Robin Ryan

Understanding the Functional Late Effects and Informational Needs of Adult Survivors of Childhood Cancer

PURPOSE/OBJECTIVES To report functional (physical and cognitive) late effects, experiences, and information needs of adult survivors of childhood cancer. DESIGN Descriptive, mixed methods survey. SETTING Two pediatric oncology programs in the Midwest. SAMPLE Convenience sample of 272 young adult survivors. METHODS Voluntary survey completion by young adult survivors regarding late effects, experiences, and educational needs to develop appropriate comprehensive care programs for care provision before, during, and after transition to adult care. Survey domains were identified from existing survivorship literature and focused on all aspects of survivorship; however, this article focuses on results specific to the functional domain. MAIN RESEARCH VARIABLES Functional late effects, experiences, information needs, age, gender, and treatment intensity of young adult survivors of childhood cancer. FINDINGS Response rate was 48%. Functional late effects, perceptions, and information needs all correlated with intensity of treatment (those survivors most heavily treated experienced the most symptoms). Survivors wanted more information about late effects and how to deal with them. Women wanted more information about fertility-related topics, and participants who received more intense treatment generally wanted more information. Brain tumor survivors perceived greater cognitive difficulties, cognitive late effects, fatigue, and financial difficulties. CONCLUSIONS Survivors experience myriad physical late effects and require ongoing access to information as needs change over time. IMPLICATIONS FOR NURSING Identifying new and innovative ways to reach survivors and better meet needs is important for care, research, and program development. KNOWLEDGE TRANSLATION The findings of the research underscore the importance of continuous learning opportunities for adult survivors of childhood cancer. The findings also highlight the need for healthcare teams to better understand the current and long-term needs of this population. In addition to traditional communication approaches, technologies such as social media and telemedicine can provide innovative ways to deliver patient-centered care.

Randomized trial of hydroxychloroquine for newly diagnosed chronic graft-versus-host disease in children: a Children's Oncology Group study.

The Childrens Oncology Group conducted a multicenter Phase III trial for chronic graft-versus-host disease (cGVHD). The double-blind, placebo-controlled, randomized study evaluated hydroxychloroquine added to standard therapy for children with newly diagnosed cGVHD. The study also used a novel grading and response scoring system and evaluated clinical laboratory correlates of cGVHD. The primary endpoint was complete response (CR) after 9 months of therapy. Fifty-four patients (27 on each arm) were enrolled before closure because of slow accrual. The CR rate was 28% in the hydroxychloroquine arm versus 33% in the placebo arm (odds ratio [OR] = 0.77, 95% confidence interval [CI]: 0.20-2.93, P = .75) for 42 evaluable patients. For 41 patients with severity assessment at enrollment, 20 (49%) were severe and 18 (44%) moderate according to the National Institutes of Health Consensus Conference global scoring system. The CR rate was 15% for severe cGVHD and 44% for moderate cGVHD (OR = 0.24, 95% CI: 0.05-1.06, P = .07). Although the study could not resolve the primary question, it provided important information for future cGVHD study design in this population.

Clinical ResearchRandomized Trial of Hydroxychloroquine for Newly Diagnosed Chronic Graft-versus-Host Disease in Children: A Children’s Oncology Group Study

The Childrens Oncology Group conducted a multicenter Phase III trial for chronic graft-versus-host disease (cGVHD). The double-blind, placebo-controlled, randomized study evaluated hydroxychloroquine added to standard therapy for children with newly diagnosed cGVHD. The study also used a novel grading and response scoring system and evaluated clinical laboratory correlates of cGVHD. The primary endpoint was complete response (CR) after 9 months of therapy. Fifty-four patients (27 on each arm) were enrolled before closure because of slow accrual. The CR rate was 28% in the hydroxychloroquine arm versus 33% in the placebo arm (odds ratio [OR] = 0.77, 95% confidence interval [CI]: 0.20-2.93, P = .75) for 42 evaluable patients. For 41 patients with severity assessment at enrollment, 20 (49%) were severe and 18 (44%) moderate according to the National Institutes of Health Consensus Conference global scoring system. The CR rate was 15% for severe cGVHD and 44% for moderate cGVHD (OR = 0.24, 95% CI: 0.05-1.06, P = .07). Although the study could not resolve the primary question, it provided important information for future cGVHD study design in this population.

Impact of Conditioning Regimen in Allogeneic Hematopoetic Stem Cell Transplantation for Children with Acute Myelogenous Leukemia beyond First Complete Remission: A Pediatric Blood and Marrow Transplant Consortium (PBMTC) Study

Total body irradiation (TBI)-based conditioning regimens for pediatric patients with acute myelogenous leukemia (AML) beyond first complete remission (CR1) are controversial. Because the long-term morbidity of busulfan (Bu)-based regimens appears to be lower, determining efficacy is critical. We retrospectively evaluated 151 pediatric patients with AML beyond CR1, comparing outcomes in 90 patients who received a TBI-based conditioning regimen and 61 patients who received a Bu-based conditioning regimen. There were no differences between the 2 groups with respect to age, sex, duration of CR1, time from most recent remission to transplantation, or donor source. The probability of relapse at 2 years also did not differ between the 2 groups (26% and 27%, respectively; P=.93). No significant difference in event-free survival (EFS) (P=.29) or overall survival (OS) (P=.11) was noted between the 2 groups. These findings were supported by a multivariate analysis in which TBI was not associated with improved EFS (hazard ratio [HR]=1.17; 95% confidence interval [CI]=0.66-2.10; P=.58) or OS (HR=1.42; 95% CI=0.76-2.64; P=.27). Shorter CR1 and receiving an HLA-mismatched transplant adversely affected EFS and OS in this cohort. Our study provides no evidence of an advantage to using TBI in children with AML beyond CR1. A prospective, randomized study is needed to confirm these results.

A Collaborative Step-Wise Process to Implementing an Innovative Clinic for Adult Survivors of Childhood Cancer

With a 5 year survival rate of approximately 80%, there is an increasing number of childhood cancer survivors in the United States. Childhood cancer survivors are at an increased risk for physical and psychosocial health problems many years after treatment. Long-term follow-up care should include education, development of individualized follow up plans and screening for health problems in accordance with the Childrens Oncology Group survivor guidelines. Due to survivor, provider and healthcare system related barriers, adult survivors of childhood cancer (ASCC) infrequently are receiving care in accordance to these guidelines. In this paper we describe the stepwise process and collaboration between a childrens hospital and an adult academic medical center that was implemented to develop the Survivorship Transition Clinic and address the needs of ASCC in our region. In the clinic model that we designed ASCC follow-up with a primary care physician in the adult setting who is knowledgeable about late effects of childhood cancer treatment and are provided transition support and education by a transition nurse navigator.

SUCCESSFUL MOBILIZATION WITH AMD3100 AND FILGRASTIM WITH ENGRAFTMENT OF AUTOLOGOUS PERIPHERAL BLOOD STEM CELLS IN A HEAVILY PRETREATED PEDIATRIC PATIENT WITH RECURRENT BURKITT LYMPHOMA

The authors report a case of a 13-year-old female with recurrent Burkitt lymphoma who was heavily pretreated with chemotherapy. During chemotherapy for relapse, she developed serious aspergillus infection of the palate and sinuses. Despite 10 μg/kg of filgrastim for 5 days, peripheral blood CD34+ cells remained ≤1/μL. Hematopoietic progenitor cells were subsequently successfully mobilized with a combination of AMD3100 and filgrastim without tumor or fungus contamination. After transplantation, the patient achieved complete engraftment without exacerbation of fungal infection. This is the first case report of a pediatric patient with relapsed Burkitt lymphoma with mobilization of hematopoietic progenitor cells by AMD3100 without any adverse reactions.

Relationship between pediatric blood and marrow transplant center volume and day +100 mortality: Pediatric Blood and Marrow Transplant Consortium experience

The number of patients receiving a BMT is currently being used as a factor in the accreditation process in determining whether a center can provide a high-quality BMT. Such criteria particularly impact pediatric BMT centers as most of them perform a relatively small number of BMTs. To determine whether patient volume is a valid marker of pediatric BMT center’s capabilities, the Pediatric Blood and Marrow Transplant Consortium (PBMTC) evaluated data from its registry to define the relationship between a pediatric transplant center’s patient volume and day +100 mortality. The analyses evaluated 2575 transplants from 60 centers reporting to the PBMTC between the years 2002 and 2004. The volume–outcome relationship was evaluated while adjusting for 46 independent data categories divided between nine variables that were known- or suspected-mortality risk factors. We found no association between transplant center volume and day +100 mortality in several analyses. A calculated intraclass correlation coefficient also indicated that differences in individual transplant center volume contributed to only 1% of the variance in day +100 mortality within the PBMTC. The results of this study suggest that factors other than transplant center volume contribute to variation in day +100 mortality among pediatric patients.

Nurse navigation: The key to a seamless transition.

84 Background: Childrens Mercy (CM) established a cancer survivorship clinic that cares for approximately 180 survivors a year. A third of the survivors are 18 years or older requiring transition to adult care. The importance of transitioning childhood cancer survivors from pediatric oncology care to adult primary care has been acknowledged in literature, but obstacles remain. Barriers include patient and provider anxiety, difficulty navigating the complex health care system and lack of knowledge regarding late effects. CM and The University of Kansas Cancer Center (KUCC) collaborated to decrease barriers to transition for childhood cancer survivors. METHODS The work group met for 2 years to develop the clinic at KUCC. Models and delivery of survivorship care, including the breast cancer survivorship clinic at KUCC, were reviewed. A shared nurse navigator was identified as an essential component to a seamless transition. Philanthropic support was obtained and job description was developed. Contracts were negotiated to allow the navigator to be present at both institutions. RESULTS The Survivorship Transition Clinic (STC) at KUCC launched July 2014, with a navigator supporting patients at CM as they begin their transition. The same navigator then meets with the patient at KUCC STC as they initiate care. The navigator provides treatment summaries, patient education and navigates the referral services for survivors. Since clinic launch, 16 survivors out of 16 have successfully transitioned from pediatric to adult survivorship care. Positive feedback was received from patient satisfaction surveys that were administered. A common theme showed a relief from anxiety due to having a contact person to facilitate communication among providers in a complex healthcare system. CONCLUSIONS The nurse navigator has minimized anxiety about transition for patients and parents as she establishes a relationship with patients at CM, and then is able to provide continuity as they transition to the STC at KUMC. The navigator also improves communication between pediatric providers, adult primary care providers and sub-specialists caring for the patients. Overall, our transition process has been effective and is now serving as a model across both institutions.