Q: My 28-year-old daughter is G6PD positive, due to which her Hb is on the lower side sometimes dropping to 7+ and her serum bilirubin is on the higher side sometimes rising to around 2. As a result, her food intake has reduced. We are trying our best to arrange for vitamins and reasonable food having higher iron, etc. At the moment, she is pregnant and due to low Hb, a lot of complications are coming forth. In her last pregnancy, perhaps due to low Hb, her child could not develop properly, and had to be aborted in the seventh month. Should she go for blood infusion or can this be rectified by proper food and medicine?

A:Glucose-6-phosphate dehydrogenase (G-6-PD) is an enzyme present in our red blood cells (RBCs), which protects it from oxidative stress caused by the production of oxidative waste products within it. When such individuals are challenged by this stress (drugs or diet or infection), the RBCs rapidly break down (haemolyse) resulting in anaemia and jaundice. The jaundice is the result of production of bilirubin, which is the normal end product of haemoglobin metabolism. The deficiency of this enzyme is quite common as this genetic disorder protects the individual from severe malaria. Different gene mutations cause different levels of enzyme deficiency and the disease is classified into several groups depending on the degree of deficiency and the clinical severity of the condition. Most treatment is aimed toward avoidance of oxidative stressors. Rarely, the anaemia may be severe enough to warrant a blood transfusion. Supplements in the form of folic acid and iron are potentially useful even though the haemolysis is usually short-lived. G-6-PD deficiency is associated with poor pregnancy outcome as women have an increased risk of spontaneous abortion, low birth-weight babies and jaundice in the newborn. Infection is the usual cause of an acute episode so preventing it is important. Encourage her to take a diet rich in iron and folic acid (lean meat, grains, lentils, green leafy vegetables, tomato, potato, green and red chilies, fruits, nuts and seeds). She would require frequent monitoring, so ideally go to a hospital with experienced staff and good neonatal facilities for the newborn. Keep a close contact with the doctor and be guided by his / her opinion on the medicines to be taken and those to be avoided.