Sickle Cell Disease: Care Instructions

Overview

Sickle cell disease turns normal, round red blood cells into abnormally curved cells that look like sickles or crescent moons. These cells can get stuck in blood vessels, blocking blood flow and causing severe pain. The sickle-shaped cells also can harm organs, muscles, and bones. It is a lifelong condition that puts you at risk for infections. Sickle cell disease is passed down in families. You can talk to your doctor about whether to have genetic tests to find out the chance of having a child with the disease. Your doctor also may recommend that your family members get tested for sickle cell disease.

Your doctor may treat you with medicines. Some people get blood transfusions or a bone marrow transplant. Managing pain and preventing infection are important parts of your treatment.

Follow-up care is a key part of your treatment and safety. Be sure to make and go to all appointments, and call your doctor if you are having problems. It's also a good idea to know your test results and keep a list of the medicines you take.

How can you care for yourself at home?

• Take your medicines exactly as prescribed. Call your doctor if you think you are having a problem with your medicine.
• Take pain medicines exactly as directed.
  • If the doctor gave you a prescription medicine for pain, take it as prescribed.
  • If you are not taking a prescription pain medicine, ask your doctor if you can take an over-the-counter medicine.
• Try to help ease pain by distracting yourself. Use guided imagery, deep breathing, and relaxation exercises. A pain specialist can teach you pain management skills.
• Avoid alcohol. It can make you dehydrated.
• Dress warmly in cold weather. The cold and windy weather can lead to severe pain.
• Don't smoke or vape. Smoking or vaping can reduce the amount of oxygen in your blood. If you need help quitting, talk to your doctor about quit programs and medicines. These can increase your chances of quitting for good.
• Get plenty of sleep.
• Get regular eye exams. Sickle cell disease can cause vision problems.
• Wear medical alert jewelry that says that you have sickle cell disease. You can buy this at most drugstores.
• Avoid infections such as COVID-19, colds, and the flu. Wash your hands often. Get a pneumococcal vaccine shot. If you have had one before, ask your doctor whether you need another dose. Get a flu shot every year. Stay up to date on your COVID-19 vaccinations.

When should you call for help?

Call 911 anytime you think you may need emergency care. For example, call if you have:

• Symptoms of a stroke. These may include:
  • Sudden numbness, tingling, weakness, or loss of movement in your face, arm, or leg, especially on only one side of your body.
  • Sudden vision changes.
  • Sudden trouble speaking.
  • Sudden confusion or trouble understanding simple statements.
  • Sudden problems with walking or balance.
  • A sudden, severe headache that is different from past headaches.
• Severe pain that can't be relieved with your usual prescription pain medicines or other pain-relief methods.
• Severe belly pain or a sudden increase in the size of your spleen. (Learn from your doctor how to feel your spleen to check its size.)
• A persistent erection of the penis (priapism) that lasts more than 2 or 3 hours or that is extremely painful.
• Symptoms of a heart attack. These may include:
  • Chest pain or pressure, or a strange feeling in the chest.
  • Sweating.
  • Shortness of breath.
  • Nausea or vomiting.
  • Pain, pressure, or a strange feeling in the back, neck, jaw, or upper belly or in one or both shoulders or arms.
  • Lightheadedness or sudden weakness.
  • A fast or irregular heartbeat.

NOTE: If you are having heart attack symptoms, the 911 operator may tell you to chew 1 adult-strength or 2 to 4 low-dose aspirin. Wait for an ambulance. Do not try to drive yourself.

Call your doctor now or seek immediate medical care if you have:

• A fever.
• An open sore (ulcer) on the leg.
• Problems with urination.

Make a management plan with your doctor that includes where and when to get treatment in case of a sickle cell emergency.

Watch closely for changes in your health, and be sure to contact your doctor if you have any problems.