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.
Mar 30, 2024
Where We Go From Here
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.
Mar 29, 2024
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.
Oct 18, 2020
14 Years
2020 has not quite been what I expected. After Covid 19 hit in March blogging just didn't feel right. It's been such a tough time for so many people in so many ways.
Our family has been doing well, all things considered. We've been keeping very isolated for Ainsley's protection since her airway is still somewhat compromised due to scar tissue above her vocal cords and she already depends on CPAP for sleep. Respiratory viruses are risky for her, Covid especially.
Since I lasted posted Steve started working from home. We are so fortunate that he has a job that is conducive to that. He's adjusted to working at home and is tolerant of me pestering him throughout the day. I think he generally likes working from his "man cave" in the garage, except for the odd occasions when he's in a meeting and the garage door opens. It is nice that he's no longer late for dinner.
Evie was enjoying life in a dorm at the University of Washington but moved back home in April. Not knowing how things would be, come fall, she signed a lease for a house with friends on Greek Row. In September, rather than break the lease we moved her in. Although her learning is all remote it's good for her to be able to live more of a college life. She has more freedom than if she were living at home, due to precautions we have to take to protect Ainsley. You probably know Evie loves working as a princess for hire at kids' parties. Like everyone she had to adapt her work when social distancing was put in place. She made some video recordings for kids that were posted on websites so it wasn't uncommon to see her setup with a video-camera and lighting in full costume. Now that restrictions have eased up a bit she's worked a few parties like here as Ariel recently where she was happy not to have to wear the sea-shell bra. Her work problems are not quite typical. She also spent a lot of time sewing and making costumes.
Adrian's school was closed back in March and and several events for him were cancelled, one after another: the State Thespian Conference trip, the Ballyhoo show of Spelling Bee, the Spring RHS show, and even the Jazz Choir trip to Disneyland. He is enrolled in the Running Start program and will be "attending" Bellevue College for his Senior Year. He went from a mid year Junior to never going back to High School! We hope things will improve enough that there will be graduations and proms this year but only time will tell. During Covid Adrian has been keeping himself busy playing board games with his dad, experimenting with new hairstyles (no better time, I'd say), playing video games and teaching himself Japanese(Kanji).
Jan 13, 2020
Hello? 2020
If you have ideas for things you'd like to see me write about please post in the comments. I'm really excited for 2020 and am hoping it brings GREAT THINGS! I am open to change. I may even consider an off-shoot of this blog. Give me your ideas!
For now it seems a good place to start is right here, right now, with a focus on my work with Ainsley. I guess I'm saying I plan to write about Ainsley, what she is up to and how she is doing, more than the rest of the family. If everyone misses hearing and seeing my big kids or seeing what we're up to around the house I may change my mind. I may even go back in time and add some posts, back dated. I'm sneaky like that. Plus I really do miss being able to use this blog as my memory of what happened when, because my actual memory is shot. But let's be real, as much as I may like to do that, I probably won't.
Since I last posted the biggest news is Ainsley turned 13! She was thrilled to get 2 new Baby Alive dolls (One is a mermaid she can take into the pool, given to her by her big sister, who paid for it herself using money she earned working as a princess for hire for birthday parties.)! She was able to blow out the candle all by herself this year! I hope she made a big wish! So YIKES! I have 3 TEENAGERS! How did that happen?!
Ainsley had 2 surgeries (to correct a leg length discrepancy, a long story for another day). Evie graduated high school and is now at college at the UW! We are so proud of her! Both Evie and Adrian got their driver's licenses in 2019, within a week or so of each other (yes Evie is 2.5 years older she just took longer, story of her life). That has been life changing! I am thrilled not to spend so much time chauffeuring but I am terrified (and the Mini was in an accident once already). Aside from that there have been no major changes.
We are still homeschooling. Although it isn't something I ever expected to do I still believe it is the best option for Ainsley. She's been really healthy (we miss out on all those school germs) and happy (when I ask her she says she wants to keep homeschooling). The best thing is that we can "do school" in our pajamas when we want to AND she can bring all the girls. She enjoys "teaching" them. One element of a 4 Blocks approach to literacy is independent reading. This week she chose this book about animal skeletons to "read" to her girls. If you are interested in literacy for individuals with disabilities you may want to check out the much anticipated and newly released: Comprehensive Literacy for All.
Although in the shallower water she does actually balance on her feet to walk (she's not just paddling) it hasn't transferred over to walking on land. Due to her Cerebellum and Pons malformation she is easily thrown off balance and lacks adequate motor planning response time to feel safe at this point. We are working to push her a little past her comfort point. Her therapist, Joe, is so patient and kind and that helps make it as fun as possible for her (she still kind of hates it). She always brings a doll and he always talks to her about it and gives her the chance to talk to him using her AAC device. As you can see here in this video she is getting so strong!
Mar 27, 2018
First and Next Steps!
When Ainsley was 2 she was really close to taking steps, or so it seemed. Her PT said no we didn't need a stander because she was good at standing with just a little arm support. She got a walker and started learning to use it around her 3rd birthday. I thought any day she would take her first independent steps. She got stronger and did well in her walker. Each birthday would pass and I would think okay, maybe it didn't happen at 3, but it will at 4. Then... not at 4 but by 5! Not 5, but 6? Not 6, but 7? Not 7, but 8? Not 9, but 10? Not 10, but 11? Today is the day that stops because today she took her first independent steps and I have them on video for you to watch!
How did this come about? It would be natural to assume this development is a result of surgery last September, however, like everything with Ainsley things are rarely so simple. As the closest firsthand observer of "everything Ainsley" I would say that there have been many factors leading us to this exciting moment, not just surgery.
For many years I've felt that actually the biggest obstacle Ainsley faced was the fear of getting hurt. Unlike many kids facing challenges like Ainsley, Ainsley never attempted to take steps and she was even fearful about standing. Though she was able to stand for up to a minute without touching, as young as age 4, before losing her balance, she would only do so if she was sure we would catch her if she fell, like when we would stand in front of her with our arms to each side of her during sit to stand exercises. Despite standing relatively well she never progressed to steps if she didn't have something to hold onto even though she walked in a walker with relative ease. Her therapist pointed out that her balance was actually pretty good like when she was on her knees. Which showed that she didn't feel confident on her feet. I could see fear was stopping her. She had a traumatizing fall from standing, when she was young, onto carpet, where her arms collapsed and her teeth cut through her lip. Many times I am thankful that Ainsley is cautious and that keeps her from getting hurt but it also holds her back.
I spent a lot of time thinking it all through and asked her Physical Therapists years ago if they could teach her to fall. I felt quite sure that she would be able to walk it if she could just get over the fear. After all she had been walking in a walker for many years. They seemed to think it was an interesting idea but none of them did. Then I learned about a training system called SafeGait. Ainsley could try to take steps in a harness connected to the ceiling so that she could not fall. When she was 10 we tried it at Children's Hospital. I thought for sure it would work but after several frustrating months I could see she was still so fearful that she refused to take steps even though she was 100% safe, so we stopped.
Of course it is to be expected that she would have difficulty due to her diagnosis, call it whatever you will: Cerebellar Hypoplasia, Ponto-Cerebellar Hypoplasia type unknown (she doesn't have the gene for PCH but the cerebellum and pons are undersized), or something so long that no doctor but a neurologist would understand it, or just Cerebral Palsy since that's easiest. Whatever you want to call it the MRI pictures are clear she is working with an unusual cerebellum and that makes everything HARD because she's affected in the following ways:
1) Balance - she can get off balance more easily than other people
2) Motor Planning - she has to really think about how she is going to do things and it isn't automatic
3) Speed she's just slow because of the way her cerebellum is
4) Vision (not the surgical complications to her eyelids or remaining ptosis) but her eyes don't track smoothly because of her cerebellum
5) Spatial Awareness- she may have difficulty knowing where her body is in space which in combination with vision is tricky when you are having to navigate over or around obstacles, and/or across different or uneven terrain (uphill, downhill, curbs, carpets, stairs etc).
In addition to her Cerebellum issue she has the following:
6) generalized weakness
7) a history of hip dysplasia that was surgically treated twice...and
8) Her hips are a bit off balance and that causes a leg length difference which also makes it harder to straighten her legs, the right leg is longer and thus bent (though she got a shoe lift recently)
9) Her knees had become contracted and would not fully straighten, likely due to #8.
10) Her feet would pronate (roll in). We tried various orthotics, she couldn't walk in rigid AFOs but hinged AFOs allowed her to feet roll in because of her flexible joints. Then she had surgery which helped but she will still need AFOs.
So the surgery addressed issue #9 and #10. #9 - Her legs are now about 5-10 degrees from fully straight, (the new shoe lift on the left helps too, but only when she is wearing the AFO's and shoes), basically she needed the surgery and a shoe lift. #10 - The goal of the calcaneal osteotomies surgery was to give her a more stable foot base. Yes in x-ray the foot is straighter but there is enough flexibility in her feet that they can still collapse and pronate under pressure, especially if she isn't in the AFOs, but not quite as badly as before. She's better when she's wearing the AFOs, but she doesn't have as much control when wearing them due to lack of feeling, range of motion, sensory feedback etc. We do ankle exercises as part of our exercise routine, for which she needs them off as you saw in the video she was barefoot. It would be interesting to see how she does taking steps with the new hinged AFOs that we got last month now that she has done it barefoot. Her preference is not to wear them but we do put them on if we expect to do a lot of standing or walking. You can be certain I will try that to see which is easier for her.
So that brings me back to today and how it came about that today Ainsley took 2 steps. We've been doing these post-surgery exercises twice a day as often as we can. We put a mattress in our bedroom so we could do the exercises on it when she was in casts. We found it useful so we've kept it since.
At our more recent appointments I was talking with her new therapist about this fear issue. She agreed and showed me ways to start teaching her to catch herself so she felt safer. We worked on sit to stands with a chair in front of her. Then at home I had the idea to have her do the sit to stands in front of the mattress so she could fall forward safely, and it was better because it was lower so harder than a chair. This really helped her gain confidence and she started to look really good when she does that exercise! It was at this time today doing sit to stands that I thought we could expand to actually taking a step.
First to build confidence I had her hold my finger while she stepped forward after standing. She did that so well and then she said she felt comfortable doing it independently. This second video shows how much effort and determination it takes her.
She was successful taking single steps so many times that I asked her to take two steps and that is when I caught it on video! I consider myself agnostic but lately I have taken to holding hands and praying with Ainsley before exercises, asking that God help her be strong and brave and guide us to what we need to do to meet our goals. It helps me remember to have patience. Perhaps these little ideas that I tried were not mine alone. And perhaps in some way it has helped Ainsley have confidence. She always smiles and gives a thumbs up after we say Amen. In addition we have been praying for our dear friend Harlie along with hundreds of other people, over this past month. Happily she is doing much better. I don't know what part prayer played in today's serendipitous events but I know prayer can't hurt. So I will say if you feel inclined please pray that today's progress continues.
I would love to believe that now that she's done this it will mean more of the same and quick progression to more steps, but nothing with Ainsley is that easy. I expect our next steps will be:
1) continue walking with the walker for endurance
2) work with the crutches to gain confidence
3) continue 2x daily exercises to build strength
4) continue with single steps to build confidence
5) lots of patience and encouragement
We celebrated tonight with a cake. Steve is in California on business and called home to give congrats. We love this girl to bits and are so happy for this day! We are so proud of her!
Feb 27, 2018
2017 Wrap Up Part 2
Her husband makes pancakes and sausage and they put out quite a spread of treats. Like me Darcy likes to entertain. I appreciate the effort. She always puts out extra touches to make it special and each year it's a little different.
Ainsley, bless her heart, tried and I would just go back later and fix where she had 3 ornaments in the same spot on a single branch.
As the snow fell on our faces it was truly a magical night. People were happy and you could feel the true Christmas spirit. I haven't had such a nice Christmas Eve ever. Though I admit that may have also been slightly in part to my relief that Snowflake Lane was finished.
The conversation was great and all were in a great mood. A really fun Christmas this year!