A multisite evaluation of summer camps for children with cancer and their siblings.

Summer camps for pediatric cancer patients and their families are ubiquitous. However, there is relatively little research, particularly studies including more than one camp, documenting outcomes associated with children's participation in summer camp. The current cross-sectional study used a standardized measure to examine the role of demographic, illness, and camp factors in predicting children's oncology camp-related outcomes. In total, 2,114 children at 19 camps participated. Campers were asked to complete the pediatric camp outcome measure, which assesses camp-specific self-esteem, emotional, physical, and social functioning. Campers reported high levels of emotional, physical, social, and self-esteem functioning. There were differences in functioning based on demographic and illness characteristics, including gender, whether campers/siblings were on or off active cancer treatment, age, and number of prior years attending camp. Results indicated that summer camps can be beneficial for pediatric oncology patients and their siblings, regardless of demographic factors (e.g., gender, treatment status) and camp factors (e.g., whether camp sessions included patients only, siblings only, or both). Future work could advance the oncology summer camp literature by examining other outcomes linked to summer camp attendance, using longitudinal designs, and including comparison groups.

Camper learning and friendship at pediatric oncology camps in North America.

Children with cancer and their families often attend specialized camps (therapeutic recreation) through their cancer treatment journey, yet little is known about the effects of these camps. A qualitative cohort study was used to assess learning and friendship development by campers attending one of four pediatric oncology summer camps during 2010 in North America. Standardized perceived change questionnaires developed by the American Camp Association were administered following camp attendance. Five-hundred and eighteen campers were enrolled: 120 (age 6-9 years) and 398 (age 10 and older). The largest positive response from the younger campers was observed for the question, "At camp did you learn to look forward to trying new activities?" For the older campers' survey, the items "Becoming better at enjoying being with my friends," "Becoming better at helping my friends have a good time when they are with me," and "Becoming better at getting to know more things about my friends" were perceived to increase the most for the majority of campers compared to other questions. Items for which older campers most often perceived little change were "Becoming better at choosing people who would be good friends to be with" and "Becoming better at understanding friends' emotions." Camp helps children learn new activities as well as enjoy good times with friends. Dealing with one's own mistakes and understanding others' emotions are areas for improvement. Ultimately it is hoped that these skills gained at camp will help build coping and resiliency for children/siblings affected by pediatric cancers.

Safety of hematopoietic stem cell transplantation in children less than three years of age.

Allogeneic hematopoietic stem cell transplantation (HSCT) is a standard treatment for a variety of hematologic conditions. However, very young children may experience different complications of HSCT compared to older patients. The authors retrospectively analyzed the results of 51 transplants performed on children less than 3 years of age between June 1987 and October 2005. Donors were matched-related (n = 21), partially mismatched related (n = 3), and unrelated (n = 27). The majority of patients had one or more grade III organ toxicities, but all nonrelapse deaths were attributable to infection. Perineal dermatitis was found in a large number (73%) of recipients of cyclophosphamide-based conditioning regimens. The 1-year transplant-related mortality (TRM) was 14%, but significantly declined in the more modern period. Grades II-IV acute graft-versus-host-disease (GvHD) was seen in 22% of patients, while chronic extensive GvHD developed in only 7% of patients. Relapse was seen in 40% of transplants performed for a malignant condition, most commonly in those patients not in remission at time of HSCT. The 5-year event-free survival (EFS) and overall survival (OS) were 53 and 64%, respectively. Recipients of fractionated total body irradiation (fTBI) were more likely to have at least one long-term sequelae than patients who received chemotherapy-based regimens (p = .014). These data demonstrate that HSCT can be performed safely in very young children, especially as supportive-care techniques improve. Cyclophosphamide-related perineal dermatitis is a unique complication in very young children. Finally, the incidence of acute and chronic GvHD in this population is low.

High-dose therapy and autologous hematopoietic stem-cell transplantation for recurrent or refractory pediatric Hodgkin's disease: results and prognostic indices.

PURPOSE: To evaluate the outcome of pediatric patients with refractory or relapsed Hodgkin's disease (HD) who undergo high-dose therapy and autologous hematopoietic stem-cell transplantation (AHSCT). PATIENTS AND METHODS: From 1989 to 2001, 41 pediatric patients with relapsed or primary refractory HD underwent high-dose therapy followed by AHSCT according to one of four autologous transplantation protocols at Stanford University Medical Center (Stanford, CA). Pretreatment factors were analyzed by univariate and multivariate analysis for prognostic significance for 5-year overall survival (OS), event-free survival (EFS), and progression-free survival (PFS). RESULTS: At a median follow-up of 4.2 years (range, 0.7 to 11.9 years), the 5-year OS, EFS, and PFS rates were 68%, 53%, and 63%, respectively. Multivariate analysis determined the following three factors to be significant predictors of poor OS and EFS: extranodal disease at first relapse, presence of mediastinal mass at time of AHSCT, and primary induction failure. Two of these factors also predicted for poor PFS (extranodal disease at time of first relapse and presence of mediastinal mass at time of transplantation). CONCLUSION: More than half of children with relapsed or refractory HD can be successfully treated with the combination of high-dose therapy and AHSCT, confirming the efficacy of this approach. Further investigation is now required to determine the optimal timing of AHSCT, as well as to develop alternative regimens for those patients with factors prognostic for poor outcome after AHSCT.

Allogeneic hematopoietic cell transplantation for patients with high-risk acute lymphoblastic leukemia in first or second complete remission using fractionated total-body irradiation and high-dose etoposide: a 15-year experience.

OBJECTIVE: The rationale for this retrospective study was to identify the long-term overall and event-free survival, relapse, and treatment-related mortality rates of high-risk pediatric and adult first (CR1) and second remission (CR2) patients with acute lymphoblastic leukemia (ALL) who were treated with a single preparatory regimen consisting of fractionated total-body irradiation (FTBI) and high-dose etoposide (VP-16) prior to allogeneic hematopoietic cell transplantation. PATIENTS AND METHODS: Over a 15-year period at Stanford University Medical Center, 85 consecutive high-risk pediatric (up to age 17 years; n=41) and adult (age 18-55 years; n=44); patients with leukemia (ALL) in CR1 (n=55) and CR2 (n=30) received HLA-matched sibling allogeneic bone marrow or peripheral blood progenitor grafts after being treated with FTBI (1320 cGy) and high-dose VP-16 (60 mg/kg) as their preparatory regimen. The majority of patients transplanted in CR1 (n=45) had high-risk features, including age above 30 years, white blood cell count at presentation exceeding 25000/microL, extramedullary disease, need for more than 4 weeks of induction chemotherapy to achieve CR, or high-risk chromosomal translocations. Most patients transplanted in CR1 were adults (n=39), whereas patients in CR2 were primarily children or adolescents (n=25). RESULTS: The 10-year Kaplan-Meier estimates of relapse were significantly (p=0.05) lower in CR1 patients (15%+/-10%) than in CR2 patients (33%+/-20%). Relapse was the most common cause of treatment failure in patients transplanted in CR2. There was a significantly (p=0.05) higher rate of chronic graft-vs-host disease in CR1 (32%+/-14%) compared with CR2 (9%+/-11%) patients; however, overall survival for patients transplanted in CR1 (66%+/-14%) was comparable (p=0.67) to that of patients transplanted in CR2 (62%+/-19%). Event-free survival rates also were similar (p=0.53) between CR1 (64%+/-14%) and CR2 (61%+/-18%) patients. Treatment-related mortality rates were equivalent (p=0.51) between CR1 and CR2, as well as between Philadelphia chromosome (Ph) positive (Ph(+))and Ph(-) (p=0.23) ALL patients. CONCLUSION: Overall, FTBI/VP-16 is a highly effective preparatory regimen that provides durable remissions for patients receiving allogeneic hematopoietic cell transplantation for high-risk ALL in CR1 or CR2.

Genetic polymorphisms in CYP3A5, CYP3A4 and NQO1 in children who developed therapy-related myeloid malignancies.

Therapy-related acute myeloid leukemia and myelodysplastic syndrome (t-ML) are serious complications that affect some patients after acute lymphoblastic leukemia (ALL) treatment. Genetic polymorphisms in the promoter of CYP3A4 (CYP3A4*1B) and in NAD(P)H:quinone oxidoreductase (NQO1609C-->T substitution) have been associated with the risk of t-ML. A polymorphism in CYP3A5 (CYP3A5*3) affects CYP3A activity and the wild-type allele (CYP3A5*1) is in partial linkage with the CYP3A4*1B allele. We compared the genotype frequencies for the CYP3A5*3, the CYP3A4*1B and the NQO1609C-->T substitution in 224 children with ALL who did not develop t-ML (controls) and in 53 children with ALL who did develop the complication. The allele frequencies differed significantly among whites, blacks and Hispanics (P < 0.001 for CYP3A5*3, P < 0.001 for CYP3A4*1B and P = 0.004 for NQO1609), thus we performed the comparisons between ALL controls and t-ML patients after accounting for race. We found no differences in the CYP3A4*1B allele distribution between ALL controls and t-ML patients in whites (P = 0.339, 6.6% vs. 9.8%), blacks (P = 0.498, 93.8% vs. 87.5%) or Hispanics (P = 0.523, 39.1% vs. 25.0%). The frequencies for the NQO1609C-->T allele did not differ between control and t-ML groups in whites (P = 0.191, 35.0% vs. 44.9%), blacks (P = 0.664, 37.5% vs. 37.5%) or Hispanics (P = 0.447, 65.2% vs. 50.0%). We found no differences between the control and t-ML group in the incidence of homozygous CYP3A5*3 genotypes: 82.0% vs. 85.4% in whites (P = 0.403), 6.5% vs. 12.5% in blacks (P = 0.508), and 69.6% vs. 75.0% in Hispanics (P= 0.663). Our data do not support an association between common CYP3A4, NQO1 or CYP3A5 polymorphisms and the risk of t-ML in children treated for ALL.

Projective drawings as measures of psychosocial functioning in siblings of pediatric cancer patients from the Camp Okizu study.

This research was conducted at a summer camp for siblings of children with cancer. Participants included 77 siblings (ages 6-17 years) and their parents. Before attending camp, 18 of the siblings had experienced the death of their brother or sister with cancer. Projective measures were administered before attending camp and 3 months after camp. These included the Human Figure Drawing (HFD) and the Kinetic Family Drawing-Revised (KFD-R). Siblings were administered both the HFD and KFD-R; parents were given the KFD-R. On the HFD, siblings' emotional distress scores decreased significantly pre- to postcamp. On the KFD-R, nonbereaved siblings and parents showed significant improvement in family environment scores. Bereaved siblings and parents also showed improvement (although nonsignificant). These results support Camp Okizu's effectiveness in increasing siblings' emotional well-being yet underscore the need to implement interventions to address family communication for both bereaved and nonbereaved families.

Antimetabolite-based therapy in childhood T-cell acute lymphoblastic leukemia: a report of POG study 9296.

PURPOSE: A previous Pediatric Oncology Group (POG) study showed high incidence of secondary acute myelogenous leukemia (AML) in children treated for T-cell acute lymphoblastic leukemia (T-ALL) or higher-stage lymphoblastic lymphoma. To prevent secondary neoplasms, induce prolonged asparagine depletion, and maintain high event-free survival (EFS) in children with newly diagnosed T-ALL or higher-stage non-Hodgkins lymphoma (NHL), we designed this pilot study to determine feasibility and safety of substituting methotrexate/mercaptopurine for teniposide/cytarabine and PEG-asparaginase for native asparaginase. PATIENTS AND METHODS: Forty-five patients were entered, 29 with T-ALL and 16 with higher-stage NHL. Forty-two of 45 patients achieved complete remission (CR), and 27 completed the therapy in continuous CR. Treatment consisted of 4-week induction then 6 weeks consolidation and ten 9-week maintenance cycles. Therapy primarily comprised antimetabolites, anthracyclines, alkylating agents, and asparaginase. Expected chemotherapy duration was 100 weeks. RESULTS: Forty-two of 45 patients achieved CR, and 27 completed therapy. The most common toxicities were Grade 3 or 4 myelosuppression after cyclophosphamide/cytarabine and allergic reactions to asparaginase. Two died of sepsis early in maintenance. Five-year EFS was 68.5% (SE 9.1%) for T-ALL and 81.3% (SE 9.8%) for NHL. Five-year EFS was 73.1% (SE 6.8%) for the entire cohort. No patients treated entirely on this study developed secondary neoplasms. One patient taken off study for asparaginase toxicity was treated with multiagent therapy that contained teniposide, and died from secondary myelodysplasia (sMDS)/AML. CONCLUSION: Substituting methotrexate/mercaptopurine for teniposide/cytarabine and PEG-asparaginase for native asparaginase in a dose-intensive regimen was feasible in children and young adults with newly diagnosed T-ALL or higher-stage NHL. EFS was not compromised and secondary neoplasms were decreased.

Siblings of pediatric cancer patients: the quantitative and qualitative nature of quality of life.

This study used both quantitative and qualitative methodologies to assess the pediatric health-related quality of life (HRQOL) in siblings (n = 77) of cancer patients attending summer camp. On quantitative measures (Pediatric Quality of Life Inventory (PedsQL) parent and child versions), siblings reported statistically significant improvements in HRQOL from pre-to post camp. The parent sample, as a whole, did not report a statistically significant improvement in the siblings' HRQOL; however, statistically significant improvements were found when the analysis controlled for the responses of bereaved parents. On the qualitative measures (Sibling Qualitative Interview and Camp Okizu Satisfaction Surveys), both children and parents described the positive impact of camp. Using grounded theory, we identified the major themes and found that the positive emotional and social experiences captured by the quotes were paralleled in the quantitative findings of improved HRQOL in psychosocial domains on the PedsQL. These findings suggest the beneficial effects of camp as a psychological intervention and illustrate the value of integrating quantitative and qualitative methodological approaches in research.