Surgical and Obstetric Outcomes in an Adult Population with Sickle
Cell Disease
Soheir Adam, MD, MRCPath 1, Jude Jonassaint, RN1, Hillary Krugar, BS1, Melanie
Kail, MS1, Eugene P. Orringer, MD2, James J. Eckman,, MD3 Allison Ashley-Koch1,
PhD, Marilyn J. Telen, MD1, Laura M. De Castro, MD1
1
Department of Medicine, Duke University, Durham, North Carolina, 27710,
Department of Medicine, University of North Carolina, Chapel Hill, North Carolina,
27599, and 3Department of Medicine, Emory University Medical Center, Atlanta,
Georgia, 30303.
2
Correspondence:
Soheir Adam, MD, MRCPath
Duke University Medical Center
Box 3939
Durham, NC 27710
TEL: 919 684-0594
FAX: 919 681-6174
Email: soheir.adam@duke.edu
Acknowledgments
*This work was supported by grants HL68959 from the National Heart, Lung and
Blood Institute, National Institutes of Health (NIH). LDC is the Sickle Cell Scholar
for the Duke-UNC Comprehensive Sickle Cell Center (HL070769). SA is a visiting
scholar and an Assistant Professor at King AbdulAziz University, Jeddah, Saudi
Arabia
Abstract
Surgical procedures in sickle cell disease (SCD) patients have been described as more
frequent, and occurring at an earlier age, when compared with the general population.
Likewise, obstetric events in females with SCD are associated with distinct
morbidities affecting both the patient and the offspring, including spontaneous
abortion, fetal growth retardation, and venous thrombosis. We therefore reviewed the
surgical and obstetrical outcomes of SCD in adult subjects. We hypothesized that 1.
SCD-related comorbidities would be associated with the type of surgery and age at
which surgery was performed and that 2.The fetal and maternal complications in
pregnancy, are part of the clinical spectrum of sickle cell disease in females.
Over a period of five years, 541 unrelated adult patients with HbSS or HbSß0thal were
enrolled in a prospective study designed to identify genetic factors associated with
clinical outcomes in SCD. Medical history was obtained by both patient interviews
and review of medical records, using standardized case report forms, and information
was then entered into our Pedigene® database. Five hundred and nine (509) subjects
were included in this analysis. For these patients, data was available on surgery and
obstetric outcomes, as well as possible predictor variables, including age, gender, total
bilirubin, complete blood count (CBC), absolute reticulocyte count, LDH, blood
pressure (BP), use of BP medication, and body mass index (BMI). Contingency
tables were used to provide descriptive frequencies of the surgical and obstetric
outcomes. Logistic regression was used to identify statistically significant
associations between outcomes and possible predictor variables. Cholecystectomy
was the most commonly reported surgery in this population (291/509, 57.17 %).
Almost half (47.5%) of all patients who had cholecystectomy , had it by the age of 47.
The MCV and the total bilirubin were found to be significantly higher for those who
had had cholecystectomy than those without cholecystectomy (p=0.0047, and 0.0084,
respectively). A history of surgical splenectomy was reported in 48 (9.52%) study
subjects, 9.3% of them by theage of 47. The hematocrit was found to be significantly
higher in the post- splenectomy group (26.3 vs 24.2) (p =0.0168). Individuals with a
higher body mass index (BMI) were also more likely to have had surgical
splenectomy (p = 0.0082). Hip replacement had been performed in 47/ 509 (9.23%)
individuals, and 7.8% reported having it by the age of 57. Therefore, cholecystectomy
was 5 times more common than other surgeries and occured at a much younger age.
Of the 284 females included in this analysis, 190 (66.9 %) had a history of pregnancy,
and a total of 410 pregnancies were reported resulting in an average pregnancy rate of
2.15. The mean age at first delivery was 23.7 years. Out of 410 reported pregnancies,
221 resulted in live births (53.9%) and 121 (29.51%) ended in miscarriages, stillbirths
or ectopic implantations .There were 68 (16.59%) voluntary abortions.
Our data provides current information about the surgical and obstetric history of
adults with SCD. We conclude that this population’s unique surgical and obstetrical
history should be used to provide insight into management guidelines and to develop
more efficient preventive approaches approaches to the medical, surgical and
obstetrical complications of SCD.