Mar 16, 2012

We Have A Date

And then some. Life has been busy and in my Happiness Photo Project posts I've alluded to there being a lot coming up with Ainsley.

There is a LOT that goes into having a "medically complex" kid. A lot of it happens behind the scenes and even our family and closest friends are unaware of what is involved. Honestly I think even my husband is, since I handle a lot during the days while he's at work. In addition to regular care of a child there are the additional demands. Everything takes extra time. Dressing, diapering, toileting, and positioning. Things that a child normal does herself. Then there is the medical stuff: tube feeding, suctioning, cleaning the stomas, ordering and stocking and cleaning medical supplies and equipment. Beyond that there is the "therapy" ie. working on eating, speaking, Ainsley's communication device, stretching her legs(from her hip surgery), standing, walking, fine motor (drawing, cutting, picking up little things) and then there are her educational needs because really she needs additional help to learn the alphabet, colors, shapes, numbers, animals.....well everything really. Her schooling requires an IEP and a fair amount of coordination and she receives PT, OT and Speech therapies through them.  And lastly there is medical planning and scheduling. This is huge and can take a LOT of time. Researching conditions, treatment options, finding products and services that help and then eventually scheduling and coordinating those appointments and surgeries. When I say it it is a full time job I'm not kidding. It's that and then some.

So here is a little (okay big) update with what's been going on. Ready?! Set. Go.

Ainsley will be in Kindergarten next year. Since she's been in preschool at the local school since she was 3 I don't expect this to be a difficult transition. We met with her teacher on 3/5 to discuss it.  The school district hasn't made assignments yet because they may be opening a new school and that would affect assignments throughout the district. However we expect that Ainsley will continue at the same school she is currently at. She will be in a "learning center" classroom, which is smaller in size to accommodate kids with special educational requirements and she will try to do some hours in the typical classroom.

I'm not sure how often I've talked about it on Ainsley's blog but she has difficulty safely swallowing large volumes, ie. she gets tired after so much swallowing(the amount varies from day to day). Just as the rest of her muscles get tired from low tone and her cerebellum issues, so do her swallowing muscles. And when she gets tired she could accidentally get food into her airway which would be very bad for her lungs. Regardless, we've worked really hard on self-feeding and it shows. She requests (with sign language) to drink her formula orally even if it is only a little. She also mouths table foods every night at dinner. Although she started OT when she was a baby and I continued the techniques I learned 4 years ago she is stuck at the same place. She is less interested in puree and won't eat any puree with any texture in it so bridging the gap between smooth purees and actually swallowing table food is where we are stuck.  Every time I add small amounts of texture she will do okay until I reach a certain point that is "too much" and she rejects it.  We need help.

A frustrating aspect of having a medically complex kid is that everything is related and is a process that takes time. I'd planned to request a referral to a feeding therapist at her cranio-facial clinic appointment. The soonest appointment was over 2 months away in December. Then December came and she got sick with RSV and the appointments all had to be rescheduled. Then it was February before we could get in and after the appointment we had to wait for a referral. Then we had to wait for an opening. So although I started the process in the fall it will be spring before she actually sees the therapist. 3/28 to be specific. Let's hope nothing happens and that the therapist has good ideas to help Ainsley.  It is not uncommon for children with trachs to have difficulty eating. Normally our physiology doesn't allow breathing and eating at the same time, but having a tracheostomy does and increases the risk of aspiration. Plus she may have diminished taste and oral sensation due to the lack of airflow through the palate. Having a cerebellum malformation in addition to a tracheostomy makes it just that much harder.  Many kids develop an oral aversion but luckily Ainsley wants to eat, so that should help. I'd love to get her into an intensive feeding program but I'm not sure they will allow that. And with her high caloric needs it's unlikely that she'll be 100% tube free anytime soon. But we shall see.

Communication Device
We started using PECS cards and sign language with Ainsley when she was under 2. She then graduated to more and more complex communication devices over the years. I have compared the options and even went through an evaluation at the hospital last year just weeks after Ainsley's eyelid surgery. The timing was bad so I decided to put off the decision. Usually insurance will only pay for one device every 5 years so it's a big decision and you really want to get the right thing. The device they need at age 4 could be very different from what they need at age 7.

When we moved into the Lake Washington school district we lost the Vantage and it was agreed that we would try an IPad with communication software on it, provided by the school. It requires a lighter touch and different skills we wanted to see how she would do. After "playing" with it for nearly 6 months she has progressed a lot. She can even swipe to scroll through pages. She manages to change the wallpaper, move icons around the desktop and even get onto YouTube (scary!).   

It's amazing to look back. There was actually a day when part of my "therapy homework" was to working on teaching her to point and it was really slow progress. It was too hard for her to keep her arm steady, she couldn't keep the other fingers out of the way and it was hard for her to guide her hand to the precise location.

Last year I started the process to get a re-evaluation. This too requires a referral in the system, copies of her IEP and speech evaluations, signed forms, completion of a lengthy questionnaire etc.  I'd heard about a new communication device that would be coming out at the end of the year called the Nova 7.  My fingers were crossed that this was the "one" and it was. Things I like about it: better pronunciation of basic words, handle and neck strap for portability, a sturdy stand(IPad doesn't have this), a built in amplifier for use in loud rooms, touch screen technology(with Gorilla glass for durability), a rubberized case in case she drops it, a child's voice(nice to have for a child so Ainsley doesn't sound like an adult with a British and Bostonian accent who doesn't know English very well), software to allow programming on the PC (instead of just the device), and continuity of use (the Nova 7 can use the same screen layout as her current AAC).

Paperwork was submitted this week and we are waiting for funding authorization to come through.  Here are some pictures and video of her with the new device. We're also waiting on a referral for a speech therapist to work on vocalizing. I haven't given up on that yet.

A close up.

You can see she loved it. It comes with a stylus too, a nice feature until you lose it.

It uses the Android platform. Here it is next to the IPad so you can compare size. I asked her and she said she likes the Nova 7 better.

I decided to get AFO's for Ainsley. She'd had a pair of soft AFO's. Not custom but the same idea. They really didn't make much difference for her and I was afraid that her ankles would become weak in the braces. She wore them at school every day until she outgrew them. We used DAFO Pollywog inserts for the past year or so. They helped keep her foot in a decent position but even after the leg plate removal more than a year ago and the progress she made initially she is no closer to standing or walking. The way I see it, it's worth trying them. Though it was a very traumatizing (it shouldn't be but it was). She started to get upset when he wet the plaster and then became really upset when he went to cut them off.   Here's the process for those who would like to see:  

1. Take off shoes and socks (Note still happy.)

2. Add stocking and making marks with Sharpie for ankle bones. Note tube inserted into stocking.
3. Pick out brace material. Fun. Ainsley chose butterflies.

4. Wrap legs with plaster coated gauze. 
5. Add water and make gooey plaster. Things start to go bad.

6. Put foot into ideal position and hold until firm.
7. Cut cast off. Tube protects skin from being cut.
8. The orthotist inspects the cast

9. Ainsley signs "all done". Sadly we were not done and when he started the wrap a second time she lost it and then when he had to re-do it she came unhinged.

10. After a week or so the braces arrive and are fitted to the patient.  She remembered him and started to cry again even though we explained there would be no casting, though it wasn't quite as bad as the casting day.

Here she is at home wearing them. Luckily I had some hand-me-down Keen's that worked. Then we worked up to wearing them an hour at a time. She wears them to school but signs "off" the second she gets home. Her pleading little face is hard to resist. They make it hard for her to get up and down from crawling and her gait is a little odd still. I hope it gets better. We need more time before I commit to an opinion.
Check out this video of Ainsley cruising (with support) the biggest obstacle for her is standing balance. In February we met the new orthopedic doctor for routine follow-up. Ainsley's x-rays look good and although she will still need an x-ray every 6 months, it is unlikely that any further surgery will be needed. Her hips have pretty good coverage over the femur and the donor bone appears absorbed. The legs look good where the plates were removed. The reason she isn't walking isn't really her hips, but having hip dysplasia really didn't help any. Plus she's spent so much of her life unable to move. Now days she's been rockin' the hammock and it is helping her a lot. She can now get in by herself sometimes. It's been really fun for her and that is good because it motivates her to be in a semi-standing position and her legs are now quite strong. She also uses a walker at school every day and weather permitting, in our driveway. She does some walking in the house every day, holding our hands. It's on Steve's to-do list to remove her arm troughs and add the hand grips to her walker. I'm going to make bumpers for it so we don't have to worry about the walls and furniture. Stay tuned for pictures. I doubt she'll use it much on her own (likely she'll prefer to crawl) but at least we can use it for PT during the rainy season.

Ainsley has gotten pretty good at climbing. At the doctor recently she climbed in and out of the wheelchair completely by herself. She can also get in and out of a chair at the dining table. And on and off a bed or couch. It's great to see her be able to be a bit more independent.

Oh my gosh is she going through a phase. I like to give my kids lots of hugs and kisses and she's learned that I like kisses (there was a time when she couldn't do it so we made a big deal when she did) so lately she uses kisses to try to get what she wants. She comes over to the computer and kisses me over and over asks, with sign language, for a DVD. It works. And now she's kissing me all the time and you can even hear her kiss. That's good for her lips. I hate that I have to think that way but after years of working with Ainsley just to try to make an "O" sound I'm rather desperate for some progress. I want her to at least learn to say a verbal yes and no. One day I'll be sorry when she learns the word no, but I know that.  For now we're using a combination of things. Vocalization, sign language, body language word approximations, and the IPad. Here is an example of how she communicates with her AAC device. 

I've been planning on taking Ainsley to Cincinnati to get a second opinion in the Spring after cold and flu season.  They have a top rated airway team and some advanced testing. The fact is Dr. Cotton probably sees more complex pediatric airways than anyone else in the world. Our surgeon has been on the fence about a cricoid split since Ainsley was a baby. We've been encouraged to wait and see if growth helps her, and every year we're asked to wait longer. I've suspected all along that she'd end up needing an airway surgery.  But if Dr. Cotton tells us he isn't confident that surgery would help then we can lay it to rest.  We hope Ainsley won't need the trach for the rest of her life but if she does so be it. If he thinks surgery is a good idea then we'd probably be hoping to get it scheduled over the summer.

We got a date today and we will be there from 5/21-23. I am hoping to meet up with some of the moms from my pediatric trach group while we are there. My sister Sheryl has agreed to come with me. Hooray!

As you probably remember, I was very disappointed with the result of Ainsley's eyelid surgery in December 2010. I had hoped that the appearance of her eyes at birth could be restored to her. The surgeon did what he felt had to be done. I won't go into it in detail again, the whole thing is rather complex. But before Ainsley's second cranio-facial surgery when she was 9 months old her eyes were far more open and straight. In fact they didn't close at night and we had to put ointment in them to keep them from drying out. Now we have the reverse problem, they are too closed.

I have struggled to understand what went wrong, and the doctors weren't sure. But after giving it countless hours of thought I think it comes down to that her eyelid function was poor at birth but the excess bone from the sagittal craniosynostosis helped lift the lid and so her eyes appeared much more normal. When the bone was removed/reshaped it created droopiness.

We've been considering a frontalis sling operation since she was a baby. She needed to be 4-5 years old and now she is. We also needed her lids to be in their final position. Both the brow implants and the tarsal switch surgery each elevated her lids a few mm's. The brow implants also give the occuloplastic surgeon a solid surface to work with because the bone was thin.

What they do is attach some material (could be synthetic or tendon from her leg) to the muscle in the lid and the forehead muscle. It helps elevate the lid(s) and compensates for the abnormal muscle tissue (which combined with the cranio-facial surgery is the reason Ainsley's lids are so droopy. The surgeon will have to be careful not to lift them too high or she might not be able to close them. Luckily this is a surgery that can be undone or adjusted if needed.

We've recently thought perhaps we should go back to the surgeon who did the tarsal switch because he's been "in there" before and knows her anatomy. Plus we would like him to open up the left eye by a mm in the corner. Also he does cosmetic work so he will have a slightly different approach than the other occuloplastic surgeon who deals more with kids who have complex vision issues. We will at least meet with him and see. Our appointment is 4/3. Our goal would be to have the surgery some time this summer.

I know that her eyes will never be the same but I do hope this helps for so many reasons. I miss being able to see in her eyes like here. My friend Christy said to me that that alone is enough of a reason to do the surgery. I'd like to think she is right. For those who don't remember here is what they looked like before.  This was her normal eye position but she could use her forehead muscle (the way the sling will work) to lift them fully open which she did a lot of the day (before the surgery). They are so dark and beautiful. I can't explain how hard it is to feel you can't see into your childs' eyes.

Physical Therapy
I also asked the craniofacial Ped for a referral for physical therapy. I would like to work with someone who can teach Ainsley how to fall. I think that if she wasn't afraid to fall she would take more risks to try standing which really is a precursor to walking. Nearly every day I stand her up and hold her gently at the hips and try to see if she can stay in position if I let go. She only needs the tiniest bit of a touch. I remember her physical therapist, Gay, doing that with her when she was 2. She was able to do the same thing then. I don't know what it's going to take for her to learn to balance on her feet. Ainsley receives PT through the school but it's just not enough.

We've had great success with the hammock. Ainsley hangs in it for hours every day and that is great for leg strengthening because she is somewhat standing in it, (with the support of the hammock under her arms). She also loves to sit in it and swing. That is great for the vestibular system (helps with balance).  I've got the supplies to make a bouncer which I hope will get her in a better more upright standing position.

So we're gearing up big time. Hopefully there will be good news to share in some of these areas in 2012.  Thanks for reading.


  1. Thank you so much for the update! I've been wondering how Ms. Ainsley has been! :-)

  2. You are crazy busy Susan! I loved this update and reading about all the goings on with Ainsley. Never a dull moment, that's for sure. Even though I don't always comment, I check up on you guys all the time.

    I can only imagine how frustrating and sad it is for you that you can't see Ainley's eyes like you used to. It is a big deal and I really hope you can find a surgeon who can help Ainsley get her eyes back.

    As always, thanks for the update ...

    Lots of love,

  3. What a great post! It must have taken you days to write! I feel like I need to read it several times to let it all sink in!

    You're doing a fabulous job, Susan. I know you probably don't believe that or feel that a lot of the time. But you really are! I'm super impressed and Ainsley is doing fantastic! Consider yourself hugged.

    Much love,
    Christy xo