The articles in this supplement represent presentations and discussions at the “International Workshop on Adolescents and Young Adults with Cancer: Towards Better Outcomes in Canada” that was held in Toronto, Ontario, March 11-13, 2010.
Workshop on Adolescents and Young Adults with Cancer: Towards Better Outcomes in Canada, Supplement to Cancer.
The impact of the diagnosis and treatment of cancer on long-term health outcomes in children has received much attention over the past decade.1 Until recently, less attention was focused on the burden of cancer on adolescents and young adults, who face unique challenges from diagnosis through survivorship. In 2006, the US National Cancer Institute (NCI) published a report from the Adolescent and Young Adult Oncology Progress Review Group, which highlighted the finding that unlike younger or older cohorts, those diagnosed with cancer between ages 15 and 39 years have seen little to no improvement in cancer survival rates for decades.2 This new focus on the adolescent/young adult population was reflected in the Canadian Cancer Society's publication, Canadian Cancer Statistics 2009, which featured “Cancer in Adolescents and Young Adults (15-29 Years)” as a Special Topic.3 The vast majority of patients diagnosed with cancer as an adolescent/young adult will become long-term survivors. From 1997 to 2004, the 5-year overall survival for those diagnosed with cancer between ages 15 and 29 years in Canada was 83%.3
It is important to consider the definition of the adolescent/young adult group, as the incidence of cancer increases with age. In Canada, there is an average of 2075 new cancer cases per year diagnosed between ages 15 and 29 years, compared with only 836 cases between ages 0 and 14 years. An expansion of the definition of adolescent/young adult to those diagnosed between ages 15 and 39 years, which matches the definition of the NCI's Progress Review Group, would dramatically increase the number of adolescent/young adult survivors being considered, and therefore increase the required capacity of programs and services to attend to adolescent/young adult patients. Unique factors are associated with a cancer diagnosis in the adolescent/young adult age range. Whereas leukemia, lymphoma, and brain tumors are the most common pediatric cancer diagnoses, and malignancies of the prostate, breast, lung, and colorectum predominate among older adults, lymphomas are the most common diagnoses among patients aged 15 to 29 years, with thyroid cancer in females and testicular cancer in males occurring with similar frequency in each sex.4 Furthermore, increasing data show that diseases such as acute lymphoblastic leukemia, breast cancer, and colorectal cancer, when diagnosed among the adolescent/young adult population, are biologically distinct from those diseases in older or younger cohorts.5 In addition to biological differences, adolescents/young adults face a unique range of psychosocial issues that may affect their care and transition into survivorship. These include geographic mobility, concerns about fertility preservation, and isolation from their peers. A cancer diagnosis during the adolescent/young adult period often derails academic or vocational goals, and can impede the transition to independence that usually occurs during the young adult years. Because the vast majority of patients diagnosed with cancer as an adolescent/young adult will go on to be long-term survivors,3 it is important for adolescent/young adult cancer patients to have access to programs that address their unique health and psychosocial issues (Table 1) to optimize the quality of their survival.
Table 1. Transition Issues That Should Be Addressed With Adolescent/Young Adult Cancer Survivors
Physical health and function
Growth and development
Promotion of healthy lifestyle
Cancer-related chronic health problems
Sexually transmitted infections/diseases
Impact of cancer on fertility/reproductive outcomes
Psychological adjustment to cancer survivorship
Reintegration into normative social systems
Educational progress/need for resources
Illicit drug Use
Risky sexual behavior
Adherence to treatment
Adherence to health screening/surveillance
Diagnostic and treatment history
Cancer-related health risks
Self-management of medical issues
Impact of health behaviors on cancer-related risks
Health screening/surveillance recommendations
Navigation of the adult health care environment
Health care access
Both children and adolescents/young adults with cancer face multiple transitions during their cancer journey, from health to a diagnosis of a potentially life-threatening illness, from diagnosis to treatment, then from treatment to survivorship or palliation (Fig. 1). In children and adolescents treated in pediatric cancer centers, there is a further transition from pediatric to adult care, whereas those adolescents and young adults treated in an adult cancer center often transition from cancer center-based care to care coordinated by a primary care physician. This paper explores these transitions in children and adolescents/young adults with cancer, with a particular focus on the transition to survivorship care. For those fortunate patients who survive their cancer, the period of survivorship will comprise the longest portion of their cancer journey.
Phases in the Transition of Adolescents/Young Adults With Cancer
Much may be learned by examining the models used in the care and transition of other chronic childhood diseases (eg, cystic fibrosis, diabetes, congenital heart disease).6-10 However, adolescents/young adults with these chronic diseases usually require some form of therapy or have symptoms of their disease at the time of transition. In contrast, survivors of adolescent/young adult or childhood cancer are frequently asymptomatic and in good health but often have limited knowledge of their prior disease or their long-term risks resulting from their disease and treatment thereof.11, 12 Because many cancer treatment-related sequelae may not become clinically apparent until the survivor attains maturity or with the aging process, education of survivors and the health care providers supervising their care represents an important component of the transition process. Educational efforts should begin early in the continuum of cancer care and respond to the information needs of children and adolescents/young adults across the cancer survivorship spectrum from diagnosis to long-term follow-up.13 A systematic transition plan14 should consider key milestones experienced at cancer diagnosis, initiation of therapy, completion of therapy, entry into long-term follow-up care, transfer from pediatric to adult medical providers, and exit from oncology care providers to primary health care providers. Transitional services that should be available to children and adolescents/young adults throughout the survivorship spectrum ideally include health-related education, health surveillance and screening, management of cancer-related complications, and psychosocial support relevant to their developmental needs.14, 15 Survivors of childhood and adolescent/young adult cancer who attended the Workshop on AYA With Cancer: Towards Better Outcomes in Canada identified the process of transition (from acute care to survivor clinics, from acute/survivor care to a primary care practitioner, or from a pediatric to an adult cancer center) as inconsistent. Frequently, discussions around transition do not occur until the last visit to a specific clinic, allowing the survivor little time to prepare for the transition or to ensure that they have sufficient knowledge about their cancer history and long-term risks to assume responsibility for their own long-term care.
At cancer diagnosis, orientation to the medical system, the planned therapy, and its potential side effects are typically prioritized to enhance self-care measures and tolerance to treatment. Assessment of the impact of cancer on academic or occupational activities and the ability of the adolescent/young adult to live independently is important to facilitate adjustment during the acute phase of therapy and access to supportive psychosocial services. Before initiation of therapy, the informed consent process must address potential late treatment-related toxicity, measures that will be undertaken to prevent or remediate adverse effects, as well as pertinent health surveillance and health-promoting behaviors that can optimize outcomes. In particular, the consequences of cancer treatment on future reproductive health should be considered in all newly diagnosed adolescent/young adult cancer patients and followed by appropriate referrals for interventions to preserve fertility when possible.16 This topic is addressed in another article of this Supplement.
Completion of therapy represents another major milestone for child and adolescent/young adult cancer patients.17, 18 This is a critical time in the cancer survivorship continuum, characterized by a transition from a focus on cancer treatment and disease surveillance to a focus on wellness and health maintenance.13 Patients and families report considerable anxiety, fears, and vulnerability when treatment ends and they are no longer actively fighting cancer; frequently, parents request advice on how to continue to safeguard their child's health. This milestone represents an opportune time to review health risks associated with cancer treatment, lifestyle factors that influence these risks, and health surveillance pertinent to specific cancer treatment modalities that can facilitate timely diagnosis and remediation of complications. Assessment and support of psychosocial functioning should also continue to ensure access to mental health resources to promote resilience after the cancer experience and the achievement of social competence expected during young adulthood.19
These same interventions continue at transition into long-term follow-up (typically somewhere from 2 to 5 years after completion of therapy), at which time sustained cancer remission will eventually mandate the transition from oncology back to primary care. In addition, survivors of child or adolescent cancer must ultimately negotiate the transfer of care from a child-centered to an adult-focused health care provider and environment, where they are required to assume primary responsibility for their own health and follow-up care. Transition to survivor care or from a pediatric to an adult center often takes place at a critical time during survivors' development into independent young adults. Many survivors view themselves as completely healthy or invincible and do not want to remain in a system that brings back memories of their prior cancer, creates fear about future risks, or treats them as chronically ill.20, 21 Thus, programs for this population must ensure that these perceptions do not obstruct survivors' willingness to attain regular care.
Because many community providers lack familiarity with the health risks associated with childhood cancer, adolescent/young adult survivors must have sufficient health knowledge to advocate for risk-based cancer-related follow-up.22 Educating and engaging survivors in self-management is difficult, as evidenced by research indicating that many survivors do not recognize their risk of serious cancer-related health problems and do not adhere to recommended cancer-related follow-up care.23 Moreover, limitations in health insurance access and employment opportunities are commonly experienced by young adults, which may further complicate adolescent/young adult survivors' ability to obtain optimal follow-up care.24 Therefore, in addition to health education and psychosocial support, providing assistance in identifying and meeting financial challenges is an important component of transition services for adolescent/young adult survivors in this phase of the cancer survivorship spectrum.14 One example of such a program is Ontario's Successful Academic and Vocational Transition Initiative (www.pogo.ca/care/savti/). This resource, created in 2002 by the Pediatric Oncology Group of Ontario, provides educational and vocational counseling to teenage survivors, particularly those who may have developed learning difficulties as a result of their prior cancer or its therapy (eg, survivors of brain tumors or acute lymphoblastic leukemia treated with cranial radiation therapy). Although other resources are provided by cancer centers, regional cancer agencies, or peer or professional support groups, survivors are frequently unaware of these programs, limiting universal access.
Models of Adolescent/Young Adult Survivor Care
The model chosen for delivering risk-based adolescent/young adult survivor care in any particular jurisdiction needs to be flexible; be able to respond to patient preferences, the risk of recurrence, and late effects; and take into account local health system resources. Proposed models include cancer center follow-up (by the primary treatment team or in specialized long-term follow-up [LTFU] clinics), primary care follow-up by the patient's family physician, or most commonly, a combination of both.15, 25, 26 Risk stratification of patients based on existing and potential late effects may help determine how follow-up care is best organized.15, 25, 27 In all models, however, ongoing primary health care, health maintenance, and treatment of intercurrent illness are the responsibility of primary care providers. This may post a particular challenge for patients diagnosed with cancer in young adulthood, whose mobility and previous good health often mean they are less likely to have a connection with a regular primary care provider.
Survivors at the highest risk for adverse long-term outcomes, such as those treated for central nervous system tumors or with hematopoietic cell transplantation, may benefit from indefinite regular follow-up at a cancer center. Specialized and separate LTFU clinics are seen as having advantages over the integration of survivorship care into on-therapy clinics, where the preventative, rehabilitative, and health-oriented nature of well follow-up visits may not receive sufficient attention because of the urgency of caring for patients on active treatment.15, 26 These LTFU clinics feature a multidisciplinary core team of a physician, nurse or nurse practitioner, and social worker, with consultative services available as needed from other medical, rehabilitative, and behavioral consultants.15 It is important to ensure that a referral pathway is established to LTFU clinics for patients in late adolescence diagnosed and treated in the adult oncology system. However, the establishment of specialized LTFU clinics will not be feasible in all Canadian jurisdictions because of lack of institutional capacity, insufficient numbers of eligible survivors, or inadequate funding.
Patients at the lowest risk for late effects, such as those treated with surgery and chemotherapy alone, as for a Wilms tumor, may be transferred to a primary care physician for follow-up care after a short period of cancer center follow-up. Family physicians are willing to assume the follow-up care of their patients who are survivors of adult and pediatric cancers, but require follow-up care guidelines, a cancer treatment summary, and a clear contact person at the cancer center to answer questions and to expedite re-referral if there are concerns.28, 29 Survivorship care plans are an important method for addressing the challenge of the safe and effective transfer of care from cancer center to primary care.30 These documents should be created by the cancer team and shared with patients, families, and primary care providers at the end of treatment. They provide a summary of cancer treatment, risk-based follow-up recommendations, and health promotion and screening advice. Examples of these comprehensive summaries and recommendations are presently being tested in Canada in a randomized trial in adult breast cancer survivors discharged to primary care.
For the majority of adolescent/young adult cancer survivors at intermediate risk for late effects, such as those who have received anthracycline-based therapy or radiation therapy, a model that features risk-based follow-up care both in the cancer center and in a primary care setting can be considered. A Dutch pilot study has demonstrated the feasibility and acceptability to patients and family practitioners of alternating cancer center and primary care visits in a cohort of 121 adult survivors of childhood cancers. They are following risk-based care guidelines developed by a cancer center-based general practitioner in oncology.31 A case management model has also been proposed that would feature periodic contact by an LTFU clinic nurse with the patient and primary care provider to monitor adherence to recommended testing, collect information for outcomes research, and provide support and updated information about survivorship care.15 These different follow-up care models have not as yet been compared in rigorously designed studies.
The close collaboration between cancer specialists and primary care that is necessary for effective cancer survivorship care will require new links between these distinct care systems. The UPCON (Uniting Primary Care and Oncology) Network links the Manitoba provincial cancer agency with 34 primary care clinics. Each partner clinic has a lead family physician or nurse practitioner who have been willing to assume the follow-up care of cancer patients with no regular provider, including young adults.32 Because many survivors do not have a primary care provider, it is important for cancer centers to find a family physician or nurse practitioner willing to take on such patients, rather than having them rely on the use of walk-in clinics or emergency departments. UPCON Network clinics also have Internet-based secure access to the cancer system electronic medical record and to a program of ongoing continuing education about cancer care, thus building confidence in the quality of care provided. Cancer agencies need to identify primary care outreach leaders to work with local universities and medical associations to create educational sessions that equip primary care providers with the knowledge needed to provide cancer survivorship care, including the unique challenges faced by young adults as they cope with new challenges after cancer. Innovative Internet portals with information for health care professionals about survivorship issues (including follow-up guidelines, such as those published by the Children's Oncology Group33) and a centralized phone number to the treating cancer center, such as exists in Alberta and Manitoba, should also be considered.
Current Canadian System for Survivors of Childhood or Adolescent/Young Adult Cancer
The majority of Canadian children who develop cancer before age 15 years are treated in a pediatric cancer center, whereas about half of those aged 15 to 17 years at diagnosis are treated in such a facility.34 Twelve (71%) of Canada's 17 pediatric cancer centers have a formal survivor program or clinic dedicated to the care of survivors during their pediatric and adolescent years. The remaining 5 centers continue to provide follow-up to survivors in their acute care oncology clinics, but do not have a specialized LTFU program or clinic.35 Once survivors reach adulthood, only 6 of 17 centers have access to a formal program for adult survivors of childhood cancer, whereas the remaining centers discharge survivors to their primary care physician at some point after the completion of therapy. In contrast, there are no formal survivor programs for adolescents/young adults who receive their acute cancer care in an adult hospital. Thus, there are obvious deficiencies in the medical services currently available to survivors of childhood, adolescent, and young adult cancer that vary by region. This deficiency is most apparent among adolescents/young adults treated in adult cancer centers.
Opportunities for Research
In parallel to the development of programs for adolescent/young adult survivor care, there is an opportunity for research into barriers and facilitators of successful transition, models of transition and survivor care, and appraisal of the health services accessed by survivors. Investigators should create and evaluate methods to empower survivors to seek lifelong care (eg, assessment of the impact of survivor care plans or Web-based resources for survivors), and evaluation should include both the content and mode of delivery of these resources. More knowledge is needed about the cost-effectiveness of regular surveillance for late effects according to published guidelines, and the relative costs of providing such care in a cancer center or primary care setting. Research that takes advantage of the databases linked to Canada's public health care system is ongoing in British Columbia,36, 37 and survivor projects using Ontario's cancer registries are underway. There is also a need for better identification of subclinical changes in survivors' health that precede clinical manifestation of late effects of therapy. Detection of subclinical disease by imaging or biomarker measurement will allow for research into interventions designed to change the natural history of these diseases. Such interventions need not be restricted to pharmacological approaches, but might include strategies to promote a healthy lifestyle.
Excellent survival rates for children and adolescents/young adults with cancer have led to a growing population of cancer survivors with many potential years of life ahead of them. There is broad consensus that these survivors require lifelong care that focuses not only on the medical risks that arise from their cancer and its therapy, but also on the psychosocial, educational, and vocational implications of surviving cancer. The availability of resources for survivors varies by geography, health care system, and survivor characteristics. Survivors who receive their care outside of a pediatric cancer center have particularly limited access. Thus, there is a need for the implementation and assessment of adolescent/young adult survivor care strategies. The limitations in cancer center resources (along with survivor preference) strongly suggest that the long-term care of survivors will need to be shared between the cancer centers and primary care providers in survivors' communities.
Funding for the national task force on adolescents and young adults with cancer has been made possible by a financial contribution from Health Canada through the Canadian Partnership Against Cancer. Funding for the workshop was provided by C17; the Advisory Board of the Institute for Cancer Research at the Canadian Institutes for Health Research (CIHR); the Public Health Agency of Canada; the Ontario Institute for Cancer Research; the Meetings, Planning and Dissemination Grants program of the CIHR; the Terry Fox Research Institute; LIVESTRONG, formerly the Lance Armstrong Foundation; the Canadian Cancer Society Research Institute; Young Adult Cancer Canada; Hope and Cope; and the Comprehensive Cancer Centre at the Hospital for Sick Children, Toronto, in addition to the support provided by the Canadian Partnership Against Cancer to the Task Force on adolescents and young adults with cancer.