Sickle Cell Disease

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Health Maintenance for People with Sickle Cell Disease

What your healthcare providers can do to help you be healthy.

Optimize your hydroxyurea dose

  • We periodically monitor your blood counts to determine if the dosage is safe. If your blood counts (especially neutrophils and platelets) get too low, we’ll know we’ve increased your dose too much and will reduce it accordingly.
  • The best dose will be the one that decreases your blood counts a lot but not too much.
  • For 20 years, hydroxyurea has shown in research studies to decrease pain crises and hospitalizations for people who take optimized doses.
  • Taking your hydroxyurea and folate every day as prescribed is something you can do to help maintain your health.

Make sure your vaccines are up to date

  • In a person with sickle cell disease, the spleen is frequently removed or nonfunctional. This puts you at an increased risk for infection.
  • To help protect you from infection, we strongly recommend the following:
    •  Yearly flu shot
    • Pneumovax (to help prevent a type of pneumonia)—2 doses, 5 years apart
    • Meningococcal vaccine (to help prevent a type of meningitis)—2 doses, 2 months apart, followed by boosters every 5 years
    • H. influenza vaccine (to help prevent a type of meningitis)—1 dose
    • Tetanus boosters every 10 years
    • Hepatitis B vaccine series—3 doses
  • In certain situations, we may recommend additional vaccines.

Screen periodically for sickle cell disease complications

  • The effects of sickle cell can accumulate in your body before you might feel different. We can check for things that could turn into big problems, and then help prevent further damage.
  • Special screening for people with sickle cell disease includes:
    • Eye exams every year to check for sickle cell retinopathy
    • Lung function tests tell us if your body is getting enough oxygen; if it’s not, you could get oxygen to use a home.
      • A sleep study may be indicated in some people. If you have a condition called sleep apnea, your body doesn’t get enough oxygen at night, which can make sickling worse, and there’s a special device that can help you get more oxygen while you sleep.
    • Echocardiogram (an ultrasound of your heart) will tell us how your heart is functioning and if you are developing pulmonary artery hypertension, a serious complication of sickle cell disease. If we get an echo while you’re healthy, we’ll be able to compare it to future studies when you’re not feeling as well.
    • Urinalysis to check for protein in your urine. This helps us know if your kidneys might be injured.
    • Ferritin – this is a blood test that indicates an elevated level of iron in your body. If you’ve had more than 10 transfusions in your life, you are at risk for iron overload, which can be harmful to your liver.
      • If your ferritin is elevated or you have known history of iron overload, you’ll need to get periodic MRIs. The T2* MRI will show how much iron is in your liver and indicate if you would benefit from chelation.

Routine wellness care recommended for all adults