Sickle cell disease
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Sickle cell disease: Essentials
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What are the symptoms of sickle cell disease?

Pain is the most common symptom of sickle cell disease. Attacks of pain happen to almost everyone with sickle cell disease at some point in their life. The pain can last for hours or days. It's sometimes only mild, but it can be extremely bad.

Normal
 
 
 
 
 
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 are disk-shaped. They look a bit like a doughnut. They can move freely through your blood vessels. If you have sickle cell disease, your red blood cells 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. You can get pain in any part of your body.

Some people get pain only every few years. But other people get pain quite often.
 
 
 
 
 
Source:
Sickle Cell Society.
Information for health professionals.
Available at http://www.sicklecellsociety.org/education/healthpr.htm (accessed on 28 August 2007).
 
 
 
 
 
1
 
 
 
 
 
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).
 
 
 
 
 
2 The pain in sickle cell disease is sometimes described as throbbing, sharp or gnawing.
 
 
 
 
 
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.
 
 
 
 
 
3

If you're looking after a child who has sickle cell disease, it's important to get used to the signs that they're in pain. Younger children can't always explain very well what they're feeling or where something hurts.

Other symptoms
Feeling very tired
Red blood cells carry oxygen around your body. Sickle cells can't carry oxygen as well as normal red blood cells. And they don't last as long. Normal red blood cells live about 120 days, but sickle cells live from only 10 to 20 days.
 
 
 
 
 
Source:
Tanyi RA.
Sickle cell disease: health promotion and maintenance and the role of primary care nurse practitioners.
Journal of the American Academy of Nurse Practitioners. 2003; 15: 389-397.
 
 
 
 
 
4 When your blood can't carry enough oxygen around your body, it's called anemia. It can mean you:

  • Get tired easily
  • Look pale
  • Get breathless
  • Have a fast heartbeat.
Anemia can get worse if you get an
 
 
 
 
 
infection
You get an infection when viruses, bacteria, fungi or other tiny organisms get into your body. These bugs are so tiny that you can't see them without a microscope. For example, an infection in your airways causes the common cold. And an infection in your skin can cause rashes such as athlete's foot.
 
 
 
 
 
infection. It can also get worse if you have a problem with your
 
 
 
 
 
spleen
Your spleen is an organ that sits on the left side of your body just below your ribs. It helps your body fight infections.
 
 
 
 
 
spleen. Red blood cells can get stuck in your spleen so they can't travel round your body. This is called a sequestration crisis. You should see a doctor if you suddenly feel a lot worse than usual. If you have a child with sickle cell disease, make sure they see a doctor if they seem worse than usual or get stomach pain.

Infections
If you have sickle cell disease, you're more likely to catch infections, especially when you're young. This is partly because sickle cell disease can damage your spleen, which is part of your body's system for fighting infections.

Swollen hands and feet
 
 
 
 
 
inflammation
If your skin or some other part of your body becomes red, swollen, hot or sore, we say it is inflamed. It means that your body is trying to protect you from germs, from something in your body tissues that can hurt you (like a thorn or sliver), or from things that cause allergies (allergens). Inflammation is part of the way the body heals an infection or injury.
 
 
 
 
 
Inflammation of the bones of the hands and feet can make them red, swollen and painful. It's common in young children. It's known as hand and foot syndrome.

Jaundice
Your eyes or skin may look slightly yellow even if you are well. This happens because your red blood cells get broken down more quickly than normal.

Sickle cell trait
If you have sickle cell trait, you won't get any of the symptoms of sickle cell disease. But you may need extra oxygen if you have
 
 
 
 
 
general anesthetic
You may have a type of medication called a general anesthetic when you have surgery. It's given to make you unconscious so you don't feel pain.
 
 
 
 
 
general anesthetic for an operation. And you're advised not to do some sports where your oxygen supply may be less, such as scuba diving or climbing very high mountains.
 
 
 
 
 
Source:
Sickle Cell Society.
What is sickle cell anaemia?
Available at http://www.sicklecellsociety.org/education/sicklecell.htm (accessed on 28 August 2007).
 
 
 
 
 
5 To read more about sickle cell trait, see What is sickle cell disease?

Sources for the information on this page:
  1. Sickle Cell Society.Information for health professionals.Available at http://www.sicklecellsociety.org/education/healthpr.htm (accessed on 28 August 2007).
  2. 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).
  3. 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.
  4. Tanyi RA.Sickle cell disease: health promotion and maintenance and the role of primary care nurse practitioners.Journal of the American Academy of Nurse Practitioners. 2003; 15: 389-397.
  5. Sickle Cell Society.What is sickle cell anaemia?Available at http://www.sicklecellsociety.org/education/sicklecell.htm (accessed on 28 August 2007).
This information was last updated on Nov 06, 2008
BMJ Group
This information is for educational use only, and is not a substitute for prompt professional medical advice. Readers should always consult a physician or other professional for advice and treatment.
© BMJ Publishing Group Limited 2009. All rights reserved.
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