Karen E. Kinahan

Monitoring for Cardiovascular Disease in Survivors of Childhood Cancer: Report From the Cardiovascular Disease Task Force of the Children's Oncology Group

Curative therapy for childhood cancer has improved significantly in the last 2 decades such that, at present, ∼80% of all children with cancer are likely to survive ≥5 years after diagnosis. Prevention, early diagnosis, and treatment of long-term sequelae of therapy have become increasingly more significant as survival rates continue to improve. Cardiovascular disease is a well-recognized cause of increased late morbidity and mortality among survivors of childhood cancer. The Childrens Oncology Group Late Effects Committee and Nursing Discipline and Patient Advocacy Committee have recently developed guidelines for follow-up of long-term survivors of pediatric cancer. A multidisciplinary task force critically reviewed the existing literature to evaluate the evidence for the cardiovascular screening recommended by the Childrens Oncology Group guidelines. In this review we outline the clinical manifestations of late cardiovascular toxicities, suggest modalities and frequency of monitoring, and address some of the controversial and unresolved issues regarding cardiovascular disease in childhood cancer survivors.

Scarring, Disfigurement, and Quality of Life in Long-Term Survivors of Childhood Cancer: A Report From the Childhood Cancer Survivor Study

PURPOSE Childhood cancer survivors are at increased risk for adverse outcomes and chronic medical conditions. Treatment-related scarring, disfigurement, and persistent hair loss, in addition to their long-term impact on psychological distress or health-related quality of life (HRQOL), have received little attention. PATIENTS AND METHODS Self-reported scarring/disfigurement and persistent hair loss were examined in 14,358 survivors and 4,023 siblings from the Childhood Cancer Survivor Study. Multivariable models were used to examine associations with demographic and cancer treatment. The impact of disfigurement and hair loss on HRQOL (ie, Medical Outcomes Short Form-36) and emotional distress (ie, Brief Symptom Inventory-18) was examined. RESULTS Survivors reported a significantly higher rate of scarring/disfigurement compared with siblings for head/neck (25.1% v 8.4%), arms/legs (18.2% v 10.2%), and chest/abdomen (38.1% v 9.1%), as well as hair loss (14.0% v 6.3%). In age-, sex-, and race-adjusted models, cranial radiation exposure ≥ 36 Gy increased risk for head/neck disfigurement (relative risk [RR], 2.42; 95% CI, 2.22 to 2.65) and hair loss (RR, 4.24; 95% CI, 3.63 to 4.95). Adjusting for cranial radiation, age, sex, race, education, and marital status, survivor hair loss increased risk of anxiety (RR, 1.60; 95% CI, 1.23 to 2.07), whereas head/neck disfigurement increased risk of depression (RR, 1.19; 95% CI, 1.01 to 1.41). Limitations due to emotional symptoms were associated with head/neck disfigurement (RR, 1.24; 95% CI, 1.10 to 1.41), arm/leg disfigurement (RR, 1.19; 95% CI, 1.05 to 1.35), and hair loss (RR, 1.26; 95% CI, 1.09 to 1.47). CONCLUSION Survivors of childhood cancer are at increased risk for disfigurement and persistent hair loss, which is associated with future emotional distress and reduced quality of life. Future studies are needed to better identify and manage functional outcomes in these patients.

Fertility Preservation and Adolescent Cancer Patients: Lessons from Adult Survivors of Childhood Cancer and Their Parents

Building on 40 years of progress in cancer detection and treatment, survival rates for childhood cancers have risen from 20 % to almost 80 % [1,2]. Approximately 270,000 Americans are childhood cancer survivors and, by 2010, an estimated 1 in every 250 adults will be living with a history of childhood cancer [2,3]. The early and late effects of treatment are beginning to take on greater importance for survivors, their families and providers [4]. Increasing numbers of childhood cancer survivors are beginning to face a new challenge in returning to normalcy after cancer. Infertility is one of the most common chronic medical problems reported by childhood cancer survivors [5] and can be a primary concern particularly among female survivors [6]. Female infertility has biological and psychosocial implications that cannot be easily addressed given the ethical and legal questions surrounding fertility preservation [7–9]. Recent advances in fertility preservation may soon offer potential methods for females of all ages to protect their reproductive capacity from damaging radiation and/or chemotherapy [10]. Current literature focuses on the further development of preservation techniques and the numerous ethical and legal questions, but little knowledge is available on the attitudes and opinions of childhood cancer patients and their parents regarding fertility preservation. Progress is being made in understanding the fertility issues that women may face after treatment. Infertility as an isolated health problem can be emotionally devastating for a woman [11] and is often viewed as a loss of one’s sense of femininity [12]. The risk of infertility touches on the most intimate aspects of a woman’s life after cancer, particularly her relationships, future plans for a family, and concerns about pregnancy and birth [4,12–16]. For cancer survivors who may be dealing with additional physical and emotional concerns, infertility may add yet another concern to an already lengthy list of fears and worries [17]. Some survivors describe that the loss of fertility can be as painful as facing cancer itself [15,18]. The situation is further complicated by the fact that female cancer survivors, particularly pediatric cancer patients, lack clear-cut options to address their fertility that are available to their male counterparts. Advancements in semen cryopreservation and intracytoplasmic sperm injection (ICSI) have revolutionized the reproductive outlook of male patients who have reached puberty [19]. Recent advances in reproductive science are beginning to change what is possible for female survivors as well. Traditionally, few options existed for female cancer patients who may want to have their own biological children in the future. The only two established techniques women have for fertility preservation are protecting the ovaries from radiation and emergency in vitro fertilization (IVF) [20,21]. While protecting a patient’s ovaries has become common practice, emergency IVF cannot be offered to patients diagnosed with cancer before puberty because mature oocytes cannot be collected [22]. The promise for female patients with childhood cancer lies in the strides made toward ovarian transplantation and in vitro follicle maturation. Ovarian transplantation involves the removal and cryopreservation of ovarian tissue before treatment and the reintroduction of tissue after treatment, either orthotopically or heterotopically, such as in muscle or subcutaneously [23]. Researchers have demonstrated that transplantation of cryopreserved ovarian tissue has led to human embryonic development when accomplished heterotopically [24] and to a live birth after orthotopic transplantation [25]. Another promising method of fertility preservation is in vitro maturation of immature oocytes. Similar to ovarian transplantation, ovarian tissue is removed and cryopreserved before fertility-threatening treatment. Once a woman is prepared to have a child, follicles can be isolated from the thawed tissue, matured in vitro in a three-dimensional culture system, and the mature oocyte can be fertilized through IVF. Murine oocytes have been collected from in vitro grown follicles, matured, and fertilized in vitro, which has resulted in live births [26]. Human trials, where one ovary is laparoscopically removed before treatment, are being conducted on adult patients in order to begin the experimental process of perhaps one day delivering this option to female cancer patients. As research begins to enter the clinical arena, a large number of unanswered questions remain regarding the application of the procedures, the legal and ethical considerations involved, and the receptiveness of patients and their families to fertility preservation. Thus far, very few studies have considered the viewpoints of childhood cancer patients and their parents [27]. Since decisions regarding fertility preservation must be made before treatment begins, parents, physicians, and patients are required to make a complex decision in a short amount of time during an extremely stressful situation, similar to the anxiety involved in the informed consent process of clinical cancer research trials [28]. In order for fertility preservation to become a realistic and valued addition to the treatment of childhood cancer patients, a better understanding of the decision-making process that parents and their children go through at the time of diagnosis and their interest in fertility preservation is needed. Further, a more thorough exploration of the patients’ and parents’ thoughts regarding the child’s fertility at the time of diagnosis as well as later in the patient’s life will be valuable in the continued advancement and eventual application of fertility preservation.

Adult survivors of childhood cancer and their parents: Experiences with survivorship and long-term follow-up

To compare the perspectives of adult childhood cancer survivors and their parents in terms of: (1) parental involvement in the survivors healthcare, (2) thoughts and discussion about their own or their sons/daughters childhood cancer, (3) concern about the survivors current health status, and (4) perceived benefits of follow-up care. Forty-two adult survivors and their parents completed a semistructured audio-taped interview via the phone responding to a parallel set of questions. Thirty-eight percent of survivors reported that one of their parents attended the adult survivor clinic with them; in 41% of patient-parent dyads the parent expressed more concern than their child about the childs health status; 45% of the parents reported thinking about the cancer experience more often than their child. The results suggest that some parents continue to worry about their childs health status into adulthood, and in turn may choose to stay involved in their adult childs healthcare. Additional research is needed to understand the survivorship needs of the adult survivor and their family. Including parents in important healthcare decisions and discussions may be a consideration when caring for this unique population of patients.

Models of Cancer Survivorship Care for Adolescents and Young Adults

OBJECTIVES To review the literature on adolescent and young adult (AYA) oncology, discuss survivorship models of care, and focus on the unique needs of AYA patients with transition of care from treatment to survivorship. DATA SOURCES Peer-reviewed literature, workshop summaries, clinical practice guidelines. CONCLUSION Advancements have been made for AYAs with regard to identifying risk factors from cancer treatment and the need for ongoing follow-up care. Survivors face several unique care transitions. Several models of survivorship care are available for AYAs. IMPLICATIONS FOR NURSING PRACTICE The responsibilities of survivorship care for AYA patients fall on clinical providers, researchers, the government, advocacy groups as well as the survivors and families themselves. Nurses must remain cognizant and educated on AYA survivorship issues.

Childhood Cancer: Fertility and Psychosocial Implications

Having your child diagnosed with cancer is one of the most trying experiences a parent can endure. Greater than 15,000 children and adolescents under the age of 19 are diagnosed with childhood cancer each year in the United States [1]. Childhood cancer is comprised of a wide spectrum of malignancies, and outcomes are dependent upon histology type, disease origin and site, race, sex, and age at diagnosis [2]. Fortunately, advances in treatment and supportive care have led to a significant increase in survival rates for childhood cancer patients. Ries et al. reported that from 1950 to 1954 the 5-year survival rate was 20 % for children diagnosed with cancer between the ages 0–14 years [3]. Almost 50 years later, by 1995–2000, the 5-year survival rate rose to 80.1%, and in the past 25 years alone this rate increased by 20 % for children ages 0–19 years [4]. Approximately 270,000 Americans are childhood cancer survivors and, by 2010, an estimated 1 in every 250 adults will be living with a history of childhood cancer [5,6]. Recent studies demonstrate that while more childhood cancer patients are surviving, a high percentage of survivors are encountering serious “late effects” from their therapy. These late effects include, but are not limited to, cardiac, pulmonary and endocrine disorders, increased morbidity and mortality, as well as moderately to severely affected status in one or more of the primary domains of health (i.e., general health, mental health, functional status, limitations in activity, fear, or anxiety) [7,8]. This chapter will not go into detail on the myriad of late effects of childhood cancer treatment. Rather, we will focus on the fertility effects that adult survivors of childhood cancer may experience, including the emotional consequences of living with threatened or impaired fertility. Finally, some of the barriers to ongoing follow-up medical care will also be addressed.

Screening for late effects in brain tumor survivors.

Brain tumor survivors frequently receive therapy that places them at risk for long-term or late sequelae involving multiple organs. These late complications, arising as a result of neurosurgery, radiation, chemotherapy, and bonemarrow/ stem cell transplant, have been discussed in detail throughout this book. The focus of this chapter is to provide the clinician with guidance regarding how to methodically assess each survivor’s risk for late complications, and how to determine appropriate risk-based surveillance, in order to provide targeted, yet comprehensive, long-term follow-up care for brain tumor survivors. Two case studies of brain tumor survivors are included to illustrate the potential and actual late effects experienced by these survivors and the need for ongoing surveillance for late complications.

Promoting Health and Care Transitions in the Long-Term AYA Survivor

A large proportion of survivors of cancer diagnosed when they were AYAs experience some adverse effects on their health, some that do not become apparent for years or even decades after the exposure to the anticancer therapies. The developing and maturing organ systems of AYAs have different sensitivities to radiation therapy, chemotherapy, and surgery than do those of younger or older cancer patients. Virtually all organ systems can be affected, depending upon the therapeutic exposure, leading to a wide array of late effects, including second cancers, cardiovascular and pulmonary disease, cognitive dysfunction, and musculoskeletal problems. Some initially subclinical effects may exacerbate common diseases associated with aging, such as cardiovascular, skeletal, and endocrine disorders, and contribute to poor quality of life and premature death. Sociodemographic factors, details of treatment, and health behaviors also influence the magnitude of impairment in specific health status domains. Through risk-based care and education about the health risks conferred by the cancer experience, clinicians caring for long-term survivors play a critical role in the prevention, diagnosis, and rehabilitation of cancer-related complications and adjustment to chronic health conditions predisposed or exacerbated by cancer. Consequently, health professionals caring for AYA cancer survivors may influence their future health positively by correcting knowledge deficits, addressing factors that enhance an individual survivor’s vulnerability to health problems, and providing personalized health counseling that promotes the practice of health-promoting behaviors. This chapter describes the healthcare of survivors of cancer diagnosed during the AYA years, including risk-based screening and surveillance for late effects, transition of AYA healthcare, and models of AYA survivorship care. The promotion of healthy lifestyle habits is discussed, emphasizing the impact of such habits on the expression of late effects.

Future health of AYA survivors

Adolescent and young adult (AYA) oncology patients (ages 15–39) have been identified as a group with healthcare disparities including gaps and challenges in diagnosis, access to care, research, clinical trial participation, and cure rates. Like other patient groups with cancer or other chronic illnesses, disparities can lead to poor future health and outcomes, which is a well‐recognized concern within the AYA population. Cancer is the leading disease‐related cause of death in this age range. Numerous interested groups including the National Cancer Institute have met to address the research and cancer care needs of AYAs. This review highlights how these gaps in care during and after treatment can affect future health of this population of patients. Access to care, models of survivorship care, and lack of provider education are discussed. Survivorship care and use of guidelines, and promotion of psychosocial support and health behaviors during treatment and beyond are essential to optimizing future health of AYA patients.