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Living with the Chronic Pain of Sickle Cell Disease

December 08, 2014, 12:00 PM
Living with the Chronic Pain of Sickle Cell Disease
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Semaj Bailey can vividly recall her first sickle cell disease “pain crisis” even though it happened nearly 20 years ago.

She was working as a stunt performer on the Austin set of the 1997 TV miniseries “True Women.” Bailey — who started riding horses almost as soon as she could walk — was there to serve as a stunt double for the more rigorous riding segments.

Bailey remembers getting sick on the set and passing out. “The next thing I knew I was in a hospital getting a blood transfusion,” the 37-year-old says.

It was the first of dozens of “pain crises” she would experience in the ensuing years. “It feels as if someone is going up and down your body with a jackhammer,” says Bailey, who loves barrel racing. “You have trouble breathing and fever.”

She averages three to five episodes of intense pain a year, and many lead to hospitalizations.

“People with sickle cell disease like Ms. Bailey often live with chronic pain,” says Modupe Idowu, M.D., who is Bailey’s primary hematologist and an assistant professor in the Division of Hematology at The University of Texas Health Science Center at Houston (UTHealth) Medical School.

The disease disfigures the red blood cells that transport oxygen through your body. Normally shaped like a donut, they become crescent or sickle shaped. This slows blood circulation and robs the body of oxygen.

The cells also don’t live as long. Whereas normal red blood cells last for 120 days in the bloodstream, the red blood cells of people with sickle cell disease may only survive for 10 to 20 days. This leads to anemia and may make patients tired.

Sickle cell disease is passed from parents to child. For example, if both mother and father have the sickle cell trait, their child may develop sickle cell disease. If one parent has the trait and the other one is unaffected, there is typically no risk of passing sickle cell disease to the child.

Bailey’s only other relative with the disease was a first cousin who died at the age of 29.

Idowu encourages her patients to stay active, drink plenty of water, cut down on caffeinated beverages, avoid extreme temperatures and take their medications as prescribed. Many patients are unable to maintain a job because of their frequent hospitalizations. However, her patients include an electrical engineer and a nurse practitioner.

“I see Ms. Bailey as someone who has risen to the challenge and who is working to do something with her life,” Idowu says.

Bailey previously worked as a medical insurance claims processor and a certified nurse assistant, and she is now taking courses to become a real estate agent. “Being a realtor would enable me to work out of my house, and I could meet clients as needed,” says Bailey, whose first name is her father’s middle name — James — spelled backward.

Right now, the only disease-modifying drug for sickle cell anemia approved by the Food and Drug Administration is hydroxyurea. It may lessen the incidence of intense pain cycles; however, some patients experience side effects.

Millions have the disease worldwide and 90,000 to 100,000 in the United States. It is particularly common among people whose ancestors came from sub-Saharan Africa, Spanish-speaking regions in the Western Hemisphere and Mediterranean countries like Greece, Turkey and Italy, reports the Centers for Disease Control and Prevention.

Sickle cell disease can reduce life expectancy, stunt growth, increase the risk of infection, cause severe anemia and damage organs.

Looking ahead, Idowu says promising treatments include stem cell transplantation and targeted medications.

Stem cell transplantation has been shown to be curative for sickle cell disease patients who do not reject the transplant. Only patients with a brother or sister who meet the criteria for a stem cell transplant are eligible. Bailey has no siblings.

Another approach involves the development of drugs that target the molecular mechanisms responsible for the damage to the red blood cells.

At UTHealth, clinicians like Idowu and basic scientists like Yang Xia, M.D., Ph.D., professor in the Department of Biochemistry and Molecular Biology at UTHealth Medical School, are working together to develop a targeted drug treatment, and their research worked in a mouse model of sickle cell disease.

If you examine the blood of people with sickle cell disease, you will find high levels of a small molecule called sphingosine-1-phosphate. When researchers manipulated the levels of the molecule in an animal model, they reduced sickling.

“We confirmed our findings in isolated blood cells from patients with sickle cell disease,” says Xia, who led the study and who believes clinical trials could be in the offing. “Our research could lead to therapeutic opportunities.”

Xia also is on the faculty of The University of Texas Graduate School of Biomedical Sciences at Houston.

Today, Bailey believes there is a greater public understanding of the nature of sickle cell disease than in the past. “Some people used to think it was contagious,” she says.

For Bailey, who has two sons, Jemaj Bailey, 11, and Robert Bailey, Jr., 9, a new treatment can’t come soon enough. “I hope they find a cure. I’m living day to day. I want to see my children grown,” she says.

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