growing up or grown up with cystic fibrosis: models of care in transition to adulthood nancy brager...
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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