Our Crazy Day!

Well, Luke and I are home, and he is doing well. Again, after much preparation and worry, Luke didn't have the Laryngotracheal Reconstruction (LTR). This is probably good news. As many of you know, Luke doesn't just have one airway issue, but many. He was trached at 6 days old because of his choanal atresia (CA) (his nose was completely blocked by bone). Then later it was discovered that he had tracheomalacia and laryngomalacia (floppiness of his trachea and larynx) and subglottic stenosis (a narrowing of his airway just below the glottic region). Since he already had the trach it was hard to know how much each of these issues affected his breathing because the trach bypassed them. So first, Luke's ENT, Dr. H., has been working on Luke's nose, which Dr. H. described as the worst case of CA he has ever treated. Luke has had more than a dozen surgeries on his nose - 2 major, the rest one-day. The good news today is that Luke's nose has stayed open and looks better than it ever has. We may finally be close to a resolution of that issue. Dr. H. did go ahead and dilate Luke's nose today since he was already in the OR.

So then we look at what else may be causing Luke's difficulty breathing. Until today every time Dr. H. has seen Luke's airway it was red and inflamed. Today there was some irritation, but it looked better than ever before. And with the reduction of inflammation Luke's subglottic stenosis was much better. It would still be on the small side, but not small enough for Luke to need the LTR to make his airway bigger. Another reason to have an LTR is if there is a collapse in the trachea caused by having a trach. This is what I thought happened when Luke's decannulation attempt went so badly. But Dr. H. didn't find any significant collapse when he did the scope today. So, as far as I understand, unless something new happens, Luke won't have an LTR at all.

If there isn't a collapse then we have to think about which of Luke's other issues could have caused the decan to fail the way it did. And we are left with the laryngomalacia (LM). Most of the time kids are able to outgrow LM without the need for a surgical repair or a trach. But when LM is severe the epiglottis can contract and cut off the airway. This wouldn't be constant and would be worse on the inhalation than the exhalation. Times it would be worst would be when upset and when lying on your back. With Luke it would explain why when he started crying hard during the decan his airway closed off (looking like a collapse), why when he is capped he does fine when he is awake (except when he is crying and obviously can't catch his breath), but when he is asleep (on his back) he has a noticeable occlusion - he can wear his PMV while asleep though with no problems (with this he is breathing in through his trach, rather than his mouth). LM is associated with aspiration, reflux and poor weight gain. Kids usually outgrow it by 24 months. Luke is 35 months and hasn't outgrown it yet. The surgical repair of LM is supraglottoplasty. It involves taking away some of the excess (floppy) tissue of the epiglottis. And this is what Dr. H. would like to do on Luke. It is a quick surgery and fairly common. He would probably come home the same day. We would then cap for a few weeks and try to decannulate again. The problem is that once the tissue is taken away it can never be added back again. And with kids who aspirate it can worsen aspiration permanently. If aspiration is bad enough, you cannot eat by mouth and you may have to have a trach to be able to suction the aspirated fluids. This is what makes this simple surgery a not so simple decision. Luke's last swallow study was last Spring. At that time he was still aspirating, but we don't know now if he is or not. We have scheduled a swallow study for Monday afternoon, and depending on the results of that, we will be able to weigh the risks of surgery for Luke. If they seem too great, then we will probably wait, keep the trach, do repeat swallow studies and see if either the aspiration improves or the LM does. If we decide on the surgery it should be scheduled for the end of next week.

If anyone has any insights or advice we would love to hear it.

Tomorrow is the day - Luke's LTR

Luke is scheduled to have a single-stage laryngotracheal reconstruction tomorrow. Dr. H. will take out Luke's trach and graft a piece of his rib cartilage into his trachea. This should make it both larger and more stable. Luke will be on a ventilator and sedated for a week. Then next week he will go back into the OR and if everything looks good they will try to ween him off the vent and we will all hope he can breath on his own. We expect him to be in the PICU for a couple of weeks and then in a room for a couple more. But everything is just going to depend on how he does. If the graft doesn't work, then Dr. H. will have to do another surgery to retrach Luke. There is some concern with Luke's nose continuing to close back up and with Luke's airway being inflamed (maybe an overactive immune response), but this seems to be the only way he can get his trach out. Life has been really good for all of us lately, so hopefully this will be just one more positive step.

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.