About

My name is Ainsley. I was born full term on 10/18/06 via c-section after 12+ hours of labor and 1 1/2 hours of pushing. I weighed 7lb 12oz and was 21 inches long. My birth ultimately ended in a c-section because I was trying to come out by the side of my head with my neck twisted, and that just wouldn't work. Plus babies with craniosynostosis frequently require a c-section since their skulls aren't as flexible but more about that later. I went without a name for a few days because my name was going to be Flora and my mom just thought that didn't suit me.
Right away I had stridor (a kind of wheezing sound when breathing) and had to be sent to the NICU for breathing support. It was decided that I needed to be intubated. The doctors never figured out what the initial problem was but I acquired polyps, swelling and scaring from the intubation tubes and scopes. It got increasingly worse, requiring that I get a tracheostomy at 5 weeks old. There is an old video here if you'd like to see what it was like.

I was so happy to have that endotracheal tube out of my throat! Even though I was doing great and didn't need any ventilator support or oxygen I still didn't get to go home yet. The following weeks were spent coordinating the details of my release from the hospital. My mom and dad would be the primary caregivers for all things medical and had to complete 30+ hours of training including: infant CPR, how to care for and replace a trach (short for tracheostomy tube), learn what types of medical equipment and supplies would be needed and most importantly recognize signs of respiratory distress. Then came training on how to use the medical equipment: a feeding pump, ambu-bag (for manual ventilation in case of emergency), oxygen (just in case), warm mist machine(for humidity), pulse-oximeter, apnea monitor and suction machine to clear secretions from my airway. In addition it took time to setup nursing services so we would have trained medical professionals to care for me while my mom and dad slept.


I didn't learn to eat as a baby. Because I had the endotracheal tube down my throat I had to be fed with a nasogastric tube. I loved to pull that thing out! I got the okay to attempt to feed orally many months later, after I was trached, but by that time I didn't have a lot of interest in learning, or maybe it was just too difficult. They called it Dysphagia.

My mom tried really really hard to help me learn to drink and eat but in June 2007 it was decided that I should have a gastrostomy so I could get that tube off my face and out of my airway. It was the right decision.


A few months later the Bard was replaced by a Mic-Key button that allowed my mom to start the process of feeding a homemade blenderized formula made from real foods. Even though I was fed through a tube I enjoyed sitting with my family at meal times and tried a little of what everyone else had. Sometimes I tasted things but swallow studies had shown that when I swallowed I was at risk for aspiration (getting food or liquid down in the lungs). For awhile we thickened my liquids with Simply Thick since thin liquids are the most difficult to swallow (they move quickly down the throat).  My mom worked really hard over the years to get me over my dislike of textures and to build my strength to eat. We celebrated every milestone along the way. We did intensive work in the Summer of 2014 and I started eating regular table foods that year, transitioning to fully oral eating in December 2014 over winter break. My g-tube was removed and surgically closed in July 2015. This was a huge accomplishment! Now days I LOVE to eat! You name it I eat it: hotdogs, pizza, popcorn, chips, salad, cake, curry, chili, chocolate the list goes on and on. But I still don't like ice cream very much.

People often want to know if my mom had a normal pregnancy. Yes she did. She had the regular pre-natal care and my 20 week ultrasound results were reported as normal. However, when she was 8 months pregnant she was carrying rather large(as you can see in this picture). Wanting to avoid another difficult labor she went in for an extra ultrasound. They found she had polyhydramnios (excess amniotic fluid), now we know that was probably because I wasn't swallowing well.  During the ultrasound the doctor thought they saw that I had a condition called sagittal craniosynostosis but we would have to wait until birth to find out.

After that ultrasound they did an amniocentesis. My mom was told there was no way she would carry to full term anyway due to the large amount of fluid so they took the risk of having an amnio.  It turned out they were wrong about delivering early and she was induced at full term. The results of the amnio showed I have a balanced chromosome translocation p(1;7)(p22;q31.2).  What does this mean? 
Most balanced translocation carriers are healthy and do not have any symptoms. But about 6% of them have a range of symptoms that may include autism, intellectual disability, or congenital anomalies. A gene disrupted or disregulated at the breakpoint of the translocation carrier is likely the cause of these symptoms.
Essentially even with the amnio results we learned that we would have to be patient and wait until I was born to find out more about me. There were a few answers but also a lot of remaining questions.

At my birth a month later, they found that sure enough the bony plates of my skull fused early at the sagittal suture point. That caused my head shape to become long and thin as well as my forehead to bulge as the skull shape changed to accommodated my growing brain. I had to have surgery quickly to open  up the suture points and give my brain enough room to continue to grow. The back part of my head was reconstructed on 1/23/07 when I was 3 months old and the front of my skull was reconstructed on 7/24/07 when I was 9 months old.

When they did the front reconstruction they also replaced a bit of bone that was missing behind my eyes (greater wing of the sphenoid), though it has started to regrow by then, as well as reconstructed the brow. Unfortunately the swelling and/or repositioning of the bone to which the eyelid muscle (levator)  is connected caused a condition I was born with called ptosis (the appearance of droopy eyelids) to get much worse and my eyes to look slanted. We aren't sure exactly what happened to cause this unexpected result. My field of vision was affected but thankfully my actual vision wasn't even if people do assume that I am sleepy or can't see.

                          Morning of Surgery 7/24/07, 38 Days Post-op, and One Year Post-Op

Then when I was three on 1/7/10 I had eyebrow prosthetics placed in preparation for future surgery. It helped elevate the lids a little to widen my field of vision and give a secure anchor point for the sling that everyone knew I would need later. I am lucky that aside from the eyelids I have good vision and can see just fine. In December 2010 I had an eyelid surgery, called a tarsal switch procedure, to try to correct the results of the prior cranio-facial surgery. We were hoping the eyelid surgery would make it easier to see my beautiful brown eyes again and read my expressions. Unfortunately the results weren't what my parents had hoped for.
In June 2012 a Frontalis Sling surgery was done anchoring tendon from the levator to the brow, and allowing me to use my brow to lift my eyelids. Although the opthalmology surgeon at Children's had told my parents at birth that my ptosis would be fully correctable with a Frontalis Sling, he admitted that after what happened there was nothing he could do to restore the appearance of my eyes. Now that I have the slings I can use my brow to lift my lids, but not as well as prior to the surgery in 2007.

When they took a CT scan for the craniosynostosis they were surprised to find my cerebellum didn't look quite as big as usual. The fancy name for that is Cerebellar Hypoplasia. I seemed to be moving pretty normally so they were surprised. We don't know exactly all the ways that this will affect me in the long run. It's like Cerebral Palsy, in that it can affect people from a mild to severe degree and it causes symptoms similar to CP though in my case it we know it is specifically due to the cerebellum difference. Anything that involves muscles and the cerebellum/brain can be affected, and that means pretty much everything, eating, talking, walking, writing etc. I have a posterior walker that I use at school and when I'm out in public, but at home  it's easier to crawl around the house to get to where I want to go. Hopefully one day I will be able to walk independently without a walker. I'm working hard with that goal in mind.

A factor may be that my hips sockets weren't very well formed. They were too shallow allowing easy dislocation. Maybe because of the excess amniotic fluid or maybe this was "just because". A Pavlik harness in the early weeks did nothing. So in November 2007 when I was one year old I had hip surgery and was placed in a spica cast and then brace for 4 1/2 months and then wore the brace to sleep for another 6 months. This was difficult and we really hoped this would do the trick but unfortunately in the end I needed a bilateral hip and femoral osteotomies. The goal of this surgery was to keep the leg bones in the hip sockets to prevent arthritis of the hip and pain when I get older. The surgery was done on 4/05/10. I was put in a spica cast again and in another brace for 3 months. In December 2010 I had the plates that had held my legs together after the surgery removed, they had been causing pain and discomfort. You can see them here in the x-ray.

For two years I had this icky problem of sometimes throwing up when I cough up my trach secretions, in other words reflux even though I spent much of my early life strapped into a very restrictive reflux wedge. After a medical trip to the world-renowned Cincinnati Children's Hospital Aerodigestive Center we learned my stomach contents made their way all the way up to my airway (even when I didn't throw up) and that caused swelling to my airway surrounding the vocal cords. In addition to the airway swelling a doctor was worried that aspirating stomach contents could cause lung disease. I had signs of Bronchiectasis already (lung CT's have shown improvement since then). In November 2008, when I was two, I had surgery. It was a huge relief to the whole family because they could finally relax a little without having to worry that I would vomit into my airway (due to the open trach). In addition I went from needing suctioning 50+ times a day to less than 10. I finally gained weight after the vomiting stopped.

Due to the fact that I breathed through a tracheostomy tube I needed a speaking valve in order to make sound with my voice. Normally the air you breathe comes up through the vocal cords and produces sound. When you have a trach  the air goes in and out the tube before reaching the vocal cords. My airway was so swollen shut that I had no voice for the first 2 years of my life, until the fundoplication. I did get a speaking valve but I didn't love how it felt and would often remove it, which left me without a voice. Lack of a voice was only part of the problem, having a cerebellum malformation makes the motor planning and coordination of learning language hard. Despite that I score high on receptive language and can understand more than you might think. I can say "mama" and "all done" and try to say a few other words. I have Complex Communication Needs and started using AAC methods early in life, a simple switch, PECS cards, a Vantage Lite, IPad with TouchChat and in 2012 I started using a Nova Chat SGD. In 2014 I transitioned from a phrase based system to word based. I can communicate quite a bit with it. One time I even did show and tell by myself about my Teddy. In addition I communicate with gestures and pointing as well as some sign language.

Having language delays there was concern that I may have difficulty hearing. They tried twice to make me show them what I could hear with a behavioral hearing test but since I HATED animatronics that did NOT work.  Eventually I had a repeat sedated hearing test and that revealed, much to my parents relief, that I have normal hearing.

I started therapy when I was just 4 months old, with in home visits from a birth-to-three center. When I was about two I started attending "pre-school" at the center 2 days a week, during which I would also receive therapy. When I turned three I started to go to special pre-school on the school bus 4 days a week with my nurses. I received OT, PT and speech therapy during that time.

After preschool was of course regular school. I do some time in a Learning Center (Special Education) and some time in General Education and still receive weekly OT, PT and Speech at school.

I love school. I have an aide that goes with me on the bus who helps keep me safe and helps me all day to access the school and playground, communicate with others and with my academic work. I am learning more each day. It is an exciting time in my life now that there is less going on medically.

I have a big IEP with lots of measurable goals and a great team that works hard to help me do the best I can.

I am a valued member of my school community and that feels great!



On 7/21/14 my trach was removed. That was a great day! I have Obstructive Sleep Apnea, a common condition for many people but especially for those with lower than average muscle tone. The trach treated it well for 7 years, but it was decided that I should be given a chance to try going without my trach, using CPAP to sleep instead. I have been using CPAP to sleep every night since then. If I didn't my breathing would become more and more labored until I was unable to breathe at which point I wake up, unable to sleep.

The other thing about me you should know is that I have the best brother and sister in the whole world! Sometimes I annoy them, it's true, but that's my job as the baby sister.

They were only 5 and 3 when I was born and have grown up by my side and been a part of all of this. They help me when I need it, play with me, and love me for who I am and will stand up for me when I needed them to. They are special. Just look through the posts and you will see.


My mom makes us do home therapy work which can be pretty fun. But I love it when we make a fort together, play dress up, dolls or Calico Critters but the best is when they play Disney Infinity or Wii games with me since I need a little help. Even though they are pretty much teenagers now they are pretty great and I know I'm a lucky sister!

In July 2015 I had my trach stoma surgically closed as well as my g-tube and have no other surgeries planned for my future. Of course we know to "Never say never." My family and I are hoping for a break and time to just be a kid doing regular kid things. You can see I've been through a lot in my short life. Luckily I am a very happy girl so all this stuff doesn't get me down. If I like you I just might flash you a smile that'll melt your heart.


If after all that you still have questions you can e-mail my mom at ainsleyblog@comcast.net. Tell her I said so.  And if you found this interesting you might like this video of my first year of life. 

26 comments:

  1. Just finally read Ainsley's story, after following your blog for a while. What a story! I totally relate to parts of it...while Ainsley and my daughter have different medical conditions, they both have trachs, g-tubes, and a slew of doctors and therapists. I am impressed that she lets you put the Passey Muir valve on---my daughter hates it and won't wear it unless in speech therapy! Anyhow, Ainsley has come such a long way. Kudos to your whole family!

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  2. Thank you for your story. My 7 month old son has a trach and I have looked for motivating stories of children with them. Thank you also for being such a wonderful mother; these "unique" children need, and thankfully are born to many times, mothers whose love surpasses all of their physical disabilities! Many times I pray to God thanks for giving me Malachi, instead of a woman who would let him die off or suffer. I thank Him for other mothers such as yourself!

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  3. what an amazing story. Please feel free to follow mine. My daughter has a trach and gtube. Its interesting...my daughter has a tendency to vomit and gag when coughing up trach secretions and I suction SOOOO much during the day. hmmm

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  4. My daughter is the same age as Ainsley and somehow your daughter reminds me so much of her. THere is an inquisitiveness and mischeviousness in the pictures you take of Ainsley. I think of your daughter often and wish you the best of luck!

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  8. Hi Ainsley! Your mom has told me all about you and your blog is really interesting. I love the video of you using your new walker, looks like you're a pro at it already! I've always loved the name Ainsley and if I were to have another baby girl I would name her Ainsley! Hopefully we can meet one day soon! Alison

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  9. Hi what is her condidition called?
    she is such a beautiful and brave girl and has got a wonderful stry

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  10. You are an amazing blogger, to say nothing of mother (and RN, OT, SLP, PT, etc.)"Be strong and courageous...for the Lord your God will be with you wherever you go." Joshua 1:9
    Stephanie
    Lynnwood, WA

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  11. Best of luck and prayers to you always. I too am a mother of an Ainsley Rae!!!

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  12. I'm very pleased to meet you, Susan. You know what? You're the sweetest mom in the world! I'm very sure that Ainsley is so happy to have you as her mother. I'll be praying for her safety and good health.

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  13. I can only say what an amazing, brave little girl you have and kudos to you mom for being there for her every step of the way. God bless you both!

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  14. Thanks for sharing. My son is 11 and has Apert Syndrome. He just got a trach last June. We are in the process of decannulation. We just downsized and scoped, he has mild tracheal stenosis. We are trying to cap but he is not getting air around it, maybe due to scope plus he is on antibiotics for tracheitis?? He also had sleep apnea but did well with Bipap. Hoping we can get sleep study and decan soon. YOur daughter is amazing and so is mommy.

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  20. She sure has grown! What a great kiddo

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  21. Thank you for sharing your story, what a great mom and beautiful daughter. You are an inspiration!

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  23. My wife was watching your videos under my YouTube username without realizing and so they showed up in my history.
    What a lovely family. How wonderful your older children are with Ainsley is inspiring. You expect mom and dad to do that hard work, you're inspired when siblings do.
    After a handful of videos I was growling right along with Ainsley while she was working on her speaking while making tiger claws at the screen and yelling "Go Ainsley" as she mastered her half crutches before I could stop myself lol.
    I don't miss a whole lot about having retired from driving cab in the big city but I sure do miss my special needs kids.
    All the best to you and yours,
    Les

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  24. Susan, Steve, Evie, Adrian, Ainsley: I love your story! Please email me back, I've longed to talk to you for so many years!! You all have been busy I see, I come here often searching for a way to contact you, and I guess this is it! We
    all think of you often and remember the good ole days in Seattle! A bond cannot
    be broken! Jen, Sydney, & Griffin...

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  25. Ainsley your truly amazing!! Wanna hear something interesting? I had a tracheotomy (trach), a gtube and I also have a full fundoplication too! I got my trach when I was 3 days old and my gtube when I was 6 months old. I think I was also 6 months old when I got my fundoplication done as well. Eventually my trach was removed when I was 3 and the aroma was surgically closed when I was 5 or 6. My gtube was taking out when I was 9 or 10 and it almost fully closed up on it own. Eventually I'll have to have it surgically closed the rest of the day cause it's still a tiny bit open. Anyway I just wanted to tell you what I went through so you know that I'm telling the truth when I sag I know almost exactly everything you went through. Also like you I have medical problems that caused me to require my trach, gtube, and fundoplication. I also had an IEP in school and had to go to a special learning center at school as well as having an aide up until 1st Grade and being in a regular classroom. If you want to chat with me that's totally fine! Just have your mom reply to this comment and I'll message you back. ALWAYS REMEMBER YOUR BEAUTIFUL!!

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