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Understanding your sickle cell disease.

What happens in SCD.

Sickle cell disease (SCD) is a group of red blood cell disorders that affect the shape of your hemoglobin. Hemoglobin is the main part of the red blood cell. Healthy red blood cells have hemoglobin A and are round and soft. They move easily through your small blood vessels to carry oxygen to all parts of the body.

With SCD, you make a different type of hemoglobin called hemoglobin S. Hemoglobin S is c-shaped like the farm tool called a sickle. This change in shape makes the red blood cells hard and sticky. When they move through your small blood vessels, they get stuck and can block the flow of blood and oxygen. This can damage the part of your body where the blood flow gets clogged. It is this damage that causes many of the possible problems of SCD.

How SCD affects you.

SCD symptoms vary from person to person. Your symptoms may also change over time. They can range from mild to severe. Depending on where the blood flow is blocked, you may have symptoms all of a sudden or they may develop over time. Here are some problems that can occur with SCD.

  • Pain. This is a common problem. You may notice pain in your chest, stomach (abdomen), arms and legs. Sometimes your hands and feet swell up. Pain can start suddenly (called a pain crisis), be mild to severe and can last for any length of time.
  • Anemia. This is another common problem. Having too few red cells is called anemia and it can make you feel very tired. With SCD, the red blood cells live for a shorter period of time, so you might not have enough healthy red blood cells to carry oxygen through your body.
  • Infection. Your spleen helps prevent infections. Your spleen may become damaged due to blocked blood flow. You’re more likely to get infections if your spleen is damaged.
  • Splenic sequestration crises. Sickle cells can get trapped in the spleen. It can cause a quick drop in the number of red blood cells you have. This is a serious condition that requires medical help right away.
  • Stroke. Blocked blood flow to the brain can cause a stroke. Watch for signs like weakness in your arms and legs, speech problems and facial drooping. Get medical help right away if you notice any of these symptoms. Your doctor may also suggest regular screenings for stroke.
  • Acute chest syndrome. Inflammation (swelling), infection or blocked blood vessels can cause this serious condition. Watch for signs like chest pain, coughing, trouble breathing and fever. Get medical help right away if you notice any of these symptoms.
  • Priapism. Males with SCD can have painful erections that last a long time. You should go to the emergency room if yours lasts longer than 2 to 3 hours.
  • Vision problems. When blood flow is blocked to the blood vessels in your eye, it can cause serious vision problems, including blindness.

What you can do.

SCD is a life-long condition. The only cure is a blood cell (also called bone marrow) transplant. A transplant has some serious risks, but it can be an option for some people. Treatments are available to help reduce symptoms and manage your SCD. Talk to your doctor about a treatment plan that’s best for you.

You can help yourself stay healthier and prevent problems with these tips:

  • Drink at least eight to ten glasses of water every day
  • Stay as active as you can
  • Avoid infections by washing your hands well and staying away from people who are sick
  • Take all your medications as directed by your doctor
  • Keep up with your vaccinations
  • Avoid high elevations, including flying in a plane
  • Avoid getting too cold or hot
  • Know the serious symptoms to watch for and how to get medical help

Managing your SCD can be a challenge but you don’t have to do it alone. Your CVS Specialty® CareTeam is here to help. Call your CareTeam at the phone number listed on your prescription label.

This information is not a substitute for medical advice or treatment. Talk to your doctor or health care provider about your medical condition and prior to starting any new treatment. CVS Specialty assumes no liability whatsoever for the information provided or for any diagnosis or treatment made as a result, nor is it responsible for the reliability of the content.

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