Surgical and obstetric outcomes in adults with sickle cell disease

Abstract

Background: Sickle cell disease patients are more likely than the general population to undergo surgery and usually do so at a younger age. Female sickle cell disease patients also have special gynecological and obstetric issues related to their disease.

Methods: We collected data through standardized clinical report forms, patient interviews, and medical records from 509 adult sickle cell disease patients. Logistic regression was used to estimate the association between multiple variables and each of the surgery types. We also determined the prevalence and outcomes of pregnancy in 284 women with sickle cell disease in this population.

Results: Almost 50% of patients aged 18-27 years had had a cholecystectomy. Mean corpuscular hemoglobin, total bilirubin, and lactate dehydrogenase were significantly higher in the postcholecystectomy group; 9.5% of 504 individuals had undergone splenectomy. Hematocrit, body mass index, and red blood cell count were significantly higher in the postsplenectomy group. Hip replacement had been performed in 9.2% of individuals, with the prevalence increasing as early as the fourth decade and continuing to increase through the sixth decade of life. A history of pregnancy was present in 190 women (67%). Of 410 pregnancies, only 53.9% resulted in live births, 16.6% were voluntarily terminated, and 29.5% were complicated by miscarriage, still birth, or ectopic implantation.

Conclusions: Sickle cell disease continues to have a strong effect on the mean age for common surgeries and impacts pregnancy outcomes. We conclude that this population has a unique surgical and obstetric history that should be further studied to provide insight into potentially more effective preventive approaches to end-organ damage.

Figure 1

Cumulative prevalence of surgical histories by age. The prevalence of cholecystectomy, splenectomy, and total hip replacement is shown. The frequency of all 3 types of surgeries appeared to plateau as patients aged.

Figure 2

Number of surgeries performed in each age group. The numbers of individuals who had had surgery in different age groups is compared. While a history of cholecystectomy is far more common than history of other surgeries in all groups, above age 38 years, a history of total hip replacement is more common than a history of splenectomy.