Transcript Sherry L. Bayliff, MD, MPH Assistant Professor of Pediatrics Division of Pediatric Hematology/Oncology KCNPNM Conference 2014 April 15, 2014

Sherry L. Bayliff, MD, MPH
Assistant Professor of Pediatrics
Division of Pediatric Hematology/Oncology
KCNPNM Conference 2014
April 15, 2014

Aziz & Rowland, Sem Rad Oncol 2003; 13:248
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.




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




~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

The Late Effects Study Group
 early 1970s
 international consortium

Cooperative Groups
 NWTS, POG, CCG, COG

The Childhood Cancer Survivor Study
(CCSS)

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








Cardio-pulmonary Abnormalities
Autoimmune Dysfunction
Endocrine Dysfunction
Eye Problems
Bone/Joint Problems
Kidney and Genitourinary Dysfunction
Secondary Malignancies
Psychosocial/Cognitive Effects






recurrence
heart problems
second cancers
obesity
GI problems
skeletal problems






sexual problems
infertility
poor quality of life
cognitive problems
school/work issues
depression

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





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 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


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 Medicine
Oeffinger et al, 2004










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

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

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






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
Surgery
Chemotherapy
Radiation





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

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





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)

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

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



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

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



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



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




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




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




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



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







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 Gyreversible; > 3 Gy irreversible
Leydig cell function preserved usually
PE, Tanner stage, bone age, sperm analysis,
hormonal evaluation





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

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


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

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)


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

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





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.




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
2
mg/m
2
mg/m
2
mg/m
2
mg/m
2
mg/m
2
mg/m

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

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

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)

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




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


Now WHAT?!?
2014