SEARCH

SEARCH BY CITATION

Keywords:

  • child;
  • cystic fibrosis;
  • inhaled corticosteroids

Abstract

  1. Top of page
  2. Abstract
  3. Cystic fibrosis
  4. Why use corticosteroids?
  5. What should we do in practice?
  6. What do the guidelines suggest?
  7. Commentary by J. Robinson
  8. Declaration of interest
  9. References

Eco-paediatrics is an occasional feature in Evidence-Based Child Health: A Cochrane Review Journal. Our goal is to contribute to the worldwide discussion on reducing waste in health care. In each instalment, we will select a recent Cochrane review highlighting a practice, still in use, which the available evidence tells us should be discontinued.

Excerpts from: Balfour-Lynn IM, Welch K. Inhaled corticosteroids for cystic fibrosis. Cochrane Database of Systematic Reviews 2012, Issue 11. Art. No.: CD001915. DOI: 10.1002/14651858.CD001915.pub3.

Cystic fibrosis

  1. Top of page
  2. Abstract
  3. Cystic fibrosis
  4. Why use corticosteroids?
  5. What should we do in practice?
  6. What do the guidelines suggest?
  7. Commentary by J. Robinson
  8. Declaration of interest
  9. References

Cystic fibrosis (CF) is a chronic disorder, of autosomal recessive inheritance, which principally affects the respiratory tract, pancreas, gastrointestinal tract and liver. Recurrent chest infections in people with CF cause inflammation and damage to the lungs which, in the long term, is the most common reason for death in CF patients.

Why use corticosteroids?

  1. Top of page
  2. Abstract
  3. Cystic fibrosis
  4. Why use corticosteroids?
  5. What should we do in practice?
  6. What do the guidelines suggest?
  7. Commentary by J. Robinson
  8. Declaration of interest
  9. References

Corticosteroids (glucocorticoids) are potent, nonspecific anti-inflammatory agents that have been widely used in a variety of inflammatory disorders. In asthma, inhaled corticosteroids (ICS) reduce bronchial hyperreactivity and airway inflammation. The rationale for their use in CF lies in observations that inflammation occurs early in the course of CF and may contribute to lung damage. Recent data from UK CF Trust Registry shows that in 2007, 21% of children and 23% of adults were prescribed regular ICS; and North American data from the Cystic Fibrosis Foundation (CFF) registry showed that in 2005, 46% of patients received ICS (UK CF Trust and CFF, personal communication). Corticosteroids are associated with a wide range of potential adverse effects (1). Some are less serious, for example oral thrush, but others such as reduced childhood growth are more serious.

What should we do in practice?

  1. Top of page
  2. Abstract
  3. Cystic fibrosis
  4. Why use corticosteroids?
  5. What should we do in practice?
  6. What do the guidelines suggest?
  7. Commentary by J. Robinson
  8. Declaration of interest
  9. References

This review has found little evidence from existing trials to support the practice of routinely prescribing inhaled steroids in CF. Specifically, it cannot be concluded that inhaled steroids are beneficial but there is some evidence that at a high dose, they adversely affect growth.

What do the guidelines suggest?

  1. Top of page
  2. Abstract
  3. Cystic fibrosis
  4. Why use corticosteroids?
  5. What should we do in practice?
  6. What do the guidelines suggest?
  7. Commentary by J. Robinson
  8. Declaration of interest
  9. References
  1. These are guidelines specific to children <2 years.

  2. ABPA, allergic bronchopulmonary aspergillosis.

CFF (2)For patients with CF, 6 years and above, and without asthma or ABPA, the CFF recommends against the routine use of ICS to improve lung function and to reduce exacerbations.
CFF (3)For infants with CF, below 2 years, and without airway reactivity or asthma, the CFF does not recommend use of ICS to improve lung function or reduce exacerbations.
European Cystic Fibrosis Society (4)

This study supports the conclusion from the Cochrane review that there is neither evidence nor benefit or harm from corticosteroid use in CF (5).

The authors suggested that the majority of patients taking ICS probably do not need to do so.

Evidence-Based Child Health, Editorial Office

*Correspondence to: Evidence-Based Child Health Canadian editorial office. E-mail: child@ualberta.ca

Commentary by J. Robinson

  1. Top of page
  2. Abstract
  3. Cystic fibrosis
  4. Why use corticosteroids?
  5. What should we do in practice?
  6. What do the guidelines suggest?
  7. Commentary by J. Robinson
  8. Declaration of interest
  9. References

At first look, it would seem reasonable to treat CF with ICS. Much of the pulmonary damage begins with lung inflammation and ICS reduce airway inflammation in other diseases. However, this review shows that lung inflammation is not altered by ICS and in addition reduces growth in children. This side effect is especially important in CF because of the expected difficulty with nutrition. Fortunately, the evidence also suggests that, with close monitoring, patients currently on ICS can be safely weaned from these medications.

References

  1. Top of page
  2. Abstract
  3. Cystic fibrosis
  4. Why use corticosteroids?
  5. What should we do in practice?
  6. What do the guidelines suggest?
  7. Commentary by J. Robinson
  8. Declaration of interest
  9. References
  • Association of British Pharmaceutical I. ABPI Compendium of Data Sheets and Summaries of Product Characteristics, 1999-2000: With the Code of Practice for the Pharmaceutical Industry. London: Datapharm Publications; 1999.
  • Flume PA, O'Sullivan BP, Robinson KA, Goss CH, Mogayzel PJ, Willey-Courand DB, et al. Cystic fibrosis pulmonary guidelines: chronic medications for maintenance of lung health. Am J Respir Crit Care Med 2007; 176: 957969.
  • Borowitz D, Robinson KA, Rosenfeld M, Davis SD, Sabadosa KA, Spear SL, et al. Cystic fibrosis foundation evidence-based guidelines for management of infants with cystic fibrosis. J Pediatr 2009; 155(6 Suppl): S73S93.
  • Heijerman H, Westerman E, Conway S, Touw D, Doring G. Inhaled medication and inhalation devices for lung disease in patients with cystic fibrosis: a European consensus. J Cyst Fibros 2009; 8: 295315. Epub June 30, 2009.
  • Balfour-Lynn IM, Welch K. Inhaled corticosteroids for cystic fibrosis. Cochrane Database of Systematic Reviews 2012, Issue 11. Art. No.: CD001915. DOI: 10.1002/14651858.CD001915.pub3