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Early detection eases sickle cell disease

The U.S. Food and Drug Administration has approved Novartis Adakveo^® (crizanlizumab), a new medication reported to significantly reduce painful sickle cell disease flare-ups.

Normal red blood cells contain hemoglobin A, are smooth, round and flexible, and pass easily through blood vessels to deliver oxygen to cells throughout the body. Sickle cell disease is a genetic blood disorder that causes red blood cells to contain mostly hemoglobin S, an abnormal form of hemoglobin. During a sickle crisis, red blood cells with hemoglobin S can become rigid and sickle-shaped and block small blood vessels. These blockages obstruct blood flow and oxygen delivery, which causes severe pain and eventually leads to tissue and organ damage. Additionally, the lifespan of sickled red blood cells is significantly shorter than normal red blood cells at 16 days vs. 120 days, leading to anemia in affected patients.

Sickle cell disease affects approximately 100,000 Americans and millions of people throughout the world.

According to the Centers for Disease Control and Prevention, sickle cell disease affects approximately 100,000 Americans and millions of people throughout the world. Symptoms of sickle cell disease vary from person to person and range from mild to severe, including fatal complications. Because the only known cure for sickle cell is a risky bone marrow or stem cell transplant, people affected by sickle cell disease often focus on health maintenance and prevention of a sickle crisis. Sickle cell disease also worsens over time, making early diagnosis critical for doctors to begin treatments that help prevent complications.

To aid in detection, Streck offers SICKLEDEX^®, the leading hemoglobin S solubility testing kit for use with human blood or sickle cell control material. The test requires 20 µL of patient or control sample and 2.0 mL of solubility buffer, and results are available in 6 minutes. SICKLEDEX kits are conveniently packaged 100-test, 50-test or 12-test kit configurations with squeeze bottles for easy dispensing and elimination of waste. SICKLEDEX provides a quick and easy way to detect the presence of sickling hemoglobins, so affected patients can begin treatment and prevent dangerous and painful complications.