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Sickle Cell Anemia / Disease (SCD) Doesnt Progress as Children Age

Sickle cell anemia is caused by an abnormal type of hemoglobin called hemoglobin S. Hemoglobin is a protein inside red blood cells that carries oxygen. Hemoglobin S, however, reduces the amount of oxygen inside the cells, distoring their shape. The fragile, sickle-shaped cells deliver less oxygen to the body's tissues, and can break into pieces that disrupt blood flow.Sickle cell anemia is inherited as an autosomal recessive trait , which means it occurs in someone who has inherited hemoglobin S from both parents.

Some children inherit one hemoglobin S gene and one for another type of abnormal hemoglobin. This results in a different form of sickle cell disease (such as S-beta thalassemia or hemoglobin SC disease).This process damages the red blood cell membrane, and can cause the cells to become stuck in blood vessels. This deprives the downstream tissues of oxygen and causes ischemia and infarction. The disease is chronic and lifelong. Individuals are most often well, but their lives are punctuated by periodic painful attacks. In addition to periodic pain, there may be damage of internal organs, such as stroke.

Sickle Cell Disease affects approximately 1 in 625 African American newborns in the United States. The sickle cell trait is present in 8% of African Americans.There are different kinds of sickle cell disease. Some kinds are mild, but others cause serious illness.Sickle cells can get stuck in blood vessels and keep blood from reaching parts of the body. This causes pain and can damage the body's internal organs. Blocked blood vessels in the arms, legs, chest or abdomen can cause strong pain.

Children with sickle cell disease might get more infections because their spleen is damaged by sickle cells. (One of the spleen's main jobs is to protect against infection.) When sickle cells block blood flow to organs and cause pain and other problems, this is called a "sickle cell crisis," or a "pain crisis."If blood supply to the brain is blocked.These painful crises, which occur in almost all patients at some point in their lives, can last hours to days, affecting the bones of the back, the long bones , and the chest. Some patients have one episode every few years, while others have many episodes per year.

Children with sickle cell disease who suffer major complications, such as lung disease or pain early in life, are not at increased risk for stroke or death later in childhood, U.S. researchers say.

Conducted by scientists at the University of Texas Southwestern Medical Center at Dallas, the study also concluded that infants and toddlers with sickle cell disease who have pain episodes or dactylitis -- painful swelling in the feet and hands -- are not at increased risk of suffering those symptoms again later in childhood.

However, young children with the disease who are hospitalized for chest problems are more likely to have a recurrence of those problems up to adulthood, the study said.

For this study, researchers looked at data from 264 children with sickle cell disease who were followed from birth through their teenage years. The findings add to efforts to identify predictors that can forecast how the disease will progress as children age.

"Everybody who has sickle cell disease is affected differently by the disease. Some seem to have a lot of problems with pain and lung disease, and some have very few problems and may have a normal life span. We really don't understand why everyone with the same disease can be so different," lead author Dr. Charles Quinn, assistant professor of pediatrics at the UT medical center, said in a prepared statement.

This makes it difficult to counsel parents of infants with the disease.

"We can't give them very much in the way of specifics, exactly what this child will likely go through or what to expect from the disease in the future," said Quinn, who is also a pediatric hematology specialist at Children's Medical Center Dallas.

It's estimated that at least 70,000 Americans have the genetic disease. Instead of soft, round red blood cells that carry oxygen through the body, people with sickle cell disease have inflexible, sickle-shaped red blood cells that cause blockages in blood vessels and prevent body tissues from receiving oxygen.

There's no cure for most people with sickle cell anemia. However, treatments can relieve pain and prevent further problems.