This was our first year that all four boys to to have ultrasounds. Our insurance won’t cover them in Florida anymore, so starting last year we have it done in Idaho before we go. I was able to take the older two before school started the Monday before we left. That way they could be “fasting” after 6 am cornstarch, and we kept a close eye on their blood sugars. Luckily they made it until 10am cornstarch with above 70 blood sugars. A miracle without eating.

The boys, of course, had a blast. The crazy kids couldn’t stop giggling, there’s just something about looking at your “guts” that 8 and 10 year old boys think is hilarious. Afterwards the ultrasound tech asked me to wait while she showed the test to the radiologist and he would be in shortly to speak with me. They’ve never done that before. Typically, the results are just sent to Dr. Weinstein and then he explains how they’re doing when we see him in Florida.

Luckily, the radiologist didn’t take too long, or I would have needed a paper bag to control my hyperventilating. The bad scenarios I can think of in a five minute wait could possibly break a world record. Thankfully, this is just a new standard procedure, and not one of those “talks” with a doctor where your world is turned upside down. He reported that everything look perfectly “normal”. He went so far as to say that he never would have thought they even had a liver disease- let alone a life threatening one like GSD. I may or may not have teared up a bit.

“You have no idea how amazing this is for us!”

“Oh, I pulled up his ultrasound from last year, and I could definitely see an improvement”

Ha! For one thing, last year’s ultrasound was “outstanding”. He has no idea how far we’ve come. I’m still in shock. My little 18 month old’s liver was so big he looked more “pregnant” than I did when I was full term. His liver was so chock full of cholesterol that a biopsy came back Cholesterol Ester Storage Disease instead of Glycogen Storage Disease (one more reason you should always do a blood test when possible). Now a doctor is surprised that my 10 year old even has a liver disease! Victory!!!

Ethan’s liver was also “perfectly normal”, so it was off to blood draws. They were able to have them done simultaneously, and they even had  a race to see who could be done first.

After I dropped off the older two, it was time for the little ones. They, too, thought ultrasounds were amazing. The tech took the time to let them hear their heartbeats, and listen to their lunch since they couldn’t fast at all before the test. They loved being told that they behaved better than their brothers, and they made sure to rub it in when they got home.

The radiologist was at lunch, so I went to get the little ones’ blood draws while we waited for the doc to come back. They’re getting so big!!! As long as they could watch the needle going in they were perfectly still, and they didn’t need to hold my hand or anything. It’s fabulous that they aren’t scared anymore, but sad that they’re growing up so fast.

Right after blood draws it was time for cornstarch, and I completely forgot to go back to see the radiologist.

Ok, I know that I always take twice as long to say anything as I need to, but I’m really going to try to keep this short. I have some seriously fun stuff to blog about next. Also, this post doesn’t have a whole lot about GSD in it, it’s mostly just me whining, so feel free to skip this one.

As we were waiting at a stoplight in Gainesville on our way back to the airport we heard a screEEEAAACH and then BAM! we were rear ended. The boys were all ok (a few headaches, but nothing serious), and they were a little scared at first. Ben’s first word was “Really?” and then they all started laughing about it. It’s a good thing we all have a weird sense of humor; I can’t tell you how many times the car accident has been reinacted with matchbox cars. Anyway, Ben was pretty stiff, but ok. I had to turn my whole body instead of just my neck to check on the boys, but we were ok enough that no one needed to go to the hospital. We decided to just exchange insurance info and get to the airport to try and catch our flight home.

About half way there my back REALLY started to hurt. By the time we got to the airport and filled out incident reports etc. I could only gimp along. Ben had to lug all of our luggage, while I did my best to keep up. We finally got home around midnight, and I have NEVER been so grateful to see our night nurse in all my life! The next day (Saturday) I was definitely worse, so Ben watched the kids while my parents took me to an urgent care/ E.R./ I don’t really remember, I was in a lot of pain. They ended up doing an x-ray to make sure I didn’t re-fracture any vertabrae, but it looked ok. She gave me a whole mess of pain killers and muscle relaxants and told me to see another doctor in a few days if I wasn’t getting better. The good news is the meds knocked me out and I got to catch up on a lot of sleep. The bad news is the meds knocked me out, so I couldn’t take them after Sunday since Ben had to go to work at his office instead of working from home.

On Tuesday I had a 504, IEP, and transition meeting at the school. I’m so grateful that I can trust this school, because while I was trying really hard, and I had made sure that I wasn’t currently on the meds, I was in so much pain I couldn’t concentrate on a thing they said.

On Thursday Dallin was riding to school on the bus with his nurse when all of a sudden he passed out. She tested his bg a couple of times- he was fine. She tried tickling him to wake him up, a sternum rub, everything. When the bus finally pulled up to the school about 5 minutes later, she and the driver carried him down the steps of the bus, and then into the nurses office. Right after she laid him on the couch, he woke up and was totally fine. Now, some of you might think that maybe he was faking it, but that kid can’t go 5 seconds without smiling if he’s pulling a prank, and there’s no way he would “normally” go from wide awake to asleep and not wake up when he’s being carried around. He’d at least open his eyes for a moment.

Of course, this is the day that I’d decided to take a bath to see if that would help my back, and I missed the call from the school. They got a hold of Ben, and he was able to rush home, terrified for Dallin, and scared for why I wasn’t answering. I had/ have massive guilt for missing something so important!

I called Dr. Weinstein, but since blood sugars were taken several times, and the glucometer was definitely working correctly (sometimes it pays to have multiple kids that need bgs tested), it really wasn’t his area. Out pediatrician just said that “These things happen sometimes, and we never know why, but they never happen again. If there’s another problem, we’ll run some tests.” So far, thankfully, it hasn’t happened again.

After a couple weeks of not being able to stand for more than 10  minutes at a time, I finally caved and went to another doctor. To sum up, I ended up getting a CT scan to make really sure that I didn’t re-break anything, and I had to see him/ the physical therapist twice a week for a few months. After about a month, I broke down and started taking the opium he prescribed, and I really did get better faster after that. I’m pretty much back to normal now, unless I overdo it. But I’m not so sure my once beautifully organized house will ever recover from me not keeping up all summer.

Now we’re on to dealing with the bills. Did you know that if you get in a car accident in the great state of Florida, that the insurance companies are only required to pay 80% of the bills- even if it wasn’t your fault. The teenager who hit us had Geico, we payed extra for Hertz, and we have our personal insurance that we could dip into, but then our rates would probably go up. Yay.

There. So much for keeping it short. Oh well, now we can move on to the fun stuff.

I now know why my mother never bothered getting in the pool when she took us swimming. After 5 minutes, kido #3 had to go back inside for the bathroom. Lucky for us we were on the first floor. By the time I got back outside, #4 had to go in. When we came back out it was time to test BGs. Most were doing well, and only one needed to eat carbs. By the time that was divied out, Ethan decided to do a cannonball. SPLASH. So much for me not getting wet. When he came up for air, he let us know that his g-tube balloon had burst. Seriously?!?! Ok, back inside to replace a g-tube.

This is the first Florida trip where we’ve had to replace a tube, so I’m really glad I still packed those bulky boxes. Unfortunately, I didn’t remember to pack the KY jelly, that makes it slide in smoothly. He decided (with just a little reasoning/ bribery) to let me replace it without the jelly. He was so brave! It took a little longer, there was a little blood, but he didn’t cry at all, and was able to calm himself down, even when it hurt.

After that, he was raring to go swimming again (which was the bribe), and Ben and the boys were still going strong. A few more bg tests, and it was time for showers and cartoons so mom and dad didn’t pass out from exhaustion.

Dr. W. came by the hotel to drop off our discharge paperwork, (I know, personally delivered, what other doctor would ever do that???) We told him about the g-tube incident and his response was something like ‘Really??? Well, you’ve had enough go wrong in one trip, the rest should be a breeze.’ If I were superstitious, I’d swear he jinxed us.

Dinner was fun because I got to use my new dietary guideline book at the Olive Garden (my favorite place to eat). It was shocking to see how much more I’d been giving the boys than they needed at restaurants. The Olive Garden was incredibly accomodating. They had new “healthier” kid menus they were testing out, and the manager came over to help us with nutrition facts, and actually showed interest in GSD. Kinda neat.

After that we went to the Bat House, and finally back to the hotel to pack. The next day we wanted to take the boys to the La Chua Trail (which was the highlight of last year’s trip), but the stress was catching up to us, so we slept in instead. After breakfast it was time to start our trip back to Orlando to catch our flight home.

Lucky for us, we decided to leave 3 hours before we needed to be at the airport, even though it’s only a 2 hour drive.

Dallin:

Cholesterol (should be less than 170)                                    2011: 144
Triglycerides (should be less than 200)                                2011: 119

LDL (bad cholesterol should be less than 100-110)            2011: 76

HDL (good Cholesterol should be above 45)                        2011: 42
Uric Acid (not sure of normal levels)                                     2011: 4.6

AST/ALT (Measures liver damage- “normal” between 5-60)

AST (should be between 5-60)                                                 2011: 53

ALT  (should be between 5-60)                                                2011: 23

Liver:                                                                                 2011: 2.9cm below costal margin

Jonny:

Cholesterol (should be less than 170)                                    2011: 128
Triglycerides (should be less than 200)                                2011: 81

LDL (bad cholesterol should be less than 100-110)            2011: 52

HDL (good Cholesterol should be above 45)                        2011: 53
Uric Acid (not sure of normal levels)                                     2011: 4.6

AST/ALT (Measures liver damage- “normal” between 5-60)

AST (should be between 5-60)                                                 2011: 54

ALT  (should be between 5-60)                                                2011: 20

Liver:                                                                                 2011: 3.4cm below costal margin

Ethan:

Cholesterol (should be less than 170)                                    2011: 107
Triglycerides (should be less than 200)                                2011: 127

LDL (bad cholesterol should be less than 100-110)            2011: 36

HDL (good Cholesterol should be above 45)                        2011: 44
Uric Acid (not sure of normal levels)                                     2011: 5.4

AST/ALT (Measures liver damage- “normal” between 5-60)

AST (should be between 5-60)                                                 2011: 72

ALT  (should be between 5-60)                                                2011: 24

Liver:                                                                                 2011: 2.9cm below costal margin

Mark:

Cholesterol (should be less than 170)                                    2011: 117
Triglycerides (should be less than 200)                                2011: 112

LDL (bad cholesterol should be less than 100-110)            2011: 48

HDL (good Cholesterol should be above 45)                        2011: 44
Uric Acid (not sure of normal levels)                                     2011: 4.2

AST/ALT (Measures liver damage- “normal” between 5-60)

AST (should be between 5-60)                                                 2011: 78

ALT  (should be between 5-60)                                                2011: 28

Liver:                                                                                 2011: 3.1cm below costal margin

All of them had slightly high Vitamin D and Iron levels, so we’re reducing their supplements.

There is a bit of a hiccup though. These tests are the results of the blood drawn in Idaho before seeing Dr. Weinstein. So, they might be a little skewed. I’m trying to remember exactly what Dr. W. said, but it was something to the effect of different hospitals use slightly different tests, so the results at one hospital are not necessarily the EXACT results that you would get at a different hospital. The “normal” ranges are listed differently in each of the hospital report. I couldn’t find my copy with Idaho’s “normal ranges”, so I just copied the normals I had for Shands. It made perfect sense when he explained it, but I’m having trouble remembering it correctly. If someone else knows exactly what he’s talking about, please clear it up for us.  Anyway, this year’s results will look slightly higher than they probably would have if taken at Shands. Plus, you have to remember they were just getting sick when the blood was drawn for these results, so that puts them a little out of whack too.

But, since they’re all still in the “normal” range, I’m not allowed to freak out.  Abdominal ultrasounds for Mark and Ethan were done in Idaho right before blood draws, and came back normal.

Anyway, we gave her the diet chart (filled to the best of our ability- I’m pretty burned out when it comes to those- thankfully, the nurses keep good notes) and she was able to tell almost immediately what we needed to change. We’re going to be working on limiting carbs for our “love it” and “like it” eaters. Their body types just won’t sustain a “normal” food intake in addition to the many calories that cornstarch gives them during the day. The good news is that even though they’re on a diet, we don’t have to count calories, only carbs. That’s not too bad, since we do that all the time to maintain blood sugars anyway.

My favorite quality in the new dietitian, is that she works closely with the GSD patient (not just the parent) to empower and have them take control of what they’re eating, and how much. She has really cool posters and handouts on “portion distortion” etc. that she uses to explain concepts. I really appreciated the “This is what needs to happen so that you can be healthy” matter of fact approach. It would have been easier for her to just give me the guidelines to take home, and have it turn into a “Mom and Dad are restricting my food” moment. We had a lot of questions answered, and it felt like we really came away with a lot of good information, and areas to improve on, without playing the blame game, or any additional guilt.

Let me be clear that I’ve never felt “guilted” at Dr. Weinstein’s hospital, but we have experienced that problem with other dietitians pre GSD diagnosis. I really liked Dr. W.’s former dietitian, but Kathy is on fire. I love how invested she is in the program, and her plans to improve it.

Soon it was time for I.V.s. Jonny did sooooo well. This year we opted not to do the numbing cream- it seems to make their veins smaller and harder to get. We took him into a separate room, and the nurses turned on Spongebob to distract him. Jonny thought this was fabulous because I HATE Spongebob with a passion, and never let them watch it, even if we had cable T.V., which we don’t. I’m pretty sure the fact that I don’t like it, is why he (and his brothers) are so obsessed.  Anyway, he was so excited that he didn’t even flinch, let alone wiggle and cry when they placed his I.V. He did so well, I wish I’d recorded it, and I’m even grateful to the annoying cartoon.

Dallin went next. Sadly, Spongebob was mid commercial break, so he wasn’t quite as distracted. Ben grabbed the camcorder, and he did really well. He sounded a bit like a monkey, but he was able to calm himself down, and it only took one poke. I’m so proud of my brave boys, and I really appreciate the amazing nurses at Shands. They always take their time to make sure the kids are as comfortable, and unafraid as possible.

The rest of the day was probably the most relaxing we’ve ever had in a hospital visit. The boys got to play way too much wii (more than 20 min. a day makes them crabby), have a pillow fight with Dr. Weinstein, and they were even able to paint a ceiling tile to put up in the unit. They were so excited about that!  There were also plenty of times that Ethan “challenged” Dr. Weinstein to a game of fussball. Dr. W. would always be winning by 8 or 9 points, when Ethan would make a fantastic recovery and score 10 times in a row for the win. Imagine that. 

And, of course, there was the typical “Who wants a present???”

Jonny loves anything Toy Story!

One of Dallin’s toys was a spider. He thought it was hilarious to put it on his spider bite scar. Boys. I’ll never understand them.

The boys were excited because there was a family using a Make a Wish, and the captain let them all see the cockpit after we landed. Ben and I were excited to get a “swagger wagon” as our rental van. It had a camera for when backing up, and a lot more room than we’re used to. We’re spoiled now.

Then, we ended up getting a little lost in Orlando trying to find a place to eat. It was late, so most places were closed. We finally found a Chile’s, and I spent most of the time in the bathroom while Ben had to deal with four slightly crabby kids who had been on an airplane ALL day. They were trying, but it had been a long day. Our waiter was fantastic though, that always helps. After leaving the parking lot for the second time (we had to go back because I forgot my purse, again) it was time to move on to Gainesville. Very luckily the rental van came with a GPS that we didn’t have to pay extra for. We usually use the GPS on Ben’s phone, but it isn’t the most accurate. There was no way I was feeling well enough to help navigate.

By the time we got to Gainesville it was about 1am local time. Right after we got there a HUGE storm hit. Normally, that’s my favorite kind of weather, but the thunder was so loud it kept us up most of the night. We found out later that it knocked out power to a lot of places. Ben made me try to sleep while he did 2am and 6am cornstarch alone. I love him.

Fortunately I’m getting better at packing, and finally figured out that it’s much easier to pack the kids’ clothes in individual backpacks, and then put all of the backpacks in one huge suitcase. That way we get the best of both worlds. We aren’t lugging a ton of different suitcases/backpacks through the airport, and we can just grab and go for the two being admitted to the hospital in the morning. It worked well. Also, folding each outfit together helped getting out the door much faster. Even with all that it was about 10:15 local time before we made it to admissions.  I hate being late.

I’d saved packing for Thursday, but wouldn’t you know it, at 2am cornstarch on Thursday, Jonny decided to get sick and throw up all over the bathroom floor. Could have been worse. Ethan followed close behind at 3am, but he didn’t make it to the bathroom. Ben spent an hour that morning shopvac-ing Ethan and Mark’s bedroom, while I was steam cleaning the floors, walls, closet door and toilet in the little boys’ bathroom. After that, we made them sleep on little mattresses in their respective bathrooms. Ethan’s bg was fine on his feeding pump, but Jonny had grown so much since his last visit that we couldn’t get his blood sugars stable. Eventually we had to call Dr. Weinstein, and get new dosages for his D20. Luckily he stabled out after that, but both of them were still throwing up the rest of the morning. We were able to wean Ethan off that night, but by then Dallin’s tummy was hurting pretty bad, so we decided to hook him up to the D20 feeding pump before it got worse. (For those of you wondering, Tolerex is better than D20, but we can’t find a medical supplier who will get Tolerex for us in Idaho, so we mix D20 as per Dr. Weinstein).

At this point, Jonny was still vomiting so we had him sleep on a crib mattress in the bathroom again. Dallin wanted to be with him, so he slept (still hooked up to the pump) in a bathtub full of blankets and pillows. According to him he was very “comfy cosy”, and I’m all about easy clean up.

This was our first adventure with a feeding pump with Dallin’s new Bard button. Our nutriport continuous feeding tubes come with a little clippy thing that holds the extension tube to the button, even if the kid tosses and turns. It’s still tricky holding the extension tube to the pump bag tube, but that’s nothing compared to trying to keep it attached to the Bard button. I ended up pulling a McGyver, and taking the little clippy part off a nutriport, and putting it on the BARD. Tricky, and it didn’t fit as well, but it did the job for one night.

Thankfully, both boys were off the pump Saturday about noon. We were worried Mark would wait until we got on the plane to catch it, but Saturday evening he had a fever of 103, and was feeling crummy. We hooked him up (he thought sleeping in the bathtub was totally awesome), and while he never threw up, his heart rate was racing so fast it was hard to count how many bpm. Eventually it woke him up, and he was so scared about it we gave Dr. Weinstein another call (around 1am EST). He wasn’t too concerned as long as his blood sugars were good. After a midnight run to the store to grab more motrin (I should start buying it by the gallon!) it was finally time for bed. His heart rate calmed down early in the morning, and by Sunday afternoon we were able to get him off the pump, but he still isn’t eating very well.

Sunday afternoon a fellow service dog raiser came to pick up Franklin. Within a couple of hours she sent me a text letting me know that Franky had just alerted to her father-in-law’s low blood sugar. That’s four times this week (on three different people)! Good dog.

Sunday night we spent scrambling to finish laundry, getting the house reasonably clean, and finishing up the packing. My awesome dad came down to spend the night and take us to the airport in the morning. We went to bed dead tired, but of course, I was so anxious about the trip, that I couldn’t sleep. Grrr!

This year we fly from Boise to Seattle, Washington (I know, wrong direction!) to Chicago, to Orlando, and then we drive the two hours to Gainesville. The fabulous part is that we’re flying Southwest (woohoo! No checked baggage charges), and even though we’ll be changing time zones five times, at least we get to stay on the same plane the whole time. Not starching x4 while running through the airport to catch a connecting flight is my idea of blissful cross country travel. Well, and the little ones can sit still for more than 10 minutes at a time now.

We’ll get to Gainesville about 11pm- 12am local time, depending on how long picking up the rental car and grabbing dinner to go takes. We’ll need to be at the hospital at 10am local time (8am our time). Have I ever mentioned I am NOT a morning person? At least we got through airport security in Boise in record time (considering we have four little boys and are bringing white powdered substances as well as liquids on board). It usually takes a lot longer, and we had about 45 minutes until it was time to board the plane. Which is how I had time to write this little blurb.