1. Survivorship in Pediatric Oncology: Reclaiming Life: The Art and Challenges Beverly Rossi Ryan, M.D. Tomorrows Children’s Institute Hackensack University Medical Center

2. Pediatric Survivorship A Growing Population 5 yr EFS has increased from 50% to 80% over the past three decades 5 yr EFS has increased from 50% to 80% over the past three decades Mortality has decreased 50% Mortality has decreased 50% Incidence of childhood malignancy has increased slightly Incidence of childhood malignancy has increased slightly By 2010, it is estimated that 1 of250 adults between 18 and 45yo will be a survivor of pediatric malignancy By 2010, it is estimated that 1 of250 adults between 18 and 45yo will be a survivor of pediatric malignancy

3. Pediatric Survivorship Advocacy Office of Survivorship – NCI Office of Survivorship – NCI Follow up of late effects has become part of the mission statement of many oncology related societies Follow up of late effects has become part of the mission statement of many oncology related societies Government funding is predicated in part upon doing outcome research Government funding is predicated in part upon doing outcome research

4. Pediatric Survivorship Different Than Adult Survivorship Types of cancer seen Types of cancer seen Incidence Incidence The use of protocol studies The use of protocol studies A child is still growing when subjected to chemotherapy and/or radiation, agents designed to alter growth A child is still growing when subjected to chemotherapy and/or radiation, agents designed to alter growth

5. Pediatric Survivorship Questions Asked on Behalf of Survivors Will there be a fast forwarding of the aging process? Will there be a fast forwarding of the aging process? How will a cancer history and genetics impact upon the risk of a second malignant neoplasm? How will a cancer history and genetics impact upon the risk of a second malignant neoplasm? What are the late effects of therapy and very late effects? Can we predict earlier menopause, early onset atherosclerosis and/or early onset of adult type cancers? What are the late effects of therapy and very late effects? Can we predict earlier menopause, early onset atherosclerosis and/or early onset of adult type cancers?

6. Pediatric Survivorship Ways That Answers to These Questions Can Help Identifying toxic outcomes allows for modification of current/future therapies. This has already happened: Identifying toxic outcomes allows for modification of current/future therapies. This has already happened:  Cranial RT was discontinued as CNS prophylaxis in ALL  In Hodgkin’s Disease ABVE partially replaced MOPP chemotherapy to reduce exposure to alkylating agents. Involved field RT has reduced exposure  In Wilms tumor,RT to the abdomen has been reduced

7. Pediatric Survivorship Ways That Answers to These Questions Can Help Answers may reassure some survivors for instance the RR of SMN is low in ALL survivors Answers may reassure some survivors for instance the RR of SMN is low in ALL survivors Answers may target certain subsets of survivors to be at higher risk for certain sequelae. This would permit a more focused surveillance Answers may target certain subsets of survivors to be at higher risk for certain sequelae. This would permit a more focused surveillance

8. Pediatric Survivorship Ways That Answers to These Questions Can Help Answers may help in modeling a cost effective health delivery system; since currently it is unclear which health care provider will follow these survivors into adulthood Answers may help in modeling a cost effective health delivery system; since currently it is unclear which health care provider will follow these survivors into adulthood

9. Pediatric Survivorship Identifying Subsets RT to the whole brain <7yo and possibly at any age will have cognitive effects. RT to the whole brain <7yo and possibly at any age will have cognitive effects. RT to the head which includes the pituitary gland may have 2º hormonal effects especially growth and fertility RT to the head which includes the pituitary gland may have 2º hormonal effects especially growth and fertility RT to the lungs, heart and kidney may result in decreased function RT to the lungs, heart and kidney may result in decreased function Depending upon dose delivered, RT to the gonads may result in infertility Depending upon dose delivered, RT to the gonads may result in infertility

10. Pediatric Survivorship Identifying Subsets Certain drugs may target organs for long term effects. Survivors with moderate to high cumulative dosages of these drugs should be followed for dysfunction Certain drugs may target organs for long term effects. Survivors with moderate to high cumulative dosages of these drugs should be followed for dysfunction  anthracycline – heart  Cyclophospamide – bladder, fertility, SMN  Cisplatinum – kidneys, hearing  Ifosfmide – bladder, kidney  VP 16 – leukemia  RT - SMN

11. Pediatric Survivorship Identifying Subsets Surveillance of premature menopause especially in adolescent females who Surveillance of premature menopause especially in adolescent females who  received RT below the diaphragm  had high cummulative doses of cytoxan and/or other alkylators

12. Pediatric Survivorship Late Effect Variables Types of diagnosis Types of diagnosis Age at diagnosis Age at diagnosis Dose intensity Dose intensity Type of therapy Type of therapy

13. Pediatric Survivorship Known Late Effects Growth Growth Endocrine Endocrine Fertility/progeny Fertility/progeny Neurocognitive Neurocognitive SMN SMN Organ damage Organ damage Psychosocial Psychosocial

14. Pediatric Survivorship Emerging Late Effects Hepatitis C Hepatitis C Obesity Obesity Fatigue Fatigue Bone morbidity Bone morbidity

15. Pediatric Survivorship The Art The measurement of the overall impact of therapy is a great deal more complicated than survival. No one person can adequately address all the medical and psychosocial issues which involve survivor, family and their quality of life. It takes a team

16. Pediatric Survivorship Psychosocial Issues Post traumatic stress syndrome especially in parents Post traumatic stress syndrome especially in parents Family dynamics – dependency/independency Family dynamics – dependency/independency Self esteem/social isolation Self esteem/social isolation Body image and its effects on intimacy/sexuality Body image and its effects on intimacy/sexuality Fears of recurrence, SMN, for progeny Fears of recurrence, SMN, for progeny Disclosure - dating Disclosure - dating

17. Pediatric Survivorship Advocacy Issues Discrimination – military, insurance Discrimination – military, insurance Vocational goals Vocational goals

18. Pediatric Survivorship The Challenges Surveying for recurrence – what is reasonable? Surveying for recurrence – what is reasonable? Educating re: healthy lifestyles Educating re: healthy lifestyles Educating re: knowledge of previous Dx & Rx Educating re: knowledge of previous Dx & Rx How do we transition to adult care? How do we transition to adult care? How do we capture outcome data? How do we capture outcome data?

19. THE COMPREHENSIVE CANCER CONTROL PLAN OF NJ Our pediatric workgroup roster included: Wond Bekele and Peri Kamalakar from Beth Israel, Alice Ettinger from St Peters, Kim Kinner from ACS, Susan Murphy, Anne Nepo and Kathy Sanok from Saint Barnabus, Bev Ryan, Steve Halpern, Libby Klein, Larissa Labay and David Gordon from HUMC and Peg Knight the Executive Director of the Office of Ca Control and Prevention;

20. THE COMPREHENSIVE CANCER CONTROL PLAN OF NJ THE COMPREHENSIVE CANCER CONTROL PLAN OF NJ We met regularly and voted upon seven areas we felt were important enough to develop strategies and objectives for further consideration and inclusion in the plan; The seven areas were AYA, SMN, Pain/Palliation, Psychosocial Health of child/family, Education, Neurocognitive Deficits and Advocacy;

21. THE COMPREHENSIVE CANCER CONTROL PLAN OF NJ The entire plan was published in July of 2002; It was validating to see that areas we selected were also highlighted in the National Action Plan for Childhood Cancer and the recently published Institute of Medicine report;

22. THE COMPREHENSIVE CANCER CONTROL PLAN OF NJ On 10/22/03 we met to narrow our focus and choose several projects to work on as a group and as NJPHON; As a way of assessing psychosocial support and opportunity to access clinical trials for AYAs we are asking the county evaluators to canvas the adult programs in their areas asking several questions concerning these issues;

23. Childhood Cancer Workgroup County Evaluator Queries 1. Do you have psychosocial support to counsel adolescents and young adults and their families; and if yes, who (e.g., social worker, nurse, psychologist)? 2. Do you have adolescent and young adult patients registered on national protocols? 3. How do you follow your AYA’s, at what time intervals, and for how long? 4. What is the youngest age accepted for treatment at the adult center?

24. THE COMPREHENSIVE CANCER CONTROL PLAN OF NJ We are partnering with the advocacy group to look at the problem of reimbursement for psychosocial services and at the problem uninsured young adults; We addressed possible ways to educate primary health care providers about survivorship and the late effects of therapy. One possibility is a speaker’s panel. We need to formulate a topics list and bring it back to NJPHON for comment and volunteer participation;

25. THE COMPREHENSIVE CANCER CONTROL PLAN OF NJ We agreed on a one day survivors’ conference for a lay audience. There are funding sources for such endeavors and a number of successful programs to model; In summary, these projects are works in progress trying to improve many of the ancillary issues facing children with cancer or a history of cancer in this state.