Experts sounding sickle cell alert

Patricia Thomas knew nothing of sickle cell anemia until doctors performed blood test during the fifth month of her first pregnancy. It was then that both Thomas and her husband tested positive for the sickle cell trait.

“(Sickle cell) testing was not done in my country,” said Thomas, a native of Jamaica. “The doctors warned me of the risks involved with both me and my husband being positive but this was my first child so I decided to take my chances.”

People with sickle cell trait do not have sickle cell disease but are carriers who can pass it on to others who then can get the disease, said Karen Smalls, president of the Sickle Cell Disease Association of Broward County.

A child being born with the disease is strictly a matter of genetics, said Dr. Astrid Mack, retired associate dean for minority affairs at University of Miami Miller School of Medicine. “There is no way around avoiding that 75 percent risk.”

If both parents have the trait and don't want to give birth to a child with sickle cell, the mother can have an amniocentesis test done to determine whether the child would have the disease, Mack said.

“If the test is positive for the disease, doctors then give the mother a choice to abort,” he said.

At 2 months old, Thomas’ son, Dennis Matthew Williams, was diagnosed with fully blown sickle cell disease.

“I told them it was a mistake when I heard the news,” Thomas, of Fort Lauderdale, said. “It couldn’t be true, I thought. They had to be wrong. This was my child, my first child.”

Dennis Williams died in May from complications of sickle cell disease. He was 27.

Thomas, in memory of her son’s’ life, joined her daughter Tricie Thomas, 17, and other participants in the Nov. 3 “2012 Cell-ebration” 5k Run/Walk with the theme “Break This Sickle Cycle.” The event, in its 35th year, took place at Central Broward Regional Park in Lauderhill, attracting about 100 runners and walkers.

The Sickle Cell Disease Association of Broward County, the Sickle Cell Foundation of Palm Beach County & Treasure Coast Inc. and the Fort Lauderdale Chapter, The Links, Incorporated, hosted the event.

Sponsors included Broward Health, Geriatric and Palliative Care of South Florida, Broward County Commissioner Dale Holness and North Lauderdale Mayor Jack Brady.

Thomas was not alone when she said she did not know that sickle cell disease and the trait are common among African Americans, said Frank Hayden, CEO, Sickle Cell Foundation of Palm Beach County.

“It’s a conversation we need to have so we can help each other,” Hayden said. “People are reluctant to talk about it, as if it’s a family secret. Meanwhile, people suffer from not knowing.”

Prevalent among people from Africa, Asia and the Pacific Coast, sickle cell disease is more common to those of African heritage, Hayden said. The ailment affects about one in every 500 blacks or African Americans. The trait occurs among about one in 12 blacks or African Americans.

Mack said the majority of black babies born in Miami with the disease are of Haitian descent. “The frequency in the [Haitian] gene is far greater than that of blacks born in America,” he said.

Sickle cell disease belongs to a group of inherited red blood cell disorders. Normal red blood cells are round like doughnuts and move through small blood tubes in the body to deliver oxygen. Sickle red blood cells become hard, sticky and shaped like sickles used to cut wheat.

When hard and pointed, red cells passing through the small blood vessel clog the artery and break apart, causing severe pain.

“A blood transfusion can be given but you can never remove all of the sickle from the system,” Hayden explained. “Thus the procedure is helpful but temporary. How long depends on the patient.”
Symptoms of the disease include lasting irritation and extreme fatigue, Hayden said.
“Sickle cell can be deadly and will break your body down,” Thomas said. “People need to know about it. My son wanted to live.”

The overall winners of the run were Otis Sanders III, 29, and Rachelle Ginsberg, 34, both of Fort Lauderdale. All participants received medals.

• For more information about sickle cell disease, treatment or testing in Palm Beach County, visit www.sicklecellpalmbeach.org or call 561-833-3113.