Youth with congenital heart disease

Recent estimates of survival rates indicate that approximately 85% of infants born with CHD will survive into adulthood. The estimates of adults with CHD in the United States are difficult to calculate accurately because no national registry of adults with CHD exists, unlike other industrialized countries, such as England, with national health care registries, although these registries also have their limitations. Experts estimate that the number of adults with CHD currently vary up to 1.8 million, more than the number of children with CHD. These estimates represent a profound change in the profile of individuals living with CHD and the system of care needed to respond to this new requirement for services. Proportionally, there are three adults with CHD for every child with CHD. Among the adults with CHD, 80,000 are projected to have severe CHD. Now, with the extended survival of individuals with CHD, most deaths occur in adulthood rather than in childhood.

Adults with CHD require life-long surveillance. Long-term monitoring is needed to detect and prevent complications associated with arrhythmias, congestive heart failure, premature death, and bacterial endocarditis. Several follow-up studies have been conducted to investigate morbidity and mortality and expenditures for services. A study of Belgian adults with CHD found that 20% required hospitalizations for cardiac and noncardiac surgical and medical purposes and their care generated higher cost expenditures compared with the general population. A Canadian study showed that 47% of adults with complex CHD were successfully transferred to adult care. Canadian adults with complex CHD were found to have high rates of hospitalizations and emergency room visits after their transition to adult providers. Other follow-up studies conducted in the United States have reported similar findings.

These and other studies identified barriers to accessing care. The Canadian and United States systems showed a lack of training programs for physicians, service integration between pediatric and adult systems, and trained providers. In the Canadian system, placement on the waiting list also was found to result in service delay. In a study examining access to care, approximately 30% of individuals with moderate to complex heart problems reported lapses with their care. Lack of follow-up occurred for several reasons: (1) patients were told that follow-up was not important (33%), (2) no transfer to an adult provider occurred (23%), (3) patients believed follow-up was not important (19%), (4) lack of insurance (19%), and (5) patients were fearful of hearing bad news (7%). Those who experienced a lapse in care were more likely to require urgent care for their cardiac problem.

As these findings indicate, adults with CHD have significant ongoing needs for care that require constant surveillance and monitoring. Additionally, adults with CHD have higher rates of hospitalization and emergency room visits than the general population. As the results of the study by Yeung and colleagues showed, and anecdotal accounts of clinicians have corroborated, the health literacy of emerging adults is inadequate, resulting in lapses of care, untoward consequences of ignoring cardiac problems, and more serious cardiac problems requiring hospitalization or urgent care. Findings of studies and surveys of professional organizations conclude that access to competent adult health care providers knowledgeable about the long-term care needs of adults with CHD is an ongoing problem.

In 2001, the American College of Cardiology (ACC) hosted the 32nd Bethesda Conference, Care of the Adult with Congenital Heart Disease , to address the emerging and pressing needs of this new population with CHD to develop and implement a strategic plan for a continuous, ongoing, comprehensive, and specialized system of care for the future. To comprehensively address the range of issues encountered by adults with CHD, five task forces were commissioned to address a specific area of need with an action plan. Each of the five task forces was delegated the responsibility of generating a set of recommendations to address the following thematic areas included in the Bethesda report: (1) the clinical profile of adults with CHD and their unmet needs for services; (2) special health care needs of adults with CHD; (3) workforce educational requirements for the growing population of adults with CHD; (4) a system of care needed for adults with CHD, and (5) access to health care and adult services for education, employment, and community living.

The guidelines recommended the establishment of regional interdisciplinary centers for treating adults with CHD. Currently 55 centers exist in the United States and 15 in Canada. And estimated 20,000 of adults with CHD are provided services at these centers, which is significantly less than the approximately 1.8 million adults with CHD identified earlier. The significant disparity between the actual numbers of adults with CHD and those served demonstrate the challenges in establishing a system to provide services required by this growing population. Currently, no uniform standards of practice have been implemented for treating adults with CHD, resulting in differing services available at each of the centers.

The goal of the recommendations generated by each of the task forces was to develop a long-term strategic plan that would provide the foundation for a system of care to competently address the unique health care needs of adults with CHD. Although the Bethesda recommendations were directed to improve health outcomes of adults with CHD, the intended audience for application was pediatric and adult cardiologists. A legitimate system of care to address the unmet needs for services encountered by adults with CHD is not possible without sufficient numbers of adult cardiologists with specialty training in managing this patient population.

Given the rising numbers of adults living with CHD, the American College of Cardiology Foundation (ACCF) and American Heart Association (AHA) developed guidelines for care of adults with CHD. The most recent version presents the evidence-based medical guidelines. The emphasis of these guidelines is on the medical aspects of care.

No formalized program or system of care for transitioning exists in the United States or in Brittan. The initiation of care transfer is not predicated on chronologic age, although experts suggest it should occur somewhere between 12 and 16 years of age; however, a combination of factors, such as emotional maturity, gender, disease status, and family functioning, affect the transfer initiation. Although the ACCF/AHA provided guidelines for implementing adult programs, the focus is on promoting medical services. This document also specified service recommendations, including age of transfer, medical summary and transfer of records, and the providers’ medical qualifications for care.

An obvious service gap is the lack of a best practice template for health care transition planning. A comprehensive health care transition planning approach incorporates the best practices advocated by the American Academy of Pediatrics (AAP) consensus statement. Best practices include support for a comprehensive, interdisciplinary service approach. An integrated and comprehensive model of care includes not only the needs related to survivorship but an examination of health-related needs pertaining to future employment, psychosocial concerns, exercise, high-risk behaviors, pregnancy and contraception, preventive approaches to minimizing the possibilities of complications such as infectious endocarditis, and need for noncardiac surgeries.

Adult survivors of childhood cancer

Each year approximately 12,400 children and youth are diagnosed with some type of cancer. The advances in treatment of pediatric cancers have improved the survivorship of children diagnosed with cancer; each year, the survival rate for adolescents increases approximately 0.9%. Currently, 80% of children diagnosed with cancer survive into adulthood, and there are an estimated 300,000 adult survivors of childhood cancer in United States.

The life expectancy for cancer survivors is on average approximately 10 years less than for the general population, although it varies according to the type of cancer. For example, the life expectancy is reduced by 4 years for individuals diagnosed with kidney tumors and by 17 years for those with Ewing’s sarcoma and brain tumors. The survivorship data do not reflect current treatment advances because they are based on patients treated from 20 to 40 years ago.

Major survivorship issues exist pertaining to the occurrence of treatment-related late effects, secondary to the primary cancer diagnosis and the psychosocial ramifications associated with the lived experience of the cancer diagnosis and the late effects. Given these circumstances, experts urge the implementation of a health care transition plan that includes the adoption of a long-term survivorship plan for life-long surveillance. Understanding of the range of potential biopsychosocial late effects and secondary conditions a child or adolescent with cancer may experience will provide a context for developing guidelines of care to address these concerns. As this discussion shows, the guidelines of care reflect the medical best practices recommended by the clinical community. Additional empiric testing is needed to show the effectiveness of care recommended.

The late effects from treatment-related complications associated with radiation, chemotherapy, hematopoietic stem cell transplantation, and surgery have been described as changes in physical appearance (ie, surgical scars, amputations), orthopedic problems (ie, short stature), endocrine dysfunctions (ie, hypo/hyperthyroidism, growth hormone deficiency), neurocognitive deficits (ie, attention deficit–hyperactivity disorder, learning disabilities), pulmonary dysfunction (ie, pulmonary fibrosis, obstructive lung disease), cardiac problems (cardiomyopathy, valvular disease), reproductive dysfunction (ie, premature menopause, ovarian failure, sterility), and second malignant tumors (ie, thyroid, breast cancer). Childhood cancer survivors are six times more likely to develop a second cancer.

These late-effects are not uncommon, occurring in two of every three survivors. Studies have concluded that cancer survivors originally diagnosed with brain tumors have more serious late effects.

Approximately 75% of cancer survivors will develop a chronic condition by 40 years of age. Nearly 50% are expected to exhibit declines in functional abilities and ability to be physically active. Many of these problems are treatable, such as those associated with endocrine and cardiovascular late effects. The key to treatment is early detection of problems by the survivor’s adult primary and specialty care physicians, which are discussed in greater detail later.

Much of the evidence on childhood cancer survivorship has been reported from analyses of Childhood Cancer Survivor Study (CCSS) data. The CCSS is a retrospective ascertained cohort of 20,346 childhood cancer survivors diagnosed before age 21 between January 1, 1970 and December 31, 1986, and 4000 siblings who served as the control group, from 26 participating clinical research centers in the United States and Canada. The data were compiled from survivors of the following cancer groups: leukemia, central nervous system cancers, Hodgkin lymphoma, Wilms’ tumor, neuroblastoma, soft tissue sarcoma, and bone tumors. More recently the cohort was expanded to include 5-year survivors diagnosed from January 1, 1987 to December 31, 1999.

An overview of the findings of studies conducted with childhood cancer survivors show the range of late effects. Follow-up studies of cancer survivors that involved measurement of behavioral and psychosocial constructs found that they scored lowered than comparison groups of siblings or matched typical peers. CCSS survivors (mean age, 14.8 years; range 12–17 years), in contrast to the comparison group of siblings (mean age, 14.9 years; same range), were more likely to have higher levels of behavioral and social problems in the domains of depression/anxiety symptoms (1.5 times higher) and attention deficits and antisocial behaviors (1.7 times) compared with siblings. Survivors diagnosed with leukemia and central nervous system tumors had more difficulties. Another study reported that one of five survivors had posttraumatic stress disorder, as did parents and siblings. Findings from a study of 103 Greek cancer survivors (mean age, 19.8 years) and their matched peer controls showed that survivors scored lower than the control subjects on measures of social functioning and self-esteem.

Several studies have examined quality of life among childhood cancer survivors. Findings showed that survivor scores were similar to comparison groups in most domains, except physical well-being for which the scores were lower. Lower quality-of-life scores were associated with smaller subgroups of survivors who had the following characteristics: older at diagnosis, longer time since diagnosis, female sex, specific diagnoses (ie, bone and central nervous system tumors), selected personal attributes (ie, low self-esteem), physical symptoms (fatigue), and demographics (ie, socioeconomic status, Hispanic ethnicity).

Studies of the academic performance of childhood cancer survivors show that they are at higher risk for academic problems and adverse outcomes. Academic problems include higher rates of special education enrollment, high school dropouts, and learning disabilities. The adverse outcomes associated with academic performance are attributable to learning difficulties that result from treatments effects, which cause declines in executive functioning, memory capacity, processing speed, and visual perceptual skills.

Successful transition to adulthood may be described by the achievement of developmental milestones. These developmental milestones include completion of postsecondary education or training programs for occupational or career purposes, employment, and development of significant relationships (ie, romantic relationships, marriage, long-term partnerships) and starting a family.

Studies investigating employment status of survivors found that rates of full-time employment were 10% to 15% lower than for the typical population. Unemployment rates were considerably higher (20%–50% ) for those who underwent radiation treatment, were diagnosed with a brain tumor, were diagnosed at a younger age, and had a chronic medical condition. Unemployment rates were higher for female survivors.

Outcomes associated with romantic relationships and marriage indicate that survivors experience more challenges compared with the general population. In a study of 60 emerging adult cancer survivors, subjects reported fewer relationships and were more upset when the relationship terminated compared with the control group. Those at higher risk for relationship difficulties were survivors who were older when diagnosed, were exposed to a more rigorous treatment regimen, and reported higher levels of anxiety. Rigorous treatment sequelae can cause physical and cognitive limitations that adversely affect social relationships.

Findings pertaining to marital status have been inconsistent regarding rates of marriage. One study suggested that rates of marriage for survivors were comparable to national averages. Other findings show that rates of marriage were lower; one study reported nearly two times lower rates for survivors when compared with their siblings. Lower marriage rates were attributed to factors such as the treatment-related effects of short stature, impaired physical functioning, decreased executive function, and memory capacity.

Investigations conducted to examine health care use by survivors show the significant gaps in adult care. In one study, 90% of survivors reported having a primary care provider, and approximately 50% had a complete physical examination in the past year. In another study, clinic nonattendance was associated with survivors who were older, of lower socioeconomic status, noninsured, off treatment for a longer period, and nonwhite. Researchers suggest that low socioeconomic status is a proxy for a greater burden of stress, a high level of competing family demands, and fewer resources available (ie, transportation and child care), which adversely affect the long-term management of survivors.

A systematic review of studies found that survivors had lower rates of smoking, alcohol use, and unprotected sex than the survivor population when compared with siblings and controls. Survivors of higher socioeconomic status were engaged in healthier behaviors. Substance abuse was associated with anxiety about cancer.

Studies have shown health literacy of long-term cancer survivors to be problematic. Findings from one study found that 70% of 121 cancer survivors did not remember receiving information from health care providers about the late effects of treatment. An investigation of Internet use among cancer survivors and their parents found that 90% accessed the Internet; approximately 30% used it to locate answers to unanswered questions after their health care visit (which were unasked for a variety of reasons, such as forgot or uncomfortable asking the question), 50% to obtain health information, and 10% to seek information about late effects.

As the CCSS findings and those from other investigations show, cancer survivors have a range of biopsychosocial needs and problems that require ongoing support and services. Their unmet needs result in unfavorable outcomes indicating problems with successful adaptation to adulthood, including lower rates of employment, academic challenges, and difficulties with social and romantic relationships, and the range of late effects described earlier.

Study findings reveal the lack of transition programs available to support survivors with the transfer to adult health care providers who are knowledgeable about late effects and health risks, and to coordinate this transfer and refer survivors to other adult social, educational, employment programs. This service gap is widespread, even in countries with universal access to health care. System barriers, such as lack of subspecialty providers, lack of knowledge by primary providers, and lack of communication between pediatric and adult providers regarding care, have been cited as contributing to the inconsistencies in follow-up. Preventive strategies must be developed to facilitate early detection and ongoing screening.

In an effort to provide clinical guidelines for health care professionals who provide long-term management for survivors of childhood cancer, the Children’s Oncology Group (COG), a national network of more than 200 institutions involved in clinical trials, developed the Long-term Follow-up Guidelines for Survivors of Childhood, Adolescent, and Young Adult Cancers (COG LTFU guidelines). The purpose of this resource is to improve the care provided to asymptomatic cancer survivors for ongoing long-term surveillance, early detection, and prevention. The COG LTFU guidelines are intended to be used by practitioners in the development of a survivorship care plan that contains individualized long-term treatment recommendations by pediatric primary care and adult providers after patients are transferred to the adult health care system. The AAP recommendations for long-term management of pediatric cancer survivors based on the COG LTFU guidelines also include health promotion guidelines and prevention recommendations to avoid high-risk behaviors pertaining to tobacco and alcohol use and engage in healthy behaviors. Together these guidelines focus on the medical and health needs of childhood cancer survivors. Guidelines addressing the range of comprehensive needs according to best practice models have not been developed, including those related to self-management.