Management of sickle cell disease: progress during the past 10 years

Despite the lack of successful drug development during the last 10 years, significant advances have been made in the management of a number of the complications of sickle cell disease. Identification of the vascular changes in the retina has led to the use of argon or zenon laser therapy to prevent progression of these changes to retinal hemorrhage and loss of visual acuity. Aseptic necrosis of the head of the femur or humerus is now successfully managed with the insertion of a bone graft or prosthesis. Exchange transfusions are believed to have prevented recurrent cerebrovascular accidents. The use of prophylactic penicillin and pneumococcal vaccine may have reduced the incidence of sepsis, pneumonia, and meningitis due to susceptible organisms in young children. While this progress can be said to have improved the quality of life of many patients, other complications of sickle cell disease remain as therapeutic dilemmas. The ability to prevent complications is hampered both by a lack of specific therapeutic measures and ignorance as to which patients are likely to develop complications.