Terri Booker was 11 when a routine blood test revealed that she had sickle cell disease (SCD), but even as a toddler she felt its pain. “It is like someone gripping your arm and twisting it in two opposite directions.”

SCD affects approximately 100,000 in the United States. People with SCD have abnormally shaped hemoglobin, the protein in red blood cells that carries oxygen from the lungs to bodily tissues. The affected cells become crescent-shaped instead of rounded and are more likely to get stuck in blood vessels, obstructing blood flow. This can lead to severe pain, inflammation, and in extreme cases, tissue and organ death.

Booker has struggled with periodic pain attacks, or crises, and has undergone regular transfusions, laser treatment on her eye, and hip surgery. “It’s a constant battle with your body,” she says. Through it all, she has learned and adapted. She knows to stay hydrated, to avoid extreme temperature or altitude changes, and to rest when she feels run down. If she takes a vacation, she makes sure there is a hospital nearby.

SCD disproportionately affects people of African heritage, and Booker posits that this is one reason the disease is still poorly understood and under-resourced. Although all newborns in the U.S. are supposed to be screened for the disease—to develop SCD a baby must possess the sickle cell trait from both parents—many people are not diagnosed until there is a crisis. Many doctors are not well trained in what to look for and how to treat patients in crisis.

Booker notes other challenges in identifying and addressing the disease. Bias can play a role; she tells of a Caucasian family where all three children were diagnosed with SCD, but only after repeated denials by their doctor, who believed it only presented in black people. Many SCD patients arrive at the emergency room seeking relief from severe pain, only to be received by a physician reluctant to dispense adequate pain medication.

Now in her late 30s, Booker has harnessed her experience with SCD to serve as an advocate for greater public awareness of it. She helped lobby Congress to pass the Sickle Cell Treatment Act of 2018 and advocates for expanded research and treatments. She emphasizes the importance of donating blood, that it can save the life of a person with SCD.

Booker is optimistic, noting that the prognosis for a young person with SCD today is so much better than it was two generations ago. She urges people to learn more and seek resources and community for the disease through the Sickle Cell Disease Association of America. “The pain is real, believe us,” she says, but adds, “I am hopeful that medicines will be created to make our everyday lives better.”

- by Jackie Faiman