Fighting Sickle Cell

As the pain of a sickle cell attack pierces Ja’Sean Smith’s legs, back and feet, he imagines it’s another visitor from the monster, “Jeepers Creepers.”

Sometimes, the pain leaves the 6-year-old Detroiter so drained, he’s unable to move, and unwilling to let his mother touch him. He can’t lie down, so he gets up. When he gets up, he’s so weak he must lie down.

His mother, Tuweka Smith of Detroit, says it’s like watching him being tortured, and all she and her husband, the Rev. Michael Smith, can do is watch and pray. The monster often sends Ja’Sean to the hospital; it’s mean-and ugly.

“He’s got a black face, black body and black neck, and he eats you,” Ja’Sean explains. “He can’t talk. He just slides in and gets you. I try to kill him, so I don’t get sick anymore.”

In the past three months, Ja’Sean was rushed to the hospital three times, once for a 10-day stay. He recently started a new daily regiment with hydroxyurea, a cancer drug used for adults with sickle cell since 1995. 

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Just last year, researchers found it effective for children with sickle cell, even those younger than 19 months. But it will take at least six months before Ja’Sean’s doctors know it will be effective for him.

“I’m keeping a positive outlook on everything,” says Tuweka Smith, 34. “I think about him living a long life with the disease with new medications and things coming out to help the disease, things helping with a better, productive life. I try to do everything the doctors tell me so I can get a positive outcome.”

With new pediatric sickle treatments on the horizon such as hydroxyurea for children and a clinical trial in Phase III with the U.S. Food and Drug Administration for L-Glutamine, an antioxidant, parents of pediatric sickle cell patients have renewed hope that someone will soon come up with a cure for the genetic blood disease-or at least a highly effective treatment. DMC Children’s Hospital of Michigan in Detroit is one of about 30 sites where the drug is being tested nationally.

When Ja’Sean describes his pain as an ugly monster, Dr. Yutaka Niihara, lead investigator at the Los Angeles Biomedical Research Institute at Harbor-UCLA Medical Center who led research for the patent-protected drug, says sickle cell, indeed can be very ugly.

“It’s the worse pain people can experience,” he says. “I use chemotherapy for cancer patients, and they sometimes need morphine for pain. I have to give more narcotics to sickle cell patients to take care of their pain. Many doctors treat [adult] sickle cell patients like drug seekers. They don’t realize their pain is really, really horrible. They have a serious medical problem, and people don’t understand.”

In early results with the drug, Niihara says his patients are not getting as sick, rushing to the hospital as often or experiencing as much pain. In fact, the antioxidant, changes their red blood cells from a sickle shape to a normal round shape.

“Patients all of a sudden had to stop taking narcotics, their ulcers were healing and patients were feeling so much better. Many of the patents who used to go to the emergency room every two weeks, didn’t have to go the emergency room hardly at all.

“This treatment had hardly any side effects; it makes the patients feel better and they improve circulation. We are very convinced that this will work.”

Currently, there is no cure for sickle cell disease, but children and adults are living longer because doctors have figured out how to conquer some of the infections and organ damage the disease causes. Still, generally, Niihara says, many sickle cell patients die in their 40’s.

Sickle cell disease, sometimes called sickle cell anemia, is an inherited blood disorder causing red blood cells to become oxidized, forming rigid and sickle-shaped cells that block small blood vessels. Besides debilitating pain, the organ damage it causes can lead to premature death.

Because it affects so few people in the United States, sickle cell is considered an “orphan” disease, but it impacts about 72,000 African Americans, according to the U.S Human Genome Project.

The disease also affects millions around the world, though it’s most common among people whose ancestors come from sub-Sahara Africa, Spanish-speaking regions (South America, Cuba, Central America); Saudi Arabia and India. About 2 million Americans, or 1 in 12 African Americans carry the sickle cell trait, researchers estimate.

That means if a man and woman with the sickle cell trait reproduce, they have a one in four chance of having a baby with sickle cell disease. Trouble is, most people who have children have no idea whether or not they have the trait because they’ve never been tested, says Dr. Wanda J. Whitten-Shurney, a pediatrician at the Comprehensive Sickle Clinic at  DMC Children’s Hospital of Michigan and CEO and medical director of the Sickle Cell Disease Association of America, Michigan Chapter Inc., in Detroit.

Shurney’s father, the late Dr. Charles F. Whitten-a renowned sickle cell disease researcher who advanced science, outreach and education for sickle cell disease-founded the organization in 1971.

She clearly is his legacy, treating patients, spreading the word around Detroit educating folks about sickle cell, helping people make decisions and traveling the nation-including a recent trip to pay tribute to her father at the National Library of Medicine, where his research papers are archived at the branch of the National Institute of Health.

“My father did such great things; people say I’m following in my father’s footsteps,” she says. “I take my shoe off and show them my shoes are not that big.”

Instead, she prefers to say she’s “standing on his shoulders.”

“I talk to the mother who didn’t beat the odds,” she says. “I also talk to adults with the trait so they can make an informed decision, not so I can say, ‘Don’t have a baby.’”

Most hospitals screen babies for sickle cell, she says, and although many people think having sickle cell is like a death sentence-it really isn’t.

“There are people who are never sick,” she says. “There are people who are a little sick and a lot sick. Every individual is different just like every snowflake is different.”

Tuweka Smith credits Shurney and the sickle team at Children’s for helping her to understand that her son also is unique, and offered her advice on how to best care for him.

“Give him his medicine,” she says. “And when he’s going through pain, put warm towels on him. Make sure he’s drinking a lot of water so the blood cells and can flow freely. When (sickle cell patients) get dehydrated, their blood cells clump and they have a pain episode.”

Dr. Sharada Sarnaik, professor of pediatrics at Wayne State University and the director of the Sickle Cell Center at Children’s Hospital, heads the team of doctors who care for Ja’Sean. She is leading the Detroit L-Glutamine clinical trial, which is still open for enrollment until Sept. 30.

Sarnaik says some reluctance exists with L-Glutamine “because it has to be taken every single day, and I don’t know that too many of our patients can commit to that.”

However, she is optimistic that it’s one more weapon in the arsenal to help fight sickle cell.

“This is one of the things that is useful,” she says. “Research is being done, and it’s very promising. This won’t be the only thing we do; it will be one of the things we do.”

To inquire about the L-Glutamine study, call the Pediatric Sickle Cell Center at 313-745-5613.

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