A case series describing causes of death in pregnant women with sickle cell disease in a low‐resource setting

Abstract

Sickle cell disease (SCD) is most prevalent in sub-Saharan Africa with 15,000 newborns per year in Ghana. 1,2 Pregnant women with SCD have increased risks of both SCD-specific and pregnancy-related complications, compared to pregnant women without SCD. 3 In a recent meta-analysis, our team reported that SCD in pregnancy increases the risk of maternal death by over 20-fold compared to pregnant women without SCD, in lowand middle-income countries. 4 In sub-Saharan Africa, maternal death rate of SCD is 7%-12%, 3,5,6 without clearly identifiable etiologies.Following our before and after study that demonstrated a 89% relative risk reduction in maternal death in women with SCD with our combined SCD-obstetric team care model, 7 we undertook a medical records and autopsy review of all SCD maternal deaths over a sevenyear period from 2010 to 2016, at the Korle-Bu Teaching hospital (KBTH), in Accra, Ghana. Ethical approval was obtained from the institutional review boards of the College of Health Sciences, University of Ghana and Vanderbilt University Medical Centre. At the KBTH, about 11 000 deliveries occurred annually including 200 women with SCD, and about two-thirds are HbSC. of all maternal deaths in women with SCD at KBTH. All deaths that occurred prior to arrival at the hospital, and those without documented evidence of hemoglobin phenotype, were excluded. Our research team, including obstetricians, hematologists, a pediatrician, anesthesiologists and nurses, adjudicated all cases of SCD maternal deaths to reach a consensus on the cause of death. Pathologists performed autopsies. In this series, we defined maternal death, direct and indirect obstetric cause of death according to WHO ICD-10 classification (WHO 2012). 8 The maternal mortality records, patients' admission and discharge files, and death certificates of all SCD-related maternal deaths within the study period were retrieved and reviewed by the team. Data were summarized as simple descriptive statistics. The Mann-Whitney U-test and Fisher exact test were used to evaluate differences between pregnant women with HbSS versus HbSC. A P-value of < .05 was considered statistically significant. SPSS version 24 (IBM Corp, USA) was used for analysis.HbSS was attributable to stroke. Two-thirds (6/9) of venous thromboembolism occurred in women with HbSC disease. There was no statistically significant difference in VTE as cause of death between HbSC and HbSS (P 5 .69).In our case series of maternal death among pregnant women with SCD at a single centre in Accra, Ghana, the main causes of death were related to SCD complications. Most maternal deaths were preceded by acute pain episodes; the commonest cause of death was ACS (86%).The number of deaths from venous thromboembolism was similar in 0 | V C 2018 Wiley Periodicals, Inc.wileyonlinelibrary.com/journal/ajh Am J Hematol. ;1-3.[8] Organization WH. The WHO application of ICD-10 to deaths during pregnancy, childbirth and puerperium: ICD MM; 2012. SUPPORTING INFORMATIONAdditional Supporting Information may be found on...

Third trimester and early postpartum period of pregnancy have the greatest risk for ACS in women with SCD

“…The definition of acute pain episode as in our previous publications was maintained. 3,4,6 During the postpartum period, "if the patient was able to judge whether the pain was of the type usually associated with crisis and reported such pain, this was considered appropriate evidence of an acute pain episode. 5 " The total number of acute pain episodes requiring hospitalization was confirmed with a chart review.…”

“…The definition of ACS as in our previous publications was maintained. 3,4,6 The research obstetricians and hematologists adjudicated all participants admitted for suspected ACS events. A consensus agreement was reached to determine the diagnosis of ACS.…”

Multidisciplinary care results in similar maternal and perinatal mortality rates for women with and without SCD in a low‐resource setting

“…During the month‐long training in Nashville the team also systematically reviewed the literature to conduct a pooled analysis on maternal and perinatal outcomes in pregnancies associated with SCD . Also, the team conducted a combined retrospective (January 2010 to April 2015) and prospective (May 2015 to December 2016) case series of all maternal deaths in women with SCD at KBTH . This process highlighted service delivery, failures and challenges to delivering improved care while allowing for providers to develop new, acceptable and feasible solutions. Creation of a multidisciplinary SCD obstetric team : In 2015, the clinic expanded to a multidisciplinary team including 3 local hematologists, 2 midwives, 2 laboratory scientists, a pediatrician, a dual‐certified anesthesiologist and pulmonologist, including SCD experts from Vanderbilt University and an obstetrician and gynecologist from Duke University Medical Center.…”

“…In a systematic review and meta‐analysis our team demonstrated that SCD is associated with an increased risk of maternal death compared to women without SCD in low‐resource settings (odds ratio [OR] 22.8 [95% CI 14.7‐35.5], P < .001) . Acute chest syndrome (ACS) is a major cause of death among pregnant women with SCD . Other adverse maternal outcomes in women with SCD include hypertensive disorders of pregnancy (preeclampsia, eclampsia), severe anemia, urinary tract infections, and increased odds of both antenatal and postnatal acute pain episodes .…”

Identifying Clinical and Research Priorities in Sickle Cell Lung Disease. An Official American Thoracic Society Workshop Report

“…Pregnancy and the postpartum period are associated with increased ACS risk. ACS is the most common cause of acute respiratory failure and death in pregnant or postpartum SCD patients in low-resource settings, with ACS responsible for >60% of the deaths in pregnant women with SCD in this population ( 31 , 51 ). In Ghana, a multidisciplinary approach using a combined obstetric and hematology team was effective in reducing ACS incidence and all-cause maternal mortality ( 52 ).…”