Sickle Cell Patients
Red blood cells are usually round and flexible, so they move easily through blood vessels. In sickle cell anemia, some red blood cells are shaped like sickles or crescent moons. These sickle cells also become rigid and sticky, which can slow or block blood flow.
sickle cell patients
Episodes of pain. Periodic episodes of extreme pain, called pain crises, are a major symptom of sickle cell anemia. Pain develops when sickle-shaped red blood cells block blood flow through tiny blood vessels to your chest, abdomen and joints.
See your health care provider right away if you or your child has symptoms of sickle cell anemia. Because children with sickle cell anemia are prone to infections, which often start with a fever and can be life-threatening, seek prompt medical attention for a fever greater than 101.5 F (38.5 C).
Sickle cell anemia is caused by a change in the gene that tells the body to make the iron-rich compound in red blood cells called hemoglobin. Hemoglobin enables red blood cells to carry oxygen from the lungs throughout the body. The hemoglobin associated with sickle cell anemia causes red blood cells to become rigid, sticky and misshapen.
If only one parent passes the sickle cell gene to the child, that child will have the sickle cell trait. With one typical hemoglobin gene and one altered form of the gene, people with the sickle cell trait make both typical hemoglobin and sickle cell hemoglobin.
For a baby to be born with sickle cell anemia, both parents must carry a sickle cell gene. In the United States, sickle cell anemia most commonly affects people of African, Mediterranean and Middle Eastern descent.
If you carry the sickle cell trait, seeing a genetic counselor before trying to conceive can help you understand your risk of having a child with sickle cell anemia. A genetic counselor can also explain possible treatments, preventive measures and reproductive options.
Sickle cell disease is a group of inherited red blood cell disorders that affect hemoglobin, the protein that carries oxygen through the body. The condition affects more than 100,000 people in the United States and 20 million people worldwide.
Sickle cell disease is a lifelong illness. A blood and bone marrow transplant is currently the only cure for sickle cell disease, but there are effective treatments that can reduce symptoms and prolong life. Your healthcare team will work with you on a treatment plan to reduce your symptoms and manage the condition. The NHLBI is leading and supporting research and clinical trials to find a cure for sickle cell disease.
Bone marrow or stem cell transplants are very risky and can have serious side effects, including death. For the transplant to work, the bone marrow must be a close match. Usually, the best donor is a brother or sister. Bone marrow or stem cell transplants are most common in cases of severe SCD for children who have minimal organ damage from the disease.
Sickle cell disease (SCD) affects millions of people throughout the world and is particularly common among those whose ancestors came from sub-Saharan Africa; Spanish-speaking regions in the Western Hemisphere (South America, the Caribbean, and Central America); Saudi Arabia; India; and Mediterranean countries such as Turkey, Greece, and Italy.
SCD occurs more often among people from parts of the world where malaria is or was common. It is believed that people who carry the sickle cell trait are less likely to have severe forms of malaria. Learn more about the protective effect of sickle cell trait against malaria.
Sickle cell anemia is rare in the United States, affecting about around 100,000 people. It mostly affects people whose ancestry links back to parts of the world where many people have malaria and carry a gene that provides partial protection against anemia. This gene also causes sickle cell anemia. In the United States, sickle cell anemia affects many people who are Black. It may also affect people from southern European, Middle Eastern or Asian Indian ancestry.
People with sickle cell anemia inherit the disease from their biological parents. In sickle cell anemia, the gene that helps make normal red blood cells mutates, or changes. People who inherit the mutated hemoglobin protein gene from both biological parents have sickle cell anemia. People who inherit the mutated gene from one biological parent have the sickle cell trait.
Sickle cell anemia symptoms typically start when babies are 5 to 6 months old. As they grow older, most people with sickle cell anemia have increased risk for developing new medical conditions. Some of these conditions are life-threatening. But by learning about conditions and symptoms, people with sickle cell anemia can seek help at the first sign of trouble so healthcare providers can treat the condition.
Healthcare providers may call this condition acute pain crisis. VOC, or acute pain crises, is the most common reason why people with sickle cell anemia go to the emergency room or need to spend time in the hospital. Symptoms include:
Anyone who has sickle cell anemia is at risk for stroke, including babies. Approximately 11% of people with sickle cell anemia have strokes by age 20, and 24% have strokes by age 45. Here is information on stroke symptoms:
Right now, allogenic stem cell transplantation is the only way healthcare providers can cure sickle cell anemia and other forms of sickle cell disease. Healthcare providers typically recommend transplantation for people who have severe complications such as stroke, acute chest syndrome or recurring VOC/acute pain crises.
Healthcare providers typically treat sickle cell anemia with blood transfusions, antibiotics to treat infections and medications that reduce symptoms caused by sickle cell anemia complications. These medications may include hydroxyurea, voxlelotor, L-glutamine therapy and crizanlizumab.
L-glutamine helps reduce some of the complications linked to sickle cell anemia. Sickled cells develop over time. L-glutamine helps protect sickled cells from becoming more misshapen. In 2017, the FDA approved Endari for treatment of children age 5 and older and adults.
Sickle cell anemia is an inherited disorder. You can have a blood test to find out if you have sickle cell trait that you could pass on to your children. People can have sickle cell trait without having sickle cell disease or sickle anemia.
New treatments are helping people with sickle cell anemia live longer and with better quality of life. The U.S. Centers for Disease Control and Prevention (CDC) has the following suggestions for living well with sickle cell anemia:
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.
Early diagnosis and prevention of complications is critical in sickle cell disease treatment. Treatment aims to prevent organ damage including strokes, prevent infection, and treat symptoms. Treatment may include:
Any and all major organs are affected by sickle cell disease. The liver, heart, kidneys, gallbladder, eyes, bones, and joints can suffer damage from the abnormal function of the sickle cells and their inability to flow through the small blood vessels correctly. Problems may include the following:
Sickle cell disease is a life-long condition. Although the complications of sickle cell disease may not be able to be prevented entirely, living a healthy life-style can reduce some of the complications.
Approximately 70,000 to 100,000 Americans have sickle cell disease, the most common form of an inherited blood disorder. This disease, which is present in affected individuals at birth, causes the production of abnormal hemoglobin. Normally, the hemoglobin protein, which resides inside red blood cells, attaches to oxygen in the lungs and carries it to all parts of the body. Healthy red blood cells are flexible so that they can move through the smallest blood vessels. In sickle cell disease, the hemoglobin is abnormal, causing the red blood cells to be rigid and shaped like a "C" or sickle, the shape from which the disease takes its name. Sickle cells can get stuck and block blood flow, causing pain and infections. Complications of sickle cell disease occur because the sickled cells block blood flow to specific organs. The worst complications include stroke, acute chest syndrome (a condition that lowers the level of oxygen in the blood), organ damage, other disabilities, and in some cases premature death.
In order for you or your child to inherit sickle cell disease, both parents must have either sickle cell disease (two sickle cell genes) or sickle cell trait (one sickle cell gene). There are variations of sickle cell disease called sickle C or sickle thalassemia, which are serious conditions but are sometimes less severe. If you have sickle cell disease, you will pass one sickle cell gene to your children.
Sickle cell trait is an inherited blood disorder that affects approximately 8 percent of African-Americans. Unlike sickle cell disease, in which patients have two genes that cause the production of abnormal hemoglobin, individuals with sickle cell trait carry only one defective gene and typically live normal lives without health problems related to sickle cell. Rarely, extreme conditions such as severe dehydration and high-intensity physical activity can lead to serious health issues, including sudden death, in individuals with sickle cell trait.
Because sickle cell disease symptoms can begin by four months of age, early diagnosis is critical. All newborns in the United States are now tested for the disease. Sickle cell disease can be identified before birth by testing a sample of amniotic fluid or tissue from the placenta. People who carry the sickle cell gene can seek genetic counseling before pregnancy to discuss options. 041b061a72