We’d love to have your feedback to know if this could be a viable community for GSD.

While we were in the NICU with our third baby (the first to be diagnosed in infancy), we set up a schedule that maintained his blood sugar levels. Every two hours he would be fed 1 ½ ounces of Enfamil ProSobee and I would nurse him on the odd hours. Obviously, his dosage went up as he grew, and we discontinued breast feeding after 3 weeks. With our fourth baby, we chose not to nurse, just feed 1 ½ ounces every two hours. What he didn’t eat, we tubed.

Some parents choose to continue to feed every two hours throughout the night- usually via ng or g-tube. With other GSD kids in the house, this was a bit too impractical for our family. We choose to hook him up to a feeding pump at night. The pump is scary because the tubing can become disconnected at night, and transitioning him off of a continuous feed is tricky as well. Their insulin levels are higher during a feed, so after about 20 minutes off of the feed, they plummet. Scary, and not fun to deal with. This is a photo of three of our boys on feed pumps during their bout with RotaVirus. Normally, only the baby is using a pump at night.

While we love g-tubes, it is also important to only use them as necessary. Many infants lose the concept of “I put something in my mouth, I swallow, and I feel full.” if they are fed too much through their g-tube. I am fortunate enough to have two Speech and Language Pathologist sisters whose job it is, in part, to re-teach how to eat. So far we’ve avoided many problems, but it has been a lot of work for poor babies.

We have also had to deal with developmental delay. Even with good metabolic control, our GSD babies have had a few issues- mostly due to not wanting to lay on their tummies with a g-tube. They were also somewhat late in wanting to sit up without assistance. I found the best product for that! I had been working for weeks trying to get our baby to sit by himself. We’d done the normal- sitting him on the floor with pillows around him, and the extreme- sessions of balancing him on exercise balls to improve balance. My aunt sent us an excellent new baby chair by the Prince Lionheart company called a “bebe pod”. WE LOVE IT!!!! He could finally sit up and play with his toys while watching his brothers run amuck. Within two weeks he could sit without assistance. When our fourth baby was showing the same signs, we got out the babe pod, and without the crazy therapy sessions he could sit without a problem. Thank you Aunt Debbie!

Starting solid foods is also a bit of a dilemma. I still haven’t figured that one out completely. It is very difficult to control blood sugar while introducing new foods and the child becomes more and more active. The time between introducing solid foods and becoming more active, and the time the infant tolerates cornstarch, is by far the most difficult in the first year. We’ve just begun that stage again with our fourth baby and I believe it just might drive me crazy this time- as if I weren’t crazy enough already.

Disclaimer: Every child is different. It is important to follow your specialist’s advice. Our pediatrician fought us tooth and nail about nursing him even a little bit. Breast milk contains lactose which is not good for GSD babies. We took precautions to monitor lactate levels, and it was only used as a supplement for the first few weeks of his life. We, as parents, felt very strongly that I should. We didn’t feel the same way with our next baby. I’ve learned that it’s important to trust parental instincts after getting as much medical information as possible. Perhaps this is why we like Dr. Weinstein so much. He doesn’t push us in any direction, he just gives us the information we need to make good decisions regarding their health care.

]]> http://www.starchwars.com/2007/07/glycogen-storage-disease-what-to-expect-the-first-year/feed/ 0 http://www.starchwars.com/2007/07/being-prepared/ http://www.starchwars.com/2007/07/being-prepared/#comments Mon, 30 Jul 2007 03:57:46 +0000 BMW http://starchwars.com/control/lib/blog/2007/07/29/being-prepared/ Just another small example on how the unexpected can happen and give you a sudden spook.

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.

3. Call ahead and request transportation. This was us last year: Two parents literally running through the Atlanta airport carrying an infant in a carseat with a blanket propping up his bottle, wearing backpacks and lugging more luggage than technically “allowed” simultaneously dragging two young children wearing small monkey backpacks/leashes while mixing up cornstarch and trying to suck it all up with a syringe because we’re 10 min. past due. Barely making the tram and praying the next flight is also delayed- or at least knows we’re coming.

This was us this year: Serenely (well, as serene as anyone can be with four kids after flying across the U.S.) driving in a bus on the tarmac, waiting for a B57 to pass before we continue our 5 min. drive to the next gate that took us 20 min. to run to last year. Arriving with a few minutes to spare before the next cornstarch dose was due. Aaaah. The peace.

In another airport they just transported us on a little go-cart to the next gate. Nice, but not as exciting as the little bus.

When you make your airline reservations, ASK FOR TRANSPORT!!!!

4. Reserve your hotel and rental car ahead of time and don’t be ashamed to ask for a medical/hospital stay discount. Try to ask someone who has stayed at a hotel in the area for a reference. Never go off pictures online-again disaster from last year, but at least I got my money back. We really liked the Hampton Inn in Gainesville. It was a bit older but so are most of the hotels in Gainesville. It has been remodeled though and while it isn’t the best looking Hampton I’ve ever stayed in they went out of their way to accomadate us. Everything from a room at almost half price because we were visiting Shand’s Hospital, to making GSD friendly eggs for the complimentary breakfast. The attendant even asked how many days we would be staying so she wouls be sure we would not have a wait for the eggs next time. There is also a pool- albeit right off the freeway. The best part of the hotel, however, would be the beds. Very nice.

I hope these few tips help. If you have any of your own please share!

As anyone who has read my other pages on this blog knows I am a huge fan of simplifying. The simplest way for our family to do and store cornstarch is in Snack sized Ziploc bags and 3oz bottles of prosobee. Some people prefer cups with a sealable lid, but that’s just too bulky with multiple GSD kids. The simplest way to bag cornstarch is to buy it in bulk. Once a month I special order two 25lb. bags of Argo cornstarch from Winco Foods. Every Tuesday morning we bag cornstarch for all the boys for the week. In the van I keep a 24 hour supply. (Their doses of cornstarch vary by time of day). In the 24 hour kit I also store three 60cc syringes, one bolus feed g-tube (they share in an emergency), one can of formula and a bottle of water. Keeping it in the car has already saved us in one instance when we just couldn’t get home in time for their next starch.

In our 72 hour kit for the family we have enough food and water to last our family a bare 72 hours. (canned food, etc.). We also keep enough doses of cornstarch and canned formula to last the full 72 hours, as well as several baby bottles for our infant, diapers and wipes. In addition we keep 8 feeding bags (our two youngest are still on continuous feeds at night), 6 syringes, 2 g-tubes, 12 I.V. sponge packets (we use these around their g-buttons), 1 roll of medical tape, a portable gram scale and a checklist that includes things we can’t keep in the kit such as feeding pumps, additional baby bottles, and medical notes from their GSD specialist.

This is a lot of stuff, but it is all necessary. We should probably keep changes of clothes, but it would simply take up too much room. All of these supplies are kept in two bags in our coat closet for easy accessabilty. Some people choose to use huge Rubbermaid Storage boxes and include tents and blankets/ sleeping bags, but if it were an emergency where we had to be bussed out (like Hurricaine Katrina victims) there is limited space, so I think packing light is the best way to go.

http://www.ipulidaho.org/

Glycogen Storage Disease type 1a kids like mine cannot regulate their own blood sugar. So we give them cornstarch every 4 hours, 24 hours a day. Even in the middle of the night. My third child (15 months) just barely started tolerating cornstarch, but is on an every three hours schedule, so yes, the time and amount vary with each child and the amount even varies with what time of day it is.

Since our kids have gastrostomy tubes (g-tubes) “starching” them is pretty easy. They have no choice. Some people prefer to drink the cornstarch, but it must be raw (uncooked) and it is a considerable amount- several tablespoons at least. That is a battle we have chosen not to fight-yet.

Every week, using a gram scale, we spend about an hour measuring cornstarch into small snack sized Ziploc bags http://www.ziploc.com. We then write the time that that dose must be given and the initial of the recipient since the dosages are different with all of our kids. Then we store all of the day time bags downstairs in a small drawer in the kitchen, and all of the night-time bags upstairs in the bathroom next to the boys’ bedroom. We have found that Rubbermaid makes a drawer that fits Ziploc bags perfectly.

Every night at 10:00pm starch, we lay out the 2:00am and 6:00am starch bags so we can tell at a glance if we’ve really woken up and starched them or if it was only a dream. Sleep depravation does crazy things to your memory.

To administer the starch all we do is pour a 3oz jar of ProSobee (soy) infant formula (http://store.enfamil.com/nursette_3_fl_oz.html ) into the Ziploc bag, and shake. It’s easy to feel if there are any clumps of cornstarch when you use a bag as opposed to a container. Then we suck up the mixture with two 60cc syringes, insert the tube into the kid’s g-button, slowly push in the cornstarch mixture, and rinse with about 15ccs of water. At night the boys don’t even wake up. It’s also important to shake the cornstarch instead of stirring because cornstarch will stick to the spoon. Pre-mixing the solution will also make the cornstarch less effective.

One last tip. Argo and Kingsford brand cornstarch are the purest, last the longest, and apparently taste the best (http://cornstarch.com/index.htm) . We buy our Argo cornstarch in 25lb bulk packages at Winco Foods for only $20. For now that lasts about three weeks, not a bad price to pay for life-saving “medicine”.

]]> http://www.starchwars.com/2007/03/shaken-not-stirred/feed/ 3 http://www.starchwars.com/2007/03/a-family-disease/ http://www.starchwars.com/2007/03/a-family-disease/#comments Mon, 19 Mar 2007 03:59:40 +0000 Jillian http://starchwars.com/control/lib/blog/2007/03/18/a-family-disease/ When a child has a rare, life-threatening disease like Glycogen Storage Disease, it doesn’t just affect the child or even his immediate family. It touches and alters the lives of extended family as well. For instance, last week when our GSD boy #3 was in the hospital, my husband needed to stay with him 24 hours a day to coordinate his care. The nurses and even our new pediatrician had never seen a child with Von Gierke before, nor will they be likely to ever again unless it’s one of our kids. He needed to make sure everyone was on the same page, which is a full time job. Since he was there and couldn’t leave, we had to rely on our family, mainly our parents to bring food up to his room for the five days our son was admitted.

On the other hand, it isn’t possible to be in good control of three other GSD kids at home by myself- let alone when they get sick too. All last week I had a member of the extended family staying the night and helping. First, my mom, then my sister-in-law, and then my mother-in law. All of them helped keep an ear out for faulty machines, alarms for the 10pm, 2am, and 6am starches, plus cleaning up various bodily fluids (thank you Mister RotaVirus), checking blood sugars, making sure g-tubes didn’t kink etc. I could never have done it by myself, I sincerely appreciate all of the help so I could get some sleep.

I also am also learning how to “use” various family members without “using them up”. For instance, i use my sisters who are both Speech and Language Pathologists to answer sporadic questions about my children’s development, referrals for good therapists in the area, etc. “Aunt Leslie” has also gone over the top in helping me advocate for my older two sons. I am very passive by nature, and fighting an authoritarian figure such as a principal is very difficult for me, even when I know that it is my sons’ right to receive certain services. It’s amazing to me how true the adage “the squeeky wheel gets the grease” is.” Then I ask my brother (in law school) whether certain laws pertain to my children, in this state, etc. My other sister is excellent at creating new sucrose, fructose and lactose-free recipes. Two of my husband’s brothers are still in high school and pros at g-tube feedings now. We love to “use” them and my mother-in-law as babysitters since we can’t trust a neighborhood teenager with this much responsibility. My ten year old sister-in-law is a wonderful “mother’s helper” and can keep the boys very entertained while I get a few chores done on Saturdays.

The list goes on and on. My point is, if my husband and I tried to do this on our own, we would have burned out long ago. Also, if we’d tried to just use one family member for everything, that poor family member would be toast too. “Using” each family member for their particular talents, taking into consideration how much time they have to devote and how much they sincerely want to help is an essential talent I am still developing.