Growing Up or Grown Up with Cystic Fibrosis:

Models of Care in Transition to Adulthood

Nancy Brager MD, FRCPCCAPM Symposium

August 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 Continued

Other 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 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

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