The pain never goes away.
Pain is a constant of Beverly Chukwudozie’s entire life. Not every minute of every day, and not always severe — what she calls a “10” level of pain.
But most minutes of most days, somewhere between discomfort and agony. And the rare times the pain vanishes altogether, it is always still in the background, “a constant fear.” Certain to return, the only question being when, and where and how severe.
Chukwudozie, 43, doesn’t remember a time when it was otherwise. As a girl in Nigeria, she was told she was suffering from rheumatoid arthritis. That’s what made her joints ache, sometimes as if they were being stabbed with a knife.
“I had a major complication that kept me in the hospital for about a month,” she said. There, a hospital resident told her, offhandedly, that no, it wasn’t arthritis. It was worse; she had sickle cell disease, and might expect to live to be 21.
She was 12 years old.
“There was really limited knowledge at the time,” she remembers. “I did my best to find out more about it.”
So did I. After Gov. J.B. Pritzker’s office announced September is Sickle Cell Disease Month. I realized that while I’d heard of the disease, I knew almost nothing about it except that it affects Black people, primarily, making their red blood cells, rather than being vaguely round, take on a crescent shape — the “sickle” part of the name.
Chukwudozie says she meets people who are completely ignorant of sickle cell, even afraid they might catch it from her.
“I’ve had people ask me, ‘Is it contagious?’ ” she said. “It is hereditary. I have one trait, the deformed sickle cell trait, from each of my parents.”
About one in 13 Black babies is born with the genetic trait; one in 365 are born with the disease. More than 80% of persons living with sickle cell are Black.
It used to be called “Herrick’s Syndrome,” named for James Herrick, the Chicago doctor who first identified the condition after looking at the blood of Walter Clement Noel, a 20-year-old dental student from Grenada. Then “sickle cell anemia.” But as understanding of the condition grew, anemia became considered the most common manifestation of a broader group of ailments, known collectively as “sickle cell disease.”
“For sickle cell disease, it’s different for different people,” said Chukwudozie. “We have sickle cell anemia, we have hemoglobin C, sickle beta-plus thalassemia, and some others. The disease manifests itself in different ways, but there is a common theme of chronic pain, chronic fatigue. Different complications that affect your bones and the way you function since red blood cells don’t carry enough oxygen. Some organs or joints don’t get enough oxygen from the blood, and that causes pain.”
For Chukwudozie, one complication is bone avascular necrosis of her left hip. That means the bone dying from lack of blood. It requires her to walk with a cane or crutches.
Because the central symptom of sickle cell disease is pain, sufferers have also collided with the opioid crisis. Black patients are generally under-medicated for pain, and chronic sufferers like Chukwudozie can struggle to get the meds they need.
“The exemptions for people living with cancer, but don’t have exemptions for people with sickle cell,” she said. “We have to do a lot of advocacy.”
She uses treatments commensurate with the pain, starting with Tylenol for mild distress, moving up through Vicodin to an ER visit for an acute crisis.
“The pain is debilitating,” she said. “It has been described in different ways. It sometimes feels extreme, like a stabbing pain, a sharp object, a knife. Very sharp, being stabbed. Sometimes feels like a blunt sore pain, like you’re being hit by something really, really hard. The pain differs. Sometimes it comes on suddenly. Sometimes it takes a few days.”
She has fought to overcome it her entire life.
“I had to struggle, always dealing with pain,” she said. “Still studying, going back to school. I was always studying in the hospital, in the ER. Always struggling to keep up with grades, keep up with my peers. I had to work twice as hard.”
The disease affected her choice of job; she became a medical researcher, and is now an administrator, developing leadership and education programs and supporting research at the University of Illinois Cancer Center.
“My ideal career was to be in military, medical doctor. But I can’t be in the military because of sickle cell. I couldn’t really go. I had to get a job where I could sit down and work, nothing that would make me walk around a lot, because of how tired I get.”
That said, it doesn’t stop her from living a full life.
“I do have hobbies. I do like traveling. I do love spending time with my family and kids,” she said — her sons are 10 and 7. “I knew that if I wanted to have my own kids, I had to marry someone without the disease. Having children with someone with the disease, or with the trait, was a concern for me. So when my husband, when we were getting married, and he didn’t have the trait, I welcomed the opportunity to have kids.”
Awareness of sickle cell lags, as well as research into it, and it’s difficult to imagine that the race of those primarily affected is not a factor.
“I feel like it’s a very slow pace,” Chukwudozie said. “The treatments are there, curative measures — stem cell transplants, that have been helping. Bone marrow transplants. There are clinical trials going on. It is much better now that there are other trials and research. There is funding. But prior to now, you cannot imagine. The only management for sickle cell was [the drug] hydroxyurea. In the last four or five years, other drugs have come on the market.”
She prefers to focus on the progress being made, rather than speculation on what might hold it back.
“There are a lot of great medical doctors trying to improve the care and research for sickle cell disease,” said Chukwudozie, who calls people living with sickle cell “warriors” who need to be their own best advocates. “Listen to your body and learn what is best for you. Stay positive, stay focused and take care of yourself.”