Sickle Cell Disease Treatment: What to Expect

If you have sickle cell disease, your red blood cells aren’t round and stretchy like they should be.   Instead, they’re stiff and sticky. They’re also shaped like a crescent or a sickle and get wedged in small blood vessels. That makes it harder for your blood to carry oxygen through your body, which can leave you tired, in pain, out of breath, and more likely to pick up infections.

Sickle cell disease can affect different organs and parts of your body. The best way to manage your condition is to see your doctor or hematologist as early as possible and to stick with your treatments for the long term.

Pain

When your red blood cells get trapped in your vessels, they not only cut off oxygen but can set off bouts of great pain. It’s called a sickle cell crisis.

You might get relief with over-the-counter pain relievers like ibuprofen or acetaminophen. Drinking lots of fluids helps, too.

You can also try:

If you’re in severe pain, you may need to go to a hospital for a day or more. You can get stronger painkillers and fluids directly into a vein.

A medicine called hydroxyurea (Droxia, Hydrea, Mylocel) prevents abnormal red blood cells from forming. This cuts down on episodes of sickle cell crises. Hydroxyurea can cause serious side effects, like a plunge in the number of white blood cells that help your body fight infections. So your doctor will watch you closely while you're on it. You shouldn’t take hydroxyurea if you’re pregnant.  

Your doctor may prescribe a newer drug called L-glutamine oral powder (Endari). It can cut down on your trips to the hospital for pain and also guard against a condition called acute chest syndrome. 

If you have long-term pain, your doctor might prescribe one of these medicines:

Severe Anemia

It’s a serious condition that can land you in the hospital. It happens when you don’t have enough red blood cells to move oxygen throughout your body.

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One way to treat anemia is with a blood transfusion. You'll get healthy red blood cells from a donor whose blood has been screened for infections and matched to your type and needs. Your doctor will pick one of several ways to do it:

Simple transfusion. The normal red blood cells from your donor’s blood are separated from the rest of the blood. The new red blood cells are added to your own blood through an IV in your arm, or a port if you have one. This usually takes about 4 hours.

Partial exchange. You replace some of your whole blood with healthy donor cells. Your blood can be drawn before or at the same time as your transfusion, with an IV or a line in each arm.

Rapid partial exchange. You switch out the same amount of your whole blood for whole blood from your donor. How much will depend on your size and the percentage of red blood cells you have. This is the quickest way to break up the sickle cells.

Infections

Sickle cells can damage your spleen, the organ that helps fight germs as part of your immune system. That can make you more likely to get pneumonia and other infections. Children with sickle cell disease should take penicillin from ages 2 months to 5 years. Some adults will also need to take antibiotics, including those who’ve had their spleen removed.

If you catch an infection and your fever is over 101 F, you'll need to get treated with antibiotics right away. You may have to stay in a hospital overnight or longer.

Vaccines can protect you and your child against germs. Everyone with sickle cell disease should get these vaccines:

  • Pneumococcus: both the PCV13 and PPSV 23 vaccines
  • Flu vaccine every year
  • Meningococcus 

Your doctor will also advise you about other vaccines that may be right for you or your child, including:

  • Hepatitis A and B
  • Measles, mumps, and rubella
  • Varicella (chickenpox)
  • Rotavirus
  • Haemophilus influenzae
  • Tetanus, diphtheria, and pertussis
  • Poliovirus

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Stroke

When sickle cells get stuck in the larger arteries that feed the brain, they can block blood flow and cause a stroke.

You can prevent this with regular blood transfusions using either the simple or exchange method. Studies show that a transfusion every 3 or 4 weeks can lower the odds of another stroke by about 90%

If you think you're having a stroke, call 911 right away. You may need a rapid exchange transfusion. If your child has sickle cell disease, your doctor will do an ultrasound test to measure blood flow to the brain and to check for strokes.

Stem Cell or Bone Marrow Transplants

They’re the only way to cure sickle cell disease. Stem cells are immature cells in your bone marrow that grow into new red or white blood cells and platelets (cells that help form blood clots).

Transplants are usually done in children under age 16 who've had sickle cell complications like pain and strokes. Your child first gets strong chemotherapy drugs to destroy her bone marrow and damaged red blood cells. Then, she gets healthy bone marrow or stem cells from another person. Her bone marrow then will start to make healthy red blood cells.

Older people don’t get transplants as often because they’re more likely to have complications.

WebMD Medical Reference Reviewed by Laura J. Martin, MD on April 14, 2018

Sources

SOURCES:

Mayo Clinic: "Sickle Cell Anemia: Symptoms and Causes,” “Sickle Cell Anemia: Diagnosis and Treatment."

National Heart, Lung, and Blood Institute: "How Is Sickle Cell Disease Treated?" "What Are the Signs and Symptoms of Sickle Cell Disease?"

Indiana Hemophilia and Thrombosis Center: “Transfusion Therapy.”

St. Jude Children's Research Hospital: "Bone Marrow (Stem Cell) Transplant for Sickle Cell Disease," “Red Blood Cell Transfusions for Sickle Cell Disease.”

The Internet Stroke Center: "Stroke as a Complication of Sickle Cell Disease," “Blood Transfusions for Children With Sickle Cell Disease.”

U.S. National Library of Medicine: "Hydroxyurea."

American Family Physician: “Practical Tips for Preventing a Sickle Cell Crisis.”

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