The move has made a tremendous change in our lives. We were able to build our home with a cornstarch station close to the kids’ bedrooms and organize the kitchen geared toward taken care of the kids. Jillian especially is much more relaxed and feels like she has a better handle on taking care of the kids.

Work has been quite demanding the last few months and I hope that I can tone it down so that we can give some attention to StarchWars.com and GSDLife.org.

We’ve posted in the past that Luke has a sensitive nose, and that we’ve experienced times where he’s reacted when the kids were having low blood sugars. We’ve since been trying to work with Luke and the kids to help him train his nose. A favorite game of the kids (and the dog) is the give one of the kids a treat and have them hide. Luke is then given the task to find a specific kid. Luke has recently gotten pretty good at it and has been able to help us find mischievous toddlers that occasionally like to play hide and go seek with out letting us know first. So tonight we decided to give Luke a real challenge.

For the 4th our family went to the city park to watch fireworks and let the kids run around. We took some treats with us and then split up for a little bit making sure that Luke was distracted while the older boys went and played. The park was very crowded and there was numerous fireworks going off in the parking lot keeping Luke a little on edge. I have to admit that I was skeptical that our pooch would be up for a challenge like this let alone be willing to even respond to the command with all the extra smells and noises that were a part of the course. He responded. My wife gave Luke the command to find Mark. He immediately focused and worked his way through the crowd following the winding and confusing path that Mark, Ethan and I took to get to the other side of the park and in a short amount of time found Mark. I was amazed.

Though Luke has come along with some extra challenges, I find the benefit of having a disciplined animal with a keen senses and a real attachment to the  kids a true blessing, and I am less and less worried about him being able to help us with eventually being on alert at all times for low blood sugars.

Now if we can just get him to never have an accident in the house again… I might actually learn to love the thing.

http://www.diabetes.org/uedocuments/df-dog-feature-0308.pdf

It came to my wife’s and my attention that despite the non-stop grind of keeping our children going four hours at a time they are able to enjoy life. They run, play, learn, jump, swim, wrestle, stunt double for one another and even find time for a brotherly quarrel here and there. For the most part they are acting and feeling like “normal” kids, and most importantly they are happy.

As parents dealing with the symptoms and effects of an unchecked and misunderstood child with Glycogen Storage Disease our fears were innumerable about what was coming next and what our children were missing out on. It broke our hearts to see our oldest son struggling to learn how to walk and forgetting things that he had learned. He broke his leg twice and was very lethargic. As he grew older and only his liver seemed to be the only part of his body that was getting better we often wondered about the quality of life that he was going to have or that we could help him have.

I’ve just mentioned a few of the negatives of what can happen and what did happen. I remember various breaking points that my wife and I had to muscle through with little answers and many of them incorrect in how we should proceed in helping our kids. I sympathize with any parent that is in a similar situation and do not envy the grief and hopelessness that comes along with it.

Fortunately, for us the right answers did come and we were blessed and relieved to finally have a correct diagnosis for our two oldest sons at Primary Children’s Hospital in Salt Lake City. Mark being 3 and Ethan almost 2 at the time. This gave my wife and I a new start and hope that we could help Mark reverse the physical and mental deterioration that resulted from the disease.

Though, we had a diagnosis and the kids were doing better, it wasn’t until we visited Dr. Weinstein in Florida that the children really started to progress. In less then one month’s time both boys we were seeing a night and day difference. They started to run and play and we could tell that they were feeling good. I’m not sure how else to better describe it other than they were sickly and now, though they still have Glycogen Storage Disease Type 1A, they are not sickly.

I hope this helps others realize that GSD is something that can be managed and that can allow the individual to do “normal” things. Our children are prime examples, and we’ve seen them on both ends of the spectrum. Yes, it is hard work, but to have a happy child that isn’t dieing on you is worth it. With the way that all four of our children with GSD are progressing we anticipate that they will all have fulfilling lives (minus the sugar addictions).

So far this season we’ve dealt with two good size episodes of different sicknesses. The first being a mean cold that took each one of us down one by one presented the first challenge and the feeding pump rotating from kid to kid. Then as to go out with a bang the nasty germ settled in on my wife and I so that we had to take care of the kids(who were now running around and healthy) in a pitifully sickened state. This time is always a great taste of patience endurance and humility. Unfortunately, I seem to fail on all three at some point or the other.

The second bout was some type of stomach bug that hit us following the same pattern as the first, by hitting each kid individually then finishing off with the parents. This one was particularly nasty; having us deal with countless vomit and poop messes. The Glycogen Storage Disease side of things unfortunatly requires that at home we keep funneling an ample supply of formula / ammunition for the germ to launch at will and the poop messes are less than to be desired, but by the grace of God we have been able to keep blood sugars stable and all the kids out of the hospital.

As a special treat this year, Ethan needed to go in for eye surgery smack dab in the middle of dealing with both sickness challenges. We were pleased however, that things went well and the doctor and hospital did a stellar job on keeping Ethan’s blood sugars up. A bit of a break afterwards would have been nice though. It seemed that as soon as he got home Mark’s stomach bug started to churn.

This all of course takes a toll on church, school, therapy and family activities, and wears us out more than we previously thought was possible.

So, I don’t wish to sound too down hearted about the holiday season. Our kids are eagerly anticipating Christmas and had a blast at Thanksgiving and eating mom’s special GSD friendly pumpkin pie. There are plenty of good things going on to balance out the bad.

Having such a large support of people has been the one thing that has kept us going. Family, friends from church, and home health aids have shown up at the right time to help cheer up a kid, or take a healthy one with them for a little while to have a chance to get out of the house and give my wife and I some chance at respite.

The most humbling of all is that we seem to find ourselves contributing to this season of giving by simply offering plenty of opportunity for others to give. So this year in our quest to keep kids healthy we’ll also be seeing what ways we can help our kids help others. Wish us luck.

Of course, we want them to feel as competent as possible, but as with the physical aspects of GSD the neurological symptoms seem to lie dormant and difficult to detect especially with good metabolic maintenance. I think many a GSD parent is familiar with others not seeing a “problem” because the kid looks normal.

We have recently met with a neuropsychologist that was able to give us some great insight as to how metabolic diseases that impact glucose levels can effect the brain. We were pleased to finally hear someone pinpoint and have a logical explanation for the concerns that we had for our child’s mental health. Apparently, automated processes in the brain that most people take for granted, are the parts that are effected by the glucose deficiencies. In short, a child may gain the ability to write and recognize letters, but the organization of those pieces may be jumbled making comprehension and verbalization complicated and frustrating. This is exactly what we were seeing happen.

We doubt that our other children will have as many of the challenges that our eldest is experiencing, but it became very apparent that because Glycogen Storage Disease disrupts glucose levels, their brains will very likely be effected.We definitely want to know what to look for so that we can minimize their frustration and maximize their learning.

So now that we enter a new dynamic of GSD and the brain we will find out more from Dr. Weinstein as well as our new neuropsychologist . Hopefully we will be able to add some more insight and more accurate information for other parents as we become more educated on the mysteries of neuropsychology.

I’ll start with reflecting on how we came to the decision that a dog was a good move for our seemingly already maxed out household. Every Sunday our family watches the show “Nature” on PBS. Last season we were watching the episode “Dogs That Changed The World” and were suprised to find a section on Medical Dogs. The story told of Delta, a German Shepard that is able to detect the changes in it’s owner’s body chemistry that occurs when he experiences low blood sugar levels. We of course started to think immediately of the benefits for a child with GSD and what the possibilities of having a dog trained and disciplined specifically for detecting the onset of hypoglycemia.

Over the next few months we began our research. We talked to trainers and breeders alike trying to determine if it was possible. The answers varied, but the overall conclusion was that if there was a detectable scent that the body produces when hypoglycemia start to kick in then yes, it was possible. We even found an organization based in California called Dogs4Diabetics that specializes specifically in training dogs to be companions for diabetic patients. Our immediate response was “Eureka!”, but we were disheartened to find out that they were only working with people in Northern California and would only train for diabetic patients. We however didn’t give up.

We found a local trainer that was willing to help us train a dog, but it seemed very iffy that he would be able to help the dog specifically target a response in low blood sugar and it would not be a cheap venture. So, we continued our research and questions. We also shared our idea with Dr Weinstein and he seemed optimistic and excited about the prospect. In fact, during our last visit to Florida we entertained the idea of adopting one of the GSD malteagle pups (that didn’t end up with GSD), but the breed didn’t seem ideal for what we were looking for.

We started to talk to the kids about the prospect of getting a dog-naturally, they were excited. We decided to set a goal to find a dog and started praying for help in finding one that could detect low blood sugars. To our surprise the boys were very diligent in keeping the request in their prayers.

Finally, in July, we came in contact with Paula DeVaney with Genesis Service Dogs (ya that’s GSD too), a local organization that raises labradoodles for various service dog organizations all over the country. Surprisingly, they knew of and had even donated to Dogs4Diabetics! Paula also had a litter of 13 newborn pups that would be ready to leave their mother the end of August. Ironically, it was the uncle of this litter that had been donated to Dogs4Diabetics. No doubt that if we were going to move forward with the decision to incorporate a dog into our family’s lifestyle this was the opportunity which we had been petitioning the Lord for, and working towards.

Since we would be raising the dog to be a service dog, Genesis Service Dogs allowed us to sign on as raisers for the dog with the option of having the dog donated to us if it all worked out. Again, another ideal situation for our family.

A few weeks before it was time to pick up the new puppy we started to attend weekly training and instruction on what we needed to do as a family to correctly condition the dog. Then the time came to pick up the dog. Our oldest son, Mark, being a Star Wars fan had finally decided on naming the dog Luke after contemplating the names Yoda and Chewbacca.

We brought the labradoodle home and he began to impress us immediately. He was rather well behaved for a new pup and took reprimand well. Despite the good behavior of the dog the load started to take it’s toll by Friday. Yet, we were given a glimpse of the possible benefit of sticking with it.

Our kids started to get sick and Ethan went to bed early. With the bustle of the day we didn’t think much of it. After the kids were in bed and the dog in his crate we heard Luke barking and acting very peculiar (he had to this point been very laid back and rarely barked). After a minute we decided we better see what was the matter. We opened his crate and he bolted out of the room and downstairs. He seemed flustered and disoriented. We weren’t sure what to make of it. We let him outside to relieve and took him back upstairs. He was still antsy and we wondered what had happened to cause the alarm.

Ethan then awoke and was in a cold sweat. We checked his sugars and they were at 40. We immediately began emergency procedures to get his blood sugar levels back into the safe zone. After we got Ethan’s blood sugar under control, Luke seemed to finally relax.

Of course, we are trying to be objective in working with Luke. We recognize that this episode could be a coincidence, but the experience has given us “a new hope” that Luke will be able to detect hypoglycemia. It also reminds us that God is blessing us in our efforts to take care of our children to the best of our ability.

A few nights ago we were getting the kids bathed and ready for bed. The process started out normal enough with the typical herding of kids and trying to keep our 18 month out of anything he can think of including the toilet. My hands were pretty full so I had asked my oldest son to take off the pad around his g-button… thud. Not only did the pad come off but the g-button did as well. My son and I shared a slow-motion moment as we looked at the funny looking device that just fell out of his stomach and lay like a slain beast on the floor.

Of course we both quickly realized that this was a bad thing and commenced the procedure of panic and calling for mom to help. Mom had to cut off the conversation that she was having with one of her friends that stopped by and was soon running up the stairs to see what we were screaming about. Luckily she had prepared and had a back up Nutriport g-button close by, which I was able to promptly replace.
Moral of the story… keep extra supplies on hand! Extra g-buttons, lubricant jelly and adrenaline are a must.

First of all the hospital stay was great. It’s a night and day experience staying at Shands Hospital in the CRC (Clinical Research Center) and any other hospital we’ve stayed in for GSD care. Instead of being on pins and needles the entire time wondering if the current nurse understands what is going on we felt that we could relax a little and not have to feel like we needed to continuously watch the nursing staff to remind them how critical a schedule they were on. There was no having to wait while everyone scrambles (or in some cases doesn’t scramble) to find some formula or cornstarch (normally we would bring our own from home) and try to track someone down to take sugar levels that are 15 minutes past due. It was actually somewhat of a relieving feeling being at Shands and our entire family was much less stressed. In fact, I was even able to enjoy playing Foosball and Video games with the kids in the play area.

The best part of course of the hospital experience was the amount of time that we were able to converse with Dr. Weinstein. He was there often to talk with us and to spend time with our children. We were constantly soaking up more information on Glycogen Storage Disease and it was great to have answers to our questions. He addressed every concerned that we had and presented great insight, and modifications to the kids changing metabolic needs. He even smoothed things over with the school which was a tremendous blessing (I believe that my wife will be writing more on the school).

We were ecstatic to learn how well our children have been doing. All of the children’s test results came back as a normal child’s would. Their livers are looking great and our oldest two have recovered tremendously over this last year. Our first having had the most damage to overcome because of the length of time that it took to diagnose him (He was 3 and a half) did not have the incredible growth that our second but had recovered from weakened bones, an extremely inflamed liver, elevated cholesterol and triglycerides and chronic fatigue. He also made an incredible recovery in mental awareness and though we feel that permanent brain damage may be there he is working hard and making good progress in that area as well.

Our second also recovered from the same things that his older brother did, but not having the severity of damage he was able to also grow at an incredible rate this past year, and even though he is one and a half years younger then his brother, they are often mistaken as twins. This is exciting for us, since with the treatment that we had between diagnosis and finding Dr. Weinstein he had plateaued in growth.

The third in line just made it through the first year and a half of Glycogen Storage Disease survival and we were finally able to teach him the ways of the starch. Since cornstarch can’t be used in the first 9 – 18 months (of course he was closer to 18), that stage is particularly difficult to keep the kid stable. So it’s a huge relief to add one more kid to the 24 hour starch regimen.

Of course the youngest is still awaiting his turn to join his brothers in the starch clan, but he is doing well and we just need to keep him that way for another six months to a year. Hopefully he’ll cooperate and it will be closer to six months.

Since we were looking at puppy options for our GSD dog project, we were able to take the kids to see Little Red and the other GSD dogs. The kids loved it and though we may not adopt one of the non-carrying pups from that litter the kids were more excited about our pursuit of bringing a dog into the home.

Unfortunately our plane and hospital schedule did not afford for us to take the kids anywhere fun, but hopefully we can slot that in next year. For now… we need to tough it out another year and hope for the best.

May the starch be with you.

Ingredients:

1 cup unsweetened soy milk
1 cup dextrose
4 eggs
1 package of Kool-Aid

I simply mixed the soy milk, the dextrose and eggs with an electric beater and then cook on the stove for 5-6 minutes. Don’t let it boil (or burn) by consistantly stirring. Remove from heat and add a package of Kool-Aid or other unsweetened package of drink mix and mix again with the beater.

To make the ice cream we purchased the Gelato Jr by Lello to make our ice cream mixtures a reality. It’s a simple machine and we’ve been satisfied with it. Since it was a hot mixture it took the machine close to an hour to freeze.

Again, I was unimpressed with the flavor but my kids liked it. Since the consistancy was the closest to real ice cream that I’ve made as of yet I will continue to tweak it. Please comment if you have any suggestions or another GSD ice cream recipe that your family enjoys.