CF Guidelines with special focus of transition from childhood to adulthood of chronically ill patient Georges Casimir, Brussels, Belgium

Transcription

1 CF Guidelines with special focus of transition from childhood to adulthood of chronically ill patient Georges Casimir, Brussels, Belgium 1

2 Unité de Transplantation Cardiaque & Pulmonaire CF Guidelines with special focus of transition from childhood to adulthood of chronically ill patient Georges Casimir, Brussels, Belgium Looking forward and moving on Transition from pediatric to adult CF care Pr.Christiane Knoop, MD, PhD, Pr. Georges Casimir, MD, PhD Department of Chest Medicine Hôpital Universitaire des Enfants Reine Fabiola Erasme University Hospital Brussels, Belgium EAP Oslo 2015

3 Cystic Fibrosisfibrosis A complex, systemic disease involving many organs (lungs, pancreas, liver, ENT sfeer ) One birth on 2500 in caucasian population Genetic defect: Cystic Fibrose Transmembrane Regulator (more than 2000 mutations) 3

4 CYSTIC FIBROSIS IN BELGIUM 1138 patients (2010) followed in 7 CF reference centres BrusselsS(Erasme-HUDERF) and St-Luc and UZ-Brussel (3 in Brussels) Leuven: UZ Leuven (KUL) - pediatric and adult Ghent: UZ Gent - pediatric and adult Antwerp: UZA (pediatric) St. Vincentius (adult) Liège: CHR Citadelle + Clinique de l Espérance pediatric and adult 4

5 CYSTIC FIBROSIS IN BELGIUM 5

6 Mid-life expectancy and progress in medicine Any girl born today has the chance to live to a hundred regular increase in the average life expectancy of men and women (about 3 months a year) with advantage for females

7 CF registry population in the United States Bethesda 7

8 ADOLESCENCE IS A PROCESS OF TRANSITION: Chronic illness adds to its complexity Puberty Autonomy Personal identity Sexuality Educational and vocational choices may all be influenced by Physical (and/or mental) impairment Medical setbacks Pain Forced dependence Perceived (bad) prognosis Blum RWM. J Adolescent Health 1993; 14:570. 8

9 TRANSITION TRANSFER Transition (process) Planned movement of adolescents and young adults with chronic medical illnesses from child-centered to adult-oriented health-care systems Provision of optimal health-care: uninterrupted coordinated developmentally appropriate psycho-socially sound comprehensive Transfer (event) Actual movement of the responsibilty for patient care from a pediatric to an adult care setting Blum RWM. J Adolescent Health 1993; 14:570. 9

10 IMPORTANCE OF TRANSITION PROGRAMS Clear positive impacts of active transition programs Sense of responsibility and personal control is enhanced Decision-making is progressively shifted from the parents to the parents-adolescent unit to the young adult Strong messages are provided Care will be permanent issue You will survive Potential negative impacts of maintaining the status quo Discussion of critical adolescent issues could be neglected Sexuality and reproductive health Substance abuse and risk behaviours Vocational counseling and independent living The difference with peers is emphasized Incorrect messages might be given Blum RWM. J Adolescent Health 1993; 14:

11 TRANSITION AND CYSTIC FIBROSIS: Perceptions of pediatric and adult program directors Survey to 110 CFF-approved pediatric and 44 adult centre directors (1998), response rate: 66 and 73% > 50% pediatric centres did not have an approved adult centre Lack of an (competent) adult CF physician was the reason in 25% It remains true for other chronic diseases (metabolic, ) Reasons for transfer to adult centre: Age 82 % (mean of 18.5 ± 1.8 years, range 15-30) Pregnancy (25%) Marriage (17%) Reasons for NOT transferring to adult centre: Patient and family resistance (51%) Disease-severity (50%) Developmental delay (47%) Flume P. Pediatr Pulmonol 2001; 31:

12 TRANSITION AND CYSTIC FIBROSIS: Perceptions of pediatric and adult program directors Patients and their families: strong relations with pediatric Staff Needs of adult staff: Medical Developmental emotional Flume P. Pediatr Pulmonol 2001; 31:

13 TRANSITION AND CYSTIC FIBROSIS: Perceptions of pediatric and adult program directors Ratings of success of transition programs were inversely correlated with pediatricians concerns about adult staff meeting patients medical and emotional needs Flume P. Pediatr Pulmonol 2001; 31:

14 TRANSITION AND TRAINING: A course on the transition to adult care University of Alabama School of Medicine Scholars Week 3rd and 4th year students, 2-4 persons Objectives rational and affective understanding of: Principal components of hospital/home management of young CF adults Influence of chronic illness on families, especially the youngs transition to independence Impact of debilitating complications on young adults independant functioning Hagood J. Academic Med 2005; 80:

15 Program: TRANSITION AND TRAINING: A course on the transition to adult care Lectures Personal readings Scientific and non-scientific literature Film Panel discussion with pre/post-transition parents/adults Free-time for interviews with young adults and family caregivers Hagood J. Academic Med 2005; 80:

16 KEY ASPECTS OF TRANSITION 7 functional domains: - Patient preparation - Patient readiness assessment - Coordination of services - Information transfer - Primary and preventive health care - Patient follow-up and program evaluation - Transition program self-evaluation McLaughlin SE. Pediatrics 2008; 121: e

17 KEY ASPECTS OF TRANSITION: Patient preparation 28% of programs offer clinics/visits focused on transition < 25% provide educational material < 50% present a transition time-line < 50% designate a specific team member < 25% dispose of a written protocol for transition McLaughlin SE. Pediatrics 2008; 121: e

18 KEY ASPECTS OF TRANSITION: Patient readiness assessment whether a patient is independently able to : Contact team members without relying on family members / other support List their medications and function Perform airway clearance without reminders / supervision Plan for and attend clinic visits without family assistance Identify conditions requiring emergency care McLaughlin SE. Pediatrics 2008; 121: e

19 TRANSITION: CLINICAL IMPACT Duguépéroux I. J Adolescent Health 2008; 43:

20 TRANSITION: CLINICAL IMPACT Duguépéroux I. J Adolescent Health 2008; 43:

21 CF TRANSITION AT OUR CENTRE Routine weekly combined pediatric-adult CF clinic at the pediatric site, NOT especially centered on transition 21

22 CF TRANSITION AT OUR CENTRE (Transition? Transfer?) Visit at adult site Prepared and accompanied by pediatric nurse Consultation Formal explanation of habitual procedures Presentation of all team members + consultation with specific adult team members as needed Visit of out-patient in-patient facilities Visible transmission of medical file and iconography 22

23 WHAT CAN PEDIATRIC TEAMS DO BETTER? Introduce the concept of transition earlier Structure the transition program Fret LESS = trust the adult team Transfer efficiently Follow-up on their patients well-being 23

24 WHAT CAN ADULT TEAMS DO BETTER? Become AWARE of transition, actually take the pediatric team into account and TALK to them, be (a bit more) helpful and understanding Fret MORE = organize physical encounters with the patient to be transferred and his physicians Structure the program Give feed-back (to the pediatricians, to build trust) 24

25 Wider world School Work Parents Spouses Family Patient Pediatric team Adult team 25

26 CONCLUSIONS: TRANSITION IN CF Lengthy preparation Ideally between years Stable medical situation Transfer efficiently when ready A number of tools to be developed and a number of open questions 26

27 Unité de Transplantation Cardiaque & Pulmonaire Many thanks to my best-loved pediatricians... Our best loved pulmonologist... and to our multidisciplinary teams SBP 2012