Morbidity associated with sickle cell disease in pregnancy
Margaret S. Villers, MD; Margaret G. Jamison, PhD; Laura M. De Castro, MD; Andra H. James, MD, MPH
The purpose of this study was to identify morbidity that is
associated with sickle cell disease (SCD) in pregnancy.
The Nationwide Inpatient Sample from the Healthcare
Cost and Utilization Project of the Agency for Healthcare Research and
Quality for the years 2000-2003 was queried for all pregnancy-related
discharges with a diagnosis of SCD.
There were 17,952 deliveries (0.1% of the total) to women
with SCD. There were 10 deaths (72.4 per 100,000 deliveries). Cere-
bral vein thrombosis, pneumonia, pyelonephritis, deep venous throm-
bosis, transfusion, postpartum infection, sepsis, and systemic inﬂam-
matory response syndrome were much more common among women
with SCD. They were more likely to undergo cesarean delivery, to ex-
perience pregnancy-related complications (such as gestational hyper-
tension/preeclampsia, eclampsia, abruption, antepartum bleeding, pre-
term labor, and fetal growth restriction), and to have cardiomyopathy or
pulmonary hypertension at the time of delivery.
Women with sickle cell disease are at greater risk for
morbidity in pregnancy than previously estimated.
Key words: morbidity, sickle cell disease
Cite this article as: Villers MS, Jamison MG, De Castro LM, James AH. Morbidity associated with sickle cell disease in pregnancy. Am J Obstet Gynecol 2008;199:
ickle cell disease (SCD), which af-
fects 1 in 600 African Americans, is
the most common hemoglobinopathy in
the United States.
Pregnancy in women
with SCD has been associated with in-
creased incidence of medical- and preg-
of medical advances in obstetrics and
neonatology, more women with SCD
are attempting pregnancy.
rates among pregnant women with SCD
have been falling consistently since the
To date, studies that have focused
on pregnancy-related events in patients
with SCD have been limited by small sam-
ple sizes or to single centers.
Although most studies of SCD in preg-
nancy document risks to the fetus that
include preterm labor and intrauterine
there are limited
data about maternal outcomes. The
women themselves appear to be at risk
for multiple pregnancy-related compli-
cations (such as pregnancy-induced hy-
pertension, preeclampsia, and pyelone-
phritis). However, current research is
contradictory regarding the impact of
Most studies fail to
demonstrate an increased risk of pre-
eclampsia or eclampsia in pregnant
women with SCD.
Most deaths in
pregnant women with SCD are attribut-
able to thromboembolic events,
overall risk of thromboembolic events in
pregnant women with SCD has never
been explored fully. The consequences of
infections, which may result in sepsis,
have not been studied.
The purpose of this study was to iden-
tify the association between SCD and the
occurrence of adverse maternal and fetal
outcomes that are associated with preg-
nancy in the United States.
The Nationwide Inpatient Sample (NIS)
from the Healthcare Cost and Utilization
Project of the Agency for Healthcare Re-
search and Quality for years 2000-2003
was queried for all pregnancy-related
discharges with a diagnosis of SCD. The
NIS contains data from 5-8 million hos-
pital stays from approximately 1000 hos-
pitals and is the largest all-payer inpa-
tient care database in the United States.
The NIS is a 20% stratiﬁed sample of all
discharges and allows for national esti-
mates. Included in this sample are gen-
eral hospitals and academic medical cen-
ters. Rehabilitation hospitals, long-term
hospitals, psychiatric hospitals, and alco-
holism or chemical-dependency treat-
ment facilities are excluded. The hospi-
tals are divided into strata based on
ownership, bed size, teaching status, ur-
ban compared with rural location, and
region. Sampling probabilities are pro-
portional to the number of hospitals in
From the Division of Gynecology and
General Obstetrics, Department of
Obstetrics and Gynecology, Medical
University of South Carolina, Charleston, SC
(Dr Villers); School of Medicine, University
of North Carolina, Chapel Hill, NC (Dr
Jamison); and the Division of Hematology,
Department of Medicine (Dr De Castro) and
the Division of Maternal-Fetal Medicine,
Department of Obstetrics and Gynecology
(Dr James), Duke University Medical
Center, Durham, NC.
This study was presented at the 27th Annual
Clinical Meeting of the Society for Maternal-Fetal
Medicine, San Francisco, CA, Feb. 5-10, 2007.
Received Oct. 18, 2007; accepted April 8,
Reprints: Andra H. James, MD, Duke
University Medical Center, Box 3967, Durham,
NC 27710. firstname.lastname@example.org.
This study was funded in part by grant 5K12-
D043446-03 from the National Institutes of
© 2008 Mosby, Inc. All rights reserved.
For Editors’ Commentary,
see Table of Contents
AUGUST 2008 American Journal of Obstetrics & Gynecology