Ounces still packing on

Gwen is continuing to gain weight right on schedule. We're up to 12 pounds, 12.5 ounces.

In other news, Apple finally put back the old "homepage" functionality, so a few new pictures are up.

EMG went fine

Today was Gwen's Electromyography, and things went fine. It was a pretty brief outpatient procedure, and we were home before 4pm. Gwen was out-of-sorts the rest of the day, but understandable and well within medical norms. So we'll see if she's back to her chipper self tomorrow.

By the way, there are lots of new pictures to share, but Apple "upgraded" the mac.com functionality to their new "me.com" site. This has left me unable to post new pictures or edit my existing photo pages in any way. Apparently the "upgrade" was to benefit the iPhone users, and they are very, very slow in returning the existing functionality to the rest of us. Sigh, Apple used to really care about customer service and getting things right...

Dane County Fair

Evie went to the fair this past Saturday and Sunday.

While she really enjoyed seeing all the animals, she is most definitely NOT a fan of being close to a horse, and if it neighs, there will be a shockwave of terror. Petting the bunnies went much better!

Hybridfest was also at the fair - most cool. While I'm not about to drive the Prius to Minneapolis and drop $10k on a plug-in upgrade, it was great talking with Hymotion, and seeing how nicely they have packaged the plug-in mod. Don't get me wrong - I'd love to do it, but can think of a couple of college funds that have greater need for those bucks. Plus, it'll give me a chance to post these two cool Opus toons!

Soon, they'll recognize me

With Gwen's EMG on Thursday of this week, we're on heightened alert for any sign of illness. Evie had some coughing fits, so she got checked out and got a clean bill of health. Perhaps it was a little virus that she's successfully fending off, perhaps it's some type of seasonal allergy developing - not sure.

Of course, that couldn't be our only scare. Monday afternoon Gwen started to not be herself, including a low-grade fever and some slightly labored breathing. So by 6pm we were at the doctor's office, and the answer was that things seemed fine, but the heavy breathing could be better evaluated by the ER. So around 7pm we were back in the ER. Blood sample, chest x-ray and a small cadre of doctors later, there was no definitive cause identified. Since pneumonia and other nasties could take another day to bloom, we were admitted to the children's hospital, again. Around 1:30am we were asleep in our room, and the day began about 6:30am.

Many, many medical folk later, the whole episode was written off as "well, she sure seemed to have had something, but fought it off". Around 6pm on Tuesday, we were all home again.

If we have any more episodes like this, they're going to have a personalized mug of coffee waiting for me on our next arrival, a-la Home Improvement.

A matter of perspective

Gwen is so tiny next to Evie, but here she is next to her newest cousin, Lydia. Keep in mind that Lydia is growing like a weed and already 45% over her birthweight. You've got a good lead Gwen, but Lydia is catching up quick!

When you're not even 3 months...

...how do you effectively communicate the fact that you're tired? By sleeping almost 14 hours. Seems that mom and dad aren't the only people around here exhausted by this week's events. Gwen fell asleep around 8pm and had the continuous feeding till 8am, and slept almost to 10am. Many lessons have been learned, like we'll need intra-night diaper changing, nuances on dealing with and resetting the pump when you run out of formula before you expected to, you shouldn't forget to unclamp the tube after refilling the bag or else the pump's alarm will wake you up as soon as you fall soundly asleep, finding out that a 4-ounce bottle can be less than 4 ounces and the pump's alarm will wake you up to reinforce that, we need a better "nest" to keep Gwen in place on an inclined mattress, and during manual feeding you have to be really careful to keep the syringe attached to the tube or else you've got some cleanup to do.

Evie, of course, came to the rescue at 6:30am today to make sure that mom and dad didn't waste daylight by catching up on sleep. Oy!

First night is going well so far

Gwen went onto the pump around 8pm, so she's getting a continuous feed of one ounce per hour till morning. Since the formula can't sit out too long, we replenish the supply every 4 hours. At the moment she's had 80 of the 120 ml in the current bag, so replenishments should be at midnight and 4am. She's sleeping peacefully - so far, so good. As a bonus, Evie was pretty tired too and didn't fuss too much about bedtime.

We're home!

We got home around 2pm. Would have been sooner, but we stopped for lunch and went to Gwen's regular doctor for a quick shot of rotavirus vaccine. Since it's normally administered orally, so with the feeding tube the vaccine was just squirted right in. The entire doctor's appointment, including waiting, was five minutes. Guinness has been alerted.

All's well, and Gwen's gotten her first G-tube meal at home. Since we were used to the old NG-tube, the new method is a bit awkward, but I'm sure we'll gain proficiency soon.

Heading home soon

It's 11:20am, and we just finished Gwen's first bolus (through a syringe, whether gravity or plunger, rather than through the pump) feeding. This is how she'll be fed during the day, and we're supposed to receive a pump for night feedings. We got training on the use and care of her new tube, and discussed how many calories she should be getting per day - which is more than previous, so open wide, Gwen!

Our pediatric neurologist stopped by. He had reviewed the MRI and did not see anything unusual, so Gwen's brain development got a clean bill of health. He also had the biggest entourage of any of the doctors, with 4 observers.

Discharge is imminent, they're working on the paperwork right now.

We are doing so great...

...that it looks like we'll be going home around lunchtime today.

We just had a visit by our GI surgeon, and she was very pleased with Gwen's progress. She was taken off of IV fluids during the night, and we're just about to stop the continuous feeding. That means she'll be back to her normal schedule of feedings, but we will have the continuous feedings going on through the nights for a while.

Right now we're just verifying that her monitors and IV port can be removed, and when that happens, the feeding tube will be the only tube/wire left. That is sure to increase Gwen's happiness, as nobody likes to be tethered to machines.

She's been in such a good mood that I'm sure it's joy at being free from the NG-tube.

If you're happy and you know it...

...wake up dad!

It's 4:20am and Gwen is falling back asleep. She sprang awake about 3:30 and has been completely bright eyed and active. Cooing, flailing the arms, and looking for a pinky to suck on - she's as awake as can be, so we had a good (albeit one-sided) conversation. It's great to see her so active and happy that it's worth it to be up in the middle of the night.

The feeding pump was changed from pedialyte to regular formula at 2am, so Gwen's back to her normal food. We're still on the continuous 30 ml/hour feed, but that will change to regular feedings about 8 this morning. Medication-wise, she's still on Tylenol for post-op soreness, but she still shows no sign of needing anything stronger, so it really is a very good recovery. The surgeon even said that she'd more likely to have soreness in her leg (muscle biopsy) than the G-tube in her stomach.

Well, back to sleep, lulled by thunder and flashes of lightening.

Awake and doin' great

Gwen is really doing well. Right now she's thrashing about, without any signs of distress - so she's just a happy baby expressing herself. She's on a little bit of Tylenol right now, so I'm sure that helps - but not needing anything heavier after having had surgery is a really great sign. Feeding-wise, she's getting 30ml/hr in a slow, constant drip. That's her normal daily intake, but she's getting pedialyte constantly instead of formula in meals. So, it looks like the new tube is working out just fine, and her stomach isn't too bothered by the day's activity.

We don't yet know when Gwen will be discharged, as it all depends on how she does through the night and tomorrow. We are expecting it'll be tomorrow sometime, but no guarantees yet.

How are all those tests going?

We had a visit from Gwen's genetic/metabolic specialist at 3pm. He and the radiologist have looked at the MRI and did not see anything abnormal, so that is good news. The neurologist hasn't had a chance to inspect it yet, so the final word is not in.

So, what do we know about what's causing the hypotonia?
SMA was ruled out previously. Prader-Willi was ruled out by recent blood work. Myotonic dystrophy is currently being checked for with the latest blood work. All other metabolic disorder checks performed so far have come back negative.

The muscle biopsy that was taken today will result in a few different tests. First, there are several myopathies that are checked for. If those are negative, the mitochondria are inspected under an electron microscope. (there were no elevated lactate levels in her blood, so the chances of a mitochondrion problem is very slight)
Then a further look at the mitochondria will be done by an outside lab, and can be a couple of months before we get that back.

There are some very rare conditions that can be checked for should all other test be exhausted without a diagnosis, so this odyssey may continue for a while yet.

One course of action will be to start taking Carnitine, a vitamin. This has shown to increase muscle tone, but exactly how it works is not known. This is something we didn't want to start on until after the muscle biopsy was done, so the regimen can begin in a couple of days when the surgical recovery is complete.

We're in the room

At 11:50 we were taken to the recovery area, and Gwen was there wide awake, and had already charmed everyone she met. She was on very light oxygen, and received a little morphine because she was frowning a bit. From there we all went into room 8 in the pediatric intensive care unit, somewhere around 1pm. We don't need the picu, but this was the room that was available.

Gwen is singing up a storm and wiggling around, displaying a remarkable recovery from the anesthesia. We can tell by her face that she's still feeling "off", but overall doing very well.

She's just now starting her first G-tube feeding, and she'll be on just pedialyte for the rest of the day. It will be pumped in at 10ml/hr for the first hour and 20ml/hr for the next hour, just to start off very slowly. Tomorrow at 9am we'll get the full lesson on how to feed her and care for the new tube.

The plan is that she'll be able to go home tomorrow, but that is TBD, depending on how the rest of the day goes.

All surgeries done

The other surgeon visited us at 11:20 to say that the biopsy is complete, and all is well. No word on when we'll see Gwen in the recovery room, but we're pretty anxious about it, as we expected to see her almost 2 hours ago.

The tube is in

Gwen's surgeon just gave us an update. The tube has been successfully inserted, the muscle biopsy is underway, and Gwen is doing just great. We even got some inside-the-tummy pictures, but don't look for me to post those. ;-)

The procedure went just as expected, and Gwen will be eating through her new tube at 2pm. To give you an idea of how small the surgery is, the 2 incisions for tube are around 1/2 centimeter each, and the biopsy site on her leg is just under one centimeter.

Just going into surgery now

9:20am and we just got an update. Gwen has been transferred into the OR for the G-tube insertion and muscle biopsy. They've decided to take the muscle sample out of her leg, rather than the stomach incision point, as it is less prone to problems and easier to recover from.

We asked what the cause of the delay was, as she was expected to be in the OR by 8:30, but there was no knowledge as to why the MRI took so much longer than expected.

Procedures in progress

It's 8:50am and we're sitting in the surgical waiting area, where we've been for around an hour. We met with lots of docs and nurses, and spent the most time with the anesthesiologists. There's two separate teams that will be working with Gwen, with a couple of folk that will be with her the whole time. The first team is the MRI folk, and that's due to be completed any minute now. Once that's complete, they'll transfer Gwen to the OR for the second team, and they'll insert the G-tube and remove the muscle sample for the biopsy.

Around 7am, Gwen let us know in no uncertain terms that she was hungry. Her cries and sounds are normally quite dainty, so while the crying may not have set any decibel level, they were very assertive for her. After about 10 minutes of that, she decided to give up and took a nap. She'll be getting IV fluids soon, so that'll take care of the blood sugar. We also expect that she'll be able to start eating through the new G-tube today, as early as 4 hours after the surgery.

So, nothing particularly interesting to report, other that things are going (for us) just as we expected. Just waiting for our beeper to go off, letting us know that there's news.

All systems are go

Gwen's surgery is in the morning, wish us well.

Evie tried to complicate matters this week, and might yet succeed. She was home with a fever the last two days, but we've minimized interaction between the girls, and Gwen does not appear to catch whatever Evie had/has.

Update: Gwen's last feeding through the NG-tube was 1am on 7/10. That means that after the feeding, we removed the tube. Always a happy event, as Gwen is simply a happier kid when there's no tube taped to her face and shoved up her nose.

Them thar medical types

Another question we get is what kind of doctors and other medical folk Gwen is seeing. Well, it started with about a million visits to her pediatrician while we were closely monitoring her weight and muscle development. We've seen a pediatric neurologist, genetics/metabolism specialist, pediatric GI, have a home-health nurse visit weekly, and periodic visits by physical therapy, occupational therapy (despite the fact that Gwen does not yet have a chosen occupation) and speech therapy (help develop mouth and facial strength to aid in eating). There was even an ER visit with a 2-day hospital stay for observation where we got to see about a dozen doctors and nurses. Last, and possibly least, we even have a dermatologist appointment coming up. We've had several blood draws, and cultures on urine and spinal fluid.

So, we're very familiar with our pediatrician's office and the UW Children's Hospital. Great kudos to our array of doctors, as they keep us informed without talking down to us or over our heads.

So, what's with the tube?

If you've seen the new pictures but haven't been clued in to the details, you're probably wondering what the deal is with the tube in Gwen's nose.

It's a feeding tube because Gwen is unable to generate enough suction to eat enough formula from a bottle, which also meant that breastfeeding was out of the question too. So, when it's feeding time, we do feed her with a special bottle, but she's only able to take 1/2 ounce or so orally, so the other 3 are gravity-fed through that tube.

In case you want the tube/feeding details - currently daytime feedings are every 3 hours, and the two nighttime feedings are 6 and 4 hours apart, more or less. Initially, we had to change the tube every few days - sometimes a medical-type did it, sometimes daddy did it. For a while, we had a special one-month tube, but Gwen managed to yank it out, and we couldn't get a replacement for it. No big deal, since this Thursday is the big tube event.

On July 10th, Gwen is having a gastric feeding tube installed. This is a surgical procedure requiring full anesthesia, so she'll have to stay overnight for observation and recovery. While she's out, she'll also have an MRI done to see if there's a neurological cause. She'll also have a small piece of muscle removed so a biopsy can be performed.

Now you're probably asking, "so, what's the problem"? Well, we don't know. Gwen has hypotonia, which simply means she has low muscle tone. So far they've eliminated some of the most common and most nasty potential causes, so testing continues. We may end up with a diagnosis, which will result in targeted treatment, but it is also quite possible that there will never be a diagnosis. Low muscle tone may just simply be a temporary condition that will go away, and that'll be that. In addition to the MRI and biopsy, there are some blood tests pending, and she'll also get an EMG test sometime to check the responses of her nerves.

So, really nothing to be overly worried or concerned about. After all, Gwen's growing, packing on weight and getting stronger every day. For now, that's aided by a feeding tube. Based on some of those smiling pictures we posted yesterday, Gwen's no worse for wear.

At long last - new pix are up

New pictures are up on both the Evie and Gwen sites, and this will be the last updating. From now on, there will be a combined site at homepage.mac.com/zadzilka/family, using the same password.