Devastation of sickle cell disease chronicled
Film documentary scheduled February 28
ATLANTA, Georgia (CNN) - Although research continues, sickle cell anemia remains an "orphan disease" attracting little notice despite its harvest of pain and early death.
But a new film documentary may help to educate viewers about the disease, which affects about 80,000 people in the United States and millions worldwide. Called "Sickle Cell: The Forgotten Disease," the film will air at 10 p.m. EST on the Discovery Health Channel. It is being co-produced by the Dr. Spock Company.
"It's hard to get funding or people interested in trials," said Dr. James Eckman, director of the Georgia Comprehensive Sickle Cell Center at Grady Health System. "But because this is a lifelong, chronic problem, it has a greater impact."
A genetic disorder, sickle cell disease attacks red blood cells, making them rigid and distorting their normally round shape into crescents and points. These misshapen cells can easily clog tiny blood vessels and deprive organs and tissues of vital oxygen, causing intractable pain crises. In addition, children with sickle cell are more prone to stroke, pneumonia, infection and other long-term complications.
Although the disorder can strike all races, it is more common among people of African, Mediterranean, Asian Indian and American Indian descent.
Berrutha Harper's son Kerry Norwood was just 2 when he was diagnosed with sickle cell disease.
"I noticed that a lot of the time he was sluggish, just laid around, and took long naps," said Harper, who works at an asthma and allergy clinic. "He didn't have a lot of energy."
Because she worked with physicians, Harper started asking questions. She was referred to a hematology clinic, where another doctor delivered the devastating news.
"He said 'This child has a disease and is probably not going to live to be 6 years old,'" she remembered. "I cried for a while, then I wiped my eyes and started doing research."
Thirty years and then some have passed since the day Harper's son was given four more years to live. Now 33, Norwood is employed and living on his own.
"Sometimes he's sick, but he's a fighter," said Harper, who has been active with a support group for parents of children with sickle cell for many years. She is also a founder of Grady's Sickle Cell Center.
"We're starting to understand the interaction of sickle blood with blood vessels," said Eckman. "That's being now applied to treatments that actually improve outcome."
Results of the STOP and STOP 2 studies - for "Stroke Prevention in Sickle Cell Anemia" -- have been particularly valuable, the physician said.
"Children are identified as at high risk for stroke by using a transcranial Doppler (a device that measures blood flow)," he said. "These are started on a transfusion program, which can (result in) a dramatic reduction in stroke."
Transfusion entails its own risk, including the possibility of iron overload. So transfused children need to be closely monitored, Eckman said.
Other studies are ongoing using compounds such as nitric oxide, N3 fatty acids and hydroxyurea, a chemotherapy agent used for cancer treatment. Hydroxyurea is the only FDA approved therapy for preventing pain crises related to sickle cell. Early evidence indicates that using N3 fatty acids - fish oil - also may have a positive impact on pain.
Research on bone marrow and stem cell transplants also been promising. But such procedures carry risks too.
"Bone marrow transplant is moving forward slowly," said Eckman.
Research on treatments is the only hope that patients with sickle cell have because the disease is theirs for life. As adults, sickle cell patients are prone to progressive lung disease and kidney failure. They also get more than their share of degenerative arthritis in the hips and shoulders that can lead to progressive disability.
"Drug companies are interested in diseases that millions of people get," said Eckman. "That's where the profits are."
U.S. Representative Donna Christian-Christiansen, a Democrat from the Virgin Islands, told CNN that "meaningful legislation" was required before true progress could be made on the disease.
"We have 10 centers, but that has not been increased," she said. "The funding has not been increased."
George Strait, a vice president for media distribution with the Dr. Spock Company, said "even the surgeon general (Dr. David Satcher) has said that cystic fibrosis affects half as many people as sickle cell but gets twice as much money (for research)."
Genetic advances should follow the mapping of the human genome. But a good first step, all agree, is advancing public awareness of the disease.
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