World Sickle Cell Day

Sickle cell disease is an inherited red blood cell disorder that affects roughly 100,000 Americans according to the Centers for Disease Control and Prevention (CDC). The Mayo Clinic describes Sickle Cell anemia as – “a condition in which there aren’t enough healthy red blood cells to carry adequate oxygen throughout your body.”

Normally, your red blood cells are flexible and round, moving easily through your blood vessels. In sickle cell anemia, the red blood cells become rigid and sticky and are shaped like sickles or crescent moons. These irregularly shaped cells can get stuck in small blood vessels, which can slow or block blood flow and oxygen to parts of the body.

Today is World Sickle Cell Day and it is intended to raise awareness about this life-threatening disease. wrote, “Although sickle cell disease can affect people of all races, it is most common among people of African, Hispanic, Mediterranean and Native American descent. About 1 in every 365 African-American children is born with sickle cell disease.”‘s online headline today read, “New Drugs Promise Hope This World Sickle Cell Day.” The excerpts below are from that story.

There is some encouraging news with, for the first time in decades, progress regarding novel treatments. Much of this is driven by better understanding of molecular biology and physiology, which in turn is resulting in identifying new targets for drugs.

The first new drug in decades to be approved for treating sickle cell disease is L-glutamine, marketed as Endari by Emmaus Life Sciences. This amino acid is taken by mouth twice daily. Small clinical trials showed a modest reduction in pain (median, 3 vs. 4) and hospitalizations (median, 2 vs. 3), 4.5 day shorter hospital stays, and a greater reduction in acute chest syndrome (9% vs. 23%).

One of the biggest issues facing people who live with sickle cell is finding ways to deal with the pain. The National Heart, Lung and Blood Institute recently pointed out that the opioid crisis is making it even more difficult for those fighting sickle cell disease.

The increasingly tight restrictions on opioid access, they say, is stirring fears that patients will face greater scrutiny than they already do when seeking relief—or worse, be forced to suffer longer from the excruciating pain the disease is known to cause.

Researchers supported by the National Heart, Lung, Blood Institute (NHLBI) say this tension—between a desire to slow a troubling drug epidemic and a desire to expedite and effectively treat sickle cell patients’ pain crises—has spurred an even greater focus on pain and sickle cell disease. Some researchers are looking more closely at how opioids are being used among sickle cell patients, while others are exploring treatment alternatives, including non-opioid painkillers and non-drug alternatives for pain, such as meditation and acupuncture.

Unfortunately, pain is not the only complication sickle cell patients have to deal with. Others include:

  • Stroke
  • Acute chest syndrome
  • Pulmonary hypertension
  • Organ damage
  • Blindness
  • Leg ulcers
  • Gallstones
  • Priapism

World Sickle Cell Day gives us a chance to bring attention to the health issues people with sickle cell face every day. And maybe that attention can one day drive new therapies and new hope, which can in turn help us take another step in our long-term goal of making Arizona the Healthiest State in the Nation!

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