Sickle Cell Anemia Awareness Night

QUESTIONS MOST OFTEN ASKED ABOUT SICKLE CELL DISEASE

What causes Sickle Cell Disease?

An abnormal hemoglobin inside of red blood cells. The hemoglobin particles inside of red blood cells are like little packets of protein and iron. The iron in hemoglobin gives red blood cells their color. Hemoglobin carries oxygen inside red blood cells. Red cells travel with the oxygen to various parts of the body. They must squeeze through tiny blood vessels to deliver oxygen - blood vessels even smaller than the cells themselves! To do this, they have to be soft enough to change shape. But sometimes the cells become stiff and have pointy edges. Then they can't get through all the blood vessels. In people with sickle cell disease, this is because there is an abnormal hemoglobin inside the red blood cells.

What is this hemoglobin called?

Hemoglobin (Hb) S. The S stands for "sickle." Red cells that contain Hb S change shape under certain conditions. One of these shapes looks like a sickle. Hemoglobin is normally in a liquid form. But Hb S is different. After it has given up its oxygen, it becomes firm and forms a gel. The hemoglobin particles then stick together and form long bundles, or fibers. These bundles grow so long that they stretch out the walls of the cell in different directions. The cells then have pointed edges and become sickled cells.

How does this cause problems?

Sickled cells can form traffic jams in small blood vessels. When sickled cells are not soft enough to squeeze through tiny blood vessels, they keep other cells from getting through. This is called vaso-occlusion. It is like a traffic jam of delivery trucks. Instead, red blood cells are trying to deliver much-needed oxygen to tissues in the body. When tissues do not get oxygen, they become painful and can be damaged. Pain can be mild or very severe. Mild pain can be treated at home, but a person with severe pain may have to be admitted to the hospital. People with sickle cell disease do not die from these painful episodes. Organs such as the kidneys and spleen can also be damaged over the long term. In very young children, the hands and feet can become swollen and painful. This is called hand-foot syndrome, and is often the first sign of sickle cell disease in a young child. Also, a small percentage of children with sickle cell disease will have stroke.

Why are children with Sickle Cell Disease at risk for serious infections?

The spleen becomes damaged from repeated red cell sickling within it. This happens early in life. The spleen is the organ in the abdomen that removes bacteria and damaged red blood cells from the blood stream. When it does not work properly, young children can become seriously ill from infections very quickly. Prompt treatment with antibiotics usually works. But infections can get bad very quickly. Penicillin is given 2 times a day beginning when the child is 2 to 3 months of age. This keeps some of the serious infections from happening.

What about Anemia?

Repeated sickling damages and finally breaks down red cells. People with sickle cell disease cannot make new cells fast enough to keep up with the loss. The result is a low blood count, or, in other words, anemia. Also, the remains of the damaged red blood cells can cause the white of the eyes to become yellowish (jaundice).

How does a person get Sickle Cell Disease?

Sickle cell disease is inherited through the genes, like hundreds of other disorders. Cystic fibrosis, hemophilia, and Cooley's anemia are all inherited. If a child has Hb SS disease, the most common form of sickle cell disease, it means that the mother contributed one gene for Hb S and the father a second gene for Hb S. These parents are said to have sickle cell trait. If the child has a variant for sickle cell disease, it means that one parent passed on a gene for Hb S and the other parent passed on a gene for another abnormal hemoglobin.

Is Sickle Cell Trait an illness?

No, not at all. It is very common. One in 12 African-Americans has sickle cell trait. There is not enough Hb S in the red blood cells of people with sickle cell trait to cause the red cells to sickle except under some very special conditions. One such condition is at high altitudes where oxygen levels are low.

Does Sickle Cell Trait ever turn into Sickle Cell Disease?

Never. And neither can sickle cell disease turn into sickle cell trait. Hemoglobin types never change. A blood test can determine the type of hemoglobin a person has.

Do only African-Americans Have Sickle Cell Trait and Disease?

No. In fact, Hb S is a world traveler. It is found in people who live in or people whose families came from Africa, Greece, Italy, Turkey, Cyprus, Saudi Arabia, Kuwait, and India. For more information contact: Comprehensive Sickle Cell Center The Children's Hospital of Philadelphia 34th Street and Civic Center Blvd. Philadelphia, PA 19104 (215) 590-3423

Aaron McKie speaks with children who are afflicted with sickle cell anemia as he and the 76ers try to raise awareness of the disease.
Jesse D. Garrabrant NBAE/Getty Images

Growing up, Aaron McKie did not fully realize it, but his brother, Woody McKie, was sufferring from a very serious illness. Aaron recalled that Woody would get sick, go into the hospital and come out.

But Aaron would personnaly be affected by the illness when Woody passed away from Sickle Cell Anemia in his early 30s. It was just before Aaron was to go off on his college.

"It’s just something that affected my family," Aaron said. "I think it’s something that doesn’t get much attention."

McKie hoped to raise awareness of Sickle Cell Anemia when the Sixers hosted Utah on Monday, Dec. 20, 2004. A group of children with the condition was in attendance, and information about sickle cell will be available throughout the game on the main concourse and during in-arena presentation.

Sickle cell anemia is an inherited, chronic disease in which the red blood cells, normally disc-shaped, become crescent shaped.  As a result, they function abnormally and cause small blood clots.  These clots give rise to recurrent painful episodes called “sickle cell pain crises.” Sickle cell disease is much more common in certain ethnic groups, affecting approximately one out of every 500 African Americans.

"One day, if not in my generation, maybe the next generation, somebody can find a cure and can help the situation with it," Aaron hopes.

It was September of 1990, when Aaron was a freshman at Temple University about to embark on his basketball career, that his older brother Woody passed away due to complications from sickle cell anemia.

“Woody was really important to me,” McKie said.  “He was a special guy, and after the way I saw him suffer with sickle cell, it became clear that I had to try to do something.  Education and awareness are the first steps.”

As part of the evening, the team invited 140 children from The Children’s Hospital of Philadelphia suffering from sickle cell disease to attend the game and enjoy a pregame meet-and-greet with Aaron.  Additionally, hospital staffers and parents were also be in attendance.

Lynne Parker, a registered nurse, cared for Woody at Germantown Hospital while he battled the disease.  A chance meeting between Aaron and Parker helped to make the awareness night possible. Parker, who is currently the director of client services for a medical data management company was unable to attend the game.   

“Woody was a very friendly guy,” said Parker.  “Even when he was discharged from the hospital, he would always stop in to see me.  All he would talk about was his brother Aaron - he was so proud of him!”