Gore-ganization 101

 This post is something that Michael and I have been wanting to blog about since we got everything organized. Before Emma came home from the hospital on April 30th I had looked up other blogs about "trach babies at home" to see how those parents organized all of the medical supplies. So here I will show and explain how we have things set up. Most of it was organized by Michael because he kind of has OCD when it comes to keeping things neat and clean, and he likes everything in certain places. Would you believe that this is the first time we had used our Canon camera to take pictures of Emma? It surprises me! But using our phones is always faster and easier for uploading to Facebook and Instagram.

Michael found this nifty four-drawer tool chest from Home Depot, which was the perfect size to hold all of the medical supplies that we knew she would have (we got the boxes a few days before she came home). It also fit so perfectly in this space between the crib and the wall. One thing that is hard to see in this picture is the metal PVC sprinkler pipe that he screwed into the wall which is what her turquoise feeding pump and bags are hooked on to. It wasn't until the second month that Emma was home (June) that Michael added the dry erase calendar and paper towel holder. On the calendar we keep track of which days we have to change out her trach to a clean one (usually every Sunday), and when we need to change her ventilator circuit tubing and clean her nebulizer pieces in vinegar (every 10 days).

  On the top of the chest (when Emma is in her room at night) we keep her pulse ox monitor. When we move her to the living room during the day, all this stuff comes with her. The top red number is her blood oxygen level - I call this her happiness level - and the green number is her heart rate. The gray thing is her suction machine to suction out her mouth if she gags while pooping and too much formula or saliva comes up that would be hard for her to swallow. We also use it to suction out her trach after she coughs a bunch and has phlegm. The clear crate has pink sodium chloride bullets that we flush the suction tubing with as well as many little bottles of purified water. Behind that, which you almost can't see, is her nebulizer, like a breathing machine where the pieces (not pictured) connect to a spot in the trach and the inhalation solution gets mixed with air and helps open up her lungs. She gets this twice a day, usually around 8am and 8pm. The bottles behind the pulse ox-monitor are just hand sanitizer and a water bottle to make it easier to clean the calendar each month.

See how Michael has every drawer labeled? So efficient!

First drawer: lots of stuff used for trach care like trach ties (the "collar"), long Q-tips, saline wipes, alcohol wipes to clean the equipment, more sodium chloride bullets, scissors, her baby toothbrush and Elmo toothpaste, stethoscope, thermometer, etc.

Second drawer: mostly feeding bags and Farrell (gas) bags. These get changed almost every day.

 Third drawer: extra circuit tubing, nebulizer pieces, suction catheters.

 Fourth drawer: Mepilex which is a thin orange foam piece that goes under the trach so it doesn't rub against her skin (you can see that in the top photos of Emma), and a ton of different sized gauze that we use to clean around her G-tube. The five bottles have distilled water that we use to refill her humidifier that is attached to her ventilator stand (not pictured.. sounds like we need more pictures here!)

 These two bags taped to the crib for easy access are for her trach. The one on the left does not have a trach in it because that is the bag for the trach she currently has in. Emma uses the Bivona Flextend 4.0 size. Michael always writes in permanent marker what date the trach was last changed.

 The third bag you can see below is just a medium size syringe that we use to deflate and inflate the G-tube balloon inside her stomach and check how much water is in it. Sometimes it has 5ml in it, so we check every few days that it is full with 6ml because that lessens the likeliness of her G-tube popping out if she pushes her stomach out while crying heavily (not common) or pooping (very common).

 Before Emma was born Michael revised the closet because he wanted her to have a changing table that was out of the way. That was before we knew she would have a trach and be attached to a ventilator at all times, so changing her on a thick pad on the floor or in her crib is much easier. Also not pictured is a Graco Pack-N-Play that we got very recently in preparation for any hurricanes. Michael said he would like us to move Emma and everything she needs into our bedroom on the other side of the house incase the tree in our backyard falls on the house/ over her bedroom. It will be fun having her in our bedroom with us for a few days, assuming another hurricane hits Florida this year. We also got a Generac generator before she came home in order to run all of her equipment during a power outage. During the last hurricane (Irma, September 2017) our power was out for almost 24 hours after the hurricane passed. I am actually surprised our power has not gone out from all the crazy lightning storms we have had this summer. So far the power has only flashed for a second without any issues.

 Items pictured here include a spot I "forced" him to keep to hang clothes. Because baby clothes are the cutest and I already forget about clothes she has if they are not hanging up for me to see. Fun fact: at almost 15 months old, Emma is currently fitting best into 3-6 month outfits. She weighs almost 14 pounds now. The dog pee pads are actually for Emma because for a while she had a terrible diaper rash so he put those under her when she was "nuding it up" to air out her butt and it is a lot easier to throw those away every day than wash dirty blankets every day. That box was 50 pads for $9.72 at Walmart. More recently we have used the pads in Emma's crib under her blanket incase her G-tube or feeding port pops out and leaks everywhere. The brown boxes are just extra medical supplies that don't currently fit into the Home Depot chest. So many more medical supplies, medicines, and diapers at the top of closet too. Since the diaper rash issue a few months ago, we switched to only using Huggies, water wipes, and Z-guard cream. Emma has the perfect baby bottom now and no longer cries during every diaper change. Hooray!!

 This is our "trach table" that Michael made out of a laptop table and changing pad. It is clipped around the bottom and Michael also sewed on velcro strips so it doesn't come apart. We sit indian style on the floor to do her trach care every night. The picture below that shows Michael's cleaning supplies. He does the cleaning of Emma's neck and stoma while I support her body and hold the trach in when the ties are off.

   Michael has made a few things to make life a little easier since Emma has been home, including this ramp that we have in the garage with anti-slip strips. Before he made this, one of us would always have to pick up the front of the stroller to get Emma in and out of the house.

   We have the Baby Jogger City Mini GT stroller and when Emma got to the point in March where she was off some of her medicines and able to go on stroller rides around the NICU, Michael made modifications so we would not need a "medical stroller". We need some normalcy in life and got the stroller from his parents before she was even born. The backpack has everything we need for all situations: extra trachs and trach supplies (same size/ 4.0 and one smaller/ 3.5), extra clothes, changing mat, diapers, various butt creams, syringes, medical tape, gloves, saline bullets, HMEs (humidifier), a copy of her 10-month discharge papers if we are ever at a hospital that is not in Orlando and they can't get her records, etc. I also have extra copies in the medical binder that bring to all of her doctor appointments. We still have at least three scheduled every month. In the undercarriage area of the stroller we have her suction machine, Ambu bag (for CPR via trach- luckily we have not had to use that!) and the battery pack to plug in the ventilator batteries when we are gone for more than 5 hours. See previous post "Local Daycations" for more about that.

   I have talked about the purple "in-VENT-tion" before, but Michael built this himself so we would be able to hook her ventilator into the Never Give Up board. He bought the dovetail bracket so the ventilator secures on and is the same bracket found on the ventilator stand. The whole purple piece clips on to the stroller in four places. We take it off in order to fold up the stroller in the car. The oxygen tank fits perfectly between the board and the back of the car seat! Isn't he just the most handsome genius!?

   Right now we are at Arnold Palmer Hospital (I'm using their Wifi to update!!) because Emma was getting a few scheduled procedures done today. Everything went very well, but we have to stay for 24-48 hours to make sure she recovers well. She needed to get her G-tube site fixed. It has been prolapsed since a month or two after she got it done in September of last year. We believe the issue was because she was on such high ventilator pressures (a PEEP of 14 in Orlando, and 18 in Michigan around Christmas) which expanded her stomach and caused a prolapse as well as the skin stretching out over time. The surgical doctor sewed up the site two different times, but it would always stretch out again. This also caused the G-tube to pop out many times since she had been home, but Michael and I were always able to get it back in without much of an issue. Of course that is something we shouldn't have to deal with, so today he closed up the hole all together. It will have a drain for a few days because closing it all together would cause an infection. Emma also got another bronchoscopy done to check to see if those pesky fistulas are all healed, as well as a hearing test. The audiologist said her hearing is perfect and she did not require ear tubes. Thank goodness!! We have been saying a lot of extra prayers lately.

Local Daycations

   Michael and I really try to give Emma the best life, especially feeling the need to make up for all the time we “missed” with her while she was in the hospital for so long, especially on the days we had to work and maybe couldn’t visit her at all. I am so glad those days are behind us and we get to see her all the time now and give her 100+ kisses each day! Often I will continue to kiss her until she pushes me away, other times she doesn’t push at all. We were feeling adventurous this weekend and tried a few new things, including keeping Emma out longer than we are used to and taking her to do fun things during our few days with the LPA and Potentials Foundation conventions, rather than skipping it all together. That would have been no fun and I already feel like there are too many days when we just sit at home, like when Michael has to work, I stay home with Emma (and vise versa will begin when I go back to work soon).

  Since we were gone for about six hours the first day of the conference, we were reminded the hard way that Emma’s ventilator Sprint battery packs only last six hours (two batteries, each holding a charge for three hours). This was the first time we had been away from our house that long. Usually we just drive to a doctor appointment and back, only being gone maybe three hours. But with Emma’s ventilator continuously alarming and flashing “Power Lost” (once both batteries died) and then “Power Low” on our drive home that evening, Michael got the Ambu Bag ready incase the 25 minute battery inside the ventilator drained and shut off too! If that happened Michael would have needed to disconnect the vent circuit from Emma’s trach and hook the Ambu Bag to her trach and basically breathe for her by squeezing the bag as each breath. We were super thankful that it didn’t come to that and when we got home we quickly grabbed Emma and everything she was connected to, ran inside, and plugged the ventilator back into the wall outlet! Since then we began looking for available outlets in all the conference rooms and when we go out to eat that way we can plug in the vent and save the battery for when charging is not available.

This was also the first time we brought extra un-mixed formula and her equipment and medicine for her nightly breathing treatments. The second night of the LPA convention, we went with our group to Kobe Japanese Steakhouse. I assumed we would skip it due to the chefs cooking food right in front of you with their traditional flame attractions. With Emma’s oxygen tank being necessary, we couldn’t have her near the table while the chef was cooking. At first Michael suggested we just keep the oxygen tank in the car and hope she sleeps through dinner, but she woke up after 15 minutes and started getting squirmy, with her blood oxygen level dropping enough that Michael had to take her back to the car. Dinner this night was at 7pm and Emma normally gets her breathing treatments (nebulizer and inhaler) at 8pm. We had noticed an outlet in the lobby that was not being used, far enough from the cooking tables, and Michael took advantage that, but unfortunately missed most of the meal. I texted him when our chef and the chef at the table next to us were done cooking, so Michael knew it would be safe to bring Emma back in with the oxygen tank on the stroller. In the future, I would probably either avoid places like that, or go earlier, but it was worth a try!

Having the un-mixed formula was really simple and probably barely any different than what a parent of a regularly healthy baby would do. The only difference is that Emma has a couple extra ingredients: 6ml of pectin (pectin is normally used to make jams, but the gastroenterologist told us it could be used to thicken up Emma’s stools- kind of gross, but it has been working) and 1/8 teaspoon of Beneprotein per 5oz bottle. Mix, shake, and pour into her feeding bag. Easy peasy!

The fourth day of the conference involved a lot of fun toursity stuff that we hadn’t done in a few years. We met our new friend families at the Orlando Eye (a huge slow moving ferris wheel that was built three or four years ago). I guess for some reason they have a “no strollers” rule for each pod, but when Michael told the ride attendant that she needed it because of her ventilator, we were allowed on without a problem. Emma slept through this, but luckily woke up before we went to the Sea Life Aquarium. All of these adventures were paid for by donations given to the Potentials Foundation, which was so great considering we are really tightening down on our budget this month, and everything is even more fun when it’s free! **Most of the money donated goes towards research for doctors as there is very limited literature about these conditions, including Microcephalic Osteodysplastic Primordial Dwarfism (MOPD) 1 and 2, and Meier-Gorlin Syndrome, which is what Emma has.

In the Aquarium we had a dad ask us about Emma because he said his 3-year-old son had several surgeries and the same feeding pump (Moog EnteraLite Infinity). His son was not on continuous feeds like Emma is, but the dad lifted the son’s shirt to show us the Mic-key button in his stomach. We hope Emma can get that one next time. Right now she still has one with a 9-inch tube attached (even when the feeding bag tube isn’t hooked in). When the dad and his son went back to looking at the fish, a girl who was probably the older sister told us that we were really brave for bringing Emma out to do fun things. She said most people just stay at home with this much medical equipment, but we told her that part of doing fun stuff is for ourselves too. I don’t like sitting at home all day either! Plus we think its really good for Emma to be in a lot of different environments and getting stimulation by seeing things she doesn’t normally see. Mostly she seems to like looking at the different colored lights, but when Michael would scoot the stroller next to each area, Emma watched the fish, turtles, and sharks swim around.  We all had a really great time!!

The timing was really good for dinner the last night when we all the Potentials Foundation families ate at Unos. We got there at 5pm and this time Emma did sleep through the whole dinner and we were even home an hour before she needed her breathing treatments. The battery for her pulse-ox did die during dinner, but that is a lot less of a big deal! That monitor tells us her blood oxygen level (I call it the Happiness Level) and her heart rate, but by now we should definitely be able to guess her levels by her skin coloring and breathing anyway- and we can! I always remember her main respiratory therapist, Gary, telling us, “Look at her- don’t look at the monitor. You didn’t give birth to the monitor.” A slight issue at dinner was that there was not a ramp to get to the area down a few stairs where everyone else had tables reserved. I guess technically we could have picked up the stroller or gently guided it down the four steps, but we found another spot on the main level next to an outlet. Michael said, “Take a breath, it will be fine”, when I began stressing about the batteries running out –since by then it had been 8 hours since we left the house, plus a little over one hour of the battery being plugged in after lunch- but I insisted on plugging the vent in. I definitely didn’t want Michael having to use the Ambu Bag while we were at dinner! I’m pretty sure Michael was glad I put my foot down about that later because when we got home, both Sprint batteries were blinking orange, meaning they were almost out of juice!

Next time we will have to actually practice taking Emma out of Orlando for the day. That would involve bringing the big, blue oxygen converter that we always use in the house to ration the oxygen tanks. That would be if we are visiting my parents for the day which we would plug in and use at their house. Michael has bought a couple car chargers that plug in to the battery, but for some reason they don’t work in my cigarette lighters. I know they work because I use them to charge my phone and listen to music. Michael tried the charger in his car and it does work, but we always take my car because I drive while Michael sits in the back with Emma. Just a few more things to figure out before taking her on a real vacation!

** If you’d like to make a donation to the Potentials Foundation to support Emma and our friends with Primordial Dwarfism, please click this link to visit the Give page. ** I am currently updating this post with pictures now (August 27th) so here are few more recent photos as well:

LPA & the Potentials Foundation

   When Emma was officially diagnosed with Meier-Gorlin Syndrome by Dr. Mark Hannibal at the University of Michigan in January, we began reaching out to different organizations that we could become a part of for her. The first one we contacted was the LPA: Little People of America. Then we searched more specifically to find Sue Connerty who lives in the UK and is the president of Walking with Giants, which is for Primordial Dwarfs. Meier-Gorlin Syndrome falls under the category of Primordial Dwarfism. Since we live in the US, she put us in contact with Christy Jordan who is the president of the Potentials Foundation- the US version of Walking with Giants. We found out that the LPA was having a week long conference at the beginning of July in Orlando and hoped to go to it, but at the time (in January) I was currently scheduled to work that weekend and did not want to think about asking for time off in the summer while I was already using a lot of medical leave to be in Michigan with Emma for her experimental 3D splint surgery. Luckily, since Emma finally came home from the hospital on April 30^th, and I was able to take 12 weeks off to enjoy a “real Maternity Leave”, the LPA fell on my 11^th week! Originally Emma had a big surgery day scheduled (she needs to get her G-tube revised again and her hearing re-tested) for July 9^th and 10^th while the convention was going on. But a week before, the surgery got postponed and we were able to make it four of the days! Michael loves to remind me that God has everything under control, even if to me at first, getting an important surgery pushed back a second time, did not seem like a good thing.

   The first day (Saturday, July 7^th) was just getting registered for the conference and then waiting around for a Meet and Greet. Everyone was very friendly and helpful. Michael said he was nervous because he didn’t know what to expect. Our name tags had a special purple ribbon attached to the bottom that said “First Timers”. I guess Emma’s cuteness drew attention because a lot of parents would come over and start asking questions, which is super normal in a conference like this.

The second day we met with the Potentials Foundation, a much smaller group (pun not intended). Everyone went around and introduced themselves before Christy Jordan talked about the foundation, what we would be doing over the next few days, and then we played a BINGO game to get to know everyone. It was cool to meet a handful of the Primordial Dwarfs that we had watched documentaries on including Bri Jordan, Nick Smith, and Hannah Kritzeck. We ended up sitting at a table that had a 32-year old girl named Jenny with Meier-Gorlin Syndrome! We talked to her a lot about the condition, but also about her jobs and regular topics. She also does not have knee caps and walks just fine, so that made us feel good bout Emma, however this girl did not have the backwards bending legs. Jenny was also surprised that Emma had a trach because of the many people she knows through social media with MGS, none of them ever had a trach. That makes me wonder if Emma is the first? Another reason why a lot more research needs to be done for doctors to be aware- to help Emma or other kids with the same conditions. We all went out to dinner that night, which was paid for by the foundation! Christy told us that about 1/3 of the donations go towards bringing everyone together once a year and the rest of the money goes towards research. And research is definitely needed!!

The third day, Michael and I brought Emma back to the hotel where the conference took place for an hour-long meeting before almost everyone went to a water park. We did not go to that for obvious reasons: Emma having a ventilator and G-tube. So we hung out at home and ran errands before leaving the house again to meet all of the families at Olive Garden. That was a really good time again. We sat next to different people than the night before, which was great, because then we got to hear all of the different stories of struggles, hope, and achievement!

The fourth day/ yesterday (our last day going) was probably my favorite. Everyone met outside the Orlando Eye – the huge, slow moving ferris wheel – and then some people did the Sea Life Aquarium after that before grabbing lunch and meeting back at the hotel for a genetics workshop. Two of the other girls, Maddi and Loini, had gone to Islands of Adventure earlier and Maddi won a stuffed toy cat in one of the arcade games. When she walked in the room she asked us if it was okay if she could give the cat to Emma. I seriously thought I was going to start crying- it was such a sweet gesture! In the workshop meeting we met Dr. Michael Bober and Dr. McKenzie, the leading researchers in genetics and orthopedics in the study of Primordial Dwarfism. They work out of Nemours Children Hospital in Wilmington, DE. I felt good when Dr. Bober said, “Meier-Gorlin Syndrome is the healthiest type of Primordial Dwarfism.” At the end the genetic counselor, Angie, handed out Parental Permission Forms for Research Study so we could give consent for them to get copies of our kids’ hospital records that way they have more (updated) information about the various types of Primordial Dwarfism, including MOPD 1 and 2. The forms were voluntary, but I think everyone still signed them. I filled out two for Emma since she was seen in Michigan for a while too.

Side note: Emma needed a diaper change during the doctors meeting. Michael is more of the “mom” so he left to change it, and on the way to the bathroom he saw actor Warwick Davis in the hallway!! J

After that work shop we had two hours until our last group dinner, so a lot of people hung around to talk and say good-bye (or as we prefer to say, “see you later!”) if they were flying home early and wouldn’t be at dinner. One of the moms, Lori Everson, passed out copies of her book “Take Me as I Am” to everyone and of course we all asked her to sign our books! I can’t wait to read it. We got to talk to her and her husband, Tim, a good bit about their 5-year-old daughter, Harmony, who was always running around and dancing. Every story is so unique, and this weekend definitely created a bond stronger in person. Although the whole foundation only gets together once a year for the LPA conference, which is in a different state each year, they have “Chapters” where everyone within 100 (?) miles of certain cities meet up a few times per year. I believe there is one near Orlando that meets up in August. Michael and I will definitely go if I’m not working. Last time I was working and Emma was still in the hospital.

 ** If you’d like to make a donation to the Potentials Foundation to support Emma and our friends, please click this link to visit the Give page. **