Transfusion (Tx) Practices in the Management of Sickle Cell Disease (SCD) among Florida Physicians.
Authors: Dunbar Levette , Coleman LaRae , Hartzema Abraham G. , Deleon Jessica M. , Lottenberg Richard 
Published: October 2019

Abstract
Limited data exist on Tx prescribing preferences in the management of SCD. To assess current Tx practices, we conducted a survey of Florida hematologists/oncologists from fall of 2005 through spring 2006. The 31-item survey addressed practice and SCD patient population characteristics, practice guidelines, Tx settings, indications/techniques, RBC phenotype specifications/modifications, iron overload, and educational resource utilization. A total of 150 physicians (75% adult-oriented, 25% pediatrics) completed the survey. Practices were primarily private (76%), with 24% in academic settings. Pediatric practices had more SCD patients (97% with 21 or more) than adult practices (83% had 10 or fewer). The majority of pediatric practices had specific Tx guidelines (61%), in contrast to adult practices (8%),(P< 0.0001). A minority of all respondents requested routine prophylactic limited or extended phenotypic matching of RBCs (37%) compared to those not matching until antibodies were identified (63%). RBC product modifications included non-leukocyte reduced, leukocyte-reduced (LR), irradiated, washed, and sickle cell negative. Overall, respondents “always” requested LR blood products (83%). There was a statistically significant difference among pediatricians (81%) and adult practitioners (11%) with respect to “always” requesting sickle cell negative RBCs (P< 0.0001). Emergent automated exchange Tx was available in the majority of pediatric (94%) and adult practices (70%). Tx was “always” indicated for acute stroke with 78% of all respondents, acute chest syndrome (56%), and acute priapism (53%). Tx was “always” or “sometimes” indicated pre-operatively for general anesthesia (86%), vitreoretinal surgery (71%), and acute painful episodes (50%). We queried 10 chronic Tx therapy indications. Pediatric practice respondents indicated that primary prevention of stroke was “always” (37%), “sometimes” (23%), rarely (14 %) and “never” (23%) an indication. Prevention of recurrence of stroke was “always” an indication for Tx in 94% of the pediatric respondents, compared to 26% of the adult practitioners (P< 0.0001). Uncomplicated pregnancy was “sometimes” (33%) and “always” (5%) an indication for chronic Tx with the adult practitioners. The majority of respondents used ferritin levels (94%) to assess iron status when starting deferoxamine. Pediatricians used liver biopsy more frequently (36%) than adult providers (12%),(P< 0.002). Most physicians felt that patient refusal (63%) and lack of compliance (61%) were “very important” reasons for not using deferoxamine, while fear of side effects (51%), poor patient understanding (47%), and costs (40%) were “somewhat important”. Analysis of clinical vignettes revealed statistically significant differences among physicians in the Tx management of elective cholecystectomy, acute chest syndrome and secondary stroke prevention after prolonged Tx therapy. While most respondents from pediatric practices used the NIH Management of SCD monograph (53%), only 27% of adult practice respondents used it (P< 0.005). In response to an open-ended question about materials that would be helpful, 18 of the 54 physicians wanted Tx guidelines. These data indicate variability in the incorporation of evidence-based approaches in Tx management of SCD. The results provide insights into the development of clinical tools and guidelines tailored to pediatric and adult practices.