Medical Monday - May 15

 I missed getting last week's post written because we were both wiped out with allergy / sinus / fun Central Texas spring air mess. This week, I wanted to give an overall medical update since the last post that had to do with medical stuff was from about 5 years ago...and some stuff has changed since then!

So, from the top down:

Lexi still struggles with some airway issues; most recently she's completed a few sleep studies that show she is having some pretty severe sleep apnea issues when she hits REM sleep. The first was in late 2021 and it showed that she was having about 39 episodes per hour. For reference, mild apnea is 5 episodes and moderate is 10. Everyone was in agreement with trying other options before going with a CPAP mask because at the time, she was still pretty sensitive to having things on her face after her stomach surgery earlier that summer. We started with a palette expander in the roof of her mouth which went in October of 2022 and came out in March of this year. Repeat sleep study results show that it did help, but not much. She still had about 27 apnea events per hour, her oxygen dipped as low as 83%, and she was retaining quite a bit of CO2. So we are going to move forward with the CPAP machine and will hopefully get that in time for her to adjust to using it before the surgeries. There is a minimal level of concern with how she will do coming out of a long period of anesthesia and hopefully this will help head that off. (That was as of her appointment today so hot off the press!)

Sleep Study pro!

Also in 2021, she was referred back to Audiology for some concerns after her hearing test at school. We went through this before in Pre-K because her ear canals curve straight upward, the school machines were not able to get a good reading. This time, the audiologists did notice a slight low-tone hearing loss in both ears. Had an MRI done to make sure everything was working the way that it should in her inner ears; those results lead them to believe that the bones in her inner ears are slowly stiffening up, like her other joints would if she weren't so active. Unfortunately, you can't really stretch out your inner ear bones so this is something that will likely continue to progress as she ages. So, December of 2022 we added hearing aids to our daily routine. While there is no concern about speech delays or her backtracking in development, the hearing loss does cross over into vocal ranges so it is more preventative to make sure she is hearing everything she needs to and to hold off as long as possible before we potentially have to take any other steps. She has adjusted well to the hearing aids and we will continue to monitor as she grows.

Fitting first set of hearing aids

Moving down: 2021 was a fun year...I didn't post the updates on this blog, but we spent 21 days in the hospital after her stomach rapidly expanded and cut off the blood supply causing all of the tissue to die. Why? We have no idea. Her surgeon said he has only seen this happen 5 times in his entire career and the only similarity was that all of the patients previously had a G-Tube. Some had been out for years, some for months so he's not even sure what the link is there. Anyway, since her gastrectomy, she has slowly worked her way from being on a 24-hour continuous feed to dropping that down to 12 hours so she is only connected at night! We've had a few late nights in the ER to get the tube put back in when it comes out, but otherwise she has adjusted as well as possible to her main source of nutrition being through the tube again. She can still eat by mouth but now that's reserved for all of the fun stuff that she wants to eat because it tastes good. She talked to her GI doctor at last Friday's appointment about repeating some allergy testing to see if she's outgrown her dairy and egg allergies, so her options might expand some after we get those results back! 

The only other thing to report on is that she is going strong with using her walker and braces to get her legs as strong as possible before surgery this summer! At school she rotates days between walking and using her chair and we have started working on her being able to carry her own backpack when walking to help move her toward more independence and not having an aide to assist with some of those tasks. It's crazy to think that we are already looking at skills for middle school, but I'm glad we are giving her plenty of time to adjust.

This year, we have gotten two big purchases approved: the first was a new power chair and the second a manual chair that she can maneuver by herself. Power chair upgrades are available every 5 years and we made the last one last for 6 so it was time. But that also means that we had to consider everything that she may need in a chair until she turns 13! The primary focus that we looked at was moving toward independence and her being able to perform age-appropriate tasks around the house so the big push was for an elevating seat so that she can reach counters, drawers, sinks, etc. The manual chair will allow her to roll herself around during quick trips to the store, between the car and buildings, etc. to save mama's back from carrying her and give Lexi a little independence instead of having to ride in the basket. We don't usually haul her power chair when we are just going around town so this will be a huge help!

Elevating seat makes her almost as tall as mom!

If you made it this far, thanks for hanging in there! We've had to make appointments with all of her specialists so they can sign off on her having surgery so I thought it would be a good time to get everyone caught up on what's been happening and what we are looking at moving into the summer.

Medical Monday - May 1

 So it's been awhile since this blog has seen a post, but I wanted to document the upcoming surgery and planning for that in the same place as all of Lexi's other adventures so here we go!

As we prep for our trip, I will do a series of posts leading up to the surgeries each Monday that we'll call Medical Monday. For round 1, an overview of what the surgery will entail:

This is obviously a very simplified version of the surgery, but gives an idea. She will have an incision from mid-thigh to just below her knee on each side. 

On the planning side of things, we are starting to look at packing, what we will need for 8 weeks, what we can live without and how to make it fit into our car. Thanks to some very generous donations, our housing has been completely covered and we are SO thankful for that. Once we have a better idea of what all the medical expenses will be, I'm sure I'll be reaching out but until then you can still send support through Venmo and earmark it for surgery expenses. 

A few minor updates... June 26

I feel like nothing has really happened since my last post in January - which is definitely a good thing medically speaking!

You were out of casts and back at school walking three days a week and using your chair two days a week by the end of January. After you learned about patterns, you were continually finding them in our everyday routines and your favorite to say is "chair walker chair walker chair". We are working on next year's pattern becoming "crutches chair crutches chair crutches!"

In February you started playing T-Ball, which you absolutely LOVED! We will definitely be doing that again next year! Unfortunately, your braces did not hold up so well and I had to do several repair jobs on them throughout the season... can't wait to hear what your doctors say about that at the next appointment :)

In March we had some fun at Spring Break with Grandpa and Nanny. Got to go swimming, fishing, took a road trip to see Pops and Gigi. 

You continue to amaze me with how fearless you are. You have started walking up and down the stair by yourself instead of scooting on your bottom one step at a time. You have no issue with wiggling your way up onto or down out of a chair, the couch, beds, etc. It doesn't surprise me anymore to walk in on situations like this:

Your class went on their field trip to the park across the street from the school in April and you thought it was the coolest thing ever to get to play with your friends. They blew bubbles and wrote with sidewalk chalk then had a snack on the play ground. So. much. fun.

School wrapped up more quickly than any of us were ready for and now here we are! It's summer time! You got to spend some time with Nanny last week while Mama was at work and came home with all sorts of new accessories -

Yesterday, we took a trip to Sea World with Daddy's side of the family and you were absolutely fascinated with watching the trainers swim with the animals. You kept asking when it was our turn to swim with them and didn't quite understand what I meant when I said they had been practicing for a long time to learn how to swim with the whales and dolphins. Apparently, you know how to swim and that should be enough! You favorites were definitely the dolphins and sharks.

Today, we went to the dentist and had an appointment with GI doctor. Dentist took some quick x-rays and said everything looks good. He also said that your roots aren't quite as long as they should be, which isn't unusual for kiddos with medical issues since the "stuff" needed for growing teeth before they pop out usually go towards other, more pressing needs in the body. He said not to be surprised if you lose you teeth earlier than your friends and have to wait longer for your adult teeth to come in since they weren't growing under the gum line yet.

GI was thrilled to hear that you have only had two tube feeds since school let out and are eating all. day. long. Unfortunately, your weight is down from our last visit so he wants us to try to find something to boost your calories. If we can't get it on the upward trend again in six weeks when we go for a weight check, we will need to start doing at least one tube meal per day to make sure you're getting those extras in. It is a negligible amount, we only need to increase your intake by 10% of the calories to make a difference; but he doesn't want the downward trend to continue. The tricky part is: you like low calorie foods. You would eat broccoli and carrots all day long, but would need to eat about 1000 times as much as you normally do in order to see the calorie difference! We'll play around with some of the cooking methods and see where we can boost some of the fats in your diet and hopefully that will make a difference because the drink they sent home to sample tastes disgusting!!!

That's all for now - we'll check in at the end of the summer after we see ENT, Ortho, and GI again!

Blended Diet - One Year Later

I’ve been meaning to write this post for a while, and even took pictures last time I did all the prep work, but haven’t sat down to put words on paper (...screen...) until now. There has been a lot of sharing and questions on one of the Facebook groups I am part of about storage methods, delivery methods, volume and calorie amount, etc so I thought I would document our blended diet system.  I will start out by saying that I will be forever thankful for the mom who shared this post about their traveling blend system because it COMPLETELY changed the game for us last summer and I have spent the last few months tweaking their system and making it work for us because it.is.A.MA.ZING!

I will also say, that I know this system won’t work for everyone, every tubie is different and has different needs, but this works for us and has simplified the feeding process for us - and in the world of medical kiddos, any system or process needs to be as simple as possible because no one has time for complicated...oh wait, that’s for parent of any kid 😊

Last intro item, we have been blending for a little over a year now and as your needs have changed, so has our system. I started out using silicone ice cube trays to freeze one ounce portions of each ingredient then would blend up one of each cube for the day. That was a storage nightmare. So I started cooking, portioning and freezing each day’s ingredients in a mason jar and would prep about a week at a time. Then I figured if I could prep for a week, I could prep for a month and have it all done. I bought the wide mouth tallest jars I could find and could fit one day at a time in each jar. I would pull it out of the freezer and put it in the fridge to thaw the night before. We would blend it, pour each portion into our daily jars and heat them up as each meal came. Again, storage.

The next solution was to use thermoses to keep the blend warm all day. So I would heat the tea kettle, pour the water into the thermoses to heat it up while I blended each morning and it would keep the blend piping hot all day long. Awesome. Except when we travelled. And then you started needing less by tube because you were eating more by mouth so we were pouring a portion out each day. Enter the post I linked above...

We used that system while we were gone for a week last summer and it was amazing. Didn’t have to worry about keeping things cold or hot, mix up one meal at a time, wash out the bottle and done! When we got back home, I went back and forth - I knew that we couldn’t afford to continue using the pouches from the store because of the expense but I loved having things ready for an individual meal. PLUS - you was getting more calories in a slightly smaller volume so you were eating even more by mouth because you weren't as full from tube feeds.

So I tried looking into methods to replicate the pouch recipes but could never figure out the best way to do that. Then it clicked one day: use the blend recipe you are already using. Duh. We know you doesn’t have any intolerances to any of the ingredients, it already works into our budget for those items, we can make some little tweaks for calories and done! So that’s how we got where we are now: 

This is part of 90 pouches, which will last anywhere from 30-45 days. Some days you gets three tube feedings, some days just two if you have eaten well orally. Each pouch is mixed with 2-3 ounces of either homemade broth, coconut water or prune juice so each feeding is 303 calories plus the liquid used and about 150-160mL, which is almost twice the calories at half the volume of our old method. Win. Win. WIN.

I pull out one tub each Saturday to go to the kitchen freezer and pull out pouches the night before for the next day.

Recipe:

Coconut milk - 1 cup

Honey - 3 oz

Olive Oil - 4 oz

Chicken - 6 oz

Rice - 126 grams (~1.5 cups)

Bananas - 180 grams

Spinach - 144 grams

Blueberries - 90 grams

Sweet Potatoes - 213 grams

Peanut Butter - 6 TBSP

Yield: 15 pouches at ~300 calories each

Process:

I spend one day cooking everything and dividing it into six portions. Each portion is a triple batch from the daily jar system and makes about 15 pouches - so the first win was taking what would have been three days worth of food and it makes five to seven days worth of pouches. I did not use coconut milk or peanut butter in her blends previously so those were huge calorie and nutrient boosters - yay healthy fats! 

The next day I blend each bowl and fill the pouches. One batch of pouches fits in one of these amazingly perfect sized ice cube trays and four trays fit on the bottom shelf of our freezer. I have had some issues with the blender not quite getting everything blended perfectly, but since I am 100% convinced it is user error and I’m overfilling the jar I am going to do a double batch instead of triple next time I make pouches. 

This method works super amazingly well for several reasons:

1. Already mentioned the higher calories in smaller volume aspect of things. We saw a huge increase in your oral intake when we started this method.
2. If you're not hungry, we don’t mix a pouch. It’s that simple. Way less waste!
3. We can vary the liquid in the blend based on what you needs. Beginning of the fall / winter sick season you are getting lots of bone broth. Random episodes of constipation, we run prune juice one mix a day for a few days to help you out. Otherwise, coconut water for the extra vitamins and minerals. 
4. I spend 2 days prepping and you're ready for up to a month and a half - and that two days is being shortened as I get more used to the amazing-ness that is the InstantPot... 12 cups of rice in 15 minutes? Yes please!
5. It is super simple for ANYONE to feed you now! For school I send a jar with the pre-measured liquid, one pouch, shaker bottle and scraper - teacher or nurse dumps it all, shakes it all up and it’s done. No measuring, no issues.

A couple downfalls:

1. I still have to worry about keeping them frozen when we travel. This is much easier to do now than with the jars, and if we are gone for more than two days I go back to the store pouch system but I always see a drop in your oral intake because of the higher volume.
2. The pouches themselves are an expense. It balanced out for us since I was able to make the food stretch a little further, but that is something to consider. We tried refillable ones but they weren’t as quick and simple as these disposable ones.
3. It’s messy. The video on the site where I ordered the pouch filler makes it look so simple and easy, and it is, but when you’re making 90 of them your kitchen won’t be as spotless as the commercial...of course, any batch cooking or blending system will do the same.

So that’s where we’re at now; as your needs change this might too, but this is a flexible and adaptable enough system that I see us sticking with this base for a while. You have gained almost three pounds since last June which is the most gain you’ve had in...ever... We will continue to encourage oral intake and drop feedings as needed but for now we are in a good holding pattern and I’m loving it!

So it’s been awhile.... July - December 2017

So I realized it had been a while since I posted, but didn’t realize exactly how long until I got on here to check... whoops!

The good thing is, nothing has really happened between July and now... just two surgeries, countless casting sessions, a new school, getting moved from special education to regular education, becoming a professional wheelchair driver, and having your 4th birthday party. Nothing important at all.

Foot surgeries - started the casting in June; every two weeks we took a trip to Houston to get the casting adjusted. Your first surgery was July 21 and it took them about two and half hours longer than planned. Not a fun waiting game. The surgery went amazingly well though and your surgeon was excited about the results. They started the casting on the second leg when they wrapped the first one after surgery so we then spent eight weeks in double casts.

The second surgery was delayed by vacation time (which I was not thrilled about) but turned out to be perfect timing or we would have been stuck in the hospital when Hurricane Harvey came through Houston! So we were delayed a little longer as they tried to get their schedule back on track and this mama was more than ready to finally get it scheduled! The second round went more quickly than the first becaus ehe knew a little more what to expect - as has been typical, your left side was tighter than your right so it took three pins to hold everything in place instead of just one.

Fast forward to November - you were suppsoed to get your post-surgical cast off the week before Thanksgiving but a double ear infection and chest cold kept us out of the ER. We spent Thanksgiving week inside doing breathing treatments, antibiotics and extra meds to get you better... and in the meantime Mama ended up with a sinus infection. Went back to Houston the week after Thanksgiving and got the cast off; we went back Today to get your final cast OFF and to get your second AFO and will go back January 3 to get your HKFOs so you can start walking again! In the meantime you have already started standing up and climbing stairs again; we also pulled out your walker last week for PT so you can start working on leg stregnth. 

That takes care of big item #1...

Number 2: SCHOOL!!!

You were so ready to start school and then we found out about a week before school that your teacher left the district AND you would not be in the class with all of your friends from last year because they had already turned four and you would be in the three-year-old class so we had no idea what would be going on! You did fantastic adjusting to a new campus, new classroom, new teacher and new friends though! So well that about a week into school your teacher called as we were getting home and asked if we could come back up to the school - they wanted to move you out of the special education / PPCD classroom and into the regular education class! 

They initally had an aide that stayed with you but as the PPCD class grew (students can begin on their third birthday so they were being added as the year went along) they pulled the aide back and you are now in there 300 minutes of your day with 70 minutes in the special ed class for “personal care” (diapers, potty time and tube feeds) as well as being pulled out for speech and motor therapy group and YOU LOVE IT! 

In fact, we had your annual review last week an dboth the special ed and general ed teachers agree they can start introducing all of the concepts for the four year old curriculum. I have no idea what they’ll do with you next year (one more year of pre-k before kinder) but we’ll cross that bridge when we get there. The other big focus is going to be on writing basics: helping you figure out how to grip the pencil, if we’ll need any adaptors, what works beat for you, etc; as well as starting to work on typing and finding areas we might to address with adaptive technology. 

So that’s where we are with school. You come home every day with a different story about a different friend. Most of your play time at home is you “teaching” your friends - numbers, letters, shapes, colors, reading books... but you’re a pretty tough task master!

Moving on: 4th Birthday Party

You don’t turn four until tomorrow but we teamed up with the two December cousins for a mega-party at Chuck E. Cheese so we wouldn’t all have to go there two weekends in a row. It was a ton of fun, played lots of games, you got to catch the tickets (which, according to you, means you are four now) and do all of things you have been talking about since April when we went for L5’s birthday party!!

The big part of your birthday this year is redoing your room! We are making some major changes to bring everything down to your level and make it all accessible to you! I’ll have to do another blog post about that (before next December hopefully!) because there will be a lot changes!

We are getting ready for all of your annual check-ups with specialists:

We see the GI doctor tomorrow; developmental pediatrician in February to do some first rounds of cognitive testing; pulmonary in April and we get to add ENT back to our rotation in January. You failed your hearing screen at school...twice. So we had to follow up with the pediatrician - which is when we found out about the ear infection mentioned earlier. Even after the double round of antibiotics and clearing out all the fluid, failed the screening (round 4...) so we went to an audiologist who said he’d rather us follow up with your ENT team since they know you and your case history. So here we go again.

That’s about all for now - although Christmas is one week away which means another post coming soon. Right now, you are taking your first full bath (not just a wipe down, sponge bath, hair wash over the sink) since July 19 and LOVING EVERY SECOND OF IT.

July 2017 - and a little bit of June and August

As usual, July has flown by!

We wrapped up June at EYL Camp and you had such a great time playing with your "new friend Lane" (as you still call him). We also used that week leading up to AMC Awareness Day as a challenge week - each day we had different staff and volunteers participate in a challeneg related to your limitations with AMC. Once again, our EYL Family stepped up to the plate and were SO supportive of helping us out. Mr. Brent even made the camp shirts blue this year so that the group photo on June 30 could also serve as a #BlueforAMC photo!

Challenge #1: Say "Arthrogryposis Multiplex Congenita" three times as fast as you can

Challenge #2: Write "Arthrogryposis" without using your hands

Challenge #3: Go up and down the stairs without using your legs

Thank you to everyone who sent us a picture of your blue shirts / outfits to support AMC Awareness Day on June 30!

We had a little down time at the beginning of July so we stepped up our timetable and got our living room floor taken care of! It has been a huge plus to have smoother floors now that you're double casted and can't use your scooter - makes it easier for you to stay on the move!

Grandpa had a little pry bar for you to help!

Not a huge fan of the noise from the saw :)

We went to watch fireworks with Aunt C and the boys - you were NOT a fan of how loud they were...but eventually decided they were too pretty to not watch them.

Excuse the blue teeth - sugar cookie frosting :)

And then we had the big event of July: your first foot surgery! They had originally told us it would be a 2(ish) hour surgery; just when we were getting to the two hour mark and I was getting stuff ready to wait for the "all done" call, they called and said they needed another 1.5-2 hours!! Yikes!! But when Dr. B came in afterward he was very excited about the positioning he was able to get with your bones and the end result so I guess the extra time was worth it :)

Pre-Op meds made you super silly and sleepy!

Finally got to get out of bed... that's what you chose for breakfast (Fritos)

We were in the hospital overnight, went home the next day, spent some time with sisters, and have just been hanging out...NOT standing up... at least trying to remind you not to stand on it. If too much weight is put on the foot it can cause the pins to slip and the bones to shift enough that he woukd have to re-do the surgery. You've spent a lot of time reading books, playing babies and watching movies; especially the first few days when you were getting used to having two casts on!

Tuesday (Aug 1) we went back for your first cast change in the left foot and he said everything looked good for both feet. Second surgery will be sometime in the later part of September, which is later than we thought but glad it's not the first week of school!

Wednesday (Aug 2) you had your developmental check-up. He wants to continue following you (still) just so we have someone we can call on when it's time to start kindergarten if we get any push back about you being in regular ed classes instead of special ed (not that we're anticipating any) and to help write letters for any accommodations you might need - we're still not sure what adaptive / assistive technology or modifications you might need. He wants to see you back in February instead of another 12 months because they will be able to do an official cognitive exam - those are tailored for 4 years and above and even though you are still testing a 4+ year old level they can't administer the test yet.

That's about it for this round! 

End of May and June 2017 - June 21

I've been awful about updating - and we've had some big things to share! Summer jumped into the picture WAY to soon and now I loom up and it's almost July...

So here goes:

You finished your first official "year" of school - and we got the good news that you will have the same teacher next year so I'm hoping it doesn't take another 3-4 months for you to settle in and be comfortable enough to start participating again! This next year, at least at home, we will be working a lot on hand work: writing, drawing, etc. Due to your foot surgeries and the casting issues that will require you to be off your feet until mid-October I figure it will be a good time to get busy with that so when you hit kindergarten we will have a better idea of whether or bot you will beed any assistive / adaptive technology or not. We had a great time at your end of school picnic and the teachers gave us these cute planters you helped make with your fingerprints so now you have "my own gardens!" - we are just waiting for your seeds to sprout!! 

We got started with casting on June 5. You tolerate the cast fairly well during the day - it definitely hasn't slowed you down any!! We are having a hard time making you understand that you can NOT stand up in your cast...I have a feeling after the first surgery you will get it! The first few nights were rough; you didn't quite understand why we couldn't take off your cast like we do your pink boots (HKFOs) so you could sleep. You woke up a few times each night for about a week before you finally settled into sleeping with it.

They opted to use a soft casting material because 1) it's easier on the skin so better for long term use; 2) they don't have to cut it off with a cast saw, it just unrolls. Unfortunately, that means it's not as stiff as a typical plaster cast so any amount of pressure can cause it to crack or shift and effect the angle they are trying to stretch your foot to. Downside number two is that it only comes in white, but we took care of that pretty quickly!!

We went back on June 19 for your first re-casting and they were really happy with the stretch and how much more loose your foot is. The first thing you asked when they took it off was "I stand up? I do nastics??" You have absolutely missed getting to do gymnastics with Little Big so you got a couple of your handstands and splits in before they recast you. We also noticed that your entire leg is much straighter - you were having to lean to stand with both feet on the table because of the nice stretch you're getting on your knee as well!

We are still on schedule for surgery on July 19, although we did find out it will be 18 weeks total casting instead of 12 like we originally thought because there is also a 6 week post-op casting period! So six weeks pre-op on the right leg, six weeks post-op right AND pre-op left, then six weeks post-op left THEN we can start PT work on regaining leg strength and walking again.

On June 20 we had a GI appointment and he pretty much told us to keep doing what we're doing because it's obviously working! You are now eating about 1/3 of your daily calories by mouth - and you eat just about everything! There are a few things you don't like, but for the most part you "want dis and dat" and whatever else is on our plates whether you know what it is or not! We'll run labs again in December to check allergies and nutrient level, but other than that we're clear to keep pushing forward!

Your favorite is still "sumsketti" but you also keep cheerios, krispies, crackers, chicken, yogurt, pretzels, toast, apples with peanut butter, apple sauce and cookies on rotation!

You've loved having Nanny around the last two weeks (and we'll have next week as well) and Mama has greatly appreciated the help as I was gone for a week, then exhausted and trying to get caught up and ready for everyone to be scattered again next week!

You got to go to your first non-family member birthday party and LOVED seeing your friends from school! You spent the next three days telling anyone and everyone you saw about how much fun you had and what presents she got and what games you played and... Needless to say, you enjoyed it :)

That's about it for now - will try to keep things updated after next week as we move closer to surgery #1!