Transitioning Children with Cystic Fibrosis to Adult Care: Recommendations and Trends

2. Most children with CF are surviving into adulthood • The Cystic Fibrosis Foundation Patient Registry (CFFPR) characterised trends in CF survival between 2000 and 2010 and projected survival for children born and diagnosed with CF in 2010 : • Between 2000 and 2010: • Median age of patients with CF increased from 14.3 to 16.7 years • Median survival of children born and diagnosed with CF in 2010 • At 2010 levels: 37 years for females; 40 years for males

3. Standards of care for patients with cystic fibrosis: a European consensus E.Kerem and Al., Journal of Cystic Fibrosis 4 (2005) • A system should be in place to ensure that all children progress to adult centered care in a seamless process. • The time of transition to an adult CF center is 16–18 years of age but should be flexible, reflecting the adolescent’s social maturity and health status. • The cooperation between the Pediatric and Adult CF specialist centers is the cornerstone of successful transition : should be focused on continuity of care • It is recommended that members of the adult MDT liaise closely with their pediatric colleagues : if possible have reviewed the children and parents before the handover of care. • The staff from the adult CF center should provide an introduction before transition takes place. • As every member of the CF team can impact on the transition process all disciplines attached to pediatric care should provide a written report on the patient. • On the first day in the adult CF center sufficient time must be made available by the CF specialist to welcome the patient.

4. Transition • 2008 survey of CF centers suggested a wide range of ages for transition from 14 to 30 years with a mean of 19 years* • The “purposeful planned movement...from child centered to adult-oriented health-care systems” : • has to be a planned, • collaborative process • A gradual and evolving process to ensure provision of uninterrupted, coordinated, developmentally appropriate and comprehensive care before, during and after the transition period • A successful transition promotes autonomy and responsibility with respect to one’s own health * McLaughlinSE and al. Improving transition from pediatric to adult cystic fibrosis care: lessons from a national survey of current practices. Pediatrics. 2008;121(5)

5. Transition • The care of patients with cystic fibrosis has changed in a short period of time: PWCF are living longer • The patient is the center of the transition and the center of the evaluation, but transition can not be successful without his 2 satellites • Involves : • The patient • His parents/family • The pediatric CF MDT • This is why protocols are complicated: it has to be tailored for each one of our patients

6. Some recommandations from the UK CF Trust: cysticfibrosis.org.uk • Early discussion of transition • The topic of transition should be introduced at least a year before transfer • Allowing time to explore feelings and resolve concerns which the young person and/or parent may have • Time of transfer • Should then be flexible, in accordance with individual needs • Emphasis on pediatric consultations should progressively be on the young person, but should not exclude parents prematurely or abruptly

7. The team: Preparation for transition • Preparation for transfer should be thorough and well planned: • The proposed procedure and adult center should be discussed with the patient and the parents • It should be modified as appropriate • It should provide opportunities for the patient to familiarise oneself with the new staff and new environment

8. Preparation for transition • The joint transition clinic: • If possible: where the patient and family can meet the pediatrician and adult physician together • The focus of the clinics should be on how the young person’s care has been, and will be, managed • Provision of information: • A booklet about the adult centre should be provided (includes information about clinical arrangements, in-patient facilities, the names of team members and how to get to the centre)

9. What will happen when my child transfers to the adult centre? • Children do not turn into adults overnight: • They begin to change and mature long before the time of transfer and will continue for some time afterwards • There is a need for pediatric CF teams to offer services which reflect this change • Preparation should begin prior to transfer

10. The Parents • The care of adults is increasingly complex and requires a specialist adult CF MDT • There is a need to respond to the growing maturity of young PWCF : they need to become more involved in decisions about their care and treatment • This should start as early as possible in the pediatric centre and continue after transfer to an adult centre • Young people do not always see the need for this to begin with but it becomes more relevant to them as they mature • It is important that they begin to understand that their feelings, opinions and attitude towards their health and treatment will have a significant impact on their future wellbeing

11. What are the differences between the pediatric and the adult CF centre? • In the pediatric centre the CF team works very closely with parents: • They are given responsibility and taught how to care for their child and how to manage treatment routines • They are encouraged to explore how best to integrate care and treatment into their personal and family life • The CF team’s relationship with the young child is a parental one where decisions are made for the child rather than with him

12. What are the differences between the pediatric and the adult CF centre? • This changes as the young person matures: • They will be encouraged to participate in making decisions about their care. This process will continue after transfer to adult care • In the adult centre the CF team works directly with the patient • It is a partnership where the CF team tries to ensure that treatment fits in with the patient’s lifestyle • Patients are encouraged to be more closely involved in making decisions about their care and treatment • Patients are encouraged to ask questions and to discuss their care with the CF team

13. What is the role of the adult CF team? • The adult CF team is there to give expert advice • To listen to what the patient has to say and to support the patient and the family • To help patients make decisions about their care and treatment • To respect the patient’s decision whatever that may be

14. What changes can you expect as a parent? • Young people gradually learn to handle more mature relationships and begin to relate to other adults as equals • They become more emotionally independent, and want to make decisions for themselves but will continue to need help and support when making difficult decisions • You will find that your relationship will change from that of protector and advisor to that of consultant and counsellor • This can be very hard and raises many difficult thoughts and feelings • You may need support at this challenging time

15. What is your role likely to be? • Parents are almost always the people to whom young adults turn when they need help and support • It is important to allow your child to make their own decision even if it differs from the decision you would have made for them • Young adults can be very challenging in their behavior • It is important that parents give positive support and encouragement to the young person when he or she is ready to start taking more responsibility

16. What is your role likely to be? • Remember you have been a key figure in your child’s life up to this point • Parents and families are still needed to provide practical and emotional support especially when there are difficulties

17. What if there’s a problem? • Try to communicate directly with those members of the CF team who are providing care for your child. They are there to help and advise you • Going back to the pediatric team for advice? • Not a good idea however comfortable this may feel : they no longer have any direct input into your child’s care • It will take time for a new relationship to develop and requires a willingness to work through difficulties together and to understand and respect each other’s roles • Young people benefit from the increasing confidence they gain by making their own decisions and parents can begin to feel more confident in their child’s ability to cope

18. A variety of tools can be used to help increase transition readiness Patient toolkits Patient workshops Patient workshops Patient workshops • Including resources to: • Collect personal health information: • Immunisation records • Medication lists • Test results • Hospitalisation summaries • Communication logs with the MDT • Inventory/checklists for equipmentand supplies • Healthy eating checklists • Transition tips • Tips for talking to members of the MDT • Work with staff on competencies: • Health literacy/what to do to stay healthy • Awareness of symptoms • Scheduling own medical appointments • Understanding what defines an emergency and how to respond • Ability to tell HCPs how they feel and answer their questions • Asking HCPs questions about their condition • Obtaining medical records when needed Online resources Parent/caregiver workshops • Transition planning guide • Health history summary • Healthcare skills checklist • Learn what to expect and how to support and coach their children in increasing self-management of their healthcare Schlucter J, et al. Pediatr Nurs. 2015;41(2):85–6.

19. Ready Steady Go www.uhs.nhs.uk • The purpose is to get you thinking about moving on and preparing for it • Your doctors and nurses may have an idea about when they feel that you might be ready but it is important that you are involved in that decision • Knowledge and skills • Knowledge • Self advocacy (speaking up for yourself) • Heath and lifestyle • Daily living • School and your future • Leisure • Managing your emotions • Transfert to adult care

21. A successful transition requires overcoming potential challenges from a range of stakeholders1–5 Parent/caregiver Adult centre Paediatric centre Patient • Transition readiness • Anxiety about leaving familiar paediatric team • Concerns about lack of support from adult services • Usual physical and psychosocial challenges of adolescence • Relinquishing control, moving from a ‘supervisory’ to a ‘consultative’ role • Concerns about health insurance • Lack of support • Reluctance to transition due to losing ‘special bond’ with patients • Concern that adult services will be unable to provide comparable care • Lack of post-transition health outcomes data • Lack of appropriate and adequately resourced adult services • Lack of regular communication with paediatric care team • Limited documentation of healthcare history and CF-related complications • Logistical differences in paediatric and adult practice 1. Towns SJ & Bell SC. Clin Respir J. 2011;5(2):64–75; 2. Tuchman LK, et al. Pediatrics. 2010;125(3):566–73; 3. Royal College of Nursing 2013. Available at: www.rcn.org.uk/__data/assets/pdf_file/0011/78617/004510.pdf (Last accessed March 2018); 4. Viner R, et al. Arch Dis Child.1999;81(3):271–5; 5. West NE & Mogayzel Jr PJ. Pediatr Clin N Am. 2016;63(5):887–97.

22. Summary • There should be a continuity between the 2 CF care teams • The 1st appointment should be schedule by the adult secretary • An out-patient visit should take place 3 months before transition : everyone can say good bye • Last consult with the CF MD • 1st consultation in the Adult unit : meeting the MDT • 2nd consultation : with the new adult CF MD

23. In summary • Transition is a process starting long before the actual transfer • Transition is a tailored process based on trust • Transition involves us all • Transition from pediatric care to adult care is a actually a good news

24. Thank you for your attention