LULU GARCIA-NAVARRO, HOST:
Now sickle cell disease. About 100,000 people in the United States suffer from it, most of them African-American. And life expectancy for people with sickle cell is dropping. Yesterday, we heard from a mother whose son died at 36. Today, we'll hear from a patient who's thriving and the clinic that's making it possible. Jenny Gold has a two-part - has Part 2 of our series on sickle cell disease and discrimination.
JENNY GOLD, BYLINE: Derek Perkins sits on the end of the exam table at the Adult Sickle Cell Center at Children's Hospital Oakland. He's short but strong, his arms and chest covered in tattoos. He's a father of four and has been with his wife for 28 years.
DEREK PERKINS: I'm a driving instructor. I teach kids how to drive and adults, also. Just a basic, normal man.
GOLD: Normal, perhaps, but also remarkable. Life expectancy for people with sickle cell disease is around 40. At 45, Perkins has already beaten the odds. Sickle cell is a genetic disorder where red blood cells bend into a crescent shape. It causes multi-organ failure and is complicated to treat. There's a hospital just three minutes from Perkins's house, but he drives nearly an hour to come here instead.
PERKINS: Without the sickle cell clinic here in Oakland, me, myself - I don't know what I would do. I don't know anywhere else I could go.
GOLD: When Perkins was 27, he once ended up at a different hospital, where doctors misdiagnosed him. He went into a coma, and the doctors gave him just a 20 percent chance of survival. His mother insisted he be transferred here to Oakland.
PERKINS: Dr. Vichinsky was able to get me here to Children's Hospital, and he found out what was wrong and within 18 hours. All I needed was a emergency blood transfusion, and I was awake.
GOLD: Perkins is talking about Dr. Elliott Vichinsky, the man visiting his exam room now. They first met when Perkins was just a kid.
ELLIOTT VICHINSKY: You know, when you see someone as an adult like Derek must be - how old are you? Forty?
GOLD: Five.
VICHINSKY: Forty-five. How old do you think I am?
GOLD: Vichinsky started the Oakland Center in 1978, and he's one of the country's leading researchers on adult sickle cell. He says that while Perkins may look robust, he has problems with his kidneys, heart, hips and breathing.
VICHINSKY: So what about getting shorter breaths?
PERKINS: All the time, yeah. I'm on a inhaler...
VICHINSKY: Inhaler?
PERKINS: Yeah.
VICHINSKY: Do you use it?
PERKINS: Yes.
GOLD: Vichinsky's clinic and the handful of others like it have made major advances in screening sickle cell patients for the early signs of organ failure, so they can intervene. This isn't easy to do, and it requires time and training. And it doesn't pay well. Many sickle cell patients are on Medicaid. But with consistent expert care, patients can expect to live to 65. The problem is that most sickle cell patients still struggle even to access treatments that have been around for decades, Vichinsky says.
VICHINSKY: I would say 40 percent or more of the deaths I've had recent have been preventable - I mean, totally preventable - 40 percent. It makes me so angry. You know, I've spent my life trying to help these people, and the harder part is you can change this. This isn't a knowledge issue. It's an access issue.
GOLD: And it's nothing new. The disease has had a long and sordid past. Sickle cell was first identified in 1910 and helped launch the field of molecular biology.
VICHINSKY: There's a long history of scientists and the government using sickle cell to study science rather than improving the disease itself.
GOLD: In the 1960s and '70s, sickle cell became a lightning rod for the Civil Rights Movement. At the time, the average patient died before the age of 20. The Black Panther Party took up the cause and began testing people. Here's Party Chairman Bobby Seale at a community event in Oakland in 1972.
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BOBBY SEALE: I'm sure we tested over 4 and a half thousand people for sickle cell anemia last night, and I think that the voter registration's running neck-and-neck with it.
GOLD: In the 1970s, Congress added additional funding for the disease, and states started screening newborns. Treatment improved. And by the 1990s, life expectancy had doubled with patients living into their 40s. But over time, funding waned, and life expectancy started dropping again. Vichinsky says discrimination is a big reason they're losing ground.
VICHINSKY: The death rate is increasing. The quality of life is going down. There's no question in my mind that class and color are major factors in impairing their survival, without question.
GOLD: Vichinsky's patient, Derek Perkins, knows he's one of the lucky ones.
PERKINS: The program that Dr. Vichinsky is running here I feel I owe my life to because if it wasn't for him and the things that he did for me, my family wouldn't have me.
GOLD: With so many patients and so few resources, it's likely that Perkins will continue to be the exception and not the rule. I'm Jenny Gold in Oakland.
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GARCIA-NAVARRO: Jenny Gold is with our partner Kaiser Health News.
(SOUNDBITE OF THIS WILL DESTROY YOU'S "THEY MOVE ON TRACKS OF NEVER-ENDING LIGHT") Transcript provided by NPR, Copyright NPR.