Childhood Cancer Survivors and Primary Care Physicians

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

Another common effect is late onset anthracy-cline-induced cardiomyopathy. More than half of the survivors of childhood cancer who were treated with moderate to high doses of an anthracycline will develop changes in left ventricular function which, over time, can lead to a stiff and poorly compliant left ventricle.¹⁰ Most patients are asymptomatic, but a significant proportion may develop overt congestive heart failure with aging or following physical stressors, such as pregnancy. A low-risk pregnancy may actually be an unrecognised high-risk condition for some childhood cancer survivors.

The impact of cancer therapy on the development of common adult health problems is just beginning to be understood. What will be the effect of mantle or chest radiation on the progression of an atherosclerotic plaque in a coronary artery? What type of cardiovascular risk will an obese, physically inactive leukemia survivor face? Early evidence¹¹ suggests that survivors of childhood acute lymphoblastic leukemia who were treated with cranial irradiation are more likely to be obese and may be at increased risk for premature cardiovascular disease.

Primary care phsicians as researchers

There are studies in progress that are investigating the risks and health needs of childhood cancer survivors. A notable example is the Childhood Cancer Survivor Study, a 25-institution National Cancer Institute-funded cohort of more than 14,000 long-term survivors.

Within this growing field of research there is a need for investigators with a primary care back-ground. Primary care physicians are trained and experienced in risk assessment, risk modification, cancer surveillance, and patient education. These same skills are vital to a research team. There are ample opportunities for primary care researchers to network with investigators from a wide array of back-grounds to study the health needs of this population.

Family physicians are likely to see an increasing number of adult survivors of childhood cancer, a population with special health problems. It is essential that our discipline become better acquainted with these health needs and collaborate in studies to determine cost-effective methods for screening for late effects and second cancers.

Acknowledgments

Dr Oeffinger received partial support for this work through the American Academy of Family Physicians Foundation Advanced Research Training Grant and the Robert Wood Johnson Foundation Generalist Physician Faculty Scholars Program. He would like to thak Dr Shelley Roaten, Jr, and Dr George Buchanan for their critical review.

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

Another common effect is late onset anthracy-cline-induced cardiomyopathy. More than half of the survivors of childhood cancer who were treated with moderate to high doses of an anthracycline will develop changes in left ventricular function which, over time, can lead to a stiff and poorly compliant left ventricle.¹⁰ Most patients are asymptomatic, but a significant proportion may develop overt congestive heart failure with aging or following physical stressors, such as pregnancy. A low-risk pregnancy may actually be an unrecognised high-risk condition for some childhood cancer survivors.

The impact of cancer therapy on the development of common adult health problems is just beginning to be understood. What will be the effect of mantle or chest radiation on the progression of an atherosclerotic plaque in a coronary artery? What type of cardiovascular risk will an obese, physically inactive leukemia survivor face? Early evidence¹¹ suggests that survivors of childhood acute lymphoblastic leukemia who were treated with cranial irradiation are more likely to be obese and may be at increased risk for premature cardiovascular disease.

Primary care phsicians as researchers

There are studies in progress that are investigating the risks and health needs of childhood cancer survivors. A notable example is the Childhood Cancer Survivor Study, a 25-institution National Cancer Institute-funded cohort of more than 14,000 long-term survivors.

Within this growing field of research there is a need for investigators with a primary care back-ground. Primary care physicians are trained and experienced in risk assessment, risk modification, cancer surveillance, and patient education. These same skills are vital to a research team. There are ample opportunities for primary care researchers to network with investigators from a wide array of back-grounds to study the health needs of this population.

Family physicians are likely to see an increasing number of adult survivors of childhood cancer, a population with special health problems. It is essential that our discipline become better acquainted with these health needs and collaborate in studies to determine cost-effective methods for screening for late effects and second cancers.

Acknowledgments

Dr Oeffinger received partial support for this work through the American Academy of Family Physicians Foundation Advanced Research Training Grant and the Robert Wood Johnson Foundation Generalist Physician Faculty Scholars Program. He would like to thak Dr Shelley Roaten, Jr, and Dr George Buchanan for their critical review.

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

Another common effect is late onset anthracy-cline-induced cardiomyopathy. More than half of the survivors of childhood cancer who were treated with moderate to high doses of an anthracycline will develop changes in left ventricular function which, over time, can lead to a stiff and poorly compliant left ventricle.¹⁰ Most patients are asymptomatic, but a significant proportion may develop overt congestive heart failure with aging or following physical stressors, such as pregnancy. A low-risk pregnancy may actually be an unrecognised high-risk condition for some childhood cancer survivors.

The impact of cancer therapy on the development of common adult health problems is just beginning to be understood. What will be the effect of mantle or chest radiation on the progression of an atherosclerotic plaque in a coronary artery? What type of cardiovascular risk will an obese, physically inactive leukemia survivor face? Early evidence¹¹ suggests that survivors of childhood acute lymphoblastic leukemia who were treated with cranial irradiation are more likely to be obese and may be at increased risk for premature cardiovascular disease.

Primary care phsicians as researchers

There are studies in progress that are investigating the risks and health needs of childhood cancer survivors. A notable example is the Childhood Cancer Survivor Study, a 25-institution National Cancer Institute-funded cohort of more than 14,000 long-term survivors.

Within this growing field of research there is a need for investigators with a primary care back-ground. Primary care physicians are trained and experienced in risk assessment, risk modification, cancer surveillance, and patient education. These same skills are vital to a research team. There are ample opportunities for primary care researchers to network with investigators from a wide array of back-grounds to study the health needs of this population.

Family physicians are likely to see an increasing number of adult survivors of childhood cancer, a population with special health problems. It is essential that our discipline become better acquainted with these health needs and collaborate in studies to determine cost-effective methods for screening for late effects and second cancers.

Acknowledgments

Dr Oeffinger received partial support for this work through the American Academy of Family Physicians Foundation Advanced Research Training Grant and the Robert Wood Johnson Foundation Generalist Physician Faculty Scholars Program. He would like to thak Dr Shelley Roaten, Jr, and Dr George Buchanan for their critical review.