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Shining a Spotlight on Sickle Cell Disease

December 20, 2021

Did you know that physicians at Texas Oncology treat more than just cancer? Many oncologists are also board-certified to practice hematology, which means that they can treat patients with blood disorders in addition to cancer patients. Since inherited blood disorders, like sickle cell disease, are diagnosed during childhood, many patients with blood disorders are treated by pediatric hematologists until they reach adulthood.

According to the Centers for Disease Control and Prevention (CDC), more than 100,000 people in the U.S. have sickle cell disease – the most common inherited blood disorder. We spoke with Joel Weinthal, M.D.,pediatric oncologist and hematologist at Texas Oncology–Medical City Dallas Pediatric Hematology– Oncology and Texas Oncology–Odessa West Texas Cancer Center to better understand sickle cell disease and what treatments are available to prevent complications and, potentially, offer a cure for the disease.

What is sickle cell disease?

Sickle cell disease is a group of inherited blood disorders that affect a patient’s red blood cells, causing them to be rigid and shaped like crescent moons or “sickles.” Normal red blood cells are disc-shaped and flexible, so that they can easily travel through blood vessels, but for sickle cell patients, red blood cells can get stuck – blocking blood flow throughout the body, causing many serious acute and chronic medical problems resulting in significant morbidity and mortality.

Who is at risk for sickle cell disease?

Approximately one out of every 500 African Americans and one out of every 36,000 Hispanic Americans, have sickle cell disease, according to data from the National Institutes of Health. Newborns in the U.S. are screened for sickle cell disease through a routine blood test that looks for hemoglobin S protein, the mutated form of the normal hemoglobin proteins that results in sickle cell disease when the abnormal gene is inherited from both parents.

What are the complications of sickle cell disease?

In the first 6 months of life, most babies with sickle cell disease don’t have sickle related symptoms because they are still expressing fetal hemoglobin, which protects red blood cells from sickling. Fetal gene expression usually disappears as children mature, so the risks of sickle cell symptoms and crisis in pediatric patients become more prevalent and can include pain, vaso-occlusive crisis, and risks of serious infection. It is imperative that sickle cell patients receive ongoing medical care throughout their childhood – with a pediatric hematologist in conjunction with their pediatrician and other specialists. Although the severity of symptoms and complications from sickle cell disease are unique to each patient, they often worsen as patients enter adulthood due to the chronic damage of many organ systems over time. In addition to being at risk for infection and acute pain crises, patients with sickle cell disease face an increased risk of heart, lung, liver, GI, eye, joint, bone, neurologic, and kidney problems. Additionally, chronic anemia and pain, stroke or silent brain injury, leg ulcers, and many other conditions contribute to the decreased quality of life experienced by patients as they get older.

We’re continuing to research sickle cell disease to enable more breakthroughs in how we care for patients and hopefully cure more patients with the disease."

How do you treat sickle cell disease?

Hematologists can best manage the disease with preventative treatments and interventions including novel medications to prevent both short- and long-term toxicity. Perhaps most exciting is the potential cure that blood and bone marrow transplants offer to some sickle cell patients. Gene therapy approaches are now being researched and studied in clinical trials, which may offer more options for curative therapy to patients in the coming years.

We’re continuing to research sickle cell disease to enable more breakthroughs in how we care for patients and hopefully cure more patients with the disease.

For upcoming webinars visit www.TexasOncologyFoundation.org.