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IOM REPORT

Better Follow-up Care Needed for Childhood Cancer Survivors

Eastman, Peggy

doi: 10.1097/01.COT.0000291625.27873.c7
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WASHINGTON, DC—Thirty years ago ongoing high-quality care for childhood cancer survivors was not an issue, because prior to 1970 few children under the age of 20 were cured. But since then, according to a new report from the National Cancer Policy Board (NCPB) of the Institute of Medicine (IOM), five-year survival rates have surged to 78%, and the number of childhood cancer survivors alive today continues to grow.

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But this medical success story has come at a high price for patients: late effects of their treatments or their disease (including second cancers), leading to pain, unwanted complications, disabilities, and adverse outcomes.

Nearly two thirds of all childhood cancer survivors—most of whom are under age 40—are likely to experience at least one unwanted late effect, and as many as one fourth may experience a late effect that is severe or life-threatening, according to the new report, Childhood Cancer Survivorship: Improving Care and Quality of Life.

New SEER Data

New data specific to cancers in children under 20 years old from the National Cancer Institute's Surveillance, Epidemiology, and End Results (SEER) program show that overall the incidence of childhood cancers rose from 1975 to about 1991 (a rise attributed in part to better diagnosis and case identification); incidence rates flattened out and dropped slightly from 1991 to 2000. During that same time period—1975 to 2000—overall mortality rates for childhood cancers declined steadily.

The risk of second cancers 20 years after primary treatment for childhood cancer varies between 3% and 10%, and is five to 20 times that in the general population.

In addition to second cancers, adverse late effects may be neurocognitive and psychological; cardiopulmonary; endocrine (such as those affecting growth and fertility); and musculoskeletal.

These late effects may be exacerbated if the survivor develops an additional health problem; for example, renal dysfunction due to treatment with ifosfamide could be accelerated if the patient develops high blood pressure or diabetes mellitus, according to the report.

To help childhood cancer survivors receive the best ongoing health care, the report makes seven specific recommendations (see box).

The 206-page report was co-edited by NCPB Chair Joseph V. Simone, MD, President of Simone Consulting in Dunwoody, Ga.; Clinical Director Emeritus of the Huntsman Cancer Institute at the University of Utah; and a member of OT's Editorial Board.

While childhood cancer survivors represent the best of the medical system, the new report emphasizes that the very system that saved their lives must not abandon these patients once the treatment for their primary cancer is over.

Another member of OT's Editorial Board, Ellen Stovall, President and CEO of the National Coalition for Cancer Survivorship, is one of three vice-chairs of the NCPB — which was established at the IOM in March 1997.

Leukemia is the most common childhood cancer, notes the report, accounting for fully 25% of all malignancies diagnosed before the age of 20. Two leukemias primarily strike children: acute lymphoblastic leukemia (ALL)—which accounts for some three fourths of all childhood leukemia—and acute myeloid leukemia (AML).

In addition to the leukemias, children also develop central nervous system and brain tumors; lymphomas; carcinomas and other epithelial tumors; germ cell tumors; soft tissue sarcomas; bone tumors; neuroblastoma; renal tumors; and retinoblastoma.

Biological Differences

The success story of childhood cancer survivorship—a 78% five-year childhood cancer survival rate vs a 62% five-year adult survivor rate—is due in part to biological differences between adult and childhood cancers, according to Dr. Simone.

His research (cited in the report) has found that while about 90% of adult cancers are carcinomas derived from epithelial tissue that lines cavities or glands—such as cancers of the breast, prostate, lung, colorectum, uterus, and ovary—childhood cancers tend to arise from non-ectodermal tissue such as bone marrow, lymph glands, bone and muscle.

The report makes the point that these age-related differences in microscopic type affect tumor development and response to therapy. In addition, latency periods are much shorter for childhood cancers: one to 10 years, vs 10 to 30 years for adult carcinomas.

Many childhood cancers appear to arise from embryonic abnormalities; these, such as Wilms' tumor of the kidney, are less resistant to radiotherapy and chemotherapy than are adult carcinomas.

Wide Range of Adverse Late Effects

The range of adverse late effects, as described in the report, is wide. Children who survive childhood leukemia into adulthood may experience learning disabilities; abnormal growth and maturation; heart problems; second cancers; and hepatitis C as an effect of blood transfusions. They may also develop osteoporosis, avascular necrosis of bone and dental problems.

Children who survive brain cancer into adulthood may have hearing loss as well as learning disabilities; kidney damage; infertility; vision problems; and second cancers.

Those with bone tumors may have to undergo amputation, with loss of mobility; have damage to soft tissues from radiation, including scarring and swelling; and have kidney problems, among others.

Adults treated as children for Wilms' tumor may experience heart and kidney problems; scoliosis; damage to soft tissues; second cancers; and fertility problems.

Some late effects in childhood cancer survivors show up years after treatment, starting with an asymptomatic period and then becoming symptomatic with disease progression.

Provocative Issue

Although most childhood cancer survivors today are under age 40, the new report raises a provocative issue: What impact late effects might have on childhood cancer survivors who live into old age.

Thus the NCPB poses these questions, among others:

  • ▪ Will survivors who experience cognitive dysfunction from cranial irradiation develop premature dementia-like illness?
  • ▪ Will survivors of soft tissue sarcoma be at increased risk for premature joint deterioration?
  • ▪ Will organ toxicity predispose survivors to premature organ failure?

To answer these questions, long-term follow-up studies of childhood cancer survivors are a must, the report emphasizes.

The NCPB's 1999 report, Ensuring Quality Cancer Care, concluded that for many Americans with cancer, there is a wide gulf between the ideal and the reality of their experience with cancer care.

While childhood cancer survivors represent the best of the medical system, the new report emphasizes that the very system that saved their lives must not abandon these patients once the treatment for their primary cancer is over.

7 Recommendations on High-Quality Care for Childhood Cancer Survivors

The following are the seven recommendations in the new IOM report:

  1. Develop evidence-based clinical practice guidelines for the care of childhood cancer survivors.
  2. Define a minimum set of standards for systems of comprehensive, multidisciplinary follow-up care that links primary care providers with specialists, and ensure the presence of such a system in cancer centers.
  3. Increase awareness of late effects and their implications for health among childhood cancer survivors and their families.
  4. Improve professional education and training on late effects and their management for both specialists and primary care providers.
  5. Support the Maternal and Child Health Bureau of the federal Health Resources and Services Administration (HRSA) in implementing the government's Healthy People 2010 goals for children with special health care needs.
  6. Enhance childhood cancer survivors' access to resources and health delivery systems through federal, state and private efforts aimed at health insurance reforms and support of safety net programs such as HRSA's Community and Migrant Health Centers.
  7. Increase support for research funded by the NCI, the American Cancer Society, and others aimed at preventing or ameliorating the long-term consequences of childhood cancer. Priority areas of research include: assessing the prevalence and etiology of late effects; testing methods that may reduce late effects during treatment; developing interventions to prevent or reduce late effects after treatment; and furthering improvements in quality of care to counter the consequences of late effects for survivors and their families.
© 2003 Lippincott Williams & Wilkins, Inc.
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