Venous thromboembolism in pediatric patients with sickle cell disease: A north American survey on experience and management approaches of pediatric hematologists

Background: There is a lack of pediatric-specific data to guide recommendations for prevention and management of venous thromboembolism (VTE) in sickle cell disease (SCD). Experience and expert opinion in this area have not been reported. Objectives: To characterize the management practices of pediatric hematologists in SCD-associated VTE. We hypothesized there is substantial variability in duration of therapy and prophylaxis preferences. Methods: Electronic survey among pediatric hematologists members of three international subspecialty societies/consortia. Results: Among 52 complete respondents (response rate, 42%), 47% of physicians reported treating 1–2 patients with SCD-associated VTE, while 20% treated 3–5 patients during the preceding 12 months. Most respondents (86%) estimated the risk of VTE recurrence at <5%. The vast majority (98%) of respondents prescribed anticoagulation for symptomatic VTE treatment. Duration of therapy varied by VTE type, 95% reported prescribing 6 weeks-3 months for provoked DVT, while 60% reported prescribing a similar duration for provoked PE with the remaining 40% reporting treating PE for longer duration. There was notable variation in the treatment practices for asymptomatic or unprovoked VTE. Lastly, half of physicians indicated to be “somewhat” (40%) and “not at all” (10%) confident making decisions regarding the duration and intensity of prophylactic anticoagulation. Conclusions: We identified significant variability in practice, a lower than expected perceived risk of recurrence, and uncertainty regarding VTE prophylaxis strategies in pediatric SCD. Cooperative multicenter studies are needed to generate evidence for future treatment guidelines development, and to identify opportunities for interventions aimed at managing and preventing VTE in pediatric SCD.