Muncie Times, Muncie, Delaware County, 2 January 1992 — Page 9

The Muricie Times, Thursday, January 2, 1992, Page 9

Girl Recovers After Bone Marrow Transplant

Charleston, S.C.-Angela and Paul Roberts of Summerville, S.C., faced a dilemma when their daughter, Tangela was diagnosed as having sickle cell anemia. The child knew little but pain during her first 3-1/2 years. She had been hospitalized many times for pain control. Her prospects were for a lifetime of illness, and possible debilitating strokes and other complications associated with sickle cell disease. Then the couple was told a bone marrow transplant could cure her sickle cell disease, free her from suffering and give her a chance at a normal life. However, the procedure was not without risk. The risk of death could be as high as 20 percent, the parents were told. But they agreed to the bone marrow transplantation for their daughter. On Oct. 23, at the Medical ypiy<a$ty. ol Sputh Carolina (Muse) Children’s Hospital, Dr. Miguel Abboud, an assistant professor, and Dr. Joseph Laver, director of hematology/oncology, performed the

transplant. It was the first bone marrow transplant on a sickle cell patient in the United States who had not already suffered from a stroke or other life-threatening problem. Because of the ethical issues involved with recommending a treatment that could result in death, the physicians consulted with a panel that was established to deal with such issues. The panel worked closely with the doctors and family in helping to make the decision. The panel consisted of two physicians outside of MUSC, with expertise in sickle cell disease and bone marrow transplantation, an MUSC general pediatrician and a lay community leader. “Making the decision was the most difficult thing we ever did,” said Paul Roberts, an instructor in the electronics division of the Ha\y Subman^ Facility in Charleston. “We thoroughly evaluated all of the alternatives. My wife and I contacted and sought the advice of sickle cell associations and research

For such a transplant to be successful, a compatible sibling usually is the best possible donor. Now a little more that a month after the transplant, the doctors are cautiously optimistic. Tangela has done remarkably well during the immediate posttransplant period, which is considered to be the riskiest time. There is still a small risk of graft-versus-host disease, where the grafter bone marrow acts against the host’s system. Tangela is fully engrafted with her brother’s marrow. However, there is a remote possibility that her own marrow will return. It will take about a year to completely certain that she is fully cured of her sickle cell disease, doctors said, centers across the nation.” Added Mrs. Roberts: “We realized that if we did nothing, we were making ^' decision. That decision was to condemn our daughter to a life of physical pain and suffering. She couldn’t go to school, play outside, have friends and lead a normal life.”

left, was the donor. Tangela had been hospit- was determined that 7-year-alized 14 times with sickle old brother, Paul, was an cell pain crises in the 18 excellent donor candidate months prior to the trans- for a transplant, plant. During that time, it

250,000 Sickle Cell Anemia Babies Born Every Year

Sickle cell disease is an inherited blood disease in which red blood cells elongate and become rigid, thus clogging capillaries. The blockage prevents oxygen normally carried in the red blood cells from reaching tissues. This can cause bouts of pain, damage to vital organs and, for some, death in childhood or early adulthood. The effects of this disease vary greatly. Sickle cell anemia is a worldwide health problem affecting many races, countries and ethnic groups. The World Health Organization estimates that each year more than 250,000 babies are bom worldwide

with this disorder. In the United States, sickle cell anemia is one of the most common hereditary disorders among people of African descent. About one in 400 black newborns in the United States has sickle cell anemia. One in 10 black Americans carries the sickle cell trait. This means such people can pass the defect on to offspring, even though their own health is unaffected. In South Carolina, 348 children under the age of 4 have been identified through newborn screening as having the sickle cell trait-not tile disease-during a screening of newborns in the ffrst 6 months of 1991.

Treatment of sickle cell anemia consists of supportive care, with emphasis on pain relief and the prevention and treatment of infections and other complications. If this type of supportive care starts early in childhood, mortality can be greatly reduced. The vast majority of children survive into adulthood. It is known however, that patients who have frequent pain crises are at a higher risk for organ damage or death in their 30s. Bone marrow transplantation is the only known way to cure the disease. However, until now, bone marrow transplants have only been performed when sickle

cell patients have had other complications such as strokes, organ failure or other life-threatening complications. The procedure involves the replacement of cells that make sidded hemoglobin with cells from a normal donor, usually a matched sister of brother. If successful, the procedure results in cure of the anemia and the disappearance of symptoms, such as pain episodes. Potentially, the damage to vital organs will be slowed, if not completely stopped. Overall, bone marrow transplantation is effective in about 80 percent of the

cases. However, the chance of cure must be balanced by tha* risks of the procedure, which include a 10 to 20 percent risk of death. The risks are high because the patient must be prepared to accept the donor graft by oblating his or her immune and marrow cells by administration of chemotherapeutic drugs before transplant. The risks of transplantation include rejection of the donor bone marrow, infection as a result of diminishing the body’s defenses through chemotherapy and graft-versus-host disease. The transplant protocol is designed to minimize the risks of these complications.