7th Annual Sickle Cell Disease Research and Educational Symposium and 36th National Sickle Cell Disease Scientific Meeting
Authors: John Strouse, Rachel Alade, Regina Crawford, Victor Uruttia, Ami Vikani, Sophie Lanzkron, Kathryn Hassell, Susan Claster, Amanda Termuhlen, Sheree Schrager, Courtney Johnson, Tony Lo, Julie Wolfson, Ellen Iverson, Diane Nugent, Melissa Rhodes, Jennifer Thornton, Laura Adkins, Tina Andrews, Sandhya Ramachandran, Mary Gerhardstein, Linda Stoverock, Coretta Jenerette
Journal: American Journal of Hematology (2013)

Background:The risk of stroke is greatly increased in children and adults with sickle cell disease (SCD).Before the widespread implementation of screening by transcranial Doppler ultrasound, 11% of people with sickle cell anemia (HbSS) and 2% with hemoglobin SC disease (HbSC) had a stroke by 20 years of age.1 Regular transfusion of sickle-negative red blood cells greatly reduces and hydroxyurea moderately reduces the risk of recurrent stroke in children.2More strokes (~75%) occur in adults than children with SCD,3 but the treatments have not been rigorously evaluated and may vary with the age of first stroke and genotype of SCD.Objectives: To describe the variation in the treatment of stroke in adults with SCD. Methods:We invited by e-mail members of the Sickle Cell Adult Provider Network (SCAPN) to complete a 41-question web-based survey (Survey Monkey, Palo Alto, CA).We asked general questions about provider and center characteristics, use of thrombolytics, and knowledge of and agreement with published stroke guidelines in addition to the preferred (>50%) acute management, evaluation, and long term treatment of 4 specific cases.We compared responses among adult hematologists, pediatric hematologists, and other physicians (general internists and Med/Peds, neurologists, transfusion medicine, and emergency medicine) using Student's t-test and the chi-squared statistic.Results: We surveyed 253 SCAPN members and received 79 responses (31%) including 52 from physicians that care for adults with SCD.This group was 56% female with a mean age of 47 ± 11 years.Most (82%) would always or mostly consult a neurologist/stroke expert to assist in the management of acute stroke in an adult with SCD.Red cell exchange was the preferred therapy (90% of respondents) for acute ischemic stroke in 21 year old with HbSS as well as a 45 year old with HbSC (71%) with simple transfusion considered an acceptable alternative by some providers for stroke in the adult with HbSS, but not HbSC.The acute treatment of ischemic stroke was similar for physicians with different residency training except for less frequent use of intravenous thrombolysis by pediatric and adult hematologists and less frequent use of simple transfusion in HbSS by adult hematologists compared to other specialties.Treatments for the secondary prevention of stroke were similar, except for the less frequent use of simple transfusion by adult hematologists compared to all others and the less frequent use of hydroxyurea by pediatric hematologists compared to adult hematologists and other physicians. Conclusions:There is substantial agreement on the acute management and secondary prevention of ischemic stroke in adults with SCD despite a paucity of evidence.The greatest variation is in the use of specialized therapies: thrombolysis for the acute treatment of stroke and hydroxyurea for secondary prevention.The lack of evidence and variation in practice present opportunities for prospective study of stroke management in adults with SCD.