December 2014 Wrap Up

Sorry for the long absence from blogging. I want to catch you up with our December and wish you a wonderful 2015!

In November (and a bit in December) I spent many many hours tweaking Ainsley's AAC vocabulary to get her a vocabulary that will last as she moves into the next level of AAC use. Also, in early December I spent a lot of time helping the kids with various things for school. Adrian had a big science project that he needed help with (an animal cell model). The big kids had a school dance recital. This was part of their physical education curriculum for which they would be graded. They each spent a lot of time choreographing a dance to a song of their choice that they performed for the school and parents, which required meeting up with their dance partners. Adrian needed matching costumes for his group but forgot to tell me until 2 days before they were due. Evie'd neglected to tell me her pointe' shoes were so small that they were falling off. So the night before we crossed the bridge just to come home empty handed since they didn't have the ideal size (Pointe shoes are complex: length, width, vamp, shank, box shape). The next morning after having visions of her shoe coming off mid solo performance I headed back over the bridge to buy the next best size and sew on all the ribbons and elastics, all without Evie present (which she should have been but was at school), before rushing off to the event to be the official photographer.   I attended school with Ainsley a few days when her nurse couldn't be there. These are the reasons I'm glad I'm a SAHM. My mom was always too busy working for that kind of stuff when I was a kid. I may not be a perfect mom but I hope my kids will remember someday how much I gave of myself to help them, when I could.

 Adrian's dance number. (In front and on the right.) 

Evie's on the left.  

I was so very proud of both my dancers. But all the years of driving Evie to dance class since she was 3... during many years of when it was quite difficult to do so (when Ainsley was young)....well it finally felt worth it, and I was so very proud to see her perform her solo pointe' piece. 

We used Ainsley's AAC to order A SCHOOL LUNCH for the first time the day before break.

It was AWESOME!!!

We transitioned her last tube feeding away during winter break 2013 so it is now official, she's been an 

ORAL EATER FOR ONE FULL YEAR!!!

*****

We made it to the tree farm a bit earlier this year (which was nice). 

Selfies among the trees.

Our new tradition, which I LOVE! - An appetizer dinner while trimming the tree. 

With cocktails.

We're going to do this one every year!

Poor Penny. Adrian made her help put an ornament on the tree. She's so tolerant!

Our elf mostly moved around from place to place this year, not making too much mischief.

That is, until this. Adrian said to Steve "Oh, boy mom's going to be mad! Pip Squeak made a big mess!"

I pretended to be really pissed about it. But the coconut snowball cookies were worth it. And I hear everyone's elf gets into mischief sometimes.

The kids got mini trees for their rooms this year and before reporting back to the North Pole Pip Squeak put chocolate presents and a small present box to put under them.

******

Steve had 2 weeks off. We made it to the mall for Santa photos(We haven't missed a year yet.) and the kids picked out a tub of Legos (as one of their presents), at the Lego store.

I forgot to say that Santa loved Ainsley so much he didn't charge us for the photo. 

How sweet is that?!

Ainsley's selfie (she keeps stealing my phone). I thought it was sweet.

Probably Adrian's favorite gift...An awesome art set from Grandpa Todd (who's an artist). You can see part of one of his painting peeking up behind the couch on the wall behind.

Putting out cookies for Santa.

I tried a crock-pot breakfast this year. It was great waking up to breakfast already made.

 Vegetarian sausage on one part for Adrian.

I love that Ainsley still loves Hot Wheels.

Perhaps Ainsley's favorite gift, a stable set.

Ahhhh!

Evie's (p)leather jacket. I made her wait to open it so she was extra excited.
She also got a loop pedal (a music thing).

Her favorite gift might have been the phone stand and shutter release for taking selfies.

Ainsley's been super into video games lately but her skills make that tough. We were able to find some old games that she can do. This one made me tear up a little. She was so happy. At recess she can't play tether ball and I could tell she wanted to, but now she can at home. It made me so happy to see her so happy!

Again...it's been a YEAR since Ainsley's been eating fully orally. It was so great to see her be able to eat all the same foods as everyone else at dinner. We're coming up on 6 months trach free. As you can see her stoma is pretty sunken. Things aren't perfect but she is still doing well. 

David played chef. Making a fabulous prime rib dinner!

They do such a beautiful job! I was so thankful not to be hosting this year. 

We had music again. It was so fun!

Evie performed a few pieces, including a couple of songs she wrote herself. We were so proud of her!

You know you're in a British home when the host breaks out the pickled eggs at the end of the night.   

Hope you all have a wonderful New Year's and that 2015 brings you joy, health and happiness. 

XOXOXO

Susan

AAC Device Vocabulary Selection and Customization.

We have been in the process selecting a new AAC vocabulary for Ainsley's NovaChat7 AAC device.  I have spent countless hours on the computer researching, testing, and mostly editing over the last month.  What started out as me editing Ainsley's existing vocab and adding new pages morphed into a major editing. After spending a 3rd weekend in a row doing little else I want to explain why with a video. First you need to understand the underlying shift in thinking that is explained in this excerpt from the blog PrAACtical AAC. I've underlined the points I want to emphasize.

Among the many changes that the AAC field has experienced in the last decade is the notion that core vocabulary is (or should be) an integral part of any AAC system. We grew to understand the limitations of AAC supports that consist primarily of nouns and descriptors, realizing that those kinds of communication displays restricted our clients to requesting and labeling. Our field realized the inadequacy of providing only prestored messages (e.g., limited the client’s ability to communicate anything novel, not flexible enough to meet most communication needs, etc.). In essence, we realized that without core vocabulary, we were imposing a ceiling on language development.

Read the full article here.

If the only way to provide the ability to truly communicate in a way similar to speaking individuals (assuming the choice of an AAC over other options like ASL) is through an AAC using a word based sentence building system (rather than a phrase based one) then that is the direction we need to go for Ainsley. We want that for her. The big question is when to make the switch. I got a bit of pushback from some of the school staff but I'm holding my ground and we are making the change now. I understand their reservations. Initially I didn't think she was ready to take on a more complex system because she has primarily used her device for requesting and labeling. As I thought more about it it dawned on me that there is no way for us to know if she was ready to build sentences because her old vocabulary didn't have the capability and limited the things she could express with it, simply by its design. She would never be able to demonstrate capability with a device that isn't capable.

Although there is logic to the idea that "if it aint broke don't fix it" how do you know it "aint broke" when your child can't tell you or show you? There is no reason to wait until she is frustrated by the limited AAC. Certainly there is no question that there are things Ainsley would like to express that she couldn't with her previous vocabulary and that will only increase over time because the old vocab had a limited number of words and phrases. Although we could expand it again, as we have in the past, that would just delay the inevitable and still leave her with a limited AAC without working on sentence building skills. I think the real the question is can she learn to use a more sophisticated system. I say YES! I know it will take time and work but she is capable. The reason to do it now is that lately she has shown a real increase in her desire to communicate. This is a mindset shift.  It is not just that she is much more interested in her AAC but also that she is asking questions using body language and signs as well as trying to comment about what she sees going on around her, using the limited means she has. In other words she WANTS TO COMMUNICATE. She is eager and ready to learn and that is why I'm working so hard to try to give her a means to do so.  The video below explains and shows the reasons we selected the new vocabulary and why we are taking on the huge task of customizing it.

It's Snot Been Fun Around Here!

Literally!

Ainsley returned to school last Wednesday after missing 11 school days in October and November. I tried to finish this post Friday but had a busy weekend programming her AAC device for a new vocab we implemented today. Though it's feeling less relevant I still want to finish the post anyway because although it borders on TMI for some, I do have some readers with kids that are trached who may like to hear about our experiences as they are considering whether or not to decannulate (the medical term for taking out the trach) their child. I have to emphasize Ainsley has NEVER missed this much school ever! And yet she wasn't all that sick! How is that for perplexing? At least when you compare it to how she typically handled a virus while trached. Going into decannulation we had been warned there was a chance that she would struggle when ill and may even have to be retrached. This post is really about how things went during her first real post-decann illness, but it is also about the pros and cons of the trach vs. decannulation with CPAP, now that we've made it through her first real illness.

Before I get into it I want to explain why I snapped a photo instead of wiping her nose since one person already felt compelled to comment about that. First of all I was in the driver seat (I pick up the middle-school carpool twice a week) and had to get out of the car to wipe. She just sneezed as I pulled into the parking lot. Thankfully. Since it's pretty difficult to wipe a kids' nose from the front seat when they are in the back and you are driving 40 mph on a long stretch of country road. I had my phone in my pocket so it was the natural thing to do since taking pictures is what I do.  At this point she'd been home with me for a week and a half and I thought she was doing better until this came out from hiding from within her nose. I had planned to send her to school the following day but sent my handy photo to her nurse and we agreed to keep her home.

Speaking of nursing: Ainsley got a continuation of nursing services for a 90 day period post-decannulation as we adjusted. Although her airway isn't perfect we feel more confident in her safety as we'd observed her working through difficulty times breathing over that period, and so we did not apply for a continuance. That's okay for a few reasons. Private duty nursing is a double edged sword. Trust me. Some foolish people thing it sounds awesome like free babysitting but it isn't. No body wants to need it. Those of us who have it would give anything for a child healthy enough to get by on their own. We have had some bad nurses over the years that I am happy to never see ever again. However Ainsley's primary nurse has been with us 2 days a week since Ainsley was two and she is great. However having someone in your home regularly is an intrusion through no fault of theirs. In September her nurse made the switch to work 5 days a week to help out as Ainsley adjusted at school to a trach-free life. She assists her with many things throughout the school day: mobility around the school and classroom, assist with the bathroom, gives a water bolus, assists her eating snack and lunch(she is more at risk of aspiration and choking), keeps her safe while accessing the climbing equipment and using her walker on the busy playground during recess, assist her with communication, her AAC device and educational materials. It's a full job even without the trach. She no longer comes into our home. That means when Ainsley doesn't attend school she misses work. We joked that she should have taken a trip to Hawaii. The biggest difference is that she isn't there in the morning to help get Ainsley ready but I've managed. What is most difficult is that we don't have as much time to talk about Ainsley's school affairs but we started a communication journal to help with that.

Now that it has been awhile we are feeling more confident about her airway and have been able to leave her with Evie and/or Adrian for periods of time. Having the flexibility to do that is really great so if I need to run to the store to pick something up I could without feeling like I am risking Ainsley's safety, or leaving my older children with too great a responsibility. At this point I think she could be left with family members.

Although there are advantages to having no trach while sick there are also disadvantages. During this illness she was far more snotty because she has air flowing through her nose for the first time(while sick). Trached the runny nose was very minimal (unless she was able to wear her cap, which she seldom was). However the snot, know by trach parents everywhere as trach-snot, was simply directed to a different point of output....the trach. I was plenty happy that the snot shown in the picture wasn't shot from the trach at the back of my head while I drove. Believe me it happened. Many times. I am really happy not to be cleaning up trach snot from my car windows, TV screens, floor, furniture, glasses etc. in the 3 months Ainsley's been trach free. I might be the best part about a decannulation. I also LOVE not having to do emergency trach-snot catches with my bare hands (preventing it from sticking to any of the previously mentioned places. So those things are good. 

On the flip side of no trach snot, Ainsley still has a pin prick stoma (hole) in her neck that oozes secretions. I have to clean it multiple times a day even when she is not sick. With a trach you have to clean the trach multiple times a day too. When sick the problem is worse and either way (trached or not) if you don't deal with it promptly and constantly her shirt will accumulate quite a lot of snot which is not a good look. I've found that for some reason Ainsley's hair seems to stick to her neck snot more than the trach snot.....like it's been glued, literally. So having two areas to keep clean when sick means double the wiping. If she makes it through this cold and flu season without having to be retrached then they will surgically close the hole which would be really nice.

Coughing has been interesting. When Ainsley was trached and developed a cough, it would often be uncontrollable and lead to bleeding. I believe the cannula (the inner tube) would rub against the trachea and cause an abrasion that would bleed. That would then lead to fever. I do not generally medicate a fever (unless it's severe) for two reasons. First because I know they are more likely to rest with a fever and second the fever itself kills off bacteria and shortens the length of the illness. This time she did eventually develop a cough but there was no bleeding and no fever (100 or higher) until the last few days when she started to develop an ear infection. So I do think these are the reasons that although she wasn't nearly as sick as she's been in the past with the trach, it did drag on longer. Throughout all of this she had a pretty decent energy level most of the time, so she basically watched videos day and night. 

Another thing about her cough is that both trached and decanned her airway seems to shut quite forcefully when she coughs. When she was trached she could still breathe, but decanned she struggles to breathe. She works through it and recovers but I worry about what could happen if the illness was worse. If she panics and cries, then that is the worst of all because the same thing happens to her airway when she cries so it's extra forceful and uncomfortable. In addition crying causes a lot of mucous which she has difficulty managing. It's the perfect storm and it is hard to watch when that happens. Poor kid. 

During the 2 week period there was one scary incident. I put her in bed for a nap. She didn't want to use her CPAP (but needed it) and had a coughing and crying fit that dropped her oxygen saturations into the 80's (unheard of for her) and she actually turned blue for the first time in years. I was a bit scared but treated her with a bit of oxygen which we still have on hand at the house and got her to calm and wear the CPAP. She was better after about 20 minutes and the oxygen was turned off. Honestly we didn't know how she would manage illness because of how her airway reacts under pressure. Her Pulmonologist told me he was expecting that she will end up in the hospital at least once during cold and flu season. Even though she missed a lot of school it is a good sign that she made it through this illness without being hospitalized, yet I say that with a word of caution since we haven't hit the worst illnesses of cold and flu season.

CPAP was extra awful during her illness. The airflow on her CPAP is fairly high pressure, and it flows in her nose and mouth. When she is snotty that means the snot is pushed into her the back of her throat. It then oozes out her mouth and into the face mask. That breaks the seal and interferes with the effectiveness of the CPAP. So while we are sleeping I will hear through the doorway that she isn't breathing well so I get up and clean out the mask and her mouth and put it back on so she can sleep. This can be necessary multiple times a night. Then there are times it's bad enough to wake her and she sits up in bed. Or she simply pulls the mask off in the middle of the night. There have been many times she removed the mask and we find it off in the morning. Other times she actually desats and the alarm of her pulse-oximeter wakes us up. Steve and I take turns nudging each other over who's turn it is to get up.

It's all very much like having a newborn baby if you are a parent you surely remember those days and know what I'm talking about. It's been like that for 8 years. In reality it's kind of a toss up and either way, trach or not, we have a lot of disruption to our sleep. It's much worse either way when she is sick. When she is well we get up in the middle of the night anywhere from 1-5 times. When recovering it is usually 5-10 and if she is really sick 8-15.  It's not uncommon that we climb back into bed just to have the alarm go off again. Even though her sats are 94+ on CPAP she breathes noisily. With the trach she slept peacefully with sats of 97-100. It was easier for us to suction her while she slept than it is for us to clean out and replace her mask. Thankfully usually in the deepest part of her sleep cycle we get a few hours without a waking. Still our sleep is very much effected. I can count on my two hands the number of times in the past 8 years that I've slept away from Ainsley and had a normal night's sleep. Comparing the two, it was quite a bit easier when she was trached than with the CPAP but we are still well prepared for having a child on CPAP by having had a child with a trach. We've lived with poor sleep for a long time now. It's just a matter of degrees.

Aside from being sick her breathing while sleeping has gotten somewhat better in the 3 months post decannulation. The above photo was taken on the morning of Nov. 11th (Veteran's Day) when she was still a bit sick. When I went to wake her up her head was completely covered by her blankets and yet she was satting at 97%. Look what the mask did to her face. At night when she falls asleep on my shoulder she can go several hours before she needs the mask. It's in the deepest periods of REM sleep that she has to have CPAP. This is good because it means I am less worried about times that she falls asleep on in the car while driving like she did this day. Still, we do need a plan for a power outage. Being technology dependent for a basic function like sleep is a bit frightening. The trach felt safer to me than this does.

Eating is an area that can be affected by a trach. I haven't seen a huge change in Ainsley's ability to eat since her decannulation however she'd improved a lot by July so I think the difference just isn't as apparent. She's eaten well in the past 3 months, as she had prior to that (for a tube fed kid who is a new eater). Although Ainsley made a lot of progress eating off and on over the years one thing was consistent. She would ALWAYS regress when she got sick. Although Ainsley wasn't terribly sick this time (not like the flu or RSV) she did still eat. This is the first time during an illness that she was able to get by without any tube feedings when sick. Of course she ate a bit less like any kid does when they are sick. But SHE DID IT! And that I would have to say is because the trach is gone.  Presumably she was better able to eat and breathe even with nasal congestion. In fact I wanted to show you this. Eating Chinese food, with CHOPSTICKS, WHILE SICK! I think this makes it official....

My girl is a real eater!

Speech is an area that can be affected by a trach, but since Ainsley was capped while trached decannulation hasn't made a huge difference, since the cap allows the air to be pushed through the vocal cords for vocalization. The source of Ainsley's inability to speak is due to the complexity of the motor planning required for speech and her difficulty with that motor skills and motor planning due to Cerebellar Hypoplasia, and was likely additionally impacted by the complete lack of a voice for the first two years of her life. This was due having a trach and an airway too swollen to wear a valve or cap(until she was older). We are expecting her to need a touchscreen AAC device for communication for the rest of her life trach or not.

You might wonder how Ainsley feels. I can say for sure that she would much rather sleep with the trach in than wear the CPAP. She slept better too. A month or so ago we had a blood draw to check her electrolytes to check for CO2 retention. It was very normal so we know the CPAP is working even when her breathing sounds bad and she'd been pulling off the mask. I do think she likes having no trach during the day. Honestly we've really just traded one set of problems for different problems which we knew going into this.

My trach mom friends are fantastic. I wouldn't have made it through this journey without their support. Many of their children moved on to decannulate over the years. I have been told that it is not uncommon to have a rough time the first cold and flu season. Only time will tell for sure.