Case Presentation: A 32-year-old woman with sickle cell HbSS disease presented due to acute encephalopathy in the setting of recent admission for diffuse urticarial rash treated with steroids and diphenhydramine. She was found to be hypotensive, tachycardic, and tachypneic with development of fever soon after presentation. Chest x-ray showed left basilar opacities. She was intubated for airway protection. Labs were concerning for disseminated intravascular coagulation. Empiric broad spectrum antibiotics were initiated and she was admitted to the medical intensive care unit. Head magnetic resonance imaging (MRI) showed ischemic changes though these were felt to be too small to be causing her presentation. There were no epileptiform abnormalities on electroencephalogram (EEG). During the first week of her hospitalization, she was gradually weaned off of vasopressors and her vent settings were minimized. However, on day nine of admission, she had onset of tonic-clonic movements, with EEG revealing status epilepticus. Lumbar puncture was unrevealing. Repeat head MRI showed extensive supra and infratentorial susceptible foci concerning for fat emboli. The patient was managed with red cell transfusions and antiepileptics. Her mental status gradually improved. Hemoglobin electrophoresis showed suppressed hemoglobin S of 7.3%. A tracheostomy was placed due to prolonged intubation, which was eventually decannulated. The patient was transferred to Hospital Medicine on day 28 of hospitalization. An individualized care plan from her outpatient team guided inpatient pain management. She worked with Physical and Occupational Therapy to regain her strength. Her primary outpatient team completed a formal consult during hospitalization to help guide her transition plan moving towards discharge. When her pain was adequately controlled on an oral regimen and family support and services were in place, she was discharged home with plan for close outpatient follow-up. She was able to transition back to life outside of the hospital.
Discussion: Fat embolism syndrome is a rare and life-threatening complication of sickle cell disease when fat globules are released into the circulation due to bone marrow infarction and necrosis. Symptoms are often nonspecific but may include pain, change in mental status, rash, and/or respiratory distress. It is more often seen in people with compound heterozygous hemoglobin S mutations with few reports of its occurrence in people with HbSS disease. Fat embolism syndrome leads to a challenging clinical course with long-term rehabilitation needs both inpatient and after discharge. While handoff is often completed with outpatient providers at the end of hospitalization, this patient’s outpatient team provided formal recommendations during hospitalization. The successful management of fat embolism syndrome in this patient underscores the importance of transitions of care from inpatient to outpatient, especially in patients with prolonged hospital courses.
Conclusions: Fat embolism syndrome is rare but associated with high morbidity and mortality. It is important for hospitalists to consider fat embolism syndrome when patients with sickle cell present with altered mental status as patients may decompensate quickly with multi-organ failure and require transfer to the intensive care unit. Once transferred back to the care of hospitalists, patients will require extensive coordination of care and close communication with outpatient teams prior to discharge for a smooth transition.