Raising the Issue of Sickle Cell; Genetic Disease Most Serious and Common among Blacks

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Benjamin Clark looks like a healthy teenager. He likes to play video games and dodge ball. An eighth-grader at Garnett Patterson Middle School in Northwest, he likes to study math and science.

However, Benjamin, 15, was diagnosed with sickle cell disease at birth. He has had 43 blood transfusions and has been hospitalized 90 times. His spleen was removed in 1996.

"It's painful and so much suffering," Benjamin says. "It's so much trouble."

Sickle cell disease, a genetic condition, occurs in about 1 in 400 black births and 1 in 1,400 Hispanic births in the United States, according to the Sickle Cell Disease Association of America in Baltimore. Eight thousand people in the United States have the illness, and 2.5 million people nationwide carry the sickle cell trait.

Pain crises are one of the most debilitating aspects of sickle cell disease, Benjamin says. A blood transfusion relieves a crisis and usually prevents another one for three to four months.

"If you've ever seen a lady in labor, that's how bad his pain is during a real bad crisis," says his 48-year-old single mother, Deborah Clark. Benjamin has four brothers. The three oldest carry the sickle cell trait but do not have the disease. The youngest brother has neither the disease nor the trait.

Benjamin and his mother have tried to raise awareness about sickle cell disease through the nonprofit organization Faces of Our Children, founded by Donald Cash Sr. in 1999. The group has its national headquarters in Danville, Va.

On Nov. 26, the organization is holding a Gospel Fest at Israel Baptist Church, 1251 Saratoga Ave. NE. The fest will include dinner from noon to 4 p.m. for $8. Luther Barnes and the Sunset Jubilaires will perform at 4 p.m. for $15. Tickets can be obtained by calling Mikki Harris at 301/459-3400, Ext. 279.

"We raise money specifically for the education of sickle cell disease," Mr. Cash says. "The disease can be eliminated through education."

Newborn screenings for sickle cell disease are mandatory in most states, says Dr. Sohail Rana, a professor in the department of pediatrics and director of hematology services at Howard University Hospital in Northwest. Benjamin is his patient.

Hemoglobin fills the red cells in the body. The cells must be flexible to pass through the body's tiny capillaries, so the hemoglobin needs to stay liquid through the process.

When oxygen in the blood is low, sickle hemoglobin forms a gel, making the cells less flexible. If all the hemoglobin becomes crystallized, the cells can get stuck in smaller blood vessels and stop the flow of blood into tissues, causing a pain crisis and tissue damage, Dr. Rana says.

"Pain crises are just one of the problems," Dr. Rana says. "Almost all children with sickle cell disease, by the time they are 4 or 5, have lost their spleen. Most children have suffered damage to their kidneys."

Low red blood count - anemia - can make sickle cell patients tired. Other complications include the possibility of strokes and serious infections. Transfusions help reduce the risk of stroke while up-to-date immunizations and antibiotics help stave off infections. Pulmonary hypertension and serious lung disease can be other complications.

"Not enough attention is paid to this disease," Dr. Rana says. "It is the most common serious genetic disease in African Americans."

Genetic counseling can reduce the number of new cases of sickle cell disease, he says. Two people with sickle cell trait have a 1-in-4 chance of having a child with sickle cell disease.

Although there isn't a certain cure for sickle cell disease, a bone-marrow transplant holds potential. …