‘Primary’ Care of theChildhood Cancer Survivor

Sherry L. Bayliff, MD, MPHAssistant Professor of Pediatrics

Division of Pediatric Hematology/Oncology

KCNPNM Conference 2014April 15, 2014

“Cancer today is a curable disease for many and a

chronic illness for most”

Aziz & Rowland, Sem Rad Oncol 2003; 13:248

Educational Objectives

To understand the multiple long term health issues our pediatric cancer survivors face.

To appreciate the services that can be provided by Long-Term Follow-Up Care.

To recognize the importance of “Risk Based Care”.

To recognize the challenges/barriers faced when trying to deliver survivorship care.

Survival Statistics

every day 42 children are diagnosed with cancer

spares no ethnic, gender or socioeconomic group

>80% of pediatric cancer patients will become “long term” survivors ~375,000 childhood cancer survivors ~1 in every 530 adults (aged 20-39 yrs)

Hewitt, Weiner, & Simone, 2003

It Doesn’t Stop at CURE . . .

~60% of CCS will suffer Late Effects

1 in 3 remain free of long term problems

42% of LE will be severe, life threatening, or fatal

The incidence increases over time for

most LE

Late Effects . . . the History

The Late Effects Study Group early 1970s international consortium

Cooperative Groups NWTS, POG, CCG, COG

The Childhood Cancer Survivor Study (CCSS)

Late Effects after Cancer Treatment

A study of 10,397 participants in the CCSS (compared with 3,034 of their siblings) 73% with at least 1 chronic health condition by

40 yrs old 42% categorized as severe, life-threatening, or

fatal 3.3 x more likely to have a chronic health

condition 4.0 x more likely to have 2 or more chronic

health conditions incidence of chronic conditions increases over

time (no plateau)

Oeffinger et al. 2006

Late Effects after Cancer Treatment

Cardio-pulmonary Abnormalities Autoimmune Dysfunction Endocrine Dysfunction Eye Problems Bone/Joint Problems Kidney and Genitourinary Dysfunction Secondary Malignancies Psychosocial/Cognitive Effects

Common Concerns of Survivors

recurrence heart problems second cancers obesity GI problems skeletal

problems

sexual problems infertility poor quality of

life cognitive

problems school/work

issues depression

Etiology of Late Effects

Underlying Diagnosis bone cancer, CNS tumors, Hodgkin's highest

risk Intensity of Treatment Regimens

radiation doses, accumulative chemo doses Transplant Related

conditioning therapies, chronic GVHD Multifactorial

genetic predisposition, age at time of dx, immunodeficiency, health behaviors

Comprehensive Long Term Follow Up (LTFU) Care Initiatives

a cooperative team effort majority of CCSs continue care not at

cancer center lack of knowledge by provider lack of understanding and risk

awareness on part of the survivor lack of recognition of the need for

Risk Based Care

A “Risk-Based” Approach

“a systematic plan for lifelong screening, surveillance, and prevention that incorporates risks based on the previous cancer, cancer therapy, genetic predispositions, lifestyle behaviors, and comorbid health conditions”

Oeffinger, 2004

A “Primary” Issue

only 53% of cancer centers had a developed LTFU program as of 1997

care provided by PCPs lack of communication lack of educating materials small percentage of the PCP’s practice large investment of resources

Annals of Family MedicineOeffinger et al, 2004

LTFU Care: Key Services

monitor for/manage physical late effects provide health education provide referrals to specialists and resources encourage wellness/health promotion activities address psychosocial needs assess/provide intervention for

educational/vocational needs assist with financial/insurance issues guide transition: pediatric adult-focused care empower survivors to advocate for their own

needs facilitate research

Models of LTFU Care

Cancer Center Models Primary Oncology Care Specialized LTFU Clinic Shared Care

Young Adult Transition Models Formalized Transition Programs Adult Oncology-Directed Care

Community-Based Care Models

Need-Based Models

Community-Based Care

close contact with the pediatric oncology team (consultative basis) individual risk factors updated screening recommendations transition at specific time points for

continued care provision of primary care

Specialized LTFU Clinic

most common model transition w/in same cancer center examines/evaluates the patient risk-based screening

recommendations education about potential late effects encourages primary care continuum

“No matter what model is chosen, an educated survivor who is empowered to be an active participant in their own life-long care is the cornerstone of all successful survivorship care”

--in “Establishing and Enhancing Services for Childhood Cancer Survivors: Long-Term Follow-Up Program Resource Guide” ; COG 2007

Late Effects by Therapy Type

Surgery

Chemotherapy

Radiation

Secondary Malignancies

overall incidence 12.6% (@25 yrs) 10-20 fold lifetime risk compared to age

matched controls leading cause of death behind

recurrence multifactorial in etiology AML most common

almost always preceded by myelodysplasia, genetic abnormalities

Solid tumors associated with history of XRT

Secondary Leukemia

Chemotherapy Alkylating agents

▪ delay to onset 5-10 yrs▪ dose related

Topoisomerase II Inhibitors▪ delay to onset 2-3 yrs▪ correlates with dose intensity and schedule

Combination therapy▪ increased risk with increased number of cycles

Radiation risk peaks at 4-9 yrs inverse relationship with dose

Secondary Solid Tumors

doses chemo family history 1o cancer was soft tissue sarcoma,

Hodgkin’s lymphoma, or bone tumor other secondary cancer Radiation (>30 Gy highest risk)

9-fold higher incidence than age matched controls

delay to onset peaks > 10 yrs post XRT (no plateau)

Solid Tumors in HL Survivors

Breast Cancer XRT rates by 10-20% at 20 yrs cumulative incidence @ 20-25 yrs post is

35% volume of radiation delivery risk begins to increase 8 yrs after XRT risk decreased if other therapies induce

premature menopause Mammography, breast exam, MRI

Cardiac Late Effects

Chemotherapy Anthracyclines & hi-dose Cyclophosphamide cumulative dose related

▪ >450 mg/m2 doxorubicin has 5-11% risk cardiac dz▪ 400-600 mg/m2 risk is nearly 23%▪ >800 mg/m2 risk is 100%

Asymptomatic ventricular dysfunction

Radiation coronary artery dz, pericarditis, ventricular

dysfunction, valvular disease risk decreases as patient ages

Evaluating for Cardiac Late Effects

Hx & Physical Exam Review of Systems ECHO/MUGA every 2-5 years

Normal: FS > 29%; LVEF > 55% Abnormal: decrease of 10% of previous

or < nl EKG—findings late and nonspecific careful evaluation during 3rd

trimester of pregnancy

Pulmonary Late Effects

Chemotherapy-related (rare) Bleomycin, nitrosurea, CTX, Ciplatinum, MTX pneumonitis, fibrosis, acute hypersensitivity,

noncardiogenic pulmonary edema cumulative dose relationship risk further increased by supplemental O2, older age,

smoking, renal dysfunction, infections, prior mediastinal XRT

Radiation 5-15% risk of pneumonitis after XRT for lung cancer with concomitant chemo, prior XRT, steroids, young age Increased w/higher cumulative doses and daily fractions

Evaluating for Pulmonary Late Effects

Hx & Physical Exam Review of Systems PFTs

baseline 6-23 months after end of therapy

repeat q 2-5 years if normal at baseline Imaging Lung biopsy

Endocrine Late Effects

most commonly growth hormone deficiency and thyroid dysfunction

present as decreased linear growth, abnormal musculoskeletal maturation or signs/sxs of thyroid dz

greatest risk associated w/XRT to neck or Hypothalamic-Pituitary-Growth Hormone axis

Growth Hormone Replacement

may occur w/o growth hormone deficiency

cancer free for 1-2 years may worsen degree of scoliosis or

induce benign intracranial hypertension

controversial risk of inducing second cancer

Thyroid Late Effects

incidence 10-28% with low dose XRT to neck

delay to onset of 5 years, increases until 20 yrs

XRT > 20-30 Gy to neck greatest risk palpable thyroid is abnormal

Ultrasound and nuclear scanning Biopsy if nodule found

screening TSH yearly FT3/FT4 if TSH increased

CNS Late Effects

Brain tumors (greatest) and ALL neurocognitive dysfunction greatest

morbidity female, < 3 years, increased time

from therapy 4 primary therapy induced

pathologies: leukoencephalopathy mineralizing microangiopathy subacute necrotizing

leukoencephalopathy secondary brain tumors

Reproductive Late Effects: Ovaries age and gender specific many survivors are unaware of their risks Ovaries:

greatest ovarian risk: postpubertal + hi-dose alkylators

standard chemo doses: retain/recover function increased risk w/increased number cycles of

combination therapy > 20 Gy pelvic XRT permanent ovarian failure Assess bone age, U/S ovaries, thyroid studies,

hormonal evaluation

Reproductive Late Effects: Testis

boys much more sensitive age and pubertal status little impact CTX 300-350 mg/kg sterility 20% may recover after combo tx;

50% remain sterile XRT 1-3 Gyreversible; > 3 Gy

irreversible Leydig cell function preserved

usually PE, Tanner stage, bone age, sperm

analysis, hormonal evaluation

GU Late Effects

markedly reduced by chemoprotectant drugs and limited cumulative dosing

acute tubular dysfunction w/alkylating agents or XRT 20-30 Gy to kidneys

Fanconi renal wasting hypo-phosphatemic rickets dribbling and nocturnal enuresis

Ocular Late Effects

Radiation TBI most common association >50 Gy: neovascularity, glaucoma,

atrophy of iris, retinal infarction, exudates, hemorrhage, optic neuropathy, decreased tearing and fibrosis of lacrimal glands

>40 Gy: ulceration, neovascularization, keratinization, edema of the cornea

Cataracts Corticosteroids and/or XRT 10-15 Gy

Auditory Late Effects

chronic OM with 40-50 Gy to middle ear

Sensorineural hearing loss 40-50 Gy radiation to middle ear Cisplatin

▪ exaggerated by aminoglycoside use continue Audiology plan made during

therapy

GI Late Effects

Radiation > 40 Gy enteritis esophagus through colon hepatitis/fibrosis/cirrhosis Intensified by concurrent use of

dactinomycin/adriamycin Early colorectal screening

Pelvic or abdominal XRT >25 Gy Start 15 yrs post treatment or age 35 yrs

(later event)

Psychological Factors Affecting Care

fear/anxiety of another cancer; a wish to leave it all behind; and unresolved feelings

Interventions discuss LTFU plans before treatment

ends familiarize the survivor with the plan for

transition encourage survivors to be proactive

—”self care” encourage healthy lifestyle behaviors

Financial/Insurance Issues Unemployed=uninsured; mobility due to

school/employment; childhood cancer as a preexisting condition; survivors may “age out” of existing insurance coverage; restriction of coverage; outright cost of healthcare prohibitive in the uninsured

Interventions provide information regarding government

programs related to special needs/disability develop a directory of community resources

and referrals provide financial/insurance counseling

The Kentucky Children’s Childhood Cancer Survivorship

Clinic

August 16th, 2007 >2-5 years off therapy Oncologist, Nurse Coordinator, Social

Worker expanded clinic visits to reduce

waiting time collaboration with the UK Med/Peds

Clinic, Pediatricians, Family Practice Groups, etc.

Services Provided

Pre-Clinic Questionnaire Physical and Psychosocial Assessment Cancer Treatment Summary Educational Materials

LAF Survivors Handbook Individualized Health Links Resource Directory

Visit Summaries Referral to Subspecialists

SUMMARY OF CANCER TREATMENT

Demographics

Name: Sex: Date of Birth:

Address:

Phone: SS# Race/Ethnicity:

Alternate contact: Relationship: Phone:

Cancer Diagnosis

Diagnosis:

Date of Diagnosis: Age at Diagnosis: Date Therapy Completed:

Sites involved/stage/diagnostic details: Laterality:

Hereditary/congenital history:

Pertinent history:

Past medical history:

Family history:

Treatment Center #1:

Medical Record #:

MD/APN Contact Information:

Treatment Center #2:

Medical Record #:

MD/APN Contact Information:

Relapse(s) Date:

Site(s): Laterality:

Date Therapy Completed:

CANCER TREATMENT SUMMARY Protocol

Acronym/Number Title/Description Initiated Completed On-Study

Surgery Date Procedure Site (if applicable) Laterality Surgeon/Institution

Chemotherapy Drug Name Route Cumulative Dose

mg/m2

mg/m2

mg/m2

mg/m2

mg/m2

mg/m2

Resources for Practitioners

http://www.survivorshipguidelines.org Children’s Oncology Group Long-Term

Follow-Up Guidelines for Survivors of Childhood, Adolescent, and Young Adult Cancers

Health Links Summary of Cancer Treatment template Late Effects Directory of Services Long-Term Follow-Up Program Resource

Guide

Resources for LTFU Care

Survivors of Childhood and Adolescent Cancer: A Multidisciplinary Approach; Heidelberg: Springer, 2005

Late Effects of Childhood Cancer; London: Arnold, 2004

Childhood Cancer Survivorship: Improving Care and Quality of Life; Washington, DC: The National Academies Press, 2003

Patient Education Materials

Childhood Cancer Survivors: A Practical Guide to Your Future (2nd Edition); Sebastopol, CA, O’Reilly Media, Inc., 2007 (www.candlelighters.org/Book_Order_Form.pdf)

Children’s Oncology Group Health Links, 2006 (www.survivorshipguidelines.org)

The Future--Research

to better understand identified late effects i.e. “metabolic syndrome” and obesity

to identify newly occurring late effects

to better understand quality of life issues

to develop targeted therapies to reduce/prevent late effects

Special Thanks . . .

Jennifer Ballard, RN, CCRP Clinic Nurse Coordinator

Kara Gore, MSW Clinical Social Worker

Pediatric H/O Division physicians, nurses, research & administrative staff

Dr. Lars Wagner Pediatric Hematology/Oncology Division Chief

Stacy Carter, RN, CPON Wendy Landier, RN, MSN, CPNP, CPON

City of Hope National Medical Center DanceBlue Northwest Mutual Cowboy Up for a Cure Kids Cancer Alliance

I SURVIVED!

Now

WH

AT?!?

2014