7/25/11 Laryngoscopy Results

Ugh. Here are the photos from today's laryngoscopy.

And here are the photos from her last laryngoscopy on 2/8/10 when they removed the granuloma and her "tonsillectomy"/adenoidectomy was done.

The main picture of interest is the one in the upper left of each "page". Does the one on the top look a lot different than the one on the bottom? I don't think so either. If anything I think the one on the bottom looks better. But then Ainsley cut her knee Sunday(go figure), it bled a lot and she cried for over 2 hours so there is a chance her vocal cords were inflamed a bit today.

Her surgeon came out of the OR saying things are better than he thought in her airway so he is back to not being sure the surgery is a good idea. We didn't nickname him Dr. Jekyl for no reason. We've been talking about doing a Posterior Cricoid Split for nearly 5 years. He says yes, then no, then yes and so on. Don't get me wrong I'd be happy not to put my daughter through an intense airway surgery the problem is she might need it. What changed in the 2 weeks since our appointment? His mind, basically.  It's rather complicated to explain but I'll try.

Here is the situation: Today he saw there is no collapse due to the trach. There is no granuloma (caused by the trach, like the one they removed last year). There is no significant subglottic stenosis (narrowness of the trachea below the vocal cords). Her vocal cords are a little short making her airway small (we've known that). They open but not super well. There is a bump, likely damage from endotracheal intubation at birth and scarring from then too (we knew that). The bump could be reducing mobility of the vocal cords plus the scarring.

Essentially her airway looks pretty good. This explains why she has been capping (covering the trach and breathing through her mouth and nose) for 2 1/2 years. Yes, things improved further in June after we started giving her Prevacid on an empty stomach in May, but really she's been doing well with the cap for a long time. The real problem is that she obstructs when she goes to sleep. Let me say this another way if she was able to do as well asleep as she does when awake we would have lost the trach last year or earlier.

The surgery could improve the situation with her sleeping by creating some space between the vocal cords. His concern is that he might do the surgery and it wouldn't help the OSA and she'd still need the trach. We knew that already. He also is concerned because there are risks: aspiration and decreased voice. We knew that too. He's also saying that the OSA may be in the nasal palate area yet 2 weeks ago he said he finally had to admit he didn't think so.

He said he would be more in favor of the surgery if we'd hit a plateau. I pointed out that in a way I think we have...like 2 years ago. He is encouraged that she is no longer removing her cap. While I agree this is a great change I think it is due to a very minor airway improvement since she'd been able to remain capped all day if her nurse was there to watch her every second. Her sleep issues still seem very much the same. And the photos are the real proof to me that things aren't much different.

He gave us as much time as he could to discuss it since he had a child in the OR waiting for him. And he understands all our points and concerns and "wished he had answers". This would leave us once again at the status of waiting and hoping she outgrows this. But I asked for a sleep study. He agreed to call it in and then said to make an appointment with him afterward. The problem there is that it takes about 3 months to get in to see him. So even if things went well (which they won't) we'd be looking at fall/winter before discussing things again which means no surgery anytime soon. So why do a sleep study when I know she can't sleep with the cap? At this point I think we need a base line to really show whether things are improving or not. And I want the doctors to see it themselves, so they aren't just relying on our reports about her sleep issues.

I respect that he is in a difficult position.  That there is a lot at stake. That there are no clear answers. I just wish he didn't keep suggesting surgery as an option just to change his mind.

ENT Follow Up, New Wheelchair & Mid-Summer Update

Yesterday's ENT appointment couldn't have gone better, really. The doc scoped Ainsley and we got the best view we've had to date. In part I was able to reason with Ainsley a bit more than in the past and she remained more calm so things weren't as wet. But it also looked like there was less redness in the upper airway, probably from the Prevacid changes. She remained capped for the entire appointment. He asked about sleeping and I told him that she still has difficulty sleeping with the cap on which is currently the biggest issue for her.

Since he got a better look he felt he could fairly safely rule out obstruction through the nasal palate area due to craniofacial issues, or in the area in the back of the tongue and he believes the area of difficulty is in the area though the vocal cords, which though they open, don't open real far. So he wants to do a laryngoscopy under anesthesia to evaluate her airway again and make sure the granuloma he removed (caused by the trach) hasn't grown back. He again talked about the options of a Cricoid Split surgery or cutting away part of the vocal cords.  We were able to get in on July 25th which is great since it's still summer so if he does decide that she should have surgery it might be possible to get it scheduled prior to the start of school in September, after which she is likely to get sick and cause the surgery to be rescheduled. Here is an image from the video of her airway with the cords open so you can see.

I took the trach out at the end of the visit so he could see how she did, while we signed consent forms. I was glad the ENT got to see how well she does for himself. For awhile we've been stuck at this point of her having a borderline airway just a bit on the small side. Well enough to do okay during the day (unless she cries or get exerted) but too small to breathe comfortably during sleep. I've always suspected she would need surgery in the end. But I also know airway surgeries fail. I would hate to put her through that and have it not work but we won't know unless we try it. Waiting for things to fix themselves also may not work. There will be more tough decisions ahead. Please think good thoughts for us for the 25th.

....And....

We finally got our Convaid Rodeo push style wheelchair last week. WooHoo, it fits her!  It was a real challenge to find the right chair for Ainsley's body type (tall, thin with a long torso but not a deep seat) and it is adjustable for growth. I didn't want to have insurance buy a chair this expensive unless it could last a few years. We wanted something that reclines so we can lay her back if she falls asleep, but in the end we got tilt instead so the chair is "transport approved" (is crash tested and has tie downs) and she can use to ride the bus to school. We'd been using the 7lb MacLaren Volo which is meant for age 3 and under. (It looks bigger because it's closer in the picture.) Now I will be hefting a 28lb + chair out of the car, but that's unavoidable. At least she seems to like it and be comfortable. I started shopping for a chair in September and we "ordered it" in December.  It was ordered without the tie downs, then had to go back, thus required reauthorization from insurance and Medicaid and was sitting at the DME since May. It is SUCH a relief to have this DONE. Although I just opened a letter from DDD/Medicaid saying certain parts were denied though the DME told me they got approval and obviously released the chair to us. So it's going to require some follow-up. Annoying. But at least we have possession of the chair!

Sunday Evie left for sleepaway horse camp. She was so cute in her cowboy boots and horse shirt. It's all she's been able to think about for weeks. She went with two of her best friends from Seattle and she was SO SO excited, had been looking forward to the day since we registered in January.  They sleep in covered wagons. I was a wee bit nervous about sending her to ride horses since her only experience on a horse is the pony rides at the fairs. Friday we go pick her up and see the "horse show". I can't wait to see her ride! I hope she has the time of her life! And doesn't come back grumpy, missing her friends! 

I'm trying to spoil Adrian a little since he's bored at home while she's gone. He turns 8 on Monday!  It feels like yesterday that HE was my baby. The years since Ainsley's birth have FLOWN by and she is not too far from turning 5! Anyway he's having a sleepover "campout" birthday party with a handful of kids in the backyard Saturday.

I LOVE this boy! (And my girls too of course.)

Since we just bought a house we have no exotic travel plans this summer. My hope was to do a lot of backyard play and entertaining. The weather here in Western Washington has been crummy, no sun and actually a bit cold at times. With all that we have going on I'm afraid summer is going to be over before it evens starts. So if you are lucky enough to be in a part of the world that is sunny and warm right now...ENJOY!

Upcoming ENT Appointment and 4th of July

Yikes I'm in a crappy mood today. Perhaps that's a sign that I need to blog. I guess it started when I got an early morning phone call that our ENT had a cancellation for this coming Tuesday(I was on the wait list). I should be happy since the earliest appointment I could get months ago was August 30th. With the new appointment perhaps if he wants to "do" anything there will be time before the end of summer. I know the minute Ainsley goes back to school she will catch a virus, and that makes scheduling any procedure (such as a laryngoscopy) under anesthesia dicey at best.

I guess I've been in my head all day thinking through all the possibilities and options. I would love to be optimistic, but I don't expect much to come from this appointment. In my fantasy he would suggest an airway surgery that would fix her airway and the trach would come out this year. I'm expecting more "wait-and-seeing". If nothing else it will be nice that he can scope her and see if the Prevacid changes have done anything to help alleviate the leftover airway swelling. The pharmacy wasn't properly labeling her prescription, so we are now giving it on an empty stomach as it should be. Thanks to my friends on the trach forum for giving me that information.  In addition we switched to the Solutabs which have a reputation for being more effective. She is having an easier time forgetting that she's wearing her cap. And she has been eating more. Since you can't see the airway the only way to know for sure what is going on is to stick the camera down her nose and look. We shall see. So stay tuned to see what the results are next week.

On a positive note, as I said, the med changes have caused enough of an improvement that we've seen a dramatic increase in her ability to swallow. I didn't want to say anything until I knew it wasn't a fluke. Now it's been nearly a month of her drinking a fair bit of her blenderized formula from a cup and straw. It's the consistency of a smoothie, but not quite as tasty because it's healthy and contains chicken and veggies in addition to the fruit and yogurt (and other ingredients). She's also become much more interested in table foods. She will put food deep into her mouth, even biting off pieces and spitting them out. She doesn't know how to chew and swallow them yet but she is enjoying the food and that is the main thing. We are probably still light years away from getting her to take all the necessary calories orally to maintain a healthy weight. But it is a massive bit of progress after years of frustration. Here are some images from the past month.

 This may be another factor. On 6/9 we taught her to spit food out. Counter intuitive, yes, but actually it gives her a greater sense of control now that she can get food out of her mouth by herself.

Over the past month she has developed a serious love of pizza crusts and will bite them into pieces (still not swallowing them though).

This is actually a bit of a problem because she's a tall girl and can now get into the pizza boxes by herself. 

And do this to the pizza. This does not make her brother happy.  Plus she is demanding of crusts. This is okay if you don't like the crust, but annoying if you do. Especially while you are trying to enjoy your pizza while she's mad at you because she wants your crust.

 



On 6/20 she completely wowed us by eating Yoplait peach yogurt which, by the way, has CHUNKS in it. Chunks which she has previously found difficult to swallow without gagging.

She even spooned it all by herself!

 Signing for more each time the bowl was empty.

Until she'd eaten then ENTIRE 8OZ!!!!!! WooHoo GO GIRL!

 She's demanding some of EVERYTHING we have to eat  at EVERY meal. This includes SALAD, which she really likes to put in her mouth now. 

One day the kids and I had a real tea party. Ainsley drank real tea from a china cup. Impressed?

She especially likes tortilla chips and popcorn but will put the "used" soggy ones back in the bowl if you aren't watching.  

On the 4th of July she impressed our family by biting a hotdog into pieces (still not swallowing obviously). Of course Nathan's are DELICIOUS. That shirt was cute and clean when I put it on her, I swear.

While I'm at it I guess I'll share some more 4th of July pics. We had a great time and it was fun to host since we live in one of the few areas still allowed to have fireworks, being in unincorporated King County.

Evie planting pinwheels.

Ainsley enjoying the lounge chair with Grandma Glenna.

 Our pergola decorated for the occasion.

The kids loved taking turns wearing this goofy hat.

Everyone had fun playing bocce. It's great to have a lawn bigger than a postage stamp.

We celebrated my mom's birthday which is actually on 7/5.

 I helped Ainsley do a sparkler. She LOVED it! And signed for more.

 As far as I'm concerned sparklers are the best. I'd be happy just with that.  But we did get other fireworks.

When the sun went down we started with the "flowers" in the street. Ainsley had fun sitting on the boulder with Daddy watching. 

Then Steve let off the rest in the backyard, mostly fountains.

Yikes. There were a few aeriels in the box but luckily we didn't catch the shake roof on fire. It's not quite like the big shows but still fun.

I feel a little better after blogging. Stay tuned for ENT appointment results next week. And pictures of Ainsley's push style wheelchair which we finally get TOMORROW after months and months of waiting. Hooray!

Father's Day 2011

Credit goes to my dear friend Lisa for giving me the inspiration and push to do this for Steve this year even though I really didn't have time. He IS a great dad. It takes a special man to parent a special child (+2). Thanks for all you do honey!

Nothing But Time

You know you've been at this special needs/medical complex kiddo thing too long when you no longer are annoyed beyond belief when you wait for 2 hours to see the doctor. Really though what's the point of getting upset? It wouldn't change anything other than making us more miserable. After its happened dozens of times you learn. So now we take it in good stride as "par for the course" as we play our favorite hospital game....."watch the clock".  Our appointment time: 12:45. Actual appointment time? 2:35.

Ainsley can be a rascal when she's overly tired, as the following pictures show. 

 Yes, she is drinking her lunch (while doing tricks). More about that another time.

 Getting bored (reallllly?!) she decided she wanted to crawl around the room. At the hospital this is ill-advised so we said no. So she then decided to get herself out of her stroller. I think that look says "Oh yeah?! Watch me."

 It's no easy effort for her.

And when she does stand the stroller and the suction machine go falling. We can't get that wheelchair soon enough. It's back from the shop but we are waiting on funding approval after their mistake of not getting the tie downs. Ridiculous is what it is. She is 42 pounds and 43.5" tall and far too big to ride in a Maclaren Volo. But hurray, she was able to get herself out by herself, another first!

She's so pleased with herself. And we are too. She's getting to be very independently minded.

Somehow she managed to stayed awake so the surgeon could see her eyes for the first time since the eyelid surgery.

Here you can kind of see that the hair of the brow is a bit lower than the actual brow bone (okay brow prosthetic). When she is relaxed and you lift the brow up a few millimeters it lines up nicely and her eyes appear more open.  You just have to love that smile, I just wish we could see her eyes when she smiles.

He didn't get the letter from the occuloplastic surgeon (it may be in his inbox) but is going to follow up with him to find out exactly what he has in mind because some styles of brow lifts (depending on how it's done/at what level) can make it harder to use the brow to lift the eyelid (not what we want). He wants to wait until it's been a year since her eyelid surgery so she is fully healed, so we are looking at December 2011 at the soonest but in the mean time they will discuss the best plan of action for her. They may determine that some other course of action would be better, such as a frontalis sling. Hopefully their plan will not involve waiting until she is a teenager.

Once again he said what an incredibly complex case Ainsley's is.  Though primarily she had sagittal craniosynostosis things were very much complicated by the absence of her greater sphenoid wings.  The fact that she has a weakness of the lid muscle in addition to the surgery that reshaped her forehead and replaced the sphenoid wings (It changed the shape of her eye orbits, her forehead shape, the position of the skin over the forehead, the position of the levator, the frontalis, the slant of her eyes as well as the possibility that there could be damage to the muscles from swelling.) makes it all rather difficult to sort out what the problem is and how to fix it, even for a highly experienced craniofacial surgeon and occuloplastic surgeon.  Luckily I had my handy photos with me to show him the progression again and he was able to see (among other things) that the brow implants that I'd suggested did in fact help her which goes to illustrate that what he would typical expect to see is not necessarily the case with Ainsley. A picture being worth a thousand words and all that I think it's always better for them to rely on images rather than their memory of how she's looked along the way.

As for her head shape: He said that unless she develops vomiting, headaches and other signs of intracranial pressure they would not operate.  So that's good. It is typical that the shape changes over time and wants to return to it's original shape and since her head size is almost mature it will most likely be okay. I was a bit concerned about the sudden change. So this was reassuring.

All this means that we will likely get through the summer without a surgery and that would be nice. I don't know about you but I am looking forward to summer!!!! Ainsley's last day of school is Friday. Evie & Adrian's is next Wednesday.  If only we could get some sun.

Looking Different

*This is the post I was drafting when the news of my friend Karin's passing came.  She will be terribly missed.
                                                               ***
Having a child with craniofacial differences is hard. People don't mean to but they judge. There is an assumption that people who look different are different. Even having a child who looks different, I have to fight against the human instinct to judge by appearance in the privacy of my own mind where no one else hears my thoughts. Such as......

Last week I went on a field trip with Ainsley's classmates, many of whom have special needs. I know and love my child in a way that others do not, simply because I am her mother. I know her life has worth, purpose and meaning. I also know that every mom out there feels the same way. And it's true. All life is precious and holds value.

I found it interesting to see reactions of these other special needs moms to my own child, who has a trach, feeding tube, is non-verbal and not walking. And from our conversations my impression was that some of them felt their own child was better off. In case you haven't met her, Ainsley is a happy child. She is a joy to be around. I can't tell you how many therapists, doctors, nurses, school workers and the like have told me how much they enjoy her. She has been through so much but she has such love for life in spite of it all. Other children may be more physically able but Ainsley's spirit shines. If I had to choose I would choose that. But as I thought about it I realize that too is a judgement. My child is no better or worse than another. More importantly there is no need to compare. We are each unique and valuable, with strengths and weaknesses. In case it's not clear I'm not just talking about those with "special needs".

And yet. Ainsley's life is not made easier by the fact that she looks different. I know that people assume things about her because of what they see.

Did you know this is what my baby looked like when she was 9 months old?

When people notice Ainsley's eyes they do not consider that her eyes look the way they do because of surgical results. Sometimes I feel like I have a hard time even getting the surgeons to acknowledge that fact and have come to appointments armed with photographic evidence. So it was with dread that I set out to her appointment on 6/1 with the occuloplastic surgeon to discuss the 6 months post surgery results. Even though it's been that long I still feel a stab in the gut when I look at her eyes and see how much of her lids were unexpectedly cut away, about 4-5mm of her upper and lower lashes. Although the surgeon didn't forewarn us that he would being doing that (beyond the scope of what we consented to) I understood the logic of his reasons why he felt it had to be done in the moment. I hate it, but understand it. And if I can find a positive it is that Ainsley has such thick beautiful lashes that I suppose only I will notice all those that are now forever gone. Somehow I was able to put on a semi-friendly face and voice my opinions and what I wanted done in a quiet factual manner and most surprisingly the surgeon agreed with me. I was shocked. Steve and I left looking at each other in disbelief that it could have gone that easily.

For several years I have said that I believe her brows are too low as a result of her 2nd craniofacial surgery because of how much bone was moved around. That is one of the reasons why they did brow implants in 2010. It helped a little, but the implants don't extend far enough toward the ears to lift the area that needed it most. The surgeon agreed that the eyebrow (hair) is in fact below the brow(bone). He is going to write a letter to the craniofacial surgeon recommending a brow lift. In addition one of Ainsley's eyes was stitched together tighter than the other and it has created a little pinching at the corner causing the eyelashes to turn in on each other and that eye looks a little smaller than the other. Though the surgeon offered electrolysis I really don't want her to loose another s-i-n-g-l-e lash, so he good-naturedly agreed to slit the corner during the brow lift (if we do it) to release those lashes.

In a stroke of extremely good fortune I was able to snag a canceled appointment for June 13th (Monday) which means not waiting months. The real reason for the appointment was that I've noticed Ainsley's head shape changing, and there is a risk that the bone that was previously fused, though operated on, will not allow proper long term growth.  Also, I think they would want to see the eyelid surgery results. Now we have the brow lift to discuss as well. Monday we could be discussing surgical plans for this summer. After what we've been through I am nervous. Plastic surgery carries risk and results are not guaranteed. You certainly don't get to pre-view the results. I'm so afraid another surgery could "make things worse" but at the same time I am not satisfied with the current results so if the craniofacial surgeon wants to do the brow lift we probably will.  I hope she doesn't need another reconstruction on top of that. Having your child's skull removed and cut into pieces and rearranged like a jigsaw puzzle is not fun.

We have a busy weekend coming up, a new speaking valve and ortho surgery follow-up tomorrow, a poker game, a baseball game, a family graduation, a ballet recital. I'm tired just thinking about it but will try update early next week to let you know what happened. I hope you all have a more relaxing weekend than I will.

True Friends

I was taking a sick day, writing a blog post from bed when I got the bad news, so that post will have to wait. A friend called with bad news about another friend.

In the years since Ainsley's birth I could not get by without the support of my friends on the Tracheostomy.com  support forum. The support it provides is a lifeline. Literally. Those who don't need that kind of support may not be able to understand it. These people are there through surgeries, medical testing, the hard times as well as to share the silly passings of everyday.  Some of these people are truly my friends even though I've never met them face to face. They are the ones who I know really care about me and my family.

At times during this journey I have questioned who my true friends are. There are a lot of things we think that a friend should do or be. A friend may not always deliver. Does this mean they are not a true friend? The subject of what makes a friend also came up this week as Evie is missing her old friends and trying to make new friends since our recent move. Can a friend be a friend if you don't see them as often, what if you never met them, what if you had a fight and miss them but haven't talked to them? There are so many ways that friendships vary. After giving it a lot of thought and today's events I think a friend is someone who knows our faults but loves us anyway. It's as simple as that.

Yesterday I was congratulating my friend Karin, from the trach forum, on the birth of her second son. After years of having a child with medical issues she deserved for things to be easy. Unfortunately she suffered an amniotic fluid embolism in the night and is not expected to live. Even though I never met her face to face I consider her a true friend and am devastated. Life can deal the unexpected. Tell your friends what they mean to you.

*Updated to say that Karin passed peacefully later that day. Here is the link to her obituary. If you feel inclinded please donate funds to her boys college fund at any Bank of America with the names Alex and Bryce Kincaid (father Mark Kincaid). On top of the cold virus I already had Ainsley picked up a super nasty stomach virus at the zoo and it's making it's way through our family. If I haven't yet contacted you to tell you that I love you, please know that I do.