I was waiting...!

Well, it finally happened. The moment I had dreaded. There always has to be a first, right?

Yesterday, after church, I was changing Evan and getting him ready for a nap. Evan was standing next to me, naked and I saw something dribble on the ground out of the corner of my eye. I thought he had spit some food out of his mouth. When I looked up, I saw more food goo on his belly and much to my surprise, his Gtube button dangling from medical tape!!!

I'm not exactly sure what happen but I'm assuming he pulled on it a little too hard. All at once I was in a slight panic. It is very strange to see a hole in your child's gut, even if it is only about a centimeter wide.

I sent one child to call their Dad, he was still at church. One child to get the emergency kit. One child to hold his arms down, the other the legs. Then began the replay in my mind of all the information they give you in the hospital for situations just like this. You see, the 'button' is held in place by a small water balloon that holds about 1tsp of water. Over time balloon can deflate (which is what was the case for us) and it is flexible so depending on the activity level of your child or even the curiosity of other children, they are bound to come out.

The nurses try to prepare you to handle the situation. We watched videos, had someone come and give us a more hands on training, etc. But it is a lot of information, and many times I was flabbergasted thinking I didn't have to have any medical training to do the things they are telling me needs to be done.
We keep an emergency kit with us at all times. Time is of the essence. Because the stomach is a muscle, the hole can close in there within 10 minutes.

I was not able to get the tube back in. I tried and tried and it seemed just ever so slightly too big. I really wasn't wanting to go to the ER. Of course, as life would have it, in the middle of this chaos his alarm for his cornstarch treatment sounded- queue more panic!

In the emergency kit there are some catheters of varying size that are used to hold the hole open while we seek medical attention in the event the button can't be replaced. I decided that like or not this is what I'm going to have to resort to.

Luckily, my knight in shining armor came home. He was little less affected by the drama and was able to get the button in with a little more patience and pressure than I was. Crisis averted. I fumbled through refilling the balloon, and got him taped back up, with extra tape of course. And then prayed we'd done it right.

All I can say now is WHEW! I never want to do that again but I know this wont be the last time. This experience will make it a lot easier for the times when I have to change it even just for  wear and tear.

I remember when I first learned that my sweetie pie had to avoid sugar that I was heart broken that he wouldn't be able to have a cake for his first birthday. It became so important for me to find a way to give him that same experience of smashing a cake and having fun making whatever mess he could. I also wanted to find a way that in later years he wouldn't notice the difference of everyone else getting fun cakes.
Here is what I came up with:

I bought a sugar free Jell-o cup. (it had to be Jell-o, the other sugar free brand was still made with fruit juice- hidden sugar!!!) Lined it with a cupcake liner. I topped it with sugar free/ lactose free Cool Whip. Sprinkles. And voila! my own impostor cupcake, mess and all!

I'm guessing he loved it!

I was excited a few weeks ago to find a sugar free cake mix by Pillsbury. I'm planning on making Evan some cookies with it for Christmas. I'm hoping it works out. I find that sugar free things don't always turn out the same way. It will be fabulous though to have a real birthday cake for him next year and another easy way to prepare a treat for him when it is needed. Pillsbury also has a sugar free frosting as well but it still contained Sorbitol which is on his "can't-have" list.

I'll let ya know how they taste!

An Emotional Outburst

I was searching through pictures today at the request of some family and friends. We haven't had a family picture in over a year, mostly because of chaos and some part due to my insecurities. This one immediately brought me to tears, not because of its tenderness but because of the look on my sweetie-pie's face.
It was a busy day. I'm trying to remember if I gave in to his constant need for food or if I tucked him in the stroller and begged him to sleep just a little longer, not knowing how dangerous it was. I had no idea at the time of this picture how desperate his situation was. The look on his face breaks my heart.
This disease can be so deceiving. If you saw my sweetie pie today you would see him running around like any other 16 month old. He's into everything, loves to dance and play with his sisters, and seems as healthy as the next kid. Many people compliment us on how well he looks asking if we are so happy that he is better now. And yes we are so happy and he is doing well. But, most people don't realize it is a daily battle to keep him that way. Its constant guilt and worry over missing an alarm or getting sidetracked on the way to prepare his cornstarch treatment. Its wondering everywhere you go if this is the day he's going to catch a stomach virus that will land him in the hospital. And after all of our best efforts there is still no guarantee that he wont have organ damage or any other complication that comes along with this disease.
I try really hard to be positive and proactive about this all. I read anything I can get my hands on and have recently endeavored to learn all I can about metabolism. Lately, though Ive been feeling quite depressed and overwhelmed.

At our last visit to the Metabolics Dept. It was frustrating as usual. I spend hours there defending choices that I make, trying to understand what they want me to do and just plain get a handle on everything. I leave confused, frustrated, and practically in tears. The cherry on top was when I was told that "I may have to make a choice. Evan being overweight or him developing liver cancer or needing a transplant" As if there is a choice in any of this!!! I don't know why but this just infuriated me. Mostly because I know its not really true anymore with proper treatment, that there is a doctor who is reversing liver damage in some of his patients and my doctors are too prideful to make the call and take his advice. So I have to continue to sit by until August and allow what ever to happen to him while we wait for our appointment in Florida. I can't stand sitting there listen to doctors who may mean well but forget what disease they are treating while talking to me or fumbling through an appointment seeming as if they may or may not understand what they should be looking for.

Another thing that has been weighing on me lately is his future. A friend of mine has recently sent her son out on a mission for our church. It is a long look forward to right of passage for our religion, and opportunity to grow and serve the Lord. A dream that I have for my own son. But in contemplating this I have realized this may not be possible for my sweetie pie. This and some other more private and delicate impressions that I have had, that I can not even bring myself to speak and just added to the heaviness on my soul.

I feel like this post is turning into a pity party, complaining session, that is not what I really want it to be. I just needed to get some anger and fear and pain out. I hope that some other mom will maybe just even get some validation from I'm saying. This is so hard, but not too hard. I have many things to be thankful for with all that has occurred and how well he truly is doing. He amazes me and is a bright shining star in my life. And if I can hold on to those positive things, I CAN DO HARD THINGS.
So I guess that is enough of my little meltdown. Ive got chubby cheeks to kiss.....

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! :)