Decannulation?!

June 10, 2014, 7 years ago today, we were pulling together all the last minute details so we could bring Brinley Noel home the next day. Supplies were organized, medical binder ready to go, oxygen concentrator, feed pump, mist machine, suction and pulse oximeters ready. The ventilator would come home with her. 

Cailyn and Raina had dress rehearsal for their ballet recital. I remember so clearly the duality of everything that we did. Excited for one, scared for another. Looking like a "normal" healthy family at an event while having a tiny baby on a ventilator at the hospital. I think those feelings and experiences are seared into my very being. 

Fast forward to today, June 10, 2021, after an incredibly hard year for everyone on the planet, our family included, we are packing up to go to the hospital for decannulation. They will put her to sleep, take the trach out in the OR, scope her to make sure everything looks good mechanically and then observe her in the PICU for 24-48 hours. 

If successful it will change many things for our family once again. Some good, some bittersweet. Brinley is excited. She is the bravest soul I know!

Please pray: 

The doctors get an accurate read and are confident in their decision. 

Brinley is safe no matter what happens.

For peace for Brinley, us and all the kids.

If successful, please pray for us during the upcoming time of transition.

I know it's been an age since our last blog post but it was only appropriate to end the way we started. If she goes home tomorrow it will be 7 years TO THE DAY, that she came home from the NICU.. Yes, I'm crying again

Sarah for us all

Happy 2 Year Homeaversary!

If you hadn't noticed, our blog has gone fairly quiet. That's a good thing in many ways because it means that life has gained a sense of normalcy (though a different normal than we used to have). Things are, in many ways, routine; Brinley is making steady progress on all fronts. She has finally crawled! She doesn't just up and go on her own but she will move her own arms and legs for PT when put in the right position. It's a great thing to see.

Brin just had her first ENT appointment since her tongue reconstruction surgery and his reaction was everything we hoped it would be. He was amazed that she actually now has a tongue! He loved it and he agreed with what we have been thinking about the trach, in that it is no longer really about helping her breath but more for secretion management. We will be scheduling her swallow study to happen in the next couple months then follow up with ENT in the early fall with a decent chance of having her trach out if the results of the swallow study are favorable. The big question now is if she will be able to keep her lungs clear and swallow her secretions properly without the trach in place to suction out anything she might aspirate.

One big reason for a post today is that this day, June 11, marks the 2 year anniversary of Brinley coming home from the NICU! She's come an amazingly long way in those 2 years as the pictures will show. We are so thankful to God for getting us all through this, for taking care of Brinley, for making her such a joy to so many people! She makes life bright. We know without a doubt that we would not be anywhere where we are without all of your prayers. Thank you, thank you, thank you!

Going forward we will probably only be posting major milestones and updates here on the blog. We still want you all to know Brinley's progress but there just isn't much to write about right now (thank God!). If we DO get the trach out this year, you can be sure we'll be throwing a party ;)

Then

Now

Post-op Update

I am so sorry that we haven't updated the blog sooner but we've been fairly busy. I hear there is still some confusion surrounding the surgery and what was worked on so I am going to try to clarify. Last January Brinley had her "duplicate toe removal" and while she was under anesthesia her ENT took a camera and looked at her mouth and airway. At that time we were told that she did not have a tongue, it was just a mass of tissue where a tongue should be. It also occluded (blocked) her airway and was the reason that she has a trach. At that time he and the plastic surgeon said that they could not do any reconstructive procedures because there simply was not enough to work with. The only option given to us was a 30 hour procedure (with 1 Dr doing the work!) when Brin hit adulthood. They would have harvested mature calf muscle and created a tongue that could be more cosmetic than functional. I digress, but please keep in mind this ENT is excellent at what he does and I am confident that the changes that have come are a result of all of our prayers and a miracle from our mighty God! 

Leading up to surgery it was thought that Brinley needed a cleft pallet repair and a reorientation of the musculature at the top/far back part of her mouth. The surgeon had us sign several wavers though as she was not sure what exactly she would find once she went in. What she found was that while Brinley does have a cleft, it is very small. She said there are a lot of people walking around eating, speaking, drinking with a cleft just like Brin's. It may or may not have to be fixed down the road and that will be determined by her speech capabilities. The musculature was also fine (she had given us a 15% chance that this would be the case) and she noticed that the tongue had grown considerably so...she took the nodules and lobes out, somehow reconstructed the base of the tongue so that it now comes down and out instead of up and into the airway. It is still shorter than a typical tongue but it has taste buds, is mostly the right shape and it's THERE! She also released 3 "ties" on the upper lip and was able to get twice the range of motion on both lips. This will help with speech. 

Wednesday we had our follow up appointment with the plastic surgeon. She was very pleased with Brinley's recovery and Brin was cleared to use her passy muir (speaking valve) again. Down the road we will be looking at:

*Phase 2 of the toe procedure, it involves the growth plate so that won't be for a few more years...

*A tongue lengthening procedure (most likely in her teens)

*Some sort of dental stuff I am sure, but we won't be referred to them for a year or two still. 

All of this brings us to our next step which will be meeting with the ENT, getting a swallow study and determining if Brinley still needs the trach. Her SATS (oxygen level in her blood) have been amazing since surgery. She doesn't really gag on her tongue anymore either. Which is simply amazing because it was so.hard.to.watch. We felt so helpless! I am excited that the ENT will be able to make his decision based solely on the airway needs and not having to keep other procudures/timing and such in mind. The swallow study will also give us a good idea if Brinley still needs to be on her reflux medication. We are hoping to be able to take her off of it soon because there are some negative side affects of prolonged usage. 

Hopefully that made things more clear. Please feel free to ask questions. Our prayer points are *that she would relearn to swallow her secretions *that she would start crawling. There are more, but those are the major two at this point.

Before and After

Doing her own neb treatment- very independent lately! 

Baby tongue!

Playtime with R (SEIT)

All sprawled out

Nurse A giving Brin a back rub. She bucks and grunts if you stop.

Friends! (Nurse H's little guy)

This little girl is such a champ and is acting two already! If you want to come celebrate her birthday with us please feel free and shoot me an e-mail or text. All the information is in the previous blog post. 

A huge thank you to each and every one of you for your prayers and meals- you continue to be God's hands and feet and bless us so much! We are excited to plug back into life with all of you now that procedures are winding down for a while!

You Are Invited!

We can hardly believe it's true, 

   Brinley Noel is turning two!                                  



Come celebrate with us!
When: Thursday, April 21 4-7pm (open house)
Where: Brinley's house! 
(Please contact us if you need the address)
Gifts: Please don't feel like you need to bring anything.
If you would like to donate any gently used items to the 
Albany Med NICU we will have a box open to collect things
like blankets, mobiles, bouncy/vibrating seats, tiny socks 
and clothes. (Garage sales are a great place to find these items.
We will donate them on June 11, Brinley's discharge anniversary,
so no rush.)

We will provide food, desserts and drinks so if you could please 
RSVP to sarajofina@gmail.com that would be great! 
Weather permitting we will enjoy the swing set that you all were part of providing as a result of last year's amazing birthday celebration! We also have a kid sized bounce house. If the weather is bad we'll all get really cozy inside ;)


*We have Brinley's post-op follow up on Wednesday and will post a progress report after that. She is doing very well as far as we can tell. Biggest prayer points would be relearning how to swallow and crawling on her own.

Post Palate (or not) Surgery Update

Brinley’s surgery went great! Her recovery is the hard part right now. She’s extremely uncomfortable and not sleeping much from a combination of pain, not being at home and maybe even some tummy troubles with all the different pain meds in her (not sure about that one). Please continue to pray for quick healing of her mouth.

As for the surgery itself, it went great, as I mentioned, but it didn’t go down how we expected. Our expectation was a palate reconstruction with maybe a tongue tie release thrown in. What we got was no palate work at all and a whole lot of tongue reconstruction work. That’s right, the whole landscape of her mouth has changed. I will try to remember everything correctly but I don’t have all the right terminology yet so I’m sure I’ll be correcting myself in coming posts (or more likely, my wife will). Her palate actually was pretty intact with only a very small cleft in it that may or may not require surgery in the future. She also has 2 uvulas (or a split one, kinda the same thing) which is fairly common. Dr. A didn’t do any work at the back because she didn’t want any scarring to get in the way of growth and development back there.

Brinley’s tongue before surgery:

·         Large lumps of flesh on either side of her tongue, almost looked like wings.

·         Many small roundish nodules under her tongue in the front

·         Tongue position was very vertical and flopping back to block her airway.

·         Didn’t really look much like a tongue to be honest.

Brinley’s tongue after surgery:

·         Those large lumps were removed

·         Most (maybe all) of those nodules were removed.

·         3 ties on her upper lip were removed and somehow Dr. A lengthened her upper and lower lips by double (not sure how that’s measured but that’s what she said) giving her much better range of motion with them.

·         Some work was done reconstructing the base of the tongue allowing it to lie much more normally and not flopping back! This is probably the most important result of this procedure and a real answer to many, many prayers the last 2 years.

·         Her tongue actually looks somewhat like a tongue. Small yes, but the texture and shape are tongue-like!

She still has a lot of swelling in her mouth so it’s hard to go into too much detail about the interior beyond what I’ve already said but the size of her lips and mouth is very apparent as soon as you look at her. She still has lots of dried caked on blood on her lips as we haven’t wanted to try cleaning it and causing her more pain and when we suction her we do get some blood still. We’re pretty sure it’s not fresh and just residual she’s coughing up and/or dried blood that gets loosened by saliva but we’ll be glad to see it gone regardless.

The only real sleep she got was when we put her with Sarah on the reclining chair and she’s sleeping again with Sarah as I write this post. Sleep is the best thing for her right now. We’re feeding her fairly small amounts but she’s tolerating it well so we’re not too concerned about nutrition. Pain meds can slow digestion so we didn’t want to give her too much too quick. Sarah and I didn’t get much sleep but we’re doing well regardless. It’s hard to see Brinley in such pain but we’re excited at the possibilities and progress she’ll make as she heals. Dr. A says she is cleared to resume therapies as soon as she is feeling up to it (just no passy muir for a couple weeks) so that’s nice. She won’t get too far behind and she usually enjoys her therapy sessions.

Oh, and the tongue tie we were expecting to be released… that was the one tie that was left alone. Go figure. We are so thankful for an awesome team led by Dr. A that did an amazing job. She is such a brilliant surgeon and we are truly blessed to have her for our Brinley. The anesthesiologist team did fantastic work too with Brinley coming out of anesthesia amazingly well this time. Thank you everyone for all the prayers and words of encouragement. We will hopefully be going home tomorrow if Brin continues to improve! Alessa had a great first day of preschool so thank you to friends and family that made that a special time for her! All our girls have been well taken care of and they are enjoying some fun time with grandparents while we are with Brinley.

Sorry this took so long to get to you. I wasn’t able to get my laptop on the wifi in the hospital so I had to wait until I got a chance to go to Panera and upload it there.

Just before surgery

Just after surgery (really)

One of her few times actually sleeping

She was only happy when mommy was with her. A nurse kicked mommy out and Brinley glared at her, then screamed for hours until she could lay down with mommy again.

Nurse C can always cheer Brin up

Took a 3 hour nap on mommy's lap. A catheter would have been handy.

Hydrocodone really helped kick the pain so she could sleep

Talking in her sleep :)

Video not playing?

Surgery #4 Impending

Mark your calendars, set you cell phone alarm, Tuesday, March 29 around 7:00am Brinley will be heading into her cleft pallet repair. This is the longest procedure that she has undergone. She will be away from us for about 5 hours. We are anticipating a 3-5 day inpatient stay as well. They are planning on putting her on a regular pediatric floor...we shall see. (check out the last post for more info)

Specific prayer requests in regard to surgery:

*The big girls are more aware now and so have been expressing concern about the procedure itself, missing Brinley, missing us, etc. We have some fabulous friends and family lined up to take care of them and even more of you wonderful people bringing meals. Please pray for peace in the girls hearts and minds. Also, Alessa will be starting pre-k that same morning! While I am very sad to miss bringing her on her first day she will be in good hands. Please pray specifically for her to adjust well.

*Peace for Brinley as she is much more aware of her world now than she was during previous hospital stays. We know some people who know some people and were able to get Brin set up for first case that morning. This helps us have more time to gown up and carry Brin into the OR ourselves. There's also a note on her chart that should allow us to see her sooner post-op than usual. Probably because we're used to ventilators and groggy/post-op Brin. 

*Great wisdom and excellence for our surgeon, Dr A. She has an excellent reputation, a lot of experience and we feel as well as we can handing our little girl over to her. 

*Wisdom for the PACU staff in their post-op care and making the final call about which floor she should recover on.

*Speedy recovery. Since Brinley's tongue is so atypical it is difficult to foresee how the procedure will affect her short term. There could be a lot of swelling which combined with her exhaustion (which causes her tongue to flop back) and pain could make the tongue rub right on the incision. This would be painful to say the least, however with Brin could also mean that she has to rely exclusively on breathing through her trach. At our last ENT appointment he told us that she was breathing through her upper airway quite a bit and if she was truly trach dependent she would need a larger trach size...So, I wonder, will we need a bigger trach? Will oxygen supplementation be enough? Will she need a ventilator? Thankfully, Dr M (her ENT) is aware of the potential complications and has assured us that we shouldn't worry, he'll be around and has a few ideas of how to help. Thank you God! So, we're praying for supernatural healing and quick positive results regarding tongue positioning, swallowing (secretion managment). tolorating food and moving her bowels. 

Thank you to each and every one of you for praying for our family! Somehow, even after 3 other surgeries it doesn't get any easier or more "normal." We will make sure to update the blog after we meet with the surgeon and see our little Brinley. 

Outside of surgery it has been two months of growth. Brinley is truly blossoming! Despite a respiratory infection and stomach bug (which hit all 6 of us!) her signing vocabulary has grown exponentially, vocalizations when the passy-muir is on are much more varied and frequent and she's making great gains in the walking/crawling department. Nurses C & A are still with us and we're so blessed by both of them! The big 3 girls are growing like weeds too, both Cailyn and Raina have teeth missing and are just too cute! 

God bless each and every one of you that have journeyed with our family for almost 2 years! Yes, we will be doing a scaled down "open house" birthday party for little miss again this year. Details are still in the works, but you're all invited! I shall leave you with these...

That time I poured my coffee into Brin's daily feed batch..

My whole heart

Thank you God for "Signing Time"

(the only thing that consoled her during the 2 week tummy bug)

Daddy Daughter Dance

Helping the nurses catch up on their charting

A 3/4 crawl, our lovely PT is only helping with the left leg.

If Video Above doesn't work

A perfect baby

It's a cold winter night in January. The day is done and the kids are asleep. A man sits in bed with his computer, his beautiful wife next to him quietly catching up on some texts and relaxing after a typical non-typical day in their lives. The youngest of four daughters dozes peacefully in the crib next to their bed, hopefully down for the night. This young one, almost two years old, has no equipment attached to her, no monitor to assure her parents that she is indeed still breathing, no vent to breathe for her, no oxygen to make sure her breaths get her the much needed saturation her blood and body demand second after second after second. This baby is perfect. The man loves his life.

“I have told you these things, so that in me you may have peace. In this world you will have trouble. But take heart! I have overcome the world.” -John 16:33

I may be echoing some sentiments that I've already stated here before but it is too important to not reiterate: whatever stuff you're in the middle of right now, God is so much bigger. God knows and so do I that sometimes we just want an easy way out of the crap we find ourselves experiencing in this life. My prayer has so often been "please God, deliver me out of this. Heal Brinley, give her a new tongue." At the same time I can't help but feel so thankful at how far we've come, not delivered from the fire but through it. Life is not an easy thing to live. I have yet to meet anyone that has had a perfect life. If you know such a person, then you don't really know them.

Rejoice in the Lord always. I will say it again: Rejoice! Let your gentleness be evident to all. The Lord is near. Do not be anxious about anything, but in every situation, by prayer and petition, with thanksgiving, present your requests to God. And the peace of God, which transcends all understanding, will guard your hearts and your minds in Christ Jesus. -Philippians 4:4-7

It's been so long since I've written a blog post that I wanted to share some of these things that are in my heart, things that God is speaking with each passing, crazy week. Sarah and I are so incredibly blessed with four incredible daughters, friends and family that are imperfect right along with us, a schedule that is more insane than you might care to believe and a God that knows exactly how much we can handle and when we just need a rest! We have been having so much fun living life with all of you, sharing joys, mourning losses and reveling in the little victories that each day brings. The God that we love continues to amaze us as we let him be in the driver's seat.
Here is a great quote from an amazing woman of God that hopefully, all of you have heard of:

“This is what the past is for! Every experience God gives us, every person He puts in our lives is the perfect preparation for the future that only He can see.” 
― Corrie ten Boom, The Hiding Place

This distills so much of what God has been working into our lives over the last couple years into something so understandable.

Now that I've gotten part of my brain on the page I can give you some of the latest on Brinley and co:

• Today we had our first appointment with ENT and plastics since last February. Dr. M, our ENT gave Brin a great report. We have the OK from him to do some food tasting and small feeding amounts (very small). The best quote from him was that soon her trach is basically going to be costume jewelry (meaning she's doing almost all her breathing using her own airway and the trach is really just a backup). The next step from there is to possibly do a new swallow study. The next step after that would be decanulation (trach removal)!
• Plastics was very pleased with how well her feet have been healing. There may be some adjustments that need to be made but that wouldn't be until at least school age.
• We have a date scheduled for her cleft palette surgery. March 29th. The procedure is about 3 hours with an hour or so before and after for pre and post op. Recovery is typically a 3-5 day stay in the hospital. This surgery will hopefully be a great step forward for a lot of development including speech and swallowing and lots of secondary things you might not expect to be connected but are.
• Big prayer request is not just for the surgery itself and the recovery but also that we can ween Brinley off her pacifier because she will not be able to have it for 4-6 weeks after surgery and it is currently the only way she is soothed to sleep. It could get rough and we don't want her to have to endure surgery and not having her binky at the same time so we will begin the weening now.
• We have a new nurse on the team, nurse A, that we are in the process of orienting/training. She's already in love with Brin and she seems to be a great fit so far with our crazy little family.

That's all for now. There's always more to tell but it's easier if you just come visit, so stop on over! Just one picture this time.