Transition from paediatric to adult care in cystic fibrosis

When should transition begin?

It is generally accepted that transition discussions should take place from at least early adolescence to normalise the process of growing up and developing independence and self-management skills. Adolescence can be defined as the phase of life stretching between childhood and adulthood, and encompasses elements of biological growth and major social role transitions. Rather than age 10–19 years, a definition of 10–24 years corresponds more closely to adolescent growth and popular understandings of this life phase and would facilitate extended investments across a broader range of settings [7]. Adolescence is also a unique stage in human development and an important time for laying the foundations of good health. Adolescents need opportunities to meaningfully participate in the design and delivery of interventions to improve and maintain their health [8]. Some CF centres discuss transition with parents on an annual basis, over time the child becomes increasingly involved and the conversations become more directed towards them. The age at which transition occurs differs between countries and occasionally within the same country. Populations and societies function differently around the world and as such, the appropriate age for transition can vary. In a recent international survey of transition in CF the ages ranged from 16 years to 21 years [9]. Regardless of the chosen age, local teams should agree an age at which they aim to transition young people within their patient cohort.

Multidisciplinary care

Paediatric and adult CF teams who work together regularly should agree a shared set of goals and guidelines related to transition for young people moving between their services. Shared working across teams and becoming dually responsible allows for an effective transition. Defining the exact age of transition can be difficult as countries and health systems accept young people into adult care at different ages. However, pre-transition preparation does not need age definition as it takes place before the move to an adult centre regardless of age. Paediatric and adult multidisciplinary teams (MDTs) should meet regularly to discuss young people who will be transitioning between their services [10]. However, in some cases this may not be possible, for example if the young person is moving away to university or if the family relocate to another part of the country. Teams in the Netherlands have successfully used virtual meetings to transition patients between centres where patients have moved great distances, this is an effective mode of communication for CF teams who may not transition patients between their centres on a regular basis. The paediatric team should provide a comprehensive written handover to the adult team, often referred to as the young person's transition document. This document should summarise details of diagnosis particularly the method of diagnosis (e.g. newborn screening), sweat chloride values and genotype (which is necessary when considering access to modulators). Additionally, a summary of care provided from birth to the point of transition should be included, containing information regarding physical and mental health, complications both related and unrelated to CF, social circumstances, and information pertaining to the individual's future aspirations in relation to education and work [11].

Meeting the adult team

It is widely reported in the literature that young people and their families may experience anxiety and concern at the prospect of transitioning from paediatric services which have been the primary care providers for their entire lives. However, there is evidence that parental anxiety may supersede that of the young person who may have a more laissez-faire attitude to transition [12]. Commonly cited concerns include meeting new team members, anxiety regarding changes in quality of care between services, a much larger adult CF service with many more patients and worries regarding infection control [13]. Meeting the adult CF team prior to transition can significantly decrease anxiety and concern, as it offers the opportunity for both the young person and their family to ask questions and discuss concerns with the adult care providers [13]. In turn, through open discussion young people and their families can be reassured about the similarities and differences between adult and paediatric care and how support will be provided throughout the transition process. Meeting with young people and their families enables the adult team to talk about expectations during the transition process, including developing independence and practicing CF-related self-management skills [9]. Engaging with the adult team prior to transition is also an opportunity for the adult team to provide information and educational resources, both written and electronic, to help facilitate the transition process.

Young people should be encouraged to take an active part in the transition process, advocating for themselves and making decisions such as where they would like their adult care to be delivered. It is important that young people are able to make an informed decision about where to have their future care, many paediatric CF services run joint transition clinics with a number of adult providers, these are commonly available on one or more occasion prior to transfer to adult care. At these clinics young people can meet members of the adult team alongside familiar members of the paediatric team during joint consultations [14]. Joint transition clinics allow young people and their families to meet different adult teams before making a final decision regarding where their adult care will be based. Some young people will choose to attend the adult centre in the same hospital as their paediatric care, others may choose to move to an adult centre which is more geographically appropriate. The reported benefits of transition clinics are improved preparedness of young people and their families, and enhanced joint working between members of the paediatric and adult MDTs.

Coordination of the transition process

In the UK, the National Institute for Health and Care Excellence (NICE) published guidance regarding transition for use in all healthcare settings in which young people move from paediatric to adult services [15]. Recommendations include discussion of transition with young people (and their families) at an early age to normalise the process and encourage them to consider this as a positive step towards adulthood and independence. Where possible, transition should take place at a time of stability in the young person's life in terms of disease progression, social circumstances and education [15].

A further recommendation suggests that young people and their families could benefit from one point of contact, or a specific team member known as the transition key worker (TKW). A TKW assists young people and their families through the transition process providing individualised support for a minimum of 6 months pre- and post-transition [13, 15]. The TKW meets the young person and their family annually from the age of 14 years to answer questions regarding transition, provide reassurance and support, and aims to develop a trusting relationship until the young person adjusts to the culture of the adult centre and settles into the service [16]. Following transition, the TKW continues to provide continuity as a main point of contact, providing details so that contact can be made independently if there are questions or concerns between hospital visits. Further, the TKW supports adjustment to care in the adult service, attends/reviews progress at the first few adult clinic appointments, supports throughout the first admission in adult services and attends MDT meetings regarding the young person, such as those with school or social services, and ensures the young person attends appointments and remains engaged [8, 17].

Developing independence

When helping young people prepare to move to adult services it is important for them to become confident when discussing their symptoms with the members of the CF MDT. The paediatric MDT should encourage the young person to practice discussing their health prior to transition, perhaps with a paediatrician or a member of the MDT they feel comfortable with. Parents can be involved in this process, for example they may wish to remain in the room, observing but not actively taking part in the discussion. An advantage of this is that the parent remains with the young person throughout the consultation and can prompt the young person to discuss any health concerns they may have forgotten to mention or rejoin the conversation at the end.

Over time, as the young person develops confidence they are encouraged to talk to the team without their parents throughout their consultation. The parents can then rejoin the consultation, with the young person summarising the discussion. The advantage of structuring the outpatient clinic in this way is that the young people can practice the skills they will need in adult services in a familiar environment, additionally parents have the opportunity to gain confidence that their child can discuss their health, treatment plans or concerns with members of the MDT. Any sensitive subjects the young person wishes to discuss, such as contraception or fertility, do not have to be shared with the parents. Independent discussions with the MDT may provide the opportunity for the young person to explore subjects they may choose not to discuss with their parents present.

Nurses are an essential part of the MDT and are often a fundamental part of the process in the coordination and management of transition within teams [18]. The TKW is commonly a member of the specialist nursing team; however, this role may be occupied by more than one person with some centres reporting the specialist nurse may work in conjunction with a member of the social work or medical team to manage transition [9]. The TKW should be known to the young adult and their family and should be used as their first point of contact if they encounter any concerns while navigating the adult service [14].

Meeting the adult team

As young people and their parents learn about the transition process, it is important they meet members of the adult team. In daily practice some CF services may find this a challenge logistically; however, a meeting with the CF nurse specialist can often be arranged in person or virtually. Meeting the team at a transition clinic provides an opportunity for the young person and their family to discuss their expectations and concerns about the differences in paediatric and adult services. It is important to let the young person introduce themselves to the new team, either in person or by writing a little about themself in the transition document. Some young people share more information than others; however, it is helpful for the adult service to gain insight into the young person's hopes for the future, hobbies and goals for future education or employment.

To help young people who transition to adult services in a different location to their paediatric care, many CF services provide short videos or tours of their facilities. These often detail the location of the hospital and introduce the service and members of the team. These resources can provide important information, such as how to get to the hospital, where the outpatient clinic is and what it looks like, and an opportunity to “meet” members of the team. A short film can be a useful alternative to an outpatient clinic before the actual transition. If CF teams do not share the same medical records system, a detailed handover letter is necessary with contact information for the paediatric CF team.

Suggested topics to include in the young person's contribution to the transition document

1. My name is:

2. The best way to reach me is:

3. Things that give me enjoyment in my life are:

4. The people I count on for support are:

5. When I am sad or am having a hard time with something, I like to (exercise, talk to friends, meditate, something else):

6. How do I feel about transferring my CF care to an adult CF team?

7. One of my goals for this year is:

8. What I like most about CF clinic or hospital stays:

9. What I like least about CF clinic or hospital stays:

10. One of the things I worry most about in life or my health is:

11. If I could change anything about my treatments it would be:

12. What topics or questions about CF do I want to learn more about this year?

13. Anything else important I would like my new adult CF team to know about me:

Failure to develop skills to self-manage care

As previously mentioned, it is recommended that young people begin to practice and develop skills to manage their disease independently prior to transition. For example, by beginning to practice skills such as contacting their care team independently. The aim of this is to prepare the young person to become more autonomous in self-management and prepare them for adult services. However, this may present a challenge, as not all young people have reached a level of maturity where they are willing to start taking over responsibility for care or they may lack the confidence to do so. Many adults with chronic diseases often require support from healthcare professionals, family members or carers to enable them to successfully manage their day-to-day care [19]. Gravelle [20] supports this by suggesting that encouraging young people to independently manage their health when they are unwilling to do so could lead to them becoming overwhelmed and resistant to developing independence, with decreased clinic attendance and lower adherence to treatment regimens. Transition takes place at a time when young people are typically beginning to develop independence in other areas of their life, making decisions regarding their future, education and employment, and beginning relationships or moving away from home. Therefore, treatment may not be a priority, their parents may be less involved with management of treatments and adherence to treatment may decrease, this may lead to an increase in symptoms and prolonged periods of illness [21].

Young people who feel empowered to independently manage their disease report increased quality of life; therefore, it is important that young people are encouraged to begin developing self-management skills with support from their families and healthcare team gradually over time in preparation for a successful transition [22]. Zack et al. [23] suggest that the ideal time to begin developing independent skills, such as speaking with a doctor alone at clinic visits, would be between 13 and 16 years of age. Cronly and Savage [19] found that encouraging young people to reflect on their adherence to treatment and its positive consequences allowed them to gain awareness of their ability to influence their health, thus acting as positive reinforcement. In turn, this increased the likelihood they would engage in healthy behaviours in the future which would positively influence their health.

Disengagement from the transition or CF service

Apprehension about any new situation is understandable, whether a new school, job or healthcare team. However, this should be addressed during the transition process and should not prevent transitioning into the adult service [24]. When transition does not run smoothly, for example if the young person or their family fail to engage in the process or if there is a no formal transition programme, there is an increased risk of young person/family disengagement and loss of follow-up in the adult service [25]. In falling between both services, the young person may not access CF-specific healthcare until the need arises, often during a deterioration in symptoms. During transition the first appointment in the adult service must be agreed by both the young person and their family, and appointments should be monitored by the TKW to ensure the young person attends clinic and is given a follow-up appointment [25]. Gravelle [20] suggests that paediatric and adult teams should share responsibility for follow-up of young people who fail to attend their first scheduled clinic appointment with the adult service.

Preventing gaps in care is a key focus of the CF clinical care guidelines in the USA. Sawicki et al. [26] sought to identify the factors associated with disengagement at the point of transition by examining the CF Foundation patient registry. They found that young people lost to follow-up usually had not attended clinic regularly in their last year of treatment under the paediatric team. Young people were lost to follow-up for several reasons, but there was an increased likelihood of disengagement if they moved to a different city, particularly if links between paediatric and adult CF centres were absent. Furthermore, these young people were unlikely to have cultured methicillin-resistant Staphylococcus aureus (MRSA) and had decreased episodes of pulmonary exacerbations, suggesting that some had lost contact because they felt well and did not need to access CF services. Disengagement was also more likely if young people did not have health insurance, suggesting they came from lower socioeconomic backgrounds. Therefore, the TWK within the adult team should monitor young people who fall within these groups to ensure they are actively encouraged to engage with the adult service and are not lost to follow-up [20].

Challenges in daily practice

There is a wealth of research regarding the importance of a positive transition for young people with chronic diseases such as CF; however, in daily practice there are many challenges to this. A variety of factors can hinder a good transition process, with potentially major consequences for the health of the young person. Some authors suggest that transition for young people with CF is particularly challenging because it occurs at a time when there is an increased risk to health [27]. Being flexible around the timing of transition can be beneficial. On rare occasions medical instability, transplant assessment or end-stage disease may delay the process or it may not be appropriate at the time. Thankfully these circumstances are rare in modern CF management.

Organisation of the transition process can pose challenges. CF services may differ in size and small centres that transition less often have less experience in the transition process and may transition young people on a more ad hoc basis. Other smaller centres may cluster their services to devise a local protocol for their CF centres. However, there is little national or international consensus on how best to organise a transition programme [9].

Preparation

Preparing young people and their families for transition can be a challenge, some young people feel ready to transition whereas others may not feel as prepared. For young people transition takes place at a time of change, including education, employment and developing relationships. Therefore, transition may be the focus for the CF team, but not for the young person. Many young people transition to adult services in good health, which may also contribute to transition feeling less of a priority [12]. The young person may just want to live a normal life, with as little burden of CF as possible. Conversely, some young people may require ongoing assistance with transition, for example those with developmental delay who may require prolonged support or assistance to navigate a multitude of services. The TKW should provide prolonged assistance to those who have severe medical problems, poor understanding, or less knowledge of CF.

Paediatric care tends to be focused upon the family, whereas adult care is primarily focused upon the individual, it may take some time for young people and their families to adapt to this change. It is generally accepted that discussion about transition (including independence and knowledge of CF) should take place from at least early adolescence, although often CF teams begin discussions earlier to ensure that transition is accepted as an expected milestone in the process of growing up with CF. Beginning to educate young people about disease management early can normalise treatment, as it becomes part of the young person's daily life and they work towards becoming independent in the management of their condition.

Confidence in the expertise of the adult CF team and the differences between paediatric and adult approaches may influence the success of the transition process. Therefore, it is important for the young person and their family to meet the adult CF team as part of the transition process. Several factors could disrupt this, such as lack of time, understaffing or disinterest/disengagement from both the young person and their family.

More recently, the coronavirus disease 2019 (COVID-19) pandemic has led to changes as people with CF report increased vigilance around cross-infection issues, a reluctance to make long journeys to hospital, and a move from physical to virtual reviews or a mixture of both. It is important to consider the wishes of the young person and their family, particularly during a process of transition.