Medicines for G6PD deficiency

What it is

G6PD deficiency is a common inherited condition in which the red blood cells lack enough of an enzyme (glucose-6-phosphate dehydrogenase) that normally protects them from certain kinds of stress. Most people with it are perfectly healthy and have no symptoms most of the time. However, exposure to particular triggers can cause the red blood cells to break down (haemolysis), leading to a sudden anaemia with tiredness, pallor, a fast heartbeat, dark urine, and sometimes yellowing of the skin and eyes (jaundice). Triggers include certain medicines (such as some antimalarials, some antibiotics, and others), fava (broad) beans, and infections. It affects far more males than females because of how it is inherited, and is more common in people with family origins in Africa, the Mediterranean, the Middle East and Asia. It can also cause prolonged jaundice in newborn babies. It is diagnosed with a blood test.

How it is treated

The mainstay of management is avoiding the known triggers, which allows most people with G6PD deficiency to live completely normal, healthy lives. People are given a list of medicines and substances to avoid (including certain drugs and fava beans), and it is important to tell doctors, pharmacists and dentists about the condition so that safe medicines are chosen. Most episodes of red cell breakdown are triggered by something identifiable and settle once the trigger is removed and the body makes new red cells, though a severe episode occasionally needs treatment such as a blood transfusion. Infections can also trigger episodes, so these are treated. In newborns, jaundice is monitored and treated as needed. Carrying information about the condition (for example a card) helps ensure safe care. The reassuring message is that, with trigger avoidance, G6PD deficiency usually causes no problems.