Affiliations: Department of Medicine, Cardeza Foundation for Hematologic Research, Sidney Kimmel Medical College, Thomas Jefferson University, Philadelphia, PA, USA
Correspondence:
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Corresponding author: Samir K. Ballas MD FACP, Cardeza Foundation for Hematologic Research, 1020 Locust Street, Philadelphia, PA 19107, USA. Tel.: +1 856 745 6380; Fax: +1 856 795 0809; E-mail: [email protected].
Abstract: 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 (SCA) using allogeneic bone marrow transplantation or cord blood transplantation. SCD is essentially a triumvirate of (1) pain syndromes, (2) anemia and its sequelae and (3) organ failure, including infection. Pain, however, is the hallmark of SCD and dominates its clinical picture throughout the life of the patients. The prevalence of these complications varies with age from infancy through adult life. However, pain, infections and anemia requiring blood transfusion occur throughout the life span of affected patients. The overall medical care of patients with SCD in developed countries has improved such that their life expectancy has almost doubled since 1951. Currently, there are at least five major approaches for the general management of SCD and its complications. These include (i) symptomatic management, (ii) supportive management, (iii) preventive management, (iv) abortive management, and (v) curative therapy.
