Red blood cells carry hemoglobin, a protein that 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 anemia, 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 anemia are a result of sickle cells blocking blood flow to specific organs, and include stroke, acute chest syndrome (a condition that lowers the level of oxygen in the blood), organ damage, and blindness. Since researchers first identified sickle cell disease more than 100 years ago, numerous advances have been made in the treatment and care of sickle cell patients.
Sickle cell anemia is an inherited disease. In order for you to get sickle cell anemia, both of your parents must have either sickle cell anemia (two sickle cell genes) or sickle cell trait (one sickle cell gene). If you inherit a sickle cell gene from both parents, you will have sickle cell disease. If you have sickle cell disease, you will pass one sickle cell gene to your children.
The Problem With Sickled Cells
If you inherit one sickle cell gene from one parent, you will carry the sickle cell trait. If you have sickle cell trait, there is a 50 percent chance that you will pass the sickle cell gene to your children.
Am I at Risk?
The National Heart, Lung, and Blood Institute reports that more than 70,000 people in the United States have sickle cell anemia. Sickle cell anemia is more common in certain ethnic groups including:
- People of African descent, including African-Americans (among whom 1 in 12 carries a sickle cell gene)
- Hispanic–Americans
- People of Saudi Arabian, Caribbean, and South and Central American Indian descent
Because sickle cell anemia symptoms usually begin at four months of age, early diagnosis is critical. All newborns in the United States are now tested for the disease. Sickle cell anemia 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.
What Are the Signs and Symptoms of Sickle Cell Anemia?
Signs and symptoms can be mild or severe enough to require frequent hospitalizations. They may include:
- Anemia
- Dark urine
- Hand-foot syndrome, which causes your hands and feet to swell
- Frequent pain episodes
- Stunted growth
- Vision problems
- Stroke
How Is Sickle Cell Anemia Treated?
There are no standard treatments that cure sickle cell anemia. However, there are treatments that help people manage and live with the disease. Treatment relieves pain, prevents infections, minimizes organ damage, and controls complications and can include medications, such as pain relievers and hydroxyurea (Hydrea), blood transfusions, and other options as needed.
Clinical trials provide access to experimental therapies for treating sickle cell anemia. ASH provides information on clinical trials for which you may be eligible. Researchers are looking at new drugs and also exploring the use of bone marrow transplants to treat sickle cell disease. Stem cell transplants are associated with significant risks and are appropriate only for some patients with severe forms of sickle cell disease and closely matched donors such as a family member.
It is important for you to talk with your doctor if you believe you may have sickle cell anemia. If you carry the sickle cell trait, make sure you tell your doctor before getting pregnant as well. Depending on your condition, your doctor may refer you to a hematologist, a doctor who specializes in blood conditions.
Sickle Cell Anemia: A Patient's Journey
Where Can I Find More Information?
If you find that you are interested in learning more about blood diseases and disorders, here are a few other resources that may be of some help:
Articles From Hematology, the ASH Education Program
The American Society of Hematology (ASH) Education, updated yearly by experts in the field, is a collection of articles about the current treatment options available to patients. The articles are categorized here by disease type. If you are interested in learning more about a particular blood disease, we encourage you to share and discuss these articles with your doctor.
Results of Clinical Studies Published in Blood
Search Blood, the official journal of ASH, for the results of the latest blood research. While recent articles generally require a subscriber login, patients interested in viewing an access-controlled article in Blood may obtain a copy by e-mailing a request to the Blood Publishing Office.
Other Resources
This section includes a list of Web links to patient groups and other organizations that provide information.
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