Impossible Decisions

Why is it, the second you make a decision and feel good about that decision, that decision get shot to hell?

Let me back up.

After arguing, crying (me, not Chad), talking, thinking, praying, and hoping, we officially decided we were not going to pursue Gabriel having the pharyngeal flap procedure.  We would continue speech therapy and supplement with sign language to further his communication skills, but would otherwise, accept what fate has thrown at us and not accept the risks associated with the procedure on behalf of our little darling.

We went to see Gabriel’s ENT on Monday and told him that we were done and were not going to do the pharyngeal flap.  He said we’d change our minds.  He told us that he has only known of two cases that ended with the child having sleep apnea and that the rewards definitely outweigh the risks.  He also told us that he would likely be able to give us an opinion on whether or not the procedure would cause sleep apnea based on the nasendoscopy Gabriel was going to have.

That brings us to today.  I took Gabriel to have the nasendoscopy done.  My intention was to try and record some of it, but since I took Gabriel alone, it was my job to hold him down while they performed the scope.  They put a tiny camera down his left nostril and asked him to say a few things, like ‘mama’, ‘all done’, bye bye’, ‘puppy’, and other assorted sounds.  The point was to see how close the soft palate was to closing off the nasal cavity to make the sounds needed for intelligible speech.

Drum roll, please…

Gabriel’s soft palate didn't move.

That’s right – not at all.  It’s so short it has no movement whatsoever.  The only option he has for intelligible speech is the pharyngeal flap procedure.  If we don’t allow it to happen, he will never be able to use any more letters than A, E, I, O, U, M, and N.  That leaves 19 letters that will never pass his lips.  What was the worst case scenario in this exam?  You guessed it.  We nailed it.

So, now we are back to arguing, crying (me, not Chad), talking, thinking, praying, and hoping.

Because I am at a loss for my own words tonight, I am going to leave you with someone else’s words.  My cousin sent this to me when Ethan was first diagnosed with Asperger’s, but it is so prevalent in both my children’s lives that I find myself constantly staring at my cubicle wall at work where these words live.

‘When you’re going to have a baby, it is like planning a fabulous vacation trip – to Italy. You buy a bunch of guidebooks and make your wonderful plans. The Coliseum. The Michelangelo David. The Gondolas of Venice. You may learn some handy phrases in Italian. It is all very exciting.

After months of anticipation, the day finally arrives. You pack your bag and off you go. Several hours later the plane lands. The stewardess comes in and says, ‘Welcome to Holland’. ‘Holland?’ you say. ‘What do you mean Holland? I signed up for Italy!!! I am supposed to be in Italy. All my life I have dreamed of going to Italy!’

But there has been a change in flight plan, they have landed in Holland and there you must stay. The important thing is that they have not taken you to a horrible, disgusting, filthy place, full of pestilence, famine and disease. It is just a different place.

So you must go out and buy new guidebooks. And you must learn a new language. And you will meet a whole new group of people you would never have met before. It is just a different place. It’s slower paced than Italy. It’s less flashy than Italy. But after you have been there for a while and you catch your breath, you look around and you begin to notice that Holland has windmills, Holland has tulips, and Holland even has Rembrandts.

But everyone you know is busy coming and going from Italy and they are all bragging about what a wonderful time they had there. And for the rest of your life you will say, ‘Yes, that is where I was supposed to go. That’s where I had planned.’

And the pain of that will never, ever go away, because the loss of that dream is a very significant loss, but if you spend your life mourning the fact that you didn’t go to Italy, you may never be free to enjoy the very special, the very lovely things about Holland.’

-Emily Perl Kingsley

Finally something to talk about

You may not believe me when I say this, but I have very valid reasons for not updating our blog right away after Gabriel’s most recent surgery. 

Reason #1 – I wanted to be sure I had good news to share…and I do!  The hole is still closed!!!!!!!!

The repair turned out to be much more difficult than anyone expected.  Our brilliant surgeon had to do a triple layer repair.  The first layer was in the nasal cavity where there was not much tissue to work with, so he used a synthetic material to add to the existing tissue in order to close the hole from the nasal side.  He then put in a layer of bone grafting, using donor bone – again, thank you to the organ/bone donors of the world.  The last layer was in the oral cavity.  There was enough tissue there without having to use anything synthetic, but not enough that the tissue wouldn't be pulled tight, making a possible opening in the future a known risk.

The surgery took about six hours and recovery was extremely difficult this time.  Gabriel was on a liquid/non-chew foods diet for two weeks and would barely eat or drink anything.  He also kept running fevers non-stop.  Not high enough to warrant possible hospitalization from infection, but enough to cause him discomfort and ridiculous amounts of crabbiness!!  Weeks three and four were spent eating easy-to-chew foods and things were getting better. 

Now, we are back to a regular diet with the exception of anything on a stick.  Halloween was interesting when we had to keep telling him what things he could not eat, but he has certainly made up for it by eating his weight in M&M’s since!  We expect that as of this Monday, we will be completely back to normal and on a 6 week break from seeing our surgeon!

In the meantime, we are still seeing some food come out of his nose, despite the hole being closed. This brings me to...

Reason #2 – I don’t know where to begin with this one.

Dr. S confirmed in this last surgery, that indeed, the soft palate is too short and we have no choice but to perform the Pharyngeal Flap procedure.  Well, we do have a choice.  We could opt to not do it at all and leave Gabriel ‘as is’. 

What does that mean?

Let’s review what we already know.  Gabriel’s speech is ‘unintelligible’ – which, by the way, is a term I hate.  Unintelligible, to me, is too close to unintelligent – which he most certainly is not.  What he is - is very hard to understand.  In fact, if you didn't live with him, you probably wouldn't ever figure out what he’s trying to say.  With two years of speech therapy behind us, we have not really seen any improvement in his speech.

When making certain sounds, such as ‘b’ or ‘p’, the soft palate has to close off the nasal cavity.  With Gabriel’s soft palate being too short, this closing off is not possible.  I have asked for our doctors to perform a nasendoscopy to see just how short and far off from closure the soft palate really is.   In a nasendoscopy, a scope is put in the child’s nose and the child is asked to make certain sounds in order to see what the palate is doing.  The hope is that if the soft palate is close to closing off the nasal cavity, then perhaps through growth and intense speech therapy, Gabriel’s speech can become intelligible.

However, the alternative is that the soft palate is nowhere near close.  We have been told by two speech therapists that if that is the case, all the speech therapy in the world will never help and we are better off bringing someone in to teach us sign language.  Since Gabriel is currently terrified of all doctors and has just been through quite a lot with his last surgery, we are going to give him a few months before we do the nasendoscopy. 

So, what if the soft palate is just too short?

Here’s where things get tough.  I've mentioned the risks associated with the Pharyngeal Flap procedure in a prior post.  Aside from the complication of not surviving the surgery, Gabriel would have a huge risk of having to live with severe obstructive sleep apnea for the rest of his life.  Additionally, in order to give him the best chance at improved speech prior to going to school, Dr. S would want to perform this procedure in 2015 – next year.

As you can imagine, Chad and I are as lost as two parents can get right now.

On one hand, I went to my niece’s choir concert recently and sat there falling into this complete sadness knowing that my Gabriel will never be able to sing with a choir if we don’t do this procedure.  He loves to sing – but unless you can pick up on the tune, you don’t know what song it is.  He will likely be alienated and not have friends because he can’t speak.  Of course, having the procedure doesn't guarantee his speech will be perfect either.

On the other hand, am I signing my child’s death certificate if we go through with the procedure?  I’m not trying to be dramatic, but that’s how this feels.  Our doctor has known of children who did not survive this operation.  Our ENT says that if Gabriel ends up with severe sleep apnea because of this procedure, his hands are tied and there is nothing he can do to help. 

This is truly a decision that one way or the other will impact the rest of his entire life.  How do we make that decision for him? 

Right now, I hate the naïve me who seriously thought everything would be ‘fixed’ with Gabriel before he was one and he’d never know about his condition until we chose to tell him.  How stupid could I have been?  And how many times does my child have to pay for whatever sins I committed?  Why, for once, can’t he just be a ‘normal’ kid with normal kid problems?

I could go on all day.

Instead, on this, the eve of Thanksgiving, I will leave you with these thoughts –

I am so thankful that Gabriel is so strong and tough and able to endure the challenges laid before him.  I am so thankful for the doctors and therapists who really seem to care about our baby and what his future looks like.  I am so thankful for my husband who, though we don’t always agree, puts me in my place when I start to spiral out of control with worry and obsession over all of this.  He is the half full to my damn near empty.  And I am thankful for all of you who provide me with an uncensored voice and at least the pretense that someone out there is listening.  

Pre-surgery Preparations

Tonight we are in the middle of our pre-surgery preparations in the Hillegass house.  As such, I thought we'd do something a little different and share some pics of how pre-surgery preparation goes.

First thing I do is take our pantry from this...

To this:

Why, you ask?  Following Gabriel's 7th surgery tomorrow, he will be on a very restricted diet.  For the first 2 weeks, he will not be allowed to chew anything.  Weeks 3 and 4 will be food that can be easily chewed (think mac & cheese).  Week 5 should be back to normal.

So now we have the largest amount of mashed potatoes, applesauce, pudding, yogurt, and ice cream.  We learned the hard way following the last surgery that if we don't hide the things he likes to eat, there is a tremendous amount of drama when we have to keep saying no.  Therefore, the pantry only contains Gabriel's diet approved foods.

And where did all the goodies go?

To the laundry room - of course!!  This is what the cabinet above my dryer looks like tonight.



In support of Gabriel's restrictive diet, I will be going on a 5-day juice detox starting Friday.  I figure I will not chew with him and be able to make extra juice to see if I can get some nutrients into him - since there is not much nutrition in a pudding cup.

Here's my new juicer.  I'm pretty excited!  Chad thinks I'm nuts.  

The last picture is the contents of our overnight bag for the hospital.  Luckily, we only live about 25 minutes from the hospital, so if we forget anything, it's not a long drive to come back.  

The contents of our bag include: a couple pairs of clothes (nothing that has to go over Gabriel's head since he will likely be swollen and sore), his blankie, his little furry friend, a puzzle for me, some puzzles for Gabriel, a Kindle for me (he's still using Chad's right now, so it's not packed yet), some chargers, some meds, and some diapers.  Yes, the hospital provides diapers and I suggest that if you are unfortunate enough to ever have a child in the hospital that you use their diapers and take any leftovers with you - because you WILL pay for them.  I have found, however, that sometimes the hospital is not prepared with diapers in Gabriel's size, so these are really just to be used in the event that happens again.

As for the rest of our preparations, we are just spending time with Gabriel and trying not to think about what happens in the morning.  Chad and I haven't slept well in the past week - which happens before every surgery.  It's amazing how it never gets any easier.  It worries me, and upsets me just as much now as it did when Gabriel was 7 weeks old going into his very first surgery.

At any rate - we will be up and heading to the hospital at 6:30 in the morning.  Surgery is scheduled for 9 am and should take approximately 3 hours.  Following surgery, we will be staying in the hospital for at least one night.  One very long night.  I can't wait until tomorrow is behind us and we are moving forward again.

So now...deep breath.

Hold...count to 10...

Exhale.

Ok.  

We're good.

Let's do this thing.

Lucky #7?

Could surgery #7 be the lucky last one?  

What is it the Magic 8 Ball says – ‘Don’t count on it,’ ‘Outlook not so good,’ ‘Very doubtful’?  Well, I suppose any one of those would work.

On October 22^nd, we will be taking Gabriel to the hospital for his 7^th surgery (that’s if you include ear tube replacement to be a surgery – which I do). 

Let’s back up, shall we?  Last Wednesday we took Gabriel to the Children’s Craniofacial Clinic to meet with our Cleft Team.  If you remember correctly from my last update, we had very serious concerns with a procedure Dr. S was proposing called a Pharyngeal Flap.  We expressed that we weren't positive the rewards of the procedure currently outweigh the risks, and thankfully, our Team agreed.  We will be waiting to see if the procedure should be done in the future when we are certain it is 100% necessary.

So why are we still have surgery in October then, you ask? 

2 reasons –

Way back in one of Gabriel’s earliest surgeries, Dr. S bone grafted the opening in the left side of Gabriel’s gum line to close it using donor bone.  He was not able to graft the right side at that time because the opening there was just too big.  Instead, he filled the opening with a synthetic gel-like material and closed the tissue around it until such a time he felt the opening had come together enough to be grafted.  That time, apparently, is now.

The second reason is that darned fistula (hole) that just refuses to stay closed.  Dr. S is still not confident he can close it since he maintains that it is the biggest fistula he’s ever seen in the largest cleft he’s ever worked on.  However, I maintain that even if he can make it smaller – that’s a win.  Chad doesn't quite agree.  He thinks putting Gabriel through another surgery that results in the hole just opening up again is not a surgery worth having.

This has been a tricky situation and, for the first time, we don’t necessarily find ourselves in agreement.  I can certainly understand and respect Chad’s stance.  He doesn't want Gabriel to experience pain, have more reasons to fear hospitals and doctors, and suffer through another month of having to eat nothing but mashed up foods.  On the flip side though, my stance is that the more we do now, the less likely he will remember any of this in the future.  Plus, even if the hole opens back up, it should at least be smaller, which means we have a better chance of getting it to stay closed next time.  

The truth is – it’s never going to be the right time.  It’s never going to be OK.  Even if the fistula is successfully and finally closed this time, Gabriel’s palate is still too short.  He may still need a jaw realignment in the future.  He will likely have dental implants.  He’ll need his tubes replaced in his ears.  And he may even need a nose job and/or lip revision in the future.  We are a long way from done, and there will just never be a good time.

I was getting coffee at work the other day and talking with the barista about Gabriel (she has been following his progress since birth).  She told me that she has a baby cousin who just had his third and final cleft repair surgery.  I was extremely excited for the little fella, but found myself once again in the land of pity for my beautiful Gabriel.  

I don’t suppose I’ll ever stop hating what’s happened to him and questioning why this can’t just be over and he can live a ‘normal’ life.  But I also don’t suppose I’ll ever have answers to my questions, so I’ll just have to keep trying to destroy my hate with hope.

Until next time…

Wow - Has it really been that long???

So, I was driving today and thinking about how much I should update our blog.  After I lost said blog and then had to find it, I realized just what a horrible person I am.  I have not written a post since January?!?!?  How can that possibly be???  Things have been happening.  It's not like life suddenly got boring.  I guess, rather, the time just got away from me.  If you still care, and take the time to read this - I'm sorry.  As things are about to get interesting in our lives, I can assure you, my next post will be within one month - not eight.

I could write the world's longest update with what has happened in the past eight months, but I won't.  Instead, I'll hit the major points and promise to write more soon.  Yes, I realize I've promised this before...

In my last post, I mentioned that Gabriel would be attending a Children's Craniofacial Clinic in March.  As it turned out, we got dumped on by more snow that day and Gabriel threw up all over Chad's backseat while waiting in the drive-through at McDonald's.  So even if it wasn't for the snow, the puke definitely had us turning around and going home.  Our Clinic appointment was then rescheduled for May.

In May, with no snow and no vomit, we made it to the Clinic.  This is how things went down:

Speech is not very good (shocker...).  We were told we could increase therapy to 2 sessions a week, should he tolerate it, and keep pushing sign language as much as possible.  After discussing this with his speech therapist, we decided to not increase his sessions at this time.  Gabriel will be in speech therapy for many, many years to come, so we really do not want to burn him out and make him hate it already.

Dr. S indicated that Gabriel's soft palate is still too short.  He said he would like to do surgery in six months to a year to both lengthen the soft palate and try (again) to repair the hole in the front of the palate.  Sounds simple, right?  It's not.  Unfortunately, after 3 semi-successful repairs, Gabriel has run out of usable tissue to lengthen the palate with, which leaves Dr. S with no choice but to perform a procedure he deems his last resort.  This procedure will take tissue from the back of Gabriel's throat in the form of a flap that will be attached up to the soft palate, effectively closing off the nasal cavity from the back of the throat.  Then, two holes would be made in this flap that will allow for drainage.  This procedure is a last resort because it comes with the 'major complication' of oftentimes causing 'severe sleep apnea' since those two drainage holes will frequently become plugged.  Especially if Gabriel continues to suffer from chronic sinus infections and drainage issues.  

Chad and I have discussed this at length and, honestly, we are still not sure what to do.  On one hand, if the soft palate is not lengthened, there is a distinct possibility he may never be able to speak clearly or without sounding very nasal (think Fran Drescher - who, by the way, because rich and famous off her voice).  Plus, we can't be sure all the food that still comes out of his nose is solely from the hole, and not coming up from behind the palate, so he could always be potentially grossing people out at the dinner table.  However, on the other hand, my child could have sleep apnea - which means always worrying about whether or not he will die in his sleep.  We have talked about possibly just waiting until Gabriel is older and can decide for himself if it is worth the risk.  I don't want my baby to not be able to use his beautiful voice, but I also don't want to have him have to take his Cpap machine with him to sleepovers or have to remember to pack it when moving away to college. This is the first time we have had to make a decision in this process since the rest were no brainers and, quite frankly, it sucks.

So, what do we do?  Well, we have another Clinic to go to on September 10th where we will talk at length with his doctors about when they think the surgery should happen and when it has to happen.  Hence my update coming in a month!

In other news, we knew Gabriel's teeth were going to be a mess, but we had no idea we'd suffer from that so soon.  I took Gabriel to see his dentist about a tooth that was broken after one of his surgeries.  That appointment led to a tooth extraction and three crowns - 3 CROWNS!!!!!  Do you know how much the tooth fairy has to pay for silver teeth?!?!?!  Needless to say, my FSA has taken a big hit this year.

Additionally, Gabriel is about to age out of First Steps.  Starting in December when he turns three, he will be attending public preschool for special education children five days a week.  He will be at the same school as Ethan, so Ethan's pretty excited!  I, on the other hand, am not excited.  It's one more piece of proof my baby is growing up.

With all that I'm going to call this update done.  I have to leave my boys with Chad for a few days while I do some traveling for work, so I probably ought to get some snuggles in before I go.  I will be in touch with all of you again in September with news of the next surgery.  Until then...

New Year, New Us!

Ok, not really new us.  Same old us; but a new year nonetheless!

So, what’s happening in the land of Hillegass, you ask?  It’s snowy, cold, and completely uneventful – for the time being anyhow.

Since I haven’t updated you on Gabriel’s last surgery (sorry), I’ll start with how that went.  It went…fine.  Just fine.

His soft palate repair was successful.  It seems to be holding well and hopefully will continue.  As a result, we have seen less and less food coming out of his nose – which is great!  Not great, however, is that the hole in the front of his palate remains.

Dr. S did take a good look at it and determined that it’s just too big to do anything with right now.  He thought he could possibly finagle a fix during the last surgery, but he had no confidence the fix would hold and then we’d likely be burning bridges we are going to need to cross further down the road.  He wants to be able to do some serious research before attempting to close the hole, but did warn us that (gasp!!!!) some kids just have to live with a hole in their palate!!!!!

NOOOOOOOOOOOOOO!!!!!!!!!!!!!!!!!!

Yep, that was the reaction in my head.  Living with a hole in Gabriel’s palate means 1) he will always require some device to try to plug said hole, 2) he will always have food coming out of his nose, 3) he will always have chronic sinus infections, and 4) he will likely never be able to speak in a fashion anyone will be able to understand.

Yeah, that 4^th one is a deal breaker.  It’s bad enough that he has been doing speech therapy for 6 months now with little to no improvement, but you mean this might be forever?!?!?  I don’t even know where to begin with that.

Gabriel is extremely smart.  I know, everyone says that about their kid, but seriously, he is.  I worry constantly that if he can’t speak and has to rely on sign language as his primary communication, then what happens when he goes to school?  Can he be with other kids his age?  Can he even go to public school?  Last I knew the average teacher didn’t know ASL (American Sign Language), so where does that leave him?

Currently, Gabriel knows upwards of 50 signs.  Believe me - even though that’s a lot for a 2 year old, it is not enough to keep him from screaming at you when you don’t understand what he wants!  His speech therapist has now decided to include sign language in with her sessions.  She doesn’t want him to rely on it and not try to keep working on his speech, but she also knows the reality we face, which is, we need to be able to understand each other.  Funny thing is I always thought I’d be learning Spanish with my kids – not ASL.  At any rate everyone he comes in contact with is supposed to be asking him to sign and try to say everything.  We all have a very long road ahead of us.

One thing I’ve been thinking about is wondering what I tell people in public when they continuously try to get him to talk to them.  This happens all the time.  I don’t want to say that he can’t talk – because he can.  He just can’t be understood.  At the same time, I don’t want them to think there is something ‘wrong’ with him – because there’s not.  I just don’t know.

In the meantime, Gabriel will be taking part in a Children’s Craniofacial Clinic on March 12^th in Fort Wayne.  Many children do this from birth, but it will be Gabriel’s first time participating.  Basically, he will sit in a room and over several hours, surgeons, orthodontists, pediatric dentists, speech therapists, dieticians, and other various folks will file into his room to check him out.  He’s going to hate it.  However, the end result is that they will all then get together and discuss what they think the next steps for him should be.  Until then, we wait.  We hope the soft palate continues to hold, we teach and learn as much ASL as possible, and we try not to think about things too seriously.  I have a very strong feeling that if I stop to think about where we’ve been, where we’re going, and all the unknowns, I might start to cry and never stop.  I can’t be that person for my boys – and I won’t.

As you know, we recently took the boys to Disney for Thanksgiving, so I have included some of my favorite pics from that.  I hope you enjoy them!  Until next time…