When we first learned how rigid this diet can be we immediately came to the conclusion that we would have to hide in our house, stop going out to eat, limit our travel, and protect Drake from being exposed to a world full of off-diet choices. I despaired that Drake would be a food jealous, food unconfident, limited in his life experiences, sad little kid. Maybe I was a little bit overdramatic at first.
So after a few months we were happy to find out that we could still take Drake out to eat again if we were careful and managed expectations. We no longer went to our favorite kid-friendly restaurants, a Mediterranean Bistro and a Mexican Restaurant, but instead favored places with a really good hamburger. You can get a burger at a grill with no bun and it is generally pretty safe and only cooked in oil (but ask your sever if you have any concerns about hidden carbs). We stress to the server that Drake can’t take a bite without two sides of mayonnaise, and that usually does the trick for bringing the all important mayo with the meal. Continue reading “Out to eat as a family”
Glut1 kid, Drake Lane, thrives on the high-fat, low-carb, low-protein Ketogenic diet. But how strict should a six year old kid who does not have seizures be?
To some degree the Glut1 Deficiency kids who don’t have epilepsy or seizures are the kids who you can experiment on; who can be the guinea pigs for doing things with less rigidity and more flexibility. We keep asking ourselves, how strict should we be with a recovered wobbly little kid who does not have seizures, who will not loose seizure control, and who will not have breakthrough seizures. After an initial period of adjustment we decided to go off script a bit. Rationalization is a powerful thing.
We do this diet within a community — within the dietitian’s guidelines, but then we understandingly find ourselves completely on our own, customized to Drake, tweaked to what works for him and for our family. We find ourselves making our own roadmap of how strict we need to be. For example, having Drake eat more often and not at regimented times, helps him maintain his energy level and his mood. This diet is supposed to be a natural appetite suppressant, but no amount of advice from medical experts or online message boards ultimately deals with the stuff we have to live through. Continue reading “Rationalization is a powerful thing”
We’ve gotten into such a good groove lately with the Keto Diet that I have to remind myself just how much of a balancing act it is for Drake to stay in ketosis. Every day that passes has thankfully, so far, been another ataxia-free day and another day of his brain growing and flourishing. The daily routine of eating keto meals and taking various vitamins has enabled Drake to be our awesome, fun-loving, lego-building, bike-riding, TaeKwonDo-kicking crazy-cool kid. It is also a reminder of how much of a balancing act it is for him to stay on the knife-edge of ketosis.
It is a fine line he has to walk. On one hand he has to eat enough fat to obtain and stay in steady ketosis. But if he eats too much fat he is in danger of ketoacidosis; which if left unchecked and becomes severe, requires hospitalization and can be life threatening. Then, on the other hand if Drake eats too many carbs and sugar, or crazily eats too much protein (which the body can turn into glucose) or too many calories, which can interfere with ketosis, he can get low-blood-sugar (Hypoglycemia) and become shaky and weak or get knocked out of ketosis altogether. Continue reading “It’s a Fine Line”
The Charlie Foundation had its yearly symposium in Banff, Canada a few weeks ago and it reminded me just how much everyone on the Ketogenic diet owes this foundation for making this fringe form of treatment for epilepsy visible and available again. We want to personally thank The Charlie Foundation for opening the floodgates and raising awareness of the Ketogenic Diet as a treatment for not just epilepsy, but also for Glut1 DS and other metabolic and genetic disorders
Like Charlie Abrahams, my son Drake will be on the diet long term. He won’t be able to wean off of it after two or three years like the majority of epilepsy kids. This is it. Self denial will have to be a permanent facet of his personality. But we feel lucky that we are doing this “special diet” in 2016, twenty-two years after this Dateline Special.
Three days after we got home from the hospital we went rebel.
I was extremely frustrated with the twenty recipes I had been given — three of which contained sugar-free jello, which is sweetened by aspartame instead of sugar, a no-no in my book (because of medical controversies associated with it). Our dietician wouldn’t give us a username and password to ketodietcalculator.org until Monday (a wonderful website that enables parents to calculate meals for their kids based on their ratio and calorie count), which was one day away, and we were entering food war territory over the amount of heavy cream Drake had to drink with every meal. The only recipe I had in which the heavy cream was cooked in, was the eggs, butter and cream recipe, and I desperately needed more like that — in which the fat is “baked in the cake” so to speak.
Drake was so stressed out from the hospital stay and the gross food combinations we were trying to get him to eat that he wouldn’t even pick up a fork and feed himself. We set up the iPad for every meal and let him watch videos while we fed him, not a sustainable scenario at all! The first time I made the Breakfast Cookie recipe I was terrified about doing so because it was at a higher ratio, a 4:1 ratio rather than Drake’s 3:1 ratio (which I could have easily remedied in the ketodietcalculator).
Glut1 Deficiency is a rare neurometabolic disease in which the protein responsible for moving glucose across the blood-brain barrier and into the brain is mutated or missing — causing a myriad of symptoms that range widely in severity among patients. The Ketogenic diet controls these symptoms by focusing on what the primary issue of Glut1 Deficiency is — an energy failure in the brain.
The Ketogenic diet is a high fat, low protein, low carb diet that causes the body to produce and burn ketones for fuel instead of glucose. Starting the diet as soon as possible is imperative so children’s brains can develop and grow and no longer be starved for energy or be permanently affected and disabled by Glut1 Deficiency. But because of the lack of awareness within the medical community many children are not being correctly diagnosed or diagnosed at all. Plus, right now 90% of Glut1 patients have seizures, so the other 10% who do not, who have the movement disorder type of Glut1 like Drake, are not even on the radar for having it. That is why I’ve started this blog —to raise awareness for this rare-disease that is also very rare in having a treatment option that works for most kids. If your kid is diagnosed with Glut1 Deficiency they will need to start the Ketogenic diet in the hospital and then continue to be medically supervised at home and do hospital/lab visits every few months.
Following a diagnostic pathway for a rare spectrum-disease can be a muddled and troublesome journey. Finding the right diagnosis can be confusing and many times involves your child being misdiagnosed or undiagnosed. When your child’s symptoms don’t quite fit the mold of a rare disease, especially one that varies in its severity, it is not uncommon to wonder if the textbook symptoms need to be elaborated upon. Or perhaps your child actually has a different diagnosis altogether. Continue reading “Glut1 Deficiency Diagnosis”
This blog is dedicated to raising awareness about the movement disorder type of Glut1 (also known as, non-epileptic Glucose Transporter Type 1 Deficiency Syndrome) and the only known treatment, the ketogenic diet.
Outside my window it’s a beautiful fall day, and I’m feeling pretty inspired that I can finally write down my thoughts about Glut1 Deficiency Syndrome (Glut1 that presents with Ataxia) while my boys are both in school. Chase has just started Pre-K. And Drake, my 7-year-old Glut1 son, is tracked back in until mid-November (yay!!! the joy of year round school) and doing very well in 1st grade.