Balance You Must Find - Glut 1 Yoda style

If you saw Max walking down the street in his favorite new hat you would never guess the complex and delicate balance that lies beneath it knitted cuteness.   I continually marvel at the amount of medical experts, medications and fat products that make my son's successful daily life possible.  I am also continually thankful to live in an age of modern medicine.  Even though the use of the Ketogenic diet has been around since ancient times, it is only in recent years that it has been perfected to treat a variety of conditions, one of which is Max's syndrome, Glut 1 DS.  

As much as I would like to think we have found the perfect balance for Max's health,  I have once again been reminded that it is and most likely will always be a work in progress.  Last week at a routine visit to Children's Hospital, for a diet check up and blood work, we learned that Max's ketones levels (Beta-Hydroxybutyrate) were quite low, 2.45 when the goal is around 5.5.  They were the lowest they had ever been, but surprisingly he was not showing any signs that would indicate this low level.  Since ketones are the primary fuel for Max's brain, one would have expected to see adverse effects at this level.  However, he seemed like his normal self.   I assume this means his levels had not been low for a long period. 

The neurology nurse and dietitian both suspected his beloved Stevita powdered drink (a stevia based Crystal Lite type drink) was the culprit.  I had my doubts since he had been drinking it before his last set of labs, which were normal, but I have learned to listen to people who know more about these things than I do.  Max has an extremely limited diet and he is quite attached to the few foods and drinks he is allowed to consume.  I hated to take away something he loved and I figured it would not go over well with him either.  However my sweet boy never ceases to amaze me with his adaptive nature.  I presented him with Aquafina water bottles, like the ones he sometimes would select at the Sam's club food counter.  For some reason he feels water just tastes better when it comes from Aquafina.  I bought six bottles and I keep refilling them, the power of the label is enough for him and he accepts the water, regardless of the source, as long as it is in the Aquafina bottle.

Within 4 days of not drinking Stevita and not eating his gummy candy made with the same powder, Max's blood ketone level was a 7.7.  Wow, 7.7!!!  He has never been that high and well...that is actually too high and now his neurology nurse is concerned he might become acidotic, so we are having to add to his daily regime of pills and powders to bring that under control since high ketones have now pushed his bicarb levels too low.  Up and down, these levels are dizzying.

After two and half years I would have thought we would have found the stable midpoint, but alas we have not.  As long as food and drink manufactures change their formulas we will always be looking for the right combination.  Along with the suspected drink we also just learned that his vitamins have added a carb per serving and so has the brand of ham he enjoys.  I joked with Caroline that she needed to become a doctor and then she could do Max's blood work at home.  That way we could stay on top of things more.  She didn't think that was funny and she said she was willing to watch, but not preform blood work.  A far cry from the little 5 1/2 year old who used to tell us it was okay, she could test her brother's urine for us, with the keto strips griped in her tiny little hand.  I guess she has moved through her medical phase....I think her current focus is on fairies and poetry, as it should be I suppose.

Balance, balance must we find....

We have been instructed to keep a food journal and keep him off the Stevita drink, as well as add a teaspoon of baking soda twice a day to keep his bicarb levels stable.  Retesting of his blood in two weeks.  

Not too high, not too low...Good levels I hope to find...