Consensus definition of essential, optimal, and suggested components of a pediatric sickle cell disease center.

Sickle cell disease (SCD) requires coordinated, specialized medical care for optimal outcomes. There are no United States (US) guidelines that define a pediatric comprehensive SCD program. We report a modified Delphi consensus-seeking process to determine essential, optimal, and suggested elements of a comprehensive pediatric SCD center. Nineteen pediatric SCD specialists participated from the US. Consensus was predefined as 2/3 agreement on each element's categorization. Twenty-six elements were considered essential (required for guideline-based SCD care), 10 were optimal (recommended but not required), and five were suggested. This work lays the foundation for a formal recognition process of pediatric comprehensive SCD centers.

How we coordinate care for uninsured children with nonmalignant hematologic disorders.

Providing medical care for uninsured children with nonmalignant hematologic diagnoses presents unique challenges to medical providers and multidisciplinary staff. Financial and psychosocial stressors can hinder optimal care of the uninsured child. Maximizing coverage of medical costs through patient enrollment in state and charity care programs and capitalizing upon community partnerships can help providers achieve comprehensive care for these children. Collaboration between primary care providers, subspecialists, and multidisciplinary teams can be optimized to facilitate provision of hematology care for uninsured children. We detail our experience in establishing these collaborations to improve access to subspecialty care for children with nonmalignant hematologic disorders.

Provider Perspective on Integrative Medicine for Pediatric Sickle Cell Disease-related Pain.

OBJECTIVE: Integrative medicine (IM) approaches are sometimes used to manage sickle cell disease (SCD)-related pain. The purpose of this research is to (1) understand provider perspective towards the use of IM for pain in children with SCD at a large urban children's hospital and (2) provide recommendations on how to better promote IM for children and adolescents with SCD. METHODS: After approval from the institutional review board, a qualitative case study approach was used with criterion-type purposeful sampling to select providers from the division of hematology to adequately inform the study. Semistructured interviews were completed using audiotape to facilitate transcription. NVivo 10 analytic software (QSR International Pty Ltd, Doncaster, Victoria, Australia) was used to organize data into themes to answer the study questions. RESULTS: Ten provider interviews were completed. Attitudes were generally positive, and most providers felt that IM is generally helpful. All providers reported that they do not optimally use integrative therapies for children with SCD. The barriers uncovered focused on lack of process for integration of IM, specifically that IM resources seem transient and based on short-term funding. Provider attitude towards CAM is generally positive, but provider comfort level is highly variable. No providers are completely comfortable with their knowledge base about IM, and increased knowledge is desired. CONCLUSIONS: Creation of protocols and processes to incorporate IM into management plans for patients with SCD could help to promote its use. Education of providers about utility and efficacy of IM for SCD-related pain and about existing resources would aid in promotion of IM for children with SCD.

Effectiveness of red blood cell exchange, partial manual exchange, and simple transfusion concurrently with iron chelation therapy in reducing iron overload in chronically transfused sickle cell anemia patients.

BACKGROUND: Chronic transfusion therapy (CTT) is indicated for stroke prevention in children with sickle cell anemia (SCA) and is complicated by iron overload and alloimmunization. CTT is performed by simple transfusion (ST), partial manual exchange (PME), or erythrocytapheresis (RCE). Although small case series have demonstrated RCE in combination with iron chelation therapy stabilizes and/or decreases ferritin, there are no reports comparing the effect of ST, PME, and RCE on liver iron concentration (LIC). CTT modality effect on serum ferritin and LIC were compared in SCA patients on iron chelation, with hemoglobin (Hb)S goal of 30%. STUDY DESIGN AND METHODS: Medical records of SCA patients on CTT and deferasirox (≥25 mg/kg/day) were retrospectively reviewed. Mean HbS%, change in ferritin and LIC, and alloimmunization rate were determined for each CTT group. RESULTS: Twenty-eight patients were included; six crossed over (one from ST to PME, one from ST to PME then to RCE, three from ST to RCE, and one from PME to RCE) to include 36 transfusion modality intervals. Median pretransfusion HbS% levels were 32.7% (ST), 36.2% (PME), and 34.7% (RCE; p = 0.732). Median ferritin changes were +15 (-17 to +45), +38 (+24 to +105), and -91 (-141 to -48) ng/mL/month (p = 0.003), and median LIC changes (available in 22 patient transfusion modality intervals) were +1.3 (-1.6 to +4.3), +2.3 (-6.5 to +8.9), and -5.7 (-10.7 to -0.5) mg/g/year (p = 0.024) in ST, PME, and RCE, respectively. There was no significant difference in alloimmunization rate between ST/PME and RCE groups. CONCLUSION: We recommend RCE plus chelation as an effective method for reducing iron overload, while maintaining HbS at 30% to 35%.