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The Case of a Female with “Sickle Cell Disease”

The Case of a Female with “Sickle Cell Disease”

Danielle Novetsky, M.D., MTS, ¹  Laurie Gordon, M.D., MA ²
¹Resident, Department of Pediatrics, Weill-Cornell Medical Center and ² Medical Director Pediatric Inpatient Unit, New York Hospital Queens, Flushing, NY

Background: The treatment of patients with sickle cell disease is fraught with difficulty. While attempting to treat a painful sickle cell crisis, the clinician must also be aware of the potential for malingering. This case report describes a patient who repeatedly simulated painful sickle cell crises without any underlying medical disease. 

Learning objectives: 1. Recognize features associated with malingering in a patient claiming sickle cell.  2. Describe how the diagnosis of sickle cell disease can be used by patients with an inclination for malingering and drug abuse.

Case presentation: A 19 year old African American female with an alleged history of Hemoglobin (Hgb) SC disease presented to the New York Hospital Queens (NYHQ) Pediatric Emergency Department (ED) with a complaint of pain in the hips and lower back. The patient presented without health insurance or an identifying Medicaid number claiming that she had moved to the US from Canada 2 weeks prior to admission.  She reported a history of multiple pain crises, one episode of acute chest, an open cholecystectomy, a prior femoral line infection, and two vaginal deliveries which resulted in two live children ages 6 years and 6 weeks. She reported a mother with Hgb SC disease, father with Hgb SS disease, and numerous affected cousins. She stated that ibuprofen, morphine, and oxycodone induced anaphylaxis. Initial CBC showed Hgb of 9.2 g/dL with MCV 78.6 fL and reticulocyte count of 1.5%. Laboratory results were otherwise normal. The patient was admitted to the pediatric ward for further management. On admission, the patient was afebrile without tachycardia or tachypnea. Despite normal vital signs, she rated her pain as “20” on a scale of 1-10. She stated that the only adequate treatment of her pain was 6 mg intravenous hydromorphone every 3 hours pre-medicated with IV diphenhydramine. She also reported a recent sexual assault by a family member while she was taking postpartum pain medication; this report initiated a social work consult.  The social worker recognized the patient from a previous hospitalization at NYHQ, under a different name, on an adult ward. At the same time, Hgb electrophoresis findings revealed 97% Hgb A, consistent with normal Hgb. Upon being presented with these findings, the patient disconnected her intravenous line and left the hospital. Of note, discussion with ACS revealed that ACS was already aware of the patient and her 6 week old child, who has been hospitalized since birth at another NYC hospital’s newborn intensive care unit undergoing hydromorphone withdrawal. The patient had been registered at that hospital under a third name. From the history and laboratory results, it was clear that the patient was addicted to narcotics and the history she shared with the pediatric staff was false.

Discussion:  Malingering has been well documented in adult patients with sickle cell disease [1-3], but has not been well described in the pediatric population. In the current case, a 19 year old patient was admitted to our pediatric floor with fictitious sickle cell disease and drug-seeking behavior.  Clinical red flags for fictitious sickle cell disease include: inconsistent CBC findings, requests for specific narcotics in very high and frequent doses and a discrepancy between reported pain and behavior. Close relatives with sickle cell disease may serve as clinical guides so that the patient knows what to say and how to act.  A high index of suspicion is needed for prompt diagnosis in order to provide appropriate care and minimize abuse of the medical system.

References:
1. Ballas SK. Factitious sickle cell acute painful episodes: a secondary type of Munchausen syndrome. Am J Hematol. 1996 Dec;53(4):254-8.
2. Ballas SK. Munchausen sickle cell painful crisis. Ann Clin Lab Sci. 1992 Jul-Aug;22(4):226-8.
3. Lindenbaum J. Hemoglobin Munchausen. JAMA. 1974 Apr 22;228(4):498

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