A Visit With the ENT and CPAP Update

We've been talking to Ainsley's otolaryngologist (ENT) on the phone every few months. Mostly because he's impossible to get an appointment with so if I have a question I call. Plus when we last saw him in March he told us to come back when she was ready to cap during sleep which she really hasn't been. Toward the end of summer, during one of these phone conversations, we talked about doing a trial decannulation but he wanted us to see her Pulmonologist first. He put the kabbash on that and steered us in the direction of CPAP. That was okay, because through my own testing and trials I'm sure she has obstructive sleep apnea and was pretty sure she would fail decannulation at night because she wouldn't be able to sleep unless she could use CPAP. The pulmonologist surprised me by setting us up with a CPAP machine for home use to get her used to it before we attempt to decannulate, maybe this spring. That seems like a good plan. She's more likely to be able to tolerate CPAP if we do it gradually than if we'd simply pulled the trach and required her to sleep with the CPAP on. So that's the CPAP history.

The respiratory therapist who gave us the CPAP machine made a comment a couple times, saying that maybe she just isn't ready to decannulate. I shouldn't have listened because she hardly knows my daughter but it started the nagging what if questions in my head. This comment had me worrying that she's not really ready to go without the trach while awake and all this work getting her to tolerate CPAP would be for nothing. In a stroke of serendipity I called the ENT out of the blue last week because I remembered in Aug/Sept. he said he would help us figure out if her pulling of the cap was behavioral or not. He agreed to see us and squeezed us in on his surgery day, and I was so appreciative! Today was the day. After talking and doing a naso-endoscopy Dr. I thinks it's probably mostly behavioral. She makes a little sound while she breathes when she's capped but he says it's okay, mostly likely it's coming from the narrowness at the vocal cords. Some kids are noisy breathers and they are fine without a trach. Her airway is still somewhat swollen at the glottis. (I'll insert photo from DVD later.) So we will try 2 months of Prevaid and do another endoscopy to see if it helps or not. He is not optimistic but since it's the least invasive "fix" it's worth a shot. We'll take another look at the airway in 2 months.

Another good thing. The subglottic stenosis that was noted in an operative report was really glottic stenosis or in other words the swelling/narrowness at the vocal cords. So we lost a diagnosis. It's always better to have less airway problems. And he felt that her lower jaw is not the problem. There is a lot of space for her tongue.

He is thinking that she is obstructing in the soft palate during sleep . It is possible that Ainsley could require surgery to her mid-face or upper jaw. Cranio-facial will not decide about a mid-face advancement until she's 8 or so. If she stays about the same it's likely they'd suggest orthodontics instead. We may be consulting with an oral surgeon here soon. They may do x-rays to determine if the sleep issues are in the nasal/palate area. Dr. I will talk to cranio-facial and put in a request to get the x-rays and a consultation. If it's not the palate then it may be that the glottic area needs to be worked on. Either with a Cricoid Split where they remove her rib cartilage and use it to widen the voice box and spread the vocal cords a bit apart in the back. This would help because her arytenoids are a bit stiff from scarring due to previous intubations and swelling. Or she may need a partial cordectomy, where they remove a little of her vocal cords.  Since the issue is sleeping, we need to know which area is the problem. It may be both.

If it were not for the sleep issue Dr. I would try decannulating her right now without surgery. Her airway isn't perfect but she could probably get by. This is exactly what I'd finally come to believe from listening to all the other trach moms on the Trach Forum. But alas she needs to be able to sleep so that brings me to CPAP.

I've worked out a pretty good system and Ainsley adapts well to routines. So she now knows what to expect and therefore does better because of that. She still HATES the CPAP. We discovered that it is FAR more uncomfortable when she breathes through her mouth. So I fought for a full face mask and am waiting to receive it from the DME, we should get it next week. For now I ask her to close her mouth (she's a mouth breather) and hold it shut. We put the nasal mask on and count to 15. Then I remove the mask and read her a book. We do it a second time. That's about as much as she can take. And it's not fun. We are busy, so I don't get to it every day but we're trying.

It's been cute, since about September Ainsley has starting signing sleep when she's tired and wants to go to bed.

Adrian helping desensitize Ainsley's fear of the mask. I admit he was bribed with Poke' Points (My new Pokemon Card Reward System).

One day I was able to get her  to sleep with the mask on (without the air flow on) for her entire nap.

She cried and cried but did do it. Her heart rate was a little elevated but she was able to sleep for several hours. That indicated to me that it's an issue of the airflow not the mask. So for the time being I've switched over to working on tolerating the feeling of the airflow because after all wearing the mask is no good unless the machine is on. And why torture her with sleeping with this thing on until I have to? 

I much prefer to see her look like this when she sleeps.

As always there is a ton going on. We are still waiting to hear word about insurance approval for her eyelid surgery. We're working toward getting Ainsley to use the walker without the sling seat.  We are still working on finding the right wheelchair for her. And we're trying to get her a communication device. After weeks of discussions we just finished a 3 hour IEP meeting on Wednesday (that could be a whole post in itself). The school finally hired a teacher for her class so I'm feeling better about her schooling. Other non-Ainsley stuff, we are busily getting ready for Halloween. Adrian is performing at the Seattle Center tomorrow for Dia De Los Muertos. We are looking at a rambler in Redmond and considering whether to try to get our house ready to sell. And Steve is super busy at work again. With the holidays just around the corner I'm concerned about how busy life is going to get but I'm trying to stay in the moment. Happy Halloween weekend!

It's So Hard

.....sometimes to watch your child ...

...want so much to be able to do things by themselves that they just can't do. Good effort though, good effort! Actually we were quite proud that she tried and was able to get both her legs into her jammies even if they were in one leg. It was cute. But then she didn't want us to do it for her.

To see your child and know she wants to be able to eat a piece of cheese but can only lick it. 

 Or want desperately to eat spaghetti and meatballs like everyone else in the family.  Every night she insists on having some of each and every item on her plate. Tonight she even put the spaghetti to her mouth. Tonight she didn't want her tube feeding. She shook her head no. After she was unable to eat the spaghetti herself she wanted me to put it in her tube. Sorry honey-baby, it doesn't work that way. Still, I was proud that she licked and tasted.  Maybe tomorrow we will have Cesar salad and spaghetti and meatballs in her puree. Still it's not the same. She's starting to know that.

Some day. Some day.

A Happy Birthday

 Monday the 18th, on her 4th birthday, Ainsley waiting for the bus on the porch. We put the crows and spider webs out the night before and she got bit between the eyes by a mosquito. It's October. Are there supposed to be mosquitos?!

That night we had dinner as a family. My sister came over that day and stayed.

Ainsley has been doing better eating bites of puree. She always wants some of what we are eating on her plate and will point and point until she has some of everything. Of course she doesn't eat any of it. But she will taste the sauces and sometimes lick stuff. Lately she's been doing more of that. She put cesar salad in her mouth and got a piece of parmesean cheese. She didn't gag or retch so that is progress. But she did very clearly show us that she wanted it out of her mouth.  

Later we opened presents.

 Her sister made her a bracelet, though Ainsley doesn't appreciate jewelry yet. It looks like she's admiring it. Actually she's trying to figure out how to get it off.

The kids love to give each other gifts.

Adrian and Ainsley have a special relationship. I love seeing them together.

She loves balloons so Evie picked this giant butterfly out for her.

It was cool to see she could open the cards by herself though the envelopes were torn to bits.

This one, from us, lit up. She was in awe.

 It was cute to see how excited she was to open the presents. She would point to the box and sign "open" over and over.

 Again, she was in awe and it was so neat to see. I think if she could talk she'd have said, "Really?! These are for me?!"

 We'd ordered the European version of Calico Critters, Sylvanian Families. They are the same but have other sets and things available that you can't get in the US.

 She kept saying open, open. But we'd run out of time to open them all. So we had to pick.

As I said in another post, her point is getting really good. But she couldn't decide. She'd point to one, then the other, back and forth. Who can decide? They are all so cute.  

We put 4 candles in the cake just like Ainsley's.

 Evie had stayed home sick and we found this cake together. Ainsley was Tinkerbell for Halloween last year. So I thought she might like this. The coolest part was that it came with jewelry on it.

I thought this would be perfect for a girl who doesn't eat cake and has this reaction to frosting.

But unfortunately this was her reaction to the jewelry. Though later in the week she decided she liked it. At least the necklace, maybe not the Tinkerbell bracelet. Overall though I'd say she had a perfect birthday.

It was a busy week. The following day was Steve and I's 14th wedding anniversary. We started dating in January 1992 which means this year we'll have been together 19 years. We had dinner at home with the kids. One disadvantage to have a trached kid.....no babysitters. Though since these days she can go without the trach for a bit I'm feeling like it could be safe (enough) to leave her with someone who doesn't know how to insert a tracheostomy (they can come out by accident). So look out. I might start hitting up family and friends soon. In fact, I left Ainsley with Sheryl for 15 minutes while I ran to get wrapping paper (yes I'm that neurotic). This was the first time I'd left Ainsley with anyone other than a nurse in 4 years.

 

 Later in the week we got all the boxes open. She loves the car. It pushes pretty easily it was cool to see her working on pushing. She can do it and that thing really goes.

I was especially excited about the school bus (and car) so we can expand her imaginative play to include activities that she does routinely like going to school on the school bus. Evie and Adrian still love Calico Critters so they were thrilled. It's a great thing they can all play together. Even I enjoy these adorable woodland creatures. They remind me of the Wind in the Willows characters.

This year Ainsley's birthday really didn't bring back painful memories of the time that she was born. It was a hard time. My oldest had just started Kindergarten. I had a busy 3 year old. And a baby in the NICU for 2 months who finally came home with an artificial airway, a feeding tube, a mini-hospital, nurses in my home, and  surgeries in the works. A lot has happened in 4 years. Our family is richer for having Ainsley in our lives. We've been through a lot together. We are better people.  There will still be struggles ahead but we are thankful for her and appreciate the small things in life and don't take our health for granted. I'm going to repost Ainsley's first year montage here for those who haven't seen it in awhile to remind everyone of how far she has come in these 4 years. 

Mama

I don't know why but I've always liked it when my children called me mama instead of mommy or mom. They don't do it all the time. Just sometimes. Special times.

I've been hoping Ainsley would call me mama because, well honestly, it's easier to say. I've been positioning her lips while she vocalizes, essentially forcing her to say mama for nearly a year. Every time she's been so close but not quite able or willing to say it on her own. And I've been sad, knowing she just couldn't do it. I was starting to give up hope.

Saturday as we were preparing all our junk to finally, after 2 years of Steve being too busy at work, to have a yard sale. Yes, it's October. In Seattle. It's dicey. This weekend was beautiful but I'm sure next weekend will be rainy and all this work for naught. But I digress..... We were there for hours sorting 20 bags of used kids clothes. Pulling out stuff that Ainsley can wear (she's now wearing size 5). It was cute to see her trying to put stuff on by herself. Anyway...

She said MAMA. Just out of the blue. For no apparent reason. And I captured it on video for you to see and hear.



Of course as I explained in this post I can never be sure at least not until she's regularly using it in context. But it sounds right and I'll take it. Just in the nick of time she did it by herself. She turns 4 on Monday. Now I can say she's said her first few words, including mama, when she was three. It's funny, I've been thinking she's 4 for a long time. She's 3. Why do we always jump ahead of ourselves?! I'm excited to see what age 4 will bring. I'm one proud mama!

Running

Walking is something that we take for granted.

All it takes is a minor injury and you realize how fortunate you are when your body is working well 

In April Ainsley's hips and legs were cut apart and reshaped and she now has plates in her legs. I often wonder what it feels like to her to try to walk and I wish it came more easily to her and we didn't have to spend so much time and energy trying to teach her something that my other kids learned with very little effort, something I didn't appreciate at the time.

Our house is a modest old Craftsman bungalow built in 1919. It doesn't have space for a walker. So when I really want to see how Ainsley can do in her walker I want to get her into an open space. Sometimes I take her to the kids' school at pickup time and we linger on the playground.

Friday was a beautiful fall day. Perfect. The 4th graders have started music lessons and they are so excited about their instruments. 

Evie picked the violin.  She has wanted to play for years (so she says). I just hope she'll practice.

A group of 4th grade girls were on the playground playing music. They were excited. The sun was exquisite. The girl on the saxophone was so enthusiastic I was inspired to take their photos though I really brought the camera to take pictures of Ainsley.

It was the perfect day to be out.

Ainsley was happy. She always is. But she was especially happy.

We tried out Ainsley's new basket for her walker. One day I'll have to blog a how-to make your own trach baby doll.

Even though I know her school therapist would disapprove because she wasn't using good form we taught her to run down the hill. For the first time in her life she felt what it is like to feel the air blowing through her hair, her feet barely able to keep up. She LOVED it.

Evie, can be so wonderful at times. She helped Ainsley walk up the hill and run back down over and over in a true testament to sisterly love and dedication.

It was truly beautiful. Ainsley was happy to do it over and over again.

She also thinks it's fun to hang off the arms of her walker. She's building some serious upper body strength.

You may notice she's got some funny boots on. Under those impostor Uggs are AFO's. I had to dig out a pair of Evie's old shoes that they would fit into. We haven't used them since we got them in the spring, for participating in a fitting workshop, before Ainsley's surgery and she's now dangerously close to outgrowing them. I thought okay, maybe they do make a difference because she was doing so well. Then right before we left I thought I should take them off and see how she did. She was very happy because you see, they aren't all that comfortable. I was glad I'd thought of it because in actuality she did almost as well without them. The only difference is that her right leg turns out a bit without the AFO, because the AFO holds the ankle into a fixed position. Her right hip is in a slightly different position which makes it appear that leg is slightly longer so I think she just doesn't know how to compensate. I want Ainsley to strengthen her muscles so that she can walk without AFO's, though she likely will need shoe inserts to help keep her feet from pronating. 

We've been loading the walker on the bus everyday so she can use it to walk into school, for free-play time and for recess. She's used it for over an hour at a time at home or out and about like at Salmon Days. And for her private physical therapy on Tuesdays, which has been great for her (a topic for another post). All this increased time in the walker has really helped her build strength. An average 4 year old spends much of their day on their feet, so we are trying to have Ainsley do more and more of that. So far it seems to be working and her little legs and butt are finally building some muscle. It's pretty exciting.

This isn't representative of good walking form but I thought you'd enjoy seeing Ainsley "running".

Salmon Days

What is "Salmon Days"?

In Issaquah, Washington the idyllic town my husband grew up in, it's the celebration of the annual return of the salmon to Issaquah Creek to spawn.

It's an amazing sight to see.

Some of the salmon are amazing colors, though mostly they blend into the their surroundings. 

...making them difficult to photograph.

The eggs.

It's a great chance for the kids to learn while having a lot of fun.

1.6 million eggs. WOW!

 The life cycle of the salmon.

My beautiful kids (minus one).

What you probably don't know is that every fall my life has revolved around Salmon Days since I met my husband nearly 20 years ago. Even my wedding was scheduled around it because my husband mixes sound  as a business hobby with his father who is a serious music lover (They both play guitar). Steve is reportedly one of the best but it's a tough industry for a family man. Together they have mixed sound for this festival for 27 years. That makes my husband sound ancient but he is 42. Because of his father he started doing a lot of interesting things at a very young age.

Here he is at the sound board with his kids. Unfortunately he is tied to the stage the entire weekend and doesn't get to enjoy the festival, this huge family tradition, with us. It's a bit ironic.

Aside from the salmon, there are thousands of people (groan), dozens of food booths, great live music and incredible craft booths that line the streets of downtown Issaquah. It's a lot of fun. I guess if you want to experience it you'll have to go yourself. Though I took hundreds of photos of interesting stuff I can only share a handful here. 

I feel about Salmon Days like I do about a lot of things. It's a bit of a chore to go but afterward I'm glad we did it. 

It was great to see Ainsley out and about walking in her walker. She loved it. Our nurse worked an extra shift so we went without Ainsley on Saturday. It was freeing to be able to navigate the crowds but ultimately it left me feeling a bit sad and feeling guilty about leaving Ainsley at home (something that I normally never do). So Sunday we went again and this time she came with us and had a great time.

On occasion I've talked about how Ainsley gets stares everywhere we go. Sometimes it's uncomfortable and unwelcome but she gets a lot of admiration too. People can see she's got spirit! She got a high-five from a middle aged man in a wheelchair. I think she made his day. It was a moment of connection I won't forget. Ainsley touches people. She is happy and you can feel it.

We love to look at all the interesting craft booths. Ainsley's "point" has really improved over the past month or two as you can see here.

Another favorite. Fire roasted corn.

 Evie and I love it! I think corn is Evie's favorite food. Adrian took this picture. It was pretty funny to see people watch a seven year old boy hold my new giant SLR camera. I was a bit nervous but for once I'm in a picture so it was worth it.

Ainsley enjoyed all the unusual sights. She's a people watcher that's for sure. Here she's watching her brother break a board in half.

Pizza and arcade games with the cousins and at the Flying Pie. Beers for the moms.

 Note the upside down table on the ceiling.

 Another favorite of the kids. Pony rides.

This year Evie got to ride a real horse. It was worth the wait in line.

 The kids had ice cream and reported it was the best they ever had!

They played with their cousins and climbed trees. 

We had fresh popped kettle corn.

Elephant ears and hot chocolate, what a perfect way to end the weekend.

 Thanks Caron for prodding me into doing it. The kids had a blast!