Pregnant People with Sickle Cell Disease Have Higher Mortality Rate, Study Says

From pregnancy to delivery, and postpartum, birthing people go through physical, emotional, and mental changes that are a lot for anyone to adjust to—but adding a preexisting condition can make a pregnancy high risk and scary.

According to a new study by Johns Hopkins Medicine, there is a substantially increased risk of maternal mortality in pregnant people with sickle cell disease (SCD). It showed the maternal mortality rate for people with SCD was 26 times greater than other pregnant people.

The study looked at why there is such a vast increase in mortality among those with SCD, and what portion of these outcomes are associated with racial disparities. Having a baby should be an exciting time, no one should fear for their lives or the lives of their newborn because of a lack of care due to a medical condition, or race.

What Is Sickle Cell Disease?

Sickle cell disease is a group of inherited red blood cell disorders. Red blood cells contain hemoglobin, a protein that carries oxygen. Healthy red blood cells are round, and they move through small blood vessels to carry oxygen to all parts of the body.

In someone who has SCD, the hemoglobin is abnormal, which causes the red blood cells to become hard and sticky and look like a C-shaped farm tool called a "sickle." The sickle cells die early, which causes a constant shortage of red blood cells. When the cells travel through small blood vessels, they get stuck and clog the blood flow, which causes pain and other serious complications such as infection, acute chest syndrome, and stroke.

SCD is a genetic condition present at birth. It is inherited when a child receives two genes—one from each parent—that code for abnormal hemoglobin. Managing SCD is focused on preventing and treating pain episodes by changing lifestyle behaviors as well as medical screening and interventions to prevent complications.

Risk For Maternal Mortality High in Those With Sickle Cell Disease

In the United States, 90% of people with SCD are Black. According to the study, published February 2 in JAMA Network Open, there is a high number of maternal deaths in SCD pregnancies. Given the prevalence of SCD in the Black community, researchers wanted to study the correlation.

They looked at data collected from 2012–2018, which included more than 5.4 million deliveries. This figure comprised of 3,901 deliveries among pregnant people with SCD and 742,164 deliveries among Black people overall. Black pregnant people made up 84% of those with sickle cell disease who delivered.

The study found maternal mortality was the highest among those with SCD, at 13.3 deaths per 10,000. That’s compared to 1.2 deaths among pregnant Black people without SCD and 0.5 deaths among non-Black, non-SCD patients. The maternal mortality rate for pregnant people with SCD overall was 26 times greater than the national average. 

According to the study, racial disparities explained 28.9% of the increased risk in deliveries to people with SCD. Race was also determined to explain between 40% and 50% of the increased risk for acute kidney failure, intrauterine fetal demise, and eclampsia.

Aside from these numbers specifically for pregnant people with sickle cell disease—there is a wider (and deepening) maternal mortality crisis in the United States. According to a September 2022 report from the Centers for Disease Control and Prevention (CDC), 80% of pregnancy-related deaths were preventable. In fact, the U.S. has the highest maternal mortality rate of all high-income nations. A separate report from the CDC showed maternal mortality rates were the highest among Black birthing people with more than 55 deaths reported for 100,000 births. That rate is almost three times higher than White birthing people.

Why Is The Risk So Much Higher For Black Pregnant People?

"There are shocking disparities in public and private funding for sickle cell disease research,” says Lydia Pecker, M.D., an assistant professor of medicine specializing in hematology at Johns Hopkins University School of Medicine. “Most people with sickle cell disease in the United States are Black and so, there are intersectional disparities affecting research on sickle cell disease and pregnancy with structural racism contributing to what we see."

Dr. Pecker also says there aren't enough sickle cell disease specialists for the number of adults with the disease.

Cassandra Trimnell, the founder and executive director of Sickle Cell 101 says the lack of research and resources plays a large part. "Many living with sickle cell disease, including myself, [are left] scrambling to find vital information and resources specifically created for pregnant individuals living with sickle cell disease,” she says.

This is an issue for many within the sickle cell community. "Couples that are family planning realistically don't have an adequate amount of information. There are too many unknowns, and many see pregnancy and sickle cell disease as a gamble," Trimnell adds.

How Can We Combat These Disproportionate Findings?

Pregnancies among people with SCD are often exposed to the added harms of systemic and interpersonal racism. That may include access to quality healthcare providers and facilities, support systems, and any potential biases from providers who care for pregnant people. These factors contribute to high rates of maternal morbidity among pregnant Black Americans and, as the study shows, this also affects people with SCD. Addressing racial health disparities, and investing in further research of sickle cell disease is imperative to saving birthing people with the disease. 

There are measures being considered in the government that could help. "We need everyone to contact their representatives in state and federal government! Congress can pass the Sickle Cell Disease Treatment Centers Act of 2022 (S 4866 / HR 8855) and other legislation aimed at addressing sickle cell disease care in the USA,” Dr. Pecker says. “Also, federal legislation aimed at addressing the generally grave maternal mortality and morbidity disparities in the USA should include funding to address comprehensive, multidisciplinary care for pregnant people with sickle cell disease."

"Ultimately if we can't educate healthcare providers to better understand sickle cell [disease], how to care for patients, and the resources pregnant women living with sickle cell need for a safe pregnancy, these mortality outcomes are only going to get worse," adds Stephen Boateng, PharmD, the director of research at Sickle Cell 101. 

What Measures Should Pregnant People With SCD Take to Protect Themselves?

Dr. Pecker says pregnant people with sickle cell disease need specialty care, ideally before they become pregnant. "They need to see a sickle cell disease expert and a high-risk obstetrician (maternal-fetal-medicine doctor). If possible, plan to deliver at a center with sickle cell disease and high-risk obstetric expertise," he adds.

The National Alliance for Sickle Cell Centers provides a list of comprehensive SCD centers across the country. "There are emerging fact sheets for people with sickle cell disease and their families making complex reproductive healthcare decisions,” says Dr. Pecker. “We are expecting the Centers for Disease Control [and Prevention] to release patient-facing information to help support preconception planning, prenatal and antenatal care for people with sickle cell disease this year."

Being able to get a specialized team is a privilege that not all people have access to, due to the lack of healthcare options for low-income families, as well as the logistical constraints of doctors that are experienced with SCD. One of the most important ways to protect yourself is self-advocacy and choosing a doctor by who you feel completely supported.  Ask questions, if something doesn’t feel right say something, and know the warning signs during your pregnancy and after.

“It is important for people with SCD who are considering pregnancy to review their health history to better assess potential risks," Trimnell adds. "This should be done in collaboration with their healthcare team.”

Being able to get a specialized team is a privilege that not all people have access to, due to the lack of healthcare options for low-income families, as well as the logistical constraints of doctors that are experienced with SCD. For those who do not have a healthcare team or primary care provider because of financial or other constraints, you can find resources at websites including the Sickle Cell Disease Organization of America and Spark Sickle Cell Change.

One of the most important ways to protect yourself is self-advocacy and choosing a doctor you feel completely supported by.  Ask questions, and if something doesn’t feel right say something.