Spring Update: April

  I'm doing all my catch up posts this weekend since there are a bunch of things I haven't written about and I need to get them done because Emma's second birthday party is in two weeks from tomorrow!

Swallow Study & NICU Visit ~ April 11th:

  Emma got her swallow study done at Arnold Palmer hospital. I don't have any pictures from that since only one parent can go back at a time, so Michael took her and I waited in the Radiology lobby. It was nice to see that her original NICU Speech Therapist, Michelle, was the one doing this test. Emma passed and showed that she was able to swallow formula and some baby food without aspirating. A copy of the study was sent to the current Speech office, so Christine (the speech therapist who comes to our house twice a week) could start giving Emma baby food and applesauce when she is here. Sometimes if Emma is having a crabby time- usually if she happens to need her nap at that time, unlike today where she is napping at Noon, which is great so she'll be awake and happy for therapy at 3pm- then Christine will just read to her and play with bubbles instead of trying to feed her. 

  After the study, Michelle urged us to visit the NICU since we hadn't been back since Emma was discharged on April 30th, 2018. Michael said it could be up to me whether we visit, which I was surprised by since he hates the security system at the front. The metal detector always beeps when he goes through because of his belt and the security guards basically make him take his shirt off to prove he doesn't have anything else on him. I voted for yes to visiting since it had been almost a year since a lot of the staff had seen Emma in person. Many of them keep in contact via social media, but we found that one of her main doctors, Doctor Lipman, had retired recently and one of her respirator therapists, Errica, had left and gone back to school.

   While we were there we saw a lot of people, many more than in the photos (including Maureen/ "Mo"- one of Emma's nurses at the beginning, Cori- a Nurse Practitioner, and Dana- her NICU physical therapist) and I wish we had gotten a picture with everyone, but it was so nice to see them. One of the girls was telling us about a gala that is happening in June (next weekend, while I'll be working) for a non-profit that is starting up called Matthew's Gift. She recently told me that Matthew's parents have heard all about us and Emma and offered to pay our tickets so we could go, but I'm training someone at work all of June, so I'm not able to get any days off. That was so sweet of them! When I said we wouldn't be able to make it, she also told me that they do a zoo event in November, so hopefully the weather is nice and I'm off so we can go to that! I'd really love us to take Emma to the zoo. She seemed to enjoy the aquarium when we went last summer.

Gore/Wilder Family Gathering ~ Mid April:
   Michael's family came down for about five days because every year his dad and older brother, Christopher, who are both doctors, have to attend conferences, so this year they picked going to Orlando that way they could also visit us! Since Michael's parents and my parents don't get to visit each other often (the previous time his parents were here at the beginning of December, my parents were on a cruise for their anniversary!), my parents made sure to come down for the day so they could spend time together. My parents did get to see Michael's mom and grandma for Emma's first birthday, but my dad hadn't seen Michael's dad since we lived in Birmingham for half of 2014!!

Emma modeling a hat my mom got her on their Myrtle Beach vacation!

     ^^ Us with Christopher and his wife, Meredith.

Although we last saw them in July 2017 when Emma was a month old in the NICU, this was the very first time Christopher and Meredith had met Emma!!

I definitely need to mention that this photo was taken after work and it was Mis-Match day during
Telecommunicator Week. I definitely wouldn't be dressed like this otherwise!  :D

First Dentist Appointment ~ April 25th:
   (I definitely thought I had written about this part already..) Emma has had 18 teeth since December, so it was recommended by the pediatrician twice that we make a dentist appointment for her. Two of the main reasons he wanted us to go is because since Emma can't close her mouth all the way and she barely ever eats anything by mouth, she gets more air and such on her teeth and her front molars do look a little bit yellow although we brush her teeth every night. Also because we don't use fluoride water on her teeth, she needed to get a fluoride cleaning done. The dentist was very nice and did really well with her. Plus we always love when doctors tell us how great of a job we are doing with her! Emma did seem to prefer to sit up in the chair, but in order for the dentist to be able to see her top teeth clearly, she was put on a soft board that was balanced on Michael's and the dentist's legs. She cried during that part when he had his gloved fingers in her mouth (she doesn't like it when the speech therapist does it either) and he rubbed the fluoride paste on her teeth with a Q-tip swab, but then she was fine after and got her Princess Belle toothbrush. She also got her name written on the "No Cavities" board. Hooray!!! We were told that she doesn't need to go back until next April.

 
   Other things that were going on in Emma's world last month ~
Although she got a smaller stemmed Mic-Key button (1.2mm) switched out in March, with her continued ab exercises, her stomach wall is thinner, so there is space between her skin and the button. We bought these cute unicorn snap patches from Etsy, which help absorb any formula leakage. Michael designed and 3D printed the purple piece, although the day after I took this photo, he made a better version which is gray. He calls it the G-Lock. (You can see the gray version in the photo at the bottom where Emma is laying on the couch.) He made it because Emma was found being able to disconnect the G-tube from the Mic-Key button. A few different times I saw her holding it and laughing while the formula continued to drip out around her! So this piece has been great to keep it secure. Once again, Bravo, Michael!! We recently ordered more unicorn patches since we put a new one around her button each night.

  Reading ~ April 7th: Back when I was doing a good job of bringing home lots of kid books from the library to read to Emma during the 15 minutes while her lung treatments are being done. I really need to get back on that! I really liked this "Smile A Lot" book by Nancy Carlson. It was just a really nice book about smiling even when bad things happen because it will make you happier and your life will be better. It really just seemed like an awesome book for Emma! She's so great about smiling a lot. :)

    One day (April 11th) when we were practicing sitting again - while holding objects!! She has a sodium chloride bullet in each hand. Not long ago she would fall back if Michael simply wiped some drool from her mouth. Now she can counter balance while being tickled or lightly pushed from either side. It's a trick they use during therapy, but I can't remember what Lara called it.

   Emma's "knee" flexion has been looking really good lately. (I put knee in quotes since part of her Meier-Gorlin Syndrome genetic condition is that she does not have kneecaps.)

   Below: during Speech therapy, Christine working Emma's cheek muscles. I laughed when I told her that I have been squeezing Emma's cheeks together so she makes a silly face, but she told me that's actually good for her oral practice! 

    Emma testing out her new swing (April 20th). We have only tried it once so far because it has been ridiculously hot outside recently. Everyone has been posting pictures of their 100+ temperatures from their vehicle dashboards (in May). The other day Emma even got sweaty in the two minutes between rolling her out of the house to putting her in the car that had the 60 degree AC on full blast.

 ^^ This is how Emma and I spend most of my days off: being lazy and watching Netflix, haha!!
She's such a happy girl and in this last photo you can see a aqua colored tube by where her hand is. That is the Passy Muir Valve (speaking valve) which we have been putting on her more lately. She has been very vocal without it, but when she wears it, it helps more air pass through her vocal cords, and she needs that practice to feel how she would breathe without the ventilator. Most kids without ventilators have a Passy Muir Valve on the end of their trach. It also allows for the senses of taste and smell, so she always wears it when we practice eating/swallowing.

   We have a lot of therapy homework that we need to do better about keeping up with. I'll have to make a chart to keep us accountable! Making sure she's always wearing her glasses (and that the lenses are clean- since she's always rubbing her fingers on them when she tries to pull them off), wearing her leg splints most of the time, wearing the Passy Muir Valve often, feeding her baby foods while we all eat dinner together, sitting up more/ timing her balancing, arm and leg bending exercises since she only has Physical Therapy once a week for 30 minutes, reading books to her and showing her the pictures, as well as sign language for a handful of words we actually use daily ~ "Good job" is the one I'm working on the most right now. I know it's not the official sign, but I'm having her associate those words with clapping. I'll clap my hands and then clap her hands together, both while saying, "Good job!" in my happy voice.

Medical Refresher Course

    Although the official "Trach Awareness Week" has now come to an end (May 19-25 this year), her whole blog is Emma McKenzie Awareness, so I figured it would be a great time to talk about the Ambu bag and how we would "bag" Emma if she ever needs it ~ but hopefully she won't!! Bagging her is basically like giving CPR but through her trach and without chest compressions.
   Once in a while Michael will start asking me medical questions regarding Emma's care or quiz me on what I would do if something went wrong and he wasn't around to help. To be honest, I would always call him before calling 911. He is extremely knowledgeable when it comes to her care. I'm pretty knowledgeable. There is a lot we have to know how to do, because even though there is a fire station less than 2 miles away in both directions of our house, that could be 5-10 minutes between Emma starting to have an emergency and the EMTs having their hands on her inside our house. It is very important for us to know what to do, and even the nurses and respiratory therapists would ask us what we would need to do in certain situations before Emma even came home from the NICU, such as what we do if her trach comes out. (The answer: Just put it back in! Sometimes that happens at night during trach care when her neck ties are off while Michael is cleaning her neck. If she coughs hard enough or leans back too far when I'm not expecting it or she suddenly pushes my hand away while I'm holding the trach in her neck). We were told that one family's baby was ready to go home from the NICU, however, the stay was extended because the parents were not comfortable enough yet to take care of the child on their own. I'm not sure if that baby also had a trach or what other medical problems the baby had, but a lot can happen or go wrong in a couple very short minutes.
   This is the case with any family! Every parent should know CPR, especially if they have a pool or live near a body of water. There have been too many 911 calls where the child dies because all of the family members are too busy panicking and yelling at each other to listen to the directions of the emergency operator and actually help the child! Sometimes it's too late because of how far the closest fire station is from the location, or because the ambulance has to actually stop at every intersection since too many drivers aren't paying attention to their surroundings.
  Anyway, so the other day Michael asked me what I would do if her ventilator broke or stopped working and Emma couldn't get the ventilator breaths she needs. My first answer was to see if she could breathe on her own with just the trach. I would disconnect the vent circuit and attach an HME (the humidifier piece with a filter on it so she wouldn't get dust or dirt in her open trach). From the many times we disconnect her vent to get a shirt over her head or to dump out the condensation water from the tubing, or when we switch out the vent tubing to a clean one every 10 days, she does really well and even laughs and pumps her arms in a fun way. But since we were talking about emergency situations and not just about how well her lungs are growing, Michael asked what I would do if she wasn't breathing well on her own and began crying and turning purple.
  I said I would get the Ambu bag and basically "be the ventilator" for her, squeezing the bag every few seconds to give her another breath. Michael asked when the last time I practiced that was. There has only been one time we thought she might need to use it (last summer, because we were away from the house for more than 6 hours and the ventilator batteries had all run out --> you can read more about that in the "Local Daycations" post) and that day Michael was in the back of the car with her. I admitted that I had not practiced it since she was still in the NICU. And that might have been even before we went to Michigan for her big 3D airway splint surgery!
   First, I made Michael run through all the steps that are needed to switch the ventilator over from the stand with the humidifier to the stroller (or the ventilator backpack, which will probably rarely be used due to it weighing 30 pounds, plus 16 more pounds when carrying Emma). Instead of just telling me what to do, he had me tell him what I thought I needed to do for each step before actually doing it. I like that; I'm a hands-on learner. And I also wrote down each step again. I originally did that after Emma came home from the NICU, but lost that paper somewhere in the house. **By the way, the reason I am rusty on this part is because Michael always does it while I clean out the suction machine and make a fresh bag of formula for her. However, I did know it in March 2018 when we were taking Emma on her stroller rides around the NICU, and I was also able to figure it out in January 2019 in order to take Emma on a walk when Michael was not home one afternoon.**

^^ Above you can see a couple pictures of how the tubing looks when it is just attached between Emma and the ventilator, without filtering through the humidifier. The bottom photo was Emma's first time at the Florida Mall in Orlando. We went there when my best friend, Mia, was visiting at the beginning of the month! Even after everything on the list I wrote down, there are a few extra steps before leaving the house:
   -- put an HME (Heat and Moisture Exchanger) on her so her throat doesn't dry out
   -- get the battery packs and spare trach (in case somehow the one she is wearing comes out all the way and then falls on the ground or gets dirty)
   -- take the feeding pump attachment that usually stays on the pole by her crib, and hook it on to the handle part of the bucket next to her so we can attach the feeding pump

   Now on to the Ambu bag part ~ we keep it under the stroller by the front door, but there is also another one, which is bigger and seems to be more for adults, that we keep in her bedroom closet. The toddler sized Ambu bag in the stroller is ready to use and has her "settings" on it so we wouldn't have to change anything if needed in an emergency.

  Emma very rarely uses oxygen anymore (hooray!), but we still always have the air concentrator on at home and bring an oxygen tank with us when we go out. The Ambu bag does not need to be attached to oxygen in order to work, however, the green part on the end is where we would attach it to the oxygen system (and turn that up!) if she was really having a hard time breathing. When we practiced the other night we did not connect it to the oxygen.
   If you see in this photo directly below, there is a red circle with the number 40 and a white tab under it that says Override with two caution triangles. If the pressure gets too high while squeezing the bag to give a breath into the lungs, the red circle can pop up to release some of the pressure. However, if the override tab is pushed up so it goes on top of the circle, it blocks the circle from releasing any extra pressure and it could puncture a lung and cause a pneumothorax (collapsed lung)!!!

  As for mentioning her "settings", this red knob on top adjusts the PEEP, which is how much air is needed to fill her lungs at the end of an exhale. Emma has been on a PEEP of six since we flew back from Michigan at the end of February 2018. Six is a great level for her to be at since five is the lowest it goes. As you can see, the knob is just past the five. The Ambu bag is then attached to the end of the trach to begin CPR (manual breaths given by squeezing the bag).

 ^^ Emma being a big girl and helping Daddy squeeze the bag. :)

^^ Mommy's turn to practice! 

    The other way to do it, which would be difficult for only one person to do, is if her trach came out all the way and we couldn't use the spare trach for some reason (if they both got dirty), we would put this plastic piece over her mouth and nose completely and attach the Ambu bag to that. BUT her trach stoma/hole would have to be covered the whole time to make sure the air goes into her lungs and doesn't come straight out of her neck. That wouldn't do her any good. We would put a piece of gauze over her stoma and then tape it down really well. Again, this would be hard for only one person because you'd also need one hand to hold the piece over her mouth and another hand to squeeze the bag to give the breaths. And possibly a third hand to hold her arms down!! As you can see, she was trying to grab the face piece and we were holding her arms down, just for the picture. The piece that would fit on her face is still wrapped up because we wanted to keep it sterile. Below you can see the unwrapped baby piece that no longer fits her face so we threw it away the other night.

   Today I was laughing when Michael was talking about why we would ever need to bag her with the piece over her mouth and nose. I joked that the trach she's using would have to literally fall in the mud, and then with butterfingers we also drop the second trach in the mud! His scenario was that if we were outside and she got stung by a bee and her throat swells up and the trach won't fit back in, then we would have to tape the gauze over her stoma and bag her with the mouth piece. Luckily those scenarios all seem like unlikely, but it could happen, so we have to be prepared for anything!
   There is another big safety scenario that I wrote about last month, talking about what we would need to do if there was a bad storm or hurricane, causing the power to go out and how we would need to use the generator. We had to get one before they would let us bring Emma home from the hospital due to her having so much equipment that requires constant electricity. This is not an Amish baby! You can read that by scrolling to the end of Emma's "Roadtrips & Preparation" blog post.

Trach Awareness Week

   It’s Trach Awareness Week! So here is some Emma specific trach/ recap life info: Since she has tracheobronchomalacia (severe floppy airway), she needed a breathing tube attached to a ventilator down her throat for the first 7 weeks of her life. They tried taking the tube out a few times, but she wasn’t able to breathe on her own. We had to wait for her to be big enough (4 & 1/2 pounds, and she was 3 pounds 3oz at birth) before they could do the surgery for the trach placement. I have heard many parents on social media talk about how they cried hearing that their baby needed a trach, but after one Fetal doctor thought Emma wouldn’t even live past birth, Michael and I had no problems with surgeries. “Whatever Emma needs, do it to help her breathe.” However, I cried so much when a respiratory therapist named Errica told me that Emma would *not* be able to come home a week after getting the trach, like the ENT doctor had told Michael. She said, “Please don’t think Emma will be able to go home any time soon.” 

At 10 days old (left) and 1 month old, before she had her trachetomy surgery. ♡

Bottom left photo from a few days after she got her trach and opened her eyes again.

   You can read the original blog post I wrote about Emma getting her trach in 2017 ---> HERE

Emma around 5 months old when she slept a lot because she was exhausted from passing out often.

   But we THANK GOD that’s old news now and she’s doing super well on her trach and vent! She still gets overheated easily, which is common for trach kids & those with dwarfism, which Emma also has (specifically Primordial Dwarfism with her Meier-Gorlin Syndrome), so we keep the house cool. Last Friday our AC went out and since Emma’s room was so stuffy, she got to sleep in our bedroom! It got up to 83 degrees inside by 4pm & stayed that hot until Saturday morning when the guys came to fix it. We had the windows open and 3 fans going in the bedroom, so she did great and did not get sweaty! Emma’s ventilator settings are slowly getting lowered, but she will most likely have the trach for many years. 

We survived the night!!! 

   A trach or tracheostomy tube is a small tube inserted into a hole into the trachea in the neck. This tube allows the person to breath without using or bypassing their mouth and nose. Emma needs hers for respiratory issues such as severe tracheomalacia and bronchomalacia, and chronic lung disease due to her Meier-Gorlin Syndrome. However, the 20 other people in the MGS group on Facebook have never had a trach (nor had Genu-Recurvatum/ backwards bending legs like Emma has). Even after she got her trach, her tracheobronchomalacia (which affects 1 in 2,200 babies) was so bad that anytime she held her breath to poop or if she took too big of a breath while crying, it caused her airway to collapse each time, which made her pass out, go limp and turn purple. The nurses always got her back pretty quickly, but Michael and I started worrying about mental damage because of the lack of oxygen to her brain from passing out so often, sometimes up to 10x per day! This happened a lot between August and December 2017 until we were finally able to go CS Mott Children’s Hospital in Michigan before Christmas so Dr. Green and Dr. Oyhe could perform the semi-experimental surgery with the bioresorbable 3D airway splints sewn over her trachea and bronchi. They are made to hold the airways open that way she would always get airflow to her lungs. She was the 16th patient to get the surgery done, the second little person to have it done, but also the one with the thinnest trachea wall. Dr. Green said it was like tissue paper! Although the surgery was super successful, and her PEEP (Positive End-Expiratory Pressure, which is the pressure in the lungs that exists at the end of expiration) went up from 14 to 18, which is a very high amount and not usually recommended, but it worked well for Emma and helped her stop passing out. 

Happy baby in Michigan after her big surgery (photos from 6-8 months old).

Top left photo on the way to trach surgery in July 2017. Bottom photos: February 2018, towards the end of our stay in Michigan when we finally got to hold Emma again. We couldn't hold her while she was on paralytics.

   Within two weeks after the Michigan surgery, her PEEP was down to a 6 (a huge, great deal for everyone because that is almost no support), she unfortunately developed little rips/holes in her trachea. Although her hair grows fast, her body heals slowly. Normally these holes heal within a few days, but because Emma's trachea wall is so thin, it takes her body so long (it's been 15 months so far) to build up scar tissue and cover the holes. She still has one or two now that we are waiting to heal. With these holes, the air that normally passes into the lungs goes into the surrounding tissues. If she is around other little kids who can easily pass along bad germs, or any adult who is sick, a virus could get into her body and she may not be able to fight it off and we could end up back in the hospital, or worse.. That's why Michael and I are so particular about who visits her and why she has not been able to meet her cousins or any of my friends’ kids yet. I sure went on a tanget there, talking about more than just why she has a trach, but it all ties together!! We are so proud of Emma and all she has been through these two years and how she has continued to smile through all of it. We are very pleased with how well her little body has healed from her six surgeries, and protected her, with the help of some medicine and lots of prayers! Happy Tracheostomy Awareness Week!

Being silly and finding another fun use for the blue cuff port.
The "real" use for it is to inflate the balloon inside her airway, but we always leave it deflated.

Doing well during our weekly trach changes! Switching it out for a clean one.

I don't normally post photos like these because I think seeing the stoma/hole is a little bit gross.
But if it is pulling down on the area (mainly bc she has her head back), that's what it looks like.

   Other notes about the trach. It is kept on with ties, which are thin, soft fabric pieces that go around her neck and hold the trach in place- the ones she has always worn are blue with little white stars. Emma's ventilator stand has a humidifier on it to make sure her throat doesn't dry out. The peach colored foam piece around the trach is so the plastic "arms" of the trach in the front of her neck don't rub on the skin. Sometimes we still have to put Hydrocortisone cream and Stoma powder (thicker powder that doesn't leave the hazardous smoke puff like regular baby powder) on the area because it still gets raw and red.

  Also I used to be confused about whether the correct long version of the word was "tracheotomy" or "tracheostomy" (with an S in it) because I thought I had heard both. And I had- Tracheotomy is the word for the actual surgery, while tracheostomy is the long version word of the trach piece that goes in her neck. Before Emma I used to think that trachs were only for older people who couldn't breathe or had lung problems because of smoking so much during their lives. Although I wonder how many babies need trachs and have lung problems because the mom smoked while she was pregnant? That is something that makes Michael and I very mad to see and we even sometimes saw it around the hospital! Not only are they hurting their own health and their unborn baby's health, they are also blowing smoke in the faces of other families where there are tons of signs around saying it was not allowed to smoke on hospital property or in the parking garages. 

   This morning I did a fun little photo shoot with Emma in the front yard by the flowers. And by "fun" I mean it was hot outside even at 8:45am and really difficult pulling the 65 pound ventilator stand through the grass, but Emma did so well. She did not mind or whine at all while sitting and laying in the grass, even with it being a little dewy, and she kept her balance the whole time. Her shirt is from a website called StomaStoma and I'm really trying to get Emma on one of the posts on their Instagram page, HiStomaStoma. Even today my BFF Mia made a cute little Trach Awareness Week collage and put it on her Instagram page for more people to see. 

dropping stuff has been her thing lately. 

   If we had a home nurse again, her only job would be to take pictures and videos of me doing all these photo shoots with Emma as well as all our medical care and doctor appointments for her and then Michael could edit together a web series and we'd be YouTube famous! #goals But for now I'll just keep blogging.