Among those taking part in the Sickle Cell Clinic’s new transition program for patients 16 and older are, from left, Annaneia Williams, Special Lawrence, Joshua Mitchell and Marque Sweeting. (photo by Susan Urmy)
Sickle Cell Clinic adds programs, options
Typical red blood cells are doughnut-shaped and glide easily through the tiniest blood vessels.
For patients with sickle cell disease, however, red blood cells are shaped like crescent moons and are hard and sticky. They block blood flow, causing pain, organ damage and stroke, and they decay quickly, resulting in anemia.
The Sickle Cell Clinic at the Monroe Carell Jr. Children's Hospital at Vanderbilt has been re-energized to give young patients their best chance at fighting this chronic disease.
“Life expectancy is in the 40s, but that number has been rising sharply,” said Elizabeth Yang, M.D., Ph.D., associate professor of Pediatrics and director of the clinic. “We want our patients to beat that. The outlook is positive if they start early and learn to take care of their bodies.”
In the U.S., sickle cell disease affects about 70,000 people, and about 2 million carry the genetic trait. The disease is most common in African-Americans, occurring in about one in every 500 births.
The clinic currently follows 260 patients, with more than 700 clinic visits last year. Each Tuesday, Yang and nurse specialist Lesley Ann Owen, M.S.N., R.N., provide comprehensive care and education to patients and families.
Diagnosis of sickle cell disease usually comes at birth through newborn screening. For infants and toddlers, the focus is on educating parents about the disease, and Yang has developed educational materials.
There is also a parent support group that discusses issues like government benefits, day care and coping.
Tinesha Weddle has been bringing her 2-year-old daughter Alyssa to Children's Hospital since the disease was discovered at her newborn screening.
“The clinic cares for all our needs, whether they are medical, outreach or the support group,” she said. “I know I'm not the only one dealing with the same emotional stress, and getting info from other parents is great.”
For children ages 4-15, the focus is on receiving proper treatment and following prevention guidelines.
“Sickle cell disease is very different now than 10 years ago. There is much greater understanding and a lot of state-of-the-art research on this complex disease,” Yang said.
“We have many treatment options today, and we want patients and families to benefit from the increased knowledge and to realize they have some control over this disease.”
Once patients turn 16 they begin an innovative new aspect of the clinic's care — the transition clinic.
These special seminars and group discussions prepare the teens to transfer to adult care and be responsible for their own health.
“Transition is a big problem for young adults, and the fall-off numbers are large,” Yang said. “We want them to understand more about their disease and to be more involved in their own health maintenance. But ultimately we want to help them set goals and feel proud of themselves and what they can accomplish.”
The transition clinic was initiated with the help of Laura Winslow, R.N., F.N.P., and Adetola Kassim, M.D., from the sickle cell clinic for adult patients. One of the sessions includes a tour of the adult side and a meeting with Kassim, so the patients are comfortable transferring their care.
Owen now runs the transition program with participation from several other disciplines, and the curriculum includes managing disease complications, insurance, job training, safe sex and genetic counseling.
“They have taught me things I didn't know before, like things that could happen if I get sick, how to keep myself healthy, why I need to take my meds and how to set a schedule to keep taking my meds,” said 17-year-old Devante Harden.
“When I do go to the adult hospital, I will know what to do and where to go,” added 17-year-old Marque Sweeting.
On Dec. 8, eight patients will graduate from the transition program, and some have already continued their care with Kassim.