Pregnancy in Women with Sickle Cell Disease

Introduction

Sickle cell disease (SCD), characterized by recurrent crisis due to vaso-occlusion, results in acute morbid outcomes and cumulative organ damage. Acute hemolysis typically occurs in times of stress such as pregnancy. Although advances in the management of SCD complications have allowed most women with SCD to reach their reproductive age (Telfer et al. Haematologica.2007), pregnancies can be complicated; increased risks of preeclampsia, maternal death, stillbirths, preterm deliveries and small-for-gestational-age newborns have been reported (Oteng-Ntim et al. BJH.2014).

The objective of our study is to assess maternal-fetal complications and vaso-occlusive crisis during pregnancy.

Methods

This is a retrospective chart review of twelve pregnancies in nine women (ages 22-34) with sickle cell disease over a 10 year period at a large urban hospital. All women had a documented history of sickle cell disease (HbSS, HbSC or HbSBthal), and met the inclusion criteria of consistent follow up with hematology for a 3 year span encompassing the year prior through the year after pregnancy. Women were excluded if they had a miscarriage, or were non-compliant with care. Number of crisis, method of delivery, fetal complications were collected.

Results

Of the twelve pregnancies, ten (83%) occurred in HbSS disease patients while two (17%) in HbSC patients. Ten out of twelve pregnancies had at least one crisis in the year prior pregnancy (baseline crisis); of these 60% had the same number of crisis during pregnancy. Interestingly, only 33% of the pregnancies had increase from their baseline crisis in the year after pregnancy.

Three pregnancies received prophylaxis exchange after a crisis admission early in the pregnancy and were noted to not have any further crisis. Of nine remaining pregnancies, seven required urgent red cell exchange; two for crisis/preeclampsia, three for acute chest syndrome and two for vaso-occlusive crisis.

Overall, 58% of pregnancies had c-section delivery; highest in women that did not receive exchange and lowest in women with prophylactic exchange (33%). Pregnancies that had crisis requiring urgent exchange had 50% rate of c-section. Three preterm deliveries and all fetal complications were noted in patients who had required urgent exchange. Fetal complications included intra-uterine growth retardation (17%), NICU admission (25%), and one fetal demise.

Conclusion

MedStar Washington Hospital Center is an urban hospital that cares for a large population of sickle cell patients however, due to sporadic follow up in our sickle cell population; data regarding maternal and fetal outcomes in pregnant patients is limited.

All pregnancies with baseline crisis will have atleast the same number of crisis during pregnancy. Rates of C-section were noted to be much less (33%) in pregnancies receiving prophylaxis exchange, which is near national average (32.6%) in healthy women (CDC, 2013). Fetal complications were also more prevalent in pregnancies with crisis requiring urgent exchange. Therefore, we conclude that maternal complications such as acute crisis requiring hospitalizations and C-section as delivery modality, and fetal complications can be prevented with prophylactic exchange.