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When cells rebel: Managing sickle cell disease

Sickle cell anemia is one of the world’s most common genetic diseases, affecting millions of people. The disorder causes red blood cells to assume a “sickle” shape instead of the normal disc shape. The misshapen cells block blood vessels and deprive tissues of oxygen, resulting in severe bone pain and over time organ failure.

“This disease has devastating consequences,” says Hedy Smith, MD, PhD, Director of the Tufts Medical Center Benign Hematology Program. “We help patients manage and cope with the disease symptoms and its psychological and social aspects. We often help them avoid hospital stays by treating them in our outpatient infusion center.” 

Here are five tips from Dr. Smith to help you or someone you love better manage sickle cell disease (SCD):

1: Establish a medical home

Management of SCD requires consistent and comprehensive care. Your medical home should have dedicated physicians, nurses and nurse practitioners, pain specialists, social workers and psychologists.

2: Avoid known triggers

Stress, poor diet, extremes of temperatures, infections and dehydration are common triggers of pain flares and complications. Tobacco use can lead to acute chest syndrome, a lung complication that is the leading cause of death in patients with SCD. Illicit drugs use will lead to exacerbations of pain.

3: Know when to seek medical attention

Call your doctor if you experience excruciating pain, fevers, shortness of breath or chest pain, which are signs of acute chest syndrome or pulmonary embolism. Sustained erections are common and can lead to sexual dysfunction.  Burning when urinating may be a sign of urinary tract or kidney infection. Make sure you’re up to date on flu and pneumonia vaccines.

4: Plan your pregnancy carefully 

Before you start a family, meet with a genetic counselor to understand risk of transmission to your baby and to get your partner tested. Care by a high-risk obstetrician is also vital for pregnant sickle cell patients.

5: Talk to someone about depression

Insomnia, feelings of isolation, fear of fatal complications and unpredictable pain flares are very disruptive in a patient’s life. It’s important to talk to someone about how you are feeling; whether it’s your physician or a psychologist.

Learn more about the Benign Hematology Program which supports our SCD patients, including our new patient support group.