Long-term sequelae in survivors of childhood leukemia with Down syndrome: A childhood cancer survivor study report.

BACKGROUND: Children with Down syndrome (DS) are at increased risk of developing acute leukemia and are more prone to acute toxicities. We studied the incidence and severity of chronic health conditions among survivors of childhood leukemia with DS compared with those without DS. METHODS: Chronic health conditions reported by questionnaire were compared between 154 pediatric leukemia survivors with DS and 581 without DS, matched by leukemia, age at diagnosis, race/ethnicity, sex, radiation location and chemotherapy exposure using Cox models to estimate hazard ratios (HRs) and 95% confidence intervals (CIs). Subjects were selected from 7139 5-year survivors of leukemia in the Childhood Cancer Survivor Study. RESULTS: Risk of at least 1 late onset chronic health condition (grade 1-5) was similar in the DS population compared with the non-DS group (HR, 1.1; 95% CI, 0.7-1.5). Serious chronic health conditions (grade 3-5) were more common in DS survivors (HR, 1.7; 95% CI, 1.1-2.6), as were ≥ 3 chronic health conditions (grades 1-5) (HR, 1.7; 95% CI, 1.2-2.4). The 25-year cumulative incidence of any condition (grades 1-5) was 83% for DS survivors and 69% for non-DS survivors. CONCLUSION: Leukemia survivors with DS have therapy-related chronic health conditions comparable to those of similarly treated survivors without DS, with a few notable exceptions: 1) an increased risk of cataracts, hearing loss, and thyroid dysfunction compared with survivors without DS (though these are known risks in the DS population), 2) decreased risk of second cancers, and 3) increased risk of severe or multiple conditions. Practitioners should be aware of these risks during and after therapy. Cancer 2018;124:617-25. © 2017 American Cancer Society.

Palliative treatment: redefining interventions to treat suffering near the end of life.

The treatment of suffering is now recognized as an essential component of medical care. By referring to this treatment as "palliative care," however, we diminish its importance. In this essay, we propose a shift in our conception of the treatment of suffering and advocate for the use of a new term: "palliative treatment." We argue that the word "treatment" is more appropriate than "care" because the interventions we employ are evidence-based, goal-directed, and effective in diminishing suffering. We articulate that this alteration in terminology is necessary because it facilitates patient and physician understanding of the importance and efficacy of palliative treatment interventions. We enumerate four distinct forms of suffering that each requires treatment: physical, psychological, existential, and spiritual. Finally, we argue that in rare cases when aggressive palliative treatment has been unsuccessful in diminishing suffering to a degree that is bearable, allowing death to occur unimpeded may be viewed as therapeutic because in death the patient's suffering is finally ended.

Optimal care for the child with cancer: A summary statement from the SIOP Working Committee on Psychosocial Issues in Pediatric Oncology.

Since its foundation in 1991, the SIOP Working Committee on Psychosocial Issues in Paediatric Oncology1 has developed and published 12 sets of Guidelines for health-care professionals treating children with cancer and their families. Those elements considered essential in the process of cure and care of children with cancer are summarized in this document as a formal statement, developed at the 2007 SIOP annual meeting in Mumbai. Elaboration of the concepts with detailed strategies for practice can be found in the referenced guidelines [1-12] and in a companion publication [13]. This article is a summary of what practitioners considered critical elements in the optimal care of the child with cancer, with the goal of stimulating a broader application of these elements throughout the SIOP membership.

Conducting a qualitative culture study of pediatric palliative care.

While conducting a grounded theory study of Chinese American and Mexican American families' experiences in pediatric palliative care, we encountered a number of unanticipated challenges regarding project development, Institutional Review Boards, recruitment, data collection, and data analysis. In this article, we describe our experiences, strategies, and insights for the benefit of other researchers and clinicians in the field.

Surviving childhood cancer: the impact on life.

With modern therapies, most children diagnosed with cancer are expected to reach adulthood. Therefore, there are large and ever-increasing numbers of children and young adults in our population who are survivors of childhood cancer. Many of the therapies responsible for improved cancer survival rates can also damage normal cells and tissues. As more children survive cancer, the physical and emotional costs of enduring cancer therapy become increasingly important. Although most childhood cancer survivors are now expected to survive, they remain at risk for relapse, second malignant neoplasms, organ dysfunction, and a negative psychologic impact. Individual risk is quite variable and is dependent on multiple factors including the type and site of cancer, the therapy utilized, and the individual's constitution. The risks are likely to change as we learn more about the specific long-term effects of cancer therapy, develop more refined and targeted therapies, and develop and apply more effective preventative strategies or therapeutic interventions. Guidelines for long-term follow-up have been established and are available to help facilitate appropriate monitoring of and care for potential late effects.

Survival after recurrence of osteosarcoma: a 20-year experience at a single institution.

BACKGROUND: Approximately one-third of patients with osteosarcoma who have a complete response to their initial treatment can be expected to relapse. It is important to define what host, tumor, or treatment characteristics determine outcome after relapse. We present findings in 59 patients treated at our institution from 1974 to 1996 who have relapsed one or more times after their initial response. METHODS: Host and tumor characteristics at diagnosis and relapse, therapeutic interventions and survival outcomes were determined from examination of medical records and a follow-up questionnaire. RESULTS: Of the 59 patients, 37 initially presented with localized disease of the extremity, 11 with localized non-extremity disease, and 11 with metastatic disease. This report focuses on those with localized disease of the extremity. For these patients, median time from original diagnosis to first recurrence was 14 months. Median survival after first recurrence was 31 months. The median post initial relapse survival was the same for patients whose first relapse occurred before or after 14 months from original diagnosis. Seventeen of 29 patients with systemic metastasis at first recurrence had complete removal of their disease and had a median post-op survival of 2.5 years, while the remaining 12 patients with no surgery, had a median survival of 2 years. Of the 37 patients who presented with primary disease only in the extremities and relapsed: 31 died (2 more than 6 years from first recurrence) and 6 are alive from 6 to 24 years from first recurrence (5 without disease and 1 with disease). Three of the five disease-free survivors had three or more relapses. CONCLUSION: With a long follow-up time, we found 15% of patients with relapsed osteosarcoma who originally presented with localized disease in the extremity are alive with no evidence of disease at 10 years from first recurrence (Kaplan-Meier estimate). Even patients with multiple relapses may have long-term disease-free survival after salvage therapy. Chemotherapy and time to first recurrence were unrelated to survival after relapse in this study. Complete surgical removal of metastatic disease may be important for long-term survival.

Health insurance coverage in survivors of childhood cancer: the Childhood Cancer Survivor Study.

PURPOSE: To examine the prevalence and predictors of health insurance coverage and the difficulties obtaining coverage in a large cohort of childhood cancer survivors. PATIENTS AND METHODS: This study included 12,358 5-year survivors of childhood cancer and 3,553 sibling controls participating in the Childhood Cancer Survivor Study. Data were collected by surveys distributed in 1994 (baseline) and 2000 (follow-up). RESULTS: At baseline, 83.9% of adult survivors, compared with 88.3% of siblings, had health insurance coverage (P < .01); 6 years later, small but significant survivor-sibling differences remained (88% v 91%; P < .01). Twenty-nine percent of survivors reported having had difficulties obtaining coverage, compared with only 3% of siblings (P < .01). In multivariate analysis of survivors 18 years of age or older, factors associated with being uninsured included younger age at diagnosis (diagnosis age of 0 to 4 years; odds ratio [OR] = 1.7; 95% CI, 1.3 to 2.2), male sex (OR = 1.3; 95% CI, 1.2 to 1.5), age at baseline survey (age 22 to 24 years; OR = 1.6; 95% CI, 1.2 to 2.1), lower level of attained education (less than high school, OR = 2.6, 95% CI, 2.1 to 3.3; high school graduate, OR = 2.1, 95% CI, 1.8 to 2.5), income less than 20,000 dollars (OR = 5.6, 95% CI, 4.5 to 7.1), marital status (widowed/divorced/separated; OR = 1.3; 95% CI, 1.1 to 1.6), smoking status (current smoker, OR = 2.0, 95% CI, 1.7 to 2.3; former smoker, OR = 1.4, 95% CI, 1.2 to 1.8), and treatment that included cranial radiation (OR = 1.3, 95% CI, 1.0 to 1.6). CONCLUSION: Compared with siblings, adult survivors of childhood cancer had significantly lower rates of health insurance coverage and more difficulties obtaining coverage. Since lack of coverage likely has serious health and financial implications for this at-risk population, any disparity in availability and quality of coverage is of great concern.

Minority adult survivors of childhood cancer: a comparison of long-term outcomes, health care utilization, and health-related behaviors from the childhood cancer survivor study.

PURPOSE: To determine the influence of race/ethnicity on outcomes in the Childhood Cancer Survivor Study (CCSS). PATIENTS AND METHODS: Of CCSS adult survivors in the United States, 443 (4.9%) were black, 503 (5.6%) were Hispanic and 7,821 (86.6%) were white. Mean age at interview, 26.9 years (range, 18 to 48 years); mean follow-up, 17.2 years (range, 8.7 to 28.4 years). Late mortality, second malignancy (SMN) rates, health care utilization, and health status and behaviors were assessed for blacks and Hispanics and compared with white survivors. RESULTS: Late mortality rate (6.5%) and 15-year cumulative incidence of SMN (3.5%) were similar across racial/ethnic groups. Minority survivors were more likely to have lower socioeconomic status (SES); final models were adjusted for income, education, and health insurance. Although overall health status was similar, black survivors were less likely to report adverse mental health (females: odds ratio [OR], 0.6; 95% CI, 0.4 to 0.9; males: OR, 0.5; 95% CI, 0.3 to 0.8). Differences in health care utilization and behaviors noted: Hispanic survivors were more likely to report a cancer center visit (females: OR, 1.5; 95% CI, 1.1 to 2.0; males: OR, 1.7; 95% CI, 1.2 to 2.3); black females were more likely (OR, 1.6; 95% CI, 1.1 to 2.4), and Hispanic females less likely to have a recent Pap smear (OR, 0.7; 95% CI, 0.5 to 1.0); black and Hispanic survivors were less likely to report smoking; black survivors were less likely to report problem drinking. CONCLUSION: Adjusted for SES, adverse outcomes in CCSS were not associated with minority status. Importantly, black survivors reported less risky behaviors and better preventive practices. Hispanic survivors had equitable access to cancer related care.

Differences in pediatric oncologists' estimates of curability and treatment recommendations for patients with advanced cancer.

BACKGROUND: When goals of therapy for children with advanced cancer are called into question, physician recommendations regarding treatment goals have been shown to be important for families. However, there has been no demonstration of the degree of variation between pediatric oncologists' recommendations in such situations. PROCEDURE: We provided 48 pediatric oncologists with two identical case histories and identical prognostic data from the literature. Individual interviews were then performed to assess variation in (1) recommended treatment goal, (2) perceived chances for cure, and (3) degree to which further curative intervention would be considered desirable for each patient. RESULTS: There was a large variability in each of the areas examined. For both patients, there was wide divergence (2:1 and 2:3) in whether to recommend cure as the goal of treatment. There were also differences in physician estimates for likelihood of cure for each patient. Finally, even among those with identical estimates for likelihood of cure, there were differences in the treatment goals physicians would recommend and how strongly they would counsel for them. CONCLUSIONS: This study demonstrates that even with identical clinical data and prognostic evidence from the literature, pediatric oncologists vary widely in their recommendations regarding goals of treatment for children with advanced cancer.

Visual attention in long-term survivors of leukemia receiving cranial radiation therapy.

The effect of cranial radiation therapy (CRT) on visual attention was examined in long-term survivors of childhood acute lymphoblastic leukemia (ALL) compared to peers with no history of ALL (n = 24) using a cued orienting task and a global-local task. ALL participants treated with CRT (n = 13) demonstrated an increased cost in response time with invalid spatial orienting cues and inefficient shifts of attention across hierarchical levels. ALL participants treated only with chemotherapy (n = 8) showed performance similar to the non-ALL comparison group. Participants with exposure to CRT early in life appeared to largely account for the attention deficits, and showed particular difficulties with shifting attention from the local level of stimuli to the global level. The data are consistent with prior reports emphasizing attention deficits following CRT, and suggest that attention shifting may be particularly affected by CRT early in life.

Education, employment, insurance, and marital status among 694 survivors of pediatric lower extremity bone tumors: a report from the childhood cancer survivor study.

BACKGROUND: With increasing numbers of childhood cancer survivors, direct sequelae of cancer therapy and psychosocial outcomes are becoming more important. The authors described psychosocial outcomes (education, employment, health insurance, and marriage) for survivors of pediatric lower extremity bone tumors. METHODS: The long-term follow-up study of the Childhood Cancer Survivor Study is a multiinstitutional cohort study comprising 14,054 individuals who have survived for 5 or more years after treatment for cancer diagnosed during childhood or adolescence. Baseline demographic and medical information were obtained. Six hundred ninety-four survivors had osteosarcoma or Ewing sarcoma of the lower extremity or pelvis and were classified by amputation status and by age at diagnosis. The median age at diagnosis was 14 years old with a median of 16 years of follow up since diagnosis. Demographic characteristics were used to analyze the rates of psychosocial outcomes. RESULTS: Amputation status and age at diagnosis did not significantly influence any of the measured psychosocial outcomes. Education was a significant positive predictor of employment, having health insurance, and being currently in their first marriage. Male gender predicted ever being employed and female gender predicted having health insurance and marriage. When compared with siblings, amputees had significant deficits in education, employment, and health insurance. CONCLUSIONS: Overall, no differences between amputees and nonamputees were found. However, gender and education play a prominent role. When compared with siblings, amputees in this cohort may benefit from additional supports.

A child and parent illustrating non-compliance with treatment: understanding non-compliance with treatment.

BACKGROUND: In children and adolescents non-compliance with treatment is a major concern. To shed light on its mechanisms, we present a family demonstrating non-compliance. PROCEDURE: B, a 10 year-old boy, treated for medulloblastoma, refused high-dose chemotherapy and Autologous Bone Marrow Transplantation. Six psychotherapeutic interviews with B, his parents and staff resolved the causative issues. RESULTS: B's behavior expressed his parents' ambivalence, despite their informed consent. Their reasons were conscious and unconscious, rational and irrational, linked to both the past and present experiences. The mother was convinced that he would die. The discussions helped both parents to assume their parental role, B perceived their ability to truly accept the treatment and support him. CONCLUSIONS: Non-compliance should not be viewed as a child's or parents' intrapsychic problem, but as the result of multi-determined interaction between the child, the parents, the staff interacting with present and past events. When the child's death is a possibility, the parents' ambivalence confuses their understanding and acception of the therapy. The development of confidence between the staff, the child and parents and clear agreement about the goals of therapy is necessary to avoid the occurrence of and escalation of non-compliance, which raises difficult clinical, legal and ethical questions.