Ok, since I haven’t blogged in a few months… here’s a quick recap of what’s been going on. What we’ve done, what we’ve learned, etc.

August: Trauma/Drama of the month: Dallin (our then 2 year old) “broke his leg good”- according to the E.R. doctor- in a freak slide accident.
What we learned: ALWAYS call Dr. Weinstein. I thought, “Hey, it’s a broken bone. What does that have to do with a liver disease. I don’t want to bother such a busy man.” WRONG! Fast forward two weeks after the accident, the orthopedic surgeon who had calmly reassured me that he was so young that he’d heal really quickly, is looking at the new x-rays a little dumbfounded and again promising that he’ll heal quickly, just not as quickly as he had expected. “The bones don’t look any more brittle than any other 2 year old’s”. He again reassured me, and told me that there was no need to “freak out” or call Dr. Weinstein. So, I e-mailed our GSD specialist extraordinaire instead- asking if we should up his calcium supplement. A few minutes later he responded and asked if we were getting him enough Vitamin D (800 IU a day) since we live so far north.
He also told me that high lactates will impair healing, so it was very important to maximize control (ie test blood sugars more frequently, and make sure he’s eating all of his scheduled meals/ snacks). Also, pain will also cause lactates to go up! (my exclamation point and bold letters- not his). I had no idea! I hadn’t been giving him too much medication to control the pain because he was acting OK, and I try to avoid meds that may cause liver damage as much as possible. Bad Mom moment.
But in my defense, the kid has an amazing pain tolerance. The E.R. Doctor wouldn’t see him at first because two little 2 year old boys came in with possible broken appendages at the same time. Dallin was sniffling, and only I could tell was in a decent amount of pain, whereas the other little boy was demonstrating his enormous lung capacity at an earsplitting pitch. So, they had us wait for a while while they treated the other boy. His arm had a hairline fracture. When they finally got around to x-raying Dallin (they weren’t entirely convinced that it was necessary) it showed a spiral fracture as well as a stress fracture.
Anyway, I followed Dr. Weinstein’s advice and maximized pain and b/g control. He healed at a faster rate, but still slower than anticipated. Again, they promised there was no way that he would still have a cast in October.

Oh, and we took the boys camping. In a tent. Broken leg and all. We are brave.  Mark caught a fish, Ethan got to roast a beloved hot dog over a fire pit, Jonny got to hold a frog, and Dallin wacked us with his  full leg cast in the middle of the night as Ben and I tried to give 2 am cornstarch in the 5 man tent all 6 of us were sleeping in.  Good times.

We then tried to take the kids to the beach. This time, relying on Naggie Maggie GPS to get us there. Unfortunately, a new freeway had been constructed since her programming, and we spent a couple hours as a blinking dot on a green screen.
We finally got to the beach, and were even able to find a parking spot. We only had time to stay about 30 minutes, but that was enough with all four boys.

This is the first time they’d ever seen the ocean, and I was able to teach them the delicate art of wave chasing, picking seashells, and building sandcastles. Ok, we didn’t actually build a sand castle because I didn’t want to spend the next 12 hours in airports with sand in my pants, but… we got to look at some nice ones.

We were able to get the airport and turn in the car without too many misdirections from Maggie, and I feel sorry for whichever poor rental car agent that had to clean out that minivan.
Checking in was a challenge because they had difficulty finding my dad’s reservations, so we were running a little late by the time we finally got to security. (“Late” by my dad’s standards means we only had 68 minutes to get through security and walk to our gate). Metal detectors and drug checks were as fun as ever, but security was exceptionally nice about it.
Our only snag was when my dad asked a security officer something about the laptop we were carrying-on and the officer said “Wait here.” So, my dad waited. The laptop came, I got all of the kids re-shod, and the stroller re-packed and still, my dad was standing next to the conveyer belt. I asked what he was waiting for and he informed me that if I wanted him arrested for a felony by disobeying a security officer’s orders of “wait here” then by all means I should drag him to our gate. Five minutes later there was still no sign of the original officer who instructed him to wait, so we asked another officer what we were waiting for. Five minutes after that, the second officer returned and said that the first had clocked out 10 minutes ago, so he assumed we’re good to go.
The gate wasn’t too far from the security station, my dad took the boys for potty breaks while I requested seating together. Then it was my turn to run the little boys (individually) to the bathroom for diaper changes before we took off. It wouldn’t have been so bad, except the closest bathroom was out of order, and the electric walkway was too. So, after jogging to and fro a couple of times, we made it back with 40 seconds to spare before boarding.
The flight was pretty easy… normal squirming, but overall, well behaved kids.
Leaving the plane Dallin, suddenly started screaming. We couldn’t figure out why until we saw a flight attendant chasing after us with the “Dr. Weinstein motorcycle”. Crisis averted, thank you Delta, thank you. We’d requested transport, as always, but of course it wasn’t there. We did, however, see a cart drive by us and I tried to stop him, but he avoided eye contact. A couple of minutes later the same guy was driving next to us. I made eye contact this time, and was about to ask for help when he stopped for the woman walking next to us- wearing the mini skirt -and left as soon as she got on. Oooooooh, I was MAD. By this time it was about 10:00 or 11:00 pm local time, so at least it wasn’t crowded. We passed the mini skirt woman a few gates before reaching our own, and before we had time to set our stuff down and make seating arrangements, my dad walked off, without saying a word. I think he needed a little “alone time”. However, he did return bearing gifts of fast food. The kids screamed and ran around no matter how much I told them to sit down, and I got some pretty interesting “Why can’t you control your children???” and “Please tell me they’re not on my plane!” looks from fellow passengers. The flight left pretty shortly after that, and the kids were calm-ish and slept most of the way home.
Ben had just driven 5 hours to get home from his business trip to Utah, and had just enough time to go home, switch cars, and come and get us. I have never in my life been so happy to see him! Since it was technically past midnight, and therefore Mother’s Day, he even had a dozen roses waiting for me. He’s awesome. I slept the whole night, and even slept in until 9 am- without any guilt.
My dad went home, and according to my mom, slept for 3 days straight. “What did you do to him?” was her only question.
He still gives a little shiver and refuses to answer any questions related to his “Florida vacation”. He also refuses to believe that this was by far the easiest trip to Gainesville that we have ever had.

THE END
~well, at least the end of the travelouge.

Now, the whole time Dr. Weinstein was excitedly telling me about Jonny and Dallin’s excellent lab numbers, I had to remind him that Mark and Ethan were definitely having more lows, and not to praise me too much, because he’d be eating his words the next day.

Second Report Card Time: Again, we had my wonderful, can’t express my appreciation enough, father watch the kids while Dr. Weinstein and I headed for the playroom.

We sat down and he said “Good news or bad news first?”

I picked bad.

“Well, Jonny was only the star for one day. The good news is, Ethan beat him”. At this point I have to grab the bottom of my seat so I don’t start dancing around the room, while I pretend that I’m one of those cool people that never cries or jumps for joy. I don’t think Dr. Weinstein bought it, but…

The results were:

Mark: Just a reminder, Mark was 3 ½ years old by the time he was FINALLY diagnosed correctly. To give you some perspective, I’ll put what his levels were in February of 2004 (several months before a firm diagnosis) and his results now. Plus, like in the previous post I’ll put what is “normal” for a “normal child”. I didn’t take a lot of time researching the normal numbers, so if they’re wrong, or you find a better/ more accurate link, someone please correct me.

Cholesterol (should be less than 170)                                    2004: 235             2009: 115
Triglycerides (should be less than 200)                                2004: 2546          2009: 91
HDL (good Cholesterol should be above 45)                       2004: ???               2009: 46
LDL (bad cholesterol should be less than 100-110)         2004: ???               2009: 51
Uric Acid (not sure of normal levels)                                     2004: 7.5              2009: 4.1
AST/ALT (Measures liver damage- normal levels between 5- 60 according to this article)
AST                                                                                                        2004: 596             2009: 28
ALT                                                                                                        2004: 668             2009: 23

Abdomen description: 2004: “ Distended, liver 11 cm below the costal margin.
2009: “ Liver with non-tender edge 3.2 ccm below costal margin; (-2.0 cm since last year)”

Woohoo Mark! Did you see how much his liver has gone down?!?!?! His stomach looks perfectly flat to me now.

Ethan: Reminder- he was about 20 months old when diagnosed.

Cholesterol (should be less than 170)                                    2004: ???                 2009: 99
Triglycerides (should be less than 200)                                2004: 655               2009: 81
HDL (good Cholesterol should be above 45)                       2004: ???                  2009: 48
LDL (bad cholesterol should be less than 100-110)         2004: ???                 2009: 35
Uric Acid (not sure of normal levels)                                     2004: 7.6                2009: 4.1
AST (5-60)                                                                                         2004: 398               2009: 27
ALT (5-60)                                                                                         2004: 434               2009: 23
Abdomen description:

2004: “protuberant.” apparently, no real measurements were taken at that visit. Perhaps because he was a screaming  17 month old?
2009: “ Liver with non-tender edge 3.6 ccm below costal margin; (-1.6 cm since last year)”

Haha! Take that you life-threatening liver disease! We’re winning this war!!!!
Again, for more bragging, all four of my boys received a description of “outstanding” and “now normal” in their evaluations.
And the sugar-free icing on the proverbial GSD friendly cake- Dr. Weinstein actually described me as “laid-back” to another physician. No one reading this blog is allowed to disillusion him.

Meanwhile, back at the research clinic, Mark, Ethan and I were having a blast. They got both of their I.V.s in on the first shot. Mark didn’t cry, whine, or squirm. As long as he could see what was going, he was fine. My kid is so brave! Ethan had the hardest time of all of them. He did a pretty good job; at least he didn’t cry or slap the nurse- this time. After the worst was over, we went to the playroom and had fun playing Sorry, Fooseball, etc. It is so much better now that we don’t have to worry about kids putting their hands on the floor, intentionally pulling out I.V.s, etc. I actually had a really fun time just playing around with my older boys without the little ones demanding attention. I guess all of those people that said “Have fun on your vacation” when we told them about Florida were on to something afterall.

My dad came back after dinner, and party time for me was over. We stayed with all four boys for a while, and then I took them back down to where my dad Valet parked. By this time it was probably 10pm. In the lobby there were a few people just hanging around on their laptops, but I heard a car backfiring and then a bunch of people in uniform walking swiftly to another exit. I went outside to pick up the car, and of course, it was closed. Duh, I’m not in Las Vegas anymore… should have known. So, I carry both Jonny and Dallin back inside to call a security guard like the little valet sign said. Back in the lobby I see a lady crying and screaming hysterically “it’s all my fault!!!!”. While her friend tries to comfort her, and I started to get a little nervous. Okay… this sort of thing just doesn’t happen in nice little sheltered Idaho. At this point, I can’t carry the boys anymore, and they’re so “done” being expected to hold my hand the whole time that they run in opposite directions if they free themselves of my deathgrip on their hands. I went upstairs to get reinforcements- AKA- my dad. We came back downstairs and called for the security guard to help us get our key. 10 min. later she shows up, but it’s her first night on the job, and she has no idea what we want to do. She calls for back up and 15 min. after that we finally get our key and walk through a dark parking garage, grateful to see two more security guards, and we’re off.
I drop my dad off at the entrance and I start driving to the hotel. While waiting to make a left hand turn at the light from Archer onto the street the hotel is on, I take a sigh of relief and think, only a few more hours of hospital stuff tomorrow, and we’ll be home in a couple of days. Everything looks brighter, I can do this. Then Jonny’s chipper little voice calls “Hiya Mom, watcha doin?” Awww, I love that kid. Huh. He sounds kinda close, his carseat should be right behind mine… I look down.
He’s sitting right next to me.
“Ack!!!! How did you get out of your carseat!?!?!?”
It’s not as if I can pull over to buckle him up again. The closest safe place to do that would be in the parking lot of the hotel.
“Oooooh that boy is gonna get it!”
Then I happened to glance at the car stopped next to us. Naturally, it’s a police car. My mind goes blank- until I desperately start praying that he won’t see my three year old sitting next to me. I don’t know how much that ticket would cost, but just traveling to see Dr. Weinstein isn’t exactly cheap, and we’re already on a budget. Panic, deep breath, panic, the light turns to a green arrow, and I proceed cautiously. We pull into the hotel parking lot without getting into a car accident- or getting a ticket. Phew. Adrenoline running high I lecture to my 3 year old exactly how bad it would be if we got hit by another car while he didn’t have his seatbelt on. I have no idea if he “got it” or not, but he hasn’t dared to get out of his carseat again.
The boys get baths and storytime and I finally get to call my husband. Life is much, much, better. He has a very calming effect. I don’t have to mix up formula for them to take their cornstarch in, and they’re on an every four hour schedule instead of three! It’s amazing what another hour of consecutive sleep twice a night will do for you. I slept very well, and woke up still tired, but not the “I can’t do this anymore” exhausted. Always a good thing.

As many of you know, you receive a parking permit in the handy dandy packet that they send you a couple weeks before your arrival. The only problem is the parking garage is on the opposite side of the hospital than admissions and the Clinical Research Center. I was used to and expecting this, but it was a bit new to my dear old dad. It wasn’t too bad this time because we’d brought the double stroller and most of the stuff we needed fit in it. But I heard a few snide comments from the peanut gallery since I had to get a running start to get up the hills (I’m glad I could make their day). The older boys had to walk, and while they weren’t too excited I knew it would be good to get some energy out before they were cooped up for any length of time.

Admissions was alright. They were very friendly at the front desk, and I think one of the receptionists was checking out my dad (ewwww- he really needs to start wearing his wedding ring), but they were pretty busy, so it took a little longer than usual. I have to say, I REALLY miss having Connie in admissions. Thankfully, we get to see her even more now as the GSD program coordinator. My dad took the boys on another potty break, and it was time for cornstarch.

I decided to race upstairs to our room to do cornstarch even though it would be a few minutes late. Unfortunately, the place is a maze. I had written directions from the little packet, verbal directions from the admissions receptionists and I still got a little lost at the elevators. I would have felt really sheepish if we hadn’t asked for directions from people who’d worked at Shand’s a couple of years- and then they got a little turned around. At least I remembered where it was for the rest of the trip, right?

As always, as soon as we walked through the CRC doors, there was immediate relief. It’s the one place where I never have to worry about cornstarch being on time, or if the food they’re giving the boys is “GSD friendly”. The same nurses the boys always have were there waiting for us (and they weren’t even dreading it). They had Jonny and Dallin’s room all ready, and we had cornstarch done in a flash. We got to see Dr. Weinstein within minutes, and we even got to meet another family with two GSD daughters. Always exciting.

My only complaint is that they can only admit two GSD patients at a time. Sad for me, but since even I get the boys confused- and i’m not taking blood every hour as well as urine samples- I can’t really blame them.     Jonny and Dallin were admitted first and later that morning my dad took Mark and Ethan for a day on the town.

It only took one poke each for Dallin and Jonny to get their I.V.s. It’s always been 2 or 3 times before, but it’s been almost a year and a half since the last time they needed one. Both of the boys were really brave and I must say I think I’ve perfected my technique for holding the boys down while the nurses are placing them. We were so proud. Hardly any screaming and no tears at all- and they even said “thank you” when they’d finished. Of course, they were loaded up with toys from the treasure box for their bravery.

Speaking of toys… Every time Dr. Weinstein came through the door “Who wants presents?!?!?” would announce his arrival- it was insane! He spoiled the kids rotten, and enjoyed it every bit as much as they did. Even so, Jonny was naturally wary of anyone wearing a stethascope. It wasn’t until later that night when things had calmed down a bit that he took Jonny (an avid Toy Story fan) to see Buzz Lightyear’s “space ship”. From that moment on Dr. Weinstein was Jonny’s best friend, and “space ship?” was the first thing out of Jonny’s mouth whenever he saw Dr. Weinstein.

Forida Museum of Natural History

Mark and Ethan had a great time that day. Grandpa took them to the Florida Museum of Natural History. They had a blast, especially at the butterfly exhibit. It’s one of their favorite memories of the trip. He even took them to Texas Roadhouse for some steak. Spoiled kids.

My dad came back at about 8:00pm and I took the older boys back to the hotel while he slept at the hospital with the little two. This sounds cruel to do to an almost senior citizen, but as anyone who wakes up multiple times a night to do cornstarch knows, Shand’s is a vacation resort. Besides, I’d promised I’d take Mark and Ethan swimming and my dad wasn’t very comfortable with checking blood sugars like you need to after swimming.

To be continued…

Our third GSD little guy has had diarrhea for a week. Our youngest little GSD one has had a stuffy nose. On Monday, when transitioning our youngest off of his feeding pump and on to eating from his bottle to control his blood sugar, he decided to throw up ALL over me. Great. Oh well, “I guess he’ll just have to be hooked up to his feeding pump for the day.” After having the boys’ two caregivers hook him up for me, I went upstairs to shower off the vomit.

Even on the continuous feed he couldn’t keep anything down. I came downstairs and immediately freaked out. Every time that maniacal little wheel on the pump would turn, so would his stomach. So, I paged Dr. Weinstein. In three minutes he called back and after explaining things, he instructed me to get him to the E.R. and start and I.V. at a particular rate. Of course, I couldn’t find his emergency letter. They’re normally filed carefully, but for some reason all four have disappeared. Nice.

Thankfully, I had two very wonderful caregivers who could take over feedings and schedules without a hitch. My sister Leslie caught the “There’s something weird at Jill’s house” vibe and showed up about five minutes after I had left. Jen had the older boys at the park across the street, and Danielle was busy mixing up the next cornstarch doses. All was calm and under control.

Fast forward 20 minutes as I race into the Emergency Room in Meridian. I think about cutting in front of a nice old lady, but figure it would be best if I at least pretend to be somewhat calm and sane. Finally, I can speak to the receptionist.

“My son has a rare metabolic disease called Glycogen Storage Disease. He’s throwing up and he needs an I.V. NOW.”

The nice girl surveys me and is prepared with her standard reply “Here is some paperwork. Have a seat and when it’s your turn…”

“No, I am not going to have a seat. My son is at risk for a hypoglycemic seizure, or even death if I can’t convince you to get him an I.V. now.” Yes, I am that melodramatic when under pressure. I can’t help it.

Out comes a very nice looking nurse who looks at how young I appear and says “This looks like your first little baby. I know throwing up can be scary, but he looks great. Dehydration doesn’t…”

At this point the baby smiles and coos. I flash a g-tube and repeat my speech. She takes us right back.

After doing my best in a somewhat panicked state to explain to the doctor on call exactly why we were here for “just throwing up” he smiles politely, says he’ll write up an order for an I.V. and leaves the room. I’m pretty sure he called our pediatrician right away, just to confirm that I wasn’t off my rocker. The I.V. is placed, the baby is playing happily on my lap, and we are admitted into the pediatric ward upstairs. I am once again as calm as I ever am with a sick kid.

I love St. Luke’s Meridian hospital. Well, as much as anyone can love a hospital. After being admitted, the nurse comes in with a warm welcome and asks how our family has been the past few months. Apparently she had treated one of our other sons when he had Rota Virus in March- and still remembers us! She even remembered that they put us in the same room as last time. Her C.N.A. and everyone else who came to treat our baby for the next 3 days he was there, remembered us. There was only one other baby in the unit and we had plenty of attention- which was good considering he had to have his blood sugar tested every half hour. Every time I turned around someone was asking if they could bring me something. A far cry from downtown Boise where the nurses are so busy I have to stand in the middle of the hallway until I can catch someone mid flight to reset the I.V. so his blood sugar won’t drop. They’re excellent nurses, just exceptionally busy.

My husband brought me something to eat about 4:00 which served as breakfast, lunch, and dinner. Thus ended Monday.

Tuesday my husband needed to be at meetings at work, so he brought me breakfast and we had a chance to speak with Dr. Joyce, our pediatrician. Later that day my mother-in-law swatted a few locusts by taking our oldest son to his therapy appointment in the same building our baby was staying. She came up to see me during his sessions. My sister Leslie also showed up to bring me lunch and work on a PowerPoint presentation for my oldest son’s school about GSD that I will be giving on Monday. My sister-in-law Bonnie also came back from Utah to help us with the boys for a semester, and she stopped by the hospital on her way to wish us well. She later told me that as she entered the hospital, that she recognized the number and said “Isn’t that where they were last time?” They laughed and nodded – there is now a joke about that being the family suite.

We were finally released Wednesday afternoon, although our baby is still on a continuous feed. That night we had a meeting with Genesis Service Dogs (ironically, also nicknamed GSD). They will be giving our oldest son a labradoodle puppy and will help us raise him to be trained to detect hypoglycemia. We will be meeting with their group once a week and doing some pretty hard core training at home. Huge responsibility, and extra expense, but they are very charitably donating a crate, feeding dish, etc. as well.

Thursday morning I had my wisdom teeth taken out. While at the doctor’s office, one of the boys’ caregivers called and reminded us about another therapy session that morning. One graciously took him and our second son, while our other caregiver stayed home with the other two boys (the baby still on a feeding pump). When I got home I was too swollen to take pain killers and was, as anyone who has had wisdom teeth removed will tell you, miserable. Danielle, the boys’ caregiver who stayed home, had recently had her wisdom teeth removed and knows all the tricks to make me as comfy as possible. At the worst point I heard a knock at the door, and I knew instinctively that it would my friend Jenny. She didn’t know any of this was happening, she just stopped by to see how things were going. She took care of bringing us dinner that night, and helping with kids since my husband had to work late again. Sister-in-law Bonnie was also here helping with the boys since I can’t bend over to give them their cornstarch… or pick them up… or talk and tell them not to do something stupid like dancing on the dinning room table…

My sister Leslie showed up last night and helped with dinner and starches as well as some more work on our PowerPoint presentation. Bonnie watched kids while we videoed how we “starch” the boys via their g-tubes. We also wanted to video tape replacing a g-tube, but I wasn’t feeling well and declined. All of a sudden, right before we put away the video camera, one dramatically fell out anyway, so we were granted the opportunity to tape me explaining how to replace a g-button, all the while, I was looking like a deranged chipmunk . Then when it was time to put the boys in bed, a breaker tripped and we lost power. How grateful I was that Bonnie happily tripped out to our garage, shoving around dusty suitcases to find the circuit breaker box and fix the problem.

One of my all time favorite caregivers also had to quit because her college schedule was too packed. Not unexpected, but depressing none the less. Thankfully Bonnie gave up a scholarship to Utah State University because she felt she needed to be here with us. Can you believe that??? Our Home Health agency hasn’t been nice to her in the past, but suddenly they’ve replaced the evil dragon boss, and hopefully things will be better managed now.

Today, my husband had volunteered himself as well as our older two boys to help clean the church house. I couldn’t take care of the two little ones by myself, so who showed up but Bonnie and my brother-in-law Andrew to help wrangle the boys.

In conclusion, I just want to thank everyone who has helped us, is still helping us, and who will continue to help us. We don’t mean to wear anyone out (but that regrettably happens, nevertheless), we’re just so grateful that whenever things seem dark and hopeless there is always an army ready to destroy the locusts that occasionally plague our family.

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/2007/03/update/ http://starchwars.com/2007/03/update/#comments Wed, 14 Mar 2007 02:48:42 +0000 Jillian http://starchwars.com/control/lib/blog/2007/03/13/update/ Well, our 14 month old is still in the hospital. He has the rotavirus, and we are having difficulty weaning him off the I.V. Everytime we try to gradually drop the I.V. rate, his blood sugar drops below 70 and they have to raise the I.V. rate again. My husband has stayed with him the entire time because we were scared to contaminate me since I need to take care of the 5 year old, 3 year old, and 2 month old at home. Unfortunately it didn’t work. Our 5 year old started acting lathargic and when we tested his blood sugar he was down to 44. Fun. After calling our favorite doctor, Dr. Weinstein, we have put him on a continuous feed since the cornstarch will be difficult for his body to absorb while he’s sick. His sugars are now up to 117. Thankfully he’s stable and hopefully things will get better soon. Apparently this virus can last up to 14 days, and people often re-contaminate themselves.

Also, the public pre-school that attends has been difficult lately. They simply do not understand the gravity of this disease, and the school nurse and psycologist were actually pushing to have our barely 5 year old weaned off his g-tube by the end of the year, and “Why don’t you have him in therapy so he can learn how to eat?” He can eat. We only use the tube for continuous feeds when necessary and cornstarch every four hours. I’ve been repeating this for 6 months, you’d think they would get it by now. Sorry if I sound bitter, but it’s hard enough dealing with just glycogen storage disease, trying to coordinate all of his special services when teachers and principals automatically assume I have no idea what I’m talking about can be even more exhausting- especailly considering I trust them with my son’s life everytime I put him on the bus.

Just before midnight on Saturday, my husband had to take him to the emergency room while I stayed home with the other three. I called ahead to see if they would prepare an I.V. so it would be ready for my son when they got there. The nurse I spoke with said she couldn’t do anything until they arrived, but fortunately another nurse overheard the conversation. She had been our son’s E.R. nurse a few weeks ago and understood that by the time he got there his blood sugar would probably be in the 55-65 range and dropping fast. When my husband walked through the door, he was immediately ushered back where this exceptional nurse had the D-10 I.V. ready. Sadly they had to place the I.V. in his head instead of his arm, but how grateful we are that she remembered us, and cared so much about our child’s well-being. They were later admitted to the hospital and will hopefully be released tonight or more likely tomorrow.