How does Endari work to treat sickle cell disease?

Endari (L-glutamine oral powder) works to treat sickle cell disease by increasing the amount of the amino acid glutamine in the blood.

Endari is used to treat sickle cell disease in adult and pediatric patients 5 years of age and older.

Sickle cell disease is an inherited blood disorder in which the red blood cells are abnormally shaped in a crescent, or "sickle," shape. This restricts the flow in blood vessels and limits oxygen delivery to the body’s tissues, and can lead to severe pain and organ damage.

Sickle cell disease most often occurs in African-Americans, Latinos and other minority groups. Sickle cell disease patients in the United States have an average life expectancy of approximately 40 to 60 years.

The mechanism of action of the amino acid L-glutamine in treating sickle cell disease is not
fully understood. It is thought to increase NAD redox potential in sickle red blood cells through increasing the availability of reduced glutathione, which may lessen oxidative damage in sickle red blood cells.

Endari is supplied as an oral powder which is mixed with a liquid, such as water, milk, or apple juice, or mixed with soft food such as applesauce or yogurt. It is taken twice daily.

Common side effects of Endari include constipation, nausea, headache, abdominal pain, cough, pain in the extremities, back pain and chest pain.