Sickle cell disease is sometimes called sickle cell anemia. That's because it causes a condition called

 

 

 

 

 

anemia
Anemia is when you have too few red blood cells. Anemia can make you get tired and breathless easily. It can also make you look pale. Anemia can be caused by a number of different things, including problems with your diet, blood loss and some diseases.

 

 

 

 

 

anemia, which can make you feel very tired.

Sickle cell disease affects a chemical in your body called hemoglobin. It's found in

 

 

 

 

 

red blood cells
Red blood cells are the part of your blood that makes it red. Their main job is to carry oxygen from your heart and lungs out to the tissues of your body. Once these cells unload oxygen, they pick up carbon dioxide. They take carbon dioxide back to your lungs so you can breathe it out of your body.

 

 

 

 

 

red blood cells. It's the hemoglobin in your red blood cells that carries oxygen around your body when you breathe. If you have sickle cell disease, your body makes a kind of hemoglobin that doesn't carry oxygen as well as it should.

Sickle cell disease is a condition that you are born with. It's caused by an abnormal

 

 

 

 

 

genes
Your genes are the parts of your cells that contain instructions for how your body works. Genes are housed on chromosomes, structures that sit in the nucleus at the middle of each of your cells. You have 23 pairs of chromosomes in your normal cells, each of which has thousands of genes. You get one set of chromosomes, and all of the genes that are on them, from each of your parents.

 

 

 

 

 

gene that can be passed down from your parents.

 

 

 

 

 

Source:
U.S. National Library of Medicine.
Medline Plus medical encyclopedia: sickle cell anemia.
February 2007. Available at http://www.nlm.nih.gov/medlineplus/ency/article/000527.htm (accessed on 24 August 2007).

 

 

 

 

 

¹

 

 

 

 

 

Source:
Sickle Cell Society.
What is sickle cell anaemia?
Available at http://www.sicklecellsociety.org/education/sicklecell.htm (accessed on 28 August 2007).

 

 

 

 

 

² You get the disease only if you inherit this abnormal gene from both parents.

You inherit two genes that tell your body how to make hemoglobin, one from your mother and one from your father.

A normal gene tells you body how to make normal hemoglobin, called hemoglobin A. An abnormal gene tells your body how to make sickle hemoglobin, or hemoglobin S. If you inherit hemoglobin S genes from both your parents, you get sickle cell disease.

Red blood cells that contain hemoglobin S don't work as well as ordinary red blood cells. Red blood cells are normally disk-shaped. They look a bit like a doughnut. They can move freely through your blood vessels. But red blood cells that contain hemoglobin S can change into a sickle, or crescent, shape. These sickle cells can get stuck in your small blood vessels. This stops blood from getting to part of your body and causes pain.

If you inherit one normal gene and one abnormal gene, you don't get sickle cell disease. But you do have something called sickle cell trait. This means that you still carry the gene, and can pass it on to your children. If your partner also carries the abnormal gene, your children could be born with sickle cell disease. If you and your partner both have sickle cell trait, you have a 1 in 4 chance of having a baby with sickle cell disease each time you're pregnant.

 

 

 

 

 

Source:
Meremikwu M.
Sickle cell disease.
Clinical Evidence. 2006; 15: 1-3.

 

 

 

 

 

³

There are other kinds of faulty hemoglobin genes. If you inherit a sickle cell gene and another kind of faulty gene, you can get a different type of sickle cell disease.

You can have a blood test to find out if you have sickle cell disease or sickle cell trait.

 

 

 

 

 

Source:
Sickle Cell Society.
What is sickle cell anaemia?
Available at http://www.sicklecellsociety.org/education/sicklecell.htm (accessed on 28 August 2007).

 

 

 

 

 

² In almost all states, newborn babies are tested for sickle cell disease.

 

 

 

 

 

Source:
March of Dimes.
Newborn screening tests.
Available at http://www.marchofdimes.com/professionals/14332_1200.asp (accessed on 24 August 2007).

 

 

 

 

 

⁴ If you're a parent, ask your doctor to explain exactly what the test results mean. If the test is positive, make sure your doctor tells you whether your child has sickle cell disease or just one abnormal gene (sickle cell trait).

People with sickle cell disease can get severe pain. These attacks are often called crises. If your red blood cells don't have enough oxygen, they change from soft round disks into hard, brittle sickle shapes. These sickle-shaped red blood cells get stuck in your small blood vessels. This can cause pain.

Babies don't usually start to get attacks of sickle cell pain until they're 3 to 6 months old.

 

 

 

 

 

Source:
Sickle Cell Society.
Information for health professionals.
Available at http://www.sicklecellsociety.org/education/healthpr.htm (accessed on 28 August 2007).

 

 

 

 

 

⁵ Another type of hemoglobin (called fetal hemoglobin, or hemoglobin F) protects babies in the womb and while they're very young.

We don't know for certain what brings on an attack of pain. But these things may play a part:

 

 

 

 

 

Source:
Meremikwu M.
Sickle cell disease.
Clinical Evidence. 2006; 15: 1-3.

 

 

 

 

 

³

You may get more sickle cell pain if you're pregnant, especially during the last three months before the birth.

 

 

 

 

 

Source:
Rees DC, Olujohungbe AD, Parker NE, et al.
Guidelines for the management of the acute painful crisis in sickle cell disease.
British Journal of Haematology. 2003; 120: 744-752.

 

 

 

 

 

⁶

 

 

 

 

 

malaria
Malaria is a dangerous but treatable disease caused by a parasite. This parasite lives in mosquitoes in parts of Asia, Africa and South America. You can catch it if you are bitten by an infected mosquito. Malaria can give you symptoms similar to the flu (influenza). The most common symptom is a fever.

 

 

 

 

 

Malaria (a condition spread by mosquito bites) can cause attacks of sickle cell pain. In tropical Africa, malaria is the most common trigger for pain.

 

 

 

 

 

Source:
Ohene-Frempong K, Nkrumah FK.
Sickle cell disease in Africa.
In: Embury SH, Hebbel RP, Mohandas N, et al (editors). Sickle cell disease: basic principles and clinical practice. Raven Press, New York, NY, USA; 1994.

 

 

 

 

 

⁷

The information on ConsumerReportsHealth.org should not be viewed as a substitute for a consultation with a medical or health professional. The information is meant to enhance communication with your doctor, not replace it. Consumers Union can not be liable for any loss, injury, or other damages related to your use of this information.

Your use of this information is subject to our User Agreement available at www.ConsumerReportsHealth.org.