Sickle cell disease in pregnancy

Abstract

Structural and quantitative changes at the polypeptide chains of haemoglobin lead to defective red blood cells with a life span 1/5th of the normal, a much smaller capacity of oxygen saturation, and a less pliable structure that easily deforms in situations of hypoxia, stress, acidosis, dehydration, cold and prolonged physical effort etc. Multi-organ vaso-occlusion and hypoxia ensues, causing severe bone pains, sequestration, infarction and anaemia. Increased physiologic demands of pregnancy, aggravate falciformation resulting to poor perfusion of the placenta predisposing to preterm pregnancy loss, intrauterine growth retardation, pre-eclampsia, severe anaemia and increased perinatal and maternal morbidity and mortality. Urinary tract, respiratory and bone infections, as well as cardiac and neurologic complications are common. Treatment is preventive and symptomatic. It aims at reducing high risk combinations, and a meticulous follow-up to prevent the woman from developing complications and not dying from these complications. Though vasso-occlusive pain crises and severe anaemia may be fatal they may be remedied by generous use of analgesics, adequate hydration and exchange transfusion. Sickle cell anaemia the most common of the three major haemoglobinopathies is more frequent with individuals of African descent, haemoglobin C is more predominant in the coast of Africa west of the river Niger while alpha thalassemia and beta thalassemia haemoglobin are frequent in the Mediterranean and Southeast Asian countries respectively.

Keywords: sickle cell anaemia, haemoglobin S, haemoglobin, thalassemia haemoglobin, haemoglobinopathy, pregnancy, vaso-occlusion, bone pain crises, exchange transfusion

Clinics in Mother and Child Health Vol. 1(1) 2004: 53-60