SICKLE CELL ANEMIA

By: Ali Rinfrette and Ann Confer

What it Sickle Cell Anemia?

Sickle cell anemia is an inherited disoeder. It is a condition in which there aren't enough healthy red blood cells to carry adequate oxygen throughout your body. Normally, your red blood cells are flexible and round and move easily through your blood vessels. In sickle cell anemia, the red blood cells become rigid and sticky and are shaped like sickles, similar to a crescent moon. These Red Blood Cells tend to get stuck in small blood vessels, which can slow or block blood flow and oxygen to parts of the body.

Symptoms of Sickle Cell Anemia

Anemia

Episodes of pain

Hand-Foot Syndrome

Frequent Infections

Delayed Growth

Vision Problems

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Causes of Sickle Cell Anemia

A person inherits Sickle Cell Anemia. The disease is a result of a mutation in the gene that makes hemoglobin. Hemoglobin is a component on red blood cells that allows oxygen to be carried throughout the body. In Sickle Cell patients their cells are Sickle shape, similar to a crescent moon

Future Cures for Sickle Cell Anemia

New research has been made that shows a transplant of adult blood stem cells could improve sickle cell disease in 9 of 10 adults who had been severely affected by the disease

Genetic factors affecting sickle cell disease symptoms

Regulation of hemoglobin production

Development of drugs to increase fetal hemoglobin production

Transplantation of blood-forming stem cells

Gene therapy

New treatments for pain

Optimal uses of blood transfusion

Management of iron overload associated with blood transfusions

Development of animal models for preclinical studies

Complications of sickle cell trait

Systems Affected

Circulatory System:

Sickle cells stick to each other. Sickle cells may clump together and may cause blood clots.

Immune System:

Immune cells reach their target areas through the blood flow. This would lead to illness, more infections, and a slower healing time. The spleen could have a complication. The complication is called splenic sequestration. It can occur when sickle cells clump together inside the spleen. This can causes a rapid decrease in the amount of hemoglobin produced. The spleen may also become enlarged, sore and scarred, and can lead to permanent damage.

Respiratory System:

Fewer normal red blood cells means less oxygen getting to organs and through the blood. Sickle cells can get trapped in the chest. This causes acute chest syndrome. It blocks the flow for the other blood and the oxygen to the lungs. Cellular respiration would also be affected due to the oxygen-carrying blood flow would be blocked or reduced from being trapped. From this happening, it leads to slower growth and development, a decreased metabolism and lower energy levels.

Nervous System:

The blood vessels can be damaged from this disease in the eyes. The retinas can also be damaged from this. Therefore blindness could occur.

Cures and Treatments

There is no cure or treatments. There is only ways to help it so it does not affect the body as bad and have less pain.

Folic acid helps the body produce more red blood cells.

Penicillin helps prevent infection if the spleen is damaged.

Hydroxyurea helps the body make fetal hemoglobin. This medicine helps the red blood cells from sickling. Hydroxyurea can also make the pain subside and helps with acute chest syndrome. One bad thing about that is the lower count of white blood cells.

Blood transfusions are for the worst cases of sickle cell anemia.

Blood and marrow stem cell transplants is a new treatment. A close family member would have to be the donor.

Gene therapy is currently being studied as a potential treatment for this therapy. It would look at certain genes and see if they could put them in bone marrow stem cells to see if they would grow normal red blood cells.

Sickle Cell Statistic

SCD affects 90,000 to 100,000 Americans.

SCD occurs among about 1 out of every 500 Black or African-American births.

SCD occurs among about 1 out of every 36,000 Hispanic-American births.

SCT occurs among about 1 in 12 Blacks or African Americans.

Sources

http://www.nhlbi.nih.gov/health/health-topics/topics/sca

http://www.mayoclinic.org/diseases-conditions/sickle-cell-anemia/basics/definition/con-20019348

http://www.sicklecelldisease.org/index.cfm?page=about-scd

http://www.cdc.gov/ncbddd/sicklecell/data.html