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/glycogen-storage-disease-get-together-in-the-west/ http://www.starchwars.com/2007/07/glycogen-storage-disease-get-together-in-the-west/#comments Fri, 13 Jul 2007 01:04:14 +0000 Jillian http://starchwars.com/control/lib/blog/2007/07/12/glycogen-storage-disease-get-together-in-the-west/ At our last visit to Florida and Dr. Weinstein, he mentioned we should try to find a way for our boys to meet others affected by Glycogen Storage Disease. Since most GSD kids in the U.S. seem to be in the east, it’s no wonder that so are most of the conferences. As far as I know, there are only five GSD kids in our state- and I have four of them.

So, it’s no wonder that I was very excited to hear from Stacy, another mom of GSD kids, in Utah the other day. Apparently Dr. Weinstein will be attending a conference in Salt Lake this fall, and she and Stephanie, mom of GSD type III kids, will be planning a mini Glycogen Storage Disease get-together and dinner while he’s in town. It’s still in the very early planning stages, but the dates will most likely be in the latter part of October in the Salt Lake City area. If anyone else would like to attend please e-mail me! We’re happy to meet all ages, all types.

We told Dr. Weinstein (www.glycogenstoragedisease.com) our woes and he offered to call the school on our behalf. He had a 20 minute conversation with the principal on the phone, came back into the hospital room and said “I just spoke with the principal. He’s a good guy and very reasonable. You won’t have any more problems with the school. I was ecstatic- yet slightly skeptical.

We had our son’s IEP meeting the following Monday. We didn’t have ANY problems with the school. He has an aide assigned to him and one other child with “redirect ional” needs. In retrospect, I think this arrangement will actually be better than a one-on-one aide. I hope it will make my son feel a little less conspicuous, while at the same time providing adequately for his needs. We will also be having a “training” meeting shortly before the new school year begins. The principal suggested that he and several other back-up people including the secretaries be taught how to administer cornstarch, test for blood sugar and how/ when to administer glucose.

For the first time in months I was able to drive by the elementary school and feel calm instead of nausea. How grateful we are. Thanks to Doctor Weinstein, I am no longer the “psycho mom whose kid can’t have ANY sugar is being treated with a home remedy of cornstarch of all things!”

]]> http://www.starchwars.com/2007/06/the-school-is-fixed/feed/ 2 http://www.starchwars.com/2007/05/bloodless-glucometer/ http://www.starchwars.com/2007/05/bloodless-glucometer/#comments Mon, 07 May 2007 20:57:06 +0000 Jillian http://starchwars.com/control/lib/blog/2007/05/07/bloodless-glucometer/ CNN just wrote an article on a new device that can test blood sugar without pricking the finger! Apparently the device penetrates the skin with near-infared rays eliminating the need for an actual drop of blood. Of the five clinical studies already performed, the new device has tested 85% accurate, whereas a glucometer has an accuracy rate of 80-85%. Here’s the best part, should be available within the next year! They also hope to use this same technology to develop a device to test lactates, cholesterol, etc. What wonderful news! Visit http://www.cnn.com/2007/HEALTH/conditions/05/07/diabetes.sugar.reut/index.html for the full article.

My greatest fear as a parent is that one of older children on 24/7 cornstarch will throw up their cornstarch and go back to bed without telling me, I’ll sleep through an alarm to give him his 2am starch, and I’ll wake up at 6am with my son either in a coma or dead. Or, another scenerio, the tube connected to the feeding pump simply detatches from the tube connected to my younger boys that don’t tolerate cornstarch yet, it falls on the floor instead of the bedwetting alarm http://www.southwestmedical.com/Incontinence/Bed_Wetting_Devices/Wet_Chek_Bed_Wetting_Control_Device/565p0 we have to notify us of just such an event, and I wake up to that kid either in a coma, or dead. (And I wonder why I have trouble sleeping at night when I’m exhausted).

Could you imagine how wonderful a service dog like this would be!?!?! On saturday when my son went swimming for an hour, by the time we got him dried off he just sat on the floor to play with blocks. We tested his blood sugar and he was down to 35. He showed no symptoms, we just knew he was acting “tired” and he had been swimming. What a tremendous peace of mind a dog would be! Not that we wouldn’t continue to test their blood glucose levels, but it would be such a relief to know there was someone, or somedog, that would be on the look-out too.

Were we able to obtain such a dog my fears of sending our children to school would be drastically diminished. Perhaps a dog trained to recognize low blood sugar would be even more effective than a person trained to do the same. The difficulty may lie in the school accepting the responsibility of being trained to respond to the dog. In Idaho I imagine even that will be a fight.

I knew dogs could be trained for a multitude of tasks, including the obvious assistance for the blind and deaf, locating missing persons, and bomb sniffing, but it never crossed my mind that they could be trained to recognize hypoglycemia- or at least it’s scent. How grateful I am to people and organizations such as Dogs 4 Diabetics http://dogs4diabetics.com. Now the only problem lies in finding someone or agency who trains these exceptional dogs. So far we haven’t had any success finding any in Idaho. If you find one, or have any suggestions as to how to go about finding one, please let us know! Obviously we prefer a Malteagle. Aside from being adorable dogs, they’re helping us cure GSD. http://glycogenstoragedisease.com/

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.

At the first meeting I was my then 4-year-olds only representative. Sitting around me were the principal, pre-k teacher, school psychologist, speech and language pathologist, school nurse, the occupational therapist and a couple other people all representing the school. I assumed (I believe incorrectly) that we were all on the same “side” for my son. He is my oldest son, so I had never done this before and comparably they have never done this for a child with GSD before. They smiled sweetly at my requests and suggestions, asked about GSD, but their eyes glazed over at “rare metabolic disease” and only caught “must have cornstarch every four hours” and “no sucrose, fructose or lactose diet”. Perhaps I down-played GSD a bit. It is, afterall, a very treatable disease and as long as it is treated aggressively children appear “normal”. Which is it’s best and worst characteristic. Because my boys are all so happy and relatively healthy people have no idea how much work it is just to keep them from dying- but I digress.

At anyrate I left the meeting feeling my child would be safe in the school. The caring teacher would not let him eat any foods that weren’t either sent to school or pre-approved by me. The school nurse would be there at all times to give him cornstarch, or in case his g-tube fell out or there was some sort of medical emergency she knew “exactly what to do”-she’s a nurse afterall. There was an aid in the classroom and only 8-9 students, and none that required extensive attention this year. I would bring him to school each morning after breakfast so as not to be tempted by sugary cafeteria food, and he would ride the bus home. If there was an emergency, I would be called immediately and I live less than 3 miles away.

All was well until early December when the teacher called and said “The nurse didn’t come to give your son his cornstarch” (he was already 45 minutes late). “Do you still want him to ride the bus home?”. I was at the school in 3 minutes and tested his blood sugar. Thankfully, he was still at 69. It is my understanding that 70 is the magic blood sugar number. Anything below that and you are considered hypoglycemic. If a person has frequent or many hypoglycemic episodes, brain damage is possible and developmental delay probable. When I arrived in his classroom only my son, and the teacher’s son (a typical peer in the pre-school) were in the room. I tested his sugar, gave him a pixie stix to boost his blood sugar, gave him his cornstarch, got his coat and back pack on, and waited another 10 minutes for his teacher to finally show up so I could ask what happened.

Apparently the school nurse was sick and didn’t come to school that day. Another school’s nurse had come the day before to give him his cornstarch, but she just didn’t show up the second day. The teacher had been in IEP meetings all day, so she didn’t know what had happened until the school day was over and the aid who had been running the classroom mentioned it to her. Thankfully, she called me immediately. When I asked why the nurse hadn’t been missed earlier in the day I was informed that she NEVER comes to school before my son’s 10:00 feed. She is in charge of several elementary schools in the district, and can’t be everywhere at once.

So, three strikes in one incident. The nurse is NOT there in case a g-tube falls out, or he becomes hypoglycemic, AND they missed a cornstarch feed. At least the teacher called and was somewhat appologetic, but he was still left alone for at least 10-15 minutes when he was hypyglycemic and in need of medical attention.

I was nine months pregnant at the time with GSD kid #4, very upset, scared, and angry- not to mention hormonal. In retrospect perhaps I should have said more at the time, but I was concerned that if I started, I wouldn’t be able to stop, and frankly I can’t trust myself when in that state to be reasonable. So, I let her know that it was completely unacceptable, that I didn’t blame her, but rather the nurse and I documented that it had happened. I called my service coordinator who agreed that it shouldn’t have happened, but “what can we do about it now? At least they know not to do it again.” I was too overwhelmed, and proud to either know what to do or ask for help.

After I had our last baby was born, our son went back to school. However, our CNA was on maternity leave herself, and I didn’t have any practical way of taking him myself after breakfast- I had two kids to transition off feeding pumps every morning not to mention a three year old who intensely wanted to go to school too and we have pretty cold winters here. Not plausible. The best I could do was say “Don’t let him eat anything I don’t send” and pack breakfast for him to eat at school.

Recently, my son came home from pre-school with a very cute letter “R” covered in raisins. We didn’t find it in his backpack until after the school day was over so I waited until the next day, took my son and a home health aid to school instead of letting him ride the bus, and asked the teacher’s aid if they knew raisins were a fruit and bad for him. She said, of course, “But we made sure he didn’t eat any at school.” When I asked where the teacher was so I could make a game-plan with her and decide what was and what was not OK to tempt my now 5 year old with, I was told that she had called in sick. When I asked to speak with the substitute so I could make sure she wouldn’t feed him, I was told that they had asked for one, but she hadn’t shown up yet. I left my aid at school with my son for the day, and found out that a substitute never showed up.

After leaving several messages for the principal which were not returned, I asked my sister (an SLP with extensive knowledge of the school system) to come with me and talk to him. He saw us almost immediately. We tried to explain that raisins are bad for him, and he just looked at me like he’d never heard it before. My sister tried to use the analogy that it was like using an asprin pill to trace the letter “a”. One asprin won’t a kid, but how many will start to cause damage? He called it a rediculous analogy and condescendingly added that “It may look like just an art project, but they’re really learning valuable fine motor skills.” I know that, but why couldn’t they use red ribbons, or rocks? We explained that the raisin wasn’t the real problem, but the miscommunication about what my son would be exposed to at school. We didn’t want the teacher to get in trouble, we just wanted everyone to be on the same page. He said there would be no more food in the classroom until we could all agree on a plan at a meeting- probably next Tuesday. He would get back to me.

After that, I sent an aid with my son every time he went to school. It was necessary anyway because we were unexpectedly “dropped” from our pediatrician’s care and the school nurse no longer had authorization to give him cornstarch.

The next school day, according to my son’s C.N.A., the teacher talked to everyone who came through the door about what happened when she was out sick. “She said one raisin could kill him. The principal chewed me out….” The response to one of these from the schools SLP was “Well, he is on your side, right?” “Of, course, but still!”

Then came our week with Mr. RotaVirus (see previous post). I never heard back from the principal, although I called once or twice to find out a time. Tuesday morning I answered the phone to hear him say “Where are you? We’ve already started the meeting.” I explained I didn’t know about the meeting, I had a child in the hospital, and the best I could do was drop off a pamphlet I had about GSD. He was short and said that it would be helpful, obviously believing he had told me about the meeting. I checked my caller ID to make sure I hadn’t just forgetten the conversation, but I didn’t have any phone calls from the school that week.

We finally had a meeting to discuss what is and is not appropriate for him at school. The superintendent of Special Ed was there as per our request. My husband also decided to miss work, and join the pow-wow. Overall it was a COMPLETELY different experience. With my husband and the superintendent there, I felt much more comfortable offering opinions than previously done. After speaking with another, very knowledgeable, GSD mom, I decided to ask for an aid to accompany my son to school. That way, the over-worked nurse and teacher wouldn’t have to worry about cornstarch, making sure he didn’t eat non-GSD friendly foods, could watch for hypoglycemia, etc.

I was told that it wasn’t practical, that they rarely ate foods, that there was ALWAYS a substitute for either the teacher or the aid, and that they had already re-done the lesson plans since the beginning of the year to accomodate the GSD diet. There were plenty of saftey nets. They also mentioned that children who have an aid assigned to them also tend to be much less independent. A valid point. It was made clear to us, that if we asked for an aid then, that our request would be denied. I only have the staff to send someone with him to school twice a week, and they were not happy about that prospect either.

I would like to add that of the 6 times in a three week period that one of my son’s aids accompanied him to school either the teacher, or the aid was missing twice. The day he missed his cornstarch, it was only the aid in the classroom. No substitute.
So, my question for you the reader is… Am I being unreasonable to ask that an aid accompany him? Will it really do more harm than good? Would an aid they supply simply forget cornstarch, and not catch hypoglycemia anyway? Do you believe that they will stick to a GSD friendly diet the remaining six weeks of school? Is it worth the fight now, so I can ensure that not only my eldest son, but the next three will be treated as they should at school? Is it unreasonable to expect perfection from a school district with only one high school, one middle school, and four elementary schools?

Believe it or not, this IS the abridged version. If you actually took the time to read this post, you must have an opinion. I really do want the truth. Please help.

http://www.ipulidaho.org/

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.

The chances, of course, are rare that even if a person happens to be packin’ the GSD gene, that they will find that special someone to marry and they also happen to have the same genetic problem. I suppose that there is always the scenario where you’re family tree doesn’t branch and that would change the statistics around a bit, but really if that’s the case, you’ve got other issues deal with.

But let’s say you do find that one in a million like myself, there’s still only a one in four chance that your children will end up with both resessive genes needed to make up the magical formula needed for a successful GSD kid. Even with the odds bordering that of the lottery, there is a concern. Heck, we beat all of the above without even really trying, so i guess anything is possible.

There is comfort in knowing where your gene pool is tainted and what the potential is. I have to admit that a heads up that our kids were going to have GDS would have been nice. A lot of guesswork and worry could have been avoided. Anyway for those that wish to know about where to go to to find out if they are indeed on of the few chosen to carry on the legacy of Von Guerke’s here is a link to Duke University Glycogen Storage Disease Lab as well as Prevention Genetics which links are both found on The University of Florida’s Glycogen Storage Disease Program web site.

I won’t pretend to know much about the process or how it works. As I learn more I will add comments to this post. But hopefully this points those interested in the right direction and I encourage those that find out more to add their comments as well to the blog here.