The Basics
Written by the doctors and editors at UpToDate � �
What is sickle cell anemia? " � Sickle cell anemia, also called sickle cell disease, is a condition that affects the red blood cells. The red blood cells are the part of the blood that carries oxygen to organs in the body. Normal red blood cells are round. � �
When people have sickle cell anemia, their red blood cells have an abnormal shape. They look like a crescent (or an old-time tool called a "sickle " �). The abnormal red blood cells get stuck in the blood vessels easily, and so they dont bring enough oxygen to the body's organs. � �
Sickle cell anemia is a life-long condition that people are born with. It is caused by an abnormal gene. To get the disease, people need to get the abnormal gene from both their mother and father. If people get the abnormal gene from only 1 parent, it's called "sickle cell trait. " � People with sickle cell trait do not usually have any of the symptoms of sickle cell anemia. � �
What are the symptoms of sickle cell anemia? " � Symptoms usually start after a baby is a few months old. Most commonly, people with sickle cell anemia have episodes of pain (also known as a sickle cell "crisis " �). The pain is usually in the bones of the back, chest, belly, arms, or legs. It can be mild or severe, and last hours to days. People can have other symptoms with their pain, including: � �
- Fever
- Swelling (for example, in the hands or feet)
- Nausea or vomiting
Often, certain things cause the pain. These things can include: � �
- Infection
- Dehydration " � Dehydration is when the body loses too much water.
- Stress
- High-altitude places " � These are places where there is less oxygen in the air, such as mountains.
Sickle cell anemia can also cause many different health problems, including: � �
- Anemia " � Anemia is a condition in which the body has too few red blood cells. It makes people tired or weak.
- Getting a lot of infections
- Strokes " � A stroke is when a part of the brain dies.
- Open sores on the skin (usually on the legs)
- An erection that lasts too long and is very painful (in men)
Is there a test for sickle cell anemia? " � Yes. A blood test can show if someone has sickle cell anemia. All newborn babies (in the US) are tested for sickle cell anemia before they leave the hospital. � �
Some parents choose to test their unborn baby for sickle cell anemia. This can be done in different ways. � �
How is sickle cell anemia treated? " � Sickle cell anemia involves life-long treatment. Some treatments help prevent infections, pain, and other problems. These include: � �
- Antibiotics " � Daily antibiotics help prevent infections in young children.
- Vaccines " � Making sure you get all your vaccines helps prevent infections
- Hydroxyurea (sample brand names: Droxia, Hydrea) " � This medicine helps prevent episodes of pain.
- Blood transfusions " � A blood transfusion is when a person gets blood that was given by another person. People who have a high chance of having a stroke might get blood transfusions to help prevent strokes. People might also get blood transfusions to treat anemia.
- A vitamin called folic acid (also called folate)
- Pain medicines
People with sickle cell anemia are also treated for: � �
- Their pain " � Doctors can use different medicines to treat pain. If the pain is severe, people might need treatment in the hospital.
- Infections " � It is important to be treated for infections right away (even if you get a fever in the middle of the night).
- Any other problems caused by the disease
A procedure called a "bone marrow transplant " � or "blood stem cell transplant " � can cure sickle cell anemia. This procedure replaces the cells that make your blood with healthy cells. But this treatment is not done very often for sickle cell anemia. That's because it has many side effects and can be done only if people meet certain conditions. � �
When should I call the doctor or nurse? " � You should call the doctor or nurse right away if the person with sickle cell anemia: � �
- Gets a fever (temperature higher than 101.5 � � � or 38.5 � � �) or infection
- Has pain that doesn't get better after it is treated at home
What else can people with sickle cell anemia do to stay healthy? " � People with sickle cell anemia should: � �
- See their doctor for regular check-ups, and follow his or her instructions about treatments and tests.
- Get the vaccines that the doctor or nurse recommends. Vaccines are treatments that can prevent certain serious infections.
- Avoid getting dehydrated, stressed, or being in high-altitude places.
What if I have sickle cell anemia and want to get pregnant? " � If you have sickle cell anemia and want to get pregnant, talk with your doctor or nurse. He or she might recommend that you see a "genetic counselor. " � This person can tell you your baby's chance of having sickle cell anemia or sickle cell trait. � �
If you have sickle cell anemia and are pregnant, your doctor will follow you and your baby closely throughout your pregnancy. That's because sickle cell anemia can cause problems for both you and your baby. � �
All topics are updated as new evidence becomes available and our peer review process is complete. � �
This topic retrieved from UpToDate on: Nov 04, 2014. � �
Topic 16730 Version 5.0 � �
Release: 22.8 - C22.206 � �
� � 2014 UpToDate, Inc. All rights reserved. � �
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