Growing Up or Grown Up with Cystic Fibrosis:

Models of Care in Transition to Adulthood

Nancy Brager MD, FRCPCCAPM SymposiumAugust 27, 2009

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

CF 101 Continued

Disease Manifestations and Complications:• Pulmonary Disease

– Chronic infection– Bronchiectasis– Pneumothorax

• Gastrointestinal Disease– Obstipation– Malnourishment– Liver Disease– Reflux– Cancer

CF 101 ContinuedOther Disease Manifestations and

Complications:• Diabetes• Osteoporosis• Infertility (male)• Psychosocial• Others

CF 101 Continued• Survival has at least doubled over the past

25 years– Diagnosis– Nutrition– Physiotherapy/exercise– Gene discovery– Transplant– Pharmaceutical

CF 201Psychiatric 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

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

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)

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

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

Transition Care(McLaughlin et al, 2008)

Transition Care

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

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, 2009 from www.vtaide.com/png/Erikson.html.

Erikson’s Stages of Psychosocial Development Theory

Transition Care

• Cognizant of developmental appropriateness

• Stages of development with respect to psychosocial crises

Transition Care

• Identity vs. Identity Confusion (too late? better during Industry vs. Inferiority)

• Intimacy vs. Isolation• Generativity vs. Stagnation (transplant)

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