Friday, May 17, 2013

GI Update

The appointment at CCMC went well. Ollie was perfect to, from, and during his appointment. Car rides have been a real challenge for us in the past few months but he was a little angel on Wednesday.

We are not doing the change to a GJ tube yet. The doctor (and we) thought it was best to try to slow his feedings and to change some if his meds. He is now on a continuous feed from the time he wakes up until the time he goes to sleep. We also are trying a low dose of erythromycin to help his stomach empty. The past two days he's had no issues and his breathing sounds crystal clear. If he gets aspiration pneumonia again then we can try even more meds but may eventually have to go with the GJ tube.

He hasn't had any trouble keeping weight on. He's up to 33 pounds now and everyone who sees him comments on his length. He's our big boy now.

Here he is at school on Thursday.

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Tuesday, May 14, 2013

GI appointment tomorrow

We have an appointment at 11:45 with GI tomorrow to discuss options. A GJ tube isn't a reflux and aspiration pneumonia "cure-all", it comes with its own risks and complications. We need to talk to some experts before we decide what to do. We've asked other MLD parents and some have had great luck with a GJ and some think it's better to stick with a G because the GJ caused all kinds of other issues. We can also "try" a GJ and see how it goes and if it's no good, we can switch him back to a G. We might also be told to not even think about a GJ now and just put him on continuous feeds instead of 3 bolus feeds. He usually gets breakfast, lunch and dinner at a rate of 90ml(3oz)/hr for 2 hours, so 6oz 3x/day and it takes 6hours to feed him those 18oz. Except when he's sick, then we slow it down substantially and usually change the pediasure to pedialyte. Or dilute one or the other with water. But even if we slow down his feeds, he's still digesting slower than molasses. He threw up pediasure today more than 5 hours after he had any.

He's also so medically fragile that we're afraid of the Drs needing to use general anesthesia on him to switch in the GJ, but apparently some doctors insist on the anesthesia and some don't see a need for it. The procedure is described as "uncomfortable" and it needs to be done in radiology so they can make sure it's placed properly in the digestive tract, but it's done through his current Gtube site, so not real "surgery" or cutting required.

So much to think about.

I'm curious what we'll hear tomorrow at the appointment. Even at a Children's Hospital they don't often have children moving backwards in their ability to eat, from eating by mouth, to Gtube, to GJ tube and pretty much nobody in the hospital, except for the neurology department have ever heard of this rare disease. And the neurologists have only read about it in text books, he's the first case ever at this hospital.

The last time we saw a Dr. in GI she said to us as we walked out the door that we were doing a "great job with his feedings" and to "keep it up, one day he may not even need a Gtube anymore." Ugh. Say what? No amount of PT in the world can teach his muscles how to function again. This disease is progressive and hearing her say that made me even more sad and lonely.

This awful disease is a big mystery even to the doctors.
Oliver has been having trouble keeping down his formula and is could be causing some of his pneumonias. Chris has been on and off the phone with Connecticut Children's trying to talk to someone in GI. We're not sure what his next step will be, whether a medication to speed up his stomach motility or changing his Gtube to a GJ tube, or something else.We'd have to go in to the hospital for a GJ tube so we're waiting for a phone call back.

Thursday, May 9, 2013

Not again!

Ollie woke up with a fever of 103* with purple lips and nail beds. His pillow was covered in yellow mucus he must have hacked up during the middle of the night. We immediately did some lung therapy to bring up the gunk then suctioned it out of his nose and mouth so it wouldn't settle back down in his lungs. When enough yuck was up and out we started him on a breathing treatment to open up his lungs, followed by a few hours of oxygen.

It's 3:00 and the color is back in his cheeks and lips, but his lungs are crackly, it's pneumonia, again.

Luckily he's doing alright at the moment, very sleepy, but breathing sounds good, nothing like last week. We're not sure if this is a brand new pneumonia or some leftover bacteria that didn't get killed off last week and started to breed again when his antibiotic course was over but our plan of attack is still the same. Antibiotics, lung therapy, supplemental oxygen, breathing treatments, kisses and cuddles.

Friday, May 3, 2013

Oliver has had a rough 18 hours. Sorry, this post gets kind of yucky, don't read if you're eating, or easily grossed out..

He's had some major gastrointestinal issues, a painfully swollen abdomen was causing lots of leakage from his gtube site. A rainbow of colors coming out that we didn't know the human body could even make! We kept trying to "vent" some of the gas through the tube but stomach contents came out instead of the air bubbles. Gross? Yes, incredibly. (I have even more respect now for nurses that deal with the bodily fluids of complete strangers on a daily basis.) The gas was causing him a lot of discomfort and is possibly being caused by the antibiotic. MLD affects all the muscles in the body, even the smooth muscles of the intestinal tract so his digestion might just be getting more sluggish as a result of the MLD. There are medications to help this speed along a little more quickly, but he's likely going to need to have his G-tube switched to a GJ tube sometime in the future. G-Tube, GJ tube differences The GJ would go directly into his intestine and bypass the stomach. This could help prevent some cases of aspiration pneumonia caused by reflux of formula. But luckily, as of this morning the bloated belly is gone, as well as several towels that were poop victims. Forget washing, those went directly into the outdoor trash can!

We've been able to keep him semi-hydrated with a mixture of pedialyte and water but with all him tummy troubles we've had to put his pump on an incredibly slow rate, and with all the leaking and retching it's hard to tell how much he's actually getting. Enough I guess, he isn't showing any signs of dehydration. He hasn't had any formula since 4oz Monday morning and 4oz diluted on Wednesday afternoon(we had to stop that feeding because he started gag/vomiting)

As of yesterday his lungs sounded better, still crackly, but there is improvement. However, as he coughs it up he gags, and gags and vomits up the gunk as it gets stuck in the back of his throat. It's awful, but it's better out than in.

Fighting MLD is a messy business.