We got back from Hopkins late Wednesday night. Overall it was a good trip and a good meeting with her doctor and dietitian. We all pretty much agree that there is something up with her metabolism making it difficult to get the diet to work. They made 2 changes to her diet. The first change is adding MCT oil to her meal. Here is info to describe it.....

MCT stands for medium chain triglycerides. Most naturally occurring fats are triglycerides - tri meaning that each molecule have three carbon chains. In the medium chain triglycerides each chain has 6 - 12 carbon atoms, and for the medically refined grades of MCT oil each chain has 8 - 10 carbon atoms. MCT oils occur naturally, and the most abundant source is coconut oil. Most MCT oil is refined from coconut oil.

MCT oil is interesting because, when it is metabolised in the body, it behaves rather more like a carbohydrate than a fat. The fuel of preference for the body is carbohydrate, and the body will use up its store of carbohydrate before using other fuels.

Long chain fats (ie the normal varieties) are converted into chemicals called chylomicrons by the digestive system, and these are then transported around the body by the lymphatic system before entering the circulatory system. This is a relatively slow process, and so fats metabolise more slowly than carbohydrates. Unlike other fats, MCT oil does not go into the lymphatic system; instead it is transported directly to the liver where it is metabolised, so releasing energy quickly, just like a carbohydrate, and creating lots of ketones in the process.

The oil has to be introduces slowly to avoid the side effects so we have added 5g to 4 of her meals and in 2 weeks we will increase to 10g per meal.

The reason the added the oil was to boost her ketones especially to try to help with her ketones dropping during the night. Well after the first day of adding the oil her ketones went pretty high but it has done something to effect her seizures. She went on to have 8 Grandmal seizures over the next day and half (It was a great birthday for her yesterday!). So I don't know if her body has to get use to it or if she can't handle it? We need to give it 2 weeks to see.

The second change was to add carnitine. Some of her blood test showed she was carnitine deficient. Since I barely understand all this here is more info to explain...

Carnitine is a protein that is essential to the metabolisation of fat. Long chain fats are metabolised by the mitochondria within the cell. Carnitine is essential to this process, because fat by itself cannot penetrate the membrane of the mitochondria. Each molecule of fat has to be transported across the mitochondrial membrane by binding with a molecule of carnitine. After the fat has been metabolised in the mitochondria, and generated the energy rich ATP, the carnitine is again required to transport the waste product out of the mitochondria as an acyl-carnitine.

Since the ketogenic diet has reduced protein and increased fat this may be the cause of her deficiency but we are wondering if she had this deficiency before she started the diet and it is just worse now?

It has taken a lot of work to locate the sugar free carnitine and thanks to Mike at Edgemont Pharmacy he had it overnight shipped to us last night.

So we are waiting to see if these 2 things work. If they don't her doctors are going to try a
24 hour fast every few days to a week. This would be an experiment to see if our theory of her body "forgetting" how to use her own fat until we shock it back by fasting her would work. We are really hoping our first option will work, it is not very fun fasting her, especially that often. But obviously we will do anything to try to control these seizures! Thanks again for all the prayers and support!