Psychological interventions for individuals with cystic fibrosis and their families

  • Review
  • Intervention

Authors


Abstract

Background

With increasing survival estimates for individuals with cystic fibrosis, long-term management has become an important focus. Psychological interventions are largely concerned with adherence to treatment, emotional and social adaptation and health-related quality of life. We are unaware of any relevant systematic reviews.

Objectives

To determine whether psychological interventions for people with cystic fibrosis provide significant psychosocial and physical benefits in addition to standard medical care.

Search methods

Studies were identified from two Cochrane trials registers (Cystic Fibrosis and Genetic Disorders Group; Depression, Anxiety and Neurosis Group), Ovid MEDLINE and PsychINFO; unpublished trials were located through professional networks and Listserves.

Most recent search of the Cystic Fibrosis and Genetic Disorders Group's register: 19 December 2013.

Most recent search of the Depression, Anxiety and Neurosis Group's register: 12 November 2013.

Selection criteria

Randomised controlled studies of a broad range of psychological interventions evaluating subjective and objective health outcomes, such as quality of life or pulmonary function, in individuals of all ages with cystic fibrosis and their immediate family. We were interested in psychological interventions, including psychological methods within the scope of psychotherapeutic or psychosomatic mechanism of action (e.g. cognitive behavioural, cognitive, family systems or systemic, psycho-dynamic, or other, e.g. supportive, relaxation, or biofeedback), which were aimed at improving psychological and psychosocial outcomes (e.g. quality of life, levels of stress or distress, psychopathology, etc.), adaptation to disease management and physiological outcomes.

Data collection and analysis

Three authors were involved in selecting the eligible studies and two of these authors assessed their risk of bias.

Main results

The review includes 16 studies (eight new studies included in this update) representing data from 556 participants. Studies are diverse in their design and their methods. They cover interventions with generic approaches, as well as interventions developed specifically to target disease-specific symptoms and problems in people with cystic fibrosis. These include cognitive behavioural interventions to improve adherence to nutrition or psychosocial adjustment, cognitive interventions to improve adherence or those associated with decision making in lung transplantation, a community-based support intervention and other interventions, such as self-hypnosis, respiratory muscle biofeedback, music therapy, dance and movement therapy, and a tele-medicine intervention to support patients awaiting transplantation.

A substantial proportion of outcomes relate to adherence, changes in physical status or other specific treatment concerns during the chronic phase of the disease.

There is some evidence that behavioural interventions targeting nutrition and growth in children (4 to 12 years) with cystic fibrosis are effective in the short term. Evidence was found that providing a structured decision-making tool for patients considering lung transplantation improves patients' knowledge of and expectations about the transplant, and reduces decisional conflict in the short term. One study about training in biofeedback-assisted breathing demonstrated some evidence that it improved some lung function measurements. Currently there is insufficient evidence for interventions aimed at other aspects of the disease process.

Authors' conclusions

Currently, insufficient evidence exists on psychological interventions or approaches to support people with cystic fibrosis and their caregivers, although some of the studies were promising. Due to the heterogeneity between studies, more of each type of intervention are needed to support preliminary evidence. Multicentre studies, with consequent funding implications, are needed to increase the sample size of these studies and enhance the statistical power and precision to detect important findings. In addition, multicentre studies could improve the generalisation of results by minimizing centre or therapist effects. Psychological interventions should be targeted to illness-specific symptoms or behaviours to demonstrate efficacy.

Résumé scientifique

Les interventions psychologiques auprès des patients souffrant de mucoviscidose et de leurs familles

Contexte

Avec l'augmentation des estimations de survie pour les patients atteints de mucoviscidose, la prise en charge à long terme est devenue un important objectif. Les interventions psychologiques portent, pour une bonne part, sur l'observance du traitement, l’adaptation émotionnelle et sociale et la qualité de vie liée à la santé. Nous n’avons connaissance d’aucune revue systématique pertinente.

Objectifs

Déterminer si les interventions psychologiques auprès des personnes atteintes de mucoviscidose apportent un bénéfice physique et psychosocial significatif en complément des soins médicaux standard.

Stratégie de recherche documentaire

Des études ont été identifiées dans deux registres d'essais cliniques Cochrane (groupes Mucoviscidose et autres maladies génétiques et Dépression, anxiété et névrose), Ovid MEDLINE et PsycINFO ; des essais non publiés ont été identifiés via les réseaux professionnels et listes de diffusion.

Recherche la plus récente effectuée dans le registre du groupe Cochrane sur la mucoviscidose et les autres maladies génétiques : 19 décembre 2013.

Recherche la plus récente effectuée dans le registre du groupe Cochrane sur la dépression, l'anxiété et la névrose : 12 novembre 2013.

Critères de sélection

Études contrôlées randomisées portant sur un large éventail d'interventions psychologiques évaluant des critères de santé objectifs et subjectifs tels que la qualité de vie ou la fonction pulmonaire, chez des personnes de tous âges atteintes de mucoviscidose et leur famille. Nous nous sommes intéressés aux interventions psychologiques, notamment aux méthodes ayant un cadre d’action d’ordre psychothérapeutique ou psychosomatique (par ex. cognitives, comportementales-cognitives, thérapies des systèmes familiaux ou systémiques, psychodynamiques ou autres, par ex. thérapie de soutien, relaxation ou biofeedback), visant à améliorer les résultats psychosociaux et psychologiques (par ex. la qualité de vie, les niveaux de stress ou de détresse, les psychopathologies, etc.), l’adaptation à la gestion de la maladie et les résultats physiologiques.

Recueil et analyse des données

Trois auteurs ont participé à la sélection des études éligibles et deux de ces auteurs ont évalué leur risque de biais.

Résultats principaux

La revue inclut 16 études (huit nouvelles études ont été incluses dans cette mise à jour) présentant des données pour 556 participants. Ces études sont diverses dans leur conception et leurs méthodes. Elles couvrent des interventions suivant une approche générique aussi bien que d’autres développées spécifiquement pour les symptômes de la mucoviscidose et les problèmes particuliers que rencontrent les patients. Ces interventions incluent des interventions comportementales-cognitives visant à améliorer l'acceptation des consignes de nutrition ou d'adaptation psychosociale, des interventions cognitives visant à améliorer l'observance ou associées à la prise d’une décision de transplantation pulmonaire, une intervention de soutien communautaire et d'autres interventions telles que l'autohypnose, le biofeedback des muscles respiratoires, la musicothérapie, la thérapie par la danse et le mouvement, et une intervention de télémédecine visant à soutenir les patients en attente de greffe.

Une proportion importante de critères de jugement sont liés à l'observance, aux changements de l'état physique ou à des questions spécifiques liées au traitement pendant la phase chronique de la maladie.

Il existe certaines preuves que les interventions comportementales ciblant la nutrition et la croissance des enfants (de 4 à 12 ans) atteints de mucoviscidose sont efficaces à court terme. Les preuves indiquent que la création d'un outil de prise de décision structurée pour les patients envisageant une greffe des poumons améliore les connaissances des patients et leurs attentes à propos de la greffe, et réduit le conflit décisionnel à court terme. Une étude sur l’entraînement respiratoire assisté par biofeedback a fourni quelques preuves d’une amélioration de certaines mesures de la fonction pulmonaire. Il n'existe actuellement pas suffisamment de preuves pour les interventions visant d'autres aspects de la maladie.

Conclusions des auteurs

Actuellement, les preuves sont insuffisantes sur les interventions ou approches psychologiques visant à soutenir les patients atteints de mucoviscidose et leurs soignants, bien que certaines des études soient encourageantes. En raison de l'hétérogénéité entre les études, il faut évaluer davantage chaque type d'intervention afin de confirmer les preuves préliminaires. Des études multicentriques, impliquant un financement adapté, sont nécessaires pour augmenter la taille des échantillons de ces études et améliorer leur puissance statistique et leur précision afin de détecter les résultats importants. En outre, elles pourraient améliorer la généralisation des résultats en minimisant les effets liés au centre ou au thérapeute. Pour avoir une efficacité démontrée, les interventions psychologiques doivent être dirigées vers les symptômes ou comportements spécifiques de la maladie.

Plain language summary

Psychological treatments to help individuals with cystic fibrosis and their caregivers manage the disease

Cystic fibrosis is a genetic disorder that damages many of the body's organs and can shorten a person's life span. The disease is progressive, stressful to manage, and needs complex and time-consuming treatments, leaving patients and caregivers stressed due to the challenges of the treatment. Thus, individuals with cystic fibrosis and their family members often need help to cope better and to deal with their thoughts and feelings. They also need assistance in managing the demands of the prescribed treatment schedules. In addition, infection control guidelines recommend the isolation of people with cystic fibrosis from others with the same disease, leading to a lack of peer support and potential social isolation.

We looked for studies of psychological treatments in individuals of all ages with cystic fibrosis and their families which aimed to reduce anxiety and depression, to improve adjustment, quality of life, and even medical outcomes, as well as knowledge, skills, and decisions regarding care. The review includes 16 studies with a total of 556 participants. Even though there many different psychological interventions, only a few have been evaluated for individuals with CF and their families. Due to the lack of high quality studies, it is not possible to currently show which psychological treatments are most helpful to those with cystic fibrosis and their caregivers. Five out of the 16 studies we found evaluated behavioural interventions to improve dietary intake. We found that in children aged 4 to 12 years receiving a nutritional intervention plus behavioural management training, consumed about 276 per day more than children just receiving the nutritional intervention. We also found that a structured decision-making tool for adults considering lung transplantation improved their knowledge, assisted in setting realistic expectations, and reduced indecision.

In summary, there is some evidence that behavioural interventions targeting specific illness-related symptoms and behaviours can work. More studies on psychological interventions with more people are urgently needed. There are several ongoing randomised controlled studies aimed at improving adherence to prescribed treatments, but final results are not yet available. We recommend multicentre studies to provide evidence for which interventions are most effective for the key issues faced by people with cystic fibrosis and their caregivers.

Résumé simplifié

Traitements psychologiques pour aider les patients souffrant de mucoviscidose et leurs soignants à gérer la maladie

La mucoviscidose est une maladie génétique qui porte atteinte à de nombreux organes et peut abréger l’espérance de vie. C’est une maladie évolutive, éprouvante, nécessitant des traitements longs et complexes dont la difficulté est source de tensions nerveuses pour les patients et les soignants. Par conséquent, les personnes atteintes de mucoviscidose et leurs proches ont souvent besoin d'aide pour mieux faire face et gérer leurs pensées et leurs émotions. Ils ont également besoin d’assistance pour respecter les exigences des traitements prescrits. En outre, les directives de lutte contre les infections préconisent d’isoler les personnes atteintes de mucoviscidose les unes des autres, ce qui limite l'entraide et crée un risque d'isolement social.

Nous avons recherché des études sur les traitements psychologiques destinés aux patients de tous âges atteints de mucoviscidose et à leurs familles et visant à réduire l'anxiété et la dépression, à améliorer l'ajustement, la qualité de vie et même les résultats médicaux, ainsi que les connaissances, les compétences et les décisions concernant les soins. La revue comprend 16 études totalisant 556 participants. Bien qu’il existe de nombreuses interventions psychologiques différentes, quelques-unes seulement ont été évaluées chez les personnes atteintes de mucoviscidose et leurs familles. En raison du manque d'études de haute qualité, il n'est pas possible actuellement de montrer quels traitements psychologiques sont les plus utiles pour les personnes atteintes de mucoviscidose et leurs soignants. Cinq des 16 études que nous avons trouvées évaluaient des interventions comportementales destinées à améliorer la nutrition. Nous avons trouvé que les enfants âgés de 4 à 12 ans ayant reçu une intervention nutritionnelle et un entraînement à la gestion comportementale, consommaient environ 276 g par jour de plus que les enfants ayant uniquement reçu l'intervention nutritionnelle. Nous avons également trouvé qu'un outil de prise de décision structurée pour les adultes qui envisagent une greffe des poumons améliorait leurs connaissances, les aidait à définir des attentes réalistes et réduisait leur indécision.

En résumé, il existe certaines preuves que les interventions comportementales ciblant des symptômes et comportements spécifiques de la maladie peuvent fonctionner. D'autres études sur les interventions psychologiques, portant sur un plus grand nombre de personnes, sont nécessaires de toute urgence. Plusieurs études contrôlées randomisées sont en cours sur les moyens d’améliorer l'observance des traitements prescrits, mais leurs résultats finaux ne sont pas encore disponibles. Nous recommandons des études multicentriques pour obtenir des preuves sur les interventions qui sont les plus efficaces sur les principaux problèmes rencontrés par les patients souffrant de mucoviscidose et leurs soignants.

Notes de traduction

Traduit par: French Cochrane Centre 13th October, 2014
Traduction financée par: Financeurs pour le Canada : Instituts de Recherche en Santé du Canada, Ministère de la Santé et des Services Sociaux du Québec, Fonds de recherche du Québec-Santé et Institut National d'Excellence en Santé et en Services Sociaux; pour la France : Ministère en charge de la Santé

Summary of findings(Explanation)

Summary of findings for the main comparison. Cognitive behavioral interventions to improve adherence
Nutrional intervention plus behavioural management training (BEH) compared with Nutritional intervention alone (NUT)for children with CF (4-12 years)

Patient or population: Children from 4-12 years with a diagnosis of CF by sweat test, pancreatic insufficiency; and weight for age and height < 40th percentile

Settings: group setting

Intervention: Nutrional intervention plus behavioural management training

Comparison: Nutritional intervention alone

OutcomesIllustrative comparative risks* (95% CI)No of Participants
(studies)
Comments
Assumed riskCorresponding risk
Nutritional intervention aloneNutrional intervention plus behavioural management training

Change in weight

pre- and post-treatment in (kg)

The mean change in weight ranged across control groups from
0.92 to 1.75kg

The mean change in weight in the intervention groups was
0.11kg higher

(0.84 lower to 1.07 higher)

75
(2)
Primary outcome

BMIz change

range: -1.00 to 1.00

2-year follow up

The mean BMIz change in the control group was
-0.22

The mean BMIz change in the intervention group was
0.35 higher

(0 to 0.7 higher)

67
(1)
Primary outcome

Total calories per day

post-intervention

The mean total calories per day ranged across control groups from
1316 to 2315 calories
The mean total calories per day in the intervention groups was
275.8 calories higher
(66.65 to 485.05 higher)
83
(3)
Secondary outcome

Change in calorie intake

pre- and post-treatment

The mean change in calorie intake ranged across control groups from
303.9 to 489 calories
The mean change in calorie intake in the intervention groups was
364.06 calories higher
(191.99 to 536.13 higher)
82
(3)
Secondary outcome

Estimated energy requirements (%EER)

post-intervention

The mean %EER in the control group was
127%
The mean %EER in the intervention group was
21% higher
(7.76 to 34.24 higher)
67
(1)
Secondary outcome

Change in estimated energy requirements (%EER)

pre- and post-treatment

The mean of change in %EER in the control group was
27%

The mean of change in %EER in the intervention groups was
21% higher

(9.22 to 32.78 higher)

67
(1)
Secondary outcome
*The basis for the assumed risk (e.g. the median control group risk across studies) is provided in footnotes. The corresponding risk (and its 95% confidence interval) is based on the assumed risk in the comparison group and the relative effect of the intervention (and its 95% CI).
CI: Confidence interval

Summary of findings 2 Cognitive interventions associated with decision making in lung transplantation

Summary of findings 2. Cognitive interventions associated with decision making in lung transplantation
Decision aid for patients considering lung transplantation compared with usual carefor patients with advanced CF considering referral for lung transplantation

Patient or population: patients with advanced CF considering referral for lung transplantation

Settings: individual

Intervention: decision aid for patients considering lung transplantation

Comparison: usual care

OutcomesIllustrative comparative risks* (95% CI)No of Participants
(studies)
Comments
Assumed riskCorresponding risk
Usual careDecision aid for patients considering lung transplantation

Participants' knowledge

4-item knowledge questionnaire (range 0 to 4)

3 week follow-up

The mean score of participants' knowledge in the control group was
1.974
The mean score of participants' knowledge in the intervention group was
0.98 higher
(0.66 to 1.31 higher)
149
(1)
Secondary outcome

Participants' knowledge - change in knowledge

4-item knowledge questionnaire (range 0 to 4)

week 3 - baseline

The mean of change in participants' knowledge in the control group was

0.3

The mean of change in participants' knowledge in the intervention group was
0.94 higher
(0.53 to 1.35 higher)
149
(1)
Secondary outcome

Patient expectations

2-item questionnaire (range 0 to 2)

3 week follow-up

The mean of the expectation score in the control group was
0.58
The mean of the expectation score in the intervention group was
0.73 higher
(0.51 to 0.95 higher)
149
(1)
Secondary outcome

Patient expectations - change in expectation score

2-item questionnaire (range 0 to 2)

week 3 - baseline

The mean change in the expectation score in the control group was
0.05
The mean change in the expectation score in the intervention group was
0.66 higher
(0.37 to 0.95 higher)
149
(1)
Secondary outcome

Decisional conflict - Total score

range 0 to 100 (low decisional conflict to high decisional conflict)

3 week follow-up

The mean of the total score

in the control group was
20.4

The mean of the total score in the intervention group was
8.8 lower
(13.7 to 3.9 lower)
149
(1)
Secondary outcome

Decisional conflict - Change in total score

range 0 to 100 (low decisional conflict to high decisional conflict)

week 3 - baseline

The mean of changes in the total score

of the decisional conflict questionnaire in the control group was
13.1

The mean of changes in the total score

of the decisional conflict questionnaire

in the intervention group was
3.3 higher
(2.28 lower to 8.88 higher)

149
(1)
Secondary outcome
*The basis for the assumed risk (e.g. the median control group risk across studies) is provided in footnotes. The corresponding risk (and its 95% confidence interval) is based on the assumed risk in the comparison group and the relative effect of the intervention (and its 95% CI).
CI: Confidence interval

Summary of findings 3 Other interventions - Biofeedback

Summary of findings 3. Other interventions - Biofeedback
Biofeedback assisted breathing re-training (BRT) compared with biofeedback assisted relaxation training (RLXT)for individuals with CF (10 - 41 years)

Patient or population: individuals with CF (10 - 41 years)

Settings: individual

Intervention: biofeedback assisted breathing re-training (BRT)

Comparison: biofeedback assisted relaxation training (RLXT)

OutcomesIllustrative comparative risks* (95% CI)No of Participants
(studies)
Comments
Assumed riskCorresponding risk
Biofeedback assisted relaxation training (RLXT)Biofeedback assisted breathing re-training (BRT)
Pulmonary function - Forced expiratory volume (FEV1) in litres per secondThe mean FEV1 in the control group was 0.78The mean FEV1 in the intervention group was
0.54 higher
(0.15 to 0.93 higher)
24
(1)
Primary outcome
Pulmonary function - Forced vital capacity (FVC) in litresThe mean FVC in the control group was
2.31

The mean FVC in the intervention group was
0.87 higher

(0.09 lower to 1.83 higher)

26
(1)
Primary outcome
Pulmonary function - FEF25-75% in litres per secondThe mean FEF25-75% in the control group was
1.39

The mean FEF25-75% in the intervention group was
0.67 higher

(0.1 to 1.24 higher)

26
(1)
Primary outcome
*The basis for the assumed risk (e.g. the median control group risk across studies) is provided in footnotes. The corresponding risk (and its 95% confidence interval) is based on the assumed risk in the comparison group and the relative effect of the intervention (and its 95% CI).
CI: Confidence interval

Summary of findings 4 Other interventions - Massage therapy

Summary of findings 4. Other interventions - Massage therapy
Massage therapycompared with bedtime reading controlfor children and adolescents with CF (5 - 18 years)

Patient or population: children and adolescents with CF (5 - 18 years)

Settings: individual

Intervention: massage therapy

Comparison: bedtime reading control

OutcomesIllustrative comparative risks* (95% CI)No of Participants
(studies)
Comments
Assumed riskCorresponding risk
Bedtime reading controlMassage therapy

Pulmonary function - Peak Air Flow (PEFR)

Follow-up day 30

The mean Peak Air Flow in the control group was
244
The mean Peak Air Flow in the intervention group was
53.9 higher
(43.27 lower to 151.07 higher)
20
(1)
Primary outcome

Parent Anxiety

State Trait Anxiety Inventory (STAI; range from 0 to 80 with a higher score reflecting more anxiety)

The mean parent anxiety score in the control group was
40
The mean parent anxiety score in the intervention group was
9.1 lower
(17.84 to 0.36 lower)
20
(1)
Primary outcome

Child Anxiety -

State Trait Anxiety Inventory for Children (STAIC; range from 0 to 80 with a higher score reflecting more anxiety)

The mean child anxiety score in the control group was
32.9
The mean child anxiety score in the intervention group was
8.2 lower
(12.36 to 4.04 lower)
20
(1)
Primary outcome

Child Mood

Profile of Mood States (POMS)- depression subscale modified (19 items 5-point-scale 0 to 4, score range 0 to 76)

The mean score of the depression subscale in the control group was
7.2
The mean score of the depression subscale in the intervention group was
5.5 lower
(8.81 to 2.19 lower)
20
(1)
Primary outcome
*The basis for the assumed risk (e.g. the median control group risk across studies) is provided in footnotes. The corresponding risk (and its 95% confidence interval) is based on the assumed risk in the comparison group and the relative effect of the intervention (and its 95% CI).
CI: Confidence interval

Background

Cystic fibrosis (CF) is the most common life-limiting, hereditary disease among those of European descent; between four and five per cent of these people carry the recessive gene and 1 per 2500 live births are affected worldwide (Bobadilla 2002). The incidence of CF is rare in native African, Asian and Oriental people (Tsui 1990). The disease is manifested by generalised dysfunction of the exocrine glands, which produce excessively viscous mucus secretions. The pancreas and lungs are the main organs affected and prognosis is largely determined by the extent of the pulmonary damage. The treatment regimen for CF is complex and time-consuming, and is primarily aimed at slowing or preventing the secondary effects of the disease. Primary components of the current daily regimen include the following.

  1. Airway clearance or chest physiotherapy and postural drainage using a variety of devices (acapella, flutter, chest wall oscillation)

  2. Nebulised medications - bronchodilators, antibiotics, mucous thinning agents

  3. Antibiotics – oral, nebulised, or intravenous

  4. Replacement of pancreatic enzymes

  5. Increased calorie intake through foods or nutritional supplementation - orally, nasogastrically or through a gastrostomy tube

  6. Gene correctors for specific mutations of the CF gene

Significant increases in life span have been achieved due mainly to the availability of specialist multidisciplinary CF centres, early diagnosis via newborn screening, and treatment breakthroughs. In the USA in 1990, the mean and median ages at diagnosis were 2.9 years and 7 months, respectively (FitzSimmons 1993). The median survival age in the USA doubled between 1969 and 1990, from 14 to 28 years (FitzSimmons 1993) and those children born in the UK in 2000 have a predicted median age of survival of greater than 50 years (Dodge 2007). Data from the CF Foundation Patient Registry show that in 2010, more than 47.5 per cent of all people with CF in the USA are 18 years or older. The median predicted age of survival in 2010 was 38.3 years, and the median age at diagnosis was five months (US Patient Registry Annual Data Report 2010).

Thus, CF is no longer solely a childhood disease, and as a consequence, long-term management issues have become more relevant, with increasing emphasis placed upon adherence to the medical regimen and the transition to adulthood while living with a chronic, life-threatening condition. In addition to the profound, emotional impact of a disease that is life-shortening, a major challenge for families is to balance the demands of daily CF care with the developmental needs of the affected individual and other family members (Quittner 1992a; Quittner 1992b; Quittner 1998). The ultimate goal is to attend to the health needs of the individual without sacrificing overall quality of life of that person or their family. Thus, the major areas for concern from a psychosocial perspective include emotional and social adjustment, adherence to treatment, and health-related quality of life. These challenges must be placed within the context of the individual's age, from infancy through adolescence to adulthood, and within the context of the disease process, from initial diagnosis to death.

Although there have been descriptive overviews of psychosocial research in the area (Stark 1995; Miller 1999; Bluebond-Langner 2001) there have been no systematic reviews of intervention studies for those affected by CF until the first version of the current review was published in 2003 (Glasscoe 2003). A meta-analysis of behavioural and emotional problems and self-concept in children aged 3 to 19 years with a variety of chronic medical conditions showed that these children had an increased risk of overall adjustment problems (Lavigne 1992). Those with CF fell into a group of moderate impact, with a pooled effect size of ∂ = 0.5 in studies comparing those with CF to normative data (n = 9) and ∂ = 0.44 in studies making comparisons to controls (n = 7) (Cohen 1992). The most prevalent psychiatric diagnoses reported by Thompson in a 7 to 14 year age group with CF were anxiety (37%) and oppositional disorder (23%) (Thompson 1990), while Pearson reported anxiety (22%) and depression (42%) as most prevalent in a 16 to 40 year age group with CF (Pearson 1991). Targeted interventions aimed at addressing these symptoms of distress are a major focus for psychological interventions, utilizing a diverse range of theoretical perspectives for example with the paediatric population (Beale 2006). Although these older, cross-sectional studies do not document the development of psychological dysfunction over time, they generally demonstrate that a majority of those affected with CF adapt well, which is partially in line with more current data. Depression is one of the major mental health problems worldwide with prevalence rates estimated between 6.9% in Europe (Wittchen 2011) and 8.6% in the USA (Kessler 2012). In the context of chronic conditions depression is of high relevance, because it is known as a risk factor for poor adherence to treatment (DiMatteo 2000). Quittner reviewed studies examining rates of depression in patients with CF and reported higher rates of depression compared to healthy population (Quittner 2008). Within a large epidemiological study (TIDES study) data from a representative sample of German patients with CF (N = 670, age range 12 to 64 years) showed that about one in 10 patients reported high levels of depressive symptoms, but elevated depression was not more common than in community samples, except for those with severe disease (Goldbeck 2011b). In summary, epidemiological studies examining symptoms of depression in CF were not conclusive in the question whether they occur more frequently in individuals with CF compared to the general population. Nevertheless, the Goldbeck study revealed that elevated anxiety scores were documented in about one in five patients and that they were more prevalent in adult German patients with CF compared to a community sample (Goldbeck 2011b).

Regarding parent caregivers, a multicentre study within the TIDES study showed that anxious and depressive symptoms are more common in German parents of minors with CF (N = 650) than in a community sample of adults matched by age and gender (Besier 2011a). More than one-third (37.2%) of parents showed elevated levels of anxious symptoms, compared to 18.9% of a population sample and significantly more parents reported elevated levels of depressive symptoms (28% versus 21%). Only a minority of affected caregivers (13.6%) received any form of specialist treatment for their mental health problems (Besier 2011b). A study conducted in the USA also found elevated levels of symptoms of depression in children (29%), in mothers (35%), and in fathers (23%), whereupon adherence to airway clearance was lowest for children with symptoms of depression and a less secure child-parent relationship (Smith 2010).

Adherence to the prescribed treatment regimen is one of the most challenging aspects of managing CF and parents often feel critically responsible for ensuring that these treatments are performed on a daily basis. Studies identifying rates of non-adherence in chronic paediatric conditions report that it affects about 50% to 75% of the children and adolescents (Rapoff 2010) and about 50% of adult patients (WHO 2003). For CF, reported rates vary widely, depending on different types of treatment and attempts to measure adherence. For example rates of approximately 45% (Quittner 2000) and 64% to 74% (Modi 2006) for adherence to airway clearance in children with CF were reported. Moreover, rates for enzyme medication were reported as ranging from 27% to 46% (Modi 2006). Abbott described amounts of adherence reported in the CF literature and pointed out, that it is higher for simple treatment-related behaviours than for behaviours of more complexity (Abbott 2009). Adherence to the major pulmonary medications, including inhaled mucolytics, inhaled antibiotics, and azithromycin, is for instance generally 50% or less (Modi 2010; Eakin 2011). Dietary management is also necessary to prevent pulmonary decline. Although nutritional status improves, for example data from the 2011 German CF Registry showed that about 26% the of children and adolescents still have a BMI scoring below the 15th percentile and approximately 23.5% of adults have a BMI lower than 19 kg/m2 (Stern 2012). Hence, a substantial proportion of patients need to improve their nutritional status. Overall, there is evidence that poor adherence reduces the effectiveness of treatment, might be responsible for disease progression and ultimately might reduce survival in chronic conditions in general (DiMatteo 2002).

As psychological interventions have to be adapted to the developmental level of the patients, the broad age range of individuals with CF (from infancy to later adulthood) and the corresponding developmental tasks, a variety of intervention methods and aims are required in order to cope with the disease. Furthermore, psychological interventions should be designed according to the stage of the disease, ranging from the normal health status of infants diagnosed by newborn screening to the severe disease of patients awaiting a lung transplantation and those in the terminal stage of CF needing palliative interventions. Interventions aimed at reducing stress and improving coping may improve both psychological outcomes and disease management, including adherence to prescribed treatments and thus hopefully improving medical outcomes (Bartholomew 1991; Smith 2010). Thus, the potential of psychological interventions goes beyond a mere improvement of mental health or psychosocial functioning, once the interactions of health-related behaviour and medical outcomes are considered. Therefore, a range of psychological interventions may be needed according to the broad scope of CF-related challenges. The scope of this review is therefore not limited to a certain type of problem, intervention, or category of outcome. Rather, the literature research is oriented towards a broad range of options for psychological interventions, with a focus on sound methodological studies.

Objectives

  1. To describe the extent and quality of existing research on the efficacy of psychological interventions for individuals with CF and their families.

  2. To establish whether the effects of psychological interventions, in addition to standard multidisciplinary care, provide significant benefits for the psychological and physical well-being of individuals with CF and their families.

  3. To compare the effectiveness of different psychological interventions within major target areas, such as newborn screening, adherence to nutrition, airway clearance, and other medications, decision making in lung transplant, coping and adapting to treatment regimen, transitioning toward independence and adult care, and maintaining or improving health-related quality of life.

  4. If psychological interventions were shown to be effective in these areas, then we planned to explore whether these effects varied depending on demographic characteristics of the family, socioeconomic status, age of the individual, or stage of the disease.

Methods

Criteria for considering studies for this review

Types of studies

All randomised controlled studies, published and unpublished.

We also considered quasi-randomised controlled studies, such as those using alternation and controlled studies, if a reasonable assumption could be made that the baseline was similar in both groups.

Types of participants

Children, adolescents and adults diagnosed with CF - individuals of all ages, from any ethnic group, and during all stages of the disease process will be included.

Parents or caregivers of a child or an adult with CF.

Siblings of a child or an adult with CF.

Nuclear family of a child or an adult with CF in which these individuals were identified collectively as a family unit.  

Types of interventions

A precise definition of psychological interventions is often missing in reviews on psychological interventions for patients with chronic illnesses (e.g. cancer (Hodges 2011)). The following definition was used to identify possible interventions for the current review (modelled after the definition used by Pai for paediatric oncology (Pai 2006).

An intervention was defined as a psychological intervention and eligible for the review if it fulfilled the following:

  1. included psychological methods within the scope of psychotherapeutic or psychosomatic mechanism of action (e.g. cognitive behavioural, cognitive, family systems or systemic, psycho-dynamic, or other like supportive, relaxation, biofeedback) and were provided in structured interactions between a participant and a facilitator;

  2. was facilitated by psychologists, psychotherapists, and therapists in training or other trained professionals supervised by a clinical psychologist or therapist;

  3. targeted areas for treatment set out in the aims and objectives for this review. The main targets for psychological interventions are: genetic screening for CF, adherence to treatments (e.g. nutrition, airway clearance, or other medication), coping or adapting to prescribed treatments, decision making in lung transplantation, and transitions towards independence and adult care;

  4. was aimed at improving psychological or psychosocial outcomes (e.g. quality of life, levels of stress or distress, psychopathology etc.), adaptation to disease management or physiological outcomes (or both);

  5. was compared to either no psychological intervention (apart from standard multidisciplinary care providing psychological support) or to an alternative psychological intervention;

  6. was conducted in an individually-oriented, family-oriented or group setting.

The following broad categories were used to classify the different types of interventions.

1. Cognitive behavioral

2. Cognitive

3. Family systems or systemic

4. Psychodynamic

5. Other interventions

Within the five categories, subcategories will be used to classify the included studies. Those subcategories arise from the predetermined target areas for psychological interventions highlighted in the objectives. The main targets with implications for psychological interventions are: genetic screening for CF; adherence to treatment; nutrition; airway clearance; decision making in lung transplantation; and transition toward independence and adult care.

A review on interventions aimed exclusively at supporting adherence to treatment for CF is currently underway. Therefore, non-psychological interventions to promote adherence are not included in this review. Interventions only containing educational methods or educational approaches aimed at improving self-management were excluded in the current update because a separate Cochrane Review on ‘Self-management education for cystic fibrosis’ was published in 2011 (Savage 2011).

Types of outcome measures

The following outcome measures were of interest for the review. Due to the range of interventions and potential outcomes we classified them into broad categories according to their clinical relevance. We accepted any measure with adequate psychometric properties demonstrating reliability and validity in relation to psychological, social outcomes, or quality of life outcomes or a combination of these. All outcome data that were reported at a single time point post-intervention (or follow up) or were calculated as change scores were included.

Primary Outcomes
  1. Well-being and functioning

    1. Measures assessing health-related quality of life or psychopathology or distress and focusing on the affected child, adolescent or adult or their family or both.

    2. Assessment of psychopathology or distress (any standardized interview that identifies a psychiatric disorder with 'The Diagnostic and Statistical Manual of Mental Disorders' (DSM) or an 'International Classification of Diseases' (ICD) category in an affected child or adult with CF or any self-report measure of emotional difficulties, such as anxiety or depression, or behavioural difficulties).

  2. Pulmonary function

    1. Objective measures of lung function (in infants younger than six years of age: chest radiograph scores and in older children, adolescents and adults: forced vital capacity (FVC), forced expiratory volume at one second (FEV1), residual volume/total lung capacity (RV/TLC), 25% to 75% of forced expiratory flow (FEF25-75%) (as % predicted)).

  3. Weight and height or body mass index (BMI)

Secondary Outcomes
  1. Adherence to treatment

    1. Measures of adherence (child, adolescent, adult or parent) to the treatment regimen including: pill counts; electronic monitors; calorie and dietary intake; self-report forms; semi-structured interviews; and diary reports.

  2. Social functioning

    1. Measures of how well the child, adolescent or adult is able to participate in developmentally appropriate social activities, to access social support systems, and to develop appropriate peer relationships and social skills.

    2. Measures of academic performance deficits or achievements or employment in a child, adolescent or adult with CF.

  3. Cognitive psychological outcomes

    1. Measures of the coping strategies.

    2. Measures of decision-making strategies or conflicts.

    3. Measures of self-esteem, social competence, perceptions of their own appearance or body image, locus of control, self-efficacy or sense of coherence.

  4. Relational or family adjustment

    1. Measures of the relationship between the parents of the affected child, adolescent or adult or with their partner, of family functioning or communication and interaction patterns.

  5. Utilization and costs of health-care services

    1. Measures estimating the financial cost of the intervention in relation to its efficacy for the individual with CF or their family (or both) or costs for the health care services and utilization.

    2. Number of hospital admissions, excluding the visits in CF centres for routine assessment.

    3. Number of days hospitalised, excluding the visits in CF centres for routine assessment.

Search methods for identification of studies

Electronic searches

Search strategies developed by the Cochrane Depression, Anxiety and Neurosis Group and the Cochrane Cystic Fibrosis and Genetic Disorders Group were undertaken. Relevant studies were identified from the Cystic Fibrosis Trials Register using the terms: motivation OR behaviour OR mental health in CF OR genetic counselling.

The Cystic Fibrosis Trials Register is compiled from electronic searches of the Cochrane Central Register of Controlled Trials (updated each new issue), quarterly searches of MEDLINE, a search of Embase to 1995 and the prospective handsearching of two journal - Pediatric Pulmonology and theJournal of Cystic Fibrosis. Unpublished work was identified by searching the abstract books of three major cystic fibrosis conferences: the International Cystic Fibrosis Conference; the European Cystic Fibrosis Conference and the North American Cystic Fibrosis Conference. For full details of all searching activities for the register, please see the relevant sections of the Cystic Fibrosis andGenetic Disorders Group Module.

For the current update of the review, searches were updated in June 2012 (Appendix 1) and were carried out for all years in the Cochrane Central Register of Controlled Trials (CENTRAL), OVID MEDLINE, OVID Embase, and OVID PsycINFO.

Date of the most recent search of the Cystic Fibrosis and Genetic Disorders Group's Cystic Fibrosis Trials Register: 19 December 2013.
Date of the most recent search of the Depression, Anxiety and Neurosis Group: 12 November 2013.

In addition, the Clinical Trial Registry (www.clinicaltrials.gov) was used to identify possible ongoing studies using the following term: 'cystic fibrosis'.

Searching other resources

Efforts were made to locate unpublished findings through personal contacts with researchers active in this field and professional networks including listserves.

Data collection and analysis

Selection of studies

Three authors (LG, AF, MH), working independently in groups of two, selected the studies relevant for inclusion in the current update of the review. The authors LG and AF checked one third of the studies, AF and MH checked a further third and LG and MH checked the remaining studies. The authors used a study eligibility form devised for the selection process. If two authors could not reach a consensus, then they asked one of the other authors to decide. In the case of studies published by one of the authors, a co-author assessed this for eligibility.

For previous versions of the review, two authors (CG and AQ) independently selected the studies that were relevant for inclusion in the review using a study eligibility form devised for this purpose.

Data extraction and management

Two authors (AF and MH) independently extracted data using a data extraction form. If they could not reach a consensus, then they asked one of the remaining authors to decide.

For previous versions of the review two authors (CG and AQ) independently extracted the data to be analysed using a data extraction form.

Measures of treatment effect

For binary outcomes with sufficient data, the authors calculated a pooled estimate of the treatment effect for each outcome. A comparison was made between the odds of an outcome occurring in the group receiving the treatment of interest versus the control group (odds ratio (OR) and 95% confidence interval (CI)).

For continuous outcomes, the authors calculated a pooled estimate of the treatment effect with a mean difference (MD) with 95% CIs.

Unit of analysis issues

In the event that groups are cluster-randomised, the authors will use the inverse variance method and report results as a fixed MD (Deeks 2011). Furthermore, the authors will explore whether the study reported appropriate analyses, taking the cluster design into account. If this is not the case they will perform correct analyses, as recommended in the Cochrane Handbook for Systematic Reviews of Interventions (Deeks 2011).

In the event that a study compared more than one intervention, the authors will compare the primary active treatment to usual care (or at least biased control condition).

Deeks recommends for cross-over studies to first of all prove whether the cross-over design is appropriate for the intervention and outcomes, which need to be reversible (Deeks 2011). Secondly, to take into consideration the possibility of substantial carry-over or period effects. Different options are available for the incorporation of cross-over study data into meta-analyses different options are available ranging from conservative methods to just analysing data from the first period. The selection of an appropriate method will be based on the characteristics of the study and the reported data.

Assessment of heterogeneity

When appropriate, the authors pooled results and they used the I2 statistic to judge whether the results of different studies were inconsistent. The authors assumed the following overlapping percentage intervals of the I2 statistic for quantifying heterogeneity (Deeks 2011): 0% to 40% (might not be important); 30% to 60% (may represent moderate heterogeneity); 50% to 90% (may represent substantial heterogeneity); and 75% to 100% (considerable heterogeneity). Additionally, the authors conducted a visual inspection of the forest plot and chi2 test.

Data synthesis

We have analysed data using a random-effects model.

The authors investigated the pooling of results when the interventions were:

  • of the same type of interventions defined in the methods section 'Types of interventions';

  • were comparable in the psychological methods used, the setting, the addressed outcomes and participants;

  • being compared to an equal control condition.

The authors extracted data at post-treatment and when available, including short- and long-term follow-up data.

Subgroup analysis and investigation of heterogeneity

If the authors identify heterogeneity of pooled results, they will use subgroup analyses to explore the possible sources of heterogeneity.

They plan to group studies into conceptually similar interventions and, if sufficient data are available, they will perform subgroup analyses with respect to the following list.

  1. Socioeconomic level of the individual and family (education level, income level, occupation)

  2. Age of the affected individual with CF (infants, toddlers, and preschool children under five years of age, school-aged children ages 5 to 12 years, adolescents ages 13 to 18 years, adults 19 years+)

  3. Stage of the disease process (pre-diagnosis stage, diagnosis phase through the the first year post-diagnosis, chronic phase - intervening years between the diagnosis and terminal phase, terminal phase - when the affected child or adult's condition was such that active treatment had ceased and only palliative measures were being offered)

Sensitivity analysis

In future, if sufficient data are available, the authors will undertake a sensitivity analysis comparing the findings of studies with a high risk of bias to results when these studies are excluded. Due to the small number of studies currently included and the different types, interventions and outcome measures, a sensitivity analysis was not feasible.

Results

Description of studies

Results of the search

Results of the search of previous versions of the review

Originally, a total of 70 RCTs (99 reports) were reviewed; 28 studies (50 reports) were relevant. In a previous version of the review, 13 RCTs (25 reports) were included (Delk 1994; Stark 1996; Cheuvront 1998; Trapp 1998; Hernandez-Reif 1999; Grasso 2000; Powers 2003; Stapleton 2001; Chernoff 2002; Davis 2002; Stark 2003; Powers 2005; Downs 2006) and five studies were classified as ongoing (Kalnins 1996; Quittner 2000; Quinn 2004; Watson 2004; Watson 2006). Two of the ongoing studies were concerned with adherence (Quittner 2000; Quinn 2004). Ten studies were classified as 'awaiting information from authors' (Williams 1987; Klig 1989; Davis 1990; Petzel 1991; Mischler 1998; Stark 1998; Cannon 1999; Christian 2006; Bryon 2000; Powers 2003a). A total of 42 studies (49 reports) were not eligible for inclusion in the review.

Results of the new search for the current update

For the current update of the review a total of 1705 titles and abstracts (1365 without duplicates) were identified. A total of 33 new studies (55 reports) were identified for reviewing as potentially relevant for inclusion. Three of these are still ongoing (Quittner 2011; Quittner 2012; Riekert 2012). We are awaiting further information from authors for 12 studies (14 reports) (Wainwright 2009; Huang 2010; Jessup 2010; Patel 2010; Cummings 2011; Hatziagorou 2011;Ruddy 2011; Irons 2012; Hass 2012; Goldbeck 2013; Powers 2013; Widman 2013). Eleven studies were not eligible for inclusion (Dodd 2001; Stark 2002; Rodrigue 2005; Ralston 2008; Bingham 2010; Pop Jordanova 2010; Castellani 2011; Georgiopoulos 2011; Goldbeck 2011a; Rault 2012; Rozenfeld 2012). Seven newly identified studies (15 reports) are included in the current update of the review (Belsky 1994; Taylor 2003; Goodill 2005; Christian 2006; Wilkinson 2008; Stark 2009; Vandemheen 2009). Details of the number of reports yielded by the new search are shown in Figure 1. Where necessary, we contacted authors of the included studies for additional data to allow quantitative analysis. We have not been able to locate contact details on any of the authors concerning one study (Belsky 1994). For three studies only narrative reporting of results were possible (Belsky 1994; Goodill 2005; Wilkinson 2008).

Figure 1.

Number of reports yielded by the new search for the current update (2014)

Four of the 12 newly identified studies awaiting classification were published as abstracts (Wainwright 2009; Jessup 2010; Patel 2010; Hatziagorou 2011) and one journal article did not include any quantitative data (Cummings 2011). The remaining seven studies were identified in the latest search in December 2013 and are still awaiting classification for inclusion (Huang 2010; Ruddy 2011; Hass 2012; Irons 2012; Goldbeck 2013; Powers 2013; Widman 2013). Information available to date on the studies is presented in the 'Characteristics of studies awaiting classification' tables.

One of the previous studies assessed as ongoing is now awaiting information from authors (Kalnins 1996) and one is now included in the review (Quinn 2004). Additionally, two previously ongoing studies were not eligible for the review due to an educational approach of the intervention (Watson 2004; Watson 2006).

Report characteristics of all previous and new relevant studies are listed in an additional table (Table 1).

Table 1. Report characteristics of relevant studies
Study IDCountry of OriginLanguage of ReportType of ReportContact AuthorReplyAssessment
Belsky 1994USAEnglishJournal articleYesNoawaiting
Bingham 2010USAEnglishJournal articleNoNoexclude
Bryon 2000UKEnglishAbstractYesNoawaiting
Cannon 1999USAEnglishAbstractYesNoawaiting
Castellani 2011ItalyEnglishJournal articleNoNoexclude
Chernoff 2002USAEnglishJournal articleNoNAinclude
Christian 1999USAEnglishAbstractYesNoawaiting
Christian 2006USAEnglishJournal articleNoNoinclude
Cummings 2011AustraliaEnglishJournal articleNoNoawaiting
Davis 1990USAEnglishAbstractYesNoawaiting
Delk 1994USAEnglishJournal articleYesNoinclude
Dodd 2001IrelandEnglishAbstractYesYesexclude
Georgiopoulus 2011USAEnglishJournal articleNoNoexclude
Goodill 2005USAEnglishJournal articleYesNoawaiting
Goldbeck 2011GermanyEnglishJournal articleNoNoexclude
Grasso 2000AustraliaEnglishJournal articleYesYesinclude
Hatziagorou 2010GreeceEnglishAbstractYesYesawaiting
Hernandez-Reif 1999USAEnglishJournal articleYesYesinclude
Jessup 2010AustraliaEnglishAbstractYesYesawaiting
Jessup 2011AustraliaEnglishAbstractYesYesawaiting
Kalnins 1996USAEnglishAbstractYesNoawaiting
Klig 1989USAEnglishAbstractYesYesawaiting
Marciel 2010USAEnglish

Journal Article /

Summary of clinical trial registration

YesYesongoing
Mischler 1998USAEnglishJournal articleNoNoawaiting
Patel 2010UKEnglishAbstractYesYesexclude
Petzel 1991USAEnglishAbstractYesNoawaiting
Pop Jordanova 2010MacedoniaEnglishJournal ArticleNoNoexclude
Powers 2003USAEnglishJournal articleYesYesinclude
Powers 2005USAEnglishJournal articleNoNAinclude
Quinn 2004UKEnglishAbstractYesYesinclude
Quittner 2000USAEnglishBook chapterYesYesawaiting
Quittner 2002USAEnglishPersonal correspondenceYesYesawaiting
Quittner 2011USAEnglishAbstractYesYesongoing
Ralston 2008USAEnglishJournal articleYesNoexclude
Ranström 2000FranceEnglishJournal articleYesNoexclude
Riekert 2012USAEnglishSummary of clinical trials registrationNoNoongoing
Rodrigue 2005USAEnglishJournal ArticleNoNoexclude
Stark 1996USAEnglishJournal articleYesYesinclude
Stark 1998USAEnglishAbstractYesYesawaiting
Stark 2001USAEnglishUnpublished reportYesYesinclude
Stark 2002USAEnglishJournal ArticleNoNoexclude
Stark 2003USAEnglishJournal articleNoNAinclude
Stark 2009USAEnglishJournal articleYesYesinclude
Taylor 2003USAEnglishJournal ArticleYesYesinclude
Vandemheen 2009Australia / CanadaEnglishJournal articleYesYesawaiting
Wainwright 2009AustraliaEnglishAbstractYesYesawaiting
Wilkinson 2008UKEnglishJournal articleYesNoawaiting
Williams 1997UKEnglishAbstractYesYesawaiting
Wolter 1997AustraliaEnglishJournal articleYesYesexclude

Included studies

Sixteen RCTs and one CCT (a total of 33 reports), representing data from 556 participants, had sufficient data for inclusion in the analyses of this review (Belsky 1994; Delk 1994; Stark 1996; Hernandez-Reif 1999; Grasso 2000; Chernoff 2002; Powers 2003; Stark 2003; Taylor 2003; Quinn 2004; Goodill 2005; Powers 2005; Christian 2006; Wilkinson 2008; Stark 2009; Vandemheen 2009).

Twelve of the included studies were conducted in the USA, one in Australia (Grasso 2000), one in Australia and Canada (Vandemheen 2009), and two in the UK (Quinn 2004; Wilkinson 2008). One study was ongoing at the time of the original review and has not yet been published in full (Quinn 2004). Summary details of these studies are given in the 'Characteristics of included studies' section.

Participants were children in one study (Christian 2006), children and their caregivers in seven of the studies (Stark 1996; Hernandez-Reif 1999; Grasso 2000; Powers 2003; Stark 2003; Powers 2005; Stark 2009), children and adolescents in one study (Belsky 1994), adolescents and their families in one study (Chernoff 2002) and only adults in two of the included studies (Quinn 2004; Vandemheen 2009). In four further studies, patients with a broad age range were enrolled (Delk 1994; Taylor 2003; Goodill 2005; Wilkinson 2008).

Due to the wide scope of this review, included studies were diverse in their design and methods. Those that were relevant for inclusion sometimes involved differing combinations of interventions for various aspects of the psychological management of CF. On occasion individual studies compared two or more types of intervention with each other.

All the included studies can be considered within the different methods the interventions contained as stated in the section 'Types of interventions'. Further distinction within these groupings arises from the predetermined target areas for psychological interventions highlighted in the 'Objectives' section (newborn screening, adherence to treatment, transplantation, adapting to treatment regimen, and maintaining or improving health-related quality of life, and transitioning toward independence and adult care).

1. Cognitive behavioral
1.1 Cognitive behavioral interventions to improve adherence

There are five studies in this group (Stark 1996; Powers 2003; Stark 2003; Powers 2005; Stark 2009). These involved educational or cognitive behavioural interventions, or both, with a stronger cognitive component in the three Stark studies (Stark 1996; Stark 2003; Stark 2009). The outcomes measured were mostly physical, i.e. dietary input specifically linked to nutrition status, and oriented towards adherence.

The Stark study compared the effectiveness of a group behavioural intervention in primary school age children and their parents in improving dietary input with a wait list control (Stark 1996). The earlier study by Powers examined the effectiveness of two nutritional interventions with children younger than three years of age and their parents (Powers 2003). Behavioural management training and nutrition education for parents were compared to nutrition education alone. The primary outcome indicator was number of calories consumed per day. The more recent Powers study is more focused and systematic in its design and assessment of individualised nutritional counselling compared to behavioural parent training, with children under five years of age (Powers 2005). Another study by Stark compared the effectiveness of two group approaches to promoting dietary input in school-age children and their parents (Stark 2003). One intervention involved nutrition education and the other involved behavioural management training for parents. The primary outcome for all of these studies was the number of calories consumed per day. The latest study by Stark also compared the effectiveness of two approaches (nutrition education versus behavioural plus nutrition education) for children ages from four to twelve years and their parents, with the aim of improving caloric intake and weight (Stark 2009).

1.2 Cognitive behavioral interventions to improve psychosocial adjustment

This subcategory includes one study that assessed the effectiveness of an intervention consisting of an individually tailored intervention during a home visit and a structured group session (Christian 2006). The intervention was based on teaching problem solving and training social skills. The children aged eight to twelve years were educated and supported with the following specific problems: finding out about the CF-diagnosis, explaining CF-related differences, dealing with teasing about CF, and keeping up with peers during physical activity. The control group received treatment as usual. Outcomes were assessed to measure differences between groups on psychosocial adjustment, functional health and physiological health status.

2. Cognitive
2.1 Cognitive interventions to improve adherence

Both studies in this group utilized a cognitive approach to improve adherence or health care utilization (Taylor 2003; Quinn 2004). One study provided a written self-disclosure intervention compared to standard care and was designed to improve health-care utilization in adolescents or adults with CF (Taylor 2003). Participants were asked to write three times over a 20-minute period (once in the CF clinic, twice at home) without being concerned with spelling or syntax. The first topic related to the most distressing experience of their life with possible connections between the topic to their relationships with others (e.g. family, friends etc.) and to their past, present, or future. For the following two writing sessions, participants could change from one topic to another. The second study assessed the effectiveness of motivational interviewing provided via telephone over a three-month period (Quinn 2004). It is a non-confrontational, patient-centred counselling style for enhancing behaviour change by helping patients explore and resolve ambivalence. Comparisons of the intervention to usual treatment were made on the primary outcome of adherence to aerosolised antibiotics; lung function was also measured pre- and post-intervention.

2.2 Cognitive interventions associated with decision making in lung transplantation

There is one study in this category (Vandemheen 2009). In addition to usual counselling, patients with advanced CF were provided with an evidence-based decision aid as they considered referral for lung transplantation. Comparisons were made with usual care. Patients received a paper version of the decision aid and could also access it online using the provided password. The aim was to improve patient knowledge about options when considering lung transplantation and facilitate more realistic expectations about transplantation to decrease decisional conflict.

3. Family systems or systemic

The study included in this category assessed its effectiveness as a psychosocial intervention for parenting a child with a chronic illness (Chernoff 2002). It was designed to mobilise community-based support linking new parent or child dyads (or both) with experienced parent or professional dyads. Each experienced dyad consisted of a trained network mother and a 'child life specialist'. This experienced pair initiated home visits, telephone calls and special family events aimed at enhancing adaptation and reducing stress. The generic program was targeted to children with diabetes, sickle cell, CF or moderate to severe asthma. However, the emotional outcomes reported by caregivers were reported separately, enabling the specific effect for CF to be estimated.

4. Psychodynamic

No studies were eligible for this category.

5. Other interventions

Six studies are included within this category (Belsky 1994; Delk 1994; Hernandez-Reif 1999; Grasso 2000; Goodill 2005; Wilkinson 2008).The CCT by Belsky assessed the effects of self-hypnosis on psychological and physiological functioning in children aged from 7 to 18 years with CF (Belsky 1994). Children were taught a hypnotic technique within a two-week period. Outcomes were physiological and psychological indicators. The Delk study was an RCT to assess the effectiveness of a respiratory muscle biofeedback technique used with adolescent and adult participants (Delk 1994). It was specifically targeted at enhancing the efficacy of breathing during physiotherapy using electromyographic trapezius muscle feedback in comparison to promoting relaxation, not specifically associated with physiotherapy. The primary outcome was pulmonary function. The Hernandez-Reif RCT that assessed the effectiveness of massage therapy compared to bedtime reading, as an adjunct to physiotherapy when delivered to primary school-aged children by parents (Hernandez-Reif 1999). The primary outcome was pulmonary function. The Grasso RCT assessed the effectiveness of music therapy in enhancing the experience of physiotherapy in mothers and infants under two years of age, diagnosed with CF (Grasso 2000). The primary outcomes were ratings of emotional (child and parent enjoyment) and cognitive (parents' perception of the time it took to perform physiotherapy) variables. The Goodill RCT assessed the effectiveness of dance and movement therapy in adult hospitalised patients compared to controls (Goodill 2005). Mood state, adherence, self-care expectations and body image were targeted for improvement. The remaining RCT by Wilkinson evaluated the feasibility of a video link to support patients on the transplantation list and their families (Wilkinson 2008). On a weekly basis tele-medicine sessions were used to discuss relevant issues between the consultant and the patient. Outcomes were quality of life, symptoms of anxiety and depression, and healthcare utilisation.

A summary of the categories of outcome measures reported in studies included in this review can be viewed in 'Additional tables' (Table 2).

Table 2. Types of outcome measure used in included studies
  1. Some parts are empty for the following reason: We summarized all reported outcomes of the 16 included studies in this table and categorized them according to our determined primary and secondary outcomes.

    BMI: body mass index
    EER: estimated energy requirements
    PEFR: peak expiratory flow rate

Study IDPrimary OutcomesSecondary Outcomes
Belsky 1994

Physiological: pulmonary function - Average PEFR
Height
Weight

Well-being: child behavior, anxiety

Impact on family
Ratings of the parents' assessment of the child's illness
Locus of control and health locus of control Self-concept
Chernoff 2002Well being: depression, anxiety 
Christian 2006

Pulmonary function

Height
Weight

Perceived Illness Experience Scale
Children's Loneliness Scale

Social Support Scale for Children

Self-Perception Profile for Children

Functional Health Status

Delk 1994Pulmonary function 
Goodill 2005Well-being: mood stateBody image
Adherence
Grasso 2000 Enjoyment & perception of time
Hernandez-Reif 1999

Well-being: anxiety/depression

Pulmonary function

 
Powers 2003WeightCalorie intake
Relational
Powers 2005Weight
Height
Calorie consumption
Quinn 2004Well-being: anxiety, depression, quality of life
Pulmonary function
Adherence behaviour to aerosolised antibiotics
Stark 1996Pulmonary function
Weight and height
Activity level
Stark 2003Weight

Daily calorie intake

Quality of parent/child interaction at mealtime
CF coping skills
Adherence to CF regimen

Stark 2009

Change in weight

BMI
Pulmonary function

Caloric intake, energy (%EER)
Taylor 2003Pulmonary function
BMI
Health care utilization
Vandemheen 2009 Participants’ knowledge and realistic expectations
Decisional conflict
Wilkinson 2008Well-being: quality of life, anxiety, depressionCoping of carers
Health care utilization
Subgroups
1. Age group of the individual with CF

Three studies involved infants (Grasso 2000; Powers 2003; Powers 2005). Seven studies involved school-aged children (Belsky 1994, Stark 1996; Hernandez-Reif 1999; Chernoff 2002; Stark 2003, Christian 2006; Stark 2009); two study involved adolescents and adults (Delk 1994; Taylor 2003); and the remaining four studies involved adults only (Quinn 2004, Goodill 2005; Wilkinson 2008, Vandemheen 2009).

2. Socioeconomic status (SES)

Eight of the included studies conducted an assessment of SES for the purposes of establishing equivalent group characteristics at baseline (Stark 1996; Hernandez-Reif 1999; Chernoff 2002; Powers 2003; Stark 2003; Powers 2005; Stark 2009; Vandemheen 2009). No attempt was made to stratify samples on the basis of SES groupings.

3. Stage of CF condition

Three studies were conducted during or soon after the diagnosis of CF (Grasso 2000; Powers 2003; Powers 2005). Two studies were conducted within a period when patients were considered for lung transplantation (Wilkinson 2008; Vandemheen 2009), but no study was conducted explicitly with those individuals affected with CF during the terminal stage of their disease state (palliative care). In the remaining 14 studies, stage of disease severity was not specified, although most occurred during the chronic management period.

Details of the subgroup membership of studies in this review can be viewed in 'Additional tables' (Table 3). No subgroup analyses were conducted due to insufficient data.

Table 3. Subgroup membership of included studies
  1. Not all characteristics were reported for every study. That is the reason why some parts are left empty.

Study IDSocioeconomic LevelAgeStage of Disease
Belsky 1994 7 - 18 years 
Chernoff 2002Mothers' educational level7 - 11 yearschronic?
Christian 2006 8 – 12 yearschronic
Delk 1994 10 - 41 years 
Goodill 2005 > 17 years 
Grasso 2000 < 2 yearspost diagnosis
Hernandez-Reif 1999Hollingshead5 - 15 yearschronic?
Powers 2003Hollingshead< 3 yearspost diagnosis
Powers 2005 18 - 48 monthspost diagnosis
Quittner 2000 

9.5 to 16.5 years

 

 
Quinn 2004Employment status  
Stark 1996Holligshead, Control group - II, Intervention group III3 -10 years control, 5 - 10 years interventionchronic?
Stark 2003Hollingshead & family income4 - 12 years 
Stark 2009Mother’s and father’s education, income before taxes4 – 12 years 
Taylor 2003 > 15 years 
Vandemheen 2009highest level of education> 18 yearsconsidering transplantation 
Wilkinson 2008 21 - 41 yearson transplantation list

Excluded studies

Fifty-nine studies with 66 reports were excluded. The reasons for exclusion for each individual study are listed in the 'Characteristics of excluded studies' section.

Five previously included studies on educational interventions (Cheuvront 1998; Trapp 1998; Stapleton 2001; Davis 2002; Downs 2006) have now been excluded following the publication of a separate review in 2011 (Savage 2011).

Studies awaiting assessment

A total of 22 studies are now awaiting information from authors (Williams 1987; Klig 1989; Davis 1990; Petzel 1991; Kalnins 1996; Mischler 1998; Stark 1998; Cannon 1999; Bryon 2000; Powers 2003a; Wainwright 2009; Huang 2010; Jessup 2010; Patel 2010; Cummings 2011; Hatziagorou 2011; Ruddy 2011; Hass 2012; Irons 2012; Goldbeck 2013; Powers 2013; Widman 2013).

Ongoing studies

To date, four studies are listed as ongoing (Quittner 2000; Quittner 2011; Quittner 2012; Riekert 2012).

Risk of bias in included studies

For judging the risk of bias for the studies being included in this update the evaluation criteria proposed by Higgins in the Cochrane Handbook for Systematic Reviews of Interventions (version 5.1) (Higgins 2011b) were used.

Details for every study and reasons for judgement are described within the risk of bias tables in the section ‘Characteristics of included studies’. Only one of the included studies was judged as adequately meeting all criteria (Figure 2).

Figure 2.

Risk of bias summary: review authors' judgements about each risk of bias item for each included study.

It should be mentioned that most of the included studies were small in their sample size, which might have influenced the ability of randomisation to deliver equivalence at baseline.

Allocation (selection bias)

Sequence generation was judged to be low risk in eight of the studies (Taylor 2003; Stark 2003; Quinn 2004; Powers 2005; Christian 2006; Wilkinson 2008; Stark 2009; Vandemheen 2009), was unclear for seven studies (Delk 1994; Stark 1996; Hernandez-Reif 1999; Grasso 2000; Chernoff 2002; Powers 2003; Goodill 2005) and was judged as high for one study (Belsky 1994).

Allocation concealment was judged to be low in six of the studies (Stark 2003; Powers 2005; Christian 2006; Wilkinson 2008; Stark 2009; Vandemheen 2009), to be high in one study (Belsky 1994) and unclear in the other nine studies (Delk 1994; Stark 1996; Hernandez-Reif 1999; Grasso 2000; Chernoff 2002; Powers 2003; Taylor 2003; Quinn 2004; Goodill 2005).

Blinding (performance bias and detection bias)

Blinding participants and providers of psychological interventions is quite difficult. To keep them unaware of the received or provided treatment is almost impossible. Details on blinding especially regarding the blinding of outcome assessors were judged as high risk in five studies (Stark 1996; Hernandez-Reif 1999; Powers 2003; Stark 2003; Powers 2005), low risk in six studies (Chernoff 2002; Taylor 2003; Quinn 2004; Goodill 2005; Christian 2006; Vandemheen 2009) and unclear in five studies (Belsky 1994; Delk 1994; Grasso 2000; Wilkinson 2008; Stark 2009).

Incomplete outcome data (attrition bias)

Judgments of possible biases due to the nature, amount or handling incomplete outcome data were assessed as the following: low risk for nine studies (Delk 1994; Stark 1996; Hernandez-Reif 1999; Powers 2003; Quinn 2004; Powers 2005; Christian 2006; Stark 2009; Vandemheen 2009), high risk for three studies (Chernoff 2002; Goodill 2005; Wilkinson 2008) and unclear risk for four studies (Belsky 1994; Chernoff 2002; Delk 1994; Powers 2003; Stark 2003; Taylor 2003).

Selective reporting (reporting bias)

Assessment of selective outcome reporting revealed the following results for the studies: low risk for four studies (Stark 2003; Taylor 2003; Stark 2009; Vandemheen 2009), high risk for four studies (Belsky 1994; Chernoff 2002; Goodill 2005; Wilkinson 2008) and unclear risk for the other eight studies (Delk 1994; Stark 1996; Hernandez-Reif 1999; Grasso 2000; Powers 2003; Quinn 2004, Powers 2005; Christian 2006).

Effects of interventions

See: Summary of findings for the main comparison Cognitive behavioral interventions to improve adherence; Summary of findings 2 Cognitive interventions associated with decision making in lung transplantation; Summary of findings 3 Other interventions - Biofeedback; Summary of findings 4 Other interventions - Massage therapy

The studies included in this review were so diverse in their methodologies that the objective of pooling results became impossible. A large number of different outcome measures were used and are described below for clarity and readability. Of the outcomes defined in 'Types of outcome measures', only those reported in one or more of the included primary studies are described below. If an outcome was not described, it is not reported in any of the target groups. Within psychological interventions adverse events or harms are not usually an issue and none of the included studies reported any such information. There were insufficient data to conduct subgroup analyses with respect to age, stage of condition or SES within any data comparisons.

Please note: the comparison groups and outcome groups refer to the graphs located in Data collection and analysis. We report only those outcomes which have relevant data included in the primary reports.

A brief overview of all main findings can be seen in the summary of findings tables. The findings were classified in accordance to the types of interventions. Main findings for cognitive behavioural interventions can be found in the Summary of findings for the main comparison, for cognitive interventions in the Summary of findings 2, and for other interventions in Summary of findings 3 and Summary of findings 4.

1. Cognitive behavioral

1.1 Cognitive behavioral interventions to improve adherence
Comparision 1 - Behavioural group treatment versus wait list control

One study with eight participants is included (Stark 1996).

PRIMARY OUTCOMES

Analysis 1.1 : Pulmonary function
Subcategory 1.1.1: FEV1% predicted (absolute values)
Subcategory 1.1.2: FVC% predicted (absolute values)
Subcategory 1.1.3: FEF25-75% predicted (absolute values)
This behavioural intervention group did not show any demonstrable effect on pulmonary function as measured by three independent indicators: FEV1 % predicted, MD -11.70 (95% CI -40.35 to 16.95); FVC % predicted, MD -6.00 (95% CI -31.16 to 19.16); and FEF25-75% predicted, MD -32.90 (95% CI -98.93 to 33.13).

Analysis 1.2 : Anthropometric measures
Subcategory 1.2.1: Weight (z score)
Subcategory 1.2.2: Height (z score)
A significant effect demonstrated that a group behavioural intervention increased children's weight when scores were converted to z scores, MD 1.02 (95% CI 0.04 to 2.00) (P = 0.04). However, no significant group differences were observed in height z scores, MD 0.00 (95% CI -1.46 to 1.46).

SECONDARY OUTCOMES

Analysis 1.3 : Adherence
Subcategory 1.3.1: Activity level - resting energy expenditure (% predicted)
Subcategory 1.3.2: Activity level - resting energy expenditure (Kcal/24 hours)
We were unable to find any evidence that a behavioural group intervention had any effect on resting energy expenditure either in terms of % predicted, MD 9.87 (95% CI -11.58 to 31.32) or Kcal/24 hours, MD 148.00 (95% CI -182.61 to 478.61).

Subcategory 1.3.3: Activity level - activity points
There was no significant effect on the activity points, MD -1.00 (95% CI -9.75 to 7.75).

Subcategory 1.3.4: Change in Kcalorie intake
The data for this outcome were reported in the source paper as calories and were converted to Kcalories in order to reduce the scale and thus contain the confidence intervals on the graph.
For change in Kcalorie intake there was a significant effect in favour of the group behavioural intervention, MD 0.93 (95% CI 0.31 to 1.55) (P = 0.003).

Analysis 1.4 : Nutrition status (additional outcome)
Subcategory 1.4.1: % Body fat
No significant group differences were observed in % body fat, MD -0.65 (95% CI -4.41 to 3.11).

Comparision 2 - Nutritional intervention plus behavioural management training (BEH) versus nutritional intervention alone (NUT)

Three studies are included: one with eight participants (Powers 2003); one with seven participants (Stark 2003); and one with 67 participants (Stark 2009). The interventions involved educational and cognitive behavioural components. The three studies utilised the same design and intervention, allowing the data to be pooled.

PRIMARY OUTCOMES

Analysis 2.1 : Anthropometric change scores

Subcategory 2.1.1: Change in weight (kg)
When data from two studies were combined, no significant differences were found for change in weight, MD 0.11 (95% CI -0.84 to 1.07) (Powers 2003; Stark 2009). The result should be interpreted carefully due to the fact that the two included studies reported converse effects and the I2 statistic coefficient for pooling the two studies is relatively high (I2 = 73%).

Subcategory 2.1.2: Change in height (cm)
Data reported by Powers showed no significant differences for change in height, MD -2.03 (95% CI -4.87 to 0.81) between the two groups post-intervention (Powers 2003).

Subcategory 2.1.3: Change in weight (kg) two-year follow-up and 2.1.4: Change in height (cm) two-year follow up
Data reported by Stark showed no significant differences at two-year follow-up for change in weight, MD 0.52 (95% CI -1.34 to 2.38) and change in height, MD -0.20 (95% -1.45 to 1.05) (Stark 2009).

Subcategories 2.1.5: BMIz change post-intervention and 2.1.6: BMIz change two-year follow up
Data reported by Stark on BMIz change scores did not show a significant difference between the two compared groups, MD 0.20 (95% CI -0.02 to 0.42), but did show a difference with borderline statistical significance (P = 0.05) at two-year follow up, MD 0.35 (95% CI 0.00 to 0.70) in favour with the behavioural management intervention (Stark 2009).

Subcategory 2.1.7: HAZ change two-year follow up
Again, this outcome was only reported by Stark, the HAZ change scores (height for age z-scores) at two-year follow up did not reveal a significant difference between the groups, MD -0.01 (95% CI -0.17 to 0.15) (Stark 2009).

Analysis 2.2 % Anthropometric

Subcategories 2.2.1 and 2.2.2: % Ideal body weight and 2.2.3 and 2.2.4: Weight % for age
The final two anthropometric measures, only reported by Powers, showed a significant effect size in favour of the intervention (Powers 2003). Although these data showed that the behavioural intervention succeeded in achieving substantial increases at follow up in % ideal body weight, MD 49.49 (95% CI 23.39 to 75.59) (P = 0.00002) and weight % for age, MD 24.70 (95% CI 12.77 to 36.63) (P < 0.0001), the values at baseline for both these measures were also significantly different in the same direction, MD 50.40 (95% CI 5.28 to 95.52) (P = 0.03); MD 25.30 (95% CI 2.70 to 47.90) (P = 0.03)).

Analysis 2.3 : Anthropometric

Subcategory 2.3.1: Weight (kg) post intervention
Subcategory 2.3.2: Weight (kg) two-year follow up
Subcategory 2.3.3: Height (cm) two-year follow up
Subcategory 2.3.4: HAZ two-year follow up
Subcategory 2.3.5: BMIz post intervention
Subcategory 2.3.6: BMIz two-year follow up
One study reported on this outcome and found no differences between the two groups in either of the assessed measures at post intervention or at two-year follow up: weight post intervention, MD -0.28 (95% CI -3.84 to 3.28); weight two-year follow up, MD -1.00 (95% CI -6.26 to 4.26); height two-year follow up, MD -2.80 (95% CI -10.88 to 5.28); HAZ two-year follow up, MD -0.15 (95% CI -0.54 to 0.24); BMIz post intervention, MD -0.08 (95% CI -0.54 to 0.38); BMIz two-year follow up, MD 0.15 (95% CI -0.26 to 0.56) (Stark 2009).

Analysis 2.4 : Pulmonary function

Subcategories 2.4.1: FEV1 two-year follow up and 2.4.2: FEV1 change two-year follow up
One study measured pulmonary function at two-year follow up via FEV1, no significant differences could be observed in the FEV1 score, MD 0.00 (95% CI -13.03 to 13.03) or in the FEV1 change score, MD 5.16 (95% CI -8.49 to 18.81) (Stark 2009).

SECONDARY OUTCOMES

Analysis 2.5 : Adherence (nutrition)

Subcategory 2.5.1: % RDA (Kcal/day)
One study showed no significant difference between the comparison groups for % RDA (Kcal/day), MD 8.52 (95% CI -18.95 to 35.99) (Powers 2003).

Subcategory 2.5.2: Total calories consumed per day
Three studies reported on this outcome (Powers 2003; Stark 2003; Stark 2009). They showed a significant difference (P = 0.010) in the total number of calories consumed per day, MD 275.85 (95% CI 66.65 to 485.05) favouring behavioural management training as compared to nutrition education alone.

Subcategory 2.5.3: Change in calorie intake
Data from the three studies showed a significant difference between the comparison groups for change in calorie intake, MD 364.06 (95% CI 191.99 to 536.13) (P < 0.0001) (Powers 2003; Stark 2003; Stark 2009).

Subcategory 2.5.4: Two-year follow up caloric intake
One study showed no significant differences at two-year follow up in caloric intake, MD 112.00 (95% CI -216.56 to 440.56) (Stark 2009).

Subcategory 2.5.5: Two-year follow up change in caloric intake
Similiarly, Stark reported no significant differences between the groups could be shown in change in caloric intake scores after the two-year follow up period, MD 188.00 (95% CI -75.57 to 451.57) (Stark 2009).

Subcategory 2.5.6: Estimated energy requirements (% EER)
Stark showed a significant difference favouring the behavioural intervention in % EER, MD 21.00 (95% CI 7.76 to 34.24) (P = 0.002) (Stark 2009).

Subcategory 2.5.7: Change in % EER
Stark showed a significant difference favouring the behavioural intervention was also found in change in % EER, MD 21.00 (95% CI 9.22 to 32.78) (P = 0.0005) (Stark 2009).

Subcategory 2.5.8: Two-year follow up % EER
In % EER at two-year follow up, Stark showed no significant difference between the two groups, MD 9.00 (95% CI -5.09 to 23.09) (Stark 2009).

Subcategory 2.5.9: two-year follow-up change in % EER
Stark showed no significant difference between the groups in change in %EER at two-year follow up, MD 9.00 (95% CI -4.73 to 22.73) (Stark 2009).

Analysis 2.6 : Relational - Parent-child interaction
Subcategory 2.1.1: Nursing Satellite Assessment Training Feeding Scale (NCAST) - carer or infant total score (Sumner 1994)
This is an observational measure administered during a family meal. It has 76-items that assess a set of empirically derived and objectively observed behaviours that characterise carer-child communication and interaction during feeding. This measure is a reliable and valid observation tool with internal consistency (Chronbach alpha) ranging from 0.73 to 0.86. From the only study reporting this outcome, we were unable to find evidence that behavioural management training improves parent-child communication and interaction during feeding as assessed by the NCAST total score, MD 4.60 (95% CI -0.40 to 9.60) (Powers 2003).

Analysis 2.7 : Nutritional status

Subcategory 2.7.1: % Body fat
Powers showed no statistical difference was evidenced between the groups in % body fat, MD 0.20 (95% CI -9.43 to 9.83) (Powers 2003).

Comparision 3 - Behavioral and nutritional intervention (BEH) versus standard care control (CTL)

One study with 10 participants was included (Powers 2005).

SECONDARY OUTCOMES

Analysis 3.1 : Total energy intake per day (kcal)
There was a substantially larger intake of calories per day in the children in the intervention group post-treatment, MD 979.10 (95% CI 266.17 to 1692.03) (P = 0.007) compared with the control group (Powers 2005). The design of the study did not permit this comparison to be repeated at follow up, although calorie intake remained stable from post-treatment to three- and 12-month follow up. This represents a clinically significant finding that should be replicated with a larger study.

Analysis 3.2 : % Fat intake
The finding for fat intake was in the predicted direction although not significant statistically, MD 10.40 (95% CI -0.51 to 21.31) (Powers 2005).

1.2 Cognitive behavioral interventions to improve psychosocial adjustment
Comparision 4 - Educational problem-solving and social skills interventions versus usual care

One study with 116 participants was included for this comparison (Christian 2006).

PRIMARY OUTCOMES

Analysis 4.1 : Pulmonary function (FEV1)

Subcategories 4.1.1: Three months; 4.1.2: six months; and 4.1.3: Nine months
There were no statistically significant differences in pulmonary function (FEV1) between the two groups at any time point: three months, MD 2.02 (95% CI -4.78 to 8.82); six months, MD 3.35 (95% CI -3.28 to 9.98); nine months, MD -2.44 (95% CI -9.04 to 4.16) (Christian 2006).

Analysis 4.2:Height in cm

Subcategories 4.2.1: Three months; 4.2.2: Six months; and 4.2.3: Nine months
No statistically significant differences in height in cm between the intervention and usual care could be found at: three months, MD 1.00 (95% CI -2.23 to 4.23); at six months, MD 1.83 (95% CI -3.03 to 6.69); and at nine months, MD -0.54 (95% CI -3.92 to 2.84) (Christian 2006).

Analysis 4.3 : Weight in kg

Subcategories 4.3.1: Three months; 4.3.2: Six months; and 4.3.3: Nine months
The outcome weight in kg did not statistically significant differ at any of the three points in time between the two groups: three months, MD 0.17 (95% CI -1.98 to 2.32); six months, MD 0.27 (95% CI -2.09 to 2.63); nine months, MD -0.53 (95% CI -3.03 to 1.97) (Christian 2006).

SECONDARY OUTCOMES

Analysis 4.4 : Perceived illness experience

Subcategories 4.4.1: Three months; 4.4.2: Six months; and 4.4.3: Nine months
This outcome was measured with 'The perceived Illness Experience Scale' (Eiser 1995). Regarding the perceived illness experience (impact of chronic illness) by the participating children, no statistically significant differences between the intervention and usual care group were found at: three months, MD -4.31 (95% CI -12.69 to 4.07); six months, MD -1.09 (95% CI -9.03 to 6.85); nine months, MD - 6.60 (95% CI -13.98 to 0.78)) (Christian 2006).

Analysis 4.5 : Children's loneliness

Subcategories 4.5.1: Three months; 4.5.2: Six months; and 4.5.3: Nine months
Children's loneliness was measured with the 'Children's Loneliness Scale' (Asher 1984). No statistically significant differences between the groups at any point in time were observed at: three months, MD -0.76 (95% CI -4.26 to 2.74); six months, MD 0.39 (95% CI -2.78 to 3.56); nine months, MD -2.17 (95% CI -5.73 to 1.39) (Christian 2006).

Analysis 4.6 : Social support peers

Subcategory 4.6.1: three months, sub-category 4.6.2: six months, and sub-category 4.6.3: nine months
This outcome is measured with the subscale 'Peers' of the 'Social Support Scale for Children' (Harter 1986). No statistically significant differences were found between the two groups at: three months MD 0.75 (95% CI -0.59 to 2.09); at six months MD -0.05 (95% CI -1.13 to 1.03); and at nine months, MD -0.09 (95% CI -1.13 to 0.95) (Christian 2006).

Analysis 4.7 : Social support classmates

Subcategories 4.7.1: Three months; 4.7.2: Six months; and 4.7.3: Nine months
This outcome is measured with the subscale 'Classmates' of the 'Social Support Scale for Children' (Harter 1986). No statistically significant differences were found between the two groups at: three months, MD 0.06 (95% CI -1.59 to 1.71); at six months, MD 0.35 (95% CI -1.11 to 1.81); and at nine months, MD 1.33 (95% CI -0.20 to 2.86) (Christian 2006).

Analysis 4.8 : Self-competence – subscale 'Global self-worth'

Subcategory 4.8.1: three months, sub-category 4.8.2: six months, and sub-category 4.8.3: nine months
This outcome is measured with the subscale 'Global self-worth' of the 'Self-perception Profile for Children' (Harter 1985). No statistically significant differences were observed at any point in time: three months, MD 0.36 (95% CI -0.84 to 1.56); six months, MD 0.31 (95% CI -0.81 to 1.43); nine months, MD 0.63 (95% CI -0.54 to 1.80) (Christian 2006).

Analysis 4.9 : Self-competence - subscale 'scholastic competence'

Subcategories 4.9.1: Three months; 4.9.2: Six months; and 4.9.3: Nine months
This outcome is measured with the subscale 'Scholastic competence' of the 'Self-perception Profile for Children' (Harter 1985). This measure did not show any statistically significant effect between the groups at any point in time: three months, MD 0.28 (95% CI -1.38 to 1.94); six months, MD 0.55 (95% CI -0.93 to 2.03); nine months, MD 0.76 (95% CI -0.65 to 2.17) (Christian 2006).

Analysis 4.10 : Self-competence – subscale 'Social acceptance'

Subcategories 4.10.1: Three months; 8.10.2: Six months; and 8.10.3: Nine months
This outcome is measured with the subscale 'Social acceptance' of the 'Self-perception Profile for Children' (Harter 1985). No statistically significant difference was found at: three months, MD -0.38 (95% CI -1.85 to 1.09); six months, MD 0.24 (95% CI -1.09 to 1.57); or nine months, MD 0.52 (95% CI -0.84 to 1.88) between the intervention and usual care group (Christian 2006).

Analysis 4.11 : Self-competence – subscale 'Athletic competence'

Subcategories 4.11.1: Three months, 4.11.2: Six months; and 4.11.3: Nine months
This outcome is measured with the subscale 'Athletic competence' of the 'Self-perception Profile for Children' (Harter 1985). No statistically significant differences between the groups at any point in time were observed: three months, MD 0.59 (95% CI -0.90 to 2.08); six months, MD 0.33 (95% CI -1.09 to 1.75); nine months, MD 0.12 (95% CI -1.38 to 1.62) (Christian 2006).

Analysis 4.12 : Self-competence – subscale 'physical appearance'

Subcategories 4.12.1: Three months; 4.12.2: Six months; and 4.12.3: Nine months
This outcome is measured with the subscale 'Physical appearance' of the 'Self-perception Profile for Children' (Harter 1985). No statistically significant differences were found between the two groups at: three months, MD 0.38 (95% CI -0.94 to 1.70); at six months, MD 0.45 (95% CI -0.82 to 1.72); and at nine months, MD 0.75 (95% CI -0.49 to 1.99) (Christian 2006).

Analysis 4.13 : Self-competence – subscale ‘behavioural conduct’

Subcategories 4.13.1: Three months; 4.13.2: Six months; and 4.13.3: Nine months
This outcome is measured with the subscale 'Behavioural conduct' of the 'Self-perception Profile for Children' (Harter 1985). No statistically significant differences between the groups at any point in time were observed: three months, MD 0.62 (95% CI -0.89 to 2.13); six months, MD 0.14 (95% CI -1.35 to 1.63); nine months, MD 0.16 (95% CI -1.24 to 1.56) (Christian 2006).

Analysis 4.14 : Functional health status

Subcategory 4.14.1: three months; sub-category 4.14.2: six months, and sub-category 4.14.3: nine months
This outcome was measured with 'The Functional Disability Inventory' (Walker 1991). Functional health status was not significantly different at all three points in time between the intervention and usual care group: three months, MD -0.05 (95% CI -1.86 to 1.76); six months, MD 0.17 (95% CI -1.43 to 1.77); nine months, MD 0.73 (95% CI -0.80 to 2.26) (Christian 2006).

2. Cognitive

2.1 Cognitive interventions to improve adherence
Comparision 5 - Written self-disclosure intervention versus standard care

One study with 39 participants was included (Taylor 2003).

PRIMARY OUTCOMES

Analysis 5.1 : Subjective health status- PHQ

Subcategory 5.1.1: Depression scale three-month follow up
Subcategory 5.1.2: Anxiety scale three-month follow up
Subcategory 5.1.3: Somatic complaints three-month follow up
Subcategory 5.1.4: Stressful life events three-month follow up
Subjective health status was measured with the ‘Patient Health Questionnaire (PHQ; Spitzer 1994). On the subscales 'Anxiety', MD -0.80 (95% CI -2.28 to 0.68), 'Depression', MD -1.50 (95% CI -4.11 to 1.11), and 'Stressful life events', MD 0.50 (95% CI -2.95 to 3.95), the two groups did not differ significantly at three-months follow up. The subscale 'Somatic complaints' showed a statistically significant difference at the three-month follow up (P = 0.0007) in favour of the written self-disclosure intervention, MD -3.10 (95% CI -5.36 to -0.84) (Taylor 2003).

Analysis 5.2 : Subjective health status –SF-12

Subcategory 5.5.1: Physical health status three-month follow up
Subcategory 5.5.2: Mental health status three-month follow up
The subjective health status of the participating adolescents and adults with CF measured with the SF-12 (Ware 1993). At the three-month follow up, the two groups did not differ statistically on the 'Physical health' subscale, MD 0.50 (95% CI -5.90 to 6.90) or on the 'Mental health' subscale, MD 4.20 (95% CI -1.01 to 9.41) (Taylor 2003).

Analysis 5.3 : FEV1 three-months follow up
FEV1 as a physiological disease severity measure did not show a statistically significant difference between the intervention and standard medical care group, MD -1.90 (95% CI -14.93 to 11.13) (Taylor 2003).

Analysis 5.4 : BMI three months follow up
At the three-month follow up, BMI as a physiological disease severity measure did not differ between the two groups, MD 0.30 (95% CI -1.35 to 1.95) (Taylor 2003).

SECONDARY OUTCOMES

Analysis 5.5 : Health care utilization

Subcategory 5.5.1: Outpatient utilization three-month follow up
Subcategory 5.5.2: Inpatient utilization three-month follow up
The data for health care utilization was retrieved from patient chart review. The number of outpatient visits and inpatient hospitalisation days were determined for the three months prior to and after enrolment in the study. There were no significantly different differences regarding outpatient visits, MD -0.80 (95% CI -1.93 to 0.33) and the inpatient hospitalisation days, MD -2.80 (95% CI -8.03 to 2.43) at three-month follow up, although the authors reported a significant reduction of the number of days patients spent in hospital due to the intervention (Taylor 2003). This indicated the importance of reporting change scores.

Comparision 6 - Motivational Interviewing via telephone (three-month period) versus treatment as usual

One study with 35 participants was included (Quinn 2004).

PRIMARY OUTCOMES

Analysis 6.1 : Quality of Life

Subcategory 6.1.1: Physical functioning
Subcategory 6.1.2: Social functioning
Subcategory 6.1.3: Treatment issues
Subcategory 6.1.4: Chest symptoms
Subcategory 6.1.5: Emotional functioning
Subcategory 6.1.6: Concerns for the future
Subcategory 6.1.7: Interpersonal relationships
Subcategory 6.1.8: Body image
Subcategory 6.1.9: Career concerns
This group of outcomes includes different domains of CF-specific quality of life. These data were collected using the CFQOL[A1]  (Gee 2000). We could only find a significant effect (P = 0.03) of the 'Motivational Interviewing' intervention on the domain ‘Body image’, MD 15.17 (95% CI 1.85 to 28.49). On the domain 'Emotional functioning', no significant effect (P = 0.07) of the intervention was observed, MD 7.88 (95% CI -0.53 to 16.29). Further, no significant group differences were found in: 'Physical functioning', MD 1.75 (95% CI -6.64 to 10.14); 'Social functioning', MD 6.78 (95% CI -3.55 to 17.11);  'Treatment issues', MD -3.09 (95% CI -20.10 to 13.92); 'Chest symptoms', MD 4.39 (95% CI -5.91 to 14.69); 'Concerns for the future', MD -1.30 (95% CI -18.06 to 15.46); 'Interpersonal relationships', MD 1.92 (95% CI -10.76 to 14.60); or 'Career concerns', MD 4.63 (95% CI -12.81 to 22.07) (Quinn 2004).

Analysis 6.2 : Psychological distress

Subcategory 6.2.1: STAI-Short Form
Subcategory 6.2.2: HADS anxiety
Subcategory 6.2.3: HADS depression
Post intervention, there were no significant effects of the 'Motivational Interviewing' intervention on symptoms of anxiety measured with STAI -Short Form, MD -5.50 (95% CI -12.05 to 1.05), symptoms of anxiety measured with HADS, MD -0.65 (95% CI -2.76 to 1.46), or symptoms of depression measured with HADS, MD 0.07 (95% CI -1.64 to 1.78) (Quinn 2004).

Analysis 6.3:Lung function

Subcategory 6.3.1: FEV1
Subcategory 6.3.2: FVC
We were unable to find any evidence that a 'Motivational Interviewing' intervention via telephone had any effect on the lung function post-intervention in either: FEV1, MD 1.88 (95% CI -11.44 to 15.20); or in FVC, MD -2.07 (95% CI -14.49 to 10.35) (Quinn 2004).

SECONDARY OUTCOME

Analysis 6.4:% Adherence behaviour

There were no significant group differences observed in rate of adherence post-intervention, MD 4.32 (95% CI -26.77 to 35.41) (Quinn 2004).

2.2 Cognitive interventions associated with transplantation
Comparision 7 - Decision aid for patients considering lung transplantation versus usual care

One study with 149 participants was included (Vandemheen 2009).

SECONDARY OUTCOMES

Analysis 7.1 : Participants’ knowledge

Subcategory 7.1.1: Three-week follow up
Subcategory 7.1.2: Change in knowledge (week three to baseline)
We were able to find evidence that a decision aid for patients considering lung transplantation improves patients’ knowledge: three-week follow-up score, MD 0.98 (95% CI 0.66 to 1.31 (P < 0.00001); and change in knowledge, MD 0.94 (95% CI 0.53 to 1.35) (P < 0.0001) (Vandemheen 2009).

Analysis 7.2 : Patients' expectations

Subcategory 7.2.1: Three-week follow up
Subcategory 7.2.2: Change in expectation score (week three to baseline)
Realistic expectations scores were used to measure patients’ perceptions of the level of risk for surgery and probabilities of survival. Significant differences between the two groups were found for scores at the three-week follow up, MD 0.73 (95% CI 0.51 to 0.95) (P < 0.00001) and change scores, MD 0.66 (95% CI 0.37 to 0.95) (P < 0.0001) in favour of the decision aid intervention (Vandemheen 2009).

Analysis 7.3 : Decisional conflict

Subcategory 7.3.1: Total score three-week follow up
Subcategory 7.3.2: Subscale - certainty three-week follow up
Subcategory 7.3.3: Subscale - informed three-week follow up
Subcategory 7.3.4: Subscale - values three-week follow up
Subcategory 7.3.5: Subscale - support three-week follow up
Subcategory 7.3.6: Subscale - satisfaction with decision three-week follow up
Subcategory 7.3.7: Change in total score (week three to baseline)
This outcome was measured with the 'Decisional Conflict Scale' (O'Connor 1995). The scale includes five subscales and one total score. Scores range from 0 (low decisional conflict) to 100 (high decisional conflict). A minimal clinically important difference was established: patients scoring of 25 or less tend to make decisions.

Significant differences between both groups in favour of the decision aid intervention were found for the total score at three-week follow up, MD -8.80 (95% CI -13.70 to -3.90) (P = 0.0004); the subscale 'certainty', MD -10.00 (95% CI -18.63 to -1.37) (P = 0.02); the subscale 'informed', MD -12.70 (95% CI -17.77 to -7.63) (P < 0.00001); the subscale 'values', MD -6.90 (95% CI -13.12 to -0.68) (P = 0.03); the subscale 'support', MD -7.60 (95% CI -12.15 to -2.75) (P = 0.002) and the subscale 'satisfaction with decision', MD -7.50 (95% CI -13.42 to -1.58) (P = 0.01). In contrast, no significant differences were observed in the change in the total score from baseline to three-week follow up, MD 3.30 (95% CI -2.28 to 8.88) (P = 0.25). All means across these scales, at the three-week follow up, fell under the MID cut-off of 25 points indicating absence of relevant decisional conflict, except the subscale 'certainty' (Decision aid group (mean (SD)): 26.4 (25.9); usual care group: 36.4 (27.8)) (Vandemheen 2009).

Analysis 7.4 : Patient's stated choice three-week follow up

Subcategory 7.4.1: Referral for transplantation
Subcategory 7.4.2: Declined referral for transplantation
Subcategory 7.4.3: Unsure
For all subcategories no significant differences in the rate of patients were found: for 'Referral for transplantation', OR 0.85 (95% CI 0.43 to 1.72); for 'Declined referral for transplantation', OR 1.69 (95% CI 0.61 to 4.71); and for 'Unsure', OR 0.88 (95% CI 0.39 to 2.00) (Vandemheen 2009).

Analysis 7.5 : Durability of patient decision (12-month follow up)

The outcome also considered whether patients chose the same option as reported at three-week follow up or not. No significant difference between groups was found on durability of the patients’ decisions observed at 12-month follow up, OR 2.47 (95% CI 0.94 to 6.52) (P = 0.06) (Vandemheen 2009).

Analysis 7.6 : Preparation for decision-making three-week follow up

This outcome was measured with a questionnaire asking whether patients felt prepared to make a decision by using the provided information. The two groups differed significantly at the three-week follow up, providing evidence that the participants in the decision aid group felt better prepared to make a decision, MD 11.30 (95% CI 2.95 to 19.65) (P = 0.009) (Vandemheen 2009).

Analysis 7.8:Values congruence

The choices of patients were consistent with their values across both groups (decision aid versus usual care) (Vandemheen 2009, p.765). Patients choosing a referral for lung transplantation rated living longer as very important, OR 1.80 (CI 95% 1.02 to 3.18) (P = 0.04); avoiding hassle, stress, and worry of lung transplantation less important, OR 0.77 (95% CI 0.60 to 0.99) (P = 0.047), in comparison to those declining lung transplantation (Vandemheen 2009).

Analysis 7.8 : Acceptability

Subcategory 7.8.1: Received the right amount of information
Subcategory 7.8.2: Materials were helpful in helping to arrive at a decision
Subcategory 7.8.3: Definitely or probably recommend the materials
Both groups received material with information on lung transplantation. Regarding acceptability of this information, a significant higher number of participants reported that they received the right amount of information, OR 4.47 (95% CI 2.22 to 9.02) (P = 0.008) and would definitely or probably recommend the materials they received, OR 4.93 (95% CI 1.04 to 23.32) (P = 0.04) in the decision aid than comparison group. In contrast to the authors, we did not find a significant difference between groups in whether the material was perceived as helpful in arriving at a decision, OR 1.72 (95% CI 0.89 to 3.33) between the two groups (Vandemheen 2009).

3. Family systems or Systemic

Comparision 8 - Community-based support program versus contact telephone number

One study with 13 participants was included (Chernoff 2002).

PRIMARY OUTCOMES

Analysis 8.1 : Psychiatric Symptom Index - Anxiety subscale - mothers

Subcategory 8.1.1: Mother's anxiety - 12 months post-baseline
This is an 11-item anxiety subscale which returns a standardised score with a possible range of 0 to 100 (Ilfeld 1976). Although the reported effects for the whole group of carers of children with a chronic illness in the source article showed reduced anxiety following the intervention, when comparisons were made for the subgroup of carers with a child with CF, no significant difference was found between groups, MD -3.60 (95% CI -18.14 to 10.94) at 12-month post-baseline. This subgroup was small and unlikely to demonstrate a clear effect, however it served to illustrate the pattern of the data within the group (Chernoff 2002).

4. Psychodynamic

No comparisons to report.

5. Other interventions

Comparision 9 - Biofeedback assisted breathing re-training (BRT) versus biofeedback assisted relaxation training (RLXT)

One study with 26 participants was included (Delk 1994).

PRIMARY OUTCOME

Analysis 9.1 : Pulmonary function (the study author's primary outcome indicator)
Subcategory 9.1.1: FEV1 (litres per second) (absolute values)
Subcategory 9.1.2: FVC (litres) (absolute values)
Subcategory 9.1.3: FEF25-75% (litres per second) (absolute values)
Two of three measures of lung function were better in the intervention than control group post-study (at four weeks). These were FEV1, MD 0.54 (95% CI 0.15 to 0.93) (P = 0.007) and FEF25-75%, MD 0.67 (95% CI 0.10 to 1.24) (P = 0.02). The FVC measure was not significantly different between the groups at this time point, MD 0.87 (95% CI -0.09 to 1.83). This set of findings shows that biofeedback-assisted breathing re-training is associated with improvement in some measures of spirometric lung function in adolescents with CF. These findings have clinical significance for the combined effects of physiotherapy and biofeedback (Chernoff 2002).

Comparision 10 - Massage therapy versus bedtime reading control

One study with 20 participants was included (Hernandez-Reif 1999).

PRIMARY OUTCOMES

Analysis 10.1 : Well-being
Three measures were utilised; one was aimed at the parents' emotional state (subcategory 10.1.1) and the remaining two assessed the child's emotional state (sub-categories 10.1.2 and 10.1.3).

Subcategory 10.1.1: State Trait Anxiety Inventory for parent (STAI) (Spielberger 1970)
This inventory measures anxiety of adults as an emotional state, i.e. the anxiety that a person experiences under certain conditions, and personality trait, i.e. a relatively enduring personality characteristic that reflects the individual's propensity to respond with anxiety to a broad range of conditions. The measure has 20 items and has acceptable concurrent validity and internal consistency. This emotional outcome measure showed a significant effect 30 days after completing the intervention in favour of massage for the parent, MD -9.10 (95% CI -17.84 to -0.36) (P = 0.04) in reducing anxiety (Hernandez-Reif 1999).

Subcategory 10.1.2: State Trait Anxiety Inventory for children (STAIC) (Spielberger 1973)
This is an adaptation of the adult STAI that has already been described above (Outcome 3.1.1) for children of school age and adolescents who are below average reading level. The scale has 20 items with acceptable internal validity. This emotional outcome measure showed a significant effect 30 days after completing the intervention in favour of massage for the child, MD -8.20 (95% CI -12.36 to -4.04) (P = 0.0001) in reducing anxiety (Hernandez-Reif 1999).

Subcategory 10.1.3: Profile of Mood States (POMS) (McNair 1971) (child only)
This measure is a modification for children of the original POMS designed for adults; items that focused on depressed symptoms were simplified based on the POMS manual. Only the depression subscale was utilised, which comprised 19 adjectives describing how the child felt right now, rated on a 5-point rating scale. The measure has good face validity for measuring depressed mood in children. Significant improvements in this outcome measure were found at post-day 30 in favour of massage for overall mood state, MD -5.50 (95% CI -8.81 to -2.19) (P = 0.001) (Hernandez-Reif 1999).

In summary, these three emotional outcome measures showed significant effects in favour of: massage for the child and the parent with respect to anxiety and mood state in children, suggesting that massage reduced anxiety levels in both the parent and the child and reduced the child's depressive symptoms. Although the sample was small, the consistency of these results indicate a clinically important result, with implications for managing affective states.

Analysis 10.2 : Pulmonary function

Subcategory 10.2.1: Peak air flow (PEFR) at day 1
Subcategory 10.2.2: Peak air flow (PEFR) at day 30.
At day 1 the two groups did not differ statistically on their pulmonary function, MD 16.50 (95% CI -64.04 to 97.04). We did not find that massage therapy affected pulmonary function at day 30, MD 53.90 (95% CI -43.27 to 151.07) (Hernandez-Reif 1999).

Comparision 11 - Music therapy versus familiar music and no audiotape control

One study with 20 participants was included (Grasso 2000).

PRIMARY OUTCOMES

Analysis 11.1 : Enjoyment (the study author's primary outcome indicator)
Subcategories 11.1.1 and 11.1.2: Enjoyment - parent & child
This outcome utilised an ad hoc seven point bi-polar rating scale measuring enjoyment of the routine. These outcome measures are highly subjective and their validity has not been tested. Parents ranked their own enjoyment and their perception of their child's enjoyment of chest physiotherapy (CPT) in the comparison groups (music therapy versus familiar music tape and no audiotape) on these scales. Significant effects were found in favour of music therapy for enjoyment during CPT by both child, MD 2.05 (95% CI 0.51 to 3.59) (P = 0.009) and parent, MD 1.20 (95% CI 0.22 to 2.18) (Grasso 2000).

Analysis 11.2 : Cognitive (the study author's primary outcome indicator)
Subcategory 11.2.1: Parent perception of time
This measured the parents' perception of how long they felt it took to complete the CPT in minutes. The score was compared to the actual time the parent recorded on a diary chart. The MD had wide CIs, so we did not find any evidence of an effect here, MD -4.70 (95% CI -16.93 to 7.53) (Grasso 2000).

Comparision 12 - Self-hypnosis versus control

One study with twelve participants was included (Belsky 1994).

We were not able to perform quantitative analysis based on the data the authors provided in their report and were not able to contact the authors to provide detailed data. Therefore we report the results the authors described.

PRIMARY OUTCOMES

Analysis 12.1 : Anxiety

The authors reported the following results: No group differences were observed (U = 15, P > 0.05) for 'State Anxiety'. A significant difference between experimental and control group was found for 'Trait Anxiety' (U = 1.5, P < 0.004) with change scores reflecting decreases in 'Trait Anxiety' for the experimental group and increases for the control group (Belsky 1994).

Analysis 12.2 : Impact on 'Family Scale'

The authors reported that there were no group differences observed (Belsky 1994).

Analysis 12.3 : 'Child Behavior Checklist'

The authors reported that there were no group differences observed (Belsky 1994).

Analysis 12.4 : Average peak flow expiratory flow rate

The authors reported the following result: The mean PEFR score showed an increase of approximately 76 points from pre- to post-test in the experimental group, whereas it dropped approximately 12 points in the control group. This difference proved to be significant (U = 0, P < 0.001). The difference between the two groups was also significant at the four-month follow up (Belsky 1994).

Analysis 12.5 : Height and weight

No group differences were observed by the authors (U = 125, P > 0.05) at four month follow up (Belsky 1994).

SECONDARY OUTCOMES

Analysis 12.6 : Rating of the parents' assessment of the child's illness

No group differences were observed (U = 15, P > 0.05) (Belsky 1994).

Analysis 12.7 : 'Locus of Control'

Significant differences were found by the authors in change of 'Locus of Control' (U = 0, P = 0.001) between the experimental and the control groups: scores of the experimental group decreased, which indicates more internality (Belsky 1994).

Analysis 12.8 : 'Health Locus of Control'

Significant differences were found in change of Children’s Health Locus of Control (U = 0, P = 0.001) between the experimental and control groups. The scores of the experimental group increased, which means that children’s internal expectancies related to health improved more in the experimental group (Belsky 1994).

Analysis 12.9 : Self-concept

Self-concept scores improved significantly more in the experimental group than control groups (U = 1.5, P = 0.003) (Belsky 1994).

Comparision 13 - Home telemedicine for patients awaiting transplantation versus usual care

One study with seven participants was included here (Wilkinson 2008).

We were not able to perform quantitative analysis based on the data the authors provided in their report and did not receive a response on our query for detailed data. However, we describe the provided results of the report.

PRIMARY OUTCOMES

Analysis 13.1 : Quality of life

The quality of life was measured with the 'Cystic Fibrosis Quality of Life Questionnaire' (Gee 2000). The authors reported that the intervention group showed a significant improvement in the subjects’ perception of body image (P = 0.002) (Wilkinson 2008).

Analysis 13.2 : Anxiety

The authors reported: "There was no significant change in anxiety before and after link-up for the telemedicine group or before and after clinic attendance for either group. There was no difference in the change of anxiety from pre to post link-up for the telemedicine group or for the control group in pre and post clinic attendance" (Wilkinson 2008, p.184).

Analysis 13.3 : Depression

The authors reported: "At the end of the study there was a higher reporting of depression, which was associated with a higher reporting of anxiety, in both groups" (Wilkinson 2008, p.184).

SECONDARY OUTCOME

Analysis 13.4 : Coping of carers

Regarding the coping of the participating carers no significant differences between the two groups were found by the authors (Wilkinson 2008).

Analysis 13.5 : health care utilization

The authors reported that the two groups did not show any significant differences on the following outcomes: number of visits; general practitioner; courses of IV antibiotics; length of hospital inpatient stay; or visits either to hospital or GP (Wilkinson 2008).

Comparision 14 - Dance/Movement therapy for adults versus control

One study with 14 participants was included here (Goodill 2005).

We were not able to perform quantitative analysis based on the data the authors provided in their report and we did not receive detailed data from the authors. Therefore, we report the results the author described in the published paper (Goodill 2005).

PRIMARY OUTCOMES

Analysis 14.1 : Mood state

The mood state was measured with 'The Profile of Mood States' (POMS) (McNair 1992).The author described that the repeated measures ANOVA revealed a statistically significant effect of time group, and gender on the subscale 'confusion-bewilderment' (F (2,20) = 5.99, P = 0.007, eta2 = 0.231). Men participating in the treatment group showed the strongest effect regarding changes in the mood state of participants (Goodill 2005).

SECONDARY OUTCOMES

Analysis 14.2 : Adherence to exercise and nutrition regimen

The author reported the following results: No significant differences on exercise or nutrition post-intervention or one-month follow up (one month later). However, significant differences were found at follow up between groups on adherence to nutrition regimens (Chi2 (1, N = 23) = 4.485, P = 0.03), with lower levels of adherence in the control group (Goodill 2005).

Analysis 14.3 : Body image

The author stated that no group differences were observed (Goodill 2005).

Discussion

Summary of main results

Psychological interventions for patients with CF are heterogenous in many respects, including their target populations (caregivers, young children, adults), focus of the intervention (e.g., adherence, nutrition, depression and anxiety, improved health-related quality of life), age and stage of disease, and outcome measures. This makes it extremely difficult to compare and evaluate the efficacy of these interventions using meta-analytic techniques. In addition, the range of psychosocial challenges faced by this population, which include high treatment burden, poor adherence, risk for depression and anxiety, transition to adult care, and ultimately, declining health, increases the difficulty of accumulating intervention data in a particular area. A recent consensus conference, organized by the European Cystic Fibrosis Society, published a prioritised list of the key issues that should be addressed in research in the next decade (Bradley 2012), along with reviews and recommendations of the best outcome measures in allied health professionals and nursing research. This included next to adherence, physical activity, nutritional interventions as well as interventions for newborns. Developing and seeking funding for psychological intervention studies in high priority areas would be one way to build the evidence base that is needed.

In terms of the complex psychological issues in coping with CF and its treatment regimen, only limited data on interventions is available. The retrieved primary studies used interventions with generic approaches utilized in the context of patients with CF, e.g. providing motivational interviewing to adult patients via telephone, behavioral group interventions for children and caregivers with nutritional problems, or interventions providing a decision aid for patients considering lung transplantation.

Given the heterogeneity of the interventions and outcome measure, it is not currently possible to identify an overall effect of psychological interventions for patients with CF and their caregivers. For approximately half of the retrieved studies, some positive evidence fort he intervention was found. Most of the successful interventions were disease-specific.

Interventions with the most evidence were those that combined educational and behavioral techniques to improve dietary intake and nutritional status. The combined intervention was compared to nutrition education alone. By pooling the results of three studies, it was shown that these behavioral interventions were effective in increasing calorie intake in underweight children with CF in the short-term (Stark 2003; Powers 2005; Stark 2009). Additionally, one of the three studies showed that this intervention was also effective in increasing the proportion of estimated energy requirements of these children over a short period of time. Longer studies are needed to see if these effects are maintained over time or if 'booster sessions' are needed.

Promising interventions were reported in single studies. A combination of an educational session plus a decision-making aid (Vandemheen 2009) improved patients' knowledge of and expectations about the transplant, with decisional conflict reduce in the short-term

Furthermore, on the basis of three single studies, we found some evidence to suggest that biofeedback, massage and music therapy might be a helpful adjunct during physical therapy. Biofeedback-assisted breathing re-training (Delk 1994) compared to biofeedback assisted relaxation training over the course of eight sessions was superior in enhancing lung function, especially FEV1. Massage therapy compared to bedtime reading (Hernandez-Reif 1999) was superior to reducing symptoms of anxiety in parents and children, and symptoms of depression in children.

Overall completeness and applicability of evidence

In relation to the broad area of psychosocial challenges in CF, only a few studies with rigorous research designs were identified for this review. A number of clinically relevant psychological issues have not yet been studied. Of note, few interventions have been developed or evaluated to address parental responses to newborn screening, transitions to adult care, or palliative care. In addition, areas of high clinical relevance across stages of the disease, such as adherence, depression and anxiety, and health-related quality of life are underrepresented or currently absent from the literature.

For example, despite published national data indicating that caregivers and other members of the family are at high risk for psychological symptoms due to the illness (Besier 2011a; Besier 2011b), no studies addressing these issues were available and eligible for inclusion. Moreover, no studies investigating the application of evidence-based psychotherapy approaches to prevent or reduce comorbid psychological distress were found. In sum, although recent evidence has demonstrated that there are significant psychological needs to be met for both caregivers and patients, the evidence on psychological interventions applied to this population is highly incomplete.

Interventions were delivered in a variety of modes, including individual, group, face-to-face, and telephone or Internet-based communication. Given recent infection control guidelines (Kerem 2005; Miroballi 2012), group-based psychological interventions for patients are not recommended. More research is needed on the efficacy and effectiveness of psychological interventions delivered via the Internet using Skype or other video-conferencing software.

Importantly, patients and family members face a number of barriers in attempting to access psychological interventions, including:

  1. distance to the centre;

  2. insurance coverage and cost;

  3. availability of a psychologist or trained mental health provider on the CF team or in hospital; and

  4. availability of a trained provider in the patient’s local community.

Applicability of group interventions into the individual outpatient treatment setting requires availability of a number of eligible families at the same time.

Another critical issue is the availability of trained mental health providers on CF specialty teams. Data from the iCARE study suggest that CF team members need additional training in counselling, but can quickly be trained to deliver a structured, behavioral intervention with brief phone supervision. If we are hoping to integrate highly structured, manualised psychological interventions into CF care, we will have to address the need for mental health training and expertise within CF teams. Currently, there are only a few comprehensive, patient-centred CF Centers that routinely collect and display data on the psychosocial functioning of its CF cohort (e.g. Sophia Children’s Hospital, ErasmusMC, Netherlands; CF Center, Medical University Innsbruck, Austria). Implementation of evidence-based psychological treatments will require carefully trained, specialized providers to implement disease-specific, evidence-based treatments. It is possible that these interventions, aimed at improving adherence for example, may provide costs savings in the long-run by reducing pulmonary exacerbations and therefore, inpatient hospitalisation costs.

Furthermore, the improvements of therapy and care in CF lead to the assumption that there is a contemporary era of care effect in the included studies. Hence, the validity of newer studies is estimated to be higher for today’s individuals with CF.

Footnote: It is important to note that this review did not include data on primarily educational interventions because they were included in a separate review (Savage 2011). However, since many psychosocial interventions combine education with other strategies (e.g., problem-solving, MI) it is somewhat artificial to draw the line between purely educational versus psychological interventions.

Quality of the evidence

The evidence presented in this review, which is based on data from only a few very heterogenous studies (16), involving a total of 556 participants, is limited by the quality of the available research. One major challenge in conducting high quality research in CF, which is a rare disease, is having large enough samples. In this review, more than half of the included studies had small sample sizes given their aims and only a few studies conducted a formal power calculation to ensure that they had the necessary sample to detect statistically significant differences. Due to the small number of studies and limited number of participants we were not able to explore whether the effect varied by type of intervention, socioeconomic status, age of the individual, or stage of the disease. Given that CF is a rare disease, larger samples with more diverse patients will require implementation and analysis of multicentre studies. This increases the complexity of obtaining funding and maintaining standardization of procedures and methods, but is likely a critical step in evaluating the efficacy of psychological interventions. Several multi-site studies are currently in process (e.g. Quittner 2011; Quittner 2012) and will likely report their results in the next two years.

The assessment of risk of bias indicated that the quality of many of the earlier intervention studies, in terms of study design and methods, fell far short of typical requirements. Several important studies were excluded from previous versions of the review, because participants were not randomised (e.g. Bartholomew 1997; Hains 1997; Johnson 2001).

We would also like to acknowledge that the quality of RCTs is improving and that most of the newly included studies paid more rigorous attention to the design, methods and reporting standards for RCTs than those in the past. Missing information on important details of the study design and procedures was associated with unclear risk of bias. Although most authors reported some information about the randomisation procedure, few provided enough detailed information to determine whether allocation concealment occurred. Although it is generally not feasible to blind therapists to the type of psychological intervention being delivered or participants from whether they are in the treatment or control arm of the study, it is always possible to blind assessors; however, this aspect of blinding was only reported in a few studies. It was also more difficult to detect significant differences between groups because data on changes in the outcome variables were not provided. Additionally, three studies of interest did not provide enough quantitative data to perform analysis. Another weakness in reporting is that some of the studies did not describe whether they pre-specified a primary outcome. Psychological interventions often have several endpoints, making it critical to specify which outcome variable is the primary and which are considered secondary. Another common limitation is inadequate correction for multiple tests in the analytic phase. Most of the retrieved studies, especially the newer ones, provided sufficient information about the intervention (duration, qualification of therapists etc.) or the treatment manuals were available online (e.g. Stark 2009; Vandemheen 2009). However, just four of the studies reported some actions or methods to ensure treatment fidelity (Grasso 2000; Chernoff 2002; Quinn 2004; Stark 2009).

Quality indicators should have been grouped for the purposes of a sensitivity analysis. However, we could not distinguish between levels of quality (e.g., good, fair, poor) due to the fact that most studies were single studies Replications by independent groups are missing for the majority of positive findings. Currently, the body of evidence is of insufficient quality and quantity to draw any firm conclusions.

Potential biases in the review process

We are confident to have identified all relevant studies guided by the objectives of this review. The selection of studies, the assessment of risk of bias, and data extraction were conducted independently by two authors. Potentially disagreements were discussed within the working group. A potential conflict of interest should be mentioned. The authors LG and AQ are investigators of some of the considered interventions studies for this review. Unfortunately, for three of the included studies, we were only able to describe the results because of insufficient quantitative data. However, most of the authors we contacted were extremely helpful and provided a great deal of data and information. There are several ongoing studies that should be ready to publish their results fairly soon (Quittner 2000; Quittner 2011; Quittner 2012; Riekert 2012).

Agreements and disagreements with other studies or reviews

The findings and respectively the drawn evidence for psychological interventions for patients with CF and their families are in line with other reviews focusing research on psychological interventions for chronically ill children, adolescents, and adults. In common with other Cochrane Reviews, e.g. on psychological interventions for children or adults with asthma (Yorke 2005; Yorke 2006) the evidence base is still lacking in quantity and quality.

In terms of other chronic illnesses, such as cancer in children and adolescents, more sufficient data of studies on psychological interventions are available to draw conclusions on the evidence in this particular illness. Two reviews highlight the support for the evidence of psychological interventions for caregivers and siblings in some of the relevant outcomes (Pai 2006; Prchal 2009). In a more recent systematic review (Sansom-Daly 2012), the most positive results were found for skills-based, multiple-session interventions for young adults with chronic illnesses; this is comparable to the results we found for the behavioral interventions for young children with feeding problems.

In general, most authors of reviews, regardless systematically or not, state that there is a need for greater rigor in designing, performing and reporting intervention studies for patients with a chronic illness and their families more rigorously (e.g. Plante 2001; Beale 2006; Seitz 2009; Sansom-Daly 2012).

Authors' conclusions

Implications for practice

The studies of CF-specific interventions developed especially for patients with CF, such as interventions for those considering lung transplantation or behavioral interventions for children with feeding problems are supported by the evidence to date. Providing an additional decision aid to patients considering lung transplantation was associated with improvements patient's knowledge and realistic expectations and enabled the patient to reduce decisional conflict. The structured decision aid used in this trial is available via the Internet (in English and French language). After failure of nutrition counselling alone to improve calorie intake, behavioral interventions aiming at improving intake should be considered, in the short-term, as an alternative to tube-feeding with its medical and psychosocial side effects (e.g. Oliver 2004; Van Biervliet 2004; Hazel 2006). Multi-component, behavioural interventions have shown the greatest promise in improving adherence, one of the greatest challenges faced by both patients and care teams. Current RCTs testing these strategies in CF are currently underway and should report results within the next year or two. Other interventions, such as biofeedback combined with physiotherapy are promising, although there are too few studies at this point to provide clear evidence. Therefore, we cannot currently recommend these interventions for implementation in clinical practice. Importantly, to utilize these interventions, psychologists or other members of the multidisciplinary team must be trained in their implementation. Health care providers with these skills are often not available in CF centres and thus, it may also be necessary to increase the behavioural expertise of providers or develop clinical pathways to referrals outside the CF centre.

Additionally, practitioners and clinical researchers should also be aware of other systematic reviews that have demonstrated that psychological interventions are beneficial. These include behavioural strategies to cope with procedural pain and distress (e.g., anxiety about needles, pic line insertions). These studies are not focused exclusively on patients with CF and thus, were not the focus of the current review (Uman 2013).

Implications for research

To summarise our findings: high quality efficacy and effectiveness studies of psychological interventions for CF are becoming more common, but there is an urgent need for studies with larger samples, more diverse characteristics (e.g., inclusion of minorities), replication of promising results, and better reporting of the methodological aspects of these studies. We are encouraged to report that some of the studies in this review utilized this approach and more are in the pipeline. We must also acknowledge that conducting large-scale, multicentre studies requires large funding sources, complex coordination of procedures and methods across sites, and complex multi-level modelling of both predictors and primary and secondary outcomes. To move this effort forward may require leadership from the various national CF associations in Europe and the US, and collaborative efforts by clinical researchers testing psychological interventions. The International TIDES study on comorbid depression and anxiety in patients with CF and their caregivers (see http://www.tides-cf.org/) with multiple funding in nine different countries, coordinated by one international study centre, involving multiple researchers and clinicians across the nine countries and finally in a joint effort collecting data of more than 6000 patients (Quittner 2012b) could serve as a model for effective international research collaboration and could be applied in international intervention studies with a need of larger samples.

A number of technological advances may also be helpful in overcoming the challenges of this rare disease, for example having online access for a decision aid for patients considering lung transplantation (Vandemheen 2009). The development of patient registries by individual countries (UK, Germany, US), as well as ECFS, represents an enormous technological advance that improves our ability to collect demographic and health outcome data on a large scale.  The implementation of electronic medical records (EMR), which may be accessed with patient permission, may also facilitate data collection in key areas, such as adherence. In addition, development of electronic monitoring devices that measure ingestion of medications and pulmonary function, may also improve the accuracy of data collection and enable data collection on a much larger scale. 

Our review also highlighted some important and common pitfalls of intervention research. Selection of a primary outcome is critical and if several outcomes are tested, attention should be paid to statistical adjustments for multiple tests to reduce the frequency of Type I errors. It is also important in the design phase to select reliable, well-validated measures that are consistent with the targeted outcome (i.e. adherence behavior, decisional conflict etc.) and are thus, able to demonstrate the efficacy of the psychological intervention.It should also be noted that the likelihood of finding evidence is higher for studies which target specific aspects of the treatment regimen (e.g. behavioural nutrition interventions) and pre-specified outcomes, rather than conducting non-specific interventions using more generic approaches and general outcomes.

We identified areas of need that have not been targeted to date in clinical research of psychological interventions. Key among these are:  interventions addressing at improving adherence, for patients at the end stage of the disease, interventions aimed at the psychological consequences of newborn screening/diagnosis, supportive interventions for  caregivers, families, and siblings of patients with CF, and psychotherapy interventions for co-morbid mental disorders. To deliver interventions to patients/caregivers more conveniently, researchers and clinicians should take advantage of new technologies, such as the Internet, in designing their studies. If effective, Internet-based interventions could improve access to a larger, more diverse group of patients and potentially reduce common barriers (e.g., distance to centre, need to miss school or work). Thus, psychological interventions could become more accessible, potentially less costly, and more convenient for patients.  

In terms of reporting, there is room for improvement in implementing the standards in reporting methods that are set out in the CONSORT statement (Moher 2001) and it's extension for non-pharmacological interventions (Boutron 2008), including the reporting of change scores.

Acknowledgements

We appreciate the support and guidance of the Cochrane Cystic Fibrosis and Gentic Disorders Group. We would like to thank the Neurosis, Anxiety and Depression Group from the Cochrane Collaboration for their help with the search strategy for psychological interventions.

Data and analyses

Download statistical data

Comparison 1. Behavioural group treatment versus wait list control
Outcome or subgroup titleNo. of studiesNo. of participantsStatistical methodEffect size
1 Pulmonary function1 Mean Difference (IV, Fixed, 95% CI)Totals not selected
1.1 FEV1 (% predicted)1 Mean Difference (IV, Fixed, 95% CI)0.0 [0.0, 0.0]
1.2 FVC (% predicted)1 Mean Difference (IV, Fixed, 95% CI)0.0 [0.0, 0.0]
1.3 FEF25-75% (% predicted)1 Mean Difference (IV, Fixed, 95% CI)0.0 [0.0, 0.0]
2 Anthropometric measures1 Mean Difference (IV, Random, 95% CI)Totals not selected
2.1 Weight (z score)1 Mean Difference (IV, Random, 95% CI)0.0 [0.0, 0.0]
2.2 Height (z score)1 Mean Difference (IV, Random, 95% CI)0.0 [0.0, 0.0]
3 Adherence1 Mean Difference (IV, Random, 95% CI)Totals not selected
3.1 Activity level - Resting energy expenditure (% predicted)1 Mean Difference (IV, Random, 95% CI)0.0 [0.0, 0.0]
3.2 Activity level -Resting energy expenditure (Kcal/24 hours)1 Mean Difference (IV, Random, 95% CI)0.0 [0.0, 0.0]
3.3 Activity level -Activity points1 Mean Difference (IV, Random, 95% CI)0.0 [0.0, 0.0]
3.4 Change in Kcalorie intake1 Mean Difference (IV, Random, 95% CI)0.0 [0.0, 0.0]
4 Nutrition status1 Mean Difference (IV, Random, 95% CI)Totals not selected
4.1 % Body Fat1 Mean Difference (IV, Random, 95% CI)0.0 [0.0, 0.0]
Analysis 1.1.

Comparison 1 Behavioural group treatment versus wait list control, Outcome 1 Pulmonary function.

Analysis 1.2.

Comparison 1 Behavioural group treatment versus wait list control, Outcome 2 Anthropometric measures.

Analysis 1.3.

Comparison 1 Behavioural group treatment versus wait list control, Outcome 3 Adherence.

Analysis 1.4.

Comparison 1 Behavioural group treatment versus wait list control, Outcome 4 Nutrition status.

Comparison 2. Nutrional intervention plus behavioural management training (BEH) versus nutritional intervention alone (NUT)
Outcome or subgroup titleNo. of studiesNo. of participantsStatistical methodEffect size
1 Anthropometric change scores2 Mean Difference (IV, Random, 95% CI)Subtotals only
1.1 Change in weight (kg)275Mean Difference (IV, Random, 95% CI)0.11 [-0.84, 1.07]
1.2 Change in height (cm)17Mean Difference (IV, Random, 95% CI)-2.03 [-4.87, 0.81]
1.3 Change in weight (kg) 2-year follow up159Mean Difference (IV, Random, 95% CI)0.52 [-1.34, 2.38]
1.4 Change in height (cm) 2-year follow up159Mean Difference (IV, Random, 95% CI)-0.20 [-1.45, 1.05]
1.5 BMIz change post intervention167Mean Difference (IV, Random, 95% CI)0.2 [-0.02, 0.42]
1.6 BMIz change 2-year follow up159Mean Difference (IV, Random, 95% CI)0.35 [0.00, 0.70]
1.7 HAZ change 2-year follow up159Mean Difference (IV, Random, 95% CI)-0.01 [-0.17, 0.15]
2 % Anthropometric1 Mean Difference (IV, Random, 95% CI)Totals not selected
2.1 % ideal body weight - baseline1 Mean Difference (IV, Random, 95% CI)0.0 [0.0, 0.0]
2.2 %Ideal body weight - follow up1 Mean Difference (IV, Random, 95% CI)0.0 [0.0, 0.0]
2.3 Weight % for age -baseline1 Mean Difference (IV, Random, 95% CI)0.0 [0.0, 0.0]
2.4 Weight % for age - follow up1 Mean Difference (IV, Random, 95% CI)0.0 [0.0, 0.0]
3 Antropometric1 Mean Difference (IV, Random, 95% CI)Totals not selected
3.1 weight (kg) post intervention1 Mean Difference (IV, Random, 95% CI)0.0 [0.0, 0.0]
3.2 weight (kg) 2-year follow up1 Mean Difference (IV, Random, 95% CI)0.0 [0.0, 0.0]
3.3 height (cm) 2-year follow up1 Mean Difference (IV, Random, 95% CI)0.0 [0.0, 0.0]
3.4 HAZ 2-year follow up1 Mean Difference (IV, Random, 95% CI)0.0 [0.0, 0.0]
3.5 BMIZ post intervention1 Mean Difference (IV, Random, 95% CI)0.0 [0.0, 0.0]
3.6 BMIZ 2-year follow up1 Mean Difference (IV, Random, 95% CI)0.0 [0.0, 0.0]
4 Pulmonary function1 Mean Difference (IV, Random, 95% CI)Totals not selected
4.1 FEV1 2-year follow up1 Mean Difference (IV, Random, 95% CI)0.0 [0.0, 0.0]
4.2 FEV1 change 2-year follow up1 Mean Difference (IV, Random, 95% CI)0.0 [0.0, 0.0]
5 Adherence (nutrition)3 Mean Difference (IV, Random, 95% CI)Subtotals only
5.1 % RDA Kcal/day18Mean Difference (IV, Random, 95% CI)8.52 [-18.95, 35.99]
5.2 Total calories per day382Mean Difference (IV, Random, 95% CI)275.85 [66.65, 485.05]
5.3 Change in calorie intake382Mean Difference (IV, Random, 95% CI)364.06 [191.99, 536.13]
5.4 2-year follow up caloric intake151Mean Difference (IV, Random, 95% CI)112.00 [-216.56, 440.56]
5.5 2-year follow up change in caloric intake151Mean Difference (IV, Random, 95% CI)188.0 [-75.57, 451.57]
5.6 Estimated energy requirements (%EER)167Mean Difference (IV, Random, 95% CI)21.0 [7.76, 34.24]
5.7 Change in %EER167Mean Difference (IV, Random, 95% CI)21.0 [9.22, 32.78]
5.8 2-year follow up %EER151Mean Difference (IV, Random, 95% CI)9.0 [-5.09, 23.09]
5.9 2-year follow up change in %EER151Mean Difference (IV, Random, 95% CI)9.0 [-4.73, 22.73]
6 Relational1 Mean Difference (IV, Random, 95% CI)Totals not selected
6.1 Nursing Child Assessment Satellite Training Feeding Scale (NCAST) - Caregiver / Infant total score1 Mean Difference (IV, Random, 95% CI)0.0 [0.0, 0.0]
7 Nutritional status1 Mean Difference (IV, Fixed, 95% CI)Totals not selected
7.1 % Body fat1 Mean Difference (IV, Fixed, 95% CI)0.0 [0.0, 0.0]
Analysis 2.1.

Comparison 2 Nutrional intervention plus behavioural management training (BEH) versus nutritional intervention alone (NUT), Outcome 1 Anthropometric change scores.

Analysis 2.2.

Comparison 2 Nutrional intervention plus behavioural management training (BEH) versus nutritional intervention alone (NUT), Outcome 2 % Anthropometric.

Analysis 2.3.

Comparison 2 Nutrional intervention plus behavioural management training (BEH) versus nutritional intervention alone (NUT), Outcome 3 Antropometric.

Analysis 2.4.

Comparison 2 Nutrional intervention plus behavioural management training (BEH) versus nutritional intervention alone (NUT), Outcome 4 Pulmonary function.

Analysis 2.5.

Comparison 2 Nutrional intervention plus behavioural management training (BEH) versus nutritional intervention alone (NUT), Outcome 5 Adherence (nutrition).

Analysis 2.6.

Comparison 2 Nutrional intervention plus behavioural management training (BEH) versus nutritional intervention alone (NUT), Outcome 6 Relational.

Analysis 2.7.

Comparison 2 Nutrional intervention plus behavioural management training (BEH) versus nutritional intervention alone (NUT), Outcome 7 Nutritional status.

Comparison 3. Behavioral and nutritional intervention (BEH) versus standard care control (CTL)
Outcome or subgroup titleNo. of studiesNo. of participantsStatistical methodEffect size
1 total energy intake per day (kcal)1 Mean Difference (IV, Random, 95% CI)Totals not selected
1.1 post-treatment1 Mean Difference (IV, Random, 95% CI)0.0 [0.0, 0.0]
2 % fat intake1 Mean Difference (IV, Random, 95% CI)Totals not selected
2.1 post-treatment1 Mean Difference (IV, Random, 95% CI)0.0 [0.0, 0.0]
Analysis 3.1.

Comparison 3 Behavioral and nutritional intervention (BEH) versus standard care control (CTL), Outcome 1 total energy intake per day (kcal).

Analysis 3.2.

Comparison 3 Behavioral and nutritional intervention (BEH) versus standard care control (CTL), Outcome 2 % fat intake.

Comparison 4. Educational problem-solving and social skills intervention versus usual care
Outcome or subgroup titleNo. of studiesNo. of participantsStatistical methodEffect size
1 Pulmonary function (FEV1)1 Mean Difference (IV, Random, 95% CI)Totals not selected
1.1 3 months1 Mean Difference (IV, Random, 95% CI)0.0 [0.0, 0.0]
1.2 6 months1 Mean Difference (IV, Random, 95% CI)0.0 [0.0, 0.0]
1.3 9 months1 Mean Difference (IV, Random, 95% CI)0.0 [0.0, 0.0]
2 Height (cm)1 Mean Difference (IV, Random, 95% CI)Totals not selected
2.1 3 months1 Mean Difference (IV, Random, 95% CI)0.0 [0.0, 0.0]
2.2 6 months1 Mean Difference (IV, Random, 95% CI)0.0 [0.0, 0.0]
2.3 9 months1 Mean Difference (IV, Random, 95% CI)0.0 [0.0, 0.0]
3 Weight in (kg)1 Mean Difference (IV, Random, 95% CI)Totals not selected
3.1 3 months1 Mean Difference (IV, Random, 95% CI)0.0 [0.0, 0.0]
3.2 6 months1 Mean Difference (IV, Random, 95% CI)0.0 [0.0, 0.0]
3.3 9 months1 Mean Difference (IV, Random, 95% CI)0.0 [0.0, 0.0]
4 Perceived illness experience1 Mean Difference (IV, Random, 95% CI)Totals not selected
4.1 3 months1 Mean Difference (IV, Random, 95% CI)0.0 [0.0, 0.0]
4.2 6 months1 Mean Difference (IV, Random, 95% CI)0.0 [0.0, 0.0]
4.3 9 months1 Mean Difference (IV, Random, 95% CI)0.0 [0.0, 0.0]
5 Children's loneliness1 Mean Difference (IV, Random, 95% CI)Totals not selected
5.1 3 months1 Mean Difference (IV, Random, 95% CI)0.0 [0.0, 0.0]
5.2 6 months1 Mean Difference (IV, Random, 95% CI)0.0 [0.0, 0.0]
5.3 9 months1 Mean Difference (IV, Random, 95% CI)0.0 [0.0, 0.0]
6 Social support - peers1 Mean Difference (IV, Random, 95% CI)Totals not selected
6.1 3 months1 Mean Difference (IV, Random, 95% CI)0.0 [0.0, 0.0]
6.2 6 months1 Mean Difference (IV, Random, 95% CI)0.0 [0.0, 0.0]
6.3 9 months1 Mean Difference (IV, Random, 95% CI)0.0 [0.0, 0.0]
7 Social support - classmates1 Mean Difference (IV, Random, 95% CI)Totals not selected
7.1 3 months1 Mean Difference (IV, Random, 95% CI)0.0 [0.0, 0.0]
7.2 6 months1 Mean Difference (IV, Random, 95% CI)0.0 [0.0, 0.0]
7.3 9 months1 Mean Difference (IV, Random, 95% CI)0.0 [0.0, 0.0]
8 Self competence - subscale 'global self-worth'1 Mean Difference (IV, Random, 95% CI)Totals not selected
8.1 3 months1 Mean Difference (IV, Random, 95% CI)0.0 [0.0, 0.0]
8.2 6 months1 Mean Difference (IV, Random, 95% CI)0.0 [0.0, 0.0]
8.3 9 months1 Mean Difference (IV, Random, 95% CI)0.0 [0.0, 0.0]
9 Self-competence - subscale 'scholastic competence'1 Mean Difference (IV, Random, 95% CI)Totals not selected
9.1 3 months1 Mean Difference (IV, Random, 95% CI)0.0 [0.0, 0.0]
9.2 6 months1 Mean Difference (IV, Random, 95% CI)0.0 [0.0, 0.0]
9.3 9 months1 Mean Difference (IV, Random, 95% CI)0.0 [0.0, 0.0]
10 Self-competence - subscale 'social acceptance'1 Mean Difference (IV, Random, 95% CI)Totals not selected
10.1 3 months1 Mean Difference (IV, Random, 95% CI)0.0 [0.0, 0.0]
10.2 6 months1 Mean Difference (IV, Random, 95% CI)0.0 [0.0, 0.0]
10.3 9 months1 Mean Difference (IV, Random, 95% CI)0.0 [0.0, 0.0]
11 Self-competence - subscale 'athletic competence'1 Mean Difference (IV, Random, 95% CI)Totals not selected
11.1 3 months1 Mean Difference (IV, Random, 95% CI)0.0 [0.0, 0.0]
11.2 6 months1 Mean Difference (IV, Random, 95% CI)0.0 [0.0, 0.0]
11.3 9 months1 Mean Difference (IV, Random, 95% CI)0.0 [0.0, 0.0]
12 Self-competence - subscale 'physical appearance'1 Mean Difference (IV, Random, 95% CI)Totals not selected
12.1 3 months1 Mean Difference (IV, Random, 95% CI)0.0 [0.0, 0.0]
12.2 6 months1 Mean Difference (IV, Random, 95% CI)0.0 [0.0, 0.0]
12.3 9 months1 Mean Difference (IV, Random, 95% CI)0.0 [0.0, 0.0]
13 Self-competence - subscale 'behavioral conduct'1 Mean Difference (IV, Random, 95% CI)Totals not selected
13.1 3 months1 Mean Difference (IV, Random, 95% CI)0.0 [0.0, 0.0]
13.2 6 months1 Mean Difference (IV, Random, 95% CI)0.0 [0.0, 0.0]
13.3 9 months1 Mean Difference (IV, Random, 95% CI)0.0 [0.0, 0.0]
14 Functional health status1 Mean Difference (IV, Random, 95% CI)Totals not selected
14.1 3 months1 Mean Difference (IV, Random, 95% CI)0.0 [0.0, 0.0]
14.2 6 months1 Mean Difference (IV, Random, 95% CI)0.0 [0.0, 0.0]
14.3 9 months1 Mean Difference (IV, Random, 95% CI)0.0 [0.0, 0.0]
Analysis 4.1.

Comparison 4 Educational problem-solving and social skills intervention versus usual care, Outcome 1 Pulmonary function (FEV1).

Analysis 4.2.

Comparison 4 Educational problem-solving and social skills intervention versus usual care, Outcome 2 Height (cm).

Analysis 4.3.

Comparison 4 Educational problem-solving and social skills intervention versus usual care, Outcome 3 Weight in (kg).

Analysis 4.4.

Comparison 4 Educational problem-solving and social skills intervention versus usual care, Outcome 4 Perceived illness experience.

Analysis 4.5.

Comparison 4 Educational problem-solving and social skills intervention versus usual care, Outcome 5 Children's loneliness.

Analysis 4.6.

Comparison 4 Educational problem-solving and social skills intervention versus usual care, Outcome 6 Social support - peers.

Analysis 4.7.

Comparison 4 Educational problem-solving and social skills intervention versus usual care, Outcome 7 Social support - classmates.

Analysis 4.8.

Comparison 4 Educational problem-solving and social skills intervention versus usual care, Outcome 8 Self competence - subscale 'global self-worth'.

Analysis 4.9.

Comparison 4 Educational problem-solving and social skills intervention versus usual care, Outcome 9 Self-competence - subscale 'scholastic competence'.

Analysis 4.10.

Comparison 4 Educational problem-solving and social skills intervention versus usual care, Outcome 10 Self-competence - subscale 'social acceptance'.

Analysis 4.11.

Comparison 4 Educational problem-solving and social skills intervention versus usual care, Outcome 11 Self-competence - subscale 'athletic competence'.

Analysis 4.12.

Comparison 4 Educational problem-solving and social skills intervention versus usual care, Outcome 12 Self-competence - subscale 'physical appearance'.

Analysis 4.13.

Comparison 4 Educational problem-solving and social skills intervention versus usual care, Outcome 13 Self-competence - subscale 'behavioral conduct'.

Analysis 4.14.

Comparison 4 Educational problem-solving and social skills intervention versus usual care, Outcome 14 Functional health status.

Comparison 5. Written self-disclosure intervention versus standard care
Outcome or subgroup titleNo. of studiesNo. of participantsStatistical methodEffect size
1 Subjective health status - PHQ1 Mean Difference (IV, Random, 95% CI)Totals not selected
1.1 PHQ- Depression scale 3-month follow up1 Mean Difference (IV, Random, 95% CI)0.0 [0.0, 0.0]
1.2 PHQ- Anxiety scale 3-month follow up1 Mean Difference (IV, Random, 95% CI)0.0 [0.0, 0.0]
1.3 PHQ- Somatic complaints 3-month follow up1 Mean Difference (IV, Random, 95% CI)0.0 [0.0, 0.0]
1.4 PHQ- Stressfull life events 3-month follow up1 Mean Difference (IV, Random, 95% CI)0.0 [0.0, 0.0]
2 Subjective health status - SF-121 Mean Difference (IV, Random, 95% CI)Totals not selected
2.1 Physical health status 3 month follow-up1 Mean Difference (IV, Random, 95% CI)0.0 [0.0, 0.0]
2.2 Mental health status 3 month follow-up1 Mean Difference (IV, Random, 95% CI)0.0 [0.0, 0.0]
3 FEV1 3-month follow up1 Mean Difference (IV, Random, 95% CI)Subtotals only
4 BMI 3-month follow up1 Mean Difference (IV, Random, 95% CI)Totals not selected
5 Health care utilization1 Mean Difference (IV, Random, 95% CI)Totals not selected
5.1 Outpatient utilization 3-month follow up1 Mean Difference (IV, Random, 95% CI)0.0 [0.0, 0.0]
5.2 Inpatient utilization 3-month follow up1 Mean Difference (IV, Random, 95% CI)0.0 [0.0, 0.0]
Analysis 5.1.

Comparison 5 Written self-disclosure intervention versus standard care, Outcome 1 Subjective health status - PHQ.

Analysis 5.2.

Comparison 5 Written self-disclosure intervention versus standard care, Outcome 2 Subjective health status - SF-12.

Analysis 5.3.

Comparison 5 Written self-disclosure intervention versus standard care, Outcome 3 FEV1 3-month follow up.

Analysis 5.4.

Comparison 5 Written self-disclosure intervention versus standard care, Outcome 4 BMI 3-month follow up.

Analysis 5.5.

Comparison 5 Written self-disclosure intervention versus standard care, Outcome 5 Health care utilization.

Comparison 6. Motivational Interviewing via telephone (3-month period) versus treatment as usual
Outcome or subgroup titleNo. of studiesNo. of participantsStatistical methodEffect size
1 Quality of life1 Mean Difference (IV, Random, 95% CI)Totals not selected
1.1 Physical functioning1 Mean Difference (IV, Random, 95% CI)0.0 [0.0, 0.0]
1.2 Social functioning1 Mean Difference (IV, Random, 95% CI)0.0 [0.0, 0.0]
1.3 Treatment issues1 Mean Difference (IV, Random, 95% CI)0.0 [0.0, 0.0]
1.4 Chest symptoms1 Mean Difference (IV, Random, 95% CI)0.0 [0.0, 0.0]
1.5 Emotional functioning1 Mean Difference (IV, Random, 95% CI)0.0 [0.0, 0.0]
1.6 Concerns for the future1 Mean Difference (IV, Random, 95% CI)0.0 [0.0, 0.0]
1.7 Interpersonal relationships1 Mean Difference (IV, Random, 95% CI)0.0 [0.0, 0.0]
1.8 Body image1 Mean Difference (IV, Random, 95% CI)0.0 [0.0, 0.0]
1.9 Career concerns1 Mean Difference (IV, Random, 95% CI)0.0 [0.0, 0.0]
2 Psychological distress1 Mean Difference (IV, Random, 95% CI)Totals not selected
2.1 STAI post intervention1 Mean Difference (IV, Random, 95% CI)0.0 [0.0, 0.0]
2.2 HADS anxiety post intervention1 Mean Difference (IV, Random, 95% CI)0.0 [0.0, 0.0]
2.3 HADS depression post intervention1 Mean Difference (IV, Random, 95% CI)0.0 [0.0, 0.0]
3 Lung function1 Mean Difference (IV, Random, 95% CI)Totals not selected
3.1 FEV1 post intervention1 Mean Difference (IV, Random, 95% CI)0.0 [0.0, 0.0]
3.2 FVC post intervention1 Mean Difference (IV, Random, 95% CI)0.0 [0.0, 0.0]
4 % adherence behavior post intervention1 Mean Difference (IV, Random, 95% CI)Totals not selected
Analysis 6.1.

Comparison 6 Motivational Interviewing via telephone (3-month period) versus treatment as usual, Outcome 1 Quality of life.

Analysis 6.2.

Comparison 6 Motivational Interviewing via telephone (3-month period) versus treatment as usual, Outcome 2 Psychological distress.

Analysis 6.3.

Comparison 6 Motivational Interviewing via telephone (3-month period) versus treatment as usual, Outcome 3 Lung function.

Analysis 6.4.

Comparison 6 Motivational Interviewing via telephone (3-month period) versus treatment as usual, Outcome 4 % adherence behavior post intervention.

Comparison 7. Decision aid for patients considering lung transplantation versus usual care
Outcome or subgroup titleNo. of studiesNo. of participantsStatistical methodEffect size
1 Participants' knowledge1 Mean Difference (IV, Random, 95% CI)Totals not selected
1.1 3 week follow-up1 Mean Difference (IV, Random, 95% CI)0.0 [0.0, 0.0]
1.2 change in knowledge (week 3 - baseline)1 Mean Difference (IV, Random, 95% CI)0.0 [0.0, 0.0]
2 Patient expectations1 Mean Difference (IV, Random, 95% CI)Totals not selected
2.1 3 week follow-up1 Mean Difference (IV, Random, 95% CI)0.0 [0.0, 0.0]
2.2 change in expectation score (week 3 - baseline)1 Mean Difference (IV, Random, 95% CI)0.0 [0.0, 0.0]
3 Decisional conflict1 Mean Difference (IV, Random, 95% CI)Totals not selected
3.1 Total score 3 week follow-up1 Mean Difference (IV, Random, 95% CI)0.0 [0.0, 0.0]
3.2 Subscale - certainty 3 week follow-up1 Mean Difference (IV, Random, 95% CI)0.0 [0.0, 0.0]
3.3 Subscale - informed 3 week follow-up1 Mean Difference (IV, Random, 95% CI)0.0 [0.0, 0.0]
3.4 Subscale - values 3 week follow-up1 Mean Difference (IV, Random, 95% CI)0.0 [0.0, 0.0]
3.5 Subscale - support 3 week follow-up1 Mean Difference (IV, Random, 95% CI)0.0 [0.0, 0.0]
3.6 Subscale - satisfaction with decision 3 week follow-up1 Mean Difference (IV, Random, 95% CI)0.0 [0.0, 0.0]
3.7 Change in total score (week 3 - baseline)1 Mean Difference (IV, Random, 95% CI)0.0 [0.0, 0.0]
4 Patient's stated choice at 3-week follow-up1 Odds Ratio (M-H, Fixed, 95% CI)Totals not selected
4.1 Referral for transplantation1 Odds Ratio (M-H, Fixed, 95% CI)0.0 [0.0, 0.0]
4.2 Declined referral for transplantation1 Odds Ratio (M-H, Fixed, 95% CI)0.0 [0.0, 0.0]
4.3 Unsure1 Odds Ratio (M-H, Fixed, 95% CI)0.0 [0.0, 0.0]
5 Durability of patient decisions (12-month follow up)1 Odds Ratio (M-H, Random, 95% CI)Totals not selected
6 Preparation for decision-making (3-week follow up)1 Mean Difference (IV, Random, 95% CI)Totals not selected
7 Values congruence1 Odds Ratio (Fixed, 95% CI)Totals not selected
7.1 Importance of living longer1 Odds Ratio (Fixed, 95% CI)0.0 [0.0, 0.0]
7.2 Importance of avoiding hassle, stress, and worry of lung transplantation1 Odds Ratio (Fixed, 95% CI)0.0 [0.0, 0.0]
8 Acceptability1 Odds Ratio (M-H, Random, 95% CI)Totals not selected
8.1 Received the right amount of information.1 Odds Ratio (M-H, Random, 95% CI)0.0 [0.0, 0.0]
8.2 Materials were helpful in helping to arrive at a decision.1 Odds Ratio (M-H, Random, 95% CI)0.0 [0.0, 0.0]
8.3 Definitely or probably recommend the materials.1 Odds Ratio (M-H, Random, 95% CI)0.0 [0.0, 0.0]
Analysis 7.1.

Comparison 7 Decision aid for patients considering lung transplantation versus usual care, Outcome 1 Participants' knowledge.

Analysis 7.2.

Comparison 7 Decision aid for patients considering lung transplantation versus usual care, Outcome 2 Patient expectations.

Analysis 7.3.

Comparison 7 Decision aid for patients considering lung transplantation versus usual care, Outcome 3 Decisional conflict.

Analysis 7.4.

Comparison 7 Decision aid for patients considering lung transplantation versus usual care, Outcome 4 Patient's stated choice at 3-week follow-up.

Analysis 7.5.

Comparison 7 Decision aid for patients considering lung transplantation versus usual care, Outcome 5 Durability of patient decisions (12-month follow up).

Analysis 7.6.

Comparison 7 Decision aid for patients considering lung transplantation versus usual care, Outcome 6 Preparation for decision-making (3-week follow up).

Analysis 7.7.

Comparison 7 Decision aid for patients considering lung transplantation versus usual care, Outcome 7 Values congruence.

Analysis 7.8.

Comparison 7 Decision aid for patients considering lung transplantation versus usual care, Outcome 8 Acceptability.

Comparison 8. Community-based support program versus contact telephone number
Outcome or subgroup titleNo. of studiesNo. of participantsStatistical methodEffect size
1 Psychiatric Symptom Index - Anxiety subscale - mothers1 Mean Difference (IV, Random, 95% CI)Totals not selected
1.1 Mothers' anxiety - baseline1 Mean Difference (IV, Random, 95% CI)0.0 [0.0, 0.0]
1.2 Mothers' anxiety - 12 months post-baseline1 Mean Difference (IV, Random, 95% CI)0.0 [0.0, 0.0]
Analysis 8.1.

Comparison 8 Community-based support program versus contact telephone number, Outcome 1 Psychiatric Symptom Index - Anxiety subscale - mothers.

Comparison 9. Biofeedback assisted breathing re-training (BRT) versus biofeedback assisted relaxation training (RLXT)
Outcome or subgroup titleNo. of studiesNo. of participantsStatistical methodEffect size
1 Pulmonary function1 Mean Difference (IV, Random, 95% CI)Totals not selected
1.1 Forced expiratory volume (FEV1) in litres per second1 Mean Difference (IV, Random, 95% CI)0.0 [0.0, 0.0]
1.2 Forced vital capacity (FVC) in litres1 Mean Difference (IV, Random, 95% CI)0.0 [0.0, 0.0]
1.3 FEF25-75% in litres per second1 Mean Difference (IV, Random, 95% CI)0.0 [0.0, 0.0]
Analysis 9.1.

Comparison 9 Biofeedback assisted breathing re-training (BRT) versus biofeedback assisted relaxation training (RLXT), Outcome 1 Pulmonary function.

Comparison 10. Massage therapy versus bedtime reading control
Outcome or subgroup titleNo. of studiesNo. of participantsStatistical methodEffect size
1 Well-being1 Mean Difference (IV, Random, 95% CI)Totals not selected
1.1 Parent Anxiety - State Trait Anxiety Inventory (STAI)1 Mean Difference (IV, Random, 95% CI)0.0 [0.0, 0.0]
1.2 Child Anxiety - State Trait Anxiety Inventory for Children (STAIC)1 Mean Difference (IV, Random, 95% CI)0.0 [0.0, 0.0]
1.3 Child Mood - Profile of Mood States (POMS)1 Mean Difference (IV, Random, 95% CI)0.0 [0.0, 0.0]
2 Pulmonary function1 Mean Difference (IV, Random, 95% CI)Totals not selected
2.1 Peak Air Flow (PEFR) - Day 11 Mean Difference (IV, Random, 95% CI)0.0 [0.0, 0.0]
2.2 Peak Air Flow (PEFR) - Day 301 Mean Difference (IV, Random, 95% CI)0.0 [0.0, 0.0]
Analysis 10.1.

Comparison 10 Massage therapy versus bedtime reading control, Outcome 1 Well-being.

Analysis 10.2.

Comparison 10 Massage therapy versus bedtime reading control, Outcome 2 Pulmonary function.

Comparison 11. Music therapy versus familiar music and no audiotape control
Outcome or subgroup titleNo. of studiesNo. of participantsStatistical methodEffect size
1 Enjoyment1 Mean Difference (IV, Random, 95% CI)Totals not selected
1.1 Parent enjoyment1 Mean Difference (IV, Random, 95% CI)0.0 [0.0, 0.0]
1.2 Child enjoyment1 Mean Difference (IV, Random, 95% CI)0.0 [0.0, 0.0]
2 Cognitive1 Mean Difference (IV, Random, 95% CI)Totals not selected
2.1 Parent perception of time1 Mean Difference (IV, Random, 95% CI)0.0 [0.0, 0.0]
Analysis 11.1.

Comparison 11 Music therapy versus familiar music and no audiotape control, Outcome 1 Enjoyment.

Analysis 11.2.

Comparison 11 Music therapy versus familiar music and no audiotape control, Outcome 2 Cognitive.

Comparison 12. Self-hypnosis versus control
Outcome or subgroup titleNo. of studiesNo. of participantsStatistical methodEffect size
1 Anxiety: State Trait Anxiety Inventory for Children (STAIC)  Other dataNo numeric data
2 Impact on Family Scale  Other dataNo numeric data
3 Child Behavior Checklist  Other dataNo numeric data
4 Average peak expiratory flow rate (PEFR)  Other dataNo numeric data
5 Height and weight  Other dataNo numeric data
6 Rating of the parents' assessment of the child's illness  Other dataNo numeric data
7 Locus of Control (LOC): Nowicki Strickland (1973) LOC for Children  Other dataNo numeric data
8 Health Locus of Control: Children's Health Locus of Control (HLOC)  Other dataNo numeric data
9 Self-concept: Piers Harris Children's Self-Concept Scale  Other dataNo numeric data

Analysis 12.1.

Comparison 12 Self-hypnosis versus control, Outcome 1 Anxiety: State Trait Anxiety Inventory for Children (STAIC).

Anxiety: State Trait Anxiety Inventory for Children (STAIC)
Study 
Belsky 1994No group differences were observed (U=15, p>.05) for State Anxiety. A significant difference between experimental and control group was found for Trait Anxiety (U=1.5, p<.004) with change scores reflecting decreases in Trait Anxiety for the experimental group and increases for the control group.

Analysis 12.2.

Comparison 12 Self-hypnosis versus control, Outcome 2 Impact on Family Scale.

Impact on Family Scale
Study 
Belsky 1994No group differences were observed at post-intervention.

Analysis 12.3.

Comparison 12 Self-hypnosis versus control, Outcome 3 Child Behavior Checklist.

Child Behavior Checklist
Study 
Belsky 1994No group differences were observed at post-intervention.

Analysis 12.4.

Comparison 12 Self-hypnosis versus control, Outcome 4 Average peak expiratory flow rate (PEFR).

Average peak expiratory flow rate (PEFR)
Study 
Belsky 1994The mean PEFR score showed an increase of approximately 76 points from pre- to post-test in the experimental group, whereas it dropped approximately 12 points in the control group. This difference proved to be significant (U=0, p<.001). The difference between the two groups was also significant at the 4-month follow up.

Analysis 12.5.

Comparison 12 Self-hypnosis versus control, Outcome 5 Height and weight.

Height and weight
Study 
Belsky 1994No group differences were observed (U=125, p>.05) at 4 month follow up.

Analysis 12.6.

Comparison 12 Self-hypnosis versus control, Outcome 6 Rating of the parents' assessment of the child's illness.

Rating of the parents' assessment of the child's illness
Study 
Belsky 1994No group differences were observed (U=15, p>.05) at post-intervention.

Analysis 12.7.

Comparison 12 Self-hypnosis versus control, Outcome 7 Locus of Control (LOC): Nowicki Strickland (1973) LOC for Children.

Locus of Control (LOC): Nowicki Strickland (1973) LOC for Children
Study 
Belsky 1994Significant differences were found in change of Locus of Control (U=0, p=.001) between experimental and control group: The scores of the experimental group decreased, which indicates more internality.

Analysis 12.8.

Comparison 12 Self-hypnosis versus control, Outcome 8 Health Locus of Control: Children's Health Locus of Control (HLOC).

Health Locus of Control: Children's Health Locus of Control (HLOC)
Study 
Belsky 1994Significant differences were found in change of Children’s Health Locus of Control (U=0, p=.001) between experimental and control group: The scores of the experimental group increased, which means that children’s internal expectancies related to health improved more in the experimental group.

Analysis 12.9.

Comparison 12 Self-hypnosis versus control, Outcome 9 Self-concept: Piers Harris Children's Self-Concept Scale.

Self-concept: Piers Harris Children's Self-Concept Scale
Study 
Belsky 1994Self-concept scores improved significantly more in the experimental group than in the control group (U=1.5, p=.003) at post-intervention.
Comparison 13. Home telemedicine for patients awaiting transplantation versus usual care
Outcome or subgroup titleNo. of studiesNo. of participantsStatistical methodEffect size
1 Quality of Life: Cystic Fibrosis Quality of Life Questionnaire (Gee 2000)  Other dataNo numeric data
2 Anxiety: Beck Anxiety Inventory (Beck 1988) + Borg anxiety scale (Borg 1982)  Other dataNo numeric data
3 Depression: Beck Depression Inventory (Beck 1987)  Other dataNo numeric data
4 Coping of carers: COPE questionnaire (Carver 1997)  Other dataNo numeric data
5 Health care utilization: seven-point questionnaire (number of visits, general practitioner, courses of IV antibiotics, length of hospital inpatient stay, visits either to hospital or GP)  Other dataNo numeric data

Analysis 13.1.

Comparison 13 Home telemedicine for patients awaiting transplantation versus usual care, Outcome 1 Quality of Life: Cystic Fibrosis Quality of Life Questionnaire (Gee 2000).

Quality of Life: Cystic Fibrosis Quality of Life Questionnaire (Gee 2000)
Study 
Wilkinson 2008Telemedicine group a significant improvement in the subjects’ perception of body image (P=.002).

Analysis 13.2.

Comparison 13 Home telemedicine for patients awaiting transplantation versus usual care, Outcome 2 Anxiety: Beck Anxiety Inventory (Beck 1988) + Borg anxiety scale (Borg 1982).

Anxiety: Beck Anxiety Inventory (Beck 1988) + Borg anxiety scale (Borg 1982)
Study 
Wilkinson 2008"There was no significant change in anxiety before and after link-up for the telemedicine group or before and after clinic attendance for either group. There was no difference in the change of anxiety from pre to post link-up for the telemedicine group or for the control group in pre and post clinic attendance." (Wilkinson 2008, p.184)

Analysis 13.3.

Comparison 13 Home telemedicine for patients awaiting transplantation versus usual care, Outcome 3 Depression: Beck Depression Inventory (Beck 1987).

Depression: Beck Depression Inventory (Beck 1987)
Study 
Wilkinson 2008"At the end of the study there was a higher reporting of depression, which was associated with a higher reporting of anxiety, in both groups." (Wilkinson 2008, p.184)

Analysis 13.4.

Comparison 13 Home telemedicine for patients awaiting transplantation versus usual care, Outcome 4 Coping of carers: COPE questionnaire (Carver 1997).

Coping of carers: COPE questionnaire (Carver 1997)
Study 
Wilkinson 2008No significant differences between the two groups were found.

Analysis 13.5.

Comparison 13 Home telemedicine for patients awaiting transplantation versus usual care, Outcome 5 Health care utilization: seven-point questionnaire (number of visits, general practitioner, courses of IV antibiotics, length of hospital inpatient stay, visits either to hospital or GP).

Health care utilization: seven-point questionnaire (number of visits, general practitioner, courses of IV antibiotics, length of hospital inpatient stay, visits either to hospital or GP)
Study 
Wilkinson 2008No significant differences between the two groups were observed.
Comparison 14. Dance/movement therapy for adults versus control
Outcome or subgroup titleNo. of studiesNo. of participantsStatistical methodEffect size
1 Mood state: ’The Profile of Mood States’ (POMS; Mc Nair 1992)  Other dataNo numeric data
2 Adherence to exercise and nutrition regimen  Other dataNo numeric data
3 Body Image: Human figure drawing task  Other dataNo numeric data

Analysis 14.1.

Comparison 14 Dance/movement therapy for adults versus control, Outcome 1 Mood state: ’The Profile of Mood States’ (POMS; Mc Nair 1992).

Mood state: ’The Profile of Mood States’ (POMS; Mc Nair 1992)
Study 
Goodill 2005Scores were analysed performing a repeated measures ANOVA: Found within-subjects interaction of Time, group, and Gender on Levels of Confusion (F (2,20) =5.99, P=.007, eta2=.231). Men in the treatment group showed the strongest effect.

Analysis 14.2.

Comparison 14 Dance/movement therapy for adults versus control, Outcome 2 Adherence to exercise and nutrition regimen.

Adherence to exercise and nutrition regimen
Study 
Goodill 2005No significant differences on exercise at post-intervention or follow-up (1 month later) and not for nutrition at post-intervention. But significant differences at follow-up between groups on adherence to nutrition regimes (Chi2 (1, N=23) = 4.485, P=.03) with lower levels in the control group.

Analysis 14.3.

Comparison 14 Dance/movement therapy for adults versus control, Outcome 3 Body Image: Human figure drawing task.

Body Image: Human figure drawing task
Study 
Goodill 2005No group differences were observed.

Appendices

Appendix 1. New Search for the updated review

Psychological Therapies for Cystic Fibrosis: Search strategies June 2012

1.      Cochrane Central Register of Controlled Trials (CENTRAL)

Intervention: Psychotherapy
#1MeSH descriptor PSYCHOTHERAPY explode all trees
#2(CBT or coping skills or counsel* or hypno* or mindfulness or psychoanal* or psychoeducat* or psychodynamic or psychotherapy* or rehabilitat*):ti,ab,kw
#3(psychologic* or behavior or behaviour or cognitive):ti,ab,kw
#4(Abreaction or (Acting NEXT Out) or Adlerian or (Adolescent NEXT Psych*) or (Anger NEXT Control) or (Anger NEXT Management) or (Art NEXT Therap*) or (Assertive* NEXT Training) or (Autogenic NEXT Training) or Autosuggestion or (Aversion NEXT Therap*) or (Balint NEXT Group) or Bibliotherap* or Biofeedback or (Caregiver NEXT Support) or (Child NEXT Psych*) or (Client NEXT Cent*) or (Color NEXT Therap*) or (Colour NEXT Therap*)  or (Mind NEXT Training) or (Conjoint NEXT Therap*) or (Contingency NEXT Management) or (Conversion NEXT Therap*) or (Conversational NEXT Therap*) or Countertransference or (Couples NEXT Therap*) or (Covert NEXT Sensitization) or (Crisis NEXT Intervention)):ti,ab,kw
#5((Dance NEXT Therap*) or Dialectic* or (Dream* NEXT Analys*) or Eclectic or (Emotion* NEXT Focus*) or (Emotional NEXT Freedom NEXT Technique) or (Encounter NEXT Group) or (Existential NEXT Therap*) or (Exposure NEXT Therap*) or (Eye NEXT Movement NEXT Desensiti*) or (Family NEXT Therap*) or (Free NEXT Association) or (Gestalt NEXT Therap*) or Griefwork  or (Group NEXT Therap*) or (Guided NEXT Image*) or Imagery or (Implosive NEXT Therap*) or (Insight NEXT Therap*) or Integrative or Interpersonal):ti,ab,kw
#6(Logotherap* or (Marital NEXT Therap*) or Meditation or (Mental NEXT Healing) or Metacognitive or (Milieu NEXT Therap*) or (Morita NEXT Therap*) or (Music NEXT Therap*) or (Narrative NEXT Therap*) or (Nondirective NEXT Therap*) or (Personal NEXT Construct) or (Person NEXT Cent*) or Persuasion or (Pet NEXT Therap*) or (Play NEXT Therap*) or (Primal NEXT Therap*) or (Problem NEXT Solv*) or Psychodrama or Psychotherapeutic):ti,ab,kw
#7((Rational NEXT Emotive) or (Reality NEXT Therap*) or (Reciprocal NEXT Inhibition) or (Relationship NEXT Therap*) or Relaxation or (Stress NEXT Manage*) or Reminiscence or (Role NEXT Play*) or (Self NEXT Analys*) or (Self NEXT Esteem) or (Sensitivity NEXT Training) or (Sex NEXT Therap*) or (Sleep NEXT Phase NEXT Chronotherap*) or (Socioenvironmental NEXT Therap*) or Sociotherap* or (Solution NEXT Focus*) or (Stress Manage*) or (Support NEXT Group*) or (Systematic NEXT Desensiti*) or (Therapeutic NEXT Communit*) or (Transactional NEXT Analysis) or (Validation NEXT Therap*)):ti,ab,kw
#8(Support NEAR/3 Psycho*):ti,ab,kw
#9MeSH descriptor ASSOCIATION explode tree 1
#10MeSH descriptor HYPNOSIS explode tree 1
#11MeSH descriptor CYSTIC FIBROSIS explode all trees with qualifiers: PX,RH,TH
#12MeSH descriptor NUTRITION THERAPY explode all trees
#13((nutrition* or diet*) NEAR/7 (behavi* or educat* or intervention or support* or therap*)):ti,ab,kw
#14MeSH descriptor DIET explode all trees with qualifiers: PX,TH
#15MeSH descriptor SELF CARE explode all trees
#16MeSH descriptor SELF EFFICACY, this term only
#17(self NEAR/3 (care* or educat* or efficacy or help or manage*)):ti,ab,kw
#18MeSH descriptor HEALTH EDUCATION explode all trees
#19MeSH descriptor COUNSELING explode all trees
#20MeSH descriptor GENETIC COUNSELING explode all trees
#21(#1 or #2 or #3 or #4 or #5 or #6 or #7 or #8 or #9 or #10 or #11 or #12 or #13 or #14 or #15 or #16 or #17 or #18 or #19 or #20)
Condition: Cystic Fibrosis
#22MeSH descriptor CYSTIC FIBROSIS, this term only
#23(cystic NEXT fibrosis):ti,ab,kw
#24Mucoviscidos*:ti,ab,kw
#25((cysti* or cisti*) NEAR/10 fibros*):ti,ab,kw
#26(fibrocystic* NEAR pancrea*):ti,ab,kw
#27(#22 or #23 or #24 or #25 or #26)
#28 (#21 and #27)

 2.      OVID MEDLINE

Intervention: Psychotherapy
1exp Psychotherapy/
2(CBT or coping skills or counsel?ing or hypno$ or mindfulness or psychoanal$ or psychoeducat$ or psychodynamic or psychotherap$ or rehabilitat$).tw.
3(psychologic$ or behavio?r or cognitive).tw.
4(Abreaction or Acting Out or Adlerian or Adolescent Psych$ or Analytical Psychotherap$ or Anger Control or Anger Management or Art Therap$ or Assertive$ Training or Autogenic Training or Autosuggestion or Aversion Therap$ or Balint Group or Behavio?r Contracting or Behavio?r Modification or Behavio?r Therap$ or Bibliotherap$ or Biofeedback or Body Psychotherap$ or Brief Psychotherap$ or Caregiver Support or Child Psych$ or Client Cent$ or Cognitive Behavio?r* or Cognitive$ Rehabilit$ or Cognitive Therap$ or Colo?r Therap$ or Compassionate Mind Training or Conjoint Therap$ or Contingency Management or Conversion Therap$ or Conversational Therap$ or Countertransference or Couples Therap$ or Covert Sensitization or Crisis Intervention).mp.
5(Dance Therap$ or Dialectic$ or (Dream$ adj3 Analys$) or Eclectic or Emotion$ Focus$ or Emotional Freedom Technique or Encounter Group Therap$ or Existential Therap$ or Experiential Psychotherap$ or Exposure Therap$ or Expressive Psychotherap$ or Eye Movement Desensiti#ation or Family Therap$ or Feminist Therap$ or Free Association or Geriatric Psychotherap$ or Gestalt Therap$ or Griefwork or Group Psychotherap$ or Group Therap$ or Guided Image$ or Holistic Psychotherap$ or Humanistic Psychotherap$ or Hypnosis or Hypnotherap$ or Hypnoti#zability or Imagery or Implosive Therap$ or Individual Psychotherap$ or Insight Therap$ or Integrative Psychotherap$ or Integrative Therap$ or Interpersonal).mp.
6(Logotherap$ or Marathon Group Therap$ or Marital Therap$ or Meditation or Mental Healing or Metacognitive Therap$ or Milieu Therap$ or Morita Therap$ or Music Therap$ or Narrative Therap$ or Nondirective Therap$ or Personal Construct Therap$ or Person Cent$ Therap$ or Persuasion Therap$ or Pet Therap$ or Play Therap$ or Primal Therap$ or Problem Solving or Psychoanaly$ or Psychodrama or Psychodynamic or Psychotherapeutic Counsel$ or Psychotherapeutic Processes or Psychotherapeutic Training).mp.
7(Rational Emotive or Reality Therap$ or Reciprocal Inhibition or Relationship Therap$ or Relaxation or Stress Management or Reminiscence Therap$ or Role Play$ or Self Analys$ or Self Esteem or Sensitivity Training or Sex Therap$ or Sleep Phase Chronotherap$ or Socioenvironmental Therap$ or Sociotherap$ or Solution Focused or Stress Management or Support Group$ or (Support adj3 Psycho$) or Systematic Desensiti#ation or Therapeutic Communit$ or Transactional Analysis or Validation Therap$).mp.
8ASSOCIATION/ or ASSOCIATION LEARNING/
9exp HYPNOSIS/
10CYSTIC FIBROSIS / px, rh, th [Psychology, Rehabilitation,Therapy]
11exp NUTRITION THERAPY/
(to include diet therapy/ and nutritional support/)
12((nutrition* or diet*) adj7 (behavi* or educat* or intervention or support* or therap*)).tw.
13DIET/px [Psychology]
14SELF CARE/
15SELF EFFICACY/
16((self) adj3 (care* or educat* or efficacy or help or manage*)).tw.
17exp HEALTH EDUCATION/
18exp COUNSELING/
19exp GENETIC COUNSELING/
20or/1-19
Condition: Cystic Fibrosis
21Cystic Fibrosis/
22cystic fibrosis.tw.
23mucoviscidos$.tw.
24((cysti$ or cisti$) adj10 fibros$).tw, ot.
25(fibrocystic* and pancreas*).tw.
26or/15-19
Filter: RCTs
27randomized controlled trial.pt.
28controlled clinical trial.pt.
29randomi#ed.ti,ab.
30randomly.ab.
31placebo.ab.
32trial.ab.
33groups.ab.
34(control$ adj3 (trial or study)).ab,ti.
35((singl$ or doubl$ or tripl$ or trebl$) adj3 (blind$ or mask$ or dummy)).mp.
36(animals not (humans and animals)).sh.
37or/27-35
3837 not 36
39 (20 and 26 and 38)

 3.      OVID EMBASE

Intervention: Psychotherapy
1exp Psychotherapy/
2(CBT or coping skills or counsel?ing or hypno$ or mindfulness or psychoanal$ or psychoeducat$ or psychodynamic or psychotherap$ or rehabilitat$).tw.
3(psychologic$ or behavio?r or cognitive).tw.
4(Abreaction or Acting Out or Adlerian or Adolescent Psych$ or Analytical Psychotherap$ or Anger Control or Anger Management or Art Therap$ or Assertive$ Training or Autogenic Training or Autosuggestion or Aversion Therap$ or Balint Group or Behavio?r Contracting or Behavio?r Modification or Behavio?r Therap$ or Bibliotherap$ or Biofeedback or Body Psychotherap$ or Brief Psychotherap$ or Caregiver Support or Child Psych$ or Client Cent$ or Cognitive Behavio?r* or Cognitive$ Rehabilit$ or Cognitive Therap$ or Colo?r Therap$ or Compassionate Mind Training or Conjoint Therap$ or Contingency Management or Conversion Therap$ or Conversational Therap$ or Countertransference or Couples Therap$ or Covert Sensitization or Crisis Intervention).mp.
5(Dance Therap$ or Dialectic$ or (Dream$ adj3 Analys$) or Eclectic or Emotion$ Focus$ or Emotional Freedom Technique or Encounter Group Therap$ or Existential Therap$ or Experiential Psychotherap$ or Exposure Therap$ or Expressive Psychotherap$ or Eye Movement Desensiti#ation or Family Therap$ or Feminist Therap$ or Free Association or Geriatric Psychotherap$ or Gestalt Therap$ or Griefwork or Group Psychotherap$ or Group Therap$ or Guided Image$ or Holistic Psychotherap$ or Humanistic Psychotherap$ or Hypnosis or Hypnotherap$ or Hypnoti#zability or Imagery or Implosive Therap$ or Individual Psychotherap$ or Insight Therap$ or Integrative Psychotherap$ or Integrative Therap$ or Interpersonal).mp.
6(Logotherap$ or Marathon Group Therap$ or Marital Therap$ or Meditation or Mental Healing or Metacognitive Therap$ or Milieu Therap$ or Morita Therap$ or Music Therap$ or Narrative Therap$ or Nondirective Therap$ or Personal Construct Therap$ or Person Cent$ Therap$ or Persuasion Therap$ or Pet Therap$ or Play Therap$ or Primal Therap$ or Problem Solving or Psychoanaly$ or Psychodrama or Psychodynamic or Psychotherapeutic Counsel$ or Psychotherapeutic Processes or Psychotherapeutic Training).mp.
7(Rational Emotive or Reality Therap$ or Reciprocal Inhibition or Relationship Therap$ or Relaxation or Stress Management or Reminiscence Therap$ or Role Play$ or Self Analys$ or Self Esteem or Sensitivity Training or Sex Therap$ or Sleep Phase Chronotherap$ or Socioenvironmental Therap$ or Sociotherap$ or Solution Focused or Stress Management or Support Group$ or (Support adj3 Psycho$) or Systematic Desensiti#ation or Therapeutic Communit$ or Transactional Analysis or Validation Therap$).mp.
8Association/
9Suggestion/ or Transference/
10CYSTIC FIBROSIS / rh, th [Rehabilitation,Therapy]
11NUTRITION EDUCATION/
12((nutrition* or diet*) adj7 (behavi* or educat* or intervention or support* or therap*)).tw.
13exp DIET THERAPY/
14DIET/th
15exp SELF CARE/
16exp SELF CONCEPT/
17((self) adj3 (care* or educat* or efficacy or help or manage*)).tw.
18exp HEALTH EDUCATION/
19exp COUNSELING/
20or/1-19
Condition: Cystic Fibrosis
21Cystic Fibrosis/
22cystic fibrosis.tw.
23mucoviscidos$.tw.
24((cysti$ or cisti$) adj10 fibros$).tw, ot.
25(fibrocystic* and pancreas*).tw.
26or/21-25
Filter: RCTs
27randomized controlled trial.de.
28randomization.de.
29placebo.de.
30placebo.ab.
31randomi#ed.ti,ab.
32randomly.ab.
33((singl$ or doubl$ or trebl$ or tripl$) adj3 (blind$ or mask$ or dummy)).mp.
34factorial$.ti,ab.
35(allocat$ or assign$).ti,ab.
36crossover procedure.de.
37(crossover$ or cross over$).ti,ab.
38(quasi adj (experimental or random$)).mp.
39(control$ adj3 (trial$ or study or studies or group$)).ti,ab.
40((animal or nonhuman) not (human and (animal or nonhuman))).de.
41or/27-39
4241 not 40
43 (20 and 26 and 42)

 4.      OVID PsycINFO (Condition + RCT filter only)

Condition: Cystic Fibrosis
1Cystic Fibrosis/
2cystic fibrosis.tw.
3mucoviscidos$.tw.
4((cysti$ or cisti$) adj10 fibros$).tw, ot.
5(fibrocystic* and pancreas*).tw.
6or/21-25
Filter: RCTs
7treatment effectiveness evaluation.sh.
8clinical trials.sh.
9mental health program evaluation.sh.
10randomly.ab.
11randomi#ed.ti,ab.
12(placebo or (attention control)).ab.
13(waitlist$ or (wait$ adj2 list$)).ab.
14trial.ti,ab.
15((singl$ or doubl$ or trebl$ or tripl$) adj3 (blind$ or mask$ or dummy)).mp.
16(control$ adj3 (trial$ or study or studies or group$)).ti,ab.
17factorial$.ti,ab.
18(allocat$ or assign$).ti,ab.
19(crossover$ or cross over$).ti,ab.
20(quasi adj (experimental or random$)).mp.
21"2000".md.
22 or/7-21
23 (6 and 22)

What's new

DateEventDescription
11 June 2014New search has been performed

Searches carried out identified 28 new relevant reports. Ten reports related to seven newly identified studies (Belsky 1994; Taylor 2003; Goodill 2005; Christian 2006; Wilkinson 2008; Stark 2009; Vandemheen 2009) were included in this review. One previous classified as ongoing is now included (Quinn 2004).

Nine of the new relevant reports were not eligible for inclusion. Three studies (Quittner 2011; Quittner 2012, Riekert 2012) were recorded as ongoing and six studies are awaiting classification/information from authors.

Due to a new Cochrane Review on 'Self-management education for cystic fibrosis' (Savage 2011) all five previously included studies with an purely educational approach were excluded (Cheuvront 1998; Trapp 1998; Stapleton 2001; Davis 2002; Downs 2006).

11 June 2014New citation required but conclusions have not changed

Leading author changed (new: Lutz Goldbeck) and new co-authors joined the team (Astrid Fidika and Marion Herle).

The title has been changed from 'Psychological interventions from people with cystic fibrosis and their families'.

The definition of psychological intervention was changed to be more precise and the classification of types of interventions, as well as specification of types of outcomes has been changed.

History

Protocol first published: Issue 3, 2001
Review first published: Issue 3, 2003

DateEventDescription
12 August 2009AmendedContact details updated.
29 April 2008AmendedConverted to new review format.
29 April 2008New search has been performed

Twenty new reports were found. Eleven reports related to five new studies (Chernoff 2002; Davis 2002; Downs 2006; Powers 2005; Stapleton 2001) which were included in this review and three reports related to studies already included.

A single report of one study was excluded and four reports related to three studies which were recorded as ongoing. It was unclear whether one further report represented a new study or simply an additional report of an existing study and so awaits classification.

29 April 2008New citation required but conclusions have not changedThe title has been changed from 'Psychological interventions for cystic fibrosis'.
21 March 2008New citation required and conclusions have changedSubstantive amendment

Contributions of authors

Lutz Goldbeck (LG) is taking principal responsibility for updating the review. He screened titles, abstracts, and reviewed papers against inclusion criteria. He drafted the updated review. He was appraising quality of papers and interpreted results of data analysis.

Astrid Fidika (AF) is co-author for the review. She was screening titles, abstracts and, retrieved papers against the inclusion criteria. She extracted data from included papers, appraised quality of papers, took the leading role in contacting authors of papers for additional information. She investigated data analysis and interpretation, data management for the review, and entered data into RevMan. She provided advice on conducting the update of the systematic review and was involved in drafting the update.

Marion Herle (MH) is co-author for the review. She was screening titles, abstracts and, retrieved papers against the inclusion criteria. She extracted data from included papers and appraised quality of papers. She also cross-checked data entry of results for each outcome to Revman.

Alexandra L. Quittner (AQ) provided general advice on the review and interpretation of results for the current update. She has contributed a broad overview to the process of updating this review. She has supported CG for the original review and previous updates, and fine-tuned the strategy for reviewing intervention studies within this vast and complex area. She also initiated the debate surrounding evidence-based interventions with the international psychosocial interest group, which enhanced the discussion of the findings.

Claire A. Glasscoe (CG) was guarantor for the previous updates and the original review. She drafted the review  and drew on the expertise of others to refine it.

Declarations of interest

None known.

Sources of support

Internal sources

  • Royal Liverpool Children's NHS Trust, UK.

  • National Institutes of Health, USA.

External sources

  • No sources of support supplied

Differences between protocol and review

The Definition of psychological intervention was been changed to a more precisely definition of psychological intervention and classification types of intervention has been changed.

Due to a new Cochrane Review on 'Self-management education for cystic fibrosis', studies with a purely educational approach are not longer included for this review (Savage 2011).

Characteristics of studies

Characteristics of included studies [ordered by study ID]

Belsky 1994

Methods

CCT.

Pilot study that assessed the effects of self-hypnosis on psychological and physiological functioning of children ages 7 to 18 years with CF.

Hypothesis: hypnosis is associated with enhanced performance on clinical rating criteria and with improved scores in self-concept and other psychological tests.

The study used a pre- and post-test design with control group and repeated measures.

Participants

12 children with CF ages 7 - 18 years attending a local CF clinic.

Population of interest: 30 (met eligibility).

Number included: 12.

Control group: 5 girls and 2 boys.

Interventions

The parents and children in the experimental group had three appointments within a 2-week period during which time the children were taught a hypnotic technique.

The hypnotic induction is described in detail in the article.

Outcomes

CF illness ratings: Shwachman- Kulzcycki system standard form has been completed by the physician at baseline, which is based on 100 maximum points measuring four categories: general activity, physical examination, nutrition, and radiographic findings. Other illness variables included each patient's average PEFR, height, weight, and number of hospital admissions during 2 years prior to the study.

Parents' measures: 'Impact on Family Scale' (measures family functioning); 'Child Behavior Checklist' (parents' perception of behavioral problems and competencies); rating of the parents' assessment of the child's illness; parents also provided a record of school days missed by their child for 3 years before the pretest.

Children's measures: LOC was assessed with the Nowicki Strickland (1973) LOC for Children and with the Children's HLOC (Parcel & Mayer, 1978) which is a 20-item area specific measure of expectancies regarding locus of control and prediction of health-related behavior. Self-concept was assessed with the Piers Harris Children's Self-Concept Scale (1964). Anxiety was assessed with the STAIC (Spielberger 1973).

NotesNone of the authors could be contacted for questions on provided data.
Risk of bias
BiasAuthors' judgementSupport for judgement
Random sequence generation (selection bias)High riskThe 2 groups were not randomised. The authors stated that "2 groups were formed on the basis of comparability of age and clinical ratings" (Belsky 1994, p. 284).
Allocation concealment (selection bias)High riskBecause groups were not randomised, participants and/or investigators enrolling participants could probably foresee assignment.
Blinding (performance bias and detection bias)
All outcomes
Unclear riskBlinding of participants and personnel providing the intervention was not possible. The authors provided no information on blinding of outcome assessment.
Incomplete outcome data (attrition bias)
All outcomes
Unclear riskNo information on dropouts were provided by the authors.
Selective reporting (reporting bias)High riskThe number of participants for post-intervention analysis were not mentioned. Outcomes of interest in the review are reported incompletely so that they cannot be entered in a an analysis (N, means and standard deviations for some of the outcomes).

Chernoff 2002

MethodsParallel RCT.
Comparing the effect for carers of children with a chronic illness of a comprehensive community-based support programme with having a contact telephone number for an experienced mother of a child with a similar illness.
For children it was hypothesised that those in the experimental group would have better mental health at the end of the intervention than controls. For the mothers it was hypothesised that those in the intervention group would report fewer psychological symptoms than control mothers.
ParticipantsPopulation of interest N = 193.
Sample agreeing to participate N = 161 children with a chronic illness and their mothers. Children with diabetes, sickle cell, CF or moderate to severe asthma were included.
For CF subgroup: n = 7 assigned to the intervention group and n = 6 controls.
Included children aged 7 - 11 years living within 80 km radius of Baltimore, with no learning disability, with a mother as a primary carer, on the telephone, speaking English, and diagnosed > 6 months ago.
Interventions1. Community-based support programme over 15 months - trained network mother + child life specialist pairing.
a. 7 home visits
b. twice-weekly telephone calls
c. 3 special family events
2. Control group are given a contact number for an experienced mother.
Outcomes Children
a. personal adjustment and role skills
b. depression
c. anxiety
d. self-perception
Mothers
a. anxiety
b. depression
c. stressful life events
Notes

POI: anxiety subscale - 11 items self-report from 'Psychiatric Symptom Index'.
The groups were equivalent at baseline although these data not reported for individual disease subgroups.
Network mothers were trained and supervised and a manual was produced.

Treatment fidelity: the authors reported that weekly meetings took place to ensure that intervention was provided as planned (Chernoff 2002, p.534/35).

Risk of bias
BiasAuthors' judgementSupport for judgement
Random sequence generation (selection bias)Unclear riskThe authors did not describe the randomisation process in detail. The stated that of the 161 families being eligible and who agreed to participate were 'randomly assigned to the experimental group and [...] to the control group (Chernoff 2002; p. 773).
Allocation concealment (selection bias)Unclear riskThe method of concealment is not described.
Blinding (performance bias and detection bias)
All outcomes
Low riskDue to the nature of the intervention blinding of participants is not possible adequately, but the authors provided the information that 'interviews were completed by paid interviewers who had undergone extensive training and were blind to group assignment' (Chernoff 2002; p. 534).
Incomplete outcome data (attrition bias)
All outcomes
High riskThe authors reported that about 15.5% of the randomised participants were not available for follow-up analysis. 'of the 161 families randomised, 86 were randomised to the experimental group and 75 to the control group. Overall, 25 families, 14 and 11 from the experimental and the control group, respectively, were lost to follow up [...] (Chernoff 2002; p. 536).
Selective reporting (reporting bias)High riskThe presentation of the results was selective. The subgroup analysis reported on p. 537 and figure 2 e.g. was not pre-specified.

Christian 2006

Methods

RCT.

2-group, experimental, repeated-measures design (baseline, 3, 6, and 9 months intervals) with randomly assigned participants to intervention and usual care group.

Hypothesis: children with CF who receive the intervention will improve significantly on 3 parameters (psychosocial adjustment, functional health status, physiologic health) over the time compared to children who received usual care.

Participants

Population of interest: N = 128.

Number of randomised participants: N = 116.

Control group 'usual care': N = 58.

Intervention group 'educational problem-solving and social skills intervention': N = 58.

Included children with CF aged 8 - 12 years receiving care from one of four CF centres in North Carolina (USA).

Interventions

Educational problem-solving and social skills intervention (N = 58) versus usual care (N = 58).

Children in the problem-solving group received individual, tailored intervention during one home visit and a structured group session (conducted approximately 2 weeks after individual home visit).

Intervention was designed to support children (8 - 12years) with the following specific problems:

1. finding out about the CF diagnosis;

2. explaining CF-related differences;

3. dealing with teasing about CF;

4. keeping up with peers during physical activity.

The intervention contains 4 modules. In every module there was a focus on one of the 4 mentioned areas of problems (1-4). The intervention team received detailed information about the intervention protocol and was trained and supervised conducting the intervention.

Outcomes

5 questionnaires which are developed for children were read aloud by research assistant to the children. The following questionnaires were used to the assess different constructs:

Psychosocial adjustment was assessed by:

- Perceived Illness Experience Scale (35 items);

- Children's Loneliness Scale (16 items);

- Social Support Scale for Children (16 items);

- Self-Perception Profile for Children (30 items).

Functional Health Status was assessed by:

- Functional Disability Inventory (15 items).

Physiolgical Health Status:

- Pulmonary function (FEV1).

- Height and weight.

- BMI.

Notes 
Risk of bias
BiasAuthors' judgementSupport for judgement
Random sequence generation (selection bias)Low riskThe authors describe that the children were randomly assigned to the intervention and control group "using a computer-generated randomization plan". (Christian 2006, p. 302)
Allocation concealment (selection bias)Low riskAssignment could not been foreseen by participants and investigators enrolling participants due to a computer-generated randomisation plan.
Blinding (performance bias and detection bias)
All outcomes
Low riskThe authors state that "group assignments were unknown to the CF clinics and research assessment team". (Christian 2006; p. 302)
Incomplete outcome data (attrition bias)
All outcomes
Low riskThe authors stated that "all children completed the baseline and 3 follow-up assessments" (Christian 2006, p.301). That means that there was no attrition or loss of any participant during the study and follow-up period.
Selective reporting (reporting bias)Unclear riskAll outcomes mentioned in the published records are reported. It is unclear if additional outcomes were pre-specified in the study protocol but not reported.

Delk 1994

MethodsParallel RCT.
Comparing the effects of therapy that it is targeted with aim of facilitating physiotherapy and when it is aimed at increasing relaxation.
ParticipantsN = 26.
Aged 10 - 41 years.
Groups matched by age and severity of disease using Shwachman-Kulczycki scoring system prior to randomisation.
Interventions1. Biofeedback assisted breathing re-training (N = 13).
2. Biofeedback assisted relaxation training (N = 13).
8 sessions over 4 weeks.
Delivered by psychology and biofeedback students under supervision.
Outcomes Lung function:
1. FVC;
2. FEV1;
3. FEF 25-75%.
Evaluation at:
1. 3 pre-study assessments, 18 months prior to study commencement;
2. baseline;
3. post-study;
4. follow up.
NotesPOI: Pulmonary function.
The groups were equivalent at baseline on age, severity of disease, height, weight, and pre-treatment lung functions.
Both groups believed they were being given an intervention so effective blinding of participants - no information about blinding of trainers and pulmonary function technicians.
Not clear how sample was selected or from what population they were drawn.
Analysed as ITT apart from one missing matched pair in FEV1 outcome measure.
Limited follow-up data.
Risk of bias
BiasAuthors' judgementSupport for judgement
Random sequence generation (selection bias)Unclear riskDetails of random generation process were not provided by the authors. They just reported that 'subjects were screened medically, matched by age and severity disease, and randomly assigned to either the experimental group receiving the biofeedback-assisted BRT or to the control groups receiving peripheral temperature biofeedback-assisted relaxation training (RLXT)' (Delk 1994, p. 23).
Allocation concealment (selection bias)Unclear riskThe method of concealment is not described.
Blinding (performance bias and detection bias)
All outcomes
Unclear riskDue to the nature of the intervention blinding of participants is not possible adequately. Insuffiecent information about blinding of outcome assessment available,
Incomplete outcome data (attrition bias)
All outcomes
Low riskOutcomes were analysed as intention-to-treat, but it has to be mentioned that for FEV1 results of one participant is missing (n = 25). Reasons were not explained by the authors.
Selective reporting (reporting bias)Unclear riskInformation is insufficient to judge whether 'low' or 'high' risk.

Goodill 2005

Methods

RCT.

Compared brief dance/movement therapy for adult CF patients during an exacerbation of CF symptomatology to standard care.

In the study it was explored whether treatment group participants would show more positive mood state, better adherence with self-care expectations, and a healthier and/or more positive body image.

Participants

Participants were 17 years or older and hospitalised for CF treatment. All were Caucasian except one, who was African-American.

Pre-test and post-test data were collected for 42 participants, follow-up data for 24 participants (14 in the treatment group and 10 in the control group).

InterventionsThe intervention consisted of 3 individual or small group DMT sessions over a 7- to l0-day time period. DMT, a creative arts psychotherapy, utilizes guided movement activity with creative and non-verbal communication processes for increasing self-awareness, enhancing expressive competence, and integrating cognitive, physical, social, and emotional functioning.
Outcomes

Mood state was measured with 'The Profile of Mood States' (POMS): scores for 'Total Mood Disturbance' and 6 subscales: 'Tension'; 'Depression'; 'Fatigue'; 'Confusion'; 'Anger'; and 'Vigor'.

Body image was assessed with human figure drawings created by the participants and rated by trained raters (levels of acceptable agreement r = .85).

Adherence: an adherence questionnaire developed for this study posed two simple questions regarding adherence with physician instructions for exercise and nutrition regimens: "How much did you do of what was instructed for exercise? for nutrition?" Response options were, "l did almost everything" (or "about half" or "little or nothing") of "what I was expected to do.”

NotesContacted the leading author for detailed data on the outcome measures.
Risk of bias
BiasAuthors' judgementSupport for judgement
Random sequence generation (selection bias)Unclear riskThe author only provided the information that "randomization formed groups similar in age, gender, and disease severity" (Goodill 2005, p. 76).
Allocation concealment (selection bias)Unclear riskThe author did not describe details of allocation concealment.
Blinding (performance bias and detection bias)
All outcomes
Low riskParticipants and personnel providing the intervention were not able to be blinded due to the nature of the intervention. Research assistants, who conducted pre- and post-testing were blinded to group assignments (Goodill 2005, p.76).
Incomplete outcome data (attrition bias)
All outcomes
High riskThe author reported a high rate of dropouts at follow up. The percentage of dropouts in each group is unclear. A complete data set was only for 14 participants available, although 42 participants have been randomised.
Selective reporting (reporting bias)High riskMeans and SDs for all outcome parameters for intervention and control group were not reported in the published article. Of the reported measure it was not prespecified which is the primary outcome.

Grasso 2000

MethodsParallel RCT.
Comparing the effects of music therapy in enhancing the experience of physiotherapy with no effective intervention.
Random allocation to 2 groups. Groups matched for spread of ages, frequency of CPT and number of rural families.
Control group experienced 2 consecutive conditions - control and placebo control with repeated measures.
ParticipantsPopulation of interest N = 26.
Five refused. Recruitment rate 87.5%.
Sample comprised N = 21.
N = 1 withdrew from intervention group.
Caregivers of one or more children with CF - parent most responsible for CPT.
Children aged 6 to 24 months with diagnosis at least two months prior to start of study.
Exclusions - children admitted to hospital with a chest infection.
InterventionsIntervention group (N = 10):
1. Music Therapy Tape (TT) with 5 minute explanation and instruction to use as often as they liked plus diary chart (N = 10).
Control group (N = 10):
2. No audiotape (NT control).
3. Familiar music tape (FT placebo control) plus diary chart .
The study lasted for 12 weeks with the control group experiencing 2 conditions each for a period of 6 weeks in the order of 2. then 3.
Outcomes1. Enjoyment - child & parent.
2. Perception of time.
Evaluation at baseline, 6 weeks and 12 weeks.
Ad hoc test measure.
Notes

POI: enjoyment in child and parent and perception of time in parent.
Equivalence at baseline for:
1. age;
2. sex;
3. stage of condition.
Analysed as ITT apart from one dropout.
Participants not informed about whether they were in treatment group.

Treatment fidelity/compliance: The authors reported that the 'participants were asked to complete a diary chart to monitor frequency of tape use' (Grasso 2000, p.373).

Risk of bias
BiasAuthors' judgementSupport for judgement
Random sequence generation (selection bias)Unclear riskThe authors did not describe details of random generation process. They just stated that 'the participants were randomly allocated into control and treatment groups.' (Grasso 2000, p. 372)
Allocation concealment (selection bias)Unclear riskThe authors did not describe the method of concealment.
Blinding (performance bias and detection bias)
All outcomes
Unclear riskThe authors reported that the participants were not informed about the group assignment, but blinding for control condition (no audiotape and later familiar music tape) is doubtful. All outcomes were assessed via self-reports by the participants.
Incomplete outcome data (attrition bias)
All outcomes
Low riskThe authors report that 21 families were assigned to one of the groups and that 'one family was withdrawn when the child was hospitalised for a chest infection, leaving a total of 10 in each group' (Grasso 2000, p.373). That means that the attrition during the study was less than 15%.
Selective reporting (reporting bias)Unclear riskThe available information is insufficient to judge whether 'low' or 'high' risk.

Hernandez-Reif 1999

MethodsParallel RCT.
Control group offered intervention on completion of study.
Comparing the effect of massage in enhancing pulmonary function.
ParticipantsPopulation of interest N = 24, aged 5 - 18 years.
1 refused and 3 dropped out.
Total sample n = 20 parent/child dyads.
Children aged 5 - 12 years.
Intervention group: 9 mothers, 1 father & 10 children.
Control group: 10 mothers & 10 children.
Mean baseline scores on NIH clinical score:
intervention group 82.2; control group 82.0.
Interventions1. Massage therapy (N = 10).
2. Bedtime reading control (N = 10).
Every evening for 30 days.
OutcomesParents: anxiety (STAI).
Children: anxiety (STAIC); mood (POMS); peak flow (PEFR).
Assessment at baseline (Day 1) & Day 30.
Assessment: pre- and post-Day 1; pre- and post-Day 30.
NotesPOI: peak flow.
Fewer than 15% refused or dropped out.
Equivalence at baseline for:
1. sex of parent;
2. age of child;
3. SES;
4. severity.
No blinding of participants or therapists and blinding of assessors unclear.
Risk of bias
BiasAuthors' judgementSupport for judgement
Random sequence generation (selection bias)Unclear riskThe authors did not describe details of random generation process. They just stated that the participants were 'randomly assigned to a massage therapy or a reading control group' (Hernandez-Reif 1999, p. 177).
Allocation concealment (selection bias)Unclear riskThe authors did not present information about the allocation concealment method.
Blinding (performance bias and detection bias)
All outcomes
High riskDue to the nature of the interventions the participants and the interventionists could not be blinded. The authors did not report whether the research assistants, who were calling the participants and checking for compliance, were blinded (as well as outcome assessment). Due to the lack of blinding the performance might have been influenced.
Incomplete outcome data (attrition bias)
All outcomes
Low riskThe authors reported that out of 23 participants, 20 were available for post analyses. Those three families 'were later dropped from the study due to noncompliance' and 'had failed to keep their 30-day appointment' (Hernandez-Reif 1999, p. 177). Two were assigned to the control condition and one family to the massage therapy condition.
Selective reporting (reporting bias)Unclear riskAll outcomes, which have been assessed, were reported.

Powers 2003

MethodsParallel RCT.
Examine the effectiveness of 2 nutritional interventions.
Hypotheses:
1. children participating in both groups will increase their calorie intake;
2. children participating in both groups will show significant increases in weight.
Sequence of randomisation - number drawn from a box.
ParticipantsN = 12.
Less than 3 years old.
Confirmed diagnosis of CF with pancreatic insufficiency.
No other disease or condition known to affect growth.
Interventions1. Nutrition intervention with strategies for enhancing calorie intake - behavioral management training for parents designed to encourage children to eat food consistent with CF dietary recommendations (N = 7).
2. Nutrition intervention only (N = 5).
Both groups received 8 sessions (45 to 60 minutes) over 1 year:
Sessions 1 to 4 (3 months) intensive education;
Sessions 5 to 8 (9 months) review of education material.
Outcomes1. Average number of calories consumed per day.
2. Weight (kg).
Assessments at baseline and follow up 1 year later.
NotesPOI: daily calorie intake.
Attrition: one family failed to complete follow-up assessment at 1 year. 3 familles withdrew after the third treatment session.
No significant difference between those that completed study and those that dropped out.
Groups were comparable with regard to age, parent's marital status and SES of family.
Equivalence at baseline doubtful.
Risk of bias
BiasAuthors' judgementSupport for judgement
Random sequence generation (selection bias)Unclear riskNot reported.
Allocation concealment (selection bias)Unclear riskNot reported.
Blinding (performance bias and detection bias)
All outcomes
High riskUnclear.
Incomplete outcome data (attrition bias)
All outcomes
Unclear riskThe authors recorded the drop-outs (33%) and presented the reasons. However, reasons for drop-outs are not reported separately for both conditions. They additionally reported that a comparison of children who withdrew from the study and those who completed the study protocol yielded no significant differences on demographic and anthropometric data' (Powers 2003 p. 304).

Powers 2005

MethodsParallel RCT.
Comparing behavioral and nutrition treatment (BEH) with usual standard care (CTL).
Cross-over from CTL to BEH offered after stage 1 of study finished with 3- and 12-month follow up.
Hypotheses:
1. BEH would improve child's energy intake relative to CTL;
2. improvements would be maintained for 12 months.
Computer-generated block randomisation code with independent blind allocation.
ParticipantsPopulation of interest N = 21 with n = 14 meeting criteria. Four withdrew or lost to follow up. Thus, n = 10 participants included aged 18 - 48 months with confirmed diagnosis of CF with pancreatic insufficiency; diagnosis for at least 3 months; and, on unrestricted fat diet. Exclusions for those with developmental delay; receiving supplemental enteral nutrition; an additional diagnosis or medication for condition known to affect growth; baseline diet record of 120% RDA or greater.
Interventions1. BEH (N = 4) nutritional counselling - targeted at 1 meal each week. 3 focus areas:
a. increasing calorie and fat intake;
b. dosage and timing of pancreatic enzymes;
c. teaching parent management skills.
Treatment over 8-week period with baseline at week 1 and 6; intervention sessions during weeks 3 - 6.
2. CTL (n = 6)
scheduled clinic visits every 3 months with CF and dietitian consulted whenever diet and growth issues were identified.
OutcomesChange in average energy intake % fat intake measured with 7-day diet diaries 1 week before intervention and 1 week after intervention (8 weeks apart).
NotesPOI: change in average energy intake per day over a 7-day period from pre- to post-treatment.
71% recruitment rate.
No significant difference between the groups at baseline for age, weight, height, sex, nutritional status ethnicity or marital status of caregiver.
Attrition: 2 families did not attend regularly and 2 declined to participate.
Risk of bias
BiasAuthors' judgementSupport for judgement
Random sequence generation (selection bias)Low riskThe authors described the randomisation process in detail. 'The computer-generated randomisation code was developed independently by the study biostatistician.' (Powers 2005, p. 1443)
Allocation concealment (selection bias)Low riskThe method of concealment was described in detail by the authors. They reported that a decentralised blinded research staff member then was given a copy of the list. Once a family was enrolled [...], another research staff member called the decentralised research staff member and obtained the random assignment.' (Powers 2005, p. 1443) The authors noted additionally that all relevant study team members were unaware of the specific allocation concealment.
Blinding (performance bias and detection bias)
All outcomes
High riskParticipants and personnel providing intervention could not be blinded to intervention as usual for psychological interventions. The primary outcome 'daily calorie intake' was assessed via self-reported daily diet dairies provided by the parents. The measure, which is not objective, might be influenced by the knowledge of allocation to one of the conditions.
Incomplete outcome data (attrition bias)
All outcomes
Low riskThe authors reported that ITT analysis has been conducted for the three-month follow up. Two families failed to complete the dietary record at this time point. Scores for the two participants were imputed 'by calculating the mean change in: (1) energy intake per day; (2) percentage of RDA; and (3) percentage of fat intake from post-treatment to 3-month follow up for the 7 completers. These mean changes then were used to calculate estimated 3-month follow up data for the 2 noncompleters' (Powers 2005, p. 1446). Furthermore, they reported that two other approaches for imputation revealed similar results.
Selective reporting (reporting bias)Unclear riskAll of the study’s pre-specified outcomes have been reported. It is unclear if additional outcomes were pre-specified in the study protocol but not reported.

Quinn 2004

Methods

RCT.

The effectiveness of brief telephone 'Motivational Interviewing' intervention in improving adherence to nebulised antibiotics in adults with CF has been evaluated (compared to treatment as usual).

Participants

Population of interest, N = 102 (patients prescribed nebulised colistimethate).

Patients who met eligibility, n = 47 (patients invited to the study).

Number of invited patients who declined, n = 8.

Prior to randomisation all participants were given a Prodose nebuliser and used it for a 3-month period to become accustomed to it and to generate baseline data on adherence.

Number of dropouts, n = 4.

Number of patients who received intended treatment, n = 35.

Inclusion criteria:

- participants were diagnosed with CF prior to the study (by 2 positive sweat tests or recognised CF genotypes);

- age > 18 years;

- attending regional CF clinic;
- prescribed Colistimethate sodium delivered via a ProdoseTM Adaptive Aerosol Delivery system nebuliser.

Exclusion criteria:

- lung function FVC < 30%;

- being involved in other trials;

- being pregnant.

Interventions

1. Treatment as usual plus brief 'Motivational Interviewing' intervention (n = 16).

Delivered via telephone by the principal investigator of the study. Was delivered over a 3-month period with up to 6 MI sessions (fortnightly). Audiotapes of intervention were supervised and checked to ensure treatment fidelity.
2. Treatment as usual alone (n = 19).

Outcomes

Primary outcome

Difference in adherence (to aerosolised antibiotics) pre- and post-intervention measured electronically monitored using Prodose. (Calculated for the previous 3 months in terms of the number of times the devise was used over the number of times its use was prescribed; 0-100+%). Lung function measured as FEV1 and FVC.
Secondary outcomes

Were measured with questionnaires for anxiety (STAI-Short Form; HADS-A), depression (HADS-D), and quality of life (CFQOL). Physiotherapists who administered questionnaires pre-randomisation and immediately post-intervention were blind to study allocation.

NotesTratement fidelity: the authors reported that audiotapes of the consultations were used in supervision, and were checked by GL to ensure treatment fidelity.
Risk of bias
BiasAuthors' judgementSupport for judgement
Random sequence generation (selection bias)Low riskThe authors provided the information that the participants were randomised using a computer generated pseudo-random number generator.
Allocation concealment (selection bias)Unclear riskParticipants were randomised using a computer generated pseudo-random number generator. But the authors did not provide information about adequate concealment of allocation.
Blinding (performance bias and detection bias)
All outcomes
Low riskParticipants and personnel providing the MI telephone intervention could not be blinded to intervention as usual for psychological interventions. Physiotherapists who administered questionnaires were blind to study allocation.
Incomplete outcome data (attrition bias)
All outcomes
Low riskThe authors provided the information that of the 39 participants randomised 4 dropped out during the study for the following reasons: possible side effects of the medication, preferring the previous nebuliser, receiving a lung transplant. Four participants were repeatedly unavailable, but were included in the intention to treat analysis.
Selective reporting (reporting bias)Unclear riskThe information is insufficient to judge whether 'low' or 'high' risk.

Stark 1996

MethodsParallel RCT with half participants receiving intervention first then other half 3 months later - not reported.
4 families changed group after randomisation - due to conflicting vacation scheduling. Thus not truly randomised.
ParticipantsN = 10.
1 withdrew from control group after randomisation.
Total sample n = 9.
Age range: 5.3 years to 10.1 years.
Mean age: 7.3 years (SD = 1.7).
Interventions1. Group behavioural intervention (n = 5).
7 weekly sessions - baseline assessment plus snack, breakfast, relaxation skills training, lunch, dinner and maintenance strategies targeted over following 7 sessions.
2. Wait list control (n = 4).
Parent meeting and 7-day food diaries at times corresponding to baseline and last week of intervention.
Outcomes1. Calorie intake.
2. Anthropometric measures - weight, height and skinfold.
3. PFT.
4. REE.
5. Physical activity - Caltrac electronic accelerator.
Assessments at baseline, then 3 months and 6 months post-treatment.
NotesUnclear what population the sample was drawn from.
POI: Daily calorie intake.
ITT - unclear.
Blinding - unclear.
No significant difference on z scores for weight or age before treatment.
Control group average SES group II - technical workers.
Intervention group average SES III - skilled labour.
Equaivalence at baseline doubtful.
Risk of bias
BiasAuthors' judgementSupport for judgement
Random sequence generation (selection bias)Unclear riskThe authors did not describe details of random generation process. It is just stated that 'the nine subjects were randomly assigned to either a behavioral intervention or a wait list control group' (Stark 1996).
Allocation concealment (selection bias)Unclear riskThe authors did not provide information about adequate concealment of allocation.
Blinding (performance bias and detection bias)
All outcomes
High riskParticipants and personnel providing the intervention were not able to be blinded due to the nature of the intervention and the study design (wait-list-control design). The main outcome 'calorie intake' is assessed via self-reported daily diet dairies. Due to that the measure is not objective and might be influenced by the knowledge of allocation to one of the conditions. All other objective measures (e.g. weight) are not likely to be influenced by the lack of blinding.
Incomplete outcome data (attrition bias)
All outcomes
Low riskThe authors reported that there was no attrition.
Selective reporting (reporting bias)Unclear riskThe authors reported alls pre-specified outcomes. It is unclear if additional outcomes were pre-specified in the study protocol but not reported.

Stark 2003

MethodsParallel RCT.
Comparing calorie intake and weight gain in 2 group interventions.
Hypothesised that those receiving a behavioral intervention would eat more calories and gain more weight.
ParticipantsPopulation of interest, N = 10.
3 dropped out.
Total sample, n = 7.
Children aged 4 to 12 years with CF and their parents.
Children had chronic pulmonary disease, pancreatic insufficiency and weight below the 40th percentile.
Mild disease, chronic phase.
InterventionsGroup educational & behavioural interventions:
1. nutritional education (n = 4);
2. behavioural intervention (n = 3).
7 sessions: baseline, 5 sessions (90 min each) plus 1 follow up session.
Outcomes1. Calorie intake.
2. Weight.
3. Quality of parent/child interaction at mealtime.
4. CF coping skills.
5. Adherence to CF regimen.
Assessments at baseline, post-treatment and 6, 12 & 24 months follow up.
NotesPOI: daily calorie intake.
Unclear what the population of interest was and how sample was selected.
Attrition - 3 dropped out. One spoke only Spanish. 1 had 3 children with CF, 1 of whom was hospitalised. 1 had conflicting time commitments.
Equivalence at baseline unclear but unlikely.
Risk of bias
BiasAuthors' judgementSupport for judgement
Random sequence generation (selection bias)Low riskThe authors described that families were assigned based on family availability to time slots, which were in a second step then randomly assigned to one of the conditions 'via a coin flip' (Stark 2003, p. 244).
Allocation concealment (selection bias)Low riskSee above.
Blinding (performance bias and detection bias)
All outcomes
High riskParticipants and personnel providing the intervention were not able to be blinded due to the nature of the intervention. One primary outcome 'daily calorie intake' is assessed via self-reported daily diet dairies. Due to that the measure is not objective and might be influenced by the knowledge of allocation to one of the conditions. The other objective measure used as primary outcome ('weight') is not likely to be influenced by the lack of blinding.
Incomplete outcome data (attrition bias)
All outcomes
Unclear riskThe authors described in detail the attrition and the reasons for families who dropped out, but it is relatively unclear what the population of interest was and how many families were randomly assigned. Out of originally 15 families, who agreed to participate, seven families completed treatment and follow up (Stark 2003, p. 240).
Selective reporting (reporting bias)Low riskAll prespecified outcome variables (primary and secondary) are reported.

Stark 2009

Methods

RCT.

Compared behavioral intervention plus nutrition education to nutrition education alone to improve caloric intake and weight in children with CF.

Hypothesis: Children receiving the behavioral plus nutrition education intervention would have a significantly greater increase from pre-treatment to post-treatment and through 24-month follow up on primary and secondary outcomes.

Participants

Population of interest, N = 177 (met eligibility).

Number randomised, n = 79.

Number of participants received the intervention = 67.

group 'nutrition education', n = 34.

group 'behavioral intervention plus nutrition education', n = 33.

There were 6 drop outs in both arms prior to treatment, leaving 67 participants for analysis.

Recruited from 5 CF centres located in the Eastern, Midwestern, and Southern USA.

Children from 4-12 years with a diagnosis of CF by sweat test, pancreatic insufficiency; and weight for age and height ≤ 40th percentile.

Interventions

Behavioral intervention and nutrition education in group setting.

1. Nutrition education (n = 34),

2. Behavioral intervention for change around nutrition an energy (Be-In-CHARGE!; n = 33) (available online at www.oup.com/us/pediatricpsych).

7 sessions (each 90 minutes): pre-treatment (session 1), 2 weeks later 5 weekly groups sessions (sessions 2 to 6), 2 weeks later post-treatment (session 7; follow up).

Outcomes

Primary Outcomes

Change in caloric intake and weight pre- to post-treatment.

Secondary outcomes

%EER (percentage of the estimated energy requirements; calculated by subtracting EER for an active child of same age and gender from individual subject's calorie intake X 100), BMIZ (BMI z scores), weight, %EER, BMIZ (assessed at baseline and post-treatment) and additionally height, HAZ, and FEV1 were examined at 24 months following treatment.

Caloric intake was assessed at baseline, post-treatment, 3, 6, 12, 18 and 24, months follow up.

Notes

In one report effects of the intervention on 'Family Interactions at Mealtime' were reported, but data was not sufficient. For detailed information on this specific outcome the leading author has been contacted and we are waiting information.

Treatment fidelity: the authors reported that 'treatment fidelity was assessed by raters coding 4 videotapes from each of the 7 sessions for each intervention.' (Stark 2009, p.917).

Risk of bias
BiasAuthors' judgementSupport for judgement
Random sequence generation (selection bias)Low riskParticipants were 'randomised to the treatment arms by coin flip by research assistant and postdoctoral fellow together' (Stark 2009, p.916).
Allocation concealment (selection bias)Low riskAssignment could not been foreseen by participants and investigators enrolling participants because of coin flipping by research assistant and postdoctoral fellow together.
Blinding (performance bias and detection bias)
All outcomes
Unclear risk

The authors of the study state that 'families were never explicitly told which treatment they had been assigned' (Stark et al 2009, p.916). But, 'as with any behavioral intervention, it is not possible to keep subjects unaware of the treatment they are receiving or therapists the treatment they are providing' (Stark 2009, p.921).

No details are provided about blinding of outcome assessors.

Incomplete outcome data (attrition bias)
All outcomes
Low risk

Of the 79 enrolled children 40 were assigned to the nutrition education group (NE) and 39 to the behaviour plus nutrition education group. There have been 6 drop outs in both arms prior to treatment. Data of 67 children was available for analysis post-treatment (NE n = 33 and behavioural plus nutrition education intervention n = 34). 24 month follow-up data of 28 children in the behaviour plus nutrition education intervention group and of 31 children in the NE group was available for analysis.

The authors provided a flow diagram of participants randomised to both study arms and assessed at each point in time from baseline to 24-month follow up (see Stark 2009, p.916 Figure 1).

Selective reporting (reporting bias)Low riskThe study protocol is available and all of the study’s pre-specified outcomes have been reported.

Taylor 2003

Methods

RCT.

Compared written self-disclosure intervention to standard care (wait-list control condition).

Hypothesis: patients who completed the written self-disclosure procedure would evidence improvement in health care utilization, disease severity and subjective health status 3 month following the intervention.

Participants

Population of interest: N = 81 (met eligibility).

Number randomised: 70.

Number of participants received the intervention: 70.

Dropouts: 14.

Excluded from data analysis because of unusual health care utilization patterns: 17.

Final sample size: 39, intervention group (WSD) n = 18, control group (SMC) n = 21.

Recruited from 2 CF centres located at 2 children’s hospitals in the southeastern part of the USA.

Initial eligibility criteria included: (1) a diagnosis of CF; (2) age at least 15 years; (3) enrolled in the CF clinic for at least 9 months; (4) physical and mental, ability to complete the research protocol as judged by, the project staff and CF physician or nurse; (5) willingness, to write for 20 min on 3 separate occasions, over a 5-day period and completion of self-report, measures on two occasions over the next 3 months; and (6) access to a telephone on the days that the participants were to write at home.

Interventions

Written self-disclosure intervention:

3-sessions written self-disclosure intervention, adapted from Pennebaker's laboratory-based protocol

First session in a private room within the CF clinic, two home-writing sessions prompted by a telephone call (each 20 minutes).

Instructions were in accord with those of Pennebaker (1989, 1993, 1997) and required that participants write about their "deepest thoughts and feelings about the most distressing experience of their entire life for a period of 20 minutes" (Pennebaker 1997).

Outcomes

Health care utilization: number of (1) outpatient visits; and (2) inpatient hospitalizations days.

Physiological disease severity: (1) FEV1; (2) BMI.

Subjective health status: (1) 'The Patient Health Questionnaire' (PHQ; Spitzer Taylor, Wallander, Anderson, Beasley, and Brown et al 1994; Spitzer, Kronke, Williams et al 1999): symptoms of (a) depression; (b) anxiety; (c) somatic complaints; and (d) psychological distress. (2) 'The Stressful Life Events Scale': 10 psychosocial complaints common among health care seeking populations (e.g., difficulties with family support, problems with significant others, and financial concerns); (3) SF-12: severity and frequency of 12 physical and mental health problems, as well as of their impact on the patients' overall perceived health status.

Feasibility and acceptability: modified version of the 'Visit Specific Satisfaction Questionnaire' (VSQ; Ware & Hays 1988).

Notes 
Risk of bias
BiasAuthors' judgementSupport for judgement
Random sequence generation (selection bias)Low riskThe authors provide insufficient information about the sequence generation. After contacting the authors they provided the information that they followed a published random numbers sequence and assigned the patient to one of the two treatments based on the next number in the sequence being odd or even.
Allocation concealment (selection bias)Unclear riskThe authors provide insufficient information on the allocation concealment.
Blinding (performance bias and detection bias)
All outcomes
Low riskThe physician and the clinic staff were not informed as to whether individual patients were in the WSD or the SMC condition. Patients and providers of the intervention knew who was in the WSD or the SMC condition (Taylor 2003).
Incomplete outcome data (attrition bias)
All outcomes
Unclear riskNumber of drop-outs in each group is not reported, demographic comparison data between those excluded and included in the final sample, using T-tests and chi-square analyses, revealed no significant differences between the two groups.
Selective reporting (reporting bias)Low riskAll of the study’s pre-specified outcomes have been reported.

Vandemheen 2009

Methods

Single blind RCT with 1-year follow up.

Compared an evidence-based decision aid for patients with advanced CF considering referral for lung transplantation to usual care.

Primary objectives: to assess whether the decision aid increased knowledge about patient options, improved realistic expectations, and decreased decisional conflict.

Participants

Population of interest: N = 155 (assessed for eligibility).

Number randomised: 149.

Number of participants received the intervention: 148 (intervention group n = 70; control group n = 79).

Final sample size after 1-year follow up: N = 125 (intervention group n = 60; control group n = 65).

Recruited from nine Canadian and five Australian outpatient CF centres.

Eligibility criteria:

1.  > 18 years of age;

2. FEV1 ≦ 40% predicted;

3. Able to read English or French.

Exclusion criteria:

1. patients who previously received a lung transplant;

2. patients on lung transplantation waiting list.

Interventions

All study patients received an educational session on the process of referral and the risks and benefits of lung transplantation from their CF physician and/or CF team. All patients received a copy of a pamphlet entitled 'Cystic Fibrosis and Lung Transplantation' and were provided addresses to access two generic websites on lung transplantation.

After randomisation:

Intervention group: Received a sealed package containing a paper version of the decision aid and a website address with a user name and password, where they could access the decision aid online (http://decisionaid.ohri.ca/decaids.html).

Control group: received a package contained blank pages and a letter explaining why blank pages were included.

Outcomes

The following primary outcomes were assessed at baseline and 3 weeks after randomisation:

participants’ knowledge and realistic expectations with designed questionnaire (structured format as per the International Patient Decision Aids Standards Guidelines); decisional conflict scores (Decisional Conflict Scale, a validated 16-item scale).

Notes 
Risk of bias
BiasAuthors' judgementSupport for judgement
Random sequence generation (selection bias)Low riskThe authors reported that randomisation was performed via a "a randomization schedule prepared through a computer-generated random listing of the two treatment allocations blocked in variable blocks of two or four and stratified by site and by infection status with Burkholderia cepacia" (Vandemheen 2009, p.762).
Allocation concealment (selection bias)Low riskThe authors reported that a central allocation was conducted (Vandemheen 2009, p. 762).
Blinding (performance bias and detection bias)
All outcomes
Low riskThe authors provided the following information regarding blinding: "Neither research staff nor medical staff as aware of the treatment assignment before and after randomization" (Vandemheen 2009, p.762).
Incomplete outcome data (attrition bias)
All outcomes
Low riskThe number of drop-outs for 3-week follow up and 12-month follow up were reported for each group.
Selective reporting (reporting bias)Low riskThe published report includes all expected outcomes, including those that were pre-specified as primary and secondary outcomes.

Wilkinson 2008

  1. a

    ACT: airway clearance techniques
    BEH: nutritional and behavioural intervention
    BMI: body mass index
    CCT: controlled clinical trial
    CF: cystic fibrosis
    CFK: CF knowledge
    CPT: chest physiotherapy
    CTL: control condition
    FEV1: forced expiratory volume at one second
    FVC: forced vital capacity
    HLOC: health locus of control
    KG: kilogram
    ITT: intention-to-treat
    LOC: locus of control
    Mean FEF25-75%: forced expiratory flow during the middle of the maneuver
    NIH: National Institute of Health
    PEFR: peak expiratory flow rate
    PFT: pulmonary functioning
    POI: primary outcome indicator
    POMS: profile of mood states
    QOL: quality of life
    RCT: randomised controlled trial
    RDA: recommended daily allowance
    REE: resting energy expenditure
    RISCS: role play inventory of situations and coping skills
    SD: standard deviation
    SES: socioeconomic status
    STAI: state trait anxiety inventory
    STAIC: state trait anxiety inventory (children)

Methods

RCT.

Prospective pilot study to investigate the feasibility of a video link to support patients on the transplantation waiting list and their families.

Participants

Patients on the transplantation list, at least 16 years of age, with a confirmed diagnosis of CF and willing to have an ISDN line installed in their home.

Number randomised: 16, median age 27 years (range 21 – 41).

Number of participants completed the intervention: 7 (4 on telemedicine and 3 in the control arm).

Of those who did not complete the study four patients died, three patients received a transplant, one withdrew following randomisation and one was too unwell to continue.

Interventions

Telemedicine additional to standard care: the participants were provided with an ISDN line to their home and a videoconferencing unit was connected to their home television set. Participants were also given a micro-spirometer, pulse-oximeter and a supply of single use clinical thermometers. Contact was made, on a weekly basis, at a time agreed by the patient and assessor (senior physiotherapist or nurse consultant). The topics which were discussed included: non-invasive ventilation; haemoptysis; physiotherapy and amount of sputum; mobility; difficulties with any clinical procedures; appetite and weight; and any other problems as appropriate.

Control group: standard medical care.

Outcomes

1. 'Cystic Fibrosis Quality of Life Questionnaire'.

2. 'Beck Anxiety Inventory'.

3. 'Beck Depression Inventory'.

4. Seven-point questionnaire which included the number of visits to the cystic fibrosis clinic, general practitioner (GP), courses of intravenous antibiotics, length of hospital inpatient stay and how many visits were made either to a hospital or to the GP.

5. 'Borg Anxiety Scale', which was completed before and after each clinic attendance and after telemedicine link-up.

6. Coping of carers: COPE questionnaire (Carver 1997).

Patients and their caregivers were asked to complete a telemedicine satisfaction questionnaire on completion of the study.

NotesAuthors were contacted for detailed quantitative data on outcome measures, but we did not receive a response within the time updating the review.
Risk of bias
BiasAuthors' judgementSupport for judgement
Random sequence generation (selection bias)Low riskParticipants were randomised by a physiotherapist distributing a pre-prepared sealed envelope, which was made by a third party not involved with recruitment (Wilkinson 2008, p. 183).
Allocation concealment (selection bias)Low riskParticipants and investigator could not foresee assignments because the authors reported that they used sequentially numbered sealed envelopes (Wilkinson 2008, p.183).
Blinding (performance bias and detection bias)
All outcomes
Unclear riskDue to the nature of the intervention the participants and teams providing the intervention could been blinded, but he authors provided no information on blinding of outcome assessment.
Incomplete outcome data (attrition bias)
All outcomes
High riskThe authors reported a high number of dropouts. The number of dropouts for each group is unclear. Reasons for dropouts were reported: 4 patients died, 3 patients received transplant, 1 withdrew, and 1 was too unwell to continue (Wilkinson 2008, p. 183).
Selective reporting (reporting bias)High riskMeans and SDs for all outcome parameters for intervention and control group were not reported in the published article.

Characteristics of excluded studies [ordered by study ID]

StudyReason for exclusion
  1. a

    CF: cystic fibrosis
    TSD: Tay Sachs Disease

Barbero 1996This is a lecture with a broad remit and does not describe an intervention study.
Bartholomew 1997This was pre-post design - participants were not randomised to the treatment and comparison groups.
Bingham 2010This is a study on breath biofeedback - the study had no comparision group (no RCT design).
Brady 1991The intervention is neither psychological nor does it have a psychosocial focus.
Brandt 1996This is not an intervention study - it is a cross-sectional survey.
Brown 1994This is not an intervention study - it is a case-control study without a psychosocial focus.
Castellani 2011This is a study on an interactive computer-based program for education in cystic fibrosis carrier tests. The participants were not a population of interest (participants did not have CF or were family members of patients with CF).
Cheuvront 1998This is a parallel RCT to evaulate two approaches to CF gene pre-test education and carrier testing. Genetic clinic education and testing versus Home-based education and testing. The approaches were both educational which are by defintion not eligible for this update of the review.
Clayton 1995This a study of population screening. The participants do not have CF nor related to anyone with CF - they were also not CF carriers and had no higher than the average risk of being a carrier.
Davis 2002This is a parallel RCT comparing an educational intervention using a CD-ROM with a wait list control. The approach was educational which is by defintion not eligible for this update of the review.
Dodd 1997This is not an intervention study - it is a study validating a psychological measure on a CF population.
Dodd 2001This is a study on motivational interviewing for exercise performance in patients with cystic fibrosis. Data has not been published in a journal article and is not available via the author anymore.
Donaldson 1995This study is a survey of attitudes and a comparison of 2 approaches to population screening. The participants do not have CF nor are they related to anyone with CF - there is no higher risk of their being a carrier than average.
Downs 2006This is a multi-centre parallel RCT investigating the effectiveness of a self-management education programme called ’Airways’. The approach was educational which is by defintion not eligible for this update of the review.
Eng 1997This was a study of attitudes towards screening for CF in a population of Askenazi Jews referred for TSD testing. There was no comparison group and no higher risk of being a CF carrier than average.
Falkman 1977This is not an intervention study - it is a descriptive study of a random sample of children with CF.
Gee 2000This is not an intervention study - it is a report of a part of the development of a psychological measure for CF.
Georgiopoulos 2011This is a study on ADHD treatment. It is a chart review.
Goa 1997This is not an intervention study it is a review of the literature. The intervention reviewed is not psychological although the reported outcomes are psychosocial.
Goldbeck 2001This study has a pre-post design - there is no comparison group.
Goldbeck 2011aThis is a study on an inpatient rehabilitation program (including psychological intervention), but there was no control group for comparing outcomes of the program.
Gonciartz 1988This study is not concerned with CF although some of the participants may have had CF. The intervention is not psychological nor does it have a psychosocial focus.
Green 1995This is a report of a survey of obstetricians views and not an intervention study. The participants do not have CF nor are they related to anyone with CF although they may have a professional relationship with someone with CF.
Gremse 1998This is a descriptive case-control study and not an intervention study.
Grody 1997This is a study of population screening - the participants have no history of CF and no higher than the average risk of being a carrier for CF.
Gulmans 1999There was no comparison group in this study.
Haddow 1999This was not an intervention study - it was a summary of the views of professionals who were attending a conference regarding screening for CF.
Hains 2001This intervention study has no comparison group.
Hains 1997This intervention study has no comparison group.
Hodson 1997This is a descriptive case-control study - it is not an intervention study.
Jelalian 1998This is not an intervention study - it is a review of the literature and meta-analysis of interventions for CF. Only 1 of the studies described is a RCT and that is reported separately here (Stark 1996).
Johnson 2001This intervention study has no comparison group.
Kollberg 1982This is a descriptive account - it is not an intervention study.
Krauth 1999The intervention in this study was not psychological nor did it have a psychosocial focus.
Le Heuzey 1997This is not an intervention study - it is a descriptive single group assessment psychological adjustment.
Leonard 1995This is not an RCT it used a quasi experimental design to compare two approaches to education information about carrier testing for CF. The participants do not have CF nor do they have any higher risk of being a carrier than average.
Lindström 1994This is not an intervention study - it is a descriptive single group study and it is not wholly concerned with CF although some of the participants had CF.
Loader 1996This was a study of attitudes and responses to population screening - the participants did not have CF nor were they and higher risk of being a carrier. Of the group identified as carriers there was no comparison group and no psychological intervention was offered.
Magnussen 1992This study was not wholly concerned with CF - it was one of several lung diseases investigated. The intervention was not psychological nor did it have a psychosocial focus.
Phillipson 2000This study had no comparison group and the intervention was not psychological nor did it have a psychosocial focus - no psychosocial outcomes were included.
Pollitt 1997This is not an intervention study it is a systematic review of the literature with respect to the cost-effectiveness of population screening. There is no review of psychological interventions nor is there a psychosocial focus.
Pop Jordanova 2010This is a study on 'Biofeedback', but there was no control group which did not receive the intervention.
Ralston 2008This is a study on of smoking cessation counselling for parents, but CF parents were not a major subgroup of the study sample. No information about the number of participating CF parents is provided.
Ramström 2000Although this study did not have a psychological intervention there was a psychosocial focus. It was however considered to be more concerned with service delivery.
Rault 2012This is a study investigating a CF quality improvement program. It is a pilot study testing the applicability and effectiveness of Clinical Microsystems Approach to improve CF care in France. No control condition.
Rodrigue 2005This study is a study on quality-of-life therapy for patients awaiting lung transplantation. The study included mostly patients with COPD (only 2 patients with CF).
Rozenfeld 2012This is a study investigating Snoezeln in infants ages 0-3 years with CF in a single centre without a comparator.
Stapleton 2001This is a parallel RCT comparing the effectiveness of an educational programme with standard care for knowledge about nutrition. The approach was educational which is by defintion not eligible for this update of the review.
Stark 2002Altough the article describes outcomes of a RCT study, only data for intervention group was collected and is provided for the outcome 'Calcium intake'.
Thompson 1990This is not an intervention study - it is a cross-sectional, controlled, descriptive study of psychological adjustment in children with CF.
Thornton 1995This is a study comparing three methods for conveying information about population screening. The participants do not have CF nor do they have any higher risk of being a carrier than average.
Trapp 1998This ia parallel CCT of 2 interventions with a convenience control group sample to investigate whether self-administration of drugs leads to greater knowledge about medications. The approach was educational which is by defintion not eligible for this update of the review.
Tullis 1995This is not an intervention study - it is an argument for a CF specific psychological measure.
Villari 1994This is not an intervention study and it is not concerned with CF although some of the participants may have had CF. There is no psychosocial focus to this cross-sectional case-control study.
Ward 1999This is not an intervention study - it is a descriptive study comparing three groups with respect to energy expenditure.
Watson 2004The previous ongoing study is now published as a randomized controlled trial of a new behavioral home-based nutrition education program, "Eat Well with CF," in adults with cystic fibrosis. The approach was educational which is by defintion not eligible for this update of the review.
Watson 2006The previous ongoing study is now published as a randomized controlled trial of a new behavioral home-based nutrition education program, "Eat Well with CF," in adults with cystic fibrosis. The approach was educational which is by defintion not eligible for this update of the review.
Welkenhuysen 1996This is not an intervention study - it is a survey of attitudes towards population screening in adolescents. The participants do not have CF nor are they related to anyone with CF. They have no higher risk of being a carrier for CF then average.
Williams 1997This intervention study does not have a comparison group.
Wolter 1997This is not a psychological intervention albeit with a psychosocial focus. It was considered to be more concerned with service delivery.

Characteristics of studies awaiting assessment [ordered by study ID]

Bryon 2000

MethodsAssessed a new developed home visit program to improve health status and psychosocial functioning, randomised allocation to intervention group vs waiting list control group.
ParticipantsChildren with CF, are from families with significant psychosocial stresses (consequences: sub-optimal treatment, irregular clinic attendance, erratic health status).
InterventionsProgram included at least four home visits from both nurse and psychologist (in addition to routine nurse specialist input) over a six-month period.
Outcomes'General Health Questionnaire', 'Parenting Stress Index', 'Harter self-esteem' (for children aged over 8 years), pulmonary function (FVC, FEV1), Take-up of clinic appointments, drug prescription and planned admissions.
NotesPublished as an abstract.

Cannon 1999

MethodsExplored the feasibility and benefit of in-home videoconferencing using inexpensive equipment interfacing to a patient’s standard telephone and television set, 2 groups (study vs control).
Participants10 patients aged 6 - 13 years.
InterventionsStudy group received reinforcement of the education message from each clinic visit with weekly in-home videoconferencing contacts by nurse, TR dietitian, or social worker, plus routine clinic education; control group received only routine clinic education.
OutcomesPFT, weight triceps skin fold thickness, mid-arm muscle circumference, prealbumin, a simple test of basic CF knowledge, the Hopelessness Scale for Children, the Family Empowerment Scale, Client Satisfaction Survey.
NotesPublished as abstract.

Cummings 2011

MethodsPilot study that aimed to assess a mentor-based behavioural modification intervention to enhance self-efficacy for self-management skills and QoL, single-blind RCT, 3 groups (mentor vs mentor plus mobile phone vs control).
Participants17 adolescents and adults aged 14 - 48 years.
InterventionsMentor-based behavioural modification intervention with mentorship and self-monitoring via a modified mobile phone with immediate visual feedback on clinical status. Duration: 6 months.
Outcomes

Primary outcome
Self-efficacy.

Secondary outcomes
CF-related and generic QoL, participant feedback on their experience via semi-structured interviews, lung function and acute healthcare utilization.

NotesPublished as abstract.

Davis 1990

MethodsExamined the psychosocial effects, safety and efficacy of HIVAT comparing to conventional hospital therapy over short term (one exacerbation).
Participants78 CF patients with acute exacerbation (1 dropout).
InterventionsHome IV antibiotic therapy as part of a multicentre randomised study, all patient were treated with IV tobramycin and ceftazidime every 8 hours (one exception: tobramycin and piperacillin).
Outcomes

Self-concept (< 18: PH; Adults: TSC), Anxiety (8th grade and under: STAIC, all other: STAI), CHIP

Weight, resting respiratory rate, NIH score, FEV1, FVC.

NotesPublished as abstract.

Goldbeck 2013

Methods 
Participants

151 CF patients aged 6 years or older recruited in 3 CF outpatient clinics.

300 patients from other CF centres receiving usual care and matched for age, sex and lung function were recruited as controls.

Interventions

Follow-up period: 2 years.

Patients in intervention group: program based on 'Individual Treatment Agreements' (ITA) between patient or caregiver and CF physician.

ITA's may focus on medical, nutritional and psychotherapeutic measures.

Outcomes

Primary outcome

Quality of care as assessed by the frequency of BMI and lung function measurements per year.

Secondary outcomes
Range of medical (mortality, lung function, BMI) and psychosocial (QoL, adherence, caregiver distress).

Use of economic resources also assessed

NotesCommenced clinically in May 2012. Co-funded by the German Ministry of Health and the German CF-association Mukoviszidose e.V.

Hass 2012

MethodsRandomised, open-label, parallel study on the effects of massage therapy on QoL in youth/young adults with CF.
Participants

Diagnosis of CF.

Ages: 8 - 21 years.

Estimated enrolment: 24.

Interventions

Active Comparator: massage therapy. Treatment group receives pre-determined massage therapy protocol x 5 over 10 - 12 weeks. massage therapy protocol includes a blend of Swedish strokes and myofascial trigger point therapy. Initially, dosing will be more frequent. Treatments will be spaced out to determine the ability of the body to maintain a more efficient musculoskeletal system, especially related to respiratory and postural efforts. Each session will end with resting hands and relaxation strokes to signal the end of the session. This protocol invites increased mobility in the musculoskeletal system. The ultimate goal is to return connective tissue (including muscles and fascia) to a more relaxed and neutral state, thus allowing expansion and ease of movement of the areas of the musculoskeletal system being worked.

Control group: no intervention.

Outcomes

Primary outcome measures

QoL

Secondary outcome measures
Pulmonary function

Other outcome measures

Pain

Notes

Study start date: July 2013; estimated completion date: December 2014.

Country: USA.

Hatziagorou 2011

MethodsCompares behavioral and nutritional intervention (BEH) with a usual care control condition (CTL) for CF children with pancreatic insufficiency.
ParticipantsTwenty-four children with CF (aged 8 months to 18 years) were assigned to the BEH group.
InterventionsBEH included nutrition counselling to increase energy intake and child behavioral management training.
OutcomesEnergy intake (calories per day), weight gain, BMI z scores.
NotesPublished as abstract. Contact to leading author. Detailed data is not published.

Huang 2010

MethodsRandomised open-label, parallel study investigating the use of texting to promote chronic disease management.
Participants

Enrolment: 81.

Ages: 14 to 22 years.

Have CF, type 1 diabetes, or inflammatory bowel disease for at least 6 months.

Interventions

Experimental: SMS and Internet. The SMS and Internet group will receive information, tips, strategies, and questions related to the self management of chronic disease (cystic fibrosis, inflammatory bowel disease, or type 1 diabetes) on a web-based program and via SMS messages.

No intervention: control. The control group will receive monthly tip sheets on various health topics for adolescents and young adults.

Outcomes

Primary outcome measures

1. Health related self-efficacy

Secondary outcome measures

1. Health knowledge

2. Disease health knowledge
3. Health literacy
4. QoL

Notes

Start date: October 2009; study completion: March 2012.

Country: USA.

Irons 2012

MethodsRCT.
Participants51 hospitalized children (mean age¼ 11.6 years, 35% male).
InterventionsParticipants undertook 8 singing or 8 recreational sessions.
OutcomesRespiratory muscle strength using maximal inspiratory and expiratory pressure (MIP and MEP, respectively), spirometry, and QoL were assessed at baseline, postintervention, and follow up.
NotesBrisbane, Australia.

Jessup 2010

Methods

RCT.

Pilot study that aimed to assist patients to achieve increased levels of self-efficacy through interactions with volunteer mentors coupled with technology supported self-monitoring.

ParticipantsAdolescents and adults with CF.
InterventionsVolunteer mentors coupled with technology supported self-monitoring.
Outcomes

QoL.

Clinical outcomes measures.

Feedback on all aspects of the intervention: the process, including the mentor relationship with the help of semi-structured interviews

NotesPublished as abstract. Contact to the leading author MJ - data is not published, yet.

Kalnins 1996

Methods 
ParticipantsN = 13.
Over ten years of age, less than 90% ideal weight for height or 5% reduction in weight over a three month period.
Exclusion criteria: routine use of supplements, liver disease, insulin dependent diabetes and oxygen dependence.
Interventions1. Dietary counselling.
2. Nutritional supplement - Boost Plus over a three month period.
Outcomes1. Pulmonary function FEV1.
2. Nutritional status:
a. weight;
b. height;
c. weight for height;
d. calorie intake;
e. calorie intake; as per cent of predicted energy needs.
Notes 

Klig 1989

MethodsEffectiveness and psychosocial advantage of a self-administered method of pulmonary therapy. FET was compared to CPT in a 2-year prospective study.
Participants50 patients with mild to percussion lung disease, age from 8 - 22 years
InterventionsFET included postural drainage, diaphragmatic breathing and forced expiration. CPT entailed postural drainage, percussion and vibration.
OutcomesFET/CPT compliant, noncompliant PET`s, clincal scores, weight, x-ray scores.
Notes 

Mischler 1998

MethodsComprehensive RCT to study both benefits and risks of newborn screening and to determine if early diagnosis would improve the prognosis of children with CF. Compares the nutritional status of patients with CF identified by neonatal screening or by standard diagnostic methods.
Participants650 340 infants, 135 families of children diagnosed as having CF, 206 false-positive families.
Interventions 
OutcomesHeight percentiles, weight percentiles, head-circumference percentile, anthropometric indexes. Assessments of reproductive knowledge, attitudes and behaviours.
Notes 

Patel 2010

Methods

Parallel randomised controlled pilot study, single centre study.

Research question: Effect of incorporating the CLCF-Q into the annual review process.

To assess the impact of a PROM on the Self Efficacy (SE) of parents and carers of children with CF.

ParticipantsParents/carers of children with CF.
InterventionsParticipants in the intervention group complete the CLCF-Q. They receive feedback at their next clinic appointment from the team in the form of colour coded tables illustrating both the positive and negative issues raised in the CLCF-Q. They are invited to talk to members of the CF team and others (consultants, CF nurses, dietitians, physiotherapists, psychologists, pharmacists, family doctors and the school) about the issues raised in the CLCF-Q.
Outcomes

SE-Questionnaire (SE-Q) as an outcome measure.

Participants complete the SE-Q at 2 time points. Between the two time points the intervention group received the intervention.

NotesInformation is retrieved from an abstract. Information from the author: Data is not published, yet.

Petzel 1991

MethodsThe study use several objective adherence indices to evaluate adherence to medical/behavioral interventions across a broad-age range of nonhospitalized CF patients, identify psychosocial attributes of patients and families associated opportunities for adherence-promoting interventions.
ParticipantsParents of 45 children with CF
Interventions

Health education intervention.

3 interventions:

- problem-solving lesson in computerized form;

- problem-solving lesson in written form.

OutcomesSociodemographic data, psychological test results, measures of the child health status (% predicted wt., x-ray scores), compliance.
Notes 

Powers 2003a

MethodsClinical study that compares a behavioral intervention to standard of care over a 2-month period
ParticipantsChildren ages 18 months to 4 years.
InterventionsBehavioral interventions to standard of care, and separate multiple baseline studies across meals (RDA guidelines).
OutcomesChange in calorie intake per day.
NotesInformation is retrieved from Symposium Session Summary.

Powers 2013

MethodsRCT. Children from 7 CF centres.
ParticipantsChildren (aged 2 - 6 years) with CF and pancreatic insufficiency.
Interventions

Behavioural and nutritional treatment versus an attention control group (CTL). Treatments occurred weekly for 8 weeks and then monthly for 4 months. Participants returned to standard care and a 12-month follow up was conducted.

CTL provided education and served as a behavioural placebo.

OutcomesDaily energy intake (7-day, weighed diet diaries) and weight for age z score change from baseline to post-treatment (6 months) and height for age z score change from baseline to follow up (18 months).
Notes

ClincialTrials.gov: #NCT00241969.

USA.

Ruddy 2011

MethodsRandomised, open-label, parallel study.
Participants

Estimated enrolment: 20.

Ages: 12 - 25 years.

Interventions

Active comparator: yoga (immediate start) group. This arm will start yoga sessions immediately after screening and will continue sessions for 8 weeks. This group will then continue with home yoga for an additional 8 weeks.

Active comparator: Yoga (waitlist) group. This arm will continue regular CF therapies for 8 weeks and will start yoga sessions at week 9.

Outcomes

Primary outcome measures

1. Number of participants with adverse events as a measure of safety

2. Pain scores on the Visual Analog Scale as a measure of tolerability

Participants will be given a tolerance questionnaire after each yoga session which will assess their degree of musculoskeletal and chest pain on a visual analog scale. Vital signs will also be measured before and after each yoga session.

Secondary outcome measures

1. QoL

2. Respiratory symptoms
3. Treatment adherence

4. Pulmonary function

5. Ease of breathing
6. BMI

Notes

Start date: February 2011, estimated end date: October 2011.

Country: USA.

Stark 1998

MethodsMulti-site, clinical study examining two innovative approaches to diet education.
Participants33 children between ages 4 and 12 years have completed treatment and 26 of these have completed the 6-month follow up.
InterventionsNutrition Education (NE) addresses the issue of lack of dietary knowledge by providing families an intensive 7-session program on nutritional
suggestions for meeting the CF RDA for energy on a meal by meal basis.
Behavioral Education (BE), combines the nutritional education component, as
described, above plus training on behavioral child management skills such as contingent attention to appropriate eating, ignoring behaviours incompatible with eating, and use of contingent privileges for meeting calorie goals.
OutcomesAt post treatment the children in the BE groups demonstrated a significantly greater increase in caloric intake than the children in the NE group t(30) = 2.24, P = The BE group also showed a significantly greater weight gain compared
to the NE. At the 6 month follow up both groups demonstrated a similar rate of weight gain from post treatment. Differences on weight gain post treatment to 6 months and pretreatment to 6 months were not significant
between the two groups.
NotesInformation is retrieved from Symposium Session Summary.

Wainwright 2009

Methods

RCT.

Pilot study of an education and behavioural adaptation program (delivered via a mentorship system, telephone and IT tools) designed to enhance self-management in adolescents.

Participants46 adolescents aged 12-19 with CF.
Interventions

3 groups: standard care (controls; N = 15); standard care + phone mentoring (N = 16); standard care, phone mentoring + IT-tool (N = 15).

Treatment lasted for 6 months with a further 6 months follow up, outcomes were re-assessed at 3, 6, and 12 months.

Outcomes

Primary outcomes
Stanford Self-Efficacy Scale
QoL (measured with CFQ-R)

Secondary outcomes
Spirometry
Height and weight z scores

Qualitative data from 10 intervention participants and mentors

NotesContacted the leading author who provided the information that they are going to publish, but the data is not published, yet.

Widman 2013

MethodsRandomised open-label cross-over study - a pilot study of behavioural intervention for nutrition in CF.
ParticipantsAnticipate 30 into 2 groups.
Interventions

This is a feasibility study to try out a multi-component behavioural intervention. The intervention is designed to help people with CF use their nutritional support to gain weight.

The first group receive the behavioural intervention immediately. The second group receive the intervention after 3 months. All participants will be followed up for 6 months. Data will be collected every 6 weeks during clinic reviews.

Experimental: Behaviour change intervention. Intensive behaviour change intervention for 3 months, followed by a maintenance phase for 3 months.

Wait-list ControlUsual care for 3 months, followed by intensive behaviour change intervention for 3 months.

Note: Behavioral: Behaviour change intervention: the behaviour change intervention consists of feedback via a web-based food diary 'coaching' with problem-solving and implementation plansThe intensive intervention phase consists of 2 home visits, 8 telephone calls and 6 reminder emails.The maintenance phase consists of 3 telephone calls and 4 reminder emails.

Outcomes

Primary outcome measures
1. Proportion of patients who accepted invitation to participate as a marker of feasibility
2. Proportion of people with CF who are randomised into the study after accepting the invitation to participate, as a marker of feasibility

Secondary outcome measures
1. Participants' opinion about the behavioural intervention
2. Participant attrition rate and phases of study whereby the attrition occurs
3. Participants' opinion about the study processes
4. Participants' suggestions for further improvement of the intervention and study processes
5. Proportion of adults who fulfils the BMI criteria for recruitment with IT facilities
6. Resources needed by the investigators to deliver the intervention
7. Proportion of days with missing nutritional data, as a marker for feasibility

Participants are requested to fill in their nutritional intake daily on a web-based food diary. The investigators are interested to find out what the proportion of such data will be missing to determine the feasibility of collecting data with the web-based food diary.

Other outcome measures

1. BMI
2. Weight
3. BMQ score
4. The mean SRBAI score

Notes

Start date: Feb 2014. Anticipated end date: March 2015.

Country: UK.

Williams 1987

  1. a

    BMQ: Beliefs about Medicines Questionnaire
    CF: cystic fibrosis
    CPT: chest physical therapy
    FET: forced expiration technique
    HIAT: home intravenous antibiotic therapy
    MAC: mid-arm circumference
    PFT: pulmonary function tests
    QoL: quality of life
    RDI: recommended dietary intake
    SRBAI: Self-Report Behavioural Automaticity Index

MethodsExplored the effects of intensive dietary counselling in adolescents with CF upon nutritional status. The participants were randomly divided into three groups (control vs counselling and normal food vs counselling and enteric coated pancreatic enzyme supplements). The groups were matched for age sex and Schwachman score.
Participants28 adolescents with CF aged 9 - 18 years.
InterventionsThe participants in the first group were the controls. Then the second group received a counselling to improve dietary intake to > 130% of RDI calculated form expected weight for height), using normal foods and simple supplements; the final group received counselling and enteric coated pancreatic enzyme supplements instead of conventional supplements. Duration: 1 year.
OutcomesChange in weight; dietary intake; improvements in MAC.
NotesPublished as an abstract.

Characteristics of ongoing studies [ordered by study ID]

Quittner 2000

Trial name or title 
Methods

RCT.

The aim of the study was to compare the effects of BFST, an empirically supported treatment, to both the Family Learning Program (FLP) and standard care (SC).

Participants

117 adolescents with CF ages 10 to 17 years.
Inclusion criteria

- Age 9.5 to 16.5 years

- Diagnosed with CF for a minimum of one year
- FEV1 greater than 35% predicted

- no other chronic disease (other than CF-specific diabetes)

- no history of psychiatric treatment within the past years

- No evidence of intellectual impairment
Stratified by age, sex, illness severity and family conflict

Interventions

Standard care versus 2 manualised and structured interventions. 11 family sessions with a registered nurse or licensed clinical psychologist

1. Standard care (n = 36)

Teens received their usual care at the CF centre.
2. Family Learning Program (n = 40)

10 90-minute psycheducational sessions over 6 months aimed at increasing knowledge about CF and its management.
3. 'Behavioral Family Systems Therapy' (n = 41)

10 treatment sessions (90 minutes) over 6 months plus 1 booster session 3 months later. Family problem-solving, communication skills training, and cognitive restructuring were included in the intervention.

Outcomes

Evaluation post-treatment and at 6, 12, & 18 months subsequently.
1. Treatment adherence:
a. electronic monitors;
b. self-report;
c. daily-phone diary.
2. Family functioning (family conflict;coping skills; communication):
a. self-report;
b. audiotaped role-play vignettes;
c. videotaped family discussions/interactions.
3. Quality of life:
a. CF questionnaire.

Additionally: long-term health outcomes and cost-effectiveness.

Starting date 
Contact informationProf. Alexandra Quittner, University of Miami, Department of Psychology.
Notes 

Quittner 2011

Trial name or titleRCT of a behavioral adherence intervention for adolescents with CF: I change adherence and raise expectations (iCARE)
Methods

Study design: clustered-randomised controlled study.

The study is evaluating two adherence promotion interventions for adolescents, implemented by CF care teams. The centres are randomised to one of the types of interventions.

Half of the centres will receive the Comprehensive Adherence Program (CAP) for 2 years. The other half will receive the Adherence Dashboard only in year 1 of the study and CAP in year 2 of the study.

Participants

Enrolled: 632 adolescents.

Population of interest: adolescents diagnosed with CF who are prescribed chronic use of a pulmonary medication.

Inclusion criteria:

1. male or female patients age 11-120 years old;.

2. patients with a diagnosis of CF;

3. patients attend the accredited care centre for regularly scheduled clinic visits;

4. patient must be prescribed at least one of the following medications for at least 6 months prior to signing the informed consent (azithromycin; hypertonic saline; Pulmozyme®; TOBI®; inhaled compounded tobramycin);

5. Patient has consented to provide data to the CF Foundation Registry prior to conversion to PORTCFv.

Interventions

Comprehensive Adherence Program (CAP) consists of:

1. Adherence Dashboard - a web-based resource offering automated/regularly updated patients adherence estimates (based on pharmacy refill patterns and health information retrieved from registry);

2. Training in the CF My Way program - a validated problem-solving adherence promotion intervention.

Outcomes

Primary outcome

Medication adherence will be assessed via MPR derived from pharmacy refill records. (Time frame 24 months.)

Secondary outcomes (Time frame 24 months.)

CF knowledge
Skills associated with CF treatments
Health-related quality of life (CFQ-R)
Lung function (FEV1)
Pulmonary exacerbation
CF hospitalizations

Starting dateOctober 2009.
Contact informationProf. Alexandra Quittner, University of Miami, Department of Psychology.
NotesThis study is ongoing and is currently recruiting participants (31st July 2012). http://www.clinicaltrials.gov/ct2/show/record/NCT01232478?term=icare+adherence&rank=1)

Quittner 2012

Trial name or titleCFfone: A cell phone support program for adolescents with CF.
Methods

The investigators propose to evaluate the efficacy of a cell phone support program for adolescents and young adults (CFfone) in improving CF knowledge, treatment adherence, and quality of life compared to a CF-related educational website intervention (control group).

Study type: interventional.

Study design: efficacy study, randomised allocation.

Participants

Inclusion criteria

1. Have a diagnosis of CF.

2. Be within the target age range of 11-20 years old at enrolment.

3. Have regular access to an internet-connected computer that does not prevent access to websites (i.e. firewall).

Exclusion criteria

1. Have a developmental disorder that would affect ability to respond to survey questions.

Estimated enrolment: n = 146.

Interventions

Cell phone support program for adolescents and young adults (CFfone):

Is a behavioral intervention which contains a password-protected, secure website, which can be accessed via web capable cell phone. The website provides age appropriate medical and behavioral information, disease management tools and social networking features for adolescents.

CF-related educational website intervention (control group):

The interventions contains a CF-related educational website which has some areas of CF information and services relevant to adolescents.

Outcomes

Primary outcome

The participants' CF-related knowledge will be assessed via the CFK. It will be administered at baseline, month 3, month 6 and month 9.

Secondary outcomes

The participants' quality of life will be assessed via the CFQ-R. The adolescent/adult version will be used for participants aged 15-20 years and the CFQ-R Older Child version will be used for participants ages 11-14 years. Quality of life will be administered at baseline, month 3, month 6 and month 9.

To measure treatment adherence a print out of CF related medications and pharmacy refill data will be collected. To obtain participants' prescription information in order to have adherence calculations for three month periods, the attending physician will fill out a prescribed treatment plan at every clinic visit during the study. Pharmacy refill data will be collected at baseline for the 12 months prior to enrolment; and again after the 9 month clinic visit for the 9 months of the study. The prescribed treatment plan will be collected at baseline, month 3, month 6 and month 9.

Exploratory end point: social support.

Starting dateSeptember 2010.
Contact informationProf. Alexandra Quittner, University of Miami, Department of Psychology.
NotesInformation provided by the investigator Prof. Quittner: "This study is ongoing, an already completed enrolment with a sample size of 100 adolescents ages 11 to 20. The study is going on at 6 CF Centers.  We anticipate that all measures will be completed in April of 2013."

Riekert 2012

  1. a

    BFST: Behavioral-Family Systems Therapy
    CF: cystic fibrosis
    CFK: Cystic Fibrosis Knowledge Questionnaire
    CFQ-R: Cystic Fibrosis Questionnaire-Revised
    FEV1: forced expiratory volume litres per second
    MPR: medication possession ratio
    QoL: quality of life
    RCT: randomised controlled trial
    SRBAI: Self-Report Behavioural Automaticity Index

Trial name or titleBuilding adherence to live with and navigate my CF experience (BALANCE).
Methods

The investigators propose to evaluate the relative efficacy of a Motivational Interviewing-focused intervention (MI) in improving adherence and reducing CF-related morbidity compared to a CF education intervention (CFE; attention control group). The investigators hypothesize that MI will result in improved regimen adherence and reduced CF morbidity compared to the CFE control group.

Study type: Interventional.

Study design: efficacy study; randomised allocation.

Participants

Inclusion criteria

  1. Have a doctor confirmed diagnosis of CF

  2. Aged 16 years old or older

  3. Prescribed an inhaled mucolytic, inhaled antibiotic therapy, chronic macrolide therapy and/or hypertonic saline therapy for the previous 12 months.

  4. Scheduled for a regular visit at either the pediatric or the adult CF clinic at Johns Hopkins Hospital.

Exclusion criteria

  1. Burkholderia cepacia complex isolated from the respiratory tract within the past 2 years.

  2. Had a lung transplant

  3. Participated in NA_00008649 A pilot study of Motivational Interviewing for adults with CF.

Estimated enrolment: n = 153.

Interventions

CF Education

This intervention is designed to increase knowledge and enhance the skills needed to optimise CF-management. The strategies used to achieve improved adherence include providing didactic education and skills training, and proscriptively using behavioral modification strategies, such as positive reinforcement for desired behaviours, and problem-solving training to overcome barriers.

Motivational Interviewing

The overarching goal for the intervention is to motivate and assist the participant to improve his/her adherence to the CF pulmonary medications. The intervention will begin by providing the patient personal feedback on their adherence(using pharmacy refill data) and health outcomes (e.g., trajectory of lung function values, frequency of exacerbations) as well as clinic-level figures showing the association between adherence and health outcomes.

Outcomes

Primary outcome

A MPR will be calculated for each prescribed drug that is being monitored for adherence (time frame: 12 months).

Secondary outcome

Change in FEV1 % predicted (time frame: 12 months).

Starting dateJuly 2009.
Contact informationKristin A. Riekert, PhD, The Johns Hopkins Adherence Research Center.
NotesThis study is ongoing, but not recruiting participants at the moment (20th July 2012). Information from http://www.clinicaltrials.gov/ct2/show/NCT01013896?term=adherence+cystic+fibrosis&rank=1).