![]() Assessment for either exchange blood transfusion or top-up transfusion may improve the acute situation. 20, 28-30 Prompt pain relief and treatment of bacterial and viral infections save lives. A differential diagnosis in the form of pulmonary embolus and severe pneumonia and in this current environment, COVID-19 pneumonitis, should be excluded by chest computed tomography. Low oxygen saturation or severe hypoxia mandate a timely transfer to the intensive care unit, as this may save lives. Essential investigations for diagnosis and prognosis should ensure a timely chest x-ray and full blood count, as well as arterial blood gas analysis. Although even rarer, HbS beta-zero also behaves like HbSS.Īcute chest syndrome is a significant complication of SCD characterized by fever and/or respiratory symptoms and new pulmonary infiltrate shown on chest x-ray. These risks may be more pronounced in HbSS than in HbSC. The babies of mothers with SCD are more likely to be born preterm, before 37 weeks' gestation, and thus there is a high need for neonatal intensive care admission, as well as an increased risk of reduced birth weight and stillbirth. 4, 5 During pregnancy, patients are more likely to require blood transfusions and admission to the critical care unit. 4 Pregnancy-related conditions such as hypertension and venous thromboembolism are significantly increased, as confirmed in other observational studies. Pregnancy in women with the condition is associated with a high risk of mortality, estimated to be up to 2%, and morbidity. Still, as most are living to reproductive age, management of this condition in pregnancy becomes more relevant. In Western countries and some developing countries, patients live up to their mid-50s. These manifestations result in reduced life expectancy. The polymerization/vaso-occlusion can result in end-organ damage, such as acute chest syndrome, pulmonary hypertension, stroke, renal dysfunction, retinal disease, and leg ulcers. 3 This leads to an increase in the breakdown of these cells, resulting in anemia and the sickle-shaped red cells polymerizing and causing the clinical features of acute pain, significant anemia, shortness of breath, and fatigue. The pathophysiology of SCD is a result of HbS in low oxygen conditions giving rise to rigid and fragile sickle-shaped red cells. 1 In the UK and France, there are estimated to be 12 000 to 15 000 affected individuals, 3 and in the UK, approximately 260 children are born with the condition each year. SCD is the most common inherited condition worldwide, with well over 300 000 children born with the disease each year, 75% of whom are born in Africa. The most common is homozygous SCD, but there are other compound heterozygous types, one of the unusual being HbS-for example, HbSC ( Table 1). SCD consists of a group of conditions caused by the inheritance of an abnormal sickle hemoglobin (HbS) gene. The mother and her baby did well post delivery without further concerns. After she was stabilized, a decision was made for a planned cesarean due to her previous cesarean. Still, a day before the delivery date she had a severe acute painful episode with significant anemia that required a top-up transfusion. Therefore, we recommended delivery at 38 weeks' gestation. The pregnancy progressed well with normal serial growth scans and minimal manifestation of SCD. She declined to go on any trials, including the TAPS2 trial (transfusion for pregnant women with SCD). ![]() During the second pregnancy, she was started on aspirin at 150 mg/d, folic acid, penicillin at 250 mg twice daily, and thromboprophylaxis in the form of dalteparin from 28 weeks onward. ![]() During that pregnancy she had an acute painful episode close to delivery and, as a result, had an exchange blood transfusion before the cesarean. Her first pregnancy was complicated by preeclampsia, necessitating delivery at 36 weeks by cesarean. Her husband was a carrier of SCD, and thus this pregnancy was conceived through preimplantation genetic diagnosis (PGD) to avoid the 50% chance of having offspring with SCD. A 28-year-old woman of African background, known to have sickle cell disease (SCD), presented to the clinic during her second pregnancy at 8 weeks' gestation.
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