Inova Health System Alexandria, Virginia, United States
Background/Case Studies: Sickle cell disease is characterized by a wide range of disease pathology requiring acute and longitudinal care, often involving emergency intervention with therapeutic red cell exchange or narcotic management for acute pain crises. Young adults who feign signs and symptoms of sickle cell disease are described as having factitious sickle cell disease. This case series describes three cases of factitious sickle cell disease and highlights the role of the Sickle Cell Solubility Test (SCST) as a rapid and inexpensive screening tool with a sensitivity above 97% for the presence of true sickle cell disease above the limit of detection of a Hemoglobin S concentration of 1.2 g/L and Hemoglobin S levels above 15%.
Study
Design/Methods: We performed a retrospective chart review of patients treated at the institution who received care for reported sickle cell disease symptoms. Patients were identified as having factitious sickle cell disease when they received a negative sickle cell solubility test and no hemoglobin S was found on hemoglobin electrophoresis. Hospital courses were summarized and presented here.
Results/Findings: Three cases were identified with factitious sickle cell disease that was discovered using sickle cell screening tests followed by hemoglobin electrophoresis.
A 27-year-old male with a reported history of sickle cell disease with stroke, pulmonary embolism, and acute chest presented to the emergency department with concerns of left-sided face, arm, and leg weakness and tingling. He received an RBC exchange with 6 units of matched RBC, however his sickle screen test was later found to be negative, and hemoglobin electrophoresis demonstrated no Hg S.
A 37-year-old female with a reported history of sickle cell disease with pain crises was admitted for concerns of right-sided weakness, dizziness, and confusion. The patient received a sickle cell screen test which resulted negative and hemoglobin electrophoresis demonstrated no Hg S. A psychiatric evaluation revealed a history of psychiatric admission for major depressive disorder and generalized anxiety disorder. She was diagnosed with factitious disorder.
A 36-year-old female with a reported history of sickle cell disease presented to the emergency department with lower extremity and back pain. She was started on a patient-controlled analgesia pump. A sickle cell screen test was conducted which was negative and hemoglobin electrophoresis demonstrated no Hg S. Her narcotic pump was discontinued, and she was discharged from the hospital.
Conclusions: Factitious sickle cell disease is a recognized phenomenon with the potential for costly and unnecessary interventions. The use of a common screening tool such as the Sickle Cell Solubility Test and high index of clinical suspicion can mitigate the need for expensive testing, reduce length of hospitalization, and allow for judicious use of vital hospital resources (blood products).
