Pain management of sickle cell disease

Sickle cell disease (SCD) is an inherited disorder of hemoglobin structure that has no established cure in adult patients. Cure has been achieved in selected children with sickle cell anemia using allogeneic bone marrow transplantation [1] or cord blood transplantation [2]. SCD is a quadrumvirate of (1) pain syndromes, (2) anemia and its sequelae, (3) organ failure, including infection, and (4) comorbid conditions. Pain, however, is the insignia of SCD and dominates its clinical picture throughout the life of the patients (Fig. 1). Pain may precipitate or be itself precipitated by the other three components of the quadrumvirate. Moreover, management of sickle cell pain must be within die framework of the disease as a whole and not in isolation. SCD is unlike other pain syndromes where the provider can make decisions on treatment based solely on the pain and its associated behavior. A primary care physician, for example, taking care of a middle-aged patient with Job-related low back pain may decide to expel the patient from his or her care if the patient in question demonstrates suspicious drug-seeking behavior. Doing the same with patients who have SGD could be counterproductive. There are anecdotes of patients with SCD who were dismissed from certain programs only to be found dead at home within 24 hours after dismissal or to be admitted to other hospitals with serious complications [3]. Sickle pain could be the prodrome of a serious and potentially fatal complication of SCD Hit some patients. This article focuses on the pathogenesis and management of acute and chronic sickle cell pain.