Sometimes progress comes by way of low-tech changes. Investigators at the Korle-Bu Teaching Hospital in Accra, Ghana, have demonstrated exactly that. Their new study looks at maternal death rates before and after implementation of a comprehensive clinical team–with hematologists, obstetricians, trained nurses and midwives, lung specialists and others–for women with sickle cell disease before, during and after childbirth.
Prior to the program’s implementation, maternal mortality for women with sickle cell disease exceeded 9% at the teaching hospital in Accra. With team-based care, the death rate fell to 1%. These findings were presented at the annual meeting of the American Society of Hematology (ASH) by Dr. Eugenia Vicky Asare, a hematologist based at the Korle-Bu hospital. The Ghanaian group collaborates with researchers at the Meharry Sickle Cell Center at Vanderbilt University in Nashville, Tenn.
The mortality rate for women with sickle cell disease in parts of Africa is “shockingly high,” said Dr. Charles Abrams, a hematologist at the University of Pennsylvania during a pre-conference phone call. He is currently president of ASH. “This study shows that doing simple things can make a big change in outcomes,” he stated. The results have implications for women’s health around the globe.
Dr. Samuel Oppong practices obstetrics and gynecology in Accra, where he belongs to the faculty of the School of Medicine and Dentistry, University of Ghana. He’s an author of the ASH report. The Korle-Bu Teaching Hospital is the largest in Ghana, he said in a Skype interview. The high-volume hospital provides care to approximately 11,000 pregnant women each year. Among those, between 200 and 250 have sickle cell disease or a related abnormality of hemoglobin, like SC disease or a severe sickle-thalassemia variant. “We have a great deal of experience with this,” he said.
Maternal deaths among women with sickle cell disease are quite common, Oppong said. “Without modern care, the death rate could be as high as 20%,” he suggested. (Most published reports indicate a maternal mortality rate closer to 10%; the rate in regions without modern medical facilities is unknown.) Women with sickling hemoglobin are prone to develop severe vascular, heart and lung problems during pregnancy, Oppong explained. “They’re already anemic,” Oppong considered. “With the slightest amount of blood loss, the impact tends to be more severe. They may also develop hypertension in pregnancy, and that can have a profound effect.”
To address this clinical problem, in 2011 Oppong revived a defunct clinic for pregnant women with sickle cell anemia at the teaching hospital in Accra. Since then, he and colleagues put together a multidisciplinary team with two obstetricians, three hematologists, a respiratory physiologist who serves also as an anesthesiologist, trained nurses and midwives, and laboratory technicians.
Clinic at the Korle-Bu Teaching Hospital in Accra, Ghana (courtesy of the Public Relations... [+]
The paper evaluates outcomes in pregnant women with sickle cell disease before implementation of the program at the Korle-Bu Teaching Hospital. Maternal deaths were defined as those that occurred during pregnancy, delivery and within 6 weeks after delivery. The study’s main limitation is that it is a non-randomized, observational analysis.
In the pre-intervention group, the number of maternal deaths was 15, out of 158 pregnant women with sickle cell disease, amounting to a 9.5% mortality rate. In the year after the interdisciplinary team took over, from May 2015 to May 2016, there was only 1 maternal death among 90 women in the program. The woman who died developed acute chest syndrome, and had “massive bilateral” clots in her lungs found at autopsy, Oppong said.
Perinatal deaths were also reduced by the changed approach to maternity care: the newborn mortality rate fell from 6.1% to 2.3%. This means that the sickle cell program for women was associated with a 63% reduction in newborn deaths.
The multidisciplinary program assigns hospitalized pregnant women with sickle cell disease to one of two units for closer observation. (Previously women were assigned rooms throughout the large hospital.) Clinicians monitor the women’s oxygen levels by pulse oximetry, intermittently, and provided supplemental oxygen–to reduce hemoglobin’s sickling–if the oxygen level drops by over 2% or falls below a certain level. They give intravenous fluid to women who are dehydrated, and transfuse based on clinical need: in case of (more than the usual) severe anemia, in women experiencing painful crisis or acute syndrome, or with hemorrhage after delivery. They use balloons, like incentive spirometers, to promote lung function.
“Without question, any time you can see such a dramatic reduction in maternal mortality rates, it’s a testament to better care,” said Dr. Alexis Thompson in a phone interview. She, who was not involved in the study, is a professor of Pediatrics at the Northwestern University Feinberg School of Medicine, where she heads the program in hematology. “The study underscores the value of providing comprehensive care in sickle cell disease.”
The mortality rate for women with sickle cell disease is approximately 10 times greater than for women of average risk in the United States, Thompson said. Death isn’t the only complication of pregnancy for which they’re at risk. “They get heart and lung complications, and infections,” she said. Lung problems include clots, pneumonia and acute chest syndrome. They develop vascular problems and may suffer strokes. Preterm births are more frequent. Painful sickle crises in pregnancy can be difficult to manage, she added.
Obstetrical guidelines support that women with sickle cell disease be seen by doctors with experience in managing patients with hemoglobin disorders, or in otherwise high-risk obstetrics practices, Thompson said. Not all pregnant patients have access to such expert practices, she acknowledged. “There clearly is room for improvement.”
The U.S. maternal death rate is rising in general, whereas in other parts of the world it is falling, Thompson noted. She referred to a recent New York Times article about this trend. “The rise in the maternal death rate is disproportionately seen in African American women,” she said. “What I don’t know is if we’re seeing an increase in maternal deaths with sickle cell that’s going up proportionately or not.”
“This study shows that a lot of clinical benefit comes from being more attentive, recognizing complications of sickle cell disease early and addressing those,” Thompson said. “Having a hematologist or other subspecialist, such as a pulmonologist, who is accustomed to managing sickle cell disease can improve care. The same is true for managing blood pressure and other complications of disease," she said. “When obstetricians become aware of these conditions and intervene, independently or by seeking out experts, the outcomes can be improved.”
A 2015 review found that mortality among affected women varies considerably. In general, the highest maternal death rates occur in women who inherit hemoglobin S from both parents, as compared to those who inherit a mix of hemoglobin S and C traits. In countries with higher income levels, maternal and perinatal outcomes exceed those in low-income regions. Overall, maternal deaths among women with SS hemoglobin are estimated to exceed that of average-risk women by six-fold or more.
Ghana has a national policy of checking pregnant women for sickle cell disease. The initial screen is usually done by a sickling test, Oppong said. Still, not everyone gets tested, and not all who screen positively for a sickling disorder get further evaluation to discern among clinically similar disorders of hemoglobin, as would typically proceed in the United States, by electrophoresis, HPLC or DNA evaluation. Women with confirmed sickling conditions, also known as hemoglobinopathies, get referred to the clinic at the teaching hospital.
“Given the particular challenges in caring for pregnant women with sickle cell disease, hematologists need be involved,” Oppong said. “We have shown that implementing simple practice changes can lower the death rate. If others adapt our strategy, they might make a significant impact when it comes to pregnant women with sickle cell disease elsewhere in the world.”
