Sickle-Cell Anemia

Sickle Cell Anemia
Sickle Cell Anemia is a genetic disorder in which red blood cells become deformed due to abnormal hemoglobin production. This abnormal hemoglobin causes the red blood cells to become sickle shaped, sticky, and stiff.

Symptoms
There are many symptoms of sickle cell anemia. Some of the most common symptoms are:
 * Shortness of breath
 * Fatigue
 * Frequent headaches and dizziness
 * Constant pale skin
 * Chest pain
 * Fever
 * Increased heart rate
 * Overactive bladder

Most symptoms only occur after 4 months of age. Most infected people have complications that consist of sudden pain throughout the body. This is called a "sickle cell crisis." When someone has a "crisis," their sickle shaped red blood cells clump in veins and block blood flow.This stops blood from reaching the extremities and results in pain and possible organ damage. These "crisis" can occur from one every few years to up to 15 per year.

Detection
Sickle Cell Anemia is caused by an abnormal type of hemoglobin, hemoglobin S. This is present when the level of oxygen is low. Hemoglobin Electrophoresis is used to detect the type of hemoglobin that is in the blood. Blood is obtained through a simple blood test, and a charge is run through the solution of hemoglobin. Different types of hemoglobin will travel different lengths. This test can determine if the indiviudal has the sickle cell disease or a sickle cell trait. Another test used to identify sickle cell anemia is the Complete Blood Count (CBC). Other less commonly used tests include Bilirubin tests, Blood Oxygen Tests, CT Scans, MRIs, or Peripheral Smears.

Treatment Options
Patients with Sickle Cell Anemia need constant treatment. Most of the time you cannot treat the actual disease, only attempt to control the symptoms. One of the most important parts is taking folic acid supplements to aid in the production of red blood cells. When having a "crisis" you should take over the counter pain medication with large amounts of water. Regular blood transfusions and antibiotics prevent strokes and bacterial infections. Most treatments are used to treat the complications that arise for Sickle Cell Anemia. These include a kidney transplant or dialysis for kidney disease, gallbladder removal, eye surgery, hip replacement or wound care. The disease can be treated by a bone marrow transplant. This is an especially complicated and painful process that poses many risks to both the donor and the debilitated patient.

Life Expectancy
In the United States, over 70,000 people have the Sickle Cell Disease; about 1,000 babies are born with the disease each year. The life expectancy for those with Sickle Cell Anemia has improved over the years but is still only 40 years old. This disease is the same as other chronic diseases, support and cautious measures are necessary. In a study measuring the Health Related Quality of Life for Sickle Cell Anemia patients, it was found that those with the Sickle Cell Disease have a lower health related quality of life than patients with cystic fibrosis. The only exception for this finding is in comparison of mental health related quality of life. This study found that the quality of life for people with Sickle Cell Anemia is worse than the general population, and similar to people with hemodialysis.

Inheritance
Sickle Cell Anemia is caused by an abnormal hemoglobin, hemoglobin S, that distorts the shape of the red blood cells. This sickled or crescent shape makes it much harder for the red blood cells to travel in the body. Because Sickle Cell Anemia is a recessive disorder, both parents must have the trait and give it to the child. If the child inherits only one allele, they are said to have the Sickle Cell Trait. This allows the person to be a carrier for the disease while not having the true symptoms. It is adventagous to have the Sickle Cell Trait, especially in Africa because it makes you more resistant to malaria while not giving you the full symptoms of Sickle Cell Anemia.

Frequency
Sickle Cell Anemia is more common in some backgrounds than others. In Africa, 1 in 4 individuals are infected with Sickle Cell Anemia. In Americans, 1 in 400 individuals are infected with Sickle Cell Anemia. In Hispanic countries, the frequency is 1 in 1,000.