Advocate Like a Mother

   It is Wednesday, August 29th as I write this. I say that because I will not "publish" this post until right before we leave. We are currently at Arnold Palmer Hospital because on Monday Emma had a scheduled surgery to get her G-tube revised. It got so stretched out over the months, especially because a different doctor put a bigger size G-tube in when we came to the ER in May. That Sunday morning the size 12 G-tube had popped out three times within 8 hours, so the doctor put in a size 16 instead.

 Here is a picture from a couple weeks ago of Emma's gastrostomy site without the mepilex foam over it. The size of the hole should not be stretched out and bigger than the tubing Michael is holding on to.

   We are also at the hospital for Emma to get a bronchoscopy done, since she has not had one (per our request) since before we left Michigan in February. We have not gotten the results back from that yet, but Emma's ENT doctor here will consult with Dr. Green in Michigan to discuss her airway and decide if she needs to remain on the Augmentin antibiotic if she still has the fistulas in her trachea. According to the images they printed for us, it looks (to me and Michael) like there is at least one fistula remaining because it shows part of the 3D splint holding her airway open, rather than only showing all pink trachea "skin". One very good thing we learned after Emma's ABR (Auditory Brainstem Response/ hearing test, which was also done while she was under anesthesia) is that her hearing is perfect! I was actually surprised by that due to how often the ENT talked about Emma needing ear tubes to drain the fluid, which she found none of on Monday. Most babies and kids with trachs are commonly found with fluid in their ear canals. Also before Emma was discharged from the NICU in April, they did a hearing screening and the outcome showed that one ear had moderate hearing loss and the other had severe hearing loss. But hooray for that test being wrong in April ~ one less procedure this week!!

 Before the surgery and procedures (above) & After (below). They had to completely sew up the original prolapsed G-tube hole except for a spot for the drain and cut an incision above her bellybutton to create a new lower stomach hole for the new (yellow) G-tube.  This was difficult as she is very small for her age and "does not have a lot of real estate to work with", as the surgical doctor phrased it.

    Yesterday morning (Tuesday) Emma was taken down to radiology to do a contrast. This is a test where they put a clear liquid through her G-tube but in the Xray video it looks black so they can see if it is moving in the right direction, out of the G-tube, into the intestines and out as it should. I liked that they let me wear the metal apron so I could stay in the room with her. Usually we have to wait outside. The contrast was moving slower than expected, so they took a few more Xrays in her hospital room every four hours to check if the contrast was still moving. Her stomach was still very hard and big, looking stretched out so they had reason to believe there was a blockage causing all the air to not be released. The doctors referred to this blockage as an ileus, explaining that during surgery the colon often falls asleep and you have to wait for it to wake back up before that part of your body starts working properly again. Instead of doing anything about it, they wanted to simply wait it out.

   I am definitely not trying to say anything bad about any of the hospitals we stay at and doctors that she has, but since Emma has such a rare condition (Meier-Gorlin Syndrome), things often affect her differently than a "normal" baby. This makes it very hard and frustrating for us as parents because we know Emma very well and know what her reactions to IV fluids and other procedures are, but if what we suggest doing is not how the doctors normally do things, they deny our request to try it. (We had this problem in the NICU after coming back from Michigan- the doctors did not want to switch her from their amazing hospital ventilator to the LTV home vent even though we knew that was best for her. Once they switched her after we begged them, Emma's breathing instantly became so much more relaxed.) The current situation is that today is now Day 3 without Emma being on formula. We turned her EleCare formula off at home at 4am on Monday since she was not able to have anything in her stomach eight hours before her surgery, which started shortly after Noon that day. Emma never does well on IV fluids, so after every surgery she has, her skin usually gets very puffy. Luckily she is not puffy (in the face and hands) this time, however, there is a lot of air in her stomach that is not coming out for some reason. She normally poops four or five times a day at home, but now she has not pooped since Monday. We really believe that IV fluids dry her up, so we just suggested that they give her even an ounce of milk over a 4 hour period (at home she gets 1 ounce per hour). But since that doesn't work in other patients, the doctor refused to let the nurse try it with Emma. Emma is a very happy girl who laughs a lot (my dad even asked if she ever makes sad faces), but with all these tummy troubles, we have not seen her smile since before her surgery on Monday. She had made several sad faces, especially this morning.

   ... Well, I guess a different doctor did not want Emma on milk because maybe an hour later her surgical doctor came in saying we could try her on 10ml of formula over an hour. An hour and a half after him saying that the formula was measured actually administered into a pump for her. They only did this every three hours because they didn't want it to cause any more backup in her intestine where all the air was. Shortly after the 10ml of formula was finished, Emma began pooping!! Yes, we prayed for poop. And we were very excited about it. During the day we positioned her from her right side to her left side after each nap in order to move her intestines around a bit to keep things progressing. Therapy brought in the Tumble Forms chair so she could sit up for about an hour today. I was even able to get some small smiles out of her in the evening. They kept this formula schedule over night of 10ml over an hour, every three hours.

   Thursday now: This morning I was very happy to change another big diaper and find Emma's tummy to be about 80% squishy, close to how squishy it normally is. I tried tickling her when I saw her awake and she looked very happy. Another GI doctor said the nurse could up her formula amount to 10ml every hour and ran it continuously throughout the day and even raised it again around 2pm to her "baseline" of 30ml per hour. Now we just have to wait for her to pass the 24 hour test. Then, around 2pm tomorrow, they will know that Emma is tolerating the full feeds well and we can go home. Her nose swab culture came back positive for MRSA this afternoon, so everyone who came in to do stuff with Emma had to wear those yellow protective gowns. She is getting Bactroban to help treat that as well as Pepcid to make sure she didn't get stomach ulcers while she was not getting any formula. Before she was able to use her G-tube for medicine, they replaced her Augmentin with Ancef which is another broad antibiotic used to treat and prevent bacterial infections, and added a low dose of Reglan to help her poop. (Side note ~ this hospital has amazing water pressure! Her Pediatric Special Care room has a bathroom with shower in it, just like the rooms at Winnie Palmer do across the street.)

  One very nice thing about today was the visitors. This morning we got to see two of Emma's primary NICU nurses, Billie and Kim, as well as Dr. Lipman and Alison the pharmacist for a few minutes. And although we are eager to leave the hospital and sleep in our own bed (Michael and I have been trading nights of who sleeps at home and who stays at the hospital overnight), to me it seems like our marriage gets stronger each time we are here. This could be due to agreeing on everything that needs to be done for Emma and making important medical decisions for her together. Our marriage was the most amazing while in Michigan at the beginning of this year, spending almost 24 hours of 72 days together.

   Friday: This morning the surgical doctor came in to see us and said he felt like Emma's yellow G-tube "mushroom" inside her stomach was in a good spot and with her being able to go potty without any problems, her tummy being back to it's soft and squishy state, and her feeding schedule back to normal, he verbally approved our request to take Emma home today since we feel comfortable doing so. We got the discharge order around 12:45pm! Next week we will go to his office for a wound care follow up appointment and then can resume her physical therapy at the house a few days later. In a couple months we will get her yellow G-tube replaced with a regular clear one like she had before. Michael wants the Mic-key button, but two of our doctors have said not-so-good things about that one. I pick whichever one will keep Emma out of the hospital! We'll pray that she does not get another prolapse over time and that the tube and balloon stays in nicely as it should until she learns to eat on her own in the future. Yesterday was Emma's fourth month "at home"! :)

Emma's Summer

   Before going into what it going on this week, here are some of my favorite Emma pictures from the past two months since I have not been able to post recently since we don't have Internet at home. We cancelled it in January while we were at the University of Michigan and not using it. One thing I love is having people come visit Emma and see how well Michael and I take care of her. The most impressed seems to be Michael's mom. She gushes about how great we are doing as parents, especially with a daughter that requires a lot of medical attention. I really enjoy hearing these things.

 Being silly with Grandpa Al (Michael's dad).

 Comparison of growth between this 4th of July and last year when she was only a few weeks old!

 
   New things: she got her glasses on her birthday (June 12th) and removable leg splints later in the month by the Occupational Therapist. Now we go to her once each month so she can heat up the splints with a heat gun and bend them a little bit. This way Emma's legs will learn to bend and grow in the correct way rather than following the Genu Recurvatum way where the "knees" bend backwards. (I put knees in quotes because Emma does not have knee caps.) We have finally got home therapy for her. *Note: if you decide against having home nursing like we did, quadruple check if they are the ones to supply therapy nurses!* The home health company that we had for a week before Michael and I quickly agreed that we liked taking care of Emma by ourselves and knew "how she works" a lot better, we cancelled the home nursing service. The company never said anything about being related to therapy. They never mentioned it when we met them, signed up, never said anything while they were actually at our house for 12 hours a day, and therapy was not mentioned when we cancelled. It wasn't that I learned about it until a few weeks later when I called the NICU to let them know that nobody from therapy had contacted us like they were supposed to during the first week Emma was home. So we had to get a new referral from Emma's pediatrician, go through the process of getting Emma in the system (we're using Early Steps), have an at home interview with a case worker, have a "play session" with some therapists at their office so they can see where her development stands so far (she got low scores, although she was graded against regular healthy kids that had never been in the NICU), then three months after we started the process, the case worker found a therapist who could come to our house. Originally the company told us that they always want kids getting therapy at home because they learn better in a familiar environment, but when we mentioned it after Emma's development "test", they said it is really hard to get home therapy. It's only for 30 minutes each week.

   The reason we need them to come to our house is because Michael and I both have to work as much as possible in order to cover our bills. And that is just the regular home bills; it does not include medical bills. We have a weird schedule worked out and luckily our supervisors and bosses are so nice and understanding with our schedules because they know we are good employees and they love Emma too. At my job (911 and Teletype operator) I am on 12 hour shifts and mostly work 2 days then have 2 days off, etc. So Michael (special makeup FX teacher) works on the weekdays I have off. However, some of the days he could work have to be used for doctor appointments. Once in a while he will work half days if a doctor appointment is short and early in the morning. But it seems like all of the offices assume that if you have a baby with a lot of medical needs, the parents don't work, and they don't understand why we can't come in on a certain day for a doctor appointment or surgery. How else would families pay for medical insurance?

   Michael and I both have to be off from work in order to take Emma places, especially with the ventilator and how often she chokes on saliva (she can't really swallow yet, hence her still having the G-tube and needing a speech therapist to teach her to eat). The home nurses we had didn't seem to "get it" with Emma and anytime she sneezed they needed to call her doctor. That is an exaggeration, but the nurse freaked out when Emma was having trouble breathing one afternoon and quickly called the nursing manager and then Emma's NICU doctor. We knew she was okay and once we laid her down (she was just positioned in an uncomfortable way), turned up her oxygen, and changed her diaper, she was perfectly fine. The nurse seemed so surprised with how we knew what to do and that our methods were just what she needed. Michael did take Emma somewhere by himself one Saturday when I was out of town. The ride to the location went fine, but on the way back Emma's alarms were sounding, she was choking, he turned her oxygen up but it wasn't helping. She needed to be suctioned, so he had to quickly pull over and jump in the back to help her. Michael admitted that he would never try that again. So having therapy come to us is so much easier and convenient for us- and the patient!! We only have physical therapy right now. She really needs speech therapy as well. It is not a problem to have her at home with one parent at a time. In the car one of us (me) always has the focus on driving while Michael's focus is on helping Emma.

   This was a new..milestone?!.. from August 15th.  She is very flexible! She has been trying to put things in her mouth recently- toes, the blue cuff piece attached to her trach, the capped end of her suction catheter, blankets, etc- all which we believe will help her with speech therapy later on! We got her the green froggy chair (below) before her birthday. It is an infant seating chair that we thought would be good for her to work her neck muscles and try to hold her head up more in a sitting position rather than laying down or being reclined in the swing. It works pretty good for her, but she still needs more practice.

 The last three pictures are Michael helping Emma with some therapy moves that we were shown recently between the physical therapist and occupational therapist. She is good about grabbing her foot with her hand on the same side of the body, so it was suggested to teach her to cross-over. Although therapy is very important, especially since she is very smart but sometimes it seems like she is (at 14 months) as developed as a 3 month old, there is no need for her to learn to crawl or walk yet while she is still ventilator dependent. Emma's pulmonologist wants to do a sleep study in the next few months to see where he can lower her vent settings and slowly wean her off. 

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.