Occurs in about one out of every 500 African American births
09-01-2009 (Dayton, OH) -
Sickle cell disease affects more than 80,000 Americans. According to the Sickle Cell Disease Association of America (SCDAA), approximately 1,000 babies are born with sickle cell disease each year in the U.S. In Ohio, approximately 70 babies are born with the disease each year. Locally, Dayton Children’s provides care for 137 pediatric patients who are affected by sickle cell disease.
Sickle cell disease (SCD) is the term used to describe a group of inherited blood disorders characterized by the predominance of “hemoglobin S” (sickle hemoglobin) in the red blood cells. Hemoglobin is a protein substance found in the red blood cells that helps carry oxygen throughout the body. Normal red blood cells are round, soft and flexible and can easily flow through blood vessels. However, when sickle blood cells loses its oxygen, a change in the structure takes place causing them to become sticky, hard and “sickle” or crescent shape. As a result, the sickle blood cells lead to blockage or “log jam” in the small blood vessels which deprives the body’s cells and tissues of blood and oxygen.
Many people often think that sickle cell disease affects only African-Americans, but Mukund Dole, MD, pediatric hematologist and director of the sickle cell program at Dayton Children’s, says that is not always the case. “Sickle cell disease has also been reported in other ethnic groups with heritage from South and Central America, Africa, parts of Asia and the Mediterranean region.”
Sickle cell disease is inherited (passed on from parent to child). At conception, if both genes for sickle hemoglobin are inherited, the baby will be born with sickle cell disease. Individuals who inherit only one gene for sickle hemoglobin and one gene for normal hemoglobin have sickle cell trait. Individuals with sickle cell trait are usually healthy, although they can pass the gene for sickle hemoglobin onto their children. There are approximately 3.5 million Americans that have sickle cell trait.
Symptoms of sickle cell disease include lifelong anemia, jaundice, episodes of pain (pain “crises”), delayed growth, infections, abnormal organ function (leading to kidney failure, heart failure), joint problems and other health issues. Most symptoms of sickle cell disease appear after four to six months of age. To accurately diagnose sickle cell disease, a special blood test, called Hemoglobin Electrophoresis, is needed. That was how Chardanai White, one of Dayton Children’s ambassadors, was diagnosed.
Less than two weeks after Tobi and Charles White brought their newborn baby girl home, they received a call from Dayton Children’s instructing them to bring her into the hospital for more testing. They were told that something came through on her newborn screening test that did not look right. After bringing her to the hospital and getting some additional tests done, Tobi and Charles learned Chardanai had sickle cell disease.
The Whites had discussed the possibility of their child having the disease before Chardanai was even born. Charles has the sickle-cell trait; however, adults with only one sickle cell gene usually have no problem. This is why Tobi and Charles were so shocked to hear about their daughter’s diagnoses.
“We had to figure out how we could take care of our daughter as well as maintain our home and keep a roof over our head. We have learned to work together as a team and make sure Chardanai is our priority,” says Tobi.
Chardanai has been treated by Mukund Dole, MD in the hematology/oncology department almost her whole life.
“Dr. Dole is the best and I tell him that all of the time,” she says. “Living life with sickle cell and von Willebrand disease can be hard sometimes. However, I have learned to deal with it and move on.”
According to Dr. Dole, “Chardanai and her family understand the importance of managing the disease. They all work together to keep Chardanai healthy. She is a bright young girl and I’m honored to know her family.”
Chardanai is now an active, 11 year old and an all “A” student at Westbrook Village Elementary in Trotwood. She is active in her church choir and Girl Scouts. Chardanai enjoys dancing and she dreams of becoming a nurse or doctor to help other kids who have blood disorders.
The only cure for sickle cell is a bone marrow transplant, but this is not common because it can be hard for patients to find a suitable match. Vigorous hydration, antibiotics, pain relievers (analgesics), chronic blood transfusions and supplemental oxygen are the general treatments used to treat this disease. Newly drugs, like hydroxyurea have been very effective in controlling symptoms of this disease. Other newer treatments, like gene therapy, hold promise for the future.
Though sickle cell can cause many complications, people with the disease can still live a productive and satisfying life—just ask Chardannai.
“I want to help other kids so that when they grow up they will become professionals at controlling their disease and not letting it control them.”
If you would like to learn more about sickle cell disease or help raise awareness, there are many local events to attend. For more information, contact Cynthia Moon, sickle cell coordinator at 937-641-5014 or Valerie Elijah, outreach educator, at 937-641-4334.
About the West Central Ohio Comprehensive Sickle Cell Center:
The centeris funded by a grant from the Ohio Department of Health and is one of six centers throughout the state of Ohio. The center uses a multidisciplinary approach to care for children and adults with sickle cell disease, sickle cell trait and other hemoglobin disorders identified by the Ohio Newborn Screening Program. The team consists of a physician, social worker, nutritionist, geneticist, nurse coordinator, newborn screening coordinator and outreach educator.
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