Health Care and Sickle Cell Anemia

One very unique  health care disorder that affects primarily people whose families come from Africa, South or Central America (especially Panama), Caribbean islands, Mediterranean countries (such as Turkey, Greece, and Italy), India, and Saudi Arabia, according to the National Institutes for Health (NIH).

In the United States, it's estimated that sickle cell anemia affects 70,000–100,000 people, mainly African Americans. The disease occurs in about 1 out of every 500 African American births. Sickle cell anemia also affects Hispanic Americans. The disease occurs in more than 1 out of every 36,000 Hispanic American births. More than 2 million Americans have sickle cell trait. The condition occurs in about 1 in 12 African Americans.

According to the NIH, it is the most common form of sickle cell disease (SCD). SCD is a serious disorder in which the body makes sickle-shaped red blood cells. “Sickle-shaped” means that the red blood cells are shaped like a crescent.

Normal red blood cells are disc-shaped and look like doughnuts without holes in the center. They move easily through your blood vessels. Red blood cells contain an iron-rich protein called hemoglobin. This protein carries oxygen from the lungs to the rest of the body.

Sickle cells contain abnormal hemoglobin called sickle hemoglobin or hemoglobin S. Sickle hemoglobin causes the cells to develop a sickle, or crescent, shape. Sickle cells are stiff and sticky. They tend to block blood flow in the blood vessels of the limbs and organs. Blocked blood flow can cause pain and organ damage which can also raise the risk for infection. Much more detailed information can be found at this website: http://www.nhlbi.nih.gov/health/health-topics/topics/sca .

According to the Sickle Cell Disease Association of America (SCDAA), sickle cell conditions are inherited from parents in much the same way as blood type, hair color and texture, eye color and other physical traits. The types of hemoglobin a person makes in the red blood cells depend upon what hemoglobin genes the person inherits from his or her parents.

Examples:

--If one parent has Sickle Cell Anemia and the other is Normal, all of the children will have sickle cell trait.

--If one parent has Sickle Cell Anemia and the other has Sickle Cell Trait, there is a 50% chance (or 1 out of 2) of having a baby with either sickle cell disease or sickle cell trait with each pregnancy.

--When both parents have Sickle Cell Trait, they have a 25% chance (1 of 4) of having a baby with sickle cell disease with each pregnancy.

A simple blood test followed by a laboratory technique called Hemoglobin Electrophoresis will determine the type of hemoglobin you have. When you pass an electric charge through a solution of hemoglobin, distinct hemoglobins move different distances, depending on their composition.

The SCDAA reports that sickle cells are destroyed rapidly in the body of people with the disease causing anemia, jaundice and the formation of gallstones. The sickle cells also block the flow of blood through vessels resulting in lung tissue damage (acute chest syndrome), pain episodes (arms, legs, chest and abdomen), stroke and priapism (painful prolonged erection).

 

It also causes damage to most organs including the spleen, kidneys and liver. Damage to the spleen makes sickle cell disease patients, especially young children, easily overwhelmed by certain bacterial infections.

Health maintenance for patients with sickle cell disease starts with early diagnosis, preferably in the newborn period and includes penicillin prophylaxis, vaccination against pneumococcus bacteria and folic acid supplementation. Treatment of complications often includes antibiotics, pain management, intravenous fluids, blood transfusion and surgery all backed by psychosocial support. Like all patients with chronic disease patients are best managed in a comprehensive multi-disciplinary program of care.

Blood transfusions help benefit sickle cell disease patients by reducing recurrent pain crises, risk of stroke and other complications. Because red blood cells contain iron, and there is no natural way for the body to eliminate it, patients who receive repeated blood transfusions can accumulate iron in the body until it reaches toxic levels.

It is important to remove excess iron from the body, because it can gather in the heart, liver, and other organs and may lead to organ damage. Treatments are available to eliminate iron overload. Much more detailed material about this disease can be found at this website: http://www.sicklecelldisease.org/index.cfm?page=about-scd.

Scientists are constantly researching ways to help people with sickle cell anemia, according to this website:  http://kidshealth.org/teen/diseases_conditions/blood/sickle_cell_anemia.html.

Several new treatments, such as the drug hydroxyurea, have helped reduce painful crises and episodes of acute chest syndrome for adults and kids with sickle cell. Bone marrow transplant, a complex and risky procedure, is the only cure for sickle cell anemia. This disease is especially tough on children and adolescents.

Scientists are also studying gene therapy as a treatment for sickle cell anemia. One day, it's hoped that doctors may be able to stop the disease by changing or replacing the abnormal gene that causes it. With the right precautions, children and teens with sickle cell disease can do most of the stuff other kids do. To stay as healthy as possible, take these steps:

--Eat a balanced, healthy diet.

--Take medications, including folic acid supplements, as prescribed.

--Drink plenty of fluids to prevent dehydration.

--Avoid extreme cold or heat.

--Exercise regularly, but in moderation. Exercise is important for staying healthy, but overdoing it can trigger a crisis in some people, particularly if they become dehydrated, overheated, or exhausted.

--Get plenty of rest.

--Avoid alcohol, drugs, and smoking which can aggravate sickle cell disease and its symptoms. Some people with sickle cell disease are prone to lung problems, so smoking is particularly risky and must be avoided.

--Avoid places low in oxygen. (For example, it's not a good idea to go hiking at high altitudes or spend lots of time swimming under water.)

--Prevent serious infections by contacting your doctor as soon as illness symptoms start. Be sure to get any immunizations (such as pneumonia and flu vaccines) that the doctor recommends, and always call your doctor if you have a high fever (over 101°F, 38°C).

--Learn as much as you can about the disease and see your doctor regularly to help prevent complications.

For a tremendous human interest story about a family’s inspirational fight against sickle cell anemia, visit this website: http://sports.espn.go.com/espn/eticket/story?page=boozers

Sickle cell anemia is a disease that can be deadly and debilitating. If you or someone you know are suffering from symptoms, see your doctor. Follow the guidance provided by specialists and support groups. Work hard at staying healthy. Know that the disease can be managed.

Until next time.