Q: My son is suffering from G-6-PD deficiency and has been advised by the doctor to avoid certain drugs which would lead to hemolysis. Can you explain the importance of G-6-PD and the drugs to be avoided by people with such ailments?
A: G-6-PD or glucose-6-phosphate dehydrogenase deficiency is a relatively common abnormality affecting more than 100 million males throughout the world. This disease is carried as a sex-linked recessive and therefore all males receiving the gene have the disease. Glucose-6-phosphate dehydrogenase is an enzyme required for the stability of red blood cells.
Without this enzyme, the blood cell membrane tends to break up easily during the metabolism of certain drugs. In a typical case of red blood cell lysis, the onset of symptoms may occur from minutes or hours after consuming the drug.
These symptoms may start with dizziness, chills, fever and abdominal pain. In severe cases of hemolysis, the presence of blood in the urine may appear, followed by jaundice.