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.

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!

Luke's Vitalstim

Vitalstim has been going well for Luke, but he is still aspirating. At least he is becoming less averse to having things in his mouth, and most of the time actually likes the games his Vitalstim therapists play with him. The plan at this point is to do another swallow study after 30 therapy sessions which should be around the beginning of December and see if he has made any improvement in coordinating his swallow. Then we are going to take a little break and go back to Vitalstim after Luke has had a few more surgeries, probably sometime in Spring. We have a lot coming up and I owe you all a big post about Luke's last ENT surgery and visit.

Here are a few photos of Luke having Vitalstim. I am amazed at the stuff they can get him to tolerate orally that I haven't been able to. Patience, patience, patience seems to be the key.

Halloween photos

We had a fun Halloween this year and even got to go to a party at Lara & Daniel's house.

Sam as the Pokemon Marill, Luke the pumpkin, Oona, a little witch,
& Gus as Pharoah from Yu-Gi-Oh

The pumpkin was a good choice for Luke because it was so warm.

Quite an honor

Sorry it has been so long between posts. We have a lot of things to update about and pictures to post, so I will try to get to it soon.

I wanted to start with a surprising honor we received last night. We were invited to The ARC of Shelby County's annual awards banquet, where our family was given the "Inspirational Family of the Year Award". We felt so honored to be chosen for this award mainly because it means that people and an organization that we respect so much, think highly of us too. I was especially glad for Sam & Gus, because they were so proud. There are a lot of things that Rob and I don't get to do with them and for them and so many times when Luke's needs come first because they are medical and more pressing. So this award was like a recognition of the sacrifices Sam & Gus make and the good-natured way in which they make them.


One of the most wonderful things we have found on our journey since having Luke is his therapists and the other members of his Early Intervention team. Between The ARC, The Bell Center and Children's Hospital over 2 dozen therapists have worked with Luke and we have not had a bad experience with any of them. Each of them is special to us for different reasons. One of the things that I love about Luke's ARC team is how accessible they are. I don't hesitate to call any of them if I have a question or need help. We never have to feel like we are in this alone - we always have backup. We know too Luke isn't just another patient to them. They care about him and us, and I am sure they feel the same about the other kids they see. It can be easy to get discouraged about how long it sometimes takes for Luke to reach milestones or achieve goals. But his therapists are always so encouraging and help keep us focused on all the progress Luke has made and is making every day. If any of you read this, I hope you know how deeply we appreciate the contribution you make to Luke's life and to our whole family.

In Memory of Lucas Weindorf

It has been a while since I have posted, and I would much rather not be posting under these circumstances. Lucas Weindorf lost his battle against Pulmonary Hypertension today. He was almost 17 months old. Rob and I are both so very saddened by his loss. Lucas and his family mean a lot to us. I thought our boys would grow up together. Even though we live far apart, this wonderful age of technology allows us to make friends and share experiences with people we never would have met before. Lucas' mother, MK, has been a great support to us as we have shared this journey through CHARGEland. If you would like to read more about Lucas or leave a message for his family, please visit their blog at http://mkweindorf.blogspot.com/


Lucas was such a beautiful, sweet boy. We will miss him so much.

G-tube update

Luke did get his g-tube on the 21st like we expected. It almost didn't happen since surgery had left him off their list, but a terrific nurse got it sorted out right at the end of the day on the 20th. Luke also had his second orchiopexy in which brought down his right testicle. He still has to have one more surgery where they bring down the left testicle which will probably happen in February or March next year. This surgery seems to have been Luke's most difficult so far. He stayed in the hospital for 4 days and had a good bit of pain, transitioning to OTC pain medicine (Tylenol) only on the last day.

Getting Luke up to speed on his g-tube feedings has happened more slowly than we expected. His goal is 5 bolus feedings of 6 ozs. each over the course of the day. This puts the feedings at about every 3 to 3.5 hours. He is currently at 1 feeding of 6 ozs. and 4 of 5 ozs. and they aren't bolus yet, but given by a pump over 45 minutes to an hour. At first Luke was throwing up anything over 4 ozs. like his stomach was just too full. We put him back on Reglan to help speed up his digestion and the throwing up has gotten much less. Unfortunately he got a cold in the last week and some of his hard coughing is making him throw up, which happened a lot in the past with his bottle feeding. The good news is that Luke already seems to have gained a few ounces going from 17 lbs. 6 ozs., when he was admitted on the 21st, to 18lbs. 1 oz., yesterday, or from 7.9 to 8.19 kg (both with clothes on). So if his weight gain is good, the nutritionists aren't as worried about getting his feeding up to goal.

With the exception of what is lost through throwing up, Luke's feeding is finally really consistent. The pump is wonderful for that. It is so easy to use and taking Luke out while he is eating is a breeze. The pump has a little back pack and it just comes right along with us. Now Luke can eat in the car and the stroller and, the best part, even when he is asleep! Another benefit of the g-tube that we have noticed is that Luke is finally well hydrated and actually producing several wet diapers a day. And the strangest thing of all is that Luke's constantly draining ears have almost totally cleared up. His left ear (the one that was cleaned out) has nothing draining, and the right can only be seen when we clean out his ear with vinegar water each night. Now, so far, we aren't seeing a big difference with his hearing, but I would have to think that there is at least some improvement. Luke did make a new sound yesterday - a very brief buh, buh, buh. When he has another booth test next week, we'll see if they can tell a difference.

The other news is that Luke finally got into VitalStim. He starts next Monday, so I will keep you all updated about his progress there.

Da, Da, Da, Da, Da...

I don't know if I mentioned it before, but Luke has been making a lot more noise lately, even without his PMV. And now when you put his PMV on he usually starts to make sounds right away. His favorite seems to be da, da, da... I took a few videos in the hospital of him making sounds. He even seemed to be queing sounds from me sometimes. So we are going to keep working on that. I apologize for the general shakiness of the video. Luke has started reaching for the camera anytime I get it near him - how wonderfully typical!



The day Luke had his swallow study, he also wore the PMV for the speech therapist. And it was an awful day all around, because he did terribly with the PMV. He did breathe through his mouth, but it was a real strain and he cried. The speech therapist thought there was some kind of obstruction between the trach and Luke's mouth, and that he should not really wear the PMV much if at all. And to be fair to us, I have never left the PMV on when he has had that kind of reaction, which has not been very often. But since that day we have not been using the PMV much. So when Luke was in the hospital last week I took advantage of the fact that he had an oxygen saturation and heart rate monitor to see how he responded physically to the PMV. He didn't cry or get upset or even seem to mind at all that he had the PMV on - in short, nothing like the day of the swallow study. Luke had the PMV on for over an hour and he did great. His sats. stayed between 98 and 100 and his heart rate was in the 120's. It gives me more confidence to know that I can tell when he is distressed and can remove the PMV. But there are definitely times when he does well with it and he should keep using it.

We're home...

... with no NG tube. Even with the x-ray machine the doctors couldn't get a tube to go down Luke's nose. I knew his anatomy was odd, but not this odd. It makes sense now that Dr. Hill is having such a difficult time with the choanal atresia. The radiologist says that he can always get the tube down with the x-ray machine, so he was quite surprised when it wouldn't work.

So the plan now is to just keep feeding Luke orally until he gets his g-tube next Thursday. As far as we know there is no problem combining the g-tube surgery with the urology surgery. They said Luke will have to stay for 3-4 days after the surgery to get his feeding schedule worked out. The plan now is to work him up to about 8 ozs. 4 times a day. This would be perfect for us, so I hope it works out. Also, Luke could keep having some solids by mouth to keep his feeding skills, and any calories from that would be bonus. He was "officially" diagnosed as "failure to thrive" on his hospital discharge papers, meaning he is under the curve for his weight compared to his height. His weight is apparently average for a 6 month old and his height for a 9 month old. The nutritionist wants Luke to have 900 calories a day to start to catch-up and we are only getting 600-700 in him right now by mouth. So Rob and I are confident now that the g-tube is the best decision for Luke.

Luke has tons of appointments next week - Dr. Hill, the dentist for the first time, and his 18 month ped. visit. I will try to update on some of those things before Thursday. Sam and Gus also want to join the cub scouts, so we have School Night for Scouting on the 19th. I hope they enjoy it. At registration they were very excited about going fishing and camping.

Hospital update

Luke was admitted to Children's today and he is doing well. In true Luke fashion though, things don't always go like you would expect them to. Three different nurse tried to put in the NG tube and they can't get it to go passed his nose. So the next option is to put it in under x-ray. Since it is so late, they will probably do that tomorrow. We will let you know when there is more news.