Wednesday Morning - A Josiah Update

Hey Guys

Two bigs things on the agenda for Josiah's day: we have the swallowing study at 2 PM and he's going for his stroller ride @ Noon! Yay!

At noon we're going to be accompanied by a nurse and all packed up with Josiah's emergency equipment so that we can take him on a little spin to enjoy the outdoors and visit our friends at Starbucks. He'll probably be able to last 30-45 minutes with all the craziness, but eh he might surprise his nurse and just chill for a long time.

At 2 PM we'll be heading down to radiology where will be having Josiah suck on a soother while Marie sneakly injects some milk-barium mix into his mouth through the soother. It will be interesting to see what he does! We're praying of course that he'll be able to handle it and swallow without any problems. Of course he just gag and puke it up, but hey God's in charge and we're content with that.

Yesterday Marie took the day to go back to Abbotsford. She did some visiting and a whole lot of house cleaning (she had to act on the motherly urge to clean something - it's been four months in Vancouver and she's going crazy!). I did a pretty good job staying with Josiah all day - he slept. He was a little upset that it was daddy giving him his bath and not mommy, but he settled down at the end. He was a real MONKEY with the trach tie changes - he would not lie still. He wasn't upset or crying, just really squirmy and was trying launch himself off the scale table. But we got it done! Further, I've spent a lot of time over the last couple of days getting his hand-mouth cordination going, and it's so cute watching him play with his soother and try to hold it in. He's also trying to figure out how he can suck on his hands (he hasn't figured it out yet that he can't have his hand in a fist!), and so excited in the process he compiles a whole lot of spit! Very cute.

To wrap, I noticed Heather (hello Heather, nice to meet you!) requested a summaritive history of Josiah's life and experiences thus far at BC Children's Hospital. For those who have joined Josiah's journey part way or what to have a refresher on what he has gone through, this portion is for you:

Josiah was born on February 16th, 16:16 at MSA Hospital in Abbotsford, BC. He was 5 weeks premature and weighed 5 pounds and 11.9 ounces.

Josiah had trouble breathing right from the womb and was put into a incubator almost immediately. We knew something was wrong, but that's it. The doctors observed quickly some physical abnormalities (6 toes on each foot, conjoined kidneys, rotated and low-set ears, and things wrong with his respiratory system to name a few). He was intubated (a tube put down his throat to breathe) a couple hours after he was born because he wasn't able to expel his CO2. At around 11 PM Friday Josiah and Marie were airlifted to BCCH. It was a few days later @ BCCH's where he was diagnosed with Pallister-Hall Syndrome a very rare sporatic genetic mutation (it happens without cause, and there may be as few as 100 recorded cases in the world). There was no indication through Marie's pregnancy that there was anything different with our child; there was a minor marker in regards to his heart, but it wasn't accompanied with anything and so it was dismissed.

Josiah was extubated that Sunday night. During his first week he breathed fine for 4-5 days before he had a 'bronch-scope' where his respiratory abnormalities were first observed: he has a short, bifid epiglotiss (split glottis) as well a narrow and floppy trachea (trachea malaysia - some of my medical terms might be misspelled!) After his bronch scope Josiah wasn't able to breathe on his own and was intubated again. He was given a few attempts over the next couple of months to breathe on his own and each time he failed to sustain himself. He also self-extubated approximately 7 times (got into an arched position and coughed hard so that the tube would come up partially, essentially briefly asphyxiating himself).

Josiah had a second and third bronch scope. His second showed a collection of abnormalities mentioned above, but the doctor didn't feel it was enough combined to prevent him from breathing so there was speculation that his inability to breathe was neurological. The third bronch scope revealed that his epiglottis would flop into the trachea, partially blocking off his airway, but enough to prevent him from getting the air he needs.

Josiah's MRI and EEG scan (both brain related) have revealed that he has abnormal brain activity and that his development and mental abilities are unclear and will be determined as he grows. His heart has been examined through three different tests because he goes bradycardic (heart rate drop dangerously low) in moments of great stress and anxiety, or whenever he is irritated at the bottom of trachea (for example, his ET tube when he had it in his throat would rub and irritate the inside of his trachea). But as far as the doctors can see, his heart is strong: there is one or two incidental things that are not perfect with his heart (and I'm sorry that I can't remember them right now) but they haven't caused him any problems. There was talk for a while that if his brady-s didn't stop, he would have to get a pacemaker!! Yikes!

What else is there? Hmmm ... Josiah for the longest time wouldn't swallow (would you blame him for not wanting to when he's got a tube in his mouth) but started doing so two months ago. He has a very strong set of lungs and is an overall extremely strong boy: there are babies, there are strong babies, and then there is Josiah - he's constantly catching his nurses off guard with his feats of strength!

We prayed hard that Josiah wouldn't need a trach and a g-tube, but God had other plans. Josiah received his trach around April 30th, and his g-tube about a week later (the trach is for breathing and the g-tube is for feeding - before he had his g-tube all his milk went through an NG tube through his nose into his stomach). Josiah was paralyzed for a week after his trach surgery so that the trach stoma would heal and set firm. Josiah didn't take long to get off his ventilator after he had his trach surgery and has been on regular room air for weeks.

There has been a few infections Josiah's had to fight off (with a trach, you don't have your nose filtering out all the germs and humidifying the air you breathe so you're more vulnerable to sickness), but he's doing very well. Every once in a while he'll have a panic attack (normal for infants with trachs) where he'll freak out because there's a moment that he can't breathe. If he is attended to and suctioned in time (we have to put a cathader through his trach to suck out the secretions that build up in his throat from having a foreign object - the trach - present) he recovers nicely and doesn't go bradycardic. If he is neglected (which is easy to do because he's in a private room and can't make any sounds of distres) than his heart drops because his throat tightens when he is stressed. He then doesn't get the air he needs and essentially goes limp and passes out. He needs to get beyond these panic attacks before he can go home.

Whew. That's as brief as I can make it folks, yet I'm certain that I've left key points out. If any of you faithful readers noticed any glaring omissions, let me know! :D

Oh, and an integral part of Josiah's journey: he is prayed for and loved by many! He's a worldwide celebrity (don't believe me - look at the map!) and is garnering support, prayer and encouragement by not only our friends, family and church, but also by many people who don't know us and have never met him. God is so good.

There you have it. I hope it's an encouragement and makes sense to those who have only recently got to know Josiah. Thanks to Troy & Trina U. dropping by yesterday.

In God's Love.
- Andrew, Marie & Josiah