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Published on September 06, 2012

Education, Treatment Key For Patients With Sickle Cell

COLUMBIA, Mo. ― September is National Sickle Cell Disease Awareness Month, and health professionals at Children’s Hospital want those who are most at risk of carrying the disease’s genetic trait to understand the importance of education and comprehensive care.

“Sickle cell is an inherited disease that causes abnormally formed red blood cells,” said Thomas Loew, M.D., medical director of the Children’s Blood Disorders and Cancer Unit at Children’s Hospital. “Normal red blood cells are soft and round, allowing them to easily flow through blood vessels. Children with sickle cell disease produce red blood cells that are rigid and half-moon shaped. These abnormally shaped cells disrupt normal blood flow and cause health complications.”

Children with the disease experience symptoms such as increased susceptibility to bacterial infections, increased risk of stroke, severe pain caused by the difficult passage of the abnormally shaped blood cells through the body’s tissue, and damage to internal organs such as the kidneys, liver and spleen.

Treatment for sickle cell anemia is usually aimed at avoiding crises, relieving symptoms and preventing complications.

“Antibiotics and pain management are typical methods we use to care for someone with sickle cell disease,” said Elizabeth Gunier, R.N., a sickle cell care coordinator at the hospital’s Children’s Blood Disorders and Cancer Unit. “However, in some instances blood transfusions or bone marrow transplants may be necessary. It is important for patients to make regular visits to their health care providers so red blood cell counts and overall health can be monitored to reduce complications.”

In the United States, sickle cell disease affects mostly African-Americans and some Latinos. Some forms of the disease may also occur in people with other ethnic backgrounds, such as those whose ancestors came from East India or Mediterranean countries such as Turkey, Greece and Italy.

All babies born in Missouri are tested for sickle cell disease, but Loew recommends that those at risk or with a family history of sickle cell get tested for the genetic trait before having children.

“It’s important for people to know that parents who carry the genetic trait but who don’t have the disease can pass the disease on to their children,” Loew said. “When it comes to sickle cell, the best defense is a good offense.”

When both parents have the sickle cell trait, there’s a 25 percent chance that their child will have sickle cell disease. But when one parent is carrying the trait and the other actually has the disease, the odds increase to 50 percent that they will pass the disorder on to their child.

“Awareness is the key with managing sickle cell disease,” said Gunier. “The most critical time is the first few years of life, which is why prenatal care, early diagnosis and comprehensive treatment are so important.”