Current pediatric cancer survivorship practices: a report from the Children's Oncology Group.

PURPOSE: The purpose of this study is to describe current survivor services provided by COG institutions. METHODS: A 190-question online survey was distributed to 209 COG member institutions over a 5-month period in 2017. Descriptive statistics were used to describe survivor services and explore their changes between 2007 and 2017. RESULTS: Representatives from 153 (73%) institutions completed the survey. Of these, 96% of institutions reported that they provide pediatric cancer survivor care either in a specialized late effects program (75%) or a regular pediatric oncology clinic (24%). However, only 29.8% of institutions reported that > 75% of eligible patients were seen in a survivorship clinic. The most prevalent reported barriers to survivor care were lack of dedicated time (58%) and lack of funding for program development (41%). In 2017, 88% of institutions provided a treatment summary compared to 31% in 2007. CONCLUSION: The majority of COG institutions have dedicated care for pediatric and young adult survivors of childhood cancer; however, at most institutions, < 75% of eligible patients access this care. Research into more efficient technology strategies is needed to ensure all survivors the opportunity to receive appropriate follow-up care. IMPLICATIONS FOR CANCER SURVIVORS: This survey provides a snapshot of the status of late effects services within COG institutions and provides information on residual gaps in services. Next steps should focus on the importance of attendance in a survivorship clinic on the physical health and psychosocial outcomes in cancer survivors.

Genomewide Approach Validates Thiopurine Methyltransferase Activity Is a Monogenic Pharmacogenomic Trait.

We performed a genomewide association study (GWAS) of primary erythrocyte thiopurine S-methyltransferase (TPMT) activity in children with leukemia (n = 1,026). Adjusting for age and ancestry, TPMT was the only gene that reached genomewide significance (top hit rs1142345 or 719A>G; P = 8.6 × 10-61 ). Additional genetic variants (in addition to the three single-nucleotide polymorphisms [SNPs], rs1800462, rs1800460, and rs1142345, defining TPMT clinical genotype) did not significantly improve classification accuracy for TPMT phenotype. Clinical mercaptopurine tolerability in 839 patients was related to TPMT clinical genotype (P = 2.4 × 10-11 ). Using 177 lymphoblastoid cell lines (LCLs), there were 251 SNPs ranked higher than the top TPMT SNP (rs1142345; P = 6.8 × 10-5 ), revealing a limitation of LCLs for pharmacogenomic discovery. In a GWAS, TPMT activity in patients behaves as a monogenic trait, further bolstering the utility of TPMT genetic testing in the clinic.

Childhood acute lymphoblastic leukemia: current perspectives.

PURPOSE/OBJECTIVES: To provide an overview of childhood acute lymphoblastic leukemia (ALL), including epidemiology, clinical presentation, diagnostic classification, prognostic factors, current treatment, long-term sequelae, and nursing management. DATA SOURCES: Journal articles, books, and clinical experience. DATA SYNTHESIS: Childhood ALL is a heterogeneous disorder, and current treatment is tailored to risk factors (e.g., initial white blood count, cytogenetic properties of the leukemic blasts). Risk-directed therapy ensures that children with a higher risk of relapse receive more intensive treatment, whereas those with lower risk disease receive less toxic therapy with decreased potential for treatment-related morbidity. Quality of life in long-term survivors is a significant issue. Late sequelae of treatment can include neurocognitive difficulties, endocrine dysfunction, secondary malignancies, and cardiomyopathy. CONCLUSIONS: With risk-directed therapy, cure rates for childhood ALL continue to improve. At least 80% of children diagnosed with ALL today are expected to survive their disease. IMPLICATIONS FOR NURSING PRACTICE: Nurses caring for children with ALL can have a significant impact on the children's overall health, from diagnosis through long-term follow-up. Nursing interventions encompass the domains of physical and psychosocial care, as well as patient and family education. Assisting the child and family to maintain normalcy in the face of chronic illness, as well as fostering the family's hope for the future and their belief in the child's potential for survival, are key nursing strategies that promote the child's growth, development, and psychological health.

Hearing loss related to ototoxicity in children with cancer.

Children undergoing treatment for cancer often receive agents that put them at risk for ototoxicity. Platinum-based chemotherapy, aminoglycoside antibiotics, loop diuretics, and radiotherapy are all capable of inducing inner ear damage, which may result in significant sensorineural hearing loss. Frequent audiological monitoring is necessary for the early detection of changes in hearing thresholds. Age-appropriate modification of audiological testing is essential to obtain accurate results and provide maximum comfort for pediatric patients. When hearing loss is identified promptly, consideration may be given to treatment modification and/or early intervention with hearing aids and other assistive devices. The consequences of hearing loss differ depending on the developmental stage of the child at the time that hearing loss occurs. Language acquisition may be affected in very young children, whereas educational and psychosocial concerns are paramount for the older child. The pediatric oncology nurse is instrumental in assisting the child and family who are coping with hearing loss related to cancer treatment.