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Outpatient Management Issues in Sickle Cell Disease Reprinted by Permission of Kenneth R. Bridges, M.D. of the Overview Overview Sickle cell disease results from an amino acid substitution of valine for glutamic acid at position 6 of the beta-globin chain. This alteration causes deoxygenated sickle hemoglobin to form polymers that ultimately destroy red cells, thereby producing anemia. If sickle cell disease only produced anemia, the problem would be relatively minor, as people can tolerate the typical hemoglobin levels of 7 or 8 g/dl reasonably well. The key difficulty with sickle cell disease is the damage to tissues and organs produced when deformed sickle erythrocytes occlude the microcirculation. The variegate manifestations of sickle cell disease are due ultimately to this problem. The routine care of people with sickle cell disease does not necessarily require a hematologist. With few exceptions, the common complications are familiar to the experienced internist. The key to care of patients with sickle cell disease is knowing which problems are likely to result from the condition, and preventing these complications where possible. This means creating a program of regular follow-up in the office, rather than intervening when crises develop. The momentum in medical care has swung away from medical subspecialists and toward primary care physicians. Physicians trained in general medicine will play an increasingly important role in the management of patients with sickle cell disease. The care of patients with sickle cell disease does require the expertise of medical subspecialists at times. Episodes of acute chest syndrome or splenic sequestration crisis are best handled by hematologists who are familiar with these complications. Sickle cell disease also requires coordination with other medical specialists, such as ophthalmologists. In this sense, the disease is no different from any other chronic medical illness, such as diabetes or hypertension. Health monitoring and prevention of complications are key. Health maintenance, which has become a shibboleth in many discussions of medical care, can truly be achieved for patients with sickle cell disease. Further, the savings to society in avoiding lost productivity and unnecessary medical expense is significant. More significant is avoiding unnecessary suffering by our fellow human beings. |
