Pregnant and Living with Sickle Cell Disease: A Push for Better Outcomes

Wemi Found, a mother of two who has sickle cell disease (SCD), barely survived her first pregnancy. The Toronto resident was elated to be pregnant; it had taken nearly a year to conceive during which she also experienced a devastating miscarriage.

The first trimester of her pregnancy was a breeze, Found recalled during a recent NHLBI-funded workshop, Reproductive Health in Individuals with Sickle Cell Disease. But as the second trimester rolled around, it was like a switch flipped in her body and the pain crises started. Like many with SCD, Found likened those episodes to having someone relentlessly hammering away at her joints. Her doctor prescribed morphine, but “taking it was extremely anxiety-inducing because all the literature out there is about babies who are addicted to drugs in utero,” she said. 

For the next few months of pregnancy, Found was hospitalized every few weeks. By month five the digital marketing manager stopped working because the pain was so intense. Her blood pressure had also increased to such high levels – a dangerous condition during pregnancy called preeclampsia – that even blood pressure medication did not stave off the need for emergency hospital visits.

“At 32 weeks, I couldn’t breathe,” she remembered. “It was so bad I would walk up two stairs at a time and have to catch my breath.”

Finally, six weeks before her due date, Found was admitted to the hospital and was told she wasn’t leaving without delivering her baby. The reason: in addition to the preeclampsia, she had mild heart failure. Doctors had to induce labor, and Found ultimately delivered her baby through emergency caesarian section.

“It was a traumatic delivery,” she said. “I didn’t get to meet my son until three hours after he was born.” 

Found and her husband quickly decided they’d had enough – they were done growing their family. But five years later, with a better care plan in place developed by Found’s medical team, the couple felt ready to try again.  Just six weeks after she became pregnant with her second baby, she began taking low-dose aspirin twice daily and getting monthly exchange blood transfusions that involved replacing her sickled red blood cells with new, healthy ones. 

“This pregnancy was like night and day,” Found said. “I had no adverse reactions and delivered a healthy baby girl at full-term.” Today, both of her children, now ages 7 and 2, are thriving. 

A formidable challenge

Experts say Found’s story highlights the extraordinary complexity of the problem for pregnant people living with SCD – and the critical importance of receiving proper, specialized care as early as possible.

This is especially true now that medical advances in care and disease-modifying therapies have helped many people living with SCD survive well through their reproductive years. For parents-to-be, that means awareness is key. Individuals with SCD are at higher risk than the general population for preeclampsia, as Found discovered; but those with preeclampsia can go on to develop a condition called eclampsia, which can lead to seizures and even coma. People with SCD are also at higher risk for sepsis and blood clots. And there are risks for the fetus, such as lower-than-normal growth in the womb, preterm delivery, and stillbirth.

 A 2023 NHLBI-funded study investigated adverse pregnancy outcomes among individuals living with SCD, comparing results among Black people with SCD with those of Black people without SCD and a control non-Black population. 

What they found was striking. With data from more than five million pregnancies, the study revealed that the maternal mortality rate among pregnant people with SCD was 26 times greater than in the non-Black control group and more than 10 times greater than among Black pregnant people without SCD. Compared with the control group, people with SCD also had higher odds of severe conditions, especially serious blot clots or blockages to the brain.

The findings highlighted how deeply-rooted the problem has become in the last 20 years, despite advancements in both treatment of SCD and high-risk obstetric care. For example, a 2007 study, which used data from 2000 to 2003, showed the maternal mortality rate among people with SCD was 7.2 deaths per 10,000 births, while the 2023 study showed rates of 13.3 deaths per 10,000 deliveries. 

Scientific experts said they are not sure what is behind the worsening problem, and that to fully understand, more studies will be needed.  But some things are becoming clearer with the help of NHLBI-funded research. “The hope,” said Julie Panepinto, M.D., M.S.P.H., director of NHLBI’s Division of Blood Diseases and Resources, “is that more scientific discoveries can lead to better treatment options to ensure healthy and successful pregnancies.” 

New insights   

One research study supported by NHLBI compared the rates of poor pregnancy results among individuals living with SCD to those without SCD, but who had iron deficiency during pregnancy, known as prenatal anemia. (Anemia is a known hallmark of most SCD pregnancies.) The study found that the risks associated with prenatal anemia and SCD were similar to risks associated with many problem pregnancies. This suggests that treating prenatal anemia might help decrease the odds of severe pregnancy-related issues in pregnant individuals with SCD.

Another NHLBI-funded study investigated a condition known as thrombosis, which occurs when blood flow is restricted to a certain area in the body due to a blood clot. Pregnancy and SCD are known risk factors for thrombosis, but researchers did not know if being pregnant and living with SCD increased that risk. Their finding: pregnant people with SCD had an eight to 11 times higher chance of developing thrombosis, compared to pregnant people without SCD. The authors identified factors such as high blood pressure and a history of blood clots as risk factors for thrombosis during pregnancy.

While NHLBI and other organizations continue to fund work like this to advance SCD research, experts say improvement in patient care will require a team effort by scientists and physicians in multiple disciplines, as well as patient education.

Heather Avant, a mother from Dallas who also shared her pregnancy story at the NHLBI workshop, underscored the critical need for such education.  “There was so much trauma [in my pregnancy],” she said. “How do we reduce that? As a sickle cell patient, I knew about [my disease] but I didn’t know about having a baby. How do we provide more education to patients who are wanting to be parents?”

Panepinto said it’s a question that often takes gatherings like the workshop to highlight – and, ultimately, try to answer. “Along with sharing individuals’ stories about pregnancy outcomes, bringing together experts raises awareness of these issues and stimulates discussion,” she said.

In the end, she added, the goal is to advance scientific discovery – and improve the health of individuals living with SCD.

Resources: 

To learn about sickle cell disease, visit https://www.nhlbi.nih.gov/health/sickle-cell-disease.  

For a fact sheet about sickle cell disease, visit https://www.nhlbi.nih.gov/resources/sickle-cell-disease-fact-sheet.