Yesterday

Yesterday was a hard day. Too hard to get on and update so I'm sorry for everyone who was waiting to hear how things went. Neither Dave or I felt very much like sending out updates.

We know Paxton would be able to be extubated... if his anatomy could only support his will.  The one good thing that came from this procedure was that we learned more about Paxton's conditions and how we need to proceed in the future.

It was very hard for the doctors to see much of anything at all down his airway, etc., with the scopes  They said it was just so small and so many secretions that they had a difficult time getting a perfect look.  They did learn that Paxton also has a laryngeal cleft, which would make it hard for him to eat or even swallow on his own without having it go down the wrong tube and possibly asphyxiating himself.


(A laryngeal cleft or laryngotracheoesophageal cleft is a rare congenital abnormality in the posterior laryngo-tracheal wall.^[1] It occurs in approximately 1 in 10,000 to 20,000 births.^[2] It means there is a gap between the oesophagus and trachea, which allows food or fluid to pass into the airway.  

asphyxiation - the condition of being deprived of oxygen (as by having breathing stopped); killing by depriving of oxygen.)


Below are some diagrams. Paxton has no palate at all, a large tongue not positioned to the bottom of his mouth, a laryngeal cleft, and possibly no epiglottis or a severly malformed one (helps you to swallow, clear throat, stop food from coming up into mouth, etc.); the drs could not see it in this exam. His laryngeal cleft is a grade 2 (grade 4 is not survivable) and the doctor thinks he can repair it, but not until Paxton is much bigger, possibly a year old. 

(Larynx and trachea lead to lungs; esophagus leads to stomach.)

Immediately after they extubated Paxton the same process occurred that happened very much in the fist 24 hours of his life: his breathing stopped, turned blue, nurses put oxygen masks on him, trying to stimulate him and open his airway, etc. It was obvious with all of this that he needed to be reintubated. Even with one of the top 3 ENT specialists doing the procedure, it took 5 tries again for Paxton to have the tube put back in. 


When we got up to see Paxton he was so worn out. You could tell he just did not want to be bothered and poked any more; he was MAD. And in pain. His fever was racing 38.3 (normally 36.5ish), his body was bright red, and he was posturing and pushing out his whole body like he does when he is in pain or really upset. He was also shaking his limbs a lot and retracting his stomach to breath very strenuously. Dave and I were really worried, wanting to know how we could help him and if there was another reason he was so mad. Finally our nurse asked us to leave, to go eat lunch (now 2 pm), so we reluctantly and weakly did. We had just had some pretty hard and overwhelming news so Dave and I were both pretty down but trying to comfort each other. When we came back from lunch we learned something had definitely been wrong with Paxton.


While we were gone Paxton had had an X-ray to check the intubation placement. Because the doctors had tried to put the tube so far down in his airway to avoid the cleft (so the tube wouldn't fall through the cleft, from his trachea into his esophagus), the breathing tube had been pushed into one side of his lungs and the other lung had been getting no oxygen. Dave was there when they repositioned it. After that process Paxton's temperature, color, and demeanor began to return to normal. However, he was still very sick of being bothered. I tried holding his hands and relaxing them down onto his body to calm him, but he would not lower his fists. He was literally in "fight" mode, "stands with a fist". 


3 hours later one fist had fallen to his side, but the other was still in the air, less tight. Poor guy has just had too much in one awful week.

So what this all means for Paxton's future is where the update gets hard. Basically Paxton does have a number of surgeries ahead of him, but most of them not until he is older and bigger. Through this process the doctors basically ruled out doing two surgeries they thought might help earlier on: the tongue adhesion (sewing tongue to bottom of mouth) or distraction surgery (making a slit in his jaw and seperating the jaw apart 1 ml / day to have a cm or more of growth in 2 weeks, to try and make more room for his throat).  They learned that what Paxton needs to be able to bypass all the problems in his airway, is a tracheostomy in his throat to breathe, sometime this next week. The doctors are also evaluating his GI to see if he should have a G-tube (feeding tube) surgically implanted into his stomach or place one down his nose (not giving his throat one more thing to deal with going down it). 

What was more difficult to hear was that most kids who need to "outgrow" their airway issues have a trach in for, on average, two and a half years. With Paxton's possible delayed growth in all areas, it is more than likely it could be five, or more. 

The most difficult part is when the doctors have said something to us about "and then you really have to consider what is best for your child." 

Dave and I have been completely flabbergasted by anyone implying anything like that at all.  To us it is not even a question. We want Paxton to be with us as long as God allows! We want that to be His decision, not ours. However, I pray that if it were to ever come to that that, the Lord would bless us to know clearly His will for Paxton, and what his sweet little heart would desire most. Thankfully that is not something we have to consider right now as Paxton still has so much hope!  His MRI looked so promising as to his possible development and interaction with the world. Also the fact that he does not have to be on a "trach with vent", meaning he will hopefully be able to breathe on his own and not with machine or oxygenated help, is great! Doctors say they do caution parents on that when you consider children who do not eat, breathe, interact, speak, move, etc. by themselves. We hope and pray we are so far from that. But you can see how hearing that several times can sort of rock your world and shake your peace a bit. At least it did mine.

Everyone is telling us it really is better for Paxton to have this surgery. It will be good to get him off the ventilator to his lungs (lots of possible risks the longer you are on it: lung disease, lung collapse, infection, pneumonia, etc.). We are also told we are actually closer to getting him to come home with this procedure, where hopefully he can thrive and grow more, and be held more. But it is obviously scary and sad. Dave and I are nervous to learn how to change it (at least weekly), clean it, keep it humidified, listen and watch for clots (would suffocate him, etc.). Apparently, and thankfully, we will have loads and loads of training on this before we would be able to bring him home. He is still no where near that but this is the first step, figuring out his breathing. Next will be feeding, then a certain amount of growth, stability, more echocardigrams, ultrasounds, xrays, eye surgeries, etc. before that is possible.  

We can't thank you enough for your prayers, and understanding. We are pretty much in a different world right now and thank you for your service, your messages, your efforts to reach out to us, to love our children as we would in our absence, and even all that without us being able to respond very much right now. We are both truly exhausted, and trying to make some time for Camden and Mackay each day, too, amidst the traveling.  It's so much more of a rollercoaster than we could have ever imagined, with so many unfulfilled wishes and moments of pain than we hoped for. In saying that, we feel the blessings of the principles of the gospel, and pure friendship, so deeply. We don't know where we would be without those things supporting us. So thank you. We know your lives go on but are humbled at the continuity of your unfailing love and prayers for our family. We feel and appreciate them so dearly. Thank you and all our sincerest love. 

Love,

Dave, Shannon, Camden, Mackay, and Paxton