Presentation, Management and Outcomes of COVID-19 Patients with Sickle Cell Disease

Background Sickle cell disease (SCD) is an inherited disorder of red blood cell (RBC) caused by a mutation in the beta-globin gene resulting in abnormal hemoglobin known as hemoglobin S (HbS) or the sickle hemoglobin. Several clinical variants of SCD have been elucidated, all driven by two fundamental pathophysiologic processes: RBC hemolysis and intermittent vaso-occlusive vasculopathy resulting in tissue ischemia/infarction. These two processes underscore the many complications and eventual multi-organ damage that may develop in patients with the most severe types of SCD. Cardiopulmonary complications including heart failure, pulmonary hypertension and acute chest syndrome (ACS) are major drivers of morbidity and mortality among patients with SCD. With regards to ACS, patients often present with fever, cough and shortness of breath caused by vaso-occlusive crisis affecting the lungs. This is particular concerning in view of its similar features to symptomatic COVID-19 infection. Methods We retrospectively identified SCD patients with COVID-19 infection admitted to Beaumont hospitals in Michigan between March 1st 2020 and July 1st 2020. Data was abstracted using the ICD 10 code of U07. 1 for COVID-19, ICD 9 and 10 codes of 282.60 and D57 for sickle cell disease. We excluded patients with sickle cell trait. Data regarding the demographics, presentation, management and outcomes were abstracted. Results A total of eleven patients with sickle cell disease were identified as having a positive SARS-Cov19 polymerase chain reaction test (Table I). All were African American and predominantly female (64%) with a mean age of 44 (22-60) years and mean BMI of 30.2 kg/m2. Genotypes identified were HbSS in 5 (45%) patients, HbSC in 4 (36%), HbS/beta-thalassemia in 1 (9%) and HbS/alpha-thalassemia in 1 (9%). All of the patients had seen a haematologist since their diagnosis but none of the patients were on hydroxyurea, voxeloter, L-glutamine or crizanlizumab at admission. The predominant clinical presentation was fever, chest pain, chills, exertional shortness of breath and cough but this was not consistent across all patients. All the patients were managed with intravenous hydration, pain management as well as hydroxychloroquine/azithromycin per institutional guideline at that time. Three patients (cases 1-3) had recurrent visits to the hospital for similar symptoms and new bone pain crises. Case 1 had a pulmonary embolus which was evident on re-admission. Two patients