MDs launch network to treat Sickle Cell
By ANDALE GROSS
Associated Press Writer
KANSAS CITY, Mo. (AP) — Ernestine Diamond says her intensely painful sickle cell crises started when she was a baby, but her doctors back then knew nothing about the disease. It wasn’t until she had complications during the birth of one of her own children about 20 years later that she learned she had sickle cell anemia — an inherited disorder in which defective hemoglobin causes red blood cells to take on a sickle or crescent shape, hindering blood flow and depriving organs and tissues of oxygen.
Now, at age 80, Diamond is proof that sickle cell doesn’t have to be a death sentence and that patients can live well into adulthood. But she and others on the disease’s front line say the health care community is lagging when it comes to providing adult sickle cell patients with consistent care.
”You face problems being an adult sickle cell patient,” said Diamond of Kansas City, Mo. ”You really have to fight to get support.”
A network of health care professionals and researchers from Missouri, Kansas and more than 30 other states is working to do something doctors and other advocates have been trying to do for years: improve the sickle cell landscape so it better addresses the needs of adult patients. The group — called the Sickle Cell Adult Provider Network — is developing what its members say will be the first-ever comprehensive set of best practices for doctors and nurses to follow when treating adult sickle cell patients.
Sickle cell specialist Dr. Kathryn Hassell of Denver, who formed the network, said the first phase of the practices will be available in the next few months.
”Everybody who works with sickle cell recognizes it’s time to start paying attention to the adults,” said Hassell, director of the Colorado Sickle Cell Treatment and Research Center. ”We’re so good at raising the children and keeping them healthier into adulthood. It’s time to do that for the adults.”
The practices will cover such issues as how to better manage pain for adults who have sickle cell pain crises, and whether regular blood transfusions can improve conditions of adult patients like they do children.
”I have younger patients who get blood exchanges once a month, and I don’t think they should be denied that once they’re adults just because there’s no research,” said Elizabeth Nelson, a University of Missouri Health Care nurse clinician and member of the Sickle Cell Adult Provider Network.
Besides feeling intense pain throughout the body for days or weeks, sickle cell patients also can suffer breathing problems and be more at risk of having a stroke or developing an infection or organ failure.
Patients generally are given pain medicine and intravenous fluids during a sickle cell pain crisis. If complications arise, they might be given a blood transfusion.
The number of people in the U.S. who have sickle cell has risen about 42 percent in recent years, said representatives of both the Sickle Cell Adult Provider Network and Sickle Cell Disease Association of America. It’s now around 100,000, they said, with more adults with the disease living longer.
Sickle cell mainly affects blacks, but also a growing number of Hispanics.
”In general people are living longer, but that shift is even more dramatic in sickle cell because of the improvements in treatment,” Hassell said. ”Now, we fully expect people with sickle cell disease to live into their 40s and 50s and even into their 60s and 70s.”
For years, sickle cell was seen as a young person’s disease.
”People honestly didn’t think people with sickle cell would live to their 40th birthday,” said Joseph Telfair, public health research professor at the University of North Carolina at Greensboro.
Better treatment for infections and pain crises has led to patients living much longer, Telfair said.
”Now, you have really healthy persons with sickle cell disease who are anywhere between 18 and over who are now showing up (at hospitals and doctor’s offices), and people don’t know how to treat them,” he said.
Dr. Zahida Yasin, who’s also a member of the Sickle Cell Adult Provider Network, said there needs to be more treatment centers for adults who have sickle cell.
”We are at the same crossroads with adults with sickle cell disease that we were with the children 30 or 40 years ago,” said Yasin, an associate hematology/oncology professor at the University of Cincinnati.
Truman Medical Center in Kansas City, Mo., has an adult sickle cell center. Within the last year, the sickle cell center was redesigned to update the examination rooms and other areas.
”We are trying to provide a very calm, relaxing, therapeutic environment, because that’s so important to sickle cell patients,” said Dr. Angela Garner, the center’s medical director.
Diamond is among the center’s 130 patients. She said the center helps patients who have received consistent treatment in their younger years continue to get the proper care as adults.
Patient Sherry Webb, 41, also of Kansas City, Mo., said she likes how the center designs treatment plans that fit each patient’s specific needs.
”We all have our different needs, and what might work for me might not work for the next person,” said Webb, whose pain crises are usually sparked by stress.
Diamond and Webb said it’s comforting to know that the center’s staff of three keeps regular, daily hours and has sickle cell expertise.
But despite bright spots like the center at Truman, they said the struggle continues for adult sickle cell patients.
”Things are coming up for us,” Diamond said. ”But they’re slow in coming.”