Experiences of UK‐based adult transition services for interstitial lung disease in childhood: “There's a lot less cushioning”

Interstitial lung disease in childhood (chILD) is rare and no longer purely a childhood issue as many survive into adulthood, and so have to transition from pediatric to adult healthcare services. Transition is a significant life event that has the potential to impact on physical and mental health outcomes. The European Respiratory Society (ERS) statement on chILD transition highlighted the lack of standardised transition services for chILD transition resulting in a haphazard process. This qualitative study explores how young people and parents in the United Kingdom experienced transition from paediatric to adult healthcare services for chILD. Participants (n = 7) were recruited from chILD patient organisations and online communities. We focused on the experience of transition exploring if there were any information packs or support provided for the transition. Such support may be generic, such as “Ready Steady Go” which provides a systematic approach to transition and disease‐specific literature. These latter have not been developed for ILD. Data were analysed by constructivist grounded theory. We present a lived experience of transition with themes of lack of transition preparation and planning, challenges of adapting to adult services, and a changing healthcare scene. Due to the complexity of chILD, parents discussed their need to remain, in part, as an advocate for the young person. Respondents provided recommendations for how transition could be improved along with tips for young people who are new to the transition process, which include educating oneself about the condition, learning medical terminology, and reaching out for support.


| INTRODUCTION
Interstitial lung disease in childhood (chILD) is a relatively newly described diverse group of more than 200 rare conditions. 1,2 Although chILD begins in childhood, patients frequently need to transition to adult services. 3 Transition can be described as "the purposeful and planned movement of adolescents and young adults with chronic physical and medical conditions from child-centered to adult-orientated healthcare systems." 4 Paediatric medicine differs greatly from adult medicine. For those with a chronic respiratory condition, pediatric care is often multidisciplinary, individualized, and heavily focuses on issues of development, growth, and family inclusion. Successful transition is a process where the young adults learn self-advocacy and to communicate their care needs; this is part of the young person taking responsibility for the management of their own healthcare. 5,6 Poor transition has been shown to have detrimental impact on long-term health and wellbeing, and is also a time when patients may be lost to follow up. 7,8 Therefore, it is of critical importance that those with chILD are able to transition smoothly to adult services.
Rare respiratory diseases who do have access to specialist centres and funded co-ordinated care, cystic fibrosis (CF) and primary ciliary dyskinesia (PCD) use the "Ready Steady Go" transition programme. 9,10 "Ready Steady Go" is disease agnostic and recommended by National Institute for Health and Care Excellence 11 ; however, not all hospitals adopt this as it requires a co-ordination of communication between pediatric and adult teams. 12 The European Cooperation in Science and Technology (COST) Action dedicated to European Network for Translational Research (EnTER) in Children's and Adult interstitial lung disease (COST action 16125) found chILD transition is highly variable and inconsistent across the EU. 2 Despite the great strides in clinical research and genetics, one aspect lacking is chILD surveillance in adulthood. There are many benefits of longterm health surveillance with respiratory conditions. These include knowledge of disease development and relapse, 3 treatment adherence and comorbidity management 13,14 ; learning of continued disease risk factors into adulthood 15 ; understanding of long-term treatment effects 16 ; along with potential missed opportunities for genetic diagnostics with idiopathic cases; these are all important aspects of longitudinal health care.
We investigated the current UK provision of transition services through interviews with young adults and parents of young people affected with chILD. Before the study, an informal UK-based prerecruitment survey indicated that only approximately 50% of young adults with chILD transitioned to adult services. With chILD services being uncoordinated and the conditions being rare-to ultra-rare, coupled with the far smaller numbers of chILD transitioning compared to CF or PCD across the United Kingdom, understanding current chILD transition is important as we aim to highlight gaps and improve services for these patients. To our knowledge, this is the first qualitative study to focus on the lived experience of transition and chILD.

| Participants
Participants were recruited from chILD-related patient organisations and online community support groups via adverts placed on forums. Potential participants were asked to contact the research team directly to receive study information and to screen for UK-based participants. We recruited three young adults and five parents of young people affected with ILD.

| The interviews
Interviews were conducted between mid-July and mid-September 2021. Seven were conducted remotely using teleconferencing software and one by telephone. Interviews lasted between 20 and 45 min. Before consenting to the interview, respondents were sent a participant information sheet via email and were asked to sign an online consent form, assuring their responses would be treated confidentially and anonymised in any study output. The interview was semi-structured; however, a chronological approach from pediatric to adult transition service changes was used, coupled with an understanding of the impact of the participants condition along with two main questions of interest "what is your experience of transition?" and "were any information packs or support provided for transition?" Parents were asked how their child's experience of transition from their perspective. Finally, participants were asked what advice they would give to others in the same situation, or any recommendation of services change that may improve future transition experiences.

| Analysis
The Zoom interviews were audio-recorded and transcribed verbatim.
All identifying features within the interviews such as names, locations and hospitals were removed from the transcripts; however, the chILD diagnosis was retained. The telephone interview was not recorded but notes and quotes were checked by the participant to confirm accuracy of information. Transcripts were analysed using the constructivist grounded theory method. 19 Line-by-line coding was primary method of the analysis as this allowed for exploration of emerging themes in the data. Quotes were selected to allow for participant "voice" and experience to come through the data, without researcher personal inference or assumptions. This was a reflexive process as the codes generated from the interview data developed into categories and each transcript was then compared to identify commonalities and broader themes. The coding, category building, and text analysis was an iterative process where central themes were developed.

| RESULTS
We present our findings in three themes of transition preparation and process; adaption and accessing adult services; the changing of healthcare relationships, and lastly, for parent respondents, the parent as a continued advocate for the young person.

| Transition preparation and process
Transition occurred predominately around the age of 18 ( Table 1).
The speed varied depending on the young person's circumstances.
YP1 required lung transplantation evaluation and so completed transition earlier than expected to access the new adult services hospital with lung transplant facilities. YP1 described a feeling that paediatric services did not wish to deal with the complexity of her condition as she was "at an awkward age where they didn't want to deal with me" (YP1). Whereas P2 positively described "a really slow transition" due to the young person requiring multidisciplinary care along with additional support for mental health and neurodevelopmental needs.
Before transition, young people were encouraged to attend a portion of their medical appointment alone. This was part of the process to build confidence and autonomy by encouraging the young person to learn to take responsibility for their own health and gaining independence from their parent/caregiver. 5 Parental encouragement is highlighted from the parental responses. P2 discussed her son's slow transition preparation to "get him more independent with his own medical needs" (P2) yet adds that her input is still required since her son has also been diagnosed with autism and mental health issues. However, already even though they are in early phases of transition discussion, P2 is seeing signs of acceptance developing in her son about a change to adult services. T A B L E 1 Respondents information. Yet, conversely, one respondent described their hopes for having an adult consultant who understands the distinction between chILD and adult ILD: "We'll probably get a really good consultant who knows we're not dealing with an adult who is developing interstitial lung disease; we're dealing with a child who's had interstitial lung disease and it's very different. And the treatment, the prognosis and the outcome is very different" (P4, Surfactant Protein C deficiency)

| The parent as continued advocate
Pediatric healthcare services are focused on growth and development, along with a strong family-centered approach to enable the young adult to build confidence, autonomy, and self-care skills. 5  Among the weaknesses in the current transition process the often the lack of a specific transition coordinator, the generally haphazard timing of first discussion about transition and for the most part the lack of involvement of professionals such as psychologists and social workers. Realistically, these professionals will have to be shared with other disease groups, because chILD is so rare, but this is Writing-original draft; writing-review and editing; formal analysis.