A 21-Year-Old Woman with Sickle Cell Disease and Vaso-Occlusive Pain Associated with Using an Electronic Nicotine Dispensing System (E-Cigarette or Vape) – a Case Report

Patient: Female, 21-year-old Final Diagnosis: Sickle cell anemia • vaso-occlusive pain Symptoms: Hypoxia • pain Clinical Procedure: — Specialty: Hematology OBJECTIVE: Rare disease BACKGROUND: Sickle cell disease is an inherited blood disorder that leads to multisystem complications. The heterogeneous course of sickle cell disease is due to both genetic modifiers and environmental factors. Cigarette smoking is a strong risk factor for sickle cell complications and even secondhand exposure to tobacco smoke can be detrimental for individuals with sickle cell disease. However, no prior reports have associated e-cigarettes and sickle cell vaso-occlusive pain. CASE REPORT: A 21 year old woman presented with sickle cell disease SS complicated by frequent pain, multiple acute chest syndrome episodes, sickle cell nephropathy, and avascular necrosis of the left hip, plus mild intermittent asthma. She developed pain in the ribs and back after her first use of e-cigarettes. After 4 days of home pain management, she came to the Emergency Department. She was mildly hypoxic and received supplemental oxygen. Chest radiograph did not show airspace consolidation, and the sites of pain were consistent with her prior pain episodes, so the diagnosis was sickle cell vaso-occlusive pain. Her hemoglobin was more than 2 g/dL below baseline and she received a red blood cell transfusion on hospital day 2. Overall, this was among her more severe pain episodes. CONCLUSIONS: The rising popularity of e-cigarettes, also known as vapes or Electronic Nicotine Delivery Systems (ENDS), is partly due to the misconception that they are safer than traditional cigarettes. Although firm conclusions will depend on studies designed to provide rigorous evidence, this case suggests that the acute adverse effects of ENDS might trigger complications of sickle cell disease, especially with asthma as a comorbidity.