Survivorship in Pediatric Survivorship in Pediatric Oncology: Reclaiming Life: Oncology: Reclaiming Life:

The Art and ChallengesThe Art and Challenges

Beverly Rossi Ryan, M.D.Beverly Rossi Ryan, M.D.

Tomorrows Children’s InstituteTomorrows Children’s Institute

Hackensack University Medical CenterHackensack University Medical Center

Pediatric SurvivorshipPediatric SurvivorshipA Growing PopulationA Growing Population

5 yr EFS has increased from 50% to 5 yr EFS has increased from 50% to 80% over the past three decades80% over the past three decades

Mortality has decreased 50%Mortality has decreased 50% Incidence of childhood malignancy Incidence of childhood malignancy

has increased slightlyhas increased slightlyBy 2010, it is estimated that 1 of250 By 2010, it is estimated that 1 of250

adults between 18 and 45yo will be a adults between 18 and 45yo will be a survivor of pediatric malignancysurvivor of pediatric malignancy

Pediatric SurvivorshipPediatric SurvivorshipAdvocacyAdvocacy

Office of Survivorship – NCIOffice of Survivorship – NCIFollow up of late effects has Follow up of late effects has

become part of the mission become part of the mission statement of many oncology related statement of many oncology related societiessocieties

Government funding is predicated Government funding is predicated in part upon doing outcome in part upon doing outcome researchresearch

Pediatric SurvivorshipPediatric SurvivorshipDifferent Than Adult Different Than Adult

SurvivorshipSurvivorshipTypes of cancer seenTypes of cancer seenIncidence Incidence The use of protocol studiesThe use of protocol studiesA child is still growing when A child is still growing when

subjected to chemotherapy and/or subjected to chemotherapy and/or radiation, agents designed to alter radiation, agents designed to alter growthgrowth

Pediatric SurvivorshipPediatric SurvivorshipQuestions Asked on Behalf Questions Asked on Behalf

of Survivorsof Survivors Will there be a fast forwarding of the aging Will there be a fast forwarding of the aging

process?process? How will a cancer history and genetics How will a cancer history and genetics

impact upon the risk of a second malignant impact upon the risk of a second malignant neoplasm?neoplasm?

What are the late effects of therapy and very What are the late effects of therapy and very late effects? Can we predict earlier late effects? Can we predict earlier menopause, early onset atherosclerosis menopause, early onset atherosclerosis and/or early onset of adult type cancers?and/or early onset of adult type cancers?

Pediatric SurvivorshipPediatric SurvivorshipWays That Answers to These Ways That Answers to These

Questions Can HelpQuestions Can Help Identifying toxic outcomes allows for Identifying toxic outcomes allows for

modification of current/future therapies. This modification of current/future therapies. This has already happened:has already happened: Cranial RT was discontinued as CNS Cranial RT was discontinued as CNS

prophylaxis in ALLprophylaxis in ALL In Hodgkin’s Disease ABVE partially replaced In Hodgkin’s Disease ABVE partially replaced

MOPP chemotherapy to reduce exposure to MOPP chemotherapy to reduce exposure to alkylating agents. Involved field RT has alkylating agents. Involved field RT has reduced exposurereduced exposure

In Wilms tumor,RT to the abdomen has been In Wilms tumor,RT to the abdomen has been reducedreduced

Pediatric SurvivorshipPediatric SurvivorshipWays That Answers to Ways That Answers to

These Questions Can HelpThese Questions Can Help

Answers may reassure some survivors Answers may reassure some survivors for instance the RR of SMN is low in for instance the RR of SMN is low in ALL survivorsALL survivors

Answers may target certain subsets of Answers may target certain subsets of survivors to be at higher risk for survivors to be at higher risk for certain sequelae. This would permit a certain sequelae. This would permit a more focused surveillancemore focused surveillance

Pediatric SurvivorshipPediatric SurvivorshipWays That Answers to Ways That Answers to

These Questions Can HelpThese Questions Can Help

Answers may help in modeling a Answers may help in modeling a cost effective health delivery cost effective health delivery system; since currently it is system; since currently it is unclear which health care provider unclear which health care provider will follow these survivors into will follow these survivors into adulthoodadulthood

Pediatric SurvivorshipPediatric SurvivorshipIdentifying SubsetsIdentifying Subsets

RT to the whole brain <7yo and possibly RT to the whole brain <7yo and possibly at any age will have cognitive effects.at any age will have cognitive effects.

RT to the head which includes the RT to the head which includes the pituitary gland may have 2pituitary gland may have 2º hormonal º hormonal effects especially growth and fertilityeffects especially growth and fertility

RT to the lungs, heart and kidney may RT to the lungs, heart and kidney may result in decreased functionresult in decreased function

Depending upon dose delivered, RT to Depending upon dose delivered, RT to the gonads may result in infertilitythe gonads may result in infertility

Pediatric SurvivorshipPediatric SurvivorshipIdentifying SubsetsIdentifying Subsets

Certain drugs may target organs for long term Certain drugs may target organs for long term effects. Survivors with moderate to high effects. Survivors with moderate to high cumulative dosages of these drugs should be cumulative dosages of these drugs should be followed for dysfunctionfollowed for dysfunction anthracycline – heartanthracycline – heartCyclophospamide – bladder, fertility, SMNCyclophospamide – bladder, fertility, SMNCisplatinum – kidneys, hearingCisplatinum – kidneys, hearing Ifosfmide – bladder, kidneyIfosfmide – bladder, kidneyVPVP16 16 – leukemia– leukemiaRT - SMNRT - SMN

Pediatric SurvivorshipPediatric SurvivorshipIdentifying SubsetsIdentifying Subsets

Surveillance of premature Surveillance of premature menopause especially in menopause especially in adolescent females whoadolescent females who received RT below the received RT below the

diaphragmdiaphragm had high cummulative doses of had high cummulative doses of

cytoxan and/or other alkylatorscytoxan and/or other alkylators

Pediatric SurvivorshipPediatric SurvivorshipLate Effect VariablesLate Effect Variables

Types of diagnosisTypes of diagnosisAge at diagnosisAge at diagnosisDose intensityDose intensityType of therapyType of therapy

Pediatric SurvivorshipPediatric SurvivorshipKnown Late EffectsKnown Late Effects

GrowthGrowthEndocrineEndocrineFertility/progenyFertility/progenyNeurocognitiveNeurocognitiveSMNSMNOrgan damageOrgan damagePsychosocial Psychosocial

Pediatric SurvivorshipPediatric SurvivorshipEmerging Late EffectsEmerging Late Effects

Hepatitis CHepatitis CObesityObesityFatigueFatigueBone morbidityBone morbidity

Pediatric SurvivorshipPediatric SurvivorshipThe ArtThe Art

The measurement of the overall The measurement of the overall impact of therapy is a great deal more impact of therapy is a great deal more complicated than survival.complicated than survival.

No one person can adequately No one person can adequately address all the medical and address all the medical and psychosocial issues which involve psychosocial issues which involve survivor, family and their quality of life.survivor, family and their quality of life.

It takes a teamIt takes a team

Pediatric SurvivorshipPediatric SurvivorshipPsychosocial IssuesPsychosocial Issues

Post traumatic stress syndrome Post traumatic stress syndrome especially in parentsespecially in parents

Family dynamics – Family dynamics – dependency/independencydependency/independency

Self esteem/social isolationSelf esteem/social isolationBody image and its effects on Body image and its effects on

intimacy/sexualityintimacy/sexualityFears of recurrence, SMN, for progenyFears of recurrence, SMN, for progenyDisclosure - datingDisclosure - dating

Pediatric SurvivorshipPediatric SurvivorshipAdvocacy IssuesAdvocacy Issues

Discrimination – military, Discrimination – military, insuranceinsurance

Vocational goalsVocational goals

Pediatric SurvivorshipPediatric SurvivorshipThe ChallengesThe Challenges

Surveying for recurrence – what is Surveying for recurrence – what is reasonable?reasonable?

Educating re: healthy lifestylesEducating re: healthy lifestylesEducating re: knowledge of Educating re: knowledge of

previous Dx & Rxprevious Dx & RxHow 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?

THE COMPREHENSIVE THE COMPREHENSIVE CANCER CONTROL PLAN OF CANCER CONTROL PLAN OF

NJNJOur 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;

THE COMPREHENSIVE CANCER THE COMPREHENSIVE CANCER CONTROL PLAN OF NJCONTROL 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;

THE COMPREHENSIVE CANCERTHE COMPREHENSIVE CANCER CONTROL PLAN OF NJCONTROL 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;

THE COMPREHENSIVE THE COMPREHENSIVE CANCER CONTROL PLAN OF CANCER CONTROL PLAN OF

NJNJOn 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;

Childhood Cancer WorkgroupChildhood Cancer WorkgroupCounty Evaluator QueriesCounty 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?

THE COMPREHENSIVE CANCER THE COMPREHENSIVE CANCER CONTROL PLAN OF NJCONTROL 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;

THE COMPREHENSIVE THE COMPREHENSIVE CANCER CONTROL PLAN OF CANCER CONTROL PLAN OF

NJNJWe 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.