Luke's decannulation attempt

I am posting this because this because I would really appreciate knowing what other parents who have been in this situation think. It is not exactly as I remembered it. Luke does breathe, but seems unable to after his stoma is covered. I don't want to scare anyone with this. Just after the video stops the trach went right back in and his sats recovered immediately. He never got lower than 77 and that was only for a second.

The BIG day out

We're going for it. The trach is coming out tomorrow. We don't know how long it's going to stay out. It could be for only 10 minutes or less, could be an hour, could be until he goes down for his nap, could be out forever. We have no idea and neither does the ENT. But he's willing to let Luke have his shot at it. If it doesn't go well, then Candi and I have to decide whether or not he gets the LTR (laryngotracheal repair) which is where they take about one inch or so of a rib and put it in his trachea make his trachea larger. At the moment his trachea is about 50% the size of what it should be, so the doc thinks he might need one but he's also undecided on that so we have to wait to see how the trach coming out goes.
So we're a bit nervous about this, but he can only do what he can do. And if he needs the LTR then so be it. We're (obviously) hoping that he won't but getting prepared for the possibility. The repair won't be tomorrow but we'll find out one way or the other, it's scheduled for Jan 7.
So there we are, a day off work (yay, I deserve it) but it's going to be a tough day for everyone, especially Luke. Fingers crossed and anything else you want to cross will be greatly appreciated.
And yes, I'm the one taking the trach out regardless of what anyone else says.

Luke's First Step

Luke was supposed to have his sleep study tonight, but his nose has closed down and he can't handle being capped for more than an hour or so when asleep.
Surgery tomorrow: CA dilation. Hopefully we can convince them that the time to do a sleep study is a couple of weeks after the CA dilation, not a month and a half later.
He is also scheduled to have a 24 hr study on Monday and maybe he can do that still. If he can stand being capped for 24 hrs then the trach can come out.
Big News - Luke walked with his walker the length of the living room. Very exciting.
Biggest News - Luke was leaning against the recliner when he pushed himself off, stood for a half second and took his first step towards Candi. He was a bit off-balance but it was definitely a step. We were both there to see it. Naturally we are both ecstatic and perhaps a bit teary.

An update - Luke

Yes, yes, I know this is the reason why you're all here. Luke is ... Luke. Since the last blog entry, he's had a number of surgeries (the last being on Thursday) including a re-trach where he had to spend a whole week in hospital. He was bored, bored, bored by the end of that week and very glad to come home.
This last surgery he went for a regular CA dilation and the plan was for the doc to take a look at his tonsils and take them out if he thought it would help Luke in breathing. We now have a little boy who has no tonsils. He still seems to be in some pain though. Plus he's been throwing up and I can't imagine how painful that must be for him - long live hydrocodone. He always seems to come out of hospital with a cold or some such malady and it's is no different this time. Which isn't helping matters. But he'll get over it.
He's now cruising quite well and seems to like standing. And climbing!! He climbs everything. He's fallen down the stairs twice and if he sees the gate open he rushes to get upstairs. He climbs onto the recliners and loves to stand up in them and rock the chair. He also loves wiping his runny nose (and it is always runny) on the nearest object - our shirts, the couch, the cloth books of his. In fact everything but the tissue we have in our hands. If we wipe his nose he is not happy and pushes our hands away.
He's also started to show some interest in clapping his own hands - he's been clapping our hands for months now. But we seen him do it himself a few times now and hopefully he'll start clapping to show he's excited or happy instead of biting his index fingers. His fingers have callouses on them where he's bitten them so much and we're hoping he starts to like clapping.
Despite everything he's gone through, he's still amazingly happy. If it was me going through all this, I would have kicked the doctor in the balls by now. But he's realized that going to the hospital means pain and the last two surgeries he's cried when going into the prep room. Candi said he tried to stay in the stroller by pushing the tray back down after she lifted it up, and then he held onto the sides of the stroller so he couldn't be picked up. And yet he almost always has a great big smile on his face. Even now with his throat and nose hurting, he's mostly happy. And his smile just makes our life a little easier.
I'm sure I've missed a whole bunch of stuff but that's just me getting older and more forgetful.
I'll try to post more as it comes up.

Luke's first day at The Bell Center this year - he likes his locker.

An update - Candi and I

Well what can I say. We're still the same: tired from lack of sleep; stressed about all the stuff dealing with Luke's issues; worried about what the next course of action is; proud of everything Sam, Gus and Luke do; sick of being tired, stressed and worried; happy that all 3 boys are smiley, joyful children; excited when any of the boys do something new and unexpected.
We haven't changed at all. :)
So this summer (winter to you in the southern hemisphere), the end of July in fact, we all went to Chicago for the 9th annual Worldwide Conference on CHARGE Syndrome. And by we, I mean all 6 of us: us 5 and Candi's mum (yes mum, not mom. I don't say mom so deal with it :) ) Well that was a roller-coaster of emotions. You go from being excited to happy to emotional to ... just name it and we experienced it. And it's all packed into 3 days. But we learned a lot, we were inspired by other people's stories and we met a whole bunch of people that we knew only through blog posts. It was wonderful to finally meet them all in person see how wonderful all of them are. There were some that couldn't make it but we fully expect to see them at the next conference, July 2011 in Orlando. :) I'm sure we'll be there the week before spending a few magical days in DisneyWorld.

An update - Sam and Gus

Well, after a month or 9, I've now hijacked this blog. Muahahaahaha. We're all doing fine, pretty much the same old, same old. Where to begin? Let's see ... Sam and Gus. They're doing brilliantly in school (2nd grade now) with reading and math above average. They're now Wolf Cub Scouts and still loving it. The 3 of us spent a night on the USS Alabama with our pack and 3 other packs. That was very cool. It really makes you respect the armed forces and what they had to endure 70 years ago. The cots were very small and triple bunked, held together with chains. 60 berths per room. And then they were at war as well. It certainly made me appreciate what I can only imagine what they went through. We had access to most of the ship and spent hours and hours going up and down steep ladders trying to find all that we could. It literally is a floating city. Then there was the submarine. I just can't imagine what it was like during war in that vessel. The boys were so tired that by 8pm they were begging to go to bed. That doesn't happen too often. The scouts are the only people that are allowed to spend the night on the Alabama, so we couldn't pass that one up. And next weekend we spend 2 nights camping out some caverns north of the city. That's just for our pack. We'll be learning knots and a bunch of other scouting stuff.

Surgery postponed

The urology surgery that Luke was supposed to have today has been rescheduled for April 10th. Luke has to take antibiotics right now (because the stints are in his nose) and they have given him terrible bm's and diaper rash. Neither would be good for a urology surgery.

Otherwise, all 3 boys are doing well. Sam had tonsilitis last weekend, but he is doing better now. Luckily no one else got sick. But Sam had to miss the Cub Scout Pinewood Derby car race. The Scouts let Gus race for him, though. Sam came in 4th and Gus came in 5th in their den. We also had to miss a surprise 50th Birthday party for my brother Don. The boys were really sad about that. They love Uncle Don; he is a great guy.

An unexpected visit to the OR

Don't worry, everything is fine. But Luke and I got to spend a long day at Children's yesterday. His nasal stints had come forward and were pressing horribly on the front of his nose. Since he is so little and the stints are sutured in they had to give him anesthesia to shorten the stints and fix the problem. Luke does seem to feel better today, so I am really glad we got that taken care of.

Luke is on antibiotics right now since he has "a foreign body" in his nose and it is giving him awful poo. So, we are going to have to reschedule his surgery with urology next week. I hope they can fit him in sometime in April. Our schedule for March just got a lot less crowded since the stints mean we have to postpone Luke's VitalStim therapy until they come out. We are adding something new though. Luke is going to have Audio Verbal Therapy at the HEAR Center (at Children's) every other week. We really need to work on Luke's receptive language, and it will be good to know what the AVT therapist thinks about how he is hearing.

We're home

Luke is eating and snoozing in the other room, so I thought I would give you guys an update. Luke's choanal atresia repair went really well. His nose is finally open - really open. The surgeons were pleased and thought it might actually take this time. Dr. Hill (who is quite an experienced ENT) said it is the most difficult CA repair he has ever done, but that he finally has a good feeling about it this time. Luke's poor little nose is swollen and still draining, but he seems to have started feeling better. The whole time at the hospital he just laid on his back in the crib. He smiled a few times, but was not himself. Then just before we left I got him dressed and that was his cue to get back to normal. He turned over and crawled in the crib and pulled up on the side. Then in his stroller he was bopping around and flirting like usual.

The not so good news from yesterday is that Dr. Hill was not able to remove the granuloma from inside Luke's airway. We didn't even know that was an option. So now the only way to get it out is to do an open procedure (basically opening his trachea and then redoing the stoma like it is a fresh trach) or removing it when doing a Laryngotracheal Reconstruction (LTR). One of these should happen on May 7th. With the first option Luke would have to stay in the hospital for a week as his new stoma starts to heal. Then after he went home we would try capping and seeing if he is ready for his trach to come out. If the capping doesn't work or Dr. Hill decides on the 7th that reconstruction is necessary, a single stage LTR will be performed. This is a pretty major surgery in which a cartilage graft is taken from Luke's ribs and inserted in his trachea to enlarge it and stabilize the area that the trach stoma is in. He would be mainly sedated for a week and be in the hospital for a few weeks (I think). The main benefits of the first procedure are that it is much less invasive and removing the trach should be less traumatic (for all of us). The main benefit of the single stage LTR is that the trach would come out with the surgery, a much faster process. But basically it comes down to whatever Dr. Hill decides is the best choice for Luke. Whichever way it goes, Luke has a good chance of being trach-free sometime this year.

We were surprised yesterday to find that Luke had a sedated hearing test scheduled. And then we were even more suprised by the results. Despite the fact that Luke has seemed to respond more and more to sounds, both with and without his hearing aid. The hearing test results weren't very good. The test showed an 80 db loss in right ear and a 90+ db loss in his left ear (both severe losses) unaided. And the biggest surprise - it showed a 60 db loss even with the bone conduction aid. As far as we were aware, Luke had normal hearing with his bone conduction aid. We are trying not to get too worried and go more with what we see Luke doing than the results of this one test. No matter what any of Luke's tests show, he is going to develop some form of communication. We just need to figure out what is going to work best for him. The test is going to be repeated when Luke's stints are removed in 6 weeks, so lets just hope these current results are an anomaly.

Here is Luke's upcoming surgery schedule:

February 26: Third and final orchiopexy surgery with urology
March 26: Stint removal and hearing test
May 7: Granuloma removal, checking Luke's nose to see if it is still open, and possible LTR

And in the midst of all that are the boys' birthdays. Luke will be 2 next Tuesday the 17th. And Sam & Gus turn 7 on April 30th. I promise to keep you all updated about what is happening. I will upload some photos too, as soon as I can get Rob to install the software that came with our new camera.

Luke is still keeping busy with VitalStim and his Bell Center and ARC therapies. And Sam & Gus are really enjoying school and Cub Scouts. (I have to post a photo of them in their uniforms - they look so cute!). So it will be a busy Spring just like last year.

Luke's surgery tomorrow

In this much overdue post, I just wanted to let you know that Luke is having surgery tomorrow. It is with ENT - a choanal revision (basically a redo of his initial choanal repair). This time Dr. Hill is going to take away the back part of the septum in the hope that Luke's nose will finally stay open. Luke is going to get stints for 6 weeks again. Both times he has had them for the 6 weeks which included his birthday (Luke turns 2 on the 17th). Dr. Hill is also going to laser away the granuloma inside Luke's trachea near the trach stoma. This should allow Luke to wear his PMV and babble again. We are expecting Luke to stay in over night, but hopefully not longer. It should be fine and pretty routine, but my nerves are acting up anyway. You would think this would get easier, but I think it gets harder in some ways.

We will try to update tomorrow or Saturday at the latest about how everything went. Until then, your positive thoughts and support would help a lot! Thanks!