Just like we knew they would be- the nasal stents have been an issue. 
Because of his size and the fact that (due to his age) he ONLY knows how to breathe out of his nose, having nasal stents requires a lot of work to ensure they remain open so he can breathe. 

Most of the day yesterday was spent trying to diagnose and treat his persistently high heart rate (like >200)… most of his labs (thankfully) back normal and dehydration was the likely culprit, given his ongoing GI distress. Fluids helped his heart rate for a short term but never for long. Yesterday evening, I noticed his breathing start to look very frazzled and almost panicky- but since his stents were patent and unoccluded, we assumed pain was the cause.

When ENT came by this morning, they noticed the same issues with his breathing and decided his stents needed to be switched out. The placement of the stent seemed like it was actually occluding his nasal airway in a way. Because of his abnormally shaped mouth/jaw, his tongue often completely occludes his throat as well- so this was leaving him with limited breathing options… the high HR was likely a compensatory mechanism to help maintain oxygenation. His stents were switched to something longer, so that it goes down deeper and doesn’t allow his tongue to block them off. This seems to be helping for the time being. 


This condition that John has is extremely rare. The incidence for PAS is 1 in every 25,000 births.
For comparison: 
Down syndrome is 1 in every 1,000 births. 
Cleft lip: 1 in every 700. 
Trisomy 18: 1 in every 8,000. 

For all these reasons, there isn’t a TON of research and tools to best manage PAS. We are SO FORTUNATE to live near a large children’s hospital- where the ENT doctors are somewhat familiar with this condition. Even so, these doctors still only treat and handful of kids with PAS in a given year. John’s case seems to be one of the most difficult, complicated ones. Every plan we’ve made has had to be adjusted and the things we hope will work just haven’t worked that well. 

For a planner like me- it’s hard to not get caught up in the long term. But the reality is- his entire life will come with so many more battles. It’s possible he will have developmental/intellectual delays. His mouth/jaw will also require surgery down the road. Because of his breathing issues, he’s been unable to drink from a bottle for the majority of his life. He will require lots of feeding therapy outpatient in hopes of avoiding an oral aversion. His feeding tube will likely remain in for months. The light at the end of the tunnel which once was in sight, is now gone, and many many miles away. Nothing has gone according to plan and probably won’t for a while. 

It can get very lonely in the hospital room and im left with a lot of time to think. (Which isn’t necessarily good!) and I keep realizing that there’s so many hard things about this journey. 
For starters- it’s hard because life doesn’t stop for my sick kid. The world keeps spinning, I’m still battling an allergy headache, I still have a toddler at home that needs normalcy and love and attention, the bills still come, the car still needs serviced, birthdays still pass, other people still have babies. 
It’s NEVER hard to be here and show up to support John- it’s easy to change his diaper, comfort him, and snuggle him when the time is right. It’s always hard to hand him over to a team of doctors- watch him be wheeled into the OR, not knowing what goes on, and being helpless and out of the loop for hours at a time. It’s hard to hear him scream when he needs a new IV and watch bright red blood pour out of his nose and mouth. It’s hard to leave him at night, never knowing what I will walk back into the next day. It’s hard because this journey is completely out of my hands and as a mother to a newborn- that’s truly heartbreaking. 


Something I’ve really struggled with is guilt. 
I feel guilty asking for help. I feel guilty that I haven’t been present for Collins. I feel guilty for missing birthdays. I feel guilty for being bitter about everyone else’s healthy babies. I feel guilty for feeling guilty. I feel guilty for sleeping too long and not getting to the hospital until 9AM. I feel guilty when I leave. I feel most guilty when I start to feel bad for myself, because I know many people that are fighting harder and longer battles with their own sick kids.

If you’re still reading this- know that it is your thoughts, prayers, love, texts, and kind gestures that are keeping me on my feet. It’s the one thing keeping me going and giving me the energy to show up for John. 

Andy and I are so tired, frustrated, and worried. We are equally comforted, supported, and hopeful because of all of you. 

No words can truly express our gratitude, so we will have to settle for a thank you.