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Sickle cell disease increases mortality risk during pregnancy
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The risk for death during or after pregnancy is six times higher among women with sickle cell disease compared with pregnant women without the disease, according to results of a systematic review and meta-analysis.
Women with sickle cell disease also were more likely to experience preeclampsia, stillbirth and preterm delivery, results showed.
“While we know that women with sickle cell disease will have high-risk pregnancies, we have lacked the evidence that would allow us to confidently tell these patients how likely they are to experience one complication over the other,” study researcher Eugene Oteng-Ntim, MD, of the Guy’s and St. Thomas’ NHS Foundation Trust, London, said in a press release. “This reality makes it difficult for us as care providers to properly counsel our sickle cell patients considering pregnancy.”
Researchers previously have been unable to define risks associated with pregnancy for women with sickle cell disease due to heterogeneity in the pathophysiology of the disease, according to the study background.
Oteng-Ntim and colleagues examined data from 21 published studies that compared outcomes among pregnant women with (n = 26,349) and without sickle cell disease (n = 26,151,746). Of the women with sickle cell disease, 1,276 had the HbSS genotype and 279 had the HbSC genotype. The other 24,794 women had unreported genotypes.
Researchers observed an increased risk for maternal mortality in women with the HbSS genotype (RR = 5.98; 95% CI, 1.94-18.44) and nonspecified sickle cell disease (RR = 18.51; 95% CI, 8.63-39.72) compared with women without sickle cell disease. Only one death was reported among women with the HbSC genotype.
The risk for preeclampsia doubled in women with the HbSC genotype (RR = 2.03; 95% CI, 1.17-3.54) and nonspecified sickle cell disease (RR = 2.06; 95% CI, 1.49-2.85) compared with women without sickle cell disease. Women with the HbSS genotype faced increased risks for preeclampsia (RR = 2.43; 95% CI, 1.75-3.39) and eclampsia (RR = 4.89; 95% CI, 1.97-12.16).
The risk for stillbirth increased fourfold among women with the HbSS genotype (RR = 3.94; 95% CI, 2.6-5.96) and nearly twofold among women with the HbSC genotype (RR = 1.78; 95% CI, 1.05-3.02).
Women with the HbSS genotype also faced significantly increased risks for neonatal death (RR = 2.68; 95% CI, 1.49-4.82) and preterm delivery (RR = 2.21; 95% CI, 1.47-3.31).
The risk for infants born small for gestational age was significantly greater for women with the HbSS genotype (RR = 3.72; 95% CI, 2.32-5.98) and the HbSC genotype (RR = 1.98; 95% CI, 1.04-3.77).
Thirteen of the studies were conducted in high-income countries ($30,000 or higher per capita income), whereas the other nine studies were conducted in low- to median-income countries.
The risks for preeclampsia, preterm delivery and small for gestational age infants persisted in women with sickle cell disease regardless of their location. However, women with sickle cell disease who lived in countries with a higher gross national income faced less significant risks for maternal mortality (OR = 0.15; 95% CI, 0.02-0.86) and stillbirth (OR = 0.28; 95% CI, 0.14-0.55).
“Even in developed countries with advanced care, there continues to be a much higher maternal mortality rate in women with sickle cell disease compared to the general population, strongly suggesting that more work must be done to improve outcomes for these patients and their families,” Oteng-Ntim said. “By improving care providers’ ability to more accurately predict adverse outcomes, this analysis is a first step toward improving universal care for all who suffer from this disease.” – by Cameron Kelsall
Disclosure: Oteng-Ntim reports no relevant financial disclosures. One researcher reports advisory roles with AesRx and Novartis.
Perspective
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Gregory M. Vercellotti, MD, FACP
The 2014 National Heart, Lung, and Blood Institute’s 2014 expert panel report titled Evidence-Based Management of Sickle Cell Disease determined that women with SCD are more likely to experience preeclampsia, small infants for gestational age, pre-term delivery, stillbirth, venous thromboembolism, pain crises, infections and maternal mortality during pregnancy. Those with pulmonary hypertension may have even more complications. The recommendations include having a reproductive life plan, screening for allo-antibodies, testing partners for SCD or thalassemia status, and realizing that pregnancy in women with SCD is considered high risk. It is suggested that additional fetal surveillance is required during a pregnancy.
Oteng-Ntim and colleagues document these findings in more than 26,000 women with SCD. But what can be done to make pregnancy safer in women with SCD? Obviously, access to care is the first step, and knowledge of sickle cell disease among primary care, obstetrical and interdisciplinary health care personnel is required. Adequate prenatal care must include ultrasounds to assess fetal-placental function; addressing folate, nutrition and iron; using aspirin to minimize pre-eclampsia; controlling nausea and ensuring adequate fluid intake.
A plan for opiate use, assessing allo-sensitization, treating pain crises and use of transfusions — especially in the third trimester — need to be addressed. VTE prophylaxis at time of delivery and post-partum may decrease fatal pulmonary emboli.
Clearly, there is an enormous need for more basic and clinical studies in SCD to understand the underlying pathophysiology and best practices to improve outcomes. All of these risks are especially elevated in low- and middle-income countries, amplifying the urgency for this research.
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