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Bone and joint complications in sickle cell disease

Alex George, MD, PhD
Michael R DeBaun, MD, MPH
Section Editors
Donald H Mahoney, Jr, MD
William Phillips, MD
Stanley L Schrier, MD
Deputy Editor
Jennifer S Tirnauer, MD


Sickle cell disease (SCD) is characterized by a marked heterogeneity in clinical and hematologic severity [1,2], with bone and joint problems being the most common manifestations. Pain caused by vasoocclusive episodes may occur as often as every week, or individuals with SCD may go with long stretches of time without any pain events. Many individuals with SCD also suffer from the long-term consequences of vasoocclusive pain episodes in the musculoskeletal system, such as avascular necrosis of the femoral heads or collapsed vertebral bodies, leading to a chronic state of pain in addition to the more acute painful episodes.

Bone and joint complications in SCD are discussed here. Other manifestations of SCD are discussed separately. (See "Overview of the clinical manifestations of sickle cell disease".)

The management of individuals with SCD is discussed separately in an overview topic and in individual topics on the treatment of specific disease manifestations. (See "Overview of the management and prognosis of sickle cell disease".)


Repeated vasoocclusive pain episodes are the hallmark of sickle cell disease (SCD). Clinicians caring for children and adults with SCD refrain from using the term "sickle cell crises" when describing a vasoocclusive pain episode because "crisis" does not appropriately depict the clinical situation.

Pain episodes can lead to bone infarcts, necrosis, and, over time, degenerative changes in marrow-containing bone. Pain episodes can occur in early infancy and into adulthood, and are a major cause of hospitalization in SCD. Among individuals with SCD in the United States, almost 90 percent of all visits to the emergency department are for painful episodes, as are almost 70 percent of all hospitalizations, with an average duration of 8 to 11 days for adults and 4 to 5 days for children. Similar numbers are found in individuals with SCD in the United Kingdom [3].


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Literature review current through: Sep 2016. | This topic last updated: Mar 1, 2016.
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