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HIV AND SICKLE CELL DISEASE

Source: www.chartcaribbean.org
Topic: Sickle Cell Disease
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Sort Desciption: Sickle cell disease (SCD) is a genetic disorder that is characterised by a chronic anaemia occurring almost exclusively in individuals of African descent. ...

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Sickle cell disease (SCD) is a genetic disorder that is characterised by a chronic anaemia occurring almost exclusively in individuals of African descent. Individuals afflicted with SCD are homozygous for a key mutation in haemoglobin, whereas individuals who are heterozygous for this mutation are generally asymptomatic and are said to have sickle cell trait. SCD is relatively common throughout the Caribbean and could present some unique clinical management issues as a co-morbidity with chronic HIV infection. CLINICAL MANIFESTATIONS The clinical manifestations of SCD are due to both anaemia and vaso-occlusive events that result in tissue ischemia and infarction. SCD patients suffering from painful vaso-occlusive events are said to be in sickle cell crisis. Common causes of death for individuals with SCD are intercurrent infections, multiple pulmonary emboli, occlusion of a vessel supplying a major organ, and renal failure. The average life span of SCD patients is age forty to forty-five years. Anaemia in SCD is usually stable but acute exacerbation of anaemia occurs in the setting of aplastic crisis, in which marrow red blood cell (RBC) production abruptly slows down. Aplastic crisis is usually the result of an acute infection; hence immunocompromised patients may be at higher risk for this complication. Individuals with sickle cell trait who are also HIV-infected are not expected to have significant worsening of any manifestations of their HIV disease or of their sickle cell trait. MANAGEMENT OF THE PATIENT WITH CO-MORBID SCD AND HIV INFECTION General Principles of Management Infection with encapsulated organisms is more common in SCD patients with functional asplaenia. HIV co-infection also appears to increase susceptibility to these infections, especially in children. Prophylactic antibiotics, pneumococcal vaccine, and early identification and treatment of serious bacterial infections are ...

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