Well, we got the final, official news on Wednesday that - despite all interventions - Job is still aspirating on extra honey thick liquids. He is absolutely unsafe to swallow liquids.
It's the best way to keep his (already impaired) lungs safe from respiratory infections developing into pneumonia. But we're devastated. I've been sobbing every few minutes for three days now. It feels like such a huge step backwards, I guess. It's also going to be a lot of work - though I've been doing oh so much work calling providers and pharmacies and taking him to so so many appointments and staying with him at the hospital for surgeries these last six weeks.
The long version:
[As I often function as Job's medical historian it helps me to write out all the minute details so I remember them better for future appointments. The following isn't even interesting to me! So feel free to disregard.]
Job has been on thickened liquids since we was 6 months old and even then always from a slow-flow bottle nipple. Until he was 3 years old (spring 2019) his cardiac condition was always so tense that we didn't have the ability to focus much on improving his swallow. We knew that his left vocal cord didn't function because the nerve to it had been damaged during his first surgery at 5 days old but hoped, at least for the first year or maybe two, that the nerve would heal. His diaphragm was also affected and he had a plication at one month old. He eventually got off oxygen but needed a feeding tube for his first few months of life and we were finally, after a lot of work, able to bottle feed. It eventually became clear that his swallow wouldn't improve significantly but by that time we were so focused on just keeping him alive that nothing else mattered.
But during transplant (fall 2018) his vocal cords were damaged during intubation. When he woke up he was completely aphonic and couldn't swallow at all, so I said I wouldn't leave the hospital without an Otolaryngology consult and, hopefully, intervention.
Oto suggested gel injections into his left vocal cord to "plump" it up and bring it to midline so that the right vocal cord could better meet it and make his voice stronger and help him swallow better. And it worked! We were really excited!
Then in March 2019, since he was almost 3 and still not able to make many verbal sounds, we started speech therapy. I vividly remember going in that morning for our very first appointment and then coming home and giving him lunch and putting him down for a nap. I was so excited to actively begin working towards the possibility of actual words!!
And then Job woke up from his nap and couldn't make noise and was struggling with his swallow. Oto had said that the gel injections would expire but I didn't expect it to be so very dramatic!!
We started working on scheduling new gel injections, but then he got back to back colds requiring not just back to back hospitalizations but back to back ICU stays. So new gel injections got pushed ahead until June 2019 until he had sufficiently recovered from his respiratory infections to be safe for anesthesia. But we also saw Pulmonology in the hopes that they would have suggestions. They were shocked he wasn't already on a G-tube (basically a hole in his stomach to put fluids in), which, honestly, was pretty off-putting to me and might be part of why I haven't been back for a six month or year or 18 month follow up with them... But probably a bigger part of the reason is that Job was so very healthy from March-October 2020 and I was very happy to avoid non-urgent hospital appointments.
His June 2019 gel injections were a different formulation than his previous injection, meant to last 9-12 months and they did! But his surgeon told me about a brand new surgery SCH was pioneering for children after it was successful for adult patients at UW. It took a while to coordinate with the UW surgeon who had to come over and partner with Job's Oto surgeon, but but in February 2020 Job had his recurrent laryngeal nerve reinnervation surgery.
The goal here was to potentially plump up his left vocal cord permanently so we wouldn't have to do gel injections over and over. Since it takes about 12 months to see results from this procedure he got another round of gel injections to hold him over until the nerve reinnervation might take effect. We were so excited that he got it done just before COVID hit and elective procedures were tabled.
We had such a blissful spring and summer from a "Job's health" perspective. Our therapies all went to online and We just had May and August cardiology appointments. Did you read that? IN SIX MONTHS WE JUST HAD TWO APPOINTMENTS at SCH. TWO APPOINTMENTS. (But don't worry: we more than made up for it this fall with at least one if not two appointments a week up there since October 18th. Oh, and three hospitalizations.) Job seemed so very healthy and happy! We've NEVER had such a respite from his medical needs. So many other aspects of our life were falling apart but Job was thriving!
Until he wasn't. His gel injections expired in mid October and wow, what a mess it has been since then.
A few days before his scheduled annual biopsy on October 18th Job's baseline cough changed into something wetter. Ezra had been feeling a bit strange so we thought it was just a cold. He was hardly symptomatic so the team said to bring him in for an assessment anyway and we'd decide that morning whether or not to proceed with the biopsy. Anesthesia felt uncomfortable with how wet his lungs sounded morning-of, so we came home and rescheduled his biopsy for November 19th to let him recover from his "cold". Except he never did. He cough got worse and worse. His lungs got wetter and wetter. Cue work for me, like appointments and phone calls and med changes, plus just being on high alert, but we weren't super worried.
I think we were all so expectant that his nerve reinnervation would fix everything that it really wasn't on my radar that his gel injections expired. Until we went to his VFSS (where he swallows foods and drinks with barium in them on x-ray to assess his swallow) on November 6th. He had seemed so healthy all summer and we were so hopeful that he would "graduate" to a thinner liquid or *gasp* something-other-than-a-baby-bottle!
Instead the study showed he was aspirating on honey thick liquids. Cue so so many appointments and phone calls and tears. We were allowed to go home for the weekend without intervention but everyone expected stuff would start happening Monday morning. But it didn't. I was calling Oto a couple times a day that week, trying to get the ball moving. His lungs sounded awful to me and I was getting really stressed out. We also kept needing to do COVID tests for each appointment/procedure.
Job has had some terribly painful medical interventions throughout the years, but always with anesthesia. His various PCR tests have always been the most emotionally scarring part of his care but until this COVID season we didn't have to do them very often. He has now had a total of SEVEN of them this fall. SEVEN. It's been awful. So awful that I've thought about making some of my decisions for his care based on whether or not we need a COVID test or not.
I have always been satisfied with Oto's care for us at our appointments/surgeries. I thought it was a little frustrating that I had to verbally walk his Oto surgeon through Job's "file" each time (thus why I have to remember all of this so intimately) and that the nurses and schedulers didn't call me back same day as I expected (like the Heart/Transplant team does) but it wasn't that big of a deal, was it?
Until he got worse and they still weren't calling me back. So I called the Transplant team and they called Oto and I got an appointment with Oto on November 13th. We showed up and Job merely walked into the room and his surgeon heard his gurgly breathing and said "it's time for new gel injections!" He said we didn't need to change anything about Job's drinking or eating and they were going to try to pair the new injections with his already scheduled biopsy on the 19th. I walked out of that appointment thrilled! Oto thinks Job's safe! We'll only have to do one COVID test and one hospitalization! Woo hoo!
But that weekend Job's sats dropped again (still not dangerously low - solidly mid 90s - but a noticeable drop) and his breathing was getting even more concerning. So I just started thickening his liquids even more (3oz liquid per packet instead of 4oz). He started drinking a bit less because that level of consistency is SO very thick.
It was a tense week but we finally made it to biopsy and gel injection day! Hooray! He had his biopsy (which looked wonderful!) and had his pulmonary artery ballooned up (surprising us all that he didn't need a stent). Yay! Results came back that he had a rejection score of 0. Yay! The surgeon doing his gel injections that day wasn't actually his regular surgeon but everything went well. Yay!
Seth and I both got to stay with him overnight and we repeated our ridiculous joke about it being a romantic weekend getaway in a luxury high rise big city hotel. Job was pretty grumpy but recovered well. No Rapid Response Team, no ICU trip! Yay!
We got home but by the time Job was feeling better and up and at 'em he also started his recurring cycle of choking (usually when crying but sometimes "just" on his own saliva) and then throwing up. It wasn't ever very much and it was usually just saliva - not stomach contents. But then it escalated. Nights were terrible and Seth and I took turns staying awake to watch him or at least sit right next to him where he was propped up on the couch and dozing while he slept. Each time we thought about calling the on call cardiologist in the middle of the night he'd suddenly improve immensely and we'd sleep a few hours before calling the regular team the next day. We told ourselves we could do it for just one more week until his gel injections really took effect and we did the VFFS.
During the VFFS (videofluoroscopy) Job eats and drinks various foods and liquids (at various thicknesses and sometimes temperatures and delivery methods) that have barium in them while x-rays are taken. This is the very best way to assess for aspirations. Job totally failed his December 16th VFSS even on extra-thick honey liquids.
We're stunned.
And we're crushed.
He's had the tube for all of 28 hours now and it's been hard.
We're expecting he will be really sad about it, in physical discomfort from the tube and really really miss the taste and feel and comfort of "liquids" in his mouth. His doctor said that often kids who aren't getting liquids by mouth will try to sneak them, so we'll have to be watchful on that front.
I am preaching to myself that God has sovereignty ordained this for His glory and my good. But I'm so sad. There have been so many losses this year - especially in fellowship - so when we're already lonely and grieving this feels like an extra heavy blow. God has always provided for us tenderly. He will again. He is right now even though I don't think I can see it yet.
We're lonely and tired too. In other seasons of such intense trial we've had friends come alongside to bring meals or take the boys on an adventure or even just send a text to say they're praying. We haven't been very vocal about this ongoing trial so I don't think many people know Job's been struggling.
I tell myself things like ultimately this will free up the 5+ hours/week I've spent the last 6 weeks taking him to appointments and making phone calls! Buuutttt then I remember from previous tube placements that will be physically irritating to have something dangling down the back of his throat. Annnddd it will be work for Seth and I to learn how to use it again/then keep up on it. But most of all we're concerned about the emotional piece for Job. Does he understand why I keep bringing him back to the hospital for more pokes and swabs and surgeries and now tubes?
The surgeries on his vocal cord did help. He's now sleeping through the night safely. He's no longer choking on his saliva to the point of frequently vomiting after a coughing fit. His voice is louder and clearer. He's no longer breathing noisily.
But the more we look at his most recent VFSS it seems that he has greater issues than "just" his vocal cord. There's something wrong with the actual swallowing mechanism. I think Oto is going to be studying this most recent study quite closely and coming up with a game plan for future interventions. Right now it doesn't seem like there are many other options. We'll wait until February (when he's a year out from his nerve reinnervation) and reassess.
Of course, first we have to get TO February on this tube. And that's still a pretty daunting prospect.
No comments:
Post a Comment