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September is Sickle Cell Awareness Month |
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SPRINGFIELD One in 12 African Americans have the trait and one in 500 African American births result in the disease, according to Dr. Richard Steingart of the Hematology Oncology Division/Baystate Regional Cancer Program at Baystate Medical Center.
Additionally, one in 25 Hispanics carries the genetic trait and one in 1,000 has the disease called sickle cell anemia, a disorder that affects red blood cells.
September is National Sickle Cell Awareness Month, designed to call attention to the disease and the need to diagnose it at an early age.
Patients with sickle cell disease have red blood cells that become hard and pointed instead of soft and round, obstructing the blood flow to all parts of the body. The physical results can lead to severe, dagger-like pain in the chest, musculo-skeletal areas and the abdomen, as well as non-healing leg ulcers. It can also lead to stroke, lung disease, gallstones and kidney disease. Teens with the disease may develop jaundice, feel tired and have trouble fighting infections, and they may reach puberty later than other teens.
Sickle cell anemia is an inherited disease which doesn't clinically manifest in infants born with it until they are three or four months old, beginning with symptoms that may include chronic ear infections, colic, pneumonia, meningitis and pain.
People who have "sickle cell trait" have inherited the gene for sickle cell disease. Sickle cell trait does not turn into the disease, but two partners with the trait can produce a child with sickle cell disease. There are about 2.5 million people in America with sickle cell trait.
Many people with sickle cell trait don't even realize they have it, as a result, it is important that teens and adults be tested to see if they can have a baby with the disease.
Babies are also tested to see if they have the disease, and the National Institutes of Health (NIH) recommends that all newborns be screened for sickle cell disease, and testing at birth is now required in almost every state so that care and treatment can be provided immediately for those with the disease.
At Baystate's Wesson Women and Infants Unit, all newborns are tested for sickle cell anemia. Newborns with the sickle cell disease are given penicillin to prevent overwhelming infections.
While there is no cure for the disease, there are some promising treatments.
According to Dr. Steingart, use of the drug Hydroxyurea has shown promising results, moderating the disease by inhibiting the cells from "sickling." Good nutrition is also important and can help prevent infections which many people have trouble fighting off. Pain medications can help ease some of the severe pain that accompanies the disease.
Dr. Steingart, one of the area's leading experts on sickle cell anemia, noted hydration is also key. "Pushing fluids can help alleviate painful crisis," he said.
"Not enough is known yet about the disease, and as the goal of National Sickle Cell Awareness implies, we have a ways to go in educating the public and providing both physical and emotional support for patients and their families living with the disease," said Dr. Steingart. "However, we have made strides with the use of new medications to ease pain, performing bone marrow transplants, and in the area of gene therapy," he added.
And it is possible to lead a productive life today while living with sickle cell anemia.
"The key is to lead a healthy lifestyle and to constantly manage your condition along with the help and support of doctors and counselors," Dr. Steingart said.
To book an appointment with Dr. Steingart, call Adult or Pediatric Hematology at the Baystate Regional Cancer Program at 794-9338.
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