One of Audrey Patricia Smith’s early defining memories of sickle cell disease was in Myrtle Beach, South Carolina, in 1959, when she was 12 years old and visiting the ocean with family friends. Before then, Smith had not yet experienced a pain crisis.

“I got in that water, and that was it,” says Smith. “That was the beginning.”

Sickle cell disease (SCD) is a chronic inherited blood disorder that causes red blood cells to form a crescent shape instead of a round disc. These misshapen cells can restrict blood flow, leading to severe pain, anemia, organ damage and other complications. About 100,000 people in the United States live with SCD. The median age at death for adults with SCD remains in the mid-to-late 40s, and many people with the disease do not receive recommended health screenings and treatments due to a lack of access to clinicians and healthcare systems with SCD expertise.

World Sickle Cell Day, observed annually on June 19, offers an opportunity to raise awareness of the disease and reflect on the need for continued progress in care.

Born in 1947, Smith came of age during a time when little was understood about how to help people with SCD survive childhood, much less manage the disease across a lifetime.

“Mrs. Smith has lived through the vast majority of contemporary sickle cell history,” says Lydia Pecker, M.D., director of research and advocacy at the Sickle Cell Center for Adults at Johns Hopkins. “In 1947, children with sickle cell disease generally did not live past age 5. Every time Mrs. Smith hit another developmental milestone, she continued to outlive what was expected.”

When Smith was born, sickle cell disease had not yet been widely understood as a genetic disease. Most elements of modern SCD care, including newborn screening, preventive penicillin for infants, expanded childhood immunizations and hydroxyurea, either did not exist or were decades away from formalized development.

Those gaps shaped the early lives of many people in Smith’s generation. By the time she came to Johns Hopkins Medicine, Smith was living with multiple organ complications related to SCD.

“I didn’t really understand how much I needed to take care of myself until I was so sick with heart failure and everything,” says Smith. “When I went to Hopkins, I began to listen, listen, listen.”

Smith has received care at Johns Hopkins for more than 20 years. In March 2003, she began a monthly automated red cell exchange procedure, a treatment built on decades of research-driven progress in sickle cell care and transfusion medicine. The procedure removes sickle red blood cells and replaces them with healthy donor red blood cells. In two decades, Smith has likely received more than 2,000 units of donated red blood cells.

“The treatment does a great job of instilling healthy donor red blood cells and minimizing chronic organ damage,” says Liz Crowe, M.D., PhD, medical director of the blood bank in the Division of Transfusion Medicine at The Johns Hopkins Hospital. “It is also helpful for preventing acute life-threatening complications when patients come consistently for transfusions.”

Crowe says patients who receive exchange transfusions spend hours at a time with apheresis nurses and staff, often month after month. These recurring visits can create strong bonds, and for Smith, the genuine connection she feels has been integral to maintaining her long-term treatment plan.

“I give them triple As for the care they give Johns Hopkins Medicine sickle cell patients,” she says. “I’m grateful for the concern and the love that they share with us all. They want us to live, and live well.”

Pecker says that trust is especially important in SCD care, a field shaped by medical complexity and a long history of inequity.

“Trustworthy care begins with deep knowledge of the disease and the willingness to keep pushing for better treatment,” she says. “And the treatments are changing quickly. People with this disease need to stay care connected.”

Smith’s advice to younger patients looking to their own futures is simple: Keep your appointments and listen to trusted specialists.

“I know as young people, other things feel more important sometimes than appointments and screenings,” she says. “But today I would tell you to follow and listen to your doctors. That’s why I’m here, despite everything, at almost 79.”

Stories like Smith’s also underscore the importance of blood donation, especially during the summer months, when donations often decline and blood inventory shortages are projected.

“Only 3% of donors who are eligible to donate blood actually do,” says Crowe. “If the donor pool increased by even 1%, shortages would be alleviated.”

Please visit this webpage to search for and sign up for upcoming blood drives at Johns Hopkins.

Smith, Pecker and Crowe are available for media interviews about this story, sickle cell disease care and the importance of blood donation for people living with SCD.