Headlines

As reported by the Danbury News-Times, January 20, 2008.

State Wants to Take on Sickle Cell Disease

Program Would Help Track Care for Patients

By Robert Miller

When Darryl Ramseur went with his classmates at South Street School in Danbury to a nature camp this September, he scrambled to the top of the climbing wall there and rang a bell to cap his achievement.

"He manages really well," said South Street School nurse Kathy Boylan. "You can't pick him out from any of his classmates."

"He's a normal 10-year-old," said his mother, Sharon Hatch.

Except that once a month, Darryl needs a blood transfusion.

"I don't like that," he said.

Asked about the reason why he needs the transfusions -- because he has sickle cell disease -- Darryl is just as adamant.

"I don't like it," he said.

With good reason. Sickle cell disease -- the most common inherited blood disease in the United States -- presents its patients with a life of medical complications. Doctors are now much better about extending the lives of sickle patients past childhood into adult years. But the problems never go away.

"The care for sickle cell disease is better and the treatment is better," said Dr. Byron Thomas, an internist at Danbury Hospital who has adults with sickle cell disease, also known as sickle cell anemia, as patients. "The problems they have are the same as children. They can be chronically anemic. They can have swelling and pain. I've had patients with kidney disease. They have to worry about heart disease and stroke. They can lose their spleens."

And, Sharon Hatch said, there's not always a lot of information available -- especially at the community level.

"Not a lot of people know about sickle cell," she said.

The state of Connecticut is determined to change that. Last week, the Department of Public Health announced it will begin a major initiative to provide comprehensive care for sickle cell patients in the state.

"We are so happy about this," said Norma Gyle, of New Fairfield, the deputy commissioner of the state Department of Public Health, who started working on sickle cell issues when she served in the General Assembly several years ago. "We want to be able to treat the 9-year-olds and the adults who are 30 to 40 years old and offer them the best care we can."

The program -- which could cost as much as $5.8 million -- will use the Hospital for Special Care in New Britain -- an institution dedicated to treating patients with chronic diseases -- as its lead agency. But Yale-New Haven Hospital, the University of Connecticut Medical Center in Farmington and the Children's Medical Center in Hartford will also take part in the program. So will the public health department and groups like the Southern Regional Sickle Cell Association Inc. in Bridgeport, and Citizens for Quality Sickle Cell Care, based in New Britain.

The program will create a statewide coordination center for state residents that will track and monitor sickle cell care in the state. It will increase community education and advocacy programs and create two hospitals in the state -- one in the northern part of the state, one in the southern part -- that will be centers of excellence for sickle cell care.

"We use the term 'medical home' sometimes," said Roger Thrall, a professor of medicine at the University of Connecticut Health Center and director of research at the Hospital For Special Care. "What we want to do is find a medical home for every sickle cell disease patient in the state."

And, Thrall said, such care will also help when a child grows into adulthood and can no longer get pediatric care for sickle cell disease, something he said is happening with increasing frequency.

Such a program is needed, advocates say. The Department of Public Health estimates it cost about $14.4 million in 2005 to treat sickle cell patients in the state. Of that, about $10.6 million was spent caring for adults, even though adults only comprise about half the sickle cell population.

"The cost is onerous to the health community and to the institutions," said Dr. Lawrence Solomon, clinical professor of medicine at the Yale School of Medicine. "But the effect is really felt on the quality of the lives of these patients and their families."

Sickle cell disease is the most common inherited blood disease in the United States, affecting around 70,000 people. Worldwide it affects millions.

Since 1964, Connecticut has screened newborns for sickle cells disease. That screening program has found nearly 400 babies with the disease, as well as nearly 12,000 people with the sickle cell trait -- the genetic mutation that can cause the disease.

Sickle cell disease alters the red blood cells. Normally, a red blood cell is round and malleable -- it squeezes through tight spaces. It also lives for about 120 days before being retired and gathered in the spleen.

If a person has sickle cell disease, however, their red blood cells are crescent-shaped. They're both more rigid and fragile than normal cells. These cells only live for about 16 days, so people with the disease often suffer from chronic anemia.

But these sickle-shaped cells don't squeeze easily through the tiny blood vessels of the body. They can pile up, blocking blood flow to the organs and extremities. The disease can also cause severe pain in different areas of the body -- in the abdomen and chest, in the long bones and in the hands and feet -- and eventually damages organs like the kidney and liver.

Because a person's spleen collects aging red blood cells, sickle cell patients often have problems with their spleens, which just wear out from too much work. In turn, people without spleens are more prone to infection.

But Dr. John Ertl, a pediatrician who is Darryl Ramseur's doctor, said the treatment of sickle cell disease has improved greatly. Children now receive antibiotics to keep infections from developing. They also get pneumococcal vaccines to prevent other infections.

"We're doing better to treat a lot of the complications," said Ertl, who practices with Pediatric Associates of Western Connecticut in Danbury. "But we still haven't found a treatment that gets rid of the disease."

But things are improving. Sharon Hatch said Darryl used to have regular, severe medical crises when he was an infant. By now, she said, her son is doing much better -- fewer medical emergencies, less pain.

"It's not the extremes he used to have," she said.

And if she keeps him away from heavy contact sports like football, she lets him play others, like baseball. She also lets him read whatever he can understand about sickle cell disease.

"I want him to know," she said. "I want him to get books, and understand, so that as he gets older, he'll be able to deal with whatever comes up."