The Latest Broncoscopy

We took Luke in this morning for Dr. McClay to have another look down his airway.  This was supposed to be Luke’s decanulation day, but Luke has been struggling quite a bit with the cap, especially for naptime.  We haven’t been able to get him to fall asleep with the cap on or cap him at all when he’s sleeping.  One of the requirements for getting Luke’s trach out is to have him pass a sleep study with his trach capped.   So we arrived at Children’s Medical Center at 7:00 this morning with no expectations for decanulation, but hope for the doctor to be able to give us an explanation for why it’s been such a battle to get Luke capped around the clock. 

They finally came and took Luke back to the OR around 9:30.  They called David and me back to the consultation room about 30 minutes later.  So here’s the scoop:

There are two possible physiological reasons why Luke is struggling so much:  1) There is a bit of floppiness in the airway tissue below the area that was reconstructed in May.  This floppiness could be causing his airway to constrict somewhat.  2)  Scar tissue on the vocal chords could responsible for constricting the airway somewhat.  Vocal chords open when you vocalize and close when you breathe.  Scar tissue on his vocal chords is causing the airway to be more narrow than usual, so this could be why Luke can vocalize very strongly but can still have difficulty in the breathing department.  Good news is that there was no granulation tissue present in the airway for Dr. McClay to remove (that’s a first).  There was a portion of tissue in the airway that looked suspicious, but McClay believes that this is a small area still swollen from the frequent surgeries/broncoscopies rather than granuloma.  In any case, it isn’t obstructing his airway enough to cause him difficulty. 

So what’s the plan?  First, more waiting.  We’re going to wait one month to see Dr. McClay in his office where he’ll “soft scope” Luke to discern the amount of damage to the vocal chords that scar tissue is causing.  It’s difficult to tell while Luke is under anesthesia, so a soft scope done while Luke is awake should give us more information.  Meanwhile, we have the go ahead to push Luke a little harder wearing the cap.  The hope is that air pressure created when Luke uses the cap will over time strengthen his airway and resolve the floppiness.  No one can tell us how long that will take. Again, we’re on Luke’s timeline.  Part of our problem has been wondering whether Luke throws fits when we try to cap him because he’s really having a difficult time getting enough air, or if he just doesn’t want to do it, so he cries to make us take off the cap.  Because we’ve been fearful of depriving him of air and harming him, we usually relent rather than making him cry through it.  We feel better about pushing him harder now that we know pushing him will only make his airway stronger and not torture him. 

Obviously the hope is that using the cap will help strengthen his airway, and the only place we have to go from here is up.  Stronger airway = more comfort with the cap = passing sleep study = decanulation.  We have the rest of July, August, and probably most of September before we hit a deadline.  McClay told us today that he won’t decanulate in winter.  If we can’t get it by September, there’s another option.   McClay talked a bit about a surgical procedure he can do to correct the floppiness.  This would take place in the spring and would result in decanulation during the same procedure. 

Realistically, we’re looking at possible decanulation later this summer/early fall.  It’s possible that we’ll have to wait another several months until the spring.  Meanwhile, we’ll most likely be getting to take Luke home to sleep in his own bed tonight (and us on ours!) and start with capping bootcamp again tomorrow. 

Please pray with us that we can be successful in capping, passing a sleep study, and decanulating by September.  Every day we long for the time Luke’s trach can come out, and we pray that we won’t have to wait another 8- 9 months before it can truly happen for our family.  Please pray that God gives us as Luke’s parents wisdom in pushing and challenging Luke to wear his cap without harming or discouraging him.  More than ever, we need to be firm, disciplined, and loving parents to this vibrant and willful two year-old. 

Thank you for your prayers and for waiting with us.  We truly love all of you.

Blessings,

Rachael, David, and Luke 

Saturday

It looks like the culprit for Luke's "now-you-see-it-now-you-don't"​ fever and illness Wednesday night can be attributed to that pesky top left molar choosing to make it's appearance. I think we'll be a go for Monday thanks to some Tylenol and your prayers.

Thursday

We seem to have hit a snag... Luke threw up twice last night and started running a fever about 4:00am. It seems to have gone down with Tylenol, and I just gave him formula. We'll see if he keeps it down. We need this to got away NOW so that we can continue with the next step in the process on Monday! Pray, please!

Another Issue?

It’s been awhile since I wrote a long post, so I want to ask you all for your prayers in the upcoming “Luke events”. 

We are nearing the end of what our ENT calls the “LTR (Laryngotracheal Resection = airway reconstruction) Protocol.  This protocol has included one major airway reconstruction surgery and several broncoscopies so far.  The point of this protocol is to enable us to cap Luke’s trach and ultimately get the trach out (called “decanulation”).  Based on the stories we heard from our ENT surgeon and one of our home health nurses, we expected the capping process after the LTR to be relatively smooth and easy.  This just hasn’t been the case.  We were able to cap Luke’s trach just a few days after the stint was removed from his airway on June 6.  In the past month, we’ve been able to get Luke to use the cap for long periods of time, but it’s been anything but smooth or easy. 

Luke often makes disconcerting “honking” and wheezing noises when breathing through his nose and mouth while his trach is capped.  He doesn’t always make those sounds, but they have troubled us.  He frequently pulls at his trach to communicate his discomfort, and has pulled out his trach or pulled it apart more than once, forcing us to do an emergency trach change and causing more concern from his parents.  When Luke has pulled out/apart his trach he panics and turns blue from lack of oxygen, which should not happen at this stage in the process—he should be able to get enough air by breathing through his nose and mouth with the trach non-functional.     

(After much going back and forth) We have a sleep study scheduled for Luke at the end of this week to see how he sleeps breathing with his trach vs. sleeping with his trach capped.  David talked with the ENT surgeon who explained that unless we can get Luke comfortable wearing the cap all day and at least able to sleep with it for some time during his naps, there is no reason to perform a sleep study at this time.  I think it goes without saying that Luke has to pass a sleep study in order for us to decanulate.   Luke absolutely panics when we lay him down to sleep with the cap on.  I have gotten him to fall asleep a handful of times with the Speaking valve (PMV)/cap hybrid (it’s a PMV with gauze taped over the opening to create more resistance when he takes a breath), but it’s a huge battle every single time.  Our attempts at getting him to fall asleep with the cap on, or trying to slip it on him after he’s asleep have failed miserably and ended in him panicking.  In addition, anytime we place the cap or the hybrid on him during sleep, his o2 sats decrease significantly. 

We have patiently increased the time he wears the cap by an hour each day, hoping that it’s a matter of him becoming acclimated to the cap and strong enough to breathe without the trach, but it hasn’t seemed to help on the sleeping front.

This brings David and me to believe that we are dealing with another, unknown, airway/pulmonary issue.  We don’t know what it is, but the ENT has agreed with us that it “should be easier than this.”  After working up to 9 hours wearing the cap with no results on the sleeping front, David and I are discouraged to say the least.  I hate torturing him by restricting his breathing, so our plan now is to again stop pushing hard and for the most part let Luke dictate when he wears the cap and when he doesn’t (within reason).  

Our original schedule has been to do a sleep study July 8 (Friday) and then a final broncoscopy and decanulation on July 18.  This no longer seems feasible, as we must figure out what this new unresolved issue is that is causing Luke such difficulty with using the cap.  We haven’t canceled the sleep study yet, but will likely do so after we talk with Luke’s ENT probably Wednesday if he cannot sleep with the cap on by then.  We are eager for this upcoming broncoscopy but no longer because we expect to decanulate Luke at that time.  We are eager because we are praying that God will give Dr. McClay wisdom as to what is causing our precious little boy to not be able to breathe on his own yet.  It could be that Luke’s airway has formed scar tissue or more granulation tissue.  It could be that the airway simply needs to be re-dilated.  Or it could be something completely off our radar that’s about to hit us out of nowhere. 

Honestly, I’m so tired of new issues (and the trach) that I could scream.  We just want to get this resolved as soon as possible so we can get on with our lives.  Please pray with us that whatever this issue is, it can be resolved easily and swiftly and result in Luke’s decanulation, if not July 18, then sometime by the end of the summer.  It will be very tough for us if we have to wait yet another year for Luke’s decanulation. 

We also are working on resolving a few (relatively) minor problems with other systems in Luke’s body.  In January, Luke’s neurologist referred us to a pediatric ophthalmologist because she thought she saw the beginnings of a lazy eye.  We visited the ophthalmologist this morning who had no concerns of that nature, but referred us to somewhere called the Retina Clinic to check out the slight nystagmus lingering from Luke’s hyponatremia (very low sodium level) last spring.  It’s imperceptible to us, so we’re not extremely concerned about this issue right now.  I’m relieved and thankful that we won’t have to deal with the headache of patching Luke’s eye to treat a lazy eye condition. 

We’re visiting a dentist tomorrow because it seems one of Luke’s lower molars seems to be growing in sideways.  It doesn’t seem to be causing him discomfort, but we don’t know how big of a deal to make of it.  So we’re seeing a dentist tomorrow.

Finally, after Luke’s last broncoscopy, I noticed the top knuckle on his left thumb doesn’t bend at all.  It’s fixed, and I can’t straighten it by applying pressure (as much as I dare).  I’m working on scheduling an appointment with an orthopedist to work on this problem. 

Besides working on the cap, Luke is doing great.  He now weighs about 25½  lbs. and is 35 in. tall.  He’s been walking since just before his second birthday.  He’s now walking everywhere he goes and can stand up on his own about 30% of the time.  He loves to climb.  His favorite activities include playing with Legos and opening and closing doors and drawers.  He loves letters and words.  Whenever he sees any kind of animal he enthusiastically signs “Cat”.  He loves making friends, singing “Itsy Bitsy Spider”, and taking a bath.  He still tastes everything.  He loves to throw things. 

Listing these stats gives me joy and lifts my mood.  But honestly, most of the time lately I feel very discouraged.  I make every attempt to count my blessings and give my discouragement to God to take care of, but it’s difficult after fighting these battles for so long.  I’m aware that there are people out there who have dealt with medical problems even bigger than Luke’s for much longer.  But standing here in the middle of it, it usually seems insurmountable.  It feels like we’re never going to get this trach out.  I’m tired of getting my hopes raised only to have them crash down once again.  I’m scared to hope any more.  I’m weary of dealing with the medical system. 

We’ll keep plugging along though.  God’s grace is sufficient for us.  More than anything, I want God’s timing.  We need patience and endurance from him to get us through these next days, weeks, months, and years.  Thank you for praying with us and for us.  Thank you for your words of encouragement.  For those of you who deal with us in person, thank you for your patience as we figure out how to raise this very special toddler.  Our biggest prayer is that any who encounter our family see the One who loves and sustains us. 

Blessings,

Rachael, David, and Luke

Sunday

Luke did 8 hours with the cap today. It's seems to be getting easier for him, but he's still not able to sleep with the cap on. Still having a hard time going to sleep and sleeping for more than 30-40 min with the PMV/cap hybrid. Praying we get him comfortable enough with the cap to complete and pass the sleep study Friday night.

Friday

Luke did 6 hours with the cap today. Talked to the doc b/c we're meeting more resistance than we expected. He says that Luke must wear the cap comfortably all day or there's no point in doing a sleep study. Good news is that even if we don't make the 7/18 deadline, there's still lots of summer left to decanulate. We'd love to have your prayers to get his trach out on the original date.