Where We Go From Here

Here we are. Ainsley is resting today post-trach, after quite an ordeal. I will have to save all that for another post. Just know that right now we are trying to bring her out of sedation slowly and at the same time wean her off the ventilator, without causing additional trauma to her already traumatized upper airway. Yes it is true that we bypassed that terrible airway by surgically placing a trach, but her ENT surgeon needs her upper airway to heal before he can operate on it. 

All week he was receiving updates from the team here and requested that she remain intubated until he returned from Chile on Monday 3/25. We viewed the video of her airway scope and discussed the plan to get us out of this mess. I knew, but he confirmed, a trach would be the only option and it was planned for later in the week as long as her health allowed it. He planned to assess the current condition of her airway (intubation and crying caused further trauma) via scope in the OR when they placed the trach. We were correct that her airway was badly affected by10 days intubated plus crying. Extubation would have failed terribly and had we tried we would have be reliving the events after her birth 17 years ago.  Post intubation pictures follow below.

This video was taken on March 19th and shows the condition of her airway at that time. It is the 2nd scope and is actually worse than the one from the 17th, the day we arrived. Her "normal" airway is similar to this, but slightly less small and swollen. 

In the best of times Ainsley has narrowness through the glottic area above the true vocal cords. Any stressors can easily collapse this area which leads to more inflammation and swelling and even more difficulty breathing. This is why this time the crying in recent weeks on top of a virus landed us in the ER, after rescue meds were used to open her airway. This is a very unusual airway problem. There is not really enough space for an intubation tube. They can cause irritation to a normal sized airway.

It's important to reiterate this video is before intubation and crying, which you can see in the following pictures, where the swelling is yet worse still. This video shows her airway in action, by filming using a camera inserted through the nose. I hope it will help you* better understand what we are dealing with and what needs to be fixed. The trach temporarily bypasses this part of the airway because it sits below the larynx, in the trachea, allowing breath to pass in and out of the tracheostomy tube. (*for our non-trach familiar friends)

These images are from the scope done in the operating room after 10 days intubated and some extreme crying from pain (again, those details will have to wait for a future post).

We have been watching some interesting anatomy videos that are fantastic for better understanding how the parts of the larynx and how it works. It's fascinating and complex. Here's a link in case you want to learn more.  It's this area where Ainsley's problems are. The airway reconstruction surgery will be a Cricoid Split. Normally done to avoid a trach, in Ainsley case the trach is necessary to buy us time for the upper airway to heal. This is likely to take up to 2-4 weeks, or more. Depending on how things go it is possible we may discharged and return for the surgery later. We won't know until we see how her airway heals over the coming weeks. In her case the trach will remain until her airway is proven to be safe and we also don't know how long that will take.

If surgery isn't done as part of this hospitalization they say we need training on the care of a trach (laughable after 8 years caring for a trach) and in-home nursing before discharge. Trust me I am working to get everything in order to get out of here as soon as possible if airway reconstruction surgery is postponed. I think it is also important to say there are risks to this surgery. It could lead to her aspirating which might mean she is no longer able to eat orally which is something she loves and has done for a decade now. Aspiration can lead to lung disease which would be very undesirable. These are the reasons we haven't proceeded with this surgery in all these years. Her tissues are not normal down there and she is "not an ideal surgical candidate". We could end up opting to keep the trach but that is not an easy lifestyle and has downsides as well. We will continue to update as things develop. Hopefully very soon I will be able to share photos of a happy smiling Ainsley. 

I am dreading her waking up with a trach unexpectedly because she had no idea it was happening.  I really don't know how she's going to react but hope she takes it in stride as she so often does. Though she is now a teenager and can be unpredictable. Keep us in your thoughts and prayers as we get through the coming days, weeks and months. Thanks for the love and support!

Almost 10 Years....

I regret that this update is to unfortunately share some bad news. After nearly 10 years without the trach Ainsley is currently in surgery as I type this, getting re-trached. Those of you who know her story well, know that she never had airway surgery, and after 8 years trached, and even though she got the trach out in 2014, she still has a small airway. She had a high degree of airway swelling that somewhat resolved after a fundoplication when she was practically a baby, but some swelling remained. She had scarring and not great opening of the vocal folds. She wasn't a great candidate for airway surgery. We even took her twice across the country to the world renowned Dr. Cotton hoping he would be brave and smart enough to come up with a miracle solution. It was a long shot. He told us there were risks to surgery and it was likely she would remained trached for the rest of her life. Discouraged we returned home but in later years worked to get her to tolerate CPAP to prepare for decannulation (trach removal). The doctors were nervous about how she might do so we left the stoma (trach hole) open for an entire year just in case the trach had to be put back in. She did well and the hole was surgically closed in 2015.

The years that followed decannulation have been pretty good. Challenging at times because her airway was small and anything that stresses the airway made it that much harder for her to breathe: illness, exertion, coughing, crying, and sleep. We had some difficult periods over the years that I didn't have a chance to blog about, but we made it through. 

In 2022 she caught Covid 19 but made it through better than expected. It eased our fears a bit. We'd been very isolated from 2020-2022 and that was especially hard on Evie & Adrian. Interestingly, her doctor said that they were surprised to find medically complex kids faired better than expected with Covid 19 and actually tend to do worse with RSV, flu and other common viruses. 

In 2023 we decided to try sending Ainsley back to school knowing she might benefit from attending Transition Academy in the 18-20 age range, after High School. It was hard to imagine her in a school of 2200 kids but after touring the space where the High School "transition classrooms" were I felt with the proper support she could do it. Ainsley is very social so we knew she would love being around people but we also knew we had to be careful due to her small airway. She did get sick nearly right away but the illness wasn't terrible (other than how it always is to be sick with her small airway - imagine being sick and breathing through a straw). She had some incidents that were very upsetting to her (behaviors of other kids that she wasn't used to) and she spent a lot of time scared and crying in September. In addition we found out later she was not being pushed in her walker for longer distances so she was getting exerted. Her airway was really stressed by the combination of these things and she had some scary episodes. We advocated so she got more consistent 1:1 support and was restricted from walking more than 5 feet at school until the end of November when we felt she'd improved enough to carefully start resuming normal activity.  Although she was ok, subsequent illnessess in the winter, although minor, were harder to tolerate. There were several times that Ainsley went to her room and put her CPAP on during the day because she felt she needed the breathing support. Her physical endurance was diminished, she would get more winded from walking down the hall. We thought it was from lack of physical activity over those 2 1/2 months but now I think it was more than that. Her already small airway was strained and looking back I think she hadn't fully recovered back to her pre-school baseline.

We consulted with her ENT at the peak of this period but the soonest we could get in to be seen was January and things had stabilized by then. A Cricoid Split Airway Reconstructive Surgery was discussed, as it has been many times over the years, but all agreed that the risks vs. benefits were a bit of a toss-up and that we should wait for a clearer sign that it was in her best interest.

That sign came in the wee hours of March 17th. All day Saturday she had been fine but at bedtime things took a turn.  She had been sick the prior weekend with a minor virus. She had a sore throat and a temp which resolved without meds by Tuesday morning. Wednesday I kept her home to be sure she was fever free for a full 24 hours and we had a great day "homeschooling" and tried speech therapy for the first time via Zoom. Thursday she went to school but had a bit of a crying episode. Friday she was fine during the day, we had a couple Zoom doctor appointments so she stayed home. Thursday and Friday night though she was breathing noisily on her CPAP at night. Since she'd been sick that week, I told her it was bedtime at 11:00 even though it was a Saturday and she cried really hard for about 30 seconds. I believe that was the straw that broke the proverbial camel's back. 

At that point she went to sleep with her CPAP on. Steve and I continued watching Oppenheimer. When I came to bed it was about1:30 and after a few minutes laying in bed I got up because I could hear she was having a hard time breathing (we have ajoined rooms). She also had removed her CPAP mask (which she really needs to sleep and normally tolerates very well) and tried to refuse putting it on. I climbed into her twin size bed, let her cling to my hand and helped her calm her breathing so she could tolerate the mask. Eventually she said to leave (it was crowded) but around 4:00 am she pulled off her CPAP again. This time I woke Steve. When I would put the mask back on it seemed to make her panic. This was the first time ever that she was unable to use CPAP. After trying to work through it we could see she was in increasing distress to the point it was scary (and I don't scare easily) and we didn't feel it would be safe to drive her to Children's Hospital. 

For the first time ever we called 911. The medics arrived quickly and got to work taking her medical history and info and they gave her racemic epinephrine. It was crazy having 6 firefighters and paramedics surrounding her bed.  That opened her airway enough that she could breathe and was stabilized. They thought she should get checked out so Ainsley got her first ride in an ambulance. The craziness sobered me up quickly (It was Saturday night) and I even managed to comb the leaves out of my hair from yardwork that day and grab a pair of shoes (*Note to self, if this ever happens again don't forget your purse!) It was chaos but the main guy who stuck with us was amazing with her and I was so grateful for their excellent care. He even gave her a fireman teddy for a souvenir. The fire department medics are the best! Although she was stable the doctors at Seattle Children's thought she should stay for observation and we reluctantly agreed. From there things went downhill like a trainwreck but that is a story for another post. 

I have missed blogging and I really need these posts to record details that I otherwise will not remember. I hope to write a bit more about what happened next and what this means for Ainsley's future, for all the people who care deeply about our sweet girl. She will continue to remain sedated for a few days and I will try to update. Keep her in your thoughts and prayers that her body heals and she is soon back to her wonderful self. Here is the last picture you will see of her bare neck for awhile. Although we are hoping for airway reconstruction surgery and eventual trach removal it is going to be a long road. We're hoping to know and share the next steps details soon.