Pregnancy outcome in sickle cell disease in the UK: have things changed over three decades?

Abstract

Sickle cell disease prevalence, albeit low, is increasing in the United Kingdom, from estimates of 5,000 in 1986 to 12,000 in 2010.1 2 Pregnancies in sickle cell disease (SCD) have, in some respects, shown worsening outcomes over time,3 4 though not, apparently, in some recent single-hospital series.5 6 7 Methods We ascertained outcomes of 206 pregnancies complicated by SCD from 11 hospitals in the UK over the period 2000 to 2004. Trend analysis compared these data with data from two previous surveys covering the 1980s and 1990s, using Z scores to assess trends in mortality rates, and the chi-square test for trends in complication rates. Results The perinatal mortality increased from 48 per 1000 in the first survey to 68 per 1000 in the latest survey. However, maternal mortality decreased by 3% (95% CI -1% to 7%), from 3.3% to 0.48% There was significant decline in severe sickle cell crises, pulmonary complications and urinary tract infections, but no reduction in severe pre-eclampsia. There was a significant decline in the use of blood transfusions in pregnancy over this time and significant increases in maternal age and in delivery by emergency caesarean section. Discussion While the UK perinatal mortality rate has fallen since 1980, that in SCD pregnancies has risen, with the rate now 9 times the national rate (68 compared with 7.6 per 1,000) Though our 2000 to 2004 survey ascertained one maternal death, CMACE reported 4 deaths during this period, indicating a continuing high maternal mortality in SCD in the UK.8