These findings demonstrate that the use of relatively brief, intensive chemotherapy regimens including marrow-ablative chemotherapy with AuHCR results in fewer endocrine sequelae than treatment schemes utilizing CNS irradiation.
Although traditional recommendations for mononephric childhood cancer survivors are to avoid contact sports in order to protect the remaining kidney, review of available evidence suggests that the majority of renal loss is caused by accidents not involving sports. An interdisciplinary team performed a review of the English literature published from 1999 to 2012 within the PubMed, Cochrane, Google Scholar, and National Guidelines Clearinghouse databases. The level of evidence and proposed recommendations were graded according to an established rubric and GRADE criteria. Our review found that kidney loss is most commonly caused by nonsports activities such as motor vehicle accidents and falls, implying that restrictions on sports-related activity in mononephric pediatric survivors are not well supported. This favors encouraging ordinary sports and related activities without restriction in mononephric childhood cancer survivors because the known benefits of exercise outweigh the exceedingly low risk of renal loss. Accordingly, activity recommendations for mononephric patients have been revised in the most current version of the Children’s Oncology Group Long-term Follow-Up Guidelines for Survivors of Childhood, Adolescent and Young Adult Cancers. This has important implications for this and similar populations who may now undertake individual and organized sports without undue regard for their mononephric status.
Molecular targeted therapies have become a fundamental treatment regimen within pediatric oncology practice, yet these medications are not without side effects, which often require interruption, dose reduction, or early discontinuation of treatment. Existing chemotherapy education curriculums focus on the side effect profile of traditional chemotherapy regimens and do not include standardized instruction on toxicities related to targeted therapies, resulting in a knowledge gap for caregivers and the clinical team. The purpose of this project was to development and implement a standardized teaching curriculum to educate patients, caregivers and clinical staff on common toxicities related to MEK inhibitors including anticipated side effects, symptom management, and toxicity surveillance. The education was presented to patients and families by a neuro-oncology nurse or nurse practitioner at the initiation of MEK inhibitor therapy. The cohort consisted of 11 patients over a 12-month period with a diagnosis of plexiform neurofibromas and neurofibromatosis type 1. Additionally, the education curriculum was presented to clinical staff during an in-service and included a pre-post intervention survey to assess effectiveness of the education curriculum. The results demonstrated that increasing patient and family knowledge at initiation of MEK inhibitor therapy likely leads to fewer dose reductions, drug interruptions, and early therapy discontinuations. Greater than 90% of the patient cohort were able to complete the treatment regimen without early termination due to toxicity, with only 18% requiring dose reduction and 9% requiring interruption of therapy. Surveys evaluating the effectiveness of the staff educational curriculum demonstrated an increased knowledge in the identification and treatment of MEK inhibitor side effects with a 26% improvement in survey results post-intervention. This project is a feasible educational approach for preventing and treating toxicities associated with MEK inhibitors and can be initiated at pediatric oncology practices to standardize MEK inhibitor education to clinicians, patients, and caregivers.
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