sickle cell anemia
1:05 pm
Thu January 30, 2014

SLU Tests New Treatment For Easing Pain Of Sickle Cell Anemia

With her oversize black frame glasses, 9-year-old Diamond Jones projects an image of being a kid who is thoughtful, inclined to study, and who loves reading and attending school. All that is true. In fact, she’s so adamant about going to school that she has been known to pout occasionally on days when her parents keep her home for health reasons. 

Her 11-year-old brother, Dayvin, also has to cope with the same sense of confinement. During winter months, he isn’t allowed to dash out the front door and romp in the snow. On hot days, he’s forbidden to rush to a community pool for vigorous water fights with other youngsters.

As children with sickle cell anemia, the brother and sister are learning to accept such restrictions as well as the excruciating pain associated with the disease. 

The Jones family (from left) Delores, Diamond, Davon, and Dayvin.
The Jones family (from left) Delores, Diamond, Davon, and Dayvin.
Credit Robert Joiner

Sickle cell anemia is an inherited blood disease where there aren't enough healthy red blood cells to carry adequate oxygen through the body. The disease disproportionately affects African Americans.

Ordinarily, red blood cells are shaped like donuts, flexible enough to move through even small vessels. But the red blood cells in the bodies of youngsters like the Jones have a crescent-like shape. Their sharp edges trap the cells inside blood vessel walls where they pile up and create blockage.  When that happens, the flow of blood to vital organs and limbs is reduced, causing great pain for several days or even weeks at a time.

Some parents of children with sickle cell anemia are putting hope in a new approach to easing the blockage and pain, a technique being pursued by Saint Louis University researchers. Scientists there believe that one drug already on the market could be used to help ease the pain and shorten the time patients are hospitalized during a crisis. This, at least, is the theory of Dr. William Ferguson, the chief researcher and head of pediatric hematology and oncology at the university. The drug is expected to be tested on about 100 people.

“We are very excited about this,” Ferguson said. “We see kids and adults with a pain crisis all the time. It’s a pretty frequent cause of (hospital) admissions among African Americans. It's heartbreaking to see people suffer and not be able to do anything other than control the symptoms.”

The drug is abciximab, which goes by the brand name of ReoPro®. It’s commonly administered to heart patients undergoing angioplasty to open blocked arteries. The drug has the potential to address  blockage caused by sickle cell anemia by inhibiting the red blood cells from sticking to blood vessel walls.

“A lot of the symptoms of sickle cell anemic aren’t related to the anemia, but are related to this tendency (of vessels) to get plugged up and literally cut off the blood supply,” Ferguson said.

The Jones family, which lives in Hillsdale, is upbeat about coping with the illness.  

“Although they have sickle cell, they still are treated like regular kids,” said Davon Jones, the father. The couple has learned to improvise and relax some restrictions on the activities they want their kids to enjoy. 

For example, they're aware that being exposed to extreme temperature changes can slow the flow blood in the children’s bodies and cause problems. Delores said that the kids aren’t supposed to go outside and play in the snow, but “we’ll let them do that anyway. You just have to time it. Go out 10 minutes, say, and come back in.”

She also confided that she and her husband have been known to allow the kids to have snow fights in the house.

“We let them be kids, just a little bit,” she said. “Although they are not supposed to go out in hot weather, we’ll go to a hotel and let them swim indoors. We still do things; we just have to do them differently.”

She noted that Dayvin has to undergo blood transfusions, among other treatments. “These can be very expensive,” Jones said. And she's happy that the youngsters have health insurance, thanks to Missouri’s relatively generous Medicaid rules for children. 

Ferguson, the researcher, said the disease itself occurs in about one of every 400 African-American births, and roughly one in every 12 African Americans carry the genetic trait. The illness can occur in a child when both parents have sickle cell traits. In such cases, there is a 50 percent chance that the baby will have sickle cell trait and a 25 percent chance the baby will have sickle cell disease.

Missouri has had a law in place since 1989 requiring that all babies be tested for sickle cell conditions. But Delores Jones said she didn’t know about the illness or that she herself carried the sickle cell trait until young Dayvin began having difficulty walking at age 2. 

“He’d try to get up and walk and his foot would cramp up,” she said. “When I asked a nurse about it, she told me he had sickle cell disease. At that time Davon was walking across the room with Diamond. The nurse asked about Diamond and said she had it, too.”

Jones said she and her husband left the medical office confused and shocked.  They gradually realized that sickle cell had been on their radar as “one of those things you always heard about but never knew much about.”

The illness is also common among people in parts of Africa, South America, Central America and Cuba; Saudi Arabia, Indian and Mediterranean countries, including Turkey, Greece and Italy, according to the Missouri Department of Health and Senior Services.

“I will see patients who are Caucasians who have sickle cell disease,” Ferguson said. “But it’s most common among African Americans and a little bit less common among Hispanics.”

The hope, he said, is that the new use of the medicine will offer a way to decrease the intensity and duration of the pain resulting from a sickle cell crisis.

“People recover from the pain over time, but you can get long-term organ damage, problems with blockage of blood vessels to the brain,” Ferguson said. “Life expectancy of someone with sickle cell anemia is in the mid-50s, compared to the 70s and 80s for people who don’t have sickle cell disease.”

Peter Ruminski, Ferguson's colleague who heads the university’s Center for World Health and Medicine, said the disease is neglected even though it affects many African Americans. He hopes the university’s work will change the quality of life and shortened lifespan of many victims of the illness.