What It’s Like to Have Sickle Cell Disease: ‘It Takes a Tribe to Sustain a Warrior’
Key takeaways:
Sickle cell disease is a genetic disorder that causes red blood cells to become shaped like a sickle, causing blockages and triggering pain.
The condition is most prominent among Black people, but it also affects people of other races.
Patient advocate Phill Okwo looks forward to promising treatments on the horizon.
When Phill Okwo was 6 years old, he went to a camp in California for kids with sickle cell disease (SCD). That is one of his earliest memories of being aware of his condition.
At the Sickle Cell Disease Foundation camp, he could do the things he loved. He’d swim, go horseback riding, do archery, even have pillow fights. In that medically supervised environment, he felt safe.
And each summer, he and the other campers learned about their condition. They discovered why they — and not their parents or their siblings, in some cases — had the disease.
“We were literally learning intro-level genetics at age 6,” Phill says.
When he wasn’t at the camp, Phill had to be careful about overexerting himself. Overexertion can sometimes send kids with SCD to the hospital with severe pain. So he often took breaks during games or sports when his siblings or classmates kept going.
The disease was very different in those days, says Phill, who is now 42 and living in Atlanta.
“When I was born, life expectancy was about 18 to 19 years old,” he says, referring to the shortened lifespan of people who have SCD. In fact, SCD was considered a pediatric disease in the 1970s, because few people who had it lived into adulthood.
Thanks to universal newborn screening and advances in treatment, people who have SCD now live an average of 54 years. But even though people are living longer, Phill says, sometimes it seems the health system hasn’t caught up.
From camper to sickle cell patient advocate
Phill loved the summer camp so much that, when he aged out as a camper, he came back as a counselor. And now, he works as an independent, professional patient advocate for people with Sickle cell disease. That role includes educating, mentoring, and coaching adults with sickle cell. And it also means helping people transition from pediatric to adult care.
Sometimes, he educates providers about the latest guidelines for patients who have SCD. He also joins podcasts and does interviews to increase awareness. And he has testified before the Texas House of Representatives about disease measures.
The only sibling with a sickle cell condition
Phill is the oldest of three siblings, and he’s the only one of them who has sickle cell disease. Growing up, he says, his siblings didn’t always understand why he sometimes seemed to get more hands-on attention from his parents.
He has two children who do not have the disease but are carriers of the trait. Because of that, they may later face decisions around the risk of having children born with the disease.
SCD is most prevalent among Black people. It affects about 100,000 Americans overall, and about 1 out of every 365 Black or African-American people at birth. It also affects about 1 out of every 16,300 Hispanic-American people at birth.
People with SCD have red blood cells that are shaped like sickles, or crescent moons — versus round, or healthy, red blood cells. The sickle shape causes the cells to sometimes get stuck together in small blood vessels and clog the flow of blood. That can lead to a pain crisis or other complications, including infection or stroke.
Avoiding triggers that can set off a pain crisis
Phill says he doesn’t think about his sickle cell disease every day. He tries to drink water regularly, which is particularly important for people with SCD. And he eats healthy foods and tries to avoid triggers that can set off a pain crisis.
For him, one major trigger is cold weather. Getting hit by a cool gust of wind after getting out of a pool could be a trigger, he says. But he knows other people who have the disease and are triggered by hot weather.
“A crisis is usually very, very intense, excruciating pain,” Phill says. “It can be hard to breathe. But, generally, that crisis is indicating a lack of or insufficient oxygen.”
The pain can feel like a dull ache or “like sharp, stabbing knives,” he adds.
During a sickle cell crisis, Phill takes pain medication to control his symptoms. He also takes vitamin D and folic acid supplements regularly to help prevent crises from occurring. And he is vigilant about getting enough sleep, because lack of sleep can trigger a crisis, he says.
"I learned that the hard way in both undergrad and grad school. I can’t do what my peers do. I can’t pull the all-nighters,” Phill says, referring to studying for final exams when he was in college and graduate school.
Holiday travel presents a potential slew of challenges for him and other people who have SCD, he adds. Traveling by plane means dealing with different oxygen levels. The climate of the destination may be different from the one at home. And sleep patterns can be thrown off.
General travel stress can also be a trigger. “It’s not unheard of for people coming into the new year to be in crisis and be hospitalized — and have used up all their sick days in the first month of the year, because they're recovering from holiday travel,” Phill says.
Promising improvements on the horizon
Phill says he’s encouraged by the progress that’s been made in stem cell transplants and treatments. But he is also frustrated with the pace of the progress.
In July, he attended a “Cheek Week” event that was aimed at promoting cheek swabs, which help identify potential blood stem cell donors. These donated cells are used in transplants that treat conditions like sickle cell disease. Up until recently, stem cell, or bone marrow, transplants were the only therapy approved by the FDA as a cure for SCD. But there are promising new gene therapy treatments being developed.
“In the same way we say that it takes a village to raise a child, I think it takes a tribe to sustain a warrior,” Phill says. “So a lot of my advocacy is about building and sustaining tribes.”
He adds that he benefits greatly from his connections to the SCD “tribe,” or community. “I think it’s as much a lifeline for me as [it is] a vocation,” he says.
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