Next week we will prove even crazier than we already thought we were. If all goes well, we will begin… 24 hour cornstarch. This is only particularly nuts for us because that may mean getting up every two hours at night to feed him, every three hours at night to feed Jonny, and every 4 hours for Mark and Ethan. Oh, don’t forget we still take Luke (the 25 pound “puppy”) out every 4-5 hours as well. Can we say “night of the living dead”? Fitting for Halloween, don’t you think?
This time last year, Jonny was also 9 months old, so, we began the same process. The difference? Jonny’s blood sugar went totally crazy, and he had many, many, many explosive poops with just 3 grams of starch. Apparently he was among the 20% of GSD babies that can’t handle cornstarch by 9 months. I thought for sure I was doing something horribly wrong, and so we gave up for a while, but tried again the next month- several times. Each time it was a “poopy” experience. We were finally able to get him on 24 hour cornstarch when we went to see Dr. Weinstein last May. It was still difficult to believe that that much feces could come out of such a small cute kid, but at least his blood sugar held, and the diarrhea gradually lessend and eventually dissapeared over the next few weeks. The trip home was considerably better hypoglycemia wise, but I really could have done without changing his diaper that many times on airplanes and in airport restrooms. I have a feeling the other travelers felt the same.

10. Set an alarm clock. Nice, but if it’s too close it’s easy to roll over and turn it off in your sleep then call the manufacturer in the morning and scream at them for making a defective clock. Don’t they understand how important cornstarch is?!?!?

9. Place the alarm clock across the room. Somehow we could still sleep walk our way through turning it off (or at least pressing snooze) and climbing back into bed.
8. Set two alarm clocks. Our first alarm goes off in the boys’ room just across the hall. If that isn’t sufficient to wake us at 2am, we have another, set 10 minutes later, that goes off in our room.

7. Booby Traps. The boys often like to covertly help us after going to bed by placing booby traps such as legos and sharp plastic toys they’ve scavenged from various birthday goody bags scattered strategically in the path to the alarm clock. This way they are certain we will step on as many as possible and be fully awake during the brief sojourn into their room to turn off the alarm- ensuring we are painfully awake at 2am.

6. Setting your cell phone alarm to the sound of your child’s voice saying “Mommy, it’s time for my cornstarch. Mommy, get up and get me my cornstarch.” etc. Ingenious. How could you possibly sleep through your child’s angelic voice pleading for cornstarch? After an entire day of three whinny boys (#4 can’t talk yet) saying “Mom… I wanna pony. Mom…. how come Dallin gets to be on a feeding pump and I don’t! Why don’t butterflies have purple eyes?” etc., I’m afraid I’d be more likely to tune out their voices than an annoying beep at 5am.

5. A quick shake. For us, that’s usually my husband grabbing my foot to make sure I’m up to starch the boys while he gets Luke out of his crate for a bladder brake. Others, however use an innovative alarm clock called Shake Awake that actually vibrates- hard. The advertisement shows a woman serenely sleeping with the alarm on the bed next to her pillow. I’ve heard of other GSDers actually having to put it under their pillows to increase the cornstarch jolt. As if we’re not shaky enough from sleep deprivation. However, it is particularly useful when trying not to wake an entire house, especially when vacationing at Grandma’s house.
4. A telephone wake-up service. Not limited to hotels anymore, there are plenty of agencies like Wake Up Land where you can pay a small fee and be woken up at a certain time every day. Who knew there were others as desperate as us to actually look for something like that.

3. The Clocky Robotic Wheeled Alarm Clock. This wacky alarm will actually roll off of your bed side table and then, like your poor meatball on top of spaghetti, will roll right out your door. I’m considering giving this one a try if not for any other reason than to watch my husband chase the thing around trying to pounce on it and finally turn it off at dark-thirty in the morning. The mental image is priceless.

2. The ball shooting alarm clock. This is my personal favorite suggestion, I’m just not sure if it’s legitimate. Supposedly you can aim it at your head and it will shoot balls at the appropriate time to ensure that you are properly awake and seriously grumpy/ lumpy. My google searches didn’t come up with anything for this one, but again, fun mental image. As long as the alarm isn’t aimed at me.

And the Top Answer is…

1. Find a poor relative/ employee you can guilt into doing cornstarch for you. Nothing is easier, or more peaceful, than finding someone you can trust to either stay up all night for starch times, or at least wake up instead of you. We currently get one full night of sleep a week, and it is heaven sent. The only problem is that it is usually our date nights also, so we end up going out late and consequently going to bed late. But somehow the lack of stress for that one night alone is enough to at least get us through the next week.

Thank you Aunt Bonnie!

When our older boys were first diagnosed with GSD our former GSD specialist put them on cornstarch every four hours during the day, and then on a continuous feed during the night so that we could “sleep through the night” (any GSD caregiver will now, undoubtly, be laughing hysterically at the thought). The dream of sleeping through the night turned into a nightmare. The boys would toss and turn so much during the night that the tube on the feeding bag that connected to the g-tube in their stomach would invariably come apart during the night. This is bad for two reasons.
#1. It means the boys aren’t getting enough calories to keep their blood glucose levels up, and
#2. With the tube unkinked, it means that any formula in their stomach could now leak out through the same tube.

I believe I can speak for all GSD 1a parents out there when I say “AAAAHHHHH!!!!!”

To help prevent the tubes from disconnecting, we used every type of tape we had, including, but not limited to, masking, medical, electrical and even duct tape. It helped, but we were still up at least once a night to reconnect the tubing and change bed sheets where the formula had leaked. We also bought a bed wetting alarm pad which was the best thing we could have done. If the tubes came apart, they would leak on the pad which would then send an ear-piercing alarm to wake up the parents while the boys slept peacefully through it. It definately isn’t fool-proof, but it does help considerably.

To make things worse our former specialist did not know, and therefore did not tell us, that when a person with Glycogen Storage Disease type 1a is put on a feeding pump their insulin levels raise to balance their blood sugar. When you discontinue a continuous feed, their insulin levels are still set at high, and their blood sugar drops even faster than it normally would without food.

We were told by our former “specialist” that the best way to transition them off of a continuous feed was to simply stop the feed and give them a dose of cornstarch in a couple of hours. There was also no need to test their blood sugar since they were just getting plenty of calories while on the feeding pump. It should come as no surprise then that while the boys were significantly more active and alert after being put on cornstarch, within a month or so their progress had again plateaued.

After a year of incomplete GSD treatment we were finally able to go to Florida and see Dr. Weinstein. What a wonderful experience. The boys gained more from his treatment than I ever thought possible. They still continue to improve. They both had huge growth spurts, and their minds have been much more alert. My oldest son had 3 therapists at the time that all independently mentioned that he had improved more in the first month right after Dr. Weinstein, than he had in the entire 9 months they had been seeing him previously. What more of a testimony to do 24 hour cornstarch do you need?

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://starchwars.com/2007/07/glycogen-storage-disease-what-to-expect-the-first-year/feed/ 0 http://starchwars.com/2007/07/malteagles-malteagles-malteagles/ http://starchwars.com/2007/07/malteagles-malteagles-malteagles/#comments Fri, 20 Jul 2007 05:06:20 +0000 Jillian http://starchwars.com/control/lib/blog/2007/07/19/malteagles-malteagles-malteagles/ Malteagle pictures

Some are so cute, some are so ugly they’re cute, but either way, we love malteagles. These photos were taken of the puppies bred specifically for helping to find a cure for Glycogen Storage Disease. Dot (mom) was a carrier, given to Dr. Weinstein by Duke University. Last December she had darling little puppies- one of whom has Glycogen Storage Disease. To tell the GSD pup apart they painted her toenails red, hence her name, Lil Red. We were able to snag a few pictures of the puppies last May when we took our boys to visit Dr. Weinstein at the University of Florida. They are housed in a small room in the Veterinarian School and have 24 hour care. Lil Red’s blood sugar is tested frequently, just like our boys, and she is given her cornstarch through a tube, just like our GSD boys. Unlike our boys, however, they couldn’t place it in her stomach because she would scratch at it. You know you want to see where they did place it. Click here to see more GSD malteagle pictures.

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.

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”.

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