MY MEDICAL STORY

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 but more about that later. Right away I had stridor (a kind of wheezing sound when breathing) and had to be intubated and rushed to the NICU. The doctors never figured out what the initial problem was but I acquired polyps, swelling and scaring from the intubation tubes and had to get a tracheostomy 5 weeks later.

I was so happy to have that tube out of my throat. Even though I was doing great and didn't need any ventilatory 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), what types of medical equipment and supplies would be needed and most importantly 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.  It was 12/22/06 when I came home, just in time for Christmas. It was so great to be home with my older sister and brother.

I never learned to eat because I had a tube down my throat so I was fed with a nasal-gastric tube. I loved to pull that thing out. I got the okay to attempt to feed orally many months later but by that time I didn't have a lot of interest in learning, or maybe it was just too difficult. My mom tried really really hard to help me learn to eat but in 06/07 it was decided that I should have a gastrostomy and get that tube off my face. I got a Mic-Key button a few months later. I am fed a homemade blenderized formula made from real foods. Even though I'm fed with a tube I enjoy sitting with my family at meal times and always want a little of what everyone else has. Sometimes I taste things but swallow studies have shown that when I swallow sometimes little bits of food get down into my lungs but the doctors say it's okay for me to try as long as we're careful and we thicken my liquids with Simply Thick. Over time I've come to enjoy drinking some of my blenderized formula so long as there is no texture.

My mommy went for an extra ultrasound when she was 8 months pregnant for having excess amniotic fluid.   The doctor thought they saw that I had a condition called sagital craniosynostosis. We would have to wait to be sure. After the ultrasound results they did an amniocentesis and found I had an apparently balanced chromosome translocation betwen 1:7. This is caused by a defect in the egg or sperm that is present even before conception.

At my birth a month later, they found that sure enough the bone plates of my skull fused early and caused my head shape to change to accommodate my growing brain. I had to have surgery to open them up and give my brain enough room to continue to grow. (And  make my head look nice and too.) The back part of my head was reconstructed on 1/23/07 and the front was reconstructed on 7/24/07. When they did the front reconstruction they also replaced some bone that was missing behind my eyes. 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. On 1/7/10 I had eyebrow prosthetics placed in preparation for an eyelid surgery. It helped elevate the lids a little to widen my field of vision. I am lucky that aside from the eyelids I have good vision and can see just fine. In December 2010 I had 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 and further surgery may be needed.

When they took a CAT 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 since the cerebellum regulates movement and many other bodily functions. Right now I'm doing therapy to try to catch up. I have a posterior walker but mostly crawl around the house to get to where I want to go. I've started to "cruise" and have plans to learn to walk in my own good time.

And if that wasn't enough my hips sockets weren't very well formed. Maybe because mommy had too much amniotic fluid because I couldn't swallow very well or maybe this is just "because". A Pavlik harness did nothing. So in November 2007 when I was one year old I had surgery and was placed in a spica cast and then brace for 4 1/2 months and wore the brace at night 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 osteotomy. The goal of this surgery was to keep the leg bones in the hip sockets, mostly to prevent arthritis of the hip and pain. 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 were removed, they had been causing pain and discomfort. Look at these pictures to see why.

For two years I had this icky problem of sometimes throwing up when I cough up my trach secretions, aka GERD . It caused acid damage to the airway (but not the esophagus) even though I spent much of my early life strapped into a very restrictive reflux wedge. In November 2008, when I was two, I had surgery to stop it. 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. Instead of needing suctioning ) 50+ times a day, to keep my airway open so I can breathe, to less than 10. Plus I finally gained weight after the vomiting stopped.

Due to the fact that I breathe through a tracheostomy tube I need 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 from the vomiting or GERD that I had no voice for the first 2 years of my life. Now I make sounds when I'm wearing my valve but I haven't learned to talk. I can say "mama" and "all done" and try to say a few other words. My vocal cords are still a bit swollen and having a cerebellum malformation makes learning language hard. Even so, I score high on receptive language and can understand more than you might think. I know close to 30 signs but having a cerebellum malformation makes sign language difficult for me.  We've also tried to use PECS but I think they are more fun to play with than to use for communicating. I am able to use gestures and sounds to get what I want most of the time. I am also learning to use a communication device to speak to people who don't know 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 HATE 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 2 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 get OT, PT and speech therapy while I'm there. I really love to learn. I especially like books. Next year I will start Kindergarten. But mostly I love to play with my brother and sister. They are the best.

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.

Here's a video of my first year of life.



If after all that you still have questions please e-mail my mom at ainsleyblog@comcast.net.