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Sickle Cell Disease

What Is Sickle Cell Disease?

Sickle cell disease is a condition in which red blood cells are not shaped as they should be. Red blood cells usually look like round discs. But in sickle cell disease, they're shaped like crescent moons, or an old farm tool known as a sickle.

These sickle shaped cells get stuck together easily, and block off small blood vessels. When blood can't get to where it should, it can lead to pain and damage to the body's organs.

sickle cell

What Are the Signs & Symptoms of Sickle Cell Disease?

People with sickle cell disease can have pain when blood can't get to parts of the body. These times are called pain crises.

Pain may happen in any part of the body and may be brought on by cold, stress, or dehydration. The pain may last a few hours, a few days, or sometimes longer. Sometimes pain can be managed at home. But someone with severe pain might need treatment in a hospital.

People with sickle cell disease often have a low number of red blood cells, or anemia. Signs of anemia include:

  • paleness, often seen in the skin, lips, or nailbeds
  • tiredness
  • dizziness
  • being short of breath
  • feeling lightheaded
  • being irritable
  • trouble paying attention
  • a fast heartbeat

People with sickle cell anemia may have . This happens because the sickle-shaped red blood cells break down faster than normal cells. Jaundice can make the skin and whites of the eyes look yellow.

What Problems Can Happen?

People with sickle cell disease can have problems that need immediate care by a doctor, such as:

  • Acute chest syndrome: Caused by inflammation, infection, and blockages of small blood vessels of the lung. Signs include chest pain, coughing, trouble breathing, and fever.
  • Aplastic crisis: This is when the body temporarily does not make enough red blood cells, and can cause severe anemia. Signs include paleness, extreme tiredness, and a fast heartbeat.
  • Infection: People with sickle cell disease are a risk for some bacterial infections. It's important to watch for fevers of 101°F (38°C) or higher, which can be signs of an infection.
  • Priapism: Guys with sickle cell disease can have painful, lasting erections. If it's not treated quickly, damage can cause problems with getting erections later on.
  • Stroke: Sickle-shaped cells can block small blood vessels in the brain, causing a stroke. Signs can include headache, , weakness of the arms and legs, speech problems, a facial droop, or loss of consciousness.

People with sickle cell disease are also at risk for problems such as leg ulcers, bone or joint damage, gallstones, kidney damage, eye damage, and delayed growth.

What Causes Sickle Cell Disease?

Sickle cell disease is not contagious, so you can't catch it from someone else or pass it to another person like a cold or an infection.

People with sickle cell disease have it because they inherited two sickle cell , one from each parent.

A person who inherits the sickle cell gene from only one parent will not develop the disease, but will have something called . People with sickle cell trait often don't have any signs of the disease, but they can pass the sickle cell gene to their children.

How Is Sickle Cell Disease Treated?

Stem cell transplant (also called bone marrow transplant) is the only known cure for sickle cell disease. Transplants are complex and risky procedures, and for now are an option only for some patients.

Scientists are studying gene therapy as a treatment for sickle cell anemia. One day, it's hoped that doctors can stop the disease by changing or replacing the abnormal gene that causes it.

But even without a cure, people with sickle cell disease can lead fairly normal lives if they follow their treatment plan. Their plan might involve:

  • Having all recommended childhood vaccinations, as well as the pneumococcal, flu, and meningococcal vaccines.
  • Taking folic acid supplements to help them continue to make new red blood cells.
  • Taking hydroxyurea, a medicine that makes sickled red blood cells less sticky. This helps people have fewer painful episodes and other complications. Hydroxyurea needs to be taken every day in order to work.
  • Taking L-glutamine, another medicine. It's used if hydroxyurea isn't working well or someone still has pain crises even with hydroxyurea.
  • Taking medicines to help when pain does happen.
  • Getting a blood transfusion.

What Can Teens With Sickle Cell Disease Do to Feel Better?

Having sickle cell disease can be frustrating and sometimes painful. To take control sickle cell disease:

  • Stay on top of your appointments and medicines.
  • Avoid your pain crisis triggers, such as extreme temperatures or stress.
  • Talk to your doctor about which activities are right for you and which you should avoid.
  • Don't smoke, drink alcohol, or use drugs.
  • Drink lots of fluid and get enough rest.
  • Let an adult know if you don't feel well.