Spring 2014 Medical Update

Here's that update I promised. Even when things are relatively calm there is a lot going on medically.

Echocardiogram
On 5/9/14 Ainsley had an echocardiogram to make sure that her heart is functioning properly. She's been having extreme foot pain that wakes her up in the middle of the night, and plus we know she doesn't have great blood flow to her feet (orthopedic surgeon said so after hip/femural surgery). Plus her heart rate has always been a bit on the high side. When the results came in we weren't terribly surprised: her blood flow is all correct and everything seems to be working well....but there is (of course) an anomaly. When we are born there is a small opening between the heart that normally closes after birth when it doesn't it is called a Patent Foreman Ovale, and in about 30% of adults this opening remains open. Thankfully we do not expect this to cause problems for Ainsley but will keep our eyes open for changes.

Orthopedic (foot) Appointment
On 5/21/14 we saw the foot specialist at Children's. He was great, had a great bedside manner, took his time examining Ainsley thoroughly and explained things well. Essentially she has a good degree of flexibility in her foot(feet) that clearly causes pronation and arch collapse. He further confirmed that AFO's do not in any way prevent long term damage to the foot. That if the foot is flat it is flat though the orthotic may help with function it is not going to "fix" the foot.  He joked that "I have it on good authority from the guy who wrote the book....me!" Since therapists will often claim that not using orthotics will cause "things to get worse" I was happy to confirm once again that when deciding whether or not to use orthotics the only consideration is whether or not they help the child function.  

About the pain mentioned earlier in this post. The episodes have become more frequent and it is not uncommon that she gets them at least once every week. The fact that her foot is not tender to the touch during the day is good. It is not uncommon for children to wake in the middle of the night with extreme pain, that is labeled "growing pains". It's responds to a hot pad, or hot bath. When I was a child I would wake up crying with intense leg pain and was diagnosed with "growing pains". The trouble is, I continued to get them in my 20's and 30's and still do from time to time. So although he expects that she'll outgrow them, I am not so sure.  

AFO's
After trying various foot orthotics over the years we decided to go ahead and try a more supportive orthotic for Ainsley.  She's been wearing insoles for awhile. She'd outgrown them so it was a good time to try something different. We wanted to make sure they didn't put too much pressure on her feet since she doesn't have great blood flow, so the therapist opted for a custom design with foam padding. After being custom fitted we went to pick up the DAFOs and found she was still pronating so they added a wedge and mailed them to us. They arrived last week so now I just need to find shoes that they will fit into. Some photos of the process.

They wrap the foot with a special tape that quickly forms into something like a cast which they use as the mold to form the orthotic at the factory. This materials is far less messy than plaster, which they use when you have AFO's casted at the hospital. 

Despite the fact that it doesn't hurt Ainsley hates it and it's so hard to see her so upset. Mostly I think she is scared of the part where they cut it off her leg, even though they use a guide (the yellow strip). Maybe it's good that she knows to be afraid of an Exacto blade, I don't know. It's a relief when that part is over. In my hand you can see the first "cast" while the therapist is cutting off the second.

The great thing about Ainsley is that she bounces back fast. Once it's done she's ready with a smile. We picked out the patterns and colors of the different components.  

We waited to get the call that they were in. At a second appointment the therapist trims the orthotic to fit and makes sure that it's perfect. They are made too long intentionally, for just this purpose.

She felt that Ainsley was still pronating too much so she sent them to the factory to have a couple wedges added to the soles and had them shipped out to us. 

When we tried them on the carpet they didn't seem to help her stand, in fact she seemed less stable.  Hopefully that will change as she gets used to how they feel. The hope is that with a more stable foot base it will help her balance, which is a particularly difficult aspect of learning to walk for Ainsley due to her cerebellar hypoplasia.

Neurodevelopmental

Speaking of cerebellar hypoplasia I was able to get a referral to a doctor at Children's who is top in the field of hind brain malformations. Our neuro developmental ped spoke with him about Ainsley (they do research together) but I would like to review the brain MRI results with him myself. Unfortunately both doctors are nearly impossible to get appointments with and after multiple follow up calls their available calendars are fully booked, AGAIN. And so it's looking like I'll be calling back in a few weeks and we'll be lucky if we get in this summer. 

IEP changes/School

Today I am too tired to write about this in detail as I'd planned.  The main thing I wanted to talk about is that after over 4 years using a walker Ainsley was really no closer to independent walking. I felt it was time for a new approach. I asked that they incorporate more standing into Ainsley's school days (since if you think about it that is where she spends the majority of her time) and they did. In addition we came up with a few creative ways to get a bit more standing time in at home. For example if she wants to watch a video she has to stand (see above picture). We also have used the dining room table to setup toys she has to stand to play with. Or I let her play at the sink with bubbles and reward Evie for watching her with additional IPod time.

Like this train set. 

After a month of that she was doing great. And she went from being able to stand independently (not holding onto something) for about 3-10 seconds to about 20-30 seconds, even reaching 55 seconds a week or so ago. Then she caught a virus...with 103 fever. She missed 3 days of school. After a full day at school she is tired anyway, but when she's not at 100% doing additional physical work is too much and we have to back off. As always we find ourselves trying not to be discouraged by getting 2 inches forward then 2 inches back. 

Ainsley qualified for extended school year and we will try it for the first time this year. It's just in July. We hope it is good for her and keeps her progress steady. With so many goals and areas to work on it is difficult to formulate an educational plan that "does it all". I've come to realize there is no perfect place for a child like Ainsley. She likes school and is well liked, the teachers and aides work hard and it's our best option but she isn't making as much progress as I would like with academics. 

Wheelchair status

We've been working to get Ainsley a new wheelchair because she'd outgrown hers and opted for another Convaid Rodeo (ours will be solid black), the recline/tilt/transit ready model. We had some delays a long the way. Sometimes I can't believe how many follow up calls I have to make for things like this to actually happen. After a year from the first evaluation the chair is in and we have a delivery scheduled for 6/26. At some point we may need to get a different type of chair, but for now I don't think Ainsley has the skills or strength to push herself for long periods, so a push chair is what we need. A transit model so that she can get on and off the bus safely. I'd love to see her progress to the point of being able to walk on and off the bus next year. We'll add that to our goals.

Oral Eating

Ainsley was transitioned to nearly 100% oral feeding over Christmas break. I'm pleased to say she is still doing well. We've had some difficulty while she's been sick, but were cautious about using the tube too much for fear of setting her up to regress and she seemed to make it through okay. I'd love to see her put on a little fat, but I think that will happen in time. This subject deserves it's own post at some point in the future. 

Surgery update
Awhile back I told you we consulted with the occuloplastic surgeon and he had a surgery in mind. Then Ainsley had a large (and suspicious) amount of discharge come out of her eye and her eyes seemed more open after that so we went back. Luckily I was smart enough to do that because he changed the surgical plan. We waited for scheduling and had a date of 6/9, then a week before we were told the hospital took or date and the new date is 7/15. It's frustrating.  Yes it's not a life threatening emergency, but if you've ever had an eyelash in your eye you know it's not comfortable. Imagine if it was rotated inward and poking your eye and it stayed that way 24/7 for months. I can't tweeze it out (what we've been doing) because they intend to do electrolysis to it, but have to have the eyelash there to do it. With our luck the lash will fall out before surgery and we'll have to reschedule. I'm tired of cleaning up eye drainage multiple times a day and just want it done for both our sakes.

CPAP/Sleep Study/Decann
It's that time again and I am conflicted but am sticking with my decision to put Ainsley through another sleep study. I felt the results from the last one were skewed by my being in the bed (to keep her from pulling off her cap). Last year she'd slept with the CPAP and cap for the week before the test so it didn't make sense to me that their CPAP machine was insufficient even at higher pressures than we'd used at home. So this time I will sleep on the cot. I fully expect the results to be poor and show that Ainsley has severe OSA that will require CPAP if she were to get decannulated (her trach out). 

Over the weekend we reviewed video of her wearing the CPAP from our preparations last year. 

She's matured a lot and though she didn't love it she wore the mask with less fuss. Last night she fell asleep on my shoulder on the recliner but after an hour or so she awoke suddenly in terror, crying. We weren't sure what happened and think she either pinched her mouth or was just disoriented and scared that the mask was on. It's hard to imagine signing up for wearing this contraption all night every night, especially when she's sick. We'd love to live a trach free life but I suspect it's not in the cards at this time. The sleep study is scheduled for this coming Sunday, the 22nd. 

I considered cancelling because this is how much she loves a sleep study. It's about the worst sleep you'll ever get and I don't know if she'll be fully well by Sunday. Every time she ends up sick a few weeks before and we are in this predicament, but the appointments are made 6 months ahead so if you reschedule every time they've been sick it might never happen. To be honest where my head is at right now is that I'm going forward so that I have data  to compare to in the future. I expect that she'll need to keep the trach and it will be nice to have it settled in my mind so we can focus on other things.  

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TOMORROW is the LAST DAY OF SCHOOL, just a half day. TODAY I am headed to Ainsley's school for an end of year celebration and then to Adrian's school for ELEMENTARY GRADUATION. It's bittersweet. Adrian is excited to be moving on but it feels like the end of an era to me. Yes Ainsley is still only going into 2nd grade, but her school experience is so different that it doesn't feel the same. Evie has one more year and then will be in HIGH SCHOOL.  Lord help me. The days go by so fast. I am hoping to cherish my kids a bit this summer before happily sending them off to school in the fall.