Mortality Plummets in Pregnant Women With Sickle Cell Disease

Roxanne Nelson, BSN, RN

December 05, 2016

SAN DIEGO — A multidisciplinary approach dramatically reduced mortality in pregnant women with sickle cell disease (SCD), after being in place for only 13 months.

The Korle-Bu Teaching Hospital, a national referral center in Accra, Ghana, treats approximately 250 pregnant women with SCD every year; up to 12% die during pregnancy and after childbirth.

But after the intervention, there was an 89% decline in maternal deaths (9.5% vs 1.1%) along with a 62% reduction in perinatal mortality (60.8% vs 23% per 1000 total births).

The findings were presented here at the American Society of Hematology (ASH) 2016 Annual Meeting (abstract 1017).

"Our hypothesis was that implementing a multidisciplinary team that included a hematologist for care of pregnant women with SCD would significantly decrease maternal mortality," said lead author Eugenia Vicky Naa Kwarley Asare, MBChB, BSc, from the Ghana Institute of Clinical Genetics, Korle-Bu, and the Korle­Bu Teaching Hospital in Accra, Ghana.

More than 300,000 infants with SCD are born every year, and about 79% are in sub-Saharan Africa, explained Dr Asare.

"Sickle cell disease has acute and chronic complications, and to manage it well, especially in the context of pregnancy and childbirth, you need to have a number of specialists on board, including a hematologist," she said.

The estimated maternal mortality ratio of women with and without sickle cell disease in Ghana is 8300 and 690 per 100,000 live births, respectively. In comparison, the maternal mortality ratio in the US general population is 14 per 100,000 live births.

In 2015, a multidisciplinary obstetric team dedicated to SCD was formed at Korle-Bu, composed of obstetricians, hematologists, pulmonologists, and nurses.

In a before-and-after study design, Dr Asare and colleagues tested the hypothesis that the implementation of a multidisciplinary team would significantly decrease maternal mortality among women with SCD.

Striking Results

The preintervention period was January 2014 to April 2015, and the postintervention period was May 2015 to May 2016. During the intervention period, members of the multidisciplinary team evaluated women at enrollment, during outpatient visits, and during acute illnesses, and simple protocols were put into place for preventing and treating acute chest syndrome.

A total of 158 deliveries by women with SCD were evaluated in the preintervention period, and 91 were evaluated after implementation.

The median gestational age at enrollment was 24 (range, 7 to 40) weeks and median gestational age at delivery was 38 (range, 26 to 41) weeks.

Perinatal mortality rates declined between the preintervention and postintervention periods: from 74.3 per 1000 total births (11/148 × 1000) to 54.9 per 1000 total births (5/91 × 1000), respectively.

Maternal mortality also significantly declined, from 9.7% (15 of 154) and 1.1% (1 of 91) of total deliveries, and the maternal mortality ratios before and after intervention were 10,949 (15/137) and 1163 (1/86) per 100,000 live births.

Before the intervention, causes of death included cardiopulmonary disease (60.0%), preeclampsia (6.67%), acute kidney injury (6.67%), severe anemia (20.0%), and hypovolemic shock (6.67%).

In contrast, the only death after the intervention was an autopsy-confirmed massive pulmonary embolism that occurred 4 days postpartum.

An additional benefit was that the rate of cesarean deliveries declined, said Dr Asare. "There was a 12.9% decrease in cesarean deliveries after the intervention."

Their next step is to implement a similar program at 3 major health facilities in Accra, which collectively see about 200 women with SCD each year. "We would like to establish a sickle cell disease center of excellence in Accra, Ghana," she concluded.

Intervention Is Reproducible

Approached for comment, Kim Smith-Whitley, MD, clinical director, Division of Hematology and director of the Comprehensive Sickle Cell Center at The Children's Hospital of Philadelphia, Pennsylvania, noted that it is incredible how "they reduced the mortality that dramatically in only 13 months."

"I think it is doable in other low- and middle-resource countries," she told Medscape Medical News.

"What is fascinating about that project is that they are going to teach us how to do things with limited resources, and that can benefit individuals who live far from academic or comprehensive sickle cell centers," Dr. Smith-Whitley said. "I also wonder if we can get local hospitals to form some sort of collaborative."

SCD is relatively uncommon in the United States, while in Africa it is far more prevalent. "They have such large populations of sickle cell disease," she pointed out, "So they are going to end up teaching us. They can teach us what we may need to do to be more practical."

The study was funded partly by Intramural Funds (Office of Research and Innovation), University of Ghana; Vanderbilt University Medical Center Gift Funds; Doris Duke Charitable Foundation; Burroughs Wellcome Foundation; Phillips Family Donation; Aaron Ardoin Foundation for Sickle Cell Anemia; and endowed chair funds from Vanderbilt University School of Medicine. Dr Asare and most of the coauthors report receiving funding from Vanderbilt University Medical Center Gift Funds:Research Funding; Intramural University of Ghana Research fund: Research Funding. Dr Smith-Whitley is an advisory committee member with Pfizer.

American Society of Hematology (ASH) 2016 Annual Meeting. Abstract 1017. Presented December 4, 2016.

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