Sickle Beta + Thalassemia (Sickle BA-ta Plus Thal-a-SEE-me-a) is a "mild" form of sickle cell disease. Your child's red blood cells contain an abnormal hemoglobin called hemoglobin S or sickle hemoglobin in addition to a small amount of the normal hemoglobin called hemoglobin A. The red blood cells have a defect called beta plus thalassemia, which results in cells which are small in size and more pale than usual. Instead of appearing round or donut shaped, your child's red blood cells are somewhat small, pale, and misshapen. Some may appear sickled or banana shaped. Because sickle beta plus thalassemia is inherited, it is a lifelong disorder. There is no treatment or cure. Your child will always have a mild anemia or slightly low blood count. This may cause occasional tiredness or weakness. Abstract source

It is possible for a person of any race or nationality to have sickle cell trait, but it is very common in African-Americans. About one (1) out of every 12 African-Americans has sickle cell trait. It also affects Hispanics, and people whose ancestors came from Africa, Latin America, Asia, India, and the Mediterranean region. Sickle cell trait affects the red blood cells. All red blood cells contain hemoglobin, which carries oxygen from the lungs to all parts of the body. People with sickle cell trait have both normal hemoglobin A and abnormal hemoglobin S in their red blood cells. People who do not have sickle cell trait or any other abnormal hemoglobin have red blood cells that contain only hemoglobin A. People with sickle cell trait do not develop sickle cell disease. People with sickle cell disease have red blood cells that contain mostly hemoglobin S. Under certain conditions these red blood cells become sickle-shaped (banana-shaped) and block circulation. Abstract source

Hydroxyurea is a medicine that can help children and adults with sickle cell disease. Research studies show that hydroxyurea lowers the following: • The numbers of acute chest syndrome (pneumonia) events • The number of pain crises • The need for blood transfusions • The number of trips to the hospital Hydroxyurea also might prevent damage to the spleen, kidneys, lungs, and brain. Hydroxyurea is given by mouth one (1) time each day. It comes in liquid or capsule form. The U.S. Food and Drug Administration (FDA) has approved it for the treatment of adults with severe sickle cell disease. St. Jude Children’s Research Hospital and other medical centers have carried out research studies that demonstrated the safety of hydroxyurea therapy for young children and infants with sickle cell disease.   Abstract source

Sickle cell disease is a blood disorder that affects red blood Cells (RBC). The main purpose of red blood cells is to deliver oxygen to the body. Blood delivers oxygen to all of the tissues in the body. The part of the blood that carries oxygen is called hemoglobin. Sickle cell disease is a blood disorder that affects the hemoglobin (HEE-muh-glowbin) within the red blood cells. The main role of hemoglobin is to deliver oxygen to the cells of the body. People with sickle cell disease have red blood cells that contain mostly hemoglobin S, an abnormal type of hemoglobin. Sometimes these red blood cells become sickle (banana) shaped, and have trouble passing through small blood vessels. Normal red blood cells are shaped like discs and are fl exible. They fl ow easily through the body’s blood vessels. Sickleshaped red blood cells are sticky, stiff, and rigid. They clog the body’s small blood vessels. When sickle-shaped cells block small blood vessels, less blood can reach that part of the body. Tissue that does not receive a normal blood fl ow eventually becomes damaged. This is what causes some of the complications of sickle cell disease. Abstract source

This type of procedure involves the transfer of red blood cells from one (1) person to another. Red blood cells are obtained when a person donates blood. The red cells are separated from the other parts of blood. These red blood cells are collected in a bag, stored in a refrigerator, and then given to another person through a vein. Donated blood cells are carefully tested for infections and closely matched to the recipient to prevent reactions. Normal red blood cells carry oxygen throughout the body. The red blood cells of people with sickle cell disease do not deliver oxygen as well as normal red blood cells do. In addition, these cells may change into a sickle shape, which blocks the flow of blood through the vessels. When a person with sickle cell disease has certain complications (health problems) from the disease, transfusing normal red blood cells may help deliver oxygen to the body and unblock blood vessels.   Abstract source

Bone marrow (stem cell) transplants have been used for the treatment and cure of a variety of cancers, immune system diseases, and blood diseases for many years. Doctors in the United States and other countries have developed studies to treat children who have severe sickle cell disease with bone marrow (stem cell) transplants. In a person with sickle cell disease, the bone marrow produces red blood cells that contain hemoglobin S. This leads to the complications of sickle cell disease. • To prepare for a bone marrow (stem cell) transplant, strong medicines, called chemotherapy, are used to weaken or destroy the patient’s own bone marrow, stem cells, and infection fighting system. – This is done so the patient does not reject the new blood cells coming from the donor. • The patient’s bone marrow then is replaced with blood-forming stem cells from a donor who does not have sickle cell disease. – This can be a donor with normal hemoglobin or sickle cell trait. – The actual transplant is given like a blood transfusion through an IV tube.   Abstract source

Sickle cell anemia is a problem passed down through genes. It affects the red blood cells (RBCs). RBCs have a protein called hemoglobin that lets them carry oxygen through the body. With sickle cell anemia, a problem with the hemoglobin causes the cells to be C-shaped (like a sickle) instead of round. As a result, the cells can’t move easily through the smallest blood vessels. Because the cells tend to stick together, this can clog the vessels and prevent normal blood flow. Also, sickled cells don’t live as long as normal RBCs. When the body doesn’t have enough healthy RBCs, this causes anemia. Sickle cell anemia is very serious and can hurt your child’s health. Work with your child’s healthcare provider to learn about treatment options for your child. Abstract source

Sickle cell disease changes normal, round red blood cells into cells that can be shaped like crescent moons. The name "sickle cell" comes from the crescent shape of the cells. (A sickle is a tool with a crescent-shaped blade.) Normal red blood cells move easily through your blood vessels, taking oxygen to every part of your body. But sickled cells can get stuck and block blood vessels, which stops the oxygen from getting through. That can cause a lot of pain. It can also harm organs, muscles, and bones. Having sickle cell disease means a lifelong battle against the health problems it can cause, such as pain, infections, anemia, and stroke. But many people are able to have a very good quality of life by learning to manage the disease. Abstract source

Sickle cell disease is an inherited blood disorder. It is marked by flawed hemoglobin. That’s the protein in red blood cells that carries oxygen to the tissues of the body. So, sickle cell disease interferes with the delivery of oxygen to the tissues. Red blood cells with normal hemoglobin are smooth, disk-shaped, and flexible, like doughnuts without holes. They can move through the blood vessels easily. Cells with sickle cell hemoglobin are stiff and sticky. When they lose their oxygen, they form into the shape of a sickle or crescent, like the letter C. These cells stick together and can’t easily move through the blood vessels. This can block small blood vessels and the movement of healthy, normal oxygen-carrying blood. The blockage can cause pain. Normal red blood cells can live up to 120 days. But, sickle cells only live for about 10 to 20 days. Also, sickle cells may be destroyed by the spleen because of their shape and stiffness. The spleen helps filter the blood of infections. Sickled cells get stuck in this filter and die. With less healthy red blood cells circulating in the body, you can become chronically anemic. The sickled cells also damage the spleen. This puts you are at greater at risk for infections.   Abstract source

Sickle cell anemia is an inherited form of anemia — 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, moving easily through your blood vessels. In sickle cell anemia, the red blood cells become rigid and sticky and are shaped like sickles or crescent moons. These irregularly shaped cells can get stuck in small blood vessels, which can slow or block blood flow and oxygen to parts of the body. There's no cure for most people with sickle cell anemia. But treatments can relieve pain and help prevent problems associated with the disease.   Abstract source