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With sickle cell disease, just living each day is a victory

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AKRON, Ohio - For 33 years, Rafael Morris has fought an enemy that flows through his veins.

At any moment, his blood can turn against him and cause pain so excruciating that he can't find the words to describe it.

Someone once compared the agony to that feeling you get when you bash your thumb with a hammer or smash your finger in a door.

When the pain takes over, Morris knows his red blood cells are morphing from their normal round shape into hard, sickle-shaped cells that lodge in his tiny veins and rob his organs of oxygen.

"It can happen anytime, anyplace," he said. "And that's the scary part of it."

For Morris, a Medina, Ohio, resident, and other adults with sickle cell disease, just living each day is a victory.

Medical advances are keeping patients with the incurable, inherited blood disorder, once considered a childhood illness, alive into their 40s, 50s or even longer.

But those success stories also are creating new challenges.

For the most part, the health-care and support system created for sickle cell patients still focuses on children, said Dr. Willarda V. Edwards, president and chief executive of the Sickle Cell Disease Association of America Inc., a nonprofit advocacy group based in Baltimore.

"It's a blessing and, at the same time, it's an issue that hasn't been addressed in terms of allowing folks to get the kind of care that they need after they get older," Edwards said. "It's a blessing in disguise. About 30 years ago, people didn't live into adulthood."

Most treatment programs are tied to pediatric hospitals and screening programs created within the last few decades to identify newborns with the disorder, said Ira Bragg-Grant, executive director of the American Sickle Cell Anemia Association of America, another nonprofit advocacy group based in Cleveland.

Newborns identified as having sickle cell disease through state-mandated blood screenings immediately are placed on antibiotics to battle infections and referred to pediatric programs.

Blood transfusions and a process known as exchange therapy also are used to reduce complications by replacing the sickle-shaped cells with normal blood cells.

"Pediatric care is very good in the area of treatment and management," Bragg-Grant said. "However, once that child or young adult begins to transition into adult care, that's when everything falls apart. All around the country, we're experiencing those issues where there is very limited emphasis and care for adults with sickle cell."

About 70,000 Americans - almost all of them black - have sickle cell disease, which causes red blood cells to become hard and pointed instead of soft and round.

The misshapen cells block and damage the small blood vessels in the spleen, kidneys, brain, bones and other organs, reducing their oxygen supply.

As a result, patients risk serious complications, including anemia, strokes, infections, joint damage, reduced lung, liver and kidney function and, for men, persistent, painful erections.

Morris sits on the advisory board for the sickle cell program at Akron Children's Hospital. But he can't benefit from the coordinated care provided there because he's an adult.

"Unfortunately, with adults, it's just completely reactive care," he said. "With adult medicine, it's not cohesive."

Morris tries to go to the same urgent care center run by his insurance company when he's having a painful sickle cell crisis.

Otherwise, he said, emergency room doctors and nurses have been known to look at him skeptically when he begs for more narcotics to control his pain.

"They say, `Well, we gave you this amount of pain medicine. Why are you saying you're still in pain?'" he said. "It's a big issue. I wish I didn't have to take pain medicine, period."

If sickle cell advocates get their way, adult patients could get help more easily in the future.

The Sickle Cell Treatment Act of 2003 calls for creation of a grant program to support 40 health centers nationwide that focus on caring for sickle cell patients.

So far, however, the $10 million needed to fund the initiative hasn't been approved by Congress.

Holly Codding, formerly education coordinator for Ohio's regional sickle cell program based at Akron Children's Hospital, was among 20 experts who attended a recent meeting with the federal Maternal and Child Health Bureau to give recommendations on how to implement the act. A report with details about the recommendations is expected later this year.

One focus of the initiative is improving adult care, said Dr. Peter van Dyck, associate administrator for the bureau.

"Because folks with sickle cell may be living longer, there's more need for coordination into the teenage years and into adulthood," he said. "There is treatment available, generally. It may not be as well-coordinated as it could be."

Dr. Zahida Yasin, director of the Adult Sickle Cell Program at the University of Cincinnati Medical Center, said more funding is needed to support the few adult programs and to create new clinics.

She recently held a conference on the issue of caring for adults with sickle cell disease.

"My fear is the comprehensive pediatric centers have a lot of clout, and they're going to get the money," Yasin said. "If there wasn't good care for children, we wouldn't have these adults now. But we need to educate our adults about their illness, about their medications. We need to support them, so they can take better care of themselves."

In the meantime, some pediatric sickle cell clinics such as the program at Akron Children's Hospital are trying to make the transition into the world of adult medicine easier.

Doctors, nurses, social workers and psychologists at Children's start sickle cell patients in a gradual transition program when they're 13 years old.

Children's covers a checklist of topics - details about their disease, the genetic risks of passing it on to their children, financial issues and general health tips.

When patients are ready to switch to adult care, they have a visit at Children's with Dr. John Petrus, who heads the hematology and oncology section for the Northeastern Ohio Universities College of Medicine.

Petrus treats about 40 adults with sickle cell disease.

"The young adults that we're getting out of the Children's program are much better prepared," Petrus said. "They have a much better understanding of their disease. They're much more willing to take ownership over their disease and its treatment. As a result of that, they tend to deal with this with much greater ease as adults."

The transition program has been helping 16-year-old Daraya Green of Akron get ready for the day when her mother won't be making her appointments.

"My nurses and doctors are really good about that," she said. "They know that I'm going to have sickle cell forever, so they talk to me a lot to make sure I'm taking care of myself."

But she's concerned that the exchange therapy she gets at Children's to replace her sickle cells with normal red blood cells without the risk of iron buildup in her blood isn't widely available to adults. (Insurers often won't cover the treatment for adults, Petrus said.)

Since starting the therapy last year after having a stroke, Daraya has cut her number of missed school days in half.

"Before the transfusions, I used to have a crisis every single month and be admitted into the hospital," she said. "Lately, I've been doing good."


(c) 2005, Akron Beacon Journal (Akron, Ohio). Distributed by Knight Ridder/Tribune News Service.

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