Sickle Cell Disease and COVID-19 Infection: Importance of COVID-19 Testing and Approach to Management

Sickle cell disease is an autosomal recessive disorder resulting in the substitution of CTG by CAG in the sixth codon of the beta-globin gene. As a result of this, the hydrophilic glutamic acid residue is replaced by hydrophobic valine residue, leading to the formation of hemoglobin tetramer HBS. This alteration in the beta-globin chain makes the red blood cells prone to sickling, especially in the presence of risk factors such as stress, hypoxia, and infection. These sickled red blood cells have the tendency to adhere to the endothelium and lead to vessel occlusion and distal tissue ischemia. The recent coronavirus disease 2019 (COVID-19) outbreak has impacted millions across the globe, putting individuals with co-morbidities at particularly high risk, and patients with sickle cell disease are no exception. We present the case of a 47-year-old African American male presenting to the emergency department with subjective fevers and a two-day history of pain in the arms, legs, and chest. A diagnosed case of sickle cell disease, the patient was on hydromorphone for pain management but ran out of his medications a few weeks before presentation. On examination, the patient was saturating well with mild tenderness upon palpation of the arms, legs, and chest. On complete blood count, the patient had a hemoglobin of 11.3 g/dL and a white cell count of 13.1 x10(3)/mcL. The patient had a normal mean corpuscular volume with reticulocytosis, hypochromia, ovalocytosis, poikilocytosis, polychromasia, and target cells. Severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) polymerase chain reaction (PCR) was positive. The chest X-ray did not reveal any significant findings. He was admitted to the medicine floor for the management of sickle cell crisis and was placed under airborne and droplet precautions. The patient was started on hydromorphone for pain management and intravenous fluid hydration. On the second day of admission, the patient reported increasing shortness of breath. He was saturating 90% on room air and 94% on 2 liters of supplemental oxygen. The white blood cell count increased to 18.42 x10(3)/mcL and the chest X-ray revealed reticular densities with patchy alveolar opacities in the left lung. Given the decline in respiratory status, the patient was started on remdesivir. Over the course of his hospital stay, the patient's pain and respiratory status improved, with the patient saturating 97% on room air. He was discharged home with instructions to follow isolation precautions for at least two weeks, folic acid, and adequate pain management. An appointment was also scheduled for the patient to follow with a sickle cell nurse practitioner upon discharge.