Mary Johnson, a 28-year-old patient whom you have followed-up for several years, comes to your office because she feels a lump in her breast. From her previous history you know that she had Hodgkin’s disease when she was 12 years old and was treated with chest radiation. She was “cured” and has not seen anyone from the pediatric cancer center in more than 10 years.
Sarah Jones is a 24-year-old who comes to you for obstetric care. As you discuss her medical history she tells you that she had cancer when she was little but cannot remember what type or anything about her treatement.
John Smith is a 27-year-old who comes to your office as a new patient complaining of a cough, sore throat, and rhinorrhea. On the office medical questionnaire under past history he lists leukemia at the age of 5 years. In addition to his upper respiratory infection you note that he is overweight with a body mass index of 31 kg per m2.
These are typical scenarios that primary care physicians will encounter with increasing regularity over the next few years. The number of survivors of childhood cancer is increasing, and this at-risk population will seek health care from primary care physicians.
The role of the primary care physician
There is a misconception that most childhood cancer survivors will be followed indefinitely by the pediatric cancer center where they received their treatement. This is generally true in the first 5 to 10 years after completing therapy. In 1997, 53% of childhood cancer centers had a program for following long-term survivors.1 Although 44% had a mechanism for following adult survivors, only 15% of the centers had established a formal database for adults. The number of follow-up programs has gradually increased in the past few years, but because of the age limitations in most pediatric institutions, there will likely be few programs developed for survivors who have entered their young adult years. Like their peers, many survivors will move several times and often reside long distances form the center where they received their treatment. Thus, as they develop medical problems or worrisome symptoms they will usually seek care from primary care physicians on their health plan or located near their home.
Optimal health care of childhood cancer survivors requires teamwork. Integral to our training and practice as primary care physicians are methods for assessing risk for each of our patients, for developing preventive strategies, including surveillance for early disease and cancer, and for educating our patients about ways to lower identified risks. These concepts are essential to the health care of child-hood cancer survivors. However, there are few peer-reviewed articles in primary care journals or chapters in textbooks that discuss the long-term health problems of this population. To provide quality health care to survivors, it is essential that primary care physicians become educated about the health care problems related to previous cancer treatment and communicate with our colleagues who specialize in the care of children with cancer.
A growing population
As a result of advances in cancer treatment during the past 3 decades, more than 70% of the children with cancer will survive into adulthood. Some of these cancers such as Hodgkin’s disease and acute lymphoblastic leukemia have cure rates exceeding 80%.2 Currently, 1 in every 900 young adults between the ages of 20 and 45 years are survivors of childhood cancer.3 Within the next 10 years, this number may increase to 1 in every 250 young adults.4 This roughly translates into 2 to 3 survivors seen each year in the average family physician’s practice, with an expected increase to approximately 6 to 9 per year. This is often enough that we will not think of a childhood cancer survivor as we do the patient with a rare syndrome seen in residency, but uncommon enough to make us pause a moment and question his or her special needs or risks.
Increased risk
This growing population is at increased risk for a number of health-related problems. Sequelae or late effects of chemotherapy and radiation are common and may be asymptomatic for extended periods. As many as two thirds of these survivors may develop a late effect, with a significant proportion experiencing a moderate-to-severe late complication.5-8
Though some late effects are observed within a few years, many are detected a decade or more after treatment. Late effects may include fertility problems, alterations in growth and development, organ system damage, hepatitis, second malignant neo-plasms, quality-of-life issues, and employability and insurability difficulties. Overall, the cumulative incidence of a second malignant neoplasm within 20 years of diagnosis of childhood cancer is 3%, with some cancer survivors facing a much higher risk. By the age of 40 years, the cumulative actuarial risk for breast cancer is 35% for women who are survivors of childhood Hodgkin’s disease who were treated with mantle or chest radiation.9