LITTLE ROCK, Ark. – The University of Arkansas for Medical Sciences is getting $400,000 to start a clinic to treat adults with sickle cell disease, a painful illness that used to regularly kill patients at a young age.
The disease affects about 1,000 people in Arkansas, according to health officials. New treatments have allowed those with the disease to live into their 50s, 60s or beyond, and the goal is to improve their quality of life.
Arkansas Children’s Hospital treats pediatric sickle cell patients, but once those patients become adults, they have to find another outlet for care. Often that means going to a local doctor, who may not be familiar with the intricacies of the condition and the vast array of other health problems it causes.
Gov. Mike Beebe this spring signed a bill by Rep. Reginald Murdock, D-Marianna, that gives the University of Arkansas for Medical Sciences $400,000 to start a clinic intended to be a center for specialized care for sickle cell patients.
UAMS Associate Vice Chancellor for Communications Leslie Taylor said more money will be needed, and the hospital is in the process of developing a plan to lay the groundwork to get the clinic started.
The clinic was the central recommendation from a task force formed two years ago to see how the state can better address the needs of sickle cell patients.
“A lot of floor-level work still needs to be done (to) make this an effective piece of legislation,” Murdock said. “We don’t have enough of the recommendations of the task force in place yet to really make this like it needs to be. Hopefully this will gain some momentum, and people will see the necessity.”
Taylor said it is too early to say how much it will cost to start the clinic. The state fiscal office says $200,000 of the money is ready to release when UAMS is ready.
Patients with the disease, who often are laid up for weeks or months at a time, say the clinic can’t arrive too soon.
“I’m 27 and I’ve never seen a sickle cell specialist,” said Tiffany Wright, who lives in Marianna and has the disease. She is also Murdock’s niece.
Wright said geography and having limited health insurance have kept her from seeing a specialist. The program envisioned at UAMS would enable patients to come in to be evaluated in Little Rock and leave with a treatment plan that their local doctors could execute.
The center would tap into UAMS’s telemedicine program so patients across the state can consult with a specialist while visiting their local doctor, if needed.
“That’s the full point of it, to have better quality of care,” said Germaine Johnson, a sickle cell patient and advocate for improved care.
Living with the disease means managing pain on a daily basis. Often, the illness causes hospitalizations that last so long it’s hard for some to hold a job.
When the body is not going through a “crisis,” a term used when symptoms become extreme, some patients can be treated with the drug hydroxyurea, which increases production of healthy hemoglobin. But the drug also has potentially serious side effects and needs to be closely monitored.
The disease is caused by red blood cells, which are ordinarily shaped like discs, that take on a crescent or “sickle” shape. Red blood cells carry the protein hemoglobin, which transports oxygen to the body. An abnormality in the hemoglobin causes the cells to sickle.
Cells that sickle are sticky and cause obstructions in blood flow, and the painful and dangerous health problems begin when stress, exertion or other triggers cause a greater restriction in blood circulation.
“Pain is the hallmark problem,” Johnson said.
At its worst, the lack of oxygen in the blood can lead to multiple organ failure. But there’s a long list of other illnesses that develop.
“It causes strokes, eye problems, ear problems, problems in the hips and joints, heart attacks … different episodes of things,” Johnson said.
Patients have problems with their spleens and are prone to developing gall bladder problems.
“It causes chest problems, which is often misdiagnosed as pneumonia, but it causes pneumonia too,” Johnson said.
Wright said Friday that she’d returned home the day before from a two-week hospital stay. Of the last eight months, she said she has spent five in the hospital, including surgery to remove her gall bladder.
“I’m still trying to recuperate. Just getting my bearings,” she said.
Wright, who has a college degree, is trying to resume her career as a medical technologist. She had been working at a hospital in Jonesboro but had to leave the job because of the time she spent in the hospital. Wright said she couldn’t keep up with her mortgage and had to move in with her uncle.
One of Wright’s motivations to fight through the pain and month after month in the hospital is to outlive her father, who died of the disease when he was 29.
“He was just two years older than me when he passed away,” she said. “I fight to stay healthy and live past the years of my father.”
Johnson is 38, an age he said he is lucky to see.
“All my life, I felt like I was prepared to die,” he said, then repeated comments he’s heard people make. “He won’t see 18; he won’t see 21.”
Every two or three weeks, Johnson has to undergo blood transfusions, a process that takes almost 24 hours.
The disease has no cure, but the understanding of the condition has expanded and that has led to improved treatment options.
Dr. David Becton, chief of hematology and oncology at Arkansas Children’s Hospital and a professor at UAMS, treats pediatric sickle cell patients. As the patients turn 18, they’re started on the path of finding their own care and the hospital doesn’t treat them after they are 21.
With treatment and carefully managed care, Becton said patients can live until their 60s or beyond. And he said quality of life is an important part of the treatment plan.
“There are plenty of reasons to want to organize the adult care clinic,” Becton said.
Murdock said he was surprised the state didn’t have an adult clinic a long time ago.
“With as many citizens there are in the state of Arkansas that (sickle cell) affects, medical costs being what they are to us, it seems like we would have been forward in having this,” Murdock said.
Presuming the clinic is successful in reducing the number of days patients spend in crisis and in the hospital, the investment would pay for itself, Murdock said.
“These patients are sometimes on disability. They can’t work because of the instability of the disease. But with better treatment, they can be taxpayers again and contributing to the economy,” Murdock said.