Sickle Cell Anemia is the most severe form of Sickle Cell Disease. Other Sickle Cell Diseases are: Sickle Cell Thalassemia (Cooley's Anemia), Sickle Cell C Disease, Sickle Cell D Disease, and any other abnormal hemoglobin combined with sickle hemoglobin. beta chains bind with other beta chains in red blood cell (RBC) when deoxygenated; polymerization occurs; Hb polymers distort RBC into sickled shapes: vaso-occlusion

Sickle cell anemia is an inherited blood disorder, characterized by chronic anemia and periodic episodes of pain. The underlying problem involves hemoglobin, a component of the red cells in the blood. The hemoglobin molecules in each red blood cell carry oxygen from the lungs to the body organs and tissues and bring back carbon dioxide to the lungs. The abnormal hemoglobin molecules tend to cluster together and from long, rod-like structures.

Sickle cell disease is a global health problem. In the United States it is estimated that over 70,000 people have sickle cell disease. About 1,000 babies are born with the disease each year in America.

In sickle cell anemia, the hemoglobin gene is defective. The defective gene tells the body to make the abnormal hemoglobin that results in deformed red blood cells. After the hemoglobin molecules give up their oxygen, some of them may cluster together and form long, rod-like structures. These structures cause the red blood cells to become stiff and to assume a sickled shape.

You can donate blood if you have sickle cell trait. However, all blood is currently filtered to help prevent reactions in the recipient. Blood with sickle cell trait does not filter well. We encourage donors with sickle cell trait to donate plasma or platelet apheresis. These two types do not get filtered

It is estimated that over 2 million people suffer from sickle cell disease, worldwide. It is particularly common among people whose ancestors come from sub- Saharan Africa; Spanish speaking regions (South America, Cuba, Central America); Saudi Arabia; India; and Mediterranean countries, such as Turkey, Greece, and Italy. In this country, it affects approximately 72,000 people, most of whose ancestors, come from Africa.

Early diagnosis of sickle cell anemia is critical. Early detection, education, and the use of penicillin may reduce life-threatening complications. More than 40 states now perform a simple, inexpensive blood test for sickle cell disease on all newborn infants. This test is performed at the same time and from the same blood samples as other routine newborn screening tests. Hemoglobin electrophoresis is the most widely used diagnostic test.

Although there is no cure for sickle cell anemia, doctors can do a great deal to help sickle cell patients, and treatment is constantly being improved. Basic treatment of painful crises relies heavily on pain-killing drugs and oral and intravenous fluids to reduce pain and prevent complications. Blood transfusions are used to treat and to prevent some of the complications of sickle cell anemia. Transfusions correct anemia by increasing the number of normal red blood cells in circulation.

Sickle cell disease is an inherited disorder that affects red blood cells. People with sickle cell disease have red blood cells that become hard and pointed instead of soft and round. Sickle cells cause anemia, pain and many other problems.

Sickle cell disease is an inherited blood disorder, characterized primarily by chronic anemia and periodic episodes of pain. The underlying problem involves hemoglobin, a component of red blood cells. The hemoglobin molecules in each red blood cell carry oxygen from the lungs to the body organs and tissues. Unlike normal red blood cells, which last about 120 days in the bloodstream, sickled red cells die after only 10 to 20 days.

Lung tissue damage, pain episodes and stroke. The blockage of blood flow caused by sickled cells also causes damage to most organs including the spleen, kidneys and liver.

The clinical course of sickle cell anemia does not follow a single pattern; some patients have, mild symptoms, and some have very severe symptoms. However, the basic problem is the same - the sickle-shaped red blood cells tend to get stuck in narrow blood vessels, blocking the flow of blood and oxygen. Insert picture of “stylized” child with glowing areas to point and click Click on area and get description Hand-foot syndrome: .

The error in the hemoglobin gene results from a genetic mutation that occurred many thousands of years ago in people in parts of Africa, the Mediterranean basin, the Middle East and India. Malaria was very common at that time and the epidemic caused the death of great numbers of people. Studies show that in areas where malaria was a problem, children who inherited one sickle hemoglobin gene and carried the sickle cell trait had a survival advantage.

Sickle cell disease has probably been recognized for centuries in African tribal medicine. However, sickle cell disease was first recognized in Western medicine in 1910 by Dr. B. Herrick when he wrote a report about a patient who suffered from a “strange disease” that included such symptoms as asthmatic conditions and blood flow problems including body ulcers. This observation eventually led to the identification of the sickle cell hemoglobin (HbS) in the 1940s by Linus Pauling.

Sickle cell disease affects millions of people throughout the world. It is particularly common among people whose ancestors come from sub-Saharan Africa, Spanish-speaking regions (South America, Cuba, Central America), Saudi Arabia, India and Mediterranean countries, such as Turkey, Greece and Italy. In this country, it affects approximately 70,000 people, most with ancestors from Africa.

A simple blood test can determine if an individual carries at least one defective hemoglobin (S) gene. Early diagnosis of sickle cell anemia is critical so children who have the disease can receive proper treatment. Currently 46 states have newborn screening programs. Texas initiated newborn screening in 1983, and sickle cell anemia is one of the diseases that infants are screened for prior to leaving the hospital.

The treatment of painful episodes includes pain medications and fluids to reduce pain and prevent complications. Blood transfusions are used to treat and to prevent some of the complications of sickle cell anemia. Transfusions correct anemia by increasing the number of normal red blood cells in circulation. Regular transfusion as a therapy can also help prevent recurring strokes in children who are at high risk of crippling nervous system complications.

Yes. By sampling the amniotic fluid or tissue taken from the placenta, doctors can tell whether a fetus has sickle cell anemia or sickle cell trait. This test can be done as early as the first trimester I of pregnancy.

Sickle Cell Anemia is clearly manifested at an early age. The child becomes pale, tires easily, eats poorly, may have swelling of the hands and feet, slow growth, pain in arms, legs, back and abdomen.

The sickled red blood cells have a shorter life span than normal red blood cells and are easy to break, thereby causing the blood count to be lower than normal (anemia). The body cannot make new cells fast enough to keep up with the destruction. Therefore, the blood count remains below normal throughout life.

Infections are treated with antibiotics at the earliest moment, particularly in children. Drinking an adequate amount of fluids is encouraged at all times. Blood transfusions and exchange transfusions are sometimes needed. The treatment varies depending upon the organs involved and the nature of the complication. You must consult your physician for physical checkups and follow up care. Sickle Cell Anemia cannot be corrected with iron medication or foods containing iron.

No cure has been found yet, but scientists are working to find a cure. Research and education are among top priorities in sickle cell programs funded through the National Heart and Lung Institute.

The first effective drug treatment for adults with severe sickle cell anemia was reported in early 1995, when a study conducted by the National Heart, Lung, and Blood Institute showed that daily doses of the anticancer drug hydroxyurea reduced the frequency of painful crises and of acute chest syndrome in these patients. Patients taking the drug also needed fewer blood transfusions.

The clinical course of sickle cell disease does not follow a single pattern. Some patients have mild symptoms, and some have very severe symptoms. However the basic problem is the same; the sickle-shaped red blood cells tend to get stuck in narrow blood vessels, blocking the flow of blood and causing pain. The presence of two defective genes (SS) is needed for sickle cell anemia.

If you have sickle cell trait, you have inherited the gene for sickle cell disease. Sickle cell trait does not turn into sickle cell disease. If someone has sickle cell trait and his partner has sickle cell trait they may produce a child with sickle cell disease. There are about 2.5 million people in America with sickle cell trait.

Try to maintain good health by eating a well balanced diet, and getting enough rest. Avoid stress such as colds and fatigue. Be careful to prevent infections from cuts and bruises. Special attention to skin care and overall personal hygiene is a must.

Answer: Median survival of individuals of all ages with sickle cell disease based on genotype and sex (Platt OS, Brambilla DJ, Rosse WF, et al. Mortality in sickle cell disease- Life expectancy and risk factors for early death. N Engl J Med 330(23): 1639-1644 (1994). Note that this was reported in 1994.

Answer: Hydroxyurea therapy for pediatric sickle cell patients is in transitionzone between "experimental therapy" and "commonly accepted therapy." In comparison to adult sickle cell patients, the number of pediatric patients treated with hydroxyurea has been small. More teenagers have been treated with hydroxyurea than younger children.