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Sickle Cell Anemia

What is sickle cell anemia?

Sickle cell anemia is a disease that causes your body to break down too many red blood cells (RBCs). RBCs carry oxygen to all the organs and tissues. Sickle cell anemia is a serious form of sickle cell disease. The disease causes RBCs to be sickle (crescent) shaped. Your risk increases if both your parents have the gene for sickle cell anemia. Your healthcare provider can confirm you have sickle cell anemia from the shape of your RBCs.

What are the signs and symptoms of sickle cell anemia?

  • Fatigue

  • Pale skin

  • Frequent headaches

  • Shortness of breath

  • Pain throughout your body

How is sickle cell anemia managed?

You may need ongoing screening for conditions that can develop because of sickle cell disease. Examples include kidney disease, hypertension (high blood pressure), retinopathy (eye problems), and problems with your lungs.

  • Medicines may be given to decrease pain or to decrease sickling of your RBCs. You may also need medicine to treat or prevent a bacterial infection.

  • NSAIDs , such as ibuprofen, help decrease swelling, pain, and fever. This medicine is available with or without a doctor's order. NSAIDs can cause stomach bleeding or kidney problems in certain people. If you take blood thinner medicine, always ask your healthcare provider if NSAIDs are safe for you. Always read the medicine label and follow directions.

  • Acetaminophen decreases pain and fever. It is available without a doctor's order. Ask how much to take and how often to take it. Follow directions. Acetaminophen can cause liver damage if not taken correctly.

  • A blood transfusion replaces blood with RBCs that are not sickle shaped.

What can I do to prevent a sickle cell crisis?

  • Take vitamins and minerals as directed. Folic acid can help prevent blood vessel problems that can occur with sickle cell anemia. Zinc may decrease how often you have pain.

  • Drink liquids as directed. Dehydration can increase your risk for a sickle cell crisis. Ask how much liquid to drink each day and which liquids are best for you.

  • Balance rest and exercise. Rest during a sickle cell crisis. Over time, increase your activity to a moderate amount. Exercise regularly. Avoid exercise or activities that can cause injury, such as football. Ask about the best exercise plan for you.

  • Stay out of the cold. Do not go quickly from a warm place to a cold place. Do not go swimming in cold water. Stay warm in the winter.

  • Do not smoke cigarettes or drink alcohol. These increase your risk for a sickle cell crisis. Smoking can also delay healing after treatment. Ask your healthcare provider for information if you currently smoke and need help quitting.

  • Ask about which vaccinations you need. Vaccinations can help prevent a viral infection that may lead to a sickle cell crisis. Get a flu shot every year as directed. You may need a pneumonia vaccine.

What do I need to know about family planning for women with sickle cell anemia?

Talk to your healthcare provider if you plan to have children. Pregnancy in a woman who has sickle cell anemia will be a high-risk pregnancy. Your healthcare provider will want to create a safe pregnancy plan. If you do not want to become pregnant, your healthcare provider may recommend birth control pills that contain only progestin. The pills will prevent pregnancy and make your periods lighter. Lighter periods may help increase your hemoglobin levels.

Call 911 for any of the following:

  • You have a new cough, shortness of breath, and chest pain.

  • You have an erection that is painful and does not go away.

  • You lose vision in one or both eyes.

  • You are short of breath, even when you rest.

When should I seek immediate care?

  • You have a fever.

  • You have a new or different headache.

  • You have new pain in any part of your body.

  • You feel like you can no longer cope with your pain, or feel like harming yourself.

  • You cannot think clearly or feel like you are going to faint.

  • You have abdominal pain, nausea, and vomiting.

  • You have new weakness or numbness in your arm, leg, or face.

  • Your urine is dark, or you are urinating less than usual or not at all.

When should I contact my healthcare provider?

  • You are more tired than usual during the day.

  • You see blood in your urine.

  • You are constipated or have diarrhea.

  • Your eyesight has changed in one or both eyes.

  • You have questions or concerns about your condition or care.

Care Agreement

You have the right to help plan your care. Learn about your health condition and how it may be treated. Discuss treatment options with your caregivers to decide what care you want to receive. You always have the right to refuse treatment. The above information is an educational aid only. It is not intended as medical advice for individual conditions or treatments. Talk to your doctor, nurse or pharmacist before following any medical regimen to see if it is safe and effective for you.

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