Living with Sickle Cell Disease

The group of red blood cell disorders known as sickle cell disease is present at birth, and for most people it’s a lifelong disease.

A stem cell transplant can cure the disease, but that treatment isn’t possible for everybody. It requires a donor with matching bone marrow. The stem cell procedure also can cause severe side effects, including life-threatening illnesses and death.

Another potential cure for sickle cell disease, gene therapy, is in clinical trial.

“There’s great hopes with these two treatments to be able to scale them up by tweaking certain aspects of the procedure,” says Enrico Novelli, MD, a hematologist and medical director of the UPMC Adult Sickle Cell Program. “So there is a cure; however, the cure is not available to everybody.”

For most people, living with sickle cell disease requires managing symptoms and trying to prevent complications. And because the disease varies by person, it’s not a simple process.

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Living With Sickle Cell: The Medicine

All 50 United States and Washington D.C. require newborn screening for sickle cell disease, among other conditions.

If a child is confirmed to have sickle cell disease, doctors explain the condition to the families and refer them to a treatment clinic.

UPMC Children’s Hospital of Pittsburgh’s Sickle Cell Program is one of three treatment centers in Pennsylvania for children with sickle cell disease. When children reach 21 years of age, they can make the transition to UPMC’s Adult Sickle Cell Program for continued treatment.

People with sickle cell disease, or parents of a child with the condition, should follow certain medical recommendations:

  • Make regular doctor’s visits: According to the Centers for Disease Control and Prevention, checkups are recommended every 2-3 months from birth through 1 year of age for children with sickle cell. Children between the ages of 1 and 2 should have a checkup every three months. Children aged 2 and older and adults should see a doctor every year.
  • Get vaccinated: Children with sickle cell disease should follow the CDC’s vaccination schedule to prevent infection. In people with sickle cell disease, there is a special vaccination schedule for Haemophilus influenzae type b (Hib), pneumococcal vaccines, and meningococcal vaccines.
  • Prevent infections: In addition to following the vaccination schedule, people with sickle cell should take other steps to prevent infections. That includes regular hand washing and taking antibiotics such as penicillin during childhood.
  • Manage your pain: The most common symptom of sickle cell disease is severe pain, which often flares in acute episodes. FDA-approved medicines like hydroxyurea, L-glutamine and crizanlizumab, can help limit the number of pain crises. Other medications, such as ibuprofen, acetaminophen, or opioids can manage the pain when it comes.
  • Get a transfusion: Blood transfusions can help treat severe anemia or prevent complications such as strokes.
  • Test for complications: Several severe, potentially life-threatening complications are linked to sickle cell disease. Doctors can screen for some of those conditions:
    • Height and weight, to see if the child is growing normally.
    • Cognitive ability, to test the child’s development
    • Eye examinations, to guard against sickle cell-caused vision problems
    • Transcranial Doppler (TCD) ultrasound, to check for the potential risk of stroke
    • Urine tests, for kidney problems
    • Blood tests, for anemia or organ damage
    • Oxygen saturation tests, to see how much oxygen the blood is carrying
    • Transthoracic echocardiogram, to screen for high blood pressure in the lungs (pulmonary hypertension)
  • Look into clinical trials: Research of sickle cell disease, including potential cures and therapies, is ongoing. You may consider joining a clinical trial at UPMC.

Living With Sickle Cell: The Lifestyle

While medical treatments are necessary to prevent symptoms and complications associated with sickle cell disease, a healthy lifestyle also is important. People with the disease should keep a healthy diet and exercise while also avoiding situations that could trigger symptoms.

“There are a lot of lifestyle changes and lifestyle measures that can directly affect the disease significantly,” Dr. Novelli says.

  • Stay hydrated: Drink eight to 10 glasses of water per day.
  • Exercise regularly: But make sure to stay hydrated and avoid workouts that are too strenuous. People with sickle cell tend to get tired more easily.
  • Eat a healthy, balanced diet
  • Get enough rest
  • Quit smoking, or don’t start
  • Limit alcohol use
  • Avoid extreme heat or cold: This includes extreme weather conditions, but people with sickle cell disease also should avoid unheated swimming pools.
  • Fly commercial: Do not fly in aircrafts with unpressurized cabins.
  • Avoid high altitudes: High altitudes can put you at risk for splenic infarct, tissue death of the spleen – a severe complication of sickle cell disease.
  • Avoid stress: Stressful situations can cause or worsen pain crises.
  • Find a support system: “Sickle cell disease, like many chronic diseases, takes a toll on the mood and on the psyche of people living with the disease,” Dr. Novelli says. There is a higher risk of depression and other emotional issues in people with sickle cell disease. Many sickle cell disease treatment centers have support groups. The UPMC Adult Sickle Cell Program’s multidisciplinary treatment team includes social workers, psychiatrists, psychologists, and more, who can help with the emotional toll of the disease.

Even without a cure for your sickle cell disease, the right treatments and a healthy lifestyle can help you manage your condition.

“With good resources and good multidisciplinary care, people living with sickle cell disease can have productive lives and can live well into their late adulthood,” Dr. Novelli says.

About UPMC

Based in Pittsburgh, UPMC is a world-renowned health care provider and insurer. We operate 40 hospitals and more than 700 doctor’s offices and outpatient centers. Our expert physicians are among the leaders in their fields, and we are leaders in groundbreaking research and treatment breakthroughs. UPMC Presbyterian Shadyside ranks as “One of America’s Best Hospitals” and No. 1 in Pennsylvania in U.S. News & World Report’s listings.