What to eat???

Sweetie Pie's meal times don't look like most peoples'. 85% of it doesn't involve his mouth.

Previous to his diagnosis, I noticed that despite his insatiable hunger he refused any solid foods. I remember being so happy the day he turned four months because I could start feeding him rice cereal and and giving him some things in a bottle. I was looking forward to him feeling full and letting me get some sleep and anything else done for that matter. That was not to be.

Every time I tried to feed him he would gag, turn blue, scream, vomit etc. You would think I was trying to feed him poison. I would back off for a few days and try again with no luck. I had never experienced a child that didn't like food.
I began to try feeding him things that he could eat on his own, all those things that  would normally appear as a choking hazard or  not really what a 6-8 month old should be eating. It worked to a degree. He really loved apple slices and celery. I found that  I could at least get him to get it in his mouth even though he still wasn't really swallowing any of it.

Then came the diagnosis and my whole perspective on feeding him had to change. At first the focus was to maintain healthy blood sugar levels and wean him. (Breast milk like all mammal milk is Lactose based) I went on a no sugar diet to keep the sugar in my milk to a minimum*. Evan went on a Nasogastric tube (NGtube) that was connected him to a feeding pump that pumped a steady low stream of Prosobee (soy baby formula). This worked like a band aid would, getting him steady but it was really annoying and impractical.  He still was refusing any thing by mouth except nursing and bottle feeding was counter productive. He fought against it so much that it would drop his sugar levels from all the exertion and fighting.  He had no desire to eat because his belly always had something in it and he never really felt hunger.

We continued this way for about 2 months, never giving up on offering food or drink.  It became apparent that we had to use an alternative means of getting him the appropriate nutrition. In August, Sweetie Pie had a Gastrostomy. It is a procedure in which a "button" device is placed in his abdominal wall that is connected to his stomach to have food pumped or gravity fed into his stomach. It is a common procedure for many medical issues and for his case has life saving benefits beyond daily maintenance. Now we were able to move forward. He was not only able to get off the 24 hour pump but we had a way to insure that he could begin cornstarch treatments.
{Cornstarch is a key to healthy living with Glycogen Storage Disease Type 1a ( and a few other of the types). Because uncooked cornstarch breaks down very slowly in the body, when they drink it, it  acts like a slow drip of glucose. The body can use it with out have any left overs to store up. Ill discuss it more in another post}

While in the hospital we had an occupational therapist come and evaluate his eating and drinking issues. She showed us some tools to help him overcome his aversions and make eating fun. She was able to get him to drink out of a straw in about 10 minutes. We will most likely have him evaluated again soon to get him eating better through a local program.
At this time Evan receives cornstarch and Prosobee 5 times a day through his tube. This, along with  1/3 of his calories coming through his pump on continuous feed while he sleeps are his main source of food. He will snack throughout the day on a few of his favorites: cucumber slices, popcorn, sunflower seeds, pretzels. None of this ever amounts to more than a  tablespoon at a time though. He will not eat anything by spoon or fork and if it feels weird to him he won't even put it in his mouth. (He finds the dog and gives it to him. HAHAHA)
I have since learned that food aversion is very common in kids with disease and many of them take years to eat normally. It is kind of ironic because food is their medicine. Everyday I see a little more progress, a little more desire and it gives me hope that he will eventually eat.

*While I did lose some weight :) the dietitian and geneticist differed on whether this was helpful or made a difference. I personally could tell in his bowel movements and demeanor when I ate too much sugar. I still breast feed a night and nap time and do not monitor my sugar intake anymore. He has shown marked improvement since making these adjustments.

Sugar Free, almost.

I've always had sympathy for mom's with children or people with food allergies or intolerance. I have lots of friends and family that deal with these issues. Maybe that empathy developed as a way to prepare me for what we were to undertake.
Evan's body cannot process sugar the same way the rest of do. Whatever unused sugars that are in his blood stream get stored up in his liver and cannot be released. This causes him to have liver damage, hypoglycemia and other metabolic complications. The best way to counteract these problems is to eliminate sugar from his diet. It sounds simple enough until you consider that 'sugar'
means more than just the white sweet powder that we add to our cereal sometimes.

Sugar comes in many forms. Sucrose, Fructose, Lactose, Glucose, Galactose, sugar alcohols, and so forth. Most forms of sugar are indigestible for Evan a few are safe.

Now I am a label reader. There are forms of sugar in everything, even things that say sugar-free have things he still can't have. It can be very confusing. Take cheese for an example. The label says 0g of sugar yet I know that lactose is in cheese and lactose is a sugar unless it is an aged cheese at which point the lactose has broken down into lactic acid. Whew- I never imagined I would ever need to know that information, I just liked eating my cheese.

There are a many items that break down differently once they enter the body so if I have a question about it I scour the Internet to see if I can find out what it breaks down into in the body.
We need 'sugars' to survive. Fortunately, simple sugars (found in complex carbohydrates) digest slowly and are safe for Evan to consume.

So here is the breakdown: Find foods that can provide usable sugars, avoid unusable ones. If it is necessary to consume sugar to provide a variety or nutritional benefit it is to be kept to less than 2.5g an meal.

Sugars to avoid: Fructose, Lactose, Sucrose, High Fructose Corn Syrup, Honey, Sorbitol
Sugars that are 'safe': Glucose, Dextrose, Dextrins, sugar alcohols, Splenda, Nutrasweet, Stevia

We have found that even small quantities of some of the safe sugars can cause Evan's blood sugar to sky rocket and then bottom out. It is a game of finding that right balance. Something I never thought I'd have to worry about or understand but knowledge is power and in this case a life saver.

New Adventure

This is the first post! Welcome to our little adventure with this little known disease. We are still a work in progress and learning daily, please pardon our dust! :)