Case Presentation: Hemophagocytic lymphohystiocytosis (HLH) is a rare cause of pancytopenia. Patients with sickle cell disease can present with pancytopenia and recognition of (HLH) in patients with sickle cell disease may be a lifesaving diagnosis.Case presentation:A 39-year-old female with past medical history of sickle cell anemia and moyamoya disease was admitted with sickle cell vaso-occlusive [...]

Case Presentation: A 12-year-old Caucasian male with no significant medical history presented with a 6-day history of bilateral leg pain following a soccer game. As an avid soccer player, he had experienced similar episodes over the past three years, managed previously as “growing pains” with NSAIDs and acetaminophen. However, this presentation raised concern for an [...]

Background: Sickle cell disease (SCD) affects about 100,000 people in the US. The disease causes significant mortality, morbidity and frequent hospitalizations secondary to primary manifestations of the disease and its complications. We conducted a retrospective, single institution study in adult patients (pts), ≥ 18 years with SCD to assess factors associated with health care utilization [...]

Background: Patients with sickle cell disease (SCD) experience painful and life threatening vaso-occlusive episodes (VOE) that cause frequent hospitalizations, account for >80% of their >$1 million lifetime healthcare costs, and can be fatal. Yet, there is a dearth of evidence addressing inpatient VOE care leading to a stark absence of evidence-informed guidelines. As a first [...]

Background: Approximately 10% adults with Sickle Cell disease (SCD) has Pulmonary hypertension (PHTN). There are several etiologies like hypoxia driven and microvascular obstruction contributing to PHTN. However, the in-hospital outcomes of PHTN on SCD has not been well studied. Methods: We queried 2018 National Inpatient Sample (NIS) database to identify SCD and different types of [...]

Background: Sickle cell disease (SCD) is an autosomal recessive disease resulting in a broad range of functional and structural renal and cardiac alterations. Chronic kidney disease is with associated proteinuria, microalbuminuria and hemoglobinuria. Cardiac complications include diastolic heart disease, dysrhythmia and sudden death. In patients with advancing age, cardio-renal dysfunction can have substantial effects on [...]

Background: Sickle cell disease (SCD) causes frequent painful episodes from vaso-occlusion. As hospitalists, we identified that SCD patients occupied a large number of hospital days. Undertreatment and lack of standardization of pain management was a likely reason for this. Many of these patients are opiate dependent at baseline. Varying levels of comfort amongst hospitalists in [...]

Background: Sickle cell disease (SCD) and its vasoocclusive sequela are significant causes of morbidity and mortality as well as acute care utilization throughout the United States, with an estimated 92,880 hospital admissions totaling $759 million in aggregate annual costs in 2014. There is a need within health care systems to identify strategies for achieving higher [...]

Background: Adults with sickle cell disease (SCD) often present with extremely painful acute vaso-occlusive crises (VOC). VOC guidelines encourage early initiation of opioids, but are lacking in a standardized approach to achieve adequate analgesia and subsequent de-escalation. Various SCD studies comparing continuous basal versus demand bolus patient-controlled analgesia (PCA) show conflicting outcomes. Thus the objective [...]

Background: Nationally, a minority of patients with complex medical and psychosocial needs consume a disproportionate amount of healthcare. In the U.S. in 2015, the top 1% of the population accounted for 23% of all healthcare expenditures and cost ten times more per year than the average patient. No one disease accounts for a large percentage, [...]