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Sickle Cell Disease

Page history last edited by PBworks 16 years ago

Sickle Cell Disease

by Victor and Amanda FC

(As displayed in the chart divided by race, Sickle Cell Diease affects blacks in the United states. Followed by Native Americans, Asians, Hispanics, and then Whites.)

What is Sickle Cell Disease?

Sickle Disease is a blood disorder that affects the red blood cells. It is an inherited disease, and people with sickle cell disease have red blood cells that contain mostly hemoglobin S. This is an abnormal type of hemoglobin. Sickle Cell Disease is caused by a change in the structure of hemoglobin. Its a mutation in the DNA code blueprint, and it can cause genetic diseases. It changes one of the amino acids of the hemoglobin which changes its function (Platt 11).

It is characterized by a different type of hemoglobin. This difference results in rigid blood cells that form a sickle shape. When these sickled red blood cells clump together they may cause blockage to the blood vessels which impedes the flow of blood causing tissue death and pain. Another characteristic of sickled red blood cells is that they are stickier than non sickle ones. This contributes to the blocking of the blood flow. Platelets are the cells that cause the blocking to occur. They become over active, leading to clotting in blood vessels that aren't cut, which stops the blood flow. However, a new born with sickle cell disease doesn't show the symptoms until 6 months of age. At that time, swelling of the hands and feet are noticied . This is caused by blocked blood flow and results in painful swelling of the small bones in the hands and the feet.

Sickle Cell Disease is a group of hemoglobin combinations:

Hemoglobin SS (Sickle Cell Anemia)
Hemoglobin SC
Hemoglobin S beta thalassemia
Hemoglobin SD-Punjab
Hemoglobin SO-Arab
Hemoglobin SE

Hemoglobin S-HPFH (hereditary persistence of fetal hemoglobin)

Hemoglobin SS (Sickle Cell Anemia) is the most common, and it is caused by inheriting two sickle cell genes: one from each parent. The symptoms include: severe anemia, tissue damage, strokes, bleeding in the eyes, loss of vision, increased infection, organ damage, and reocurrent pain. As patients age, some other symptons also include: damage to the lungs and kidneys, and an inability to filter toxins from the blood. Treatement consists of: blood transfusions and removal of the spleen.

Hemoglobin SC is the second most common, and it is caused by inheriting the Hemoglobin C gene from one parent, and Hemoglobin SC gene from the other. The symptoms include: swelling of the spleen, eye problems, bone damage, and chronic pain.

Hemoglobin S Beta Thalassemia are inherited disorders of beta globin production, a part of the hemoglobin molecule. It occurs in two types: sickle beta0 thalessemia and sickle beta+ thalessemia. People with sickle cell beta+ have milder syptoms, however, as they age they develop more symptoms. People with sickle cell beta0 thalessemia have more severe symptoms because the hemoglobin levels are higher. The spleens stop working later in childhood and the enlargement of the spleen is common into adulthood. Some symptons are: pain episodes, organ damage and eye problems.

Some other symptoms that are not physical include depression, low self esteem, social isolation, and poor family relationships due to chronic illness. Economically, dealing with Sickle Cell Disease is not easy. Extended hospitalizations, blood transfusions, IV, and other treatments can all be very expensive. In addition, the patient with Sickle Cell is more prone to infection, especially in the first five years of their life. Although infections may occur at any age, because the spleen (which is the bodies major defense against germs)is not fuctioning correctly. Infections of the bladder and kidney are also common among sickle cell patients but are treated with antibiotics.

One treatment is a prenatal diagnosis and to have genetic counseling to see if either parent is a carrier. Other treatments are meant to lessen the severity of the symptoms. For example, it has been proven that by increasing water intake the process known as the sickling effect can be dramatically delayed. This helps flush out the kidneys and prevents patients from becoming dehydrated. Patients with Sickle Cell should avoid caffeinated beverages becuase it causes the kidneys to release more water into their urine. An important part of dealing with the management of Sickle Cell is to make sure that you take in enough air and oxygen into the lungs (which means into the red blood cells). It is very important that patients refrain from smoking and smokers. It is also important to make sure the body has sufficient rest. "Exercising to exhaustion makes the body chemistry change to a state called lactic acidosis"(Platt 22) which could trigger Sickle Cell pain. Babies with Sickle Cell, although symptoms are not shown until 6 months of age, should be placed on daily doses of penicillin to prevent serious infections. They should be immunized as well. The recommended immunizations include: pneumococcal, influenza vaccine, hemophilus influenza type B, and meningococcus.

Hemoglobin SD, SO, SE have milder symptoms. Hemoglobin SE is extremley rare with only five reported cases, and these cases have all been mild. The Hemoglobin E gene is common in Southeast Asia, India and China.

There are many resources available for Sickle Cell patients and their families such as: