Surveillance of sickle cell disease in pregnancy

Summary

There are some historical data, mostly from outside the UK, showing a high incidence of maternal and fetal complications in sickle cell disease (SCD), but no contemporary or recent prospective data from the UK. The numbers of deliveries in women with SCD has increased markedly over recent years, from 25-30 deliveries across the whole UK in the 1970s, to the current situation of approximately 150-250 deliveries per year. There is a lack of consensus about the best management strategies for optimum care of these women, although it is clear that good committed obstetric care is of vital importance. The lack of knowledge about incidence of pregnancy in affected women, makes it difficult to plan services, to plan optimal care, or in the long term to plan further trials into best practice. This study collected data about the incidence across the UK and identified patterns of management practice. There were 108 confirmed cases of sickle cell disease. The majority of women were from African (68%) or Caribbean (20%) background. Patients were anaemic at booking with haemoglobin levels of 5.5-12.5g/dl (median 9.3g/dl). Problems were commonly reported during the pregnancy and 57 women (52%) experienced a painful sickle cell crisis during pregnancy, 14/57 were admitted to hospital with painful crisis, 8 on a single occasion, 5 patients on 2 occasions and 1 women on 4 occasions. Seven women (6%) had acute chest syndrome, a life threatening complication of sickle cell disease characterised by pulmonary signs and symptoms and infiltration on the Chest X-Ray. Twenty-six women (24%) received ante-natal blood transfusion.Four miscarriages/terminations, 3 stillbirths and 94 live births were reported. Eleven women had not delivered at the time of data completion, so outcome data on this pregnancy is not available. 24% of women were admitted to ITU in the peri-partum period. 15% of women reported a sickle cell crisis in the 6 weeks following delivery. These results results suggest that pregnancy in women with sickle cell disease is a time of significant morbidity. There were no reports of maternal mortality, but morbidity was high with over half of women reporting antenatal painful crises and a quarter of women admitted to intensive care after delivery. The high incidence of complications and high numbers of women receiving blood transfusion in this study suggest that the risks and benefits of prophylactic antenatal transfusion should be further investigated.