Long Term Follow Up of Childhood Cancer Transition of Care to Adult Care

1. Long Term Follow Up of Childhood CancerTransition of Care to Adult Care Sheila Pritchard,MD Angela Pretula,RN

2. Improvement in Cure Rate • Prior to 1970 most patients did not survive • Over the last 30 years the cure rate has steadily increased

3. Improvement in Cure Rate • Due to intensive combinations of treatment, including • Surgery • Chemotherapy • Radiation • Bone Marrow Transplant • Immune modulation • Supportive Care

4. Cost of Survival • Almost 3000 people in BC are long term survivors of childhood cancer • At least 70% will have at least one late effect of the cancer or it’s treatment • 25% will have severe or life threatening late effects • Late effects may be obvious or subtle • Can occur at any time after treatment from early childhood to late adulthood • Late effects may be exacerbated or precipitated by other health problems later in life • Late effects impact quality of life and quantity of life

5. Long Term Challenges • Second Malignancy • Cardiac • Lungs • Neurocognitive • Psychosocial • Endocrine • Fertility • Growth, Bone composition • Immunologic

6. Second Malignancy • Cumulative incidence of 3-10% at 20 years post treatment ie 5-10x increased • Breast cancer • Cumulative incidence of 35% at 20-25 years post mantle XRT • Median incidence at 15 years post XRT • Median age 31 • Thyroid,Salivary gland, Skin, Brain, Bone cancer 20 to XRT • Leukemia 20 to alkylating agents and topoisomerase II inhibitors • Genetic predisposition to cancer • Awareness of risk and early detection will improve survival

7. Second Malignancy Long Term survivor study • 8831 children with ALL (CCSG) diagnosed 1983-1995 • 63 2nd malignancies • Brain 19 • Parotid 4 • Thyroid 4 • STS 4 • Other solid tumours 4 • AML/MDS 16 • Lymphoma 8 • Cumulative incidence of 2nd malignancy 1.18% at 10 years = 7.2xincreased risk • Risk increased in females, XRT,relapse Bhatia et al, Blood02,4257-64

8. Cardiac Late Effects • Anthracycline induced cardiomyopathy • More severe in • Young age at treatment • Females • Mediastinal radiation • May be precipitated by • Pregnancy • Sudden strenuous exercise • Drugs, alcohol • Co-morbid conditions – Diabetes, smoking, obesity • Early identification and aggressive management can decrease morbidity and improve quality of life

9. Pulmonary Late Effects • Not a common late effect but significant cause of morbidity • XRT can decrease growth of chest wall and lungs • BCNU, Cyclophosphamide can cause lung damage • Chronic graft vs host disease can cause bronchilitis obliterans

10. Neurocognitive Late Effects • Cognitive impairment is one of the most debilitating sequelae • Due to • Tumour • Surgery • Radiation- Young age, High dose, large volume • Chemotherapy – HD MTX, IT chemo • Infection • Non Verbal abilities most impaired • Short term memory • Visual motor integration • Sequencing • Attention and concentration • Affects school performance, learning and social functioning

11. Psychosocial and Behavioral • Despite the intense stress of treatment most survivors achieve normal psychological and social function • A small minority are impaired by psychological problems similar to PTSD • Brain tumour survivors are less popular at school and are less likely to marry • Adult survivors of childhood cancer have less social contacts

12. Endocrine dysfunction • Affects 20-50% • Thyroid dysfunction • Growth hormone deficiency • Sex hormones • Fertility • Adrenal insufficiency • Obesity

13. Musculo Skeletal • Amputation, Limb salvage • Osteoporosis – 30% of ALL • Scoliosis

14. Organization of Follow Up Care • Who should be followed? • Why? • Where? • By Whom? • For how long? • Who should pay?

15. Why Should Patients be Followed? • For the patient • Prevention, Detection and Treatment of late effects • Advice and counselling • Security of knowing that their status is understood • For the health care team • Research into late effects and translation into improvements in current treatment • Job satisfaction • Avoid litigation! • For Society • ?Cost benefit of prevention and early detection of disease • Surveillance of offspring of survivors

16. Who should be Followed? • Contact should be maintained with all patients for life • Level of contact should be variable dependent on the likelihood of late effects • Annual visits • Letter, phone, e mail follow up with possibility of attending clinic

17. Where should they be followed? • Under age 18 • Currently >90% of patients are followed at BCCH • 2 Clinics per week • Multidisciplinary team available • Pediatric subspecialists available • Counselling, rehabilitation services need improvement • 10% of patients followed in Surrey , Victoria or by GP • Aim to increase community follow up clinics • Need appropriate training, multidisciplinary team, subspecialists

18. Where should they be followed? • Over 18 • Currently about 25% of patients are followed at the post pediatric clinic at BCCA • Mainly patients with radiation induced late effects • Rest of the patients are referred back to their GP for follow up • Most of these patients do not get regular follow up and there is minimal information received back • Most patients do not understand their risk of late effects. In many cases these risks were not known at the time they were discharged from pediatric care

19. Who Should Pay? • Ontario • $0.25 million/year provided for adult aftercare program • Aim to offer comprehensive, co-ordinated aftercare program for all adult survivors • Traceback of all patients lost to follow up • BC • Current funding for post pediatric clinic – BCCA • Aim to increase funding so that we can offer same level of follow up care as Ontario and as recommended by the Institute of Medicine in USA • Aim to set up adult follow up programs in communities • ?Linked to cancer agency programs • ?Followed by adult oncologists,internists,GP’s