Sickle Cell Disease - Topic Overview

What is sickle cell disease?

Sickle cell disease changes normal, round red blood cells into cells that can be shaped like crescent moons. The name "sickle cell" comes from the crescent shape of the cells. (A sickle is a tool with a crescent-shaped blade.)

Normal red blood cells move easily through your blood vessels, taking oxygen to every part of your body. But sickled cells can get stuck and block blood vessels, which stops the oxygen from getting through. That can cause a lot of pain. It can also harm organs, muscles, and bones.

See a picture of sickle cells blocking a blood vessel .

Having sickle cell disease means a lifelong battle against the health problems it can cause, such as pain, infections, anemia, and stroke. But many people are able to have a very good quality of life by learning to manage the disease.

What causes sickle cell disease?

Sickle cell disease is inherited, which means it is passed from parent to child. To get sickle cell disease, a child has to inherit two sickle cell genes-one from each parent.

When a child inherits the gene from just one parent, that child has sickle cell trait. Having this trait means that you don't have the disease but you are a carrier and could pass the gene on to your children.

What are the symptoms?

Painful events (sickle cell crises) are the most common symptom of sickle cell disease. They are periods of pain that happen when sickled cells get stuck in blood vessels and block the blood flow. These events usually cause pain in the hands, feet, belly, back, or chest. The pain may last for hours or for days.

People with sickle cell disease often have anemia, caused by a shortage of red blood cells. Anemia makes you feel weak and tired. People with sickle cell anemia may look pale or washed out. Their skin and the whites of their eyes may have a yellowish look (jaundice).

How is sickle cell disease diagnosed?

A simple blood test can show whether a person has sickle cell disease. Most states test for sickle cell disease before infants go home from the hospital.

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How is it treated?

Self-care and medical treatment can help you manage pain and avoid other health problems.

Early treatment includes daily antibiotics from 2 months to 5 years of age to help prevent infections. Routine childhood and adult immunizations are also important.

Managing pain is often a big part of having sickle cell disease. You can prepare for a painful event ahead of time by creating a pain management plan with your doctor. The plan should include what you can do at home to relieve pain for yourself or your child. The plan should also tell you when it is best to call a doctor or go to a hospital.

Some people need regular blood transfusions to lower the risk of stroke and treat anemia and other problems. Some people take medicine to prevent complications. In rare cases, a stem cell transplant might be an option.

Regular checkups are an important part of life with this disease. People with sickle cell disease need a good working relationship with a doctor who is an expert in treating it.

How do you manage life with sickle cell disease?

Tips for you

• Learn what triggers, or sets off, painful events called sickle cell crises. Triggers often include cold temperatures, wind, dehydration, and too much exercise. Low oxygen caused by cigarette smoke, high altitude, and plane flights is another common trigger.
• People with sickle cell disease and their families face ongoing stress. A support network can help ease stress and worry. Ask your doctor if there is a support group in your area.

Tips for helping your child

• Make sure that your child takes antibiotics regularly until age 5 to prevent infections. And make sure he or she receives all the usual immunizations on schedule.
• Your child can take part in normal school activities. Make sure that teachers understand your child's special needs, like needing frequent drinks and bathroom trips and avoiding overexertion and cold temperatures.

Frequently Asked Questions

Learning about sickle cell disease:	What is sickle cell disease? What causes it? What are the symptoms? What are the different types of sickle cell disorders?
Being diagnosed:	Who can diagnose sickle cell disease? How is it diagnosed?
Getting treatment:	How is sickle cell disease treated? Will I need surgery? Which medicines will I need to take?
Ongoing concerns:	When should I call my doctor? What complications can develop?
Living with sickle cell disease:	What skills will help me manage pain? What can I do to stay healthy?