BY JENNIFER FINK

If you have sickle cell disease (SCD), pain often is a part of your life.

That’s because your normally round, red blood cells can change into stiff, curved, “sickle” shapes that get stuck in small blood vessels, drastically reducing the flow of oxygen to your organs and tissues. When that happens, you experience excruciating pain, a sensation that some describe as “millions of needles going through every cell in your body.”

You can’t work through that kind of pain. You need help—and your organs desperately need oxygen.

Illustration courtesy of Shutterstock

What happens next largely depends on your access to excellent medical care. If you’re already being treated by specialists in sickle cell disease, you’ll likely receive prompt care. If not, you may have to visit several emergency rooms before finding one that understands the problem and can provide necessary care. Too often, people experiencing an SCD crisis and in need of pain medication may be misunderstood and inappropriately perceived as “drug-seeking.”

For far too long, the second scenario has been the norm. Although SCD affects approximately 100,000 Americans, many people with SCD do not receive recommended medical care. A 2019 study published by the National Institutes of Health (NIH), found that only about one quarter of adults with SCD received regular care from sickle cell specialists. An even smaller percentage of people receive effective treatment to prevent sickle cell-related complications.

Today, the estimated life expectancy for people with SCD in the United States is more than 20 years shorter than the average life expectancy. According to a paper published by the American Journal of Managed Care (AJMC), a person with SCD can expect to live 52.6 years, with females having a longer life expectancy (55 years) than males (49.3).

The Centers for Disease Control and Prevention (CDC) notes that among the overall population in the U.S., life expectancy for women is 79.3 years and for men 73.5. People with SCD also have a quality-adjusted life expectancy— a measure of how long a person can expect to live in good health—that is more than 30 years shorter than the overall population.

Because SCD blocks delivery of oxygen to cells, it affects every organ of the body. As a result, in addition to causing pain, SCD also can cause other health complications, including, among others, high blood pressure, blood clots, anemia, infection, and stroke. Access to quality medical care designed to diagnose, treat, and prevent complications can dramatically improve the quality of life for people with SCD.

Understanding sickle cell disease

Sickle cell disease is an inherited blood disorder caused by a genetic variant that changes the structure of hemoglobin, the oxygen-carrying protein within red blood cells. The variant provides a survival advantage to people who live in parts of the world where malaria is common. People with one copy of the sickle cell gene—what’s known as sickle cell trait—are more likely to survive malaria infection. Over time, the sickle cell trait has become prevalent in that population.

In the U.S. today, approximately 1 in 12 Black Americans and 1 in 145 Americans of Latino descent carry the sickle cell trait. Many don’t know they have it because sickle cell trait doesn’t cause health problems. However, if both parents have the trait, there is a 25% chance with each pregnancy that their child will inherit two copies of the gene and develop SCD.

Life for adults with SCD

Fifty years ago, children born with SCD weren’t expected to survive to middle age. “In the 1980s, parents were told their children would live to about 20 or so,” says Morey Blinder, MD, WashU Medicine hematologist at Barnes-Jewish Hospital. But in the mid 1980s, researchers discovered that giving penicillin to infants with SCD—and immunizing them against contagious diseases—dramatically decreased deaths from infection. Today, almost all children born with SCD in the U.S. survive to adulthood.

Despite advances in treatment, most adults with sickle cell disease live with pain and disability. Thirty to 40% of adults with SCD suffer from chronic pain, and episodic pain crises interfere with the ability to work or attend school. Studies have shown that 53% of adults with SCD suffer what’s known as a “silent stroke”—brain damage caused by lack of oxygen to part of the brain—by age 30, says Sana Saif Ur Rehman, MD, WashU Medicine hematologist at Barnes-Jewish Hospital.

In 1998, the Food and Drug Administration (FDA) approved a drug called hydroxyurea, now a widely available, inexpensive oral medication for SCD. Extensive research over the years also has shown the value of regular, red-cell transfusions to decrease the risks for stroke and lung disease. Current research is focused on curative treatment for SCD using cell-based gene therapy, which offers much promise. Regular blood transfusions can also minimize the frequency of pain crises and reduce the risk of stroke and other complications. Two cell-based gene therapies, approved by the FDA in 2023, offer a potential cure for some.

Despite these advances, barriers to treatment exist, including stigma, financial and logistical challenges, and limited access to specialty care. While most big cities and large hospital systems have well-established pediatric sickle cell programs, far fewer have treatment centers dedicated to treating adults. Evidence suggests that gaps in care contribute to a spike in sickle cell-related deaths among young adults in their 20s and 30s.

TRISTAN, PICTURED HERE, RECENTLY TRANSITIONED FROM SICKLE CELL CARE AT SITEMAN KIDS AT ST. LOUIS CHILDREN’S HOSPITAL TO ADULT CARE AT BARNES-JEWISH HOSPITAL.

Photo Courtesy of Barnes-Jewish Hospital

A new era: the Sickle Cell Treatment Center

In April 2024, the Sickle Cell Treatment Center opened at the Center for Advanced Medicine at Barnes-Jewish Hospital. At the time, it was one of only 12 such centers in the U.S., and it is the first dedicated infusion space for adult patients with SCD in the St. Louis region. The Center offers specialized care that includes a comfortable place where IV fluids and pain relief medications are given to those experiencing a sickle cell crisis.

But long before the opening of the new treatment center in St. Louis, Barnes-Jewish Hospital and St. Louis Children’s Hospital worked together with WashU Medicine physicians to provide care for children and adults with sickle cell disease. Recently, the hospitals and clinicians have focused on further improving care for patients as they transition from childhood to adulthood. That transition involves more than a change in medical providers—it’s also a shift in responsibility and advocacy.

Marie Turner, DNP, CPNP-PC, a pediatric nurse practitioner in the young adult sickle cell transition clinic, highlights key differences. “In pediatrics, we focus on ensuring families understand SCD and can advocate for their child’s needs,” Marie says. “Once patients enter adult care, they are ready to acquire the skills needed to advocate for themselves, which can be challenging—especially when they may face stigma in emergency settings related to their need for pain management.”

Recognizing this, BJC’s multidisciplinary task force is actively working to improve the experience for adult patients across the health care system. “One of the biggest concerns we have heard from families in our community is that they’re fearful their young person will suffer when they transition from pediatric to adult care,” says Winston Wright, MPH, senior consultant of Belonging and Inclusion at BJC Health System. When BJC initiated public listening sessions focused on that concern, it became clear there was a need for a dedicated adult SCD clinic— one with a permanent, specialized space staffed by providers who understand the complexities of the disease, who know their patients, and can offer consistent, expert care tailored to specific needs.

The Sickle Cell Treatment Center was designed to meet those needs. Increased collaboration between pediatric and adult care teams ensures a smooth transition for young adults with SCD. People ages 18 to 25 can now see their pediatric provider at the new clinic. “They get a feel for coming here, but still get to see their pediatric specialist, the one they’re familiar with,” Dr. Blinder says. At age 25, patients officially transfer their care to one of the adult SCD specialists—and continue to receive care in a comfortable setting.

Although the Sickle Cell Treatment Center has been open for just a year, it’s already made a big difference. “It means so much to our patients that they have a place that belongs to them,” Dr. Saif says. “We’re here to care for them.”