Growing Up or Grown Up with Cystic Fibrosis: Models of Care in Transition to Adulthood

1. Growing Up or Grown Up with Cystic Fibrosis: Models of Care in Transition to Adulthood Nancy Brager MD, FRCPC CAPM Symposium August 27, 2009

2. CF 101 • Most common autosomal recessive fatal gene, chromosome 7 • 71 % DF508 or DI507, 95% Caucasian • 1:3600 children born in Canada have CF • Mean age of diagnosis 3.5 y (2002) • Median age of survival in Canada 37 years (2002) • 3500 individuals attend CF Clinics (38) • >50% adults

3. CF 101 Continued Disease Manifestations and Complications: • Pulmonary Disease • Chronic infection • Bronchiectasis • Pneumothorax • Gastrointestinal Disease • Obstipation • Malnourishment • Liver Disease • Reflux • Cancer

4. CF 101 Continued Other Disease Manifestations and Complications: • Diabetes • Osteoporosis • Infertility (male) • Psychosocial • Others

5. CF 101 Continued • Survival has at least doubled over the past 25 years • Diagnosis • Nutrition • Physiotherapy/exercise • Gene discovery • Transplant • Pharmaceutical

6. CF 201 Psychiatric conditions in CF • Depression – MDD, BDD, Substance induced • Anxiety Disorders • ADHD • Substance abuse, addiction • Adjustment disorders • Relationship problems and occupational (school) • Eating disorder (CFED) • Poor adherance to medical care • Psychological factors… • ? Low incidence of psychotic disorders

7. Transitions in CF • Diagnosis • Pediatric to adult care • CF Clinic to Transplant Clinic • Transplant Clinic to CF Clinic • Palliative care

8. Transition to Adult Care • CFF mandated adult care models and transition for patients over 21 in 1996 • Transition is defined “the purposeful, planned movement of adolescents and young adults with chronic physical and medical conditions from child-centered to adult-oriented health care systems” • Median age of transition 19y (US)

9. Transition Activities (McLaughlin et al, 2008) • Patient preparation • Patient readiness assessment • Coordination of services/benefits • Information transfer • Primary and preventative health care • Patient follow up • Program evaluation

10. Transition Care(McLaughlin et al, 2008) • Discussion about transition, median age 17 • Transition process can include: • Family support • Age-appropriate preventive care: • Substance abuse • Smoking • Dieting behaviour • Sexual activity • Self management skills

11. Transition Care(McLaughlin et al, 2008)

12. Transition Care

13. Erikson’s Stages of Psychosocial Development Theory Meng, A. and Meng, H. Jan. 27, 2006 Erik Erikson’s Stages of Psychosocial Development – Summary Chart. Retrieved August 24, 2009from www.vtaide.com/png/Erikson.html.

14. Erikson’s Stages of Psychosocial Development Theory Meng, A. and Meng, H. Jan. 27, 2006 Erik Erikson’s Stages of Psychosocial Development – Summary Chart. Retrieved August 24, 2009from www.vtaide.com/png/Erikson.html.

15. Transition Care • Cognizant of developmental appropriateness • Stages of development with respect to psychosocial crises

16. Transition Care • Identity vs. Identity Confusion (too late? better during Industry vs. Inferiority) • Intimacy vs. Isolation • Generativity vs. Stagnation (transplant)

17. Transition Care Summary • Multiple transitions • Early, developmentally appropriate introduction for patients and families • Communication • Formalized programs likely have benefit but tend to be more site specific • Options for some aspects of transitional care