A long way...

Hi Connor,

You are snoozing right now and its nice and quiet here in your room and your POD so I thought I would take this opportunity and write you a little note. Before we get too far along though, we need to say something very special to someone who means a lot to you. Happy Birthday Gigi! I know if you could you would be cuddling with her for a birthday present but maybe you can just give her a rain check on that. You know what though.... you share something with Gigi today even though you are over one thousand miles away.

Today is your birthday too. Well sort of. I mean its technically not your birthday (don't tell your Grandma that, she really looks forward to these days) but today you are four months old. Its hard to believe that you have been here with us for four months. Its even harder to believe that the three of us have been living here in this hospital for four months. We thought we would have scooped you up and taken you home by now but that just wasn't in the cards. The doctors told us that you were going to have a really hard time getting through that marathon heart surgery of yours but that once you were on the other side of that hurdle you would be able to just keep on trucking and hopefully get out of here quickly. I think everyone (your mommy and I included) saw just how well you were doing after that heart surgery and we thought that there would be nothing that could possibly stand in your way. It isn't that we were wrong, we just didn't know your timetable.

You decided to take a different path when you came to us. First you were in a hurry and wanted to be here way too early and we did everything we could to slow you down. You weren't having it. You had your own plans and we learned for the first time that your schedule would be the one dictating our lives. You showed up early and gave the world quite a grand entrance. Then we thought you would need a heart surgery within three days of your birth but again you had other thoughts. Turns out you made your heart work its own way for a full three weeks and held off that surgery until you gained weight and had yourself settled. Then of course we were told that you would struggle so much after the heart surgery. Well as I mentioned before you flew through that. Within a week you were closed up and people were already talking about getting you out of here.

Then your strong will (that is the meaning of your name after all) stepped in again and taught us once more that your timetable is the one that will run the show. You have certainly taken your time traveling down the road that followed your surgery. It has snaked back and forth a few times and has even gone backwards more than once. But something that your daddy had to climb a mountain in Africa to learn is something that you apparently knew instinctively when you were born. Sometimes you can't just run straight to where you are going. Sometimes you have to go back and forth until you are accustomed to your surroundings and then you move forward. If you move forward too soon you won't be able to handle the journey but if you take your time and follow the path of patience you can go a long way.

And that is what you have done in your first four months of life son. You have taken your time and ignored the urge to rush right up the side of the mountain so that you can make the entire journey and not be stopped short because you aren't ready. So take your time, weave back and forth as much as you want, just keep moving forward every time you are ready and we will be right there beside you to help if you ever need it. And if you ever start to look up at the top of the mountain that seems so far away just remember that you have already come a long way. Sometimes we forget that when we don't look back and only focus on what is in front of us but its important to remember that we are all on a journey with things in front of us and behind us and every once and a while its okay to look back and say "wow, look how much I've done already".

Keep up the good work little man. Four months old and look what you have done, look how much you have overcome, remember you have come a long way.

We love you Connor.

- Daddy

Eat it up!

Hi little buddy,

I have been sitting by your crib for several hours now watching you sleep peacefully.  As much as I love our play time and watching your little hands move about I have to say it's wonderful to see you rest.  The last two days have been filled with some rough times for us.  You have been pretty upset several times a day and it's obvious that something is bothering you.  I wish it was as obvious what that thing was but as of now it's a mystery.  I also wish I could do something to calm you down and comfort you but right now the only thing that seems to do the trick is medicine.  The doctors have run through a series of ideas but no answers yet.  More specialists, more tests and more possibilities of scary problems but as your smart daddy told your upset mommy last night, "we don't know that of those things are true, they are all just theories."  So for now we aren't going to waste time and energy talking about those things and mommy will try not to let her mind get ahead of things.

Despite the fact that the past two days have been pretty rough there has been a good development.  On Thursday the occupational and speech therapist came to see you together.  They had gotten the green light from the doctors (thanks to ENT for pushing for this) to try tastings with you.  This means they put the littlest bit of milk in your mouth and see what you do with it.

When the therapists arrived you were sound asleep and I mean SOUND asleep.  They both looked at you and said, " I doubt this is going to work out today."   Mommy asked that they at least try and wake you up before they wrote the day off.  The OT sat you up and we rubbed your face, feet, belly and you still kept sleeping.  It took a good 10 minutes to get you fully awake but you were pretty happy when you finally cleared the sleep from your eyes.  The speech therapist started with your favorite paci which you sucked on right away.  She then explained that because it has a flat nipple it's easier to keep in your mouth but doesn't use your muscles in the same way you would need to suck out of a bottle and swallow so she switched to your other paci.   We call this one your therapy paci.  You surprised her when you took to paci number 2 without much trouble.  She was then a little hesitant to try the actual bottle nipple because she said that's a lot of change in one session but your OT told her you were a rock star and so she tried.  Much to their surprise you started sucking on the bottle nipple like you had been doing it your whole life.  The therapists were giddy with excitement.  The next step was to put some milk in the nipple and see if you could get it out.  They started with one milliliter of milk and in a split second the nipple was empty and you were looking for more.   They gave you a second milliliter and you took care of that too but slowed down a good bit.  Mommy and the therapists couldn't be happier.  It might not have been much but swallowing anything was a huge victory.

Yesterday the speech therapist came by early in the morning before mommy was here and tried to work with you  but you weren't very interested in trying (you had recently had a dose of sleepy medicine so I can certainly understand not being interested in therapy).  Mommy was disappointed that she missed the trial but one of the occupational therapists said she would be back later to try.  While mommy was at lunch with one of her good friends, Julie, the OT returned as she had promised.  Luckily your daddy was here and got to be a big part of your second day of tasting.  The OT had daddy hold you and put the nipple in your mouth while she dropped the milk in.  You ate a whole 8 milliliters of milk in a row.  BIG PROGRESS!!!!  Daddy and I are so proud of you little man.  The therapist said they would do a few more trials next week and if you kept eating about the same they would move onto a swallow study.  The swallow study allows them to make sure everything is going where it's suppose to and not sneaking into your lungs.  Since we haven't had to suction any of the milk out of your trach after you've eaten these two time it seems that you're off to a great start.

Although sometimes it seems that the mountain you have to climb is insurmountable you always amaze us by tackling each obstacle as if it's nothing.  There's no doubt that we have a very long road ahead of us but your strength and fearless attitude pick me up when I am worn down.  Connor you are my hero.   To a lot of people drinking a little milk may not seem like a big deal but for you it's HUGE.  This will be the case for most milestones you will achieve.  It may take us a little longer to get there and I'm certain it will take a lot more work but that will only make them mean so much more.  Let's try our best to celebrate your victories and worry less about the obstacles left to conquer.

Mommy and Daddy love you Connor.

-Mommy

Bumps...

Hey Bubba,

Before we get to anything else I am going to start off and just say I'm sorry. You know why, and in case you forgot I'll tell you again in a minute but I had to say sorry before going any further.

So we learned a lot the last couple of days, and we still have some questions about a few things. We have now learned how to clean and care for your trach, we have learned how to clean and care for your g-tube, we have learned how to cath you, and we have learned how to suction your trach. Maybe that doesn't sound like all that much learning, and maybe to a person who has any background in the medical world at all it wouldn't be. But for your daddy that is a lot of learning. However, I do love to learn so that part isn't too bad. When it comes to the "doing" part I am not nearly as ready to go as I may appear.

I have been trying really hard to remember that these things we are learning to do right now aren't forever and they are things that you will need when you finally do get out of this hospital. Keeping that in mind has gotten me through cath'ing you (which to be honest I never thought I would be able to do after watching the first one) and it has gotten me through cleaning your g-tube (here again I thought I would toss my cookies if I ever had to do anything with that tube) and it got me through suctioning your trach (this was probably the easiest but that was because I had the help of your respiratory therapist). Today though, we ran into a problem.

Mommy and I were getting all ready to do your trach care again (this is day three) but this time she was going to be the one holding your trach in place and I was going to be the one cleaning your little neck and putting all the ties and padding back on you. This sounded quite simple and after watching mommy and a couple of your respiratory therapists do it I thought I should be able to do it just as easily. So the first two parts went pretty well, we got all the stuff from today off and I cleaned up everywhere to make sure you weren't hiding any of your slimy stuff. Next it came time to put your new collar on and fasten it. Here I realized that the dexterity in my fingers is somewhat lacking. It took me forever to thread that little velcro strip through your collar and get it ready to fasten back down. I was all kinds of stressed out and I am pretty sure my heart rate was higher than yours the entire time I was working on it. Unfortunately that was the easier part. Next I had to try and put a little padding strip down on your neck and chin and get it under your trach collar. This is when I realized that having hands big enough to palm a basketball is actually a bad thing. I fumbled around with it and tried several times and despite my best efforts, and a bucketful of sweat dripping from my forehead, I could not get it on correctly. Thankfully mommy kept her cool and got it done for me.

The worst part about that experience was the look on your face though. You were so upset and it was clear that you were really uncomfortable during that entire debacle but no matter how hard I tried I couldn't seem to make it any better or go any faster. For that I am so sorry. I am told that it will get easier with practice and time and even easier than that when you get to growing a little and your neck gets a little longer so we have some room to work with. In the meantime though I feel terrible about it and dread the thought of doing it tomorrow. Just know that I am doing my best and will do everything I can to get better at it so it isn't so hard on you.

Now even though we have had some answers come out of the last few weeks we have had a few questions pop up in the last few days. It seems that something is bothering you again and no one knows what it is. We thought you were having your "episodes" before because of your upper airway problems but they have started happening again. The trach didn't quite fix everything like we thought it would, or so it seems. Mommy and I spent a long time with you last night trying to calm you down after your heart rate and agitation made a slow climb into the unhealthy zone over the course of a few hours. It took some medicine and a trach change to finally calm you down enough that you could sleep. That didn't work for too long though, you needed another big dose of meds early this morning before mommy got here to keep you calm. Now it has happened two more times today and the doctors have no idea what is going on or what to do to help. We are trying buddy, and we are hoping that this is just another one of those bumps in your road out of here.

Now there is some good news from today but that is mommy's story to tell and I am not going to spoil the secret. She will write you a note later or tomorrow and fill you in but it is some pretty exciting stuff. In the meantime lets try and get some rest since you have been so agitated and worked up today. I think we could all use a good night's sleep. So sweet dreams little man, keep on growing and keep on fighting, you are the toughest little guy your mommy and I have ever met and we are SO proud of you every day.

We love you Connor.

- Daddy

Learning, again...

Hey there Bubba,

Your mommy and I are going to try and get back into a better routine for writing you notes because we know that you like them and they help us to express what all has been going on with you and in our heads. Oh, and we have to update your "fans" as mommy calls them. There are so many people who tell us all the time that they start every day with you or go to bed with you every night. You are continuing to touch more and more people each and every day. I'm pretty sure you are going to have a VERY long waiting list of people looking forward to their first face to face visit with the amazing Connor. Maybe we should hand out tickets with numbers on them like they do at the deli?

But back to being serious. Your mommy and I have been so proud of you the last few days, even more than before. Like mommy told you last night you are being so strong and facing everything the doctors are throwing at you without so much as a flinch or a quiver. They have backed down on more of your meds, they have put you on a twenty-four hour breathing trial, they have changed your trach for the first time, they have switched your feeding schedule to two hours on and one hour off, and to top it all off they even switched you rooms. There is plenty of space in this new room and what is even better is you are almost neighbors with your fellow fighter Ava. She and her parents are now just a few doors down from us so we can visit with them more often. You're right, it is about time we got that worked out.

I know I merely brushed over it just now, but that trach change is a pretty big deal. Changing that first trach was the big step that the doctors and nurses had been waiting on so that we could start moving you around again. I know you probably don't like being stuck to that bed and trust me we don't like seeing you stuck there either. So with that in mind you got to do some moving and shaking today. Not just the kind that you do when you are mad at us or telling us that you need a new diaper either. Nope, not you, you were sitting up and looking all over and playing with some of your friends.

First your respiratory therapist came in, one of the ones that mommy is a huge fan of, and told us that it was time for us to learn how to take care of your trach. So we got some long q-tips, some water, some peroxide, some new trach ties, and some of that sticky foam stuff that you have stuck to your neck. We laid it all out and got ready and the respiratory therapist walked us through what we had to do step by step. He was very good about telling us the things we needed to be careful about but still letting us learn all the stuff we would need to do. We tried to be as quick as we could and as efficient as we could but we still weren't quite quick enough. After a short while (it was probably a lot longer in your world) you were pretty upset with us and we had to hurry it up. We did get everything done though, after a minute or fifteen. I promise, we will get better.

Then your favorite occupational therapist came in and brought a friend with her, a speech therapist. Now that may sound confusing since you aren't really talking or anything like that but they are the ones who are going to teach you to swallow and possibly eat. No promises though, its not likely that you will be able to eat any time soon but they are sure going to try their best to get you ready to do that when the rest of your body tells you it is time. She watched you suck on your pacifier for a while and when you said you were done with that (apparently you speak a language the therapists here can understand) it was time for your occupational therapy for the day. That included sitting up, rolling over on each side, playing with your hands, moving your head around a little bit, and making all kinds of faces. You were pretty happy about it for a little while, even smiling at the therapists. Unfortunately that didn't last too long and you were ready for some rest. But you did get to move around quite a bit and it was great to see you sitting up and smiling.

Then yet another learning experience for daddy. Your nurse came in and told us it was time to cath you and lately we have had a lot of nurses ask us if we are ready to that on our own. Well we have never been formally shown how to do or even had a nurse ask us if we wanted to learn so today I figured it was about that time. So for the second time in a few short hours I gloved up and helped out with one of your daily procedures. It was more mentally painful for me than it was for you. I know this because I was grimacing the entire time and you slept right through the entire thing. I guess that means I did a pretty good job but I would like to say that it is because you have had some really great nurses who have shown me just how to do it so you won't get upset. Another thing checked off the list to get you home.

And now you are resting after all the excitement this afternoon. You look like you need it because you are zonked right now. You aren't the only one though, your daddy is really worn out too. I have been trying to keep up with everything in the world right now so that we can hopefully somehow get a semi-normal life when we get out of here but it seems like just when I start to think I am making some progress and maybe we can stop worrying about these things that are both frivolous and outright malicious I get an email or a phone call that drags me backward again. I hope that it won't last too long buddy but just know that even though I may be exhausted and stressed it has nothing to do with you and I will always find a way to make time for you.

Keep up the good work little man, keep teaching us new things like you have all along. We are so proud of you and still just amazed at you every day.

We love you Connor.

- Daddy

So expressive!

Hi little buddy,

I don't have much time to write you a note but I wanted to at least say hi and update your fans.  The past several days have been the best you've had in a while.  At first mommy and daddy were worried that you were so sleepy and that your heart rate kept dipping down lower then it has ever been.  You have certainly never set alarms off for your heart rate being too low before.   Turns out that the doctors think you have just been working and fighting so hard to breath this whole time that your heart rate was always elevated.  The sleepy medicines were bringing your heart rate down to an acceptable level but since your trach operation things are different.

You aren't working hard to breath at all and those sleepy medicines have slowed things down a little too much.  Therefore we've started the process of getting rid of those medicines.  You were able to kick the two continuous ones (or drips as they call them) within the first day and a half without much trouble.  Now we're onto working on the other two scheduled medicines.  These will take a little longer but we're going to go slow in hopes of preventing you from having withdrawal like you did last time (that was terrible).  It's amazing to watch you breath without struggling.  Your little chest barely moves when you take a breath and the breaths you take seem much deeper.  You don't look scared and when you get upset you're able to calm down much easier.

The next step is to work on getting off the vent.  You are already flying through the tests they have set up for you and surprising everyone.  The machine is set at the lowest settings and you are breathing over it so you really aren't needing it much at all.  Yesterday you were on a pressure trial for 12 hours, meaning you did all the breathing on your own, and today you have will stay on it all day and night if you can tolerate it (as if there's a question).  Connor, you are amazing.  Every nurse, doctor and respiratory therapist that walks in your room says that you are a new little boy.  I would disagree.  You are still the strong-willed and brave little man you have always been you just don't have to use all of your energy on breathing.

So what are you doing with that extra energy?  Making all kinds of fun faces.   Mommy and daddy don't know what's more exciting; watching you breath without a struggle or looking at your cute little face without any tape or tubes.  A lot of kids with CHARGE syndrome have some palsy on one side of their face.  It seems like you may have this on your left side which is why you can't always close your eye all the way.  We weren't sure how much it affected your mouth since we could never see it all the way.  I can't promise how much it will affect you in the long run but right now you are moving that mouth all over the place and super excited to have your tongue free.  You have very distinct looks and your little eyes can say a million words.  So although I may not be able to hear you cry or make your little noises I think I have a pretty good idea what you are thinking.  Connor your face is so expressive.

Here are a few cute pictures although they aren't the best because mommy has been enjoying watching them more than wasting time taking pictures of them.

"Here's a little smile for you mom"

"Check out my tongue"

"This mobile is amazing"

"Enough with the camera already"

Keep up the awesome job buddy.  You are a special special little miracle and I am so proud to be your mommy.  I can't wait to see all the amazing things you have in store to show us.  Sweet dreams.

Mommy and daddy love you Connor!

-Mommy

Big Pictures...

Hey there Bubba,

Yep, its daddy again, and its been a little while since I have written you anything because mommy and I have either been bombarded with doctors and specialists or trying our best to wrap our brains around what the doctors call "the bigger picture". I know mommy updated you on how you have been doing and all the things that you went through the other day (it was quite the laundry list of procedures) but I think maybe its time to talk about the other stuff we have been talking about the last few days as well. It isn't bad stuff per say but we have had to take a lot in and adjust our outlook on quite a number of things in the last seven days, and not all of those adjustments have come easy. In fact, most of them have been everything short of physically painful. But don't get too bogged down, there are a few good things coming at the end too.

So lets get to the meat of the quandary at hand right now. No beating around the bush or putting it politely, you have been through enough that we want you to know exactly what has been going on. Over the course of the last seven days your mommy and I have had to sit down and take a long hard look at "the bigger picture", or in slightly more terrifying terms, "quality of life". Not just for you either, for us and the rest of our family as well. Now this is really only terrifying because of the fact that the context in which it is normally used is terrifying. When people, or little boys, are happy and healthy they have a quality of life. Some good, some bad, most somewhere in the middle, but it is a quality of life none the less. Most of the time when people talk about quality of life though, they refer to someone who is in bad shape, or someone who has a grim future ahead of them. Sounds scary right? Well the conversations we have had over the last week have been about some grim things, but more about weighing those grim things against the possibilities for good things as well.

As I am sure you can imagine this hasn't been an easy set of conversations, mostly because the doctors don't seem to have their crystal balls working quite right and can't predict the future. Sometimes we forget that forecasting future events isn't a class offered in medical school and we get frustrated about answers like "we just don't know that yet" or "we will just have to wait and see how he does". But the bottom line has been that neither we nor your doctors know exactly what lies ahead for the three of us. Here is what they do know:
1) your little heart is as strong as they could have ever hoped it would be and maybe even stronger
2) your little lungs, despite some earlier concerns, are strong and working just like they should as well
3) your little brain is showing us all the signs it should be showing for a little boy your age
4) you have a lot of challenges you have to face yet
5) you will have to work really hard to get past all those challenges
6) mommy and daddy will have to work really hard along side you to help you get past all those challenges
7) because of your CHARGE diagnosis there is just no way to tell how well you are going to do until you have a little bit more time to show us
Those sound kind of vague don't they? Well that is what mommy and I thought as well. We weren't very satisfied with those answers when we got them, even though every doctor told us the same thing. We wanted their crystal balls to work but no matter how many times we asked the answers were the same. Facing the unknown is scary, facing the unknown with the chance for bad things happening just as much (if not more) than good things is absolutely terrifying. But that was, and is, the reality of our situation. The statistics aren't good for little boys with your specific set of problems. The information is daunting. The images are saddening. The "bigger picture" looked pretty bleak.

The doctors told us you were going to need a trach. The doctors told us you were going to possibly need a vent at home. The doctors told us there was no way to know how long you would need either of them. They told us you were going to need up to sixteen hours of nursing support after you left the hospital, that you would need special machines and mommy and daddy would need all kinds of training. They said that you would need someone to watch you every minute of the day as long as the trach is in. We wouldn't be able to take you anywhere in the car unless both of us (or another trained adult) were there. You weren't going to be able to talk for an undetermined amount of time, maybe ever. You were not going to be able to eat, again for an indefinite amount of time. When were we going to be able to take you to see Gigi and Grandad Rooster? When would we hear your little voice? When would mommy and I ever be able to have some alone time without worrying ourselves to death about you? Would you go to school? Would mommy be able to work ever again? Would you be able to play with other little boys and girls? All of those things scared us for a lot of reasons, for you and for us. But the only other option was to tell you goodbye.... The little bit of light that we had been holding on to so tightly was going out right before our eyes.

That is what we thought anyway. Then we started thinking about some of the things the doctors were saying and we realized that we had been missing something. They told us that there could be some really bad repercussions of you getting this trach and that there was a chance you could not do very well at all going forward. But what they ever so cautiously told us was that all the signs you had been showing were pointing to the fact that you weren't headed down that path. Instead you have been telling us (in all the little ways you can right now) that there is every chance you could be playing with friends and going to school and talking and eating and running and even going to visit Gigi. They couldn't predict the future but they could tell us that right now the signals you are sending paint a pretty good picture for the three of us. That picture will never be what we thought it would be and it will never be "normal" (but we have learned normal is relative to a degree we had never before comprehended) but it can very well be a very good picture, maybe a great picture.

But, even though we had this realization, mommy and daddy were still apprehensive about letting the doctors put in the trach. We both felt terrible about it and were sick about it all week long. We decided it was the best decision for you but we were still so uneasy. Then we received a gift. The kind of gift that comes without warning and without asking (like the good kind of Christmas gift we talked about before). After I wrote your note about catching up we received comments and stories from other families who had been in our shoes. They told us to hold on to hope because they had been in our shoes, they had faced the same scary scenario, and they had come out on the other side. We saw pictures of some beautiful little CHARGErs (I think that is what mommy called them?) who went though this and come out on the other side doing better than expected. I'm sure you saw but you also received a note from your little buddy Ronan, and he told you he was pulling for you too. It was these notes that carried us through Tuesday. They helped us see that even though things might be gloomy now they also might be bright and sunny one day as well.

So we held on to each other tight and we walked down with you to the operating room and there stood your ENT surgeon and right before we kissed you good luck he told us just how hopeful he was for you and your future. Mommy and I were still nervous but we felt better and better and better. When you came back from surgery you looked like... well you looked like you had just had a bunch of surgeries. But you rested all night and when we came in that next day you will never guess who we met.

The cutest little boy in the world (yes I know I am biased and I just don't care). That is you. You may not recognize you with out something taped to your nose or your mouth or at least your cheeks but believe it or not you have all kinds of skin under there and it looks great. I can't say that you are happy about this trach but I can say that you aren't upset about it right now. You are breathing easier and when you rest you look so much more comfortable. Maybe that picture is getting brighter already.

So keep on sleeping, keep on healing, and keep on looking as cute as you do. Your mommy and I are so proud of you for yet again showing us that you can handle anything, even if we can't. You are going to just keep showing us more and more I think. Maybe this is the first step to something, maybe its the opening of a door, just enough to let us see the light making things brighter.

We sure do love you Connor, with all of our hearts.

- Daddy

A tough pill to swallow....

Hi little buddy,

Sorry we haven't written much the past few days.  Yesterday mommy and daddy literally had a line of people waiting at the door to talk to us and it's been pretty much like that since Saturday.  All of those people were coming to talk to mommy and daddy about you.  A few of the doctors even came in over the weekend just for you.  See you've made quite a few friends throughout the hospital and so they all wanted to weigh in on your status.  It's amazing what an impact one little boy can have on so many people.  Unfortunately most of that talk centered around a topic that's been mommy's biggest fear since the week after your heart surgery.... a trach.  Every time mommy thinks about that word she gets a lump in her throat and has a hard time swallowing.

After our super scary blue episode last Wednesday mommy and daddy got pretty insistent that the doctors do whatever they needed to find out the root of your breathing problems.  On Thursday the doctors did a test called a flexible bronchoscopy.  That test allows them to watch what happens throughout your airway when you breath.  You have had bronchs before but they have always been when you had the breathing tube in with the purpose of cleaning gunk out.  This test was a little different.  You didn't have your oxygen on and you weren't sedated.  They were worried you wouldn't tolerate it or cooperate but they were pleasantly surprised that you were an all-star  (mommy and daddy aren't surprised in the least).  The test showed that when your little chin gets tilted down at all the top of your airway closes off.  The fancy word for this is laryngeal malaysia. Another term for this is "floppy" upper airway.

This sounds pretty scary and I imagine has been terrifying for you the past 3 plus months.  All of the "episodes" you have had are most likely related to the fact that no matter how hard your little lungs worked they couldn't get air up or down.  I can't imagine how that has felt and I'm so sorry we haven't figured it out sooner.  Now that we knew the culprit we had to talk about the solution.  That part was actually a pretty short discussion.  There isn't a solution.  Most kids outgrow this condition with time.  Kids with CHARGE syndrome take an extra long time but it is possible.  There is a fancy surgery that can fix some types of laryngeal malaysia but not for the severity that you have at this time.  That leaves the only one option, bypass the problem which equates to getting a trach.

Mommy and daddy were very leery of this option.  We knew this meant any entirely different type of life and level of care for you for many years.  Mommy and daddy are more than willing to get you whatever kind of care you need but we wanted to make sure we were making a decision that left you the ability to have a happy life.   I won't rehash all the specialists we met with and the specifics of what lies ahead for you with each of them (we'll face those when we get there) but I will tell you the basic summary.  First, you have a heart that is working extremely well.  In fact they said most kids in the ICU wouldn't have been able to push through so many of these episodes without more problems and make it where you have.  Next, you have a good set of lungs.  The fact that your lungs keep bouncing back from these episodes and have supported you off the vent for so long is impressive.  Lastly, you have a smart little brain.  All the pictures they've taken and exams they have done show you are developing impressively well considering all you have been through and how confined you have been to the ICU.  You are such a little miracle Connor.

So today you went in for what mommy calls the ENT buffet of procedures.  They cleaned out your ears and put tubes in to drain the fluid.  They re-did your hearing test which showed about the same results.  The hearing in your right ear is moderately impaired and your left is severely to profoundly impaired.  This sounds scary but mommy and daddy are going to work really hard to get you all the help we can for that.  Next they looked in your nose which was still clear and opened but they dilated it to make the hole a little bit bigger.  Next they clipped the part of your tongue that attaches to the bottom of your mouth because mommy noticed you were a little tongue tied.  Lastly they took another look at your airway and put in the trach.

Whew.  You must be exhausted.  I'm tired just thinking about it.  It's a good thing that you are the toughest little boy I know.  Now you are back in your room and resting quietly.  As of now you have to be back on the vent hooked to the trach.  The doctors hope is that they can wean you off the vent and that you won't need it at home.  Mommy and daddy are going to focus all of our prayers on that for now.  For the next few days we just want you to rest and heal.  Let us worry about next week, next month, and next year.

I know you can't be happy about that tube under your neck but try to hang in there.  The doctors, nurses and therapists tell us that kiddos adjust very quickly to them so give it a try.  Mommy and daddy are trying to swallow the tough pill that is your new trach.  We hope and pray this is a way to help you breath easier and get home but most importantly enjoy life.  Connor we love you more than anything and are your biggest fans.  Sweet dreams little superhero.

I love you
-Mommy

Grandpa weekend

Hi Connor,

The past few days have been pretty quiet ones for you buddy.  You have been happy and getting a lot of rest.  Mommy and daddy have had a lot of fun playing with you and you were lucky enough to have visits from both your pappaw and grandpa this weekend.  Today you spent about 3 hours napping on grandpa's lap.  It makes mommy's heart smile seeing you two together.  Although you were asleep most of the time you seemed pretty happy about who was holding you each time you woke up.  You kept smiling at grandpa and making all kinds of faces.

 Mommy and daddy have been pretty busy the past three days.  We have been talking to doctor after doctor about what lies ahead in your journey.  I'll be honest the past few days have felt a lot like the first week you were born.  Every time we think we might walk out of your room or leave the hospital for a second another person is waiting to talk to us.  The doctors are trying to give mommy and daddy all the information they can about each little system in your body and what they foresee happening with it in the future so that mommy and daddy can make some important decisions.  The hard part is that you are truly one of a kind so despite how smart the doctors are they can't predict the future for you.  Tomorrow we will finish up the last of our meetings and then be able to explain things a little better to you.

For now there's no reason for you to worry about all those doctors and their fancy words.  Just rest and dream sweet dreams about all the people that love you.  Your belly is full from milkshakes again, you are breathing pretty easy and you are completely passed out so mommy and daddy are going to go get some dinner.  We love you more and more each day Connor.  Thanks for brightening our day each and every day.

I love you
-Mommy

Catch up...

Hey Bubba,

That's the new name I have taken to calling you lately, you seem to respond to it well and there is a nostalgic quality there for me as well. I used to call your uncle Sebastian that when I was little. Not because that was his name but because I wanted so badly to call him brother and despite my best efforts the word that came out over and over again was "bubba". It stuck with him a while, just like I'm sure it will stick with you a while. It is one of those names that, in the case of children, just says "love" in a way all its own. You're my Bubba, I love you, that's just how it is.

I am certain you have noticed a few things in the last several days. One, we haven't written you any notes lately. Two, the notes you have been getting aren't quite as cheerful as the ones from a few weeks ago. Three, you have been awfully upset lately. Four, mommy and daddy have been too. I'll catch you up with these things one at a time so we can keep them all straight, even though they are all basically the same problems just materializing in different ways.

One. We haven't written you because of some problem we are having with our computer and the internet service at Ronald McDonald. I am pretty sure it was something that I did wrong or updated when I shouldn't have but I cannot figure out what it is and are therefore without internet in the late evenings. Now mommy and I had a plan about that, we were going to make sure and either write your blogs in the morning or in the early evening, right before or after dinner. It sounded like a great idea. It should have worked beautifully. However, you had some other plans for those times of day. Most of the mornings you have made sure to greet mommy with some sort of fit or episode (we are back to the sitcom these days) and therefore tie up most of her morning. Then, somehow, the doctors and nurses and breathing treatments and therapy sessions seem to take up most of the middle of the day. So when either mommy or I settle in on the love seat in your room to write you a little note you decide that it would only be fitting if you both began the day with a fit and then ended it with one as well. And that, my little man, has kept us busy until late each of the last several nights, keeping us from getting anything written before heading to sleep for a few hours at Ronald McDonald.

Two. The notes haven't been nearly as cheerful lately because the steady stream of positive energy and signs of progress has screeched to a halt since last Wednesday. To be completely truthful here I have to tell you that not only are we not making any significant progress these days, most of your doctors seem to agree that you are declining daily. That is a pretty scary thing, I know. Try not to worry too much, the doctors are still doing everything they can to try and get you better even if they don't know what will work at this point. Every one of them that stops in makes sure to tell us that they are still working diligently to figure out what is going on. Even Dr. Nelson with his busy schedule took a good chunk of time out today to sit down with mommy and daddy and discuss what some of the next steps are. Looks like we have some more tests coming soon little man. Hopefully those will bring forth some new answers and some good news and we can get some of these notes back on track.

Three. The last week has seen some of your most epic fits. I feel like you have been battling on and off for the last week, struggling with something that we can't identify and you can't quite seem to beat. You keep waking up, from a complete sleep, gasping for air, fighting for every breath. The first time mommy and I saw this we thought maybe you were just upset and you had to go to the bathroom or something but it quickly became apparent to us that there was something more at work here. It has been taking us a long time to calm you down and you even had to go back on CPAP a few times to help get your lungs working and your airways open. It seems like no matter how much sedation you have on board or how strong the breathing support is you can't seem to fight off whatever this unseen enemy is that keeps attacking your breathing. Today was especially bad, you woke up fighting, after a long night of restlessness and battles, and even though you were sedated and had your CPAP mask on you couldn't quite catch your breath. You turned blue quickly and mommy and the nurses and respiratory therapists had to act fast to get you to come back to them. From what I understand today was the closest call you have had since the first day you showed up here at Children's, and that isn't a good thing at all.

Four. This past week has been very hard on you, but it has been pretty hard on mommy and daddy as well. There are two things that seem to wear on us the most. The first is that we hate to see you in pain and it hurts us every time we do. But the thing that hurts us even more than seeing you in pain is knowing that we are completely helpless to do anything about it. We try to hold you and rock you and calm you down. We try to pat your chest and rub your head and tell you it will be okay. We try to sing to you and talk to you and play with your mobile. But nothing works. Nothing helps. Nothing fixes the problem. Nothing seems to make you feel any better. That is our pain, that is why we are upset. We want nothing more in the world than to make it better yet there is nothing we can do but watch and hope that the doctors and nurses can help you. The other thing we keep facing this week that hurts us is the future. Mommy and I keep hearing things like "tracheotomy" that terrify us. We don't know what the future holds for you and like any parents we want yours to be bright and full of wonderful things, but lately the light has seemed to dim down and the wonderful things have become less possible. This week we have had a hard time feeling helpless when it comes to the moment we are in, and even more helpless when it comes to the days that lie ahead of us, and in particular you.

So I know I didn't give you a ton of detail today but I don't think there is much more to say that will be any more helpful. The next week will bring with it a myriad of tests and procedures, each having the ability to lighten the burden we currently bear, or hand us yet another weight to carry. We will hopefully find answers this week, be they good or bad, that will help us to start moving forward. These tests will shed some light on the path we are headed down, now lets just hope that the light leads us down a happier path than the one we have been on recently.

So sleep now Bubba. Dream of that crazy tiger and his zebra buddy (on your mobile) and the games they play while they dance over your head. Get rested up and get ready for the next round of fights and tests because tomorrow we start again. Know that your mommy and I are so proud of you, and that we are in awe of your strength.

We love you Connor, with all of our hearts.

- Daddy

ZZZZ....

Hey there Sleepy Head,

I'm guessing after last night's note you have a pretty good idea what the doctors wanted you to do all of last night and all of today. Yep, that's right, they wanted you to sleep. So after your fits last night Dr. Ilias got you all fixed up with plenty of medicine and plenty of breathing support so you could get some much needed rest. Your lungs have been doing well today, your secretions have been less than usual, and your heart rate has been doing pretty well too. But of course all of those good numbers and good signs come with a cost, and today that cost has been extra doses of your sleepy medicine. Lots of extra doses.

I think because you are keeping this respiratory issue of yours a secret for now the doctors have made the decision that it would be better for you to be sleeping and resting than awake and potentially throwing a fit. Every time you get upset lately your color gets bad, your lungs start to work too hard, and your heart rate jumps and won't come back down. Mommy and I had gotten to the point of not freaking out every time you had a little fit but the events of the last few days have brought us right back to where we were two months ago. We see you start to get a little fidgety or upset and we rush over to your bed and do everything we can to calm you down before you start to work too hard.

Even though mommy and I are doing our best to help keep you calm (mommy has been doing way more work at this than I have, you have put her through a pretty rough week little man) we can't seem to be here all the time and stop all the fits. So because of that the doctors just keep upping your medicine more and more in the hopes that you will eventually just level out and rest. If we get to that point (maybe we are close today?) then we can start weaning back off of your medicines and your breathing support again and see if we can figure out what is going on and why we can't seem to get over this hurdle. That is the plan for now anyway.

This week should be an interesting week for you. Dr. Morales really wants you to have some fancy scan done this week that will watch you breathe and hopefully see something we can point to and fix. Your ENT friends want you to have another MRI, this time to take a good look at your ears and see if they can determine what all is going on with them and how they can help. You are supposed to have a follow up audiology test but I think maybe that one has been pushed back. And then, of course, we are going to start weaning you off your breathing support and your medicines again. Oh, and we can't forget your milkshakes. They will make a comeback too I'm sure.

Maybe that doesn't sound like a lot compared to some of your weeks since you have been here, but as you know these weeks where we try to wean you off of things are never easy. And one test always leads to another test. So for now how about you just keep on keeping on and sleep your little day away. You deserve it, and odds are you are going to need it. But know that mommy and I are here, and we love you, and we are SO proud of you and your strength. You continue to inspire and amaze us every day.

We love you Connor,

- Daddy

We love you!

Hi Connor,

Mommy and daddy don't have much to say tonight.  It has been one of your longest and hardest days yet.  You have worked extra hard to breath all day and tonight it got especially rough.  Luckily Dr. Ilias is here and knows you so well that he knew despite your numbers being ok you were far from ok.  He put you back on CPAP and gave you some extra medicine to help you relax.  You are finally resting for the first time today.  Mommy and daddy just wanted to say how very much we love you.  We are so proud of how hard you continue to fight especially on these extra tough days.  You have won over the hearts of so many people that work here, that when you have a hard day your room is flooded by people wanting to help out.  You are the most amazing little person I have ever met and I am so thankful you came into my life.  You are my life little man.  I am praying extra hard that you have a peaceful night and your body is able to recover from all the hard work you put in today.

Mommy and daddy love you Connor.

-Mommy

Back again...

Good Morning Connor,

Its been a rough thirty-six hours for you big boy. You almost made it through Gigi's visit with nothing but good days and better days, almost. But just as soon as daddy finished writing your note the other night you started to fuss a little bit. I put away the laptop, put on my jacket, started to kiss you good night when all of a sudden you were upset in a big way. Luckily for us Gigi had found your new pacifiers and that calmed you down pretty quickly. We all could breathe easy again. Then Gigi walked over to kiss you goodbye and you started to fuss again. This went on for about half an hour until you finally let loose and went into a full fit. That's really when the trouble started.

You had one of your favorite nurses that night and thankfully she was able to stay calm and think clearly because after almost an hour of you going back and forth between a calm and happy sleeping baby and a full blown "hot mess" (as mommy likes to say) mommy, daddy, and Gigi couldn't think straight. We started to get worried about you because you didn't look good, your color was off and your breathing seemed awfully labored. It was at that point that your labored breathing and fussing started causing your oxygen levels to drop, and drop quick. You didn't go into a crash or anything but you dropped to the sixties and seventies and no matter what we did with your sensor the numbers weren't coming up.

That is when your nurse's level head saved the day. We were all trying to calm you, assuming that you would look worse or turn blue if your oxygen was really that low. Your color wasn't good but it wasn't that bad either. Lather, rinse, repeat, we have done this a thousand times. But your nurse knew something was wrong, something was different. She ordered a blood gas for you and when the numbers came back people started moving fast. You may not remember this, but your aunt Alisa once told your daddy that in a hospital you don't ever have to worry until people start to move fast. Crap.

Turns out your CO2 levels were the highest they have ever been and your other numbers (most of those I don't understand completely) were way off as well. As soon as mommy heard them she was worried too, but the nurses and the respiratory therapists were already in action. The doctors had been called. Now it was time start panicking. Even Gigi's face, which I have seen stay calm in some of the most intense situations,  started to show some concern. Not again, not now. Mommy and I were worried that breathing tube was going back in.

Dr. Ilias was on the case though, he got your high flow pushed back up and your oxygen turned to one hundred percent. He ordered some more medicine for you and he promised mommy and daddy that he would do everything he could to keep you from moving too far backwards. Too far, we were holding on to that idea because it was clear you were going backwards, again. So we held on, with your Gigi right there with us, and hoped that Dr. Ilias could keep you on your high flow. It was a really tense forty-five minutes or so until you finally settled in and your breathing slowed down.

During that time the doctors took an x-ray of your lungs, they just wanted to make sure that you didn't have anything in there. The good news was that you didn't have anything in there. The bad news was that you almost completely collapsed your left lung, again. So the respiratory therapists went to work on you and started doing your chest percussion therapy and suctioning to see if they could get your lung to open back up without us having to put the tube back in and bronch you again. Thankfully you have some great nurses and doctors and respiratory therapists because they got your lung back and finally, after about three hours, you were calm and sleeping. Granted it was with the assistance of some serious drugs but serious times call for serious measures.

That was just Wednesday night. Maybe when I have caught up on some sleep and have a little more energy I will fill you in on yesterday but for now you are stable again. You are back on super-high high-flow and you are no longer getting any milkshakes but you are stable. So sleep and rest and maybe we can make another run at the mountain next week.

Mommy and Daddy love you Connor.

- Daddy

New Year's Resolutions...

Connor,

Hey there buddy boy. So we haven't been able to write you any notes the last few days because our connection to the internet hasn't been working very well. I know that is no excuse but it is the reason for the lack of writing lately. Next time we have a plan though, the notes may not be posted but they will be written and just added when we can get back on the internet. But enough of that, there has been a lot going on lately so its time to catch you up.

Your Gigi has been here since last Friday and she has been with you almost non-stop since she set foot in Kentucky and crossed the river to your hospital room. She has found a whole new side of you that we didn't even know existed yet. Apparently you like to be picked up and bounced around and cuddled and just held all the time. We knew that from time to time you enjoyed snuggling up in mommy or daddy's arms but we had no idea just how much you liked being up out of that bed of yours. But Gigi used her magic to figure out how you like to be played with and how to keep you calm when you get up and around so that she can play with you. Mommy and I are going to try and replicate this magic but forgive us if it takes a few attempts, we are kinda behind Gigi in baby experience after all.

In the days since your last note you have been busy working too, not just playing with Gigi. You got the doctors to turn your feeds back on and you are back up to five milliliters an hour. Now that is a long way from your goal of twenty-two an hour but I bet it has to feel good to have some food back in your belly. You seem to like it and it seems to be doing its job because you are growing again. I think mommy said your last weight was over four kilograms, that means you are getting closer and closer to nine pounds. That may still sound like a small number but considering where you started and what all you have been through I think getting you up to nine pounds will be a huge accomplishment. But then I guess that will just be another notch on your wall of  achievements, one more milestone knocked down and out of the way.

You have also been doing so well with your breathing that they are weaning your high-flow down every few hours and you are back down to two liters per minute. The next step will be one liter per minute, then one-half, then, assuming you are still kicking butt, they will take the oxygen off completely sometime tomorrow. This would mean a lot of big steps. Aesthetically speaking you will, for the FIRST TIME IN YOUR LIFE, have no tubes on your face, no tape on your face, no tagaderm on your face, nothing bothering your adorable little face any more. However important we all may think that step is I have to tell you that it is going to be an even more important step forward to have you breathing all on your own. There will be no more assistance from any machines and no more oxygen flowing in your nose and bothering you while you sleep. That is one of the biggest hurdles to get over before getting you out of this hospital room and back home with mommy and daddy. So of course you are acting like its nothing and just flying right past it. That's how you roll.

So if your feeding and your breathing are almost under control now that means there is only more more thing we need to work really really hard on to get out of here. The doctors are going to have to start slowly (VERY VERY SLOWLY) weaning down your sedation medication. They tried this a little while back and it didn't go so well but hopefully we learned a thing or two from that last attempt and we can do a little bit better this time. So today they started weaning you down a little bit (it was Catherine's idea, but it was such as mall amount that I'm not sure they can really call it weaning...) and tomorrow we will move a little bit more and then the next day a little bit more and hopefully, after a few days, we will be able to get you off those medicines completely and we can really start talking about things like moving to the step down unit and maybe getting out of the hospital. One step at a time though, so lets not get too far ahead of ourselves today.

In the world outside of this room another big thing came and went, New Year's Eve. The year 2012, your birth year, wrapped up and the whole world welcomed in the start of 2013. As the world waits and watches together people all over join in an old tradition and make resolutions. These are kind of like goals, except they are supposed to be even stronger than goals because they are tied to the start of the new year and the beginning of something new. Now most people come up with some generic New Year's Resolutions like "this year I am going to drop 15 pounds" or "this year I will learn to play the guitar" but mommy and I have be learning that we can't think the same way we used to before you came along so our resolutions were a little different.

This year we will take you home. This year we will make sure and pick you up and hold you at least once every day (that will become significantly easier once we get past that other resolution). This year we are going to thank God for every day we have with you. This year we are going to make sure that as soon as we get out of here we give back to Children's Hospital and the Ronald McDonald house because we wouldn't be this far without all of those wonderful people. This year we are going to take LOTS of pictures. This year we are going to take naps, all three of us, together. The list goes on, and even though not all of these resolutions are in our control we have every intention of following through on all of them.

Right now you and Gigi are saying your goodbyes and giving hugs and kisses and Gigi is leaking some of her love for you. Remember what I told you about the tears you see when the people you love are holding you, they are just the love we feel in our hearts finding a way out. And Gigi has a lot of that love so you may see a lot of it leaking out as she tells you bye tonight. Don't you worry though, she will be back, and next time with your Grandad Rooster too, and you will be able to play again.

So how about you close your eyes and take a nap and think of some resolutions of your own. Don't be scared to dream big son, you have already proven that you can do big things and I think the older you get and the bigger you get the more you will be able to do. But for now you are in need of some sleep so close your eyes and rest, dream of Gigi's hugs and mommy's kisses.

Mommy and Daddy love you Connor.

- Daddy