This post was originally published on Michigan Chronicle

By J. Nathan

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

Sometimes, the pain left the then six-year-old Detroiter so drained, he was unable to move, and unwilling to let his mother touch him. He couldn’t lie down, so he got up. When he got up, he was so weak he had to lie down.

His mother, Tuweka Smith, 44, of Detroit, said it was like watching him being tortured, and all she and her husband, the Rev. Michael Smith, could do was watch and pray. The monster often sent Ja’Sean to the hospital; it was mean—and ugly.

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

As a youngster, Ja’Sean was rushed to the hospital three times in a three-month period, once for a 10-day stay. His health turned around when he began taking hydroxyurea, a cancer drug used for adults with sickle cell since 1995.

A decade ago, researchers found it effective for children with sickle cell, even those younger than 19 months. Within months of taking hydroxyurea, Ja’Sean’s doctors knew it would be effective for him.

Now, he’s 16, and able to play sports and rarely has severe pain or hospital visits except for monthly blood transfusions that help keep his blood from clotting and preventing strokes.

“I’m keeping a positive outlook on everything,” said his mother. “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.”

When Ja’Sean described 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.

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“It’s the worse pain people can experience,” Niihara said. “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 said his patients were 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 were very convinced that this would 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 said, 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 effects 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 Detroit pediatrician affiliated with multiple area hospitals and CEO and medical director of the Sickle Cell Disease Association of America, Michigan Chapter Inc., in Detroit.

Whitten-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 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 said. “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 said. “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 Whitten-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.”