Purpose
Young adult survivors (YAS) of childhood cancer require annual adult-focused, cancer-related follow-up given their risk for late effects of treatment. This study describes perception of and ...engagement with adult-focused, cancer-related follow-up care and general health care among YAS formally transferred to adult care from pediatric survivorship care.
Methods
YAS transferred from pediatric survivorship care in the prior 1–5 years completed measures indicating engagement with cancer-related follow-up care, other health care utilization, content of communication by providers, quality of cancer-related care, and satisfaction with health care in the prior year.
Results
Eighty YAS (
M
age = 27.7 years,
M
time since diagnosis = 10.4 years) participated. Just over half of YAS surveyed (
n
= 44, 55 %) endorsed continuing cancer-related follow-up care since transfer. Those with cancer-related follow-up endorsed seeing subspecialty survivorship providers (
n
= 16, 44 %) and primary care providers (
n
= 22, 50 %) or utilizing a shared care model (
n
= 6, 14 %). About a third of YAS endorsed seeing subspecialists (
n
= 29, 36 %) or using other support services (
n
= 22, 27 %). YAS-perceived content of communication varied significantly depending on care model with less cancer-related content being discussed by primary care providers, though perceived quality of cancer-related care and satisfaction with health care was generally favorable.
Conclusions
YAS report less than optimal engagement in cancer-related follow-up care and communication in their health care encounters.
Implications for Cancer Survivors
Young adult survivors should receive anticipatory guidance about expectations for delivery and content of adult-focused cancer-related follow-up care.
Purpose
Survivors of pediatric brain tumors often have neurodevelopmental late effects, such as inattention. Symptoms may mirror those of attention-deficit/hyperactivity disorder (ADHD), which ...affects ~ 5–8% of the general population. This retrospective study of survivors followed at a large tertiary care center examined the prevalence of a clinical diagnosis of ADHD, and risk factors associated with ADHD diagnosis and ADHD-related medication use.
Methods
A retrospective chart review of brain tumor survivors (n = 528), diagnosed between 2000 and 2015, who were at least 6 years old and 2 years from the end of tumor-directed therapy or from diagnosis, if no interventions were received. Clinical and demographic data were extracted from the medical record.
Results
Survivors were 55.7% male with mean age 8.15 ± 4.4 (0.0–16.0) years at brain tumor diagnosis. The most common diagnoses were low-grade glioma, medulloblastoma, and craniopharyngioma, with 52.5% of tumors supratentorial. Of the survivors, 81.3% received surgery, 40.0% radiation therapy, and 36.6% chemotherapy. Sixty-nine survivors (13.1%) had ADHD diagnoses, 105 (19.9%) had symptoms of ADHD without diagnoses, and 64 (12.1%) had ADHD medication use. ADHD diagnosis was associated with younger age at tumor diagnosis (p = 0.05) and supratentorial tumor location (p = 0.001). ADHD diagnosis was not associated with gender, tumor type, or treatment type.
Conclusions
Survivors of brain tumors are at increased risk of ADHD and related symptoms. The greatest increase in risk occurs for survivors with diagnoses at younger ages and supratentorial tumors. Additional research is warranted, as select survivors may benefit from behavioral or pharmacologic ADHD treatments to optimize functioning.
Objective: Caregivers of adolescents and young adults (AYA) with complex medical conditions, including brain tumor survivors, have protracted and often complex roles, yet a gap exists in ...understanding their perceived competence. The aim of this study is to test a hypothesized model based on the theoretical and empirical literature: better caregiver health, better survivor health, and better family functioning contribute directly to fewer caregiving demands, which in turn contribute to greater caregiver competence. Method: Telephone interviews using structured self-report questionnaires were conducted in this cross-sectional study with a sample of 186 caregivers (mothers) of childhood brain tumor survivors aged 14-40 years old who live with at least one parent. Structural equation modeling (SEM) was used to test the hypothesized model. Results: The final SEM model suggests that survivor health and family functioning directly predict caregiver competence. Caregiver health indirectly predicts caregiver competence through caregiver demands and then family functioning. Family income directly predicts family functioning. The model showed adequate fit (CFI = 0.905, TFI = 0.880, and RMSEA = 0.081). Overall, the model accounted for 45% of variance in caregiver competence. Conclusions: For this sample of caregivers of AYA with medically complex conditions, family functioning and the health of survivors are both important to how they evaluate their skills as caregivers. The results of this study underscore the crucial role of care models that focus on optimizing the health of the survivor, caregiver, and family, along with supporting a family centered approach to their care.
Background
Infertility can be the result of some common cancer treatments and can significantly impact quality of life. Semen cryopreservation allows for fertility preservation. We analyzed the semen ...parameters of specimens collected from pubertal males from the Children's Hospital of Philadelphia (CHOP) in order to expand current knowledge on the quality of these specimens and inform a standard clinical practice.
Procedure
Males who were at least Tanner stage III and newly diagnosed with cancer at CHOP were approached regarding sperm banking. The success and quality of the samples collected were analyzed and compared in relation to prior treatment, age, and diagnosis.
Results
From 399 patients approached for semen collection, 339 (85%) attempted to bank sperm, of which 265 (78%) were successful and 60 (15%) refused to participate. Therapy prior to sperm banking significantly impacted a successful collection (P < 0.01). Only 16.9% of the untreated patients were azoospermic, whereas 84.0% of the treated subjects were azoospermic. Older patients were less likely to be azoospermic and have a greater quality collection when compared with younger patients (P < 0.01). However, 65% of our youngest patients still were able to cryopreserve semen. There was no difference in azoospermia across diagnostic groups (P = 0.35), though there were differences in quality of semen parameters across diagnoses.
Conclusion
Our data support that sperm banking pubertal males prior to the initiation of therapy is feasible. While there were differences in quality of semen parameters across age and diagnostic groups, most males, regardless of age or diagnosis, had adequate specimens for cryopreservation.
Background
Self‐management interventions for adolescent and young adult (AYA) survivors of childhood cancer are needed. The present study reports on the acceptability and feasibility of delivering ...survivorship care plans (SCPs) and an accompanying app to AYA.
Procedure
AYA (n = 224) ages 15‐29 who completed treatment for cancer were randomized and received a digital SCP only or an SCP plus a mobile app intended to enhance self‐management. For 16 weeks, the app delivered one to two daily messages complementing information in their SCP and tailored based on age, treatment, and health goal. Data are presented on feasibility, self‐reported acceptability (including satisfaction and perceived benefits) and its relationship to app engagement (for those in app group), and feedback from qualitative interviews conducted with 10 AYA.
Results
The SCP and app proved feasible as evidenced by high recruitment and retention, access to technology, time analysis, moderate app engagement, and minimal technical issues. However, 12% reported never reading the SCP and 8% never used the app. The app and SCP were acceptable to AYA, and SCP acceptability ratings did not differ between groups. For those with the app, acceptability was positively related to message engagement. AYA recommended enhanced individualization and design features of the SCP and app.
Conclusions
Results support the use of tailored SCPs and mobile health interventions for most AYA, as well as the need for further refinement and research. Delivery of SCPs and digital interventions are acceptable and feasible to AYA survivors, and may help promote health‐related knowledge and survivorship self‐management.
Introduction
Survivors of childhood cancers undergo routine pulmonary function testing as they are at an increased lifetime risk for significant lung disease. However, this population also ...demonstrates growth abnormalities that could influence the interpretation of these tests, as reference equations are based on standing height. We aim to determine the impact of the relative thoracic growth deficiency in childhood cancer survivors on the interpretation of pulmonary function testing.
Methods
Standing height and upper segment length (USL) in childhood cancer survivors undergoing pulmonary function testing at a single academic center were compared to age‐matched historical standards. Additionally, pulmonary function tests were compared to reference values generated from standing height and doubled USL.
Results
Data were obtained from 107 cancer survivors. While the subjects demonstrated an overall 6.8% lower standing height vs historical standards, they also demonstrated relative thoracic growth abnormality with a further 9.9% decrement in the ratio USL to standing height. The use of doubled upper segment length as a surrogate measure for standing height in pulmonary function reference equations decreased the number of patients with restrictive lung disease as indicated by spirometry.
Conclusions
Childhood cancer survivors have disproportionately worse thoracic growth deficiency vs appendicular growth deficiency. As a result, their USL is disproportionately short for their standing height, which is most commonly used in pulmonary function testing reference equations. This leads to an increased likelihood in these patients meeting pulmonary function test criteria for restrictive lung disease.
Purpose
The purpose of this study was to explore the decision-making influences, perceived level of control over decision-making, and mood states of parents and patients who were offered OTC prior to ...gonadotoxic therapy.
Methods
Parents and patients, at least 12 years old, who required gonadotoxic therapy and were offered OTC prior to therapy, were asked to complete questionnaires. Two validated instruments were also used: the Decision-Making Control Instrument (DMCI) and the Profile of Mood States (POMS). The factors that influenced decision-making were compared using Student’s
t
test, and the scores of DMCI and POMS were compared using the Mann-Whitney test.
Results
Thirty-six parents and 16 patients who elected ovarian tissue cryopreservation (OTC) completed questionnaires. Five parents who declined OTC also completed questionnaires. Accepters thought OTC was a good idea and that, in the future, science would enable cryopreserved ovarian tissue to be used to restore fertility (100% parents, 93.8% patients). Among accepters, the desire for genetically related children and prevention of the stress of infertility drove parents’ and patients’ decisions (90.9 and 100%, respectively). The desire to prevent the stress of infertility was important to parents, but patients were less likely to report that a desire to prevent the stress of infertility factored into their decision-making (66.7 vs. 50.0%;
p
< 0.001). All respondents felt in control of their decision and displayed low levels of mood disturbance.
Conclusions
Though the decision to undergo experimental OTC is difficult and often urgent, this study suggests that families feel in control of their decision-making and report little emotional disturbance.
Cystic kidney disease includes a wide range of hereditary, developmental, and acquired conditions of the kidneys. Some of the inherited causes of cystic kidney disease include autosomal dominant ...polycystic kidney diseases (caused by mutations in PKD1 or PKD2 ), autosomal recessive polycystic kidney disease, tuberous sclerosis complex, von Hippel-Lindau disease, oral-facial-digital syndrome type I, and Hadju-Cheney syndrome. Acquired cystic kidney disease has been reported in patients receiving long-term hemodialysis or peritoneal dialysis and in children after liver transplantation. Acute kidney injury can occur in patients with neuroblastoma, usually as a result of thrombotic microangiopathy associated with bone marrow transplantation. End-stage renal disease is described in long-term survivors. However, in this case report, we provide what is to our knowledge the first description of multiple kidney cysts in long-term survivors of stage IV neuroblastoma. None of the 7 patients we describe with neuroblastoma and multiple kidney cysts had a family history of autosomal dominant polycystic kidney disease. Also, all lacked stigmata of tuberous sclerosis complex, von Hippel-Lindau disease, or Hadju-Cheney syndrome. Two patients progressed to end-stage renal disease; in addition, one of them developed an oncocytoid renal cell carcinoma.
Background: Pallister-Killian syndrome (PKS) is typically recognized by its features that include developmental delay, seizures, sparse temporal hair, and facial dysmorphisms. PKS is most frequently ...caused by mosaic supernumerary isochromosome 12p. Case Presentation: Here, we report a patient with PKS who was subsequently diagnosed with Burkitt lymphoma. Following the successful treatment of lymphoma, this patient demonstrated very mild intellectual disability despite the diagnosis of PKS, which is usually associated with severe developmental delay. Discussion: This is the first reported patient with PKS and a hematologic malignancy. Although there is no significant reported association of tetrasomy 12p with cancer, the co-occurrence of two rare findings in this patient suggests a potential relationship. The localization of AICDA, a gene for which overexpression has been implicated in promoting t(8;14) noted in our patient’s lymphoma, raises a potential mechanism of pathogenesis. In addition, this case indicates that children with PKS can demonstrate near-normal cognitive development.
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