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Sickle Cell Disease: A Comprehensive Review Of Inpatient Management
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Publication Date

September 2014 (Volume 2, Number 9)

CME

This issue includes 4 AMA PRA Category 1 CreditsTM; and 4 AOA Cetegory 2A or 2B CME credits

Authors

Raji Shameem, MD
Hematology/Oncology Fellow, Department Of Hematology/Oncology, Fox Chase Cancer Center, Temple University Hospital, Philadelphia, PA

Pooja Kumar, MD
Primary Care and Hospital Medicine, Ace Medical Group, Los Angeles, CA

Trushar M. Dungarani, DO
Clinical Assistant Professor, Assistant Director of Hospitalist Medicine, Department of Medicine, North Shore LIJ/Lenox Hill Hospital, New York, NY

Peer Reviewers

Susan Claster, MD
Clinical Professor of Medicine, Division of Hematology/Oncology, University of California, Irvine, Irvine, CA

Kathryn Hassell, MD
Professor of Medicine, Division of Hematology, University of Colorado; Director, Colorado Sickle Cell Treatment and Research Center, Aurora, CO

Abstract

Since sickle cell disease was first formally reported in 1910, its management and treatment have greatly advanced, but hospitalists are still faced with a persistent challenge in managing adult patients with this disease. There continues to be a large number of emergency department visits and hospitalizations for adults with this chronic condition, leading to substantial healthcare costs in the United States, even with protocols to prevent readmissions and avoidable prolonged hospitalizations. This review examines relevant topics in the hospital management of sickle cell disease and aims to assist physicians in providing comprehensive and cost-effective care for their patients. The initial presentation, differential diagnosis, and treatment of common life-threatening complications in sickle cell disease (such as acute pain crisis and acute chest syndrome) are discussed. Treatment strategies, including the role of hydroxyurea, appropriate transfusions practices, cost-effective strategies, and quality improvement measures are also discussed.

Excerpt From This Issue

A 28-year-old man with a history of homozygous sickle cell disease (HbSS) presents to the emergency department with severe pain. He said he has had 6 pain crisis episodes requiring hospitalization in the last year, but that he takes no medications at home. He is admitted for severe pain of his chest, ribs, and legs, but he is hemodynamically stable. An initial diagnosis of acute pain crisis is made and he is treated with intravenous analgesics. Forty-eight hours later, he develops new complaints of cough and shortness of breath. His temperature is 38.6C°, and pulse oximetry on room air is 89%. A chest x-ray reveals a new left lower lobe infiltrate compared to imaging done at admission. What complication has he developed? How would you treat him?

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