A Basic Overview of Why Nutrition Matters: Even With ERT

            We need to understand a few basic things. Pompe falls into many disease classifications the most common being that it is a Lysosomal Storage Disease (LSD) and a Glycogen Storage Disease (GSD). I will be focusing on the glycogen portion of the disease today. The body makes glycogen when your blood sugar goes above 100 (typically). Your blood sugar can rise for several reasons, when you wake up in the morning it rises, when you are sick it rises, adrenaline will make it rise and some others but the most common way we raise our blood sugar and keep it elevated is what we choose to eat. Our food intake has a bigger influence on our glucose levels than all other ways combined.

            Now that we know those things let’s talk about enzyme replacement therapy (ERT). ERT gives you the enzyme that your body lacks to break down glycogen and is a critical part of the fight if you have Pompe. You will never hear anyone at Pompe Warrior Foundation say anything different. ERT also has its limitations in its effectiveness. It in fact can gradually stop working all together for a lot of people the longer they are on ERT. Many patients also experience “ERT fatigue”. Which means if you get your infusion every Friday you will feel the best Friday and Saturday, slowly feel worse the farther and farther away you get from the day of infusion.

So why does nutrition matter? We know that what we eat has the biggest influence on the production of glycogen; we know that ERT is a great tool in the fight but has limitations, so let’s put these two things together.

            You get your infusion on Fridays and by the next Thursday you are feeling crummy again, why?? Let’s say ERT can get rid of 10 glycogen molecules a week (hypothetical) but because of your diet your body produces 15 glycogen molecules a week. This means after ERT comes in and does its thing you still have a remaining 5 glycogen molecules. You do this week after week and soon the 5 remaining molecules have compounded into 50 and ERT starts to stop working because it can’t keep up!! Simple argument....right?          

Think of Pompe as a car driving around your body crashing into everything. Eating a lower glycolic diet will not change the fact that you have a car (Pompe) inside your body but it will take the fuel (glycogen) away, leaving it parked and in a world where we still don’t have a cure, leaving your disease in park is a win and that is just one reason why nutrition matters.