Michigan Photos, Part 2

  Here is the second helping of favorite photos while we were living in Ann Arbor, Michigan for two and a half months while Emma prepared for and recovered from her 3D Splint surgery with Dr. Green. It is still considered to be an experimental procedure and she was the 16th patient to receive the splints.

 1/16/18: When you can't bring the baby to the snow, you bring the snow to the baby!! I loved that one of Emma's primary nurses, Bridget, let me fill up a bucket of snow to bring to Emma so she could feel it. I also loved that none of the desk staff questioned it.

 Emma always loves getting her hair shampooed with a head massage during bath time!

 1/18/18: Occupational Therapy with Alice to practice reaching for and grabbing objects.

 1/24/18: Around this time, although the rest of Emma's recovery from surgery was going well, because her trachea tissue is abnormally thin (less than 1mm thick) tearing occurred which created a couple small fistulas. One of the fistulas caused air to leak into her chest, and too much air caused a pneumothorax (collapsed lung). This happened on two different occasions, so twice she had to get a pericardial drain placed to suck out the air, and the chest tube to keep the lung open.

 1/27/18: Fresh air in the Law Quad, part of the University of Michigan.

 1/29/18: Snuggles with her new best friend. On this day Emma was having trouble maintaining a warm temperature and stable blood pressure. There were several reasons for this (blood needed for testing, but her body was not making enough new blood fast enough, and some of the meds she needed included a side effect of low blood pressure, etc) so they had to give her a blood transfusion on a few different days and put a thing that blew warm air on her under warm blankets. She was back on sedation and paralytics during this time so her ventilator pressures would stay low and keep the fistuals closed, in hopes that less air would leak into her chest. Poor baby.

 Sweet kisses from Daddy before one of her weekly bronchoscopies (2/5/18) and being read a Valentines book that was sent by Billie, one of Emma's primary nurses in Orlando (2/8/18)!

 2/8/18: Holding the baby's arm up while the nurse changed her chest tube dressing. She did very well and did not squirm or make any sad faces. She is such a brave little trooper!!

 2/10/18: I absolutely LOVED laying in the crib with Emma. I did this about six times? I liked it even more than holding her. I loved watching Fixer Upper or the Simone Biles biopic at night and talking to Emma during the commercials.

 2/12/18: Very excited to hold Emma after three weeks of not being able to because she had been on paralytics. Since I had my crib time with her, I definitely felt that it was fair to let Michael hold her first.

 2/14/18: After a meeting with Dr. Green, Michael and I decided that the best option was to vote against another surgery (a risky/ low survivable trachea replacement surgery which involved using her esophogas to cover up the fistulas)

 One big thing that Michael and I worked on with Emma while in Michigan was teaching her to hold the monkey pacifier herself. Every time we put it in her mouth to soothe and calm her, we also put her hands on the pacifier. Finally around her 7 month "birthday" she caught on and one day when I put the pacifier in, both hands reached up to hold the pacifier before I could reach for her hands myself. That was another proud parent day for sure!

2/20/18: Emma being her usual silly entertaining self and playing with her various tubes while sitting up in her fancy new Tumble Forms chair. 

We all flew back to Orlando on February 21st. :)

Michigan Photos, Part 1

  We have been back in Florida for just over a month now, so here are the first half of my top favorites from our time while we were in Michigan for two and a half months. If you have not seen the PICU Christmas post that I wrote in January, please check it out since I just added pictures that were not originally on there.

  One of the first pictures I got of Emma when we saw her after we arrived in Michigan on Monday, December 11th. She had a 9-minute cardiac arrest when she got to the PICU around 3pm (due to her trach being clogged), but was smiling and happy to see us when we arrived around 6pm. Originally I was pretty bummed when I was told the day before that I was not able to fly up with her in the medical jet, but after hearing about the arrest, I am so very thankful that I was not there to witness the event.

 12/15/17: Emma got a visit from Denver, one of the therapy dogs at Mott Children's Hospital.

12/16/17: Six months old now and laughing about something! :)

 12/22/17: Working on holding her head up and tracking objects with Alice and Sarah, the Occupational and Physical Therapists at Mott. I was very excited that they worked with her on so many things, when I assumed they would only deal with the 3D airway splints, which was the main point of our stay. It is a really great hospital.

 This is Emma's oral brush given to us by the Speech Therapist, Samantha. She showed us how to get Emma used to different feelings (to later use a spoon to eat with) and instructed us to practice holding Emma's cheeks together while using the pacifier to work on her sucking muscles. We either dipped the brush and pacifier into sterile water or formula.

 12/27/17: Heading down early in the morning for the big surgery!! Lots of prayers were said the night before, during and after this surgery. Emma did so well. We could not be more proud of our girl!

 12/29/17: Although heavily sedated and on paralytics, Emma peeked her eyes open for us this morning and gave us a quick smile before going back to sleep for a long time.

 When Emma had recovered more from surgery and it was safe to do so, the nurse and respiratory therapists would use this percussor to "hit" Emma's chest and back in order to get the secretions moving up so they could be suctioned out of her trach. The tool was loud against her skin, but very soft and she really seemed to like it being used.

1/3/18: Emma was lifted off of the sedation and paralytics on New Years Eve. She still slept most of the day. This was a few days later when she did not require so many naps. This day her PICC line was moved from her left arm to the right arm because one of the lumin wires had migrated up her neck instead of being in a central spot near the heart where it should be. 

 1/9/18: I was very happy when one of the nurses, DeSharra, said I was allowed to sit in the crib with Emma! She seemed a bit confused by it, ha. :)

 1/10/18: A few pictures early in the morning before Emma went down for another surgery- a tracheostomy revision so it would be easier to change the trach with the 3D splints placed.

1/13/18: Emma in her favorite place ~ Daddy's arms! He is so great at getting her to laugh by making silly noises, making her toys give hugs, and massaging her head.

Slow and Steady Wins the Race

Since the last update almost two weeks ago, the biggest thing is how much Emma's PEEP has come down! PEEP is the positive end expiratory pressure, which is the amount of air that gets pushed through the ventilator, trach, and airway to keep her lungs open. It was at 12 last Tuesday before she went down to the OR to get the bigger pericardial drain since the smaller one she had in before was not helping get the air out of her chest enough. Down in the OR the surgeon saw that her lungs were over inflated with the PEEP of 12, so they turned it down to 8! She did so well with the lower PEEP that they kept it there over night to make sure she was still ventilating and oxygenating well. The next morning they tried turning it down to 7 and that evening it was at 6! Two days later (this past Friday) her PEEP was at 5. They told us that 5 is basically the starting point and compensates for the circuit tubing. 

Doctor Green came in exclaiming, "This is ridiculous! Her PEEP is at five! She is doing so well!" All of us are frustrated about her stupid fistulas (the two holes in her trachea tissue that are causing air from her ventilator to be pushed into her chest). If it wasn't for those holes we would have been back in Orlando two weeks ago. But we also might not have known how well Emma could do with a PEEP so low, which is incredible. A few weeks ago when her PEEP was 12, Doctor Green assumed Emma would need to stay on the ventilator long term, but now he says she might not need the ventilator too much longer. Maybe a year? Maybe less! He didn't give us a time estimate, but that's me being hopeful. Unfortunately the air in her chest has caused three pneumothorax (collapsed lung, on the right side) in the past two weeks, so we really need those fistulas to heal. Two of the times (on January 24th and yesterday morning) they had to put in a chest tube in to keep the lung open. The other time it happened was not severe enough to need a chest tube, and when it happened yesterday they would not have known except that she has been getting daily X-rays. All of her vital signs stayed stable, so I think Emma is trying to tell us that she doesn't need her ventilator or her right lung, ha! But she'll keep both of them for now. 

Today was a big day. Emma had another bronchoscopy for Doctor Green to put the tiny camera light down her trachea to see if the fistulas had healed enough to keep waiting. If they had not, or if they had gotten bigger, she would need a surgery scheduled at the end of the week to patch the holes, meaning they would have to open her chest again. But at 1:30pm with ten doctors and nurses in the room (13 people total including me, Michael, and Emma) he did the bronchoscopy and we all received good news. The fistulas had enough granulation tissue around them that we could keep waiting for them to heal on her own. The main downside about waiting for her to heal is that they have to keep her sedated and paralyzed, which she has been for the past two weeks. Doctor Green wants to keep her sedated for one more week now. We are not able to hold her when she is on paralytics, and that has been hard for us, as well as not seeing her eyes open or being able to make her laugh, but a few times lately I have been able to snuggle next to her in the hospital bed for a couple hours. It is obvious that Emma knows when we are with her because tonight her heart rate was at 150, which is high for her being sedated without trying to move over the paralytics. But when Michael held her hand for 15 minutes her heart rate got down to 130, and when I was also holding her hand, her heart rate eventually got down to 112. That makes us feel good. 

Backing up to last month: On the day before Emma turned 7 months old (January 11th) her genetic testing results came back and she was officially diagnosed with Meier-Gorlin Syndrome, which is under the umbrella of Primordial Dwarfism. She has type ORC1, which is the type with the shortest stature, along with ORC4. Emma will probably grow to be between three and four feet tall. The geneticist told us that of all the people who have Meier-Gorlin, 29% of them have this type. There are 8 types (strands), but as of a 2015 report, there were only 67 known cases in the world. Some of the most common characteristic features include short stature, underdeveloped or missing knee caps (the geneticist told us that the part I thought were her knees, are really the end of her femur bone), Genu Recurvatum which is the backward bending knees, small low-set ears, and a tiny jaw. 

To get very technical and throw out a bunch of science/ math equations, her sequencing results are as follows ~

Pathogenic Variants:
1. ORC1 Exon 4-Heterozygous
2. ORC1 IVS9-Heterozygous

Nucleotide:
1. c.314G>A
2. c.1482-2A>G (ISV9-2A>G)

Protein:
1. Arg 105GIn
2. N/A

The geneticist explained what this means and even drew out a little DNA graph of exons and introns, but a lot of it went right over our heads. Maybe if we were explained it five more times it would make more sense? He did say that the introns "code" is ISV1, ISV2, and so on, so in the Pathogenic Variant (#2 above) it's Emma's 9th intron in that strand that has the change. Because she has two of the heterozygous, it means Michael and I both carried the recessive gene creating a compound heterozygous variant. He said we have a 25% chance of having another baby with the same thing, but we agreed that we would only have one kid even before we found out in May that Emma was half the size of an average baby. 

The geneticist printed out a paper from 2011 that reported two other people with the exact same mutations: a 47 year old man in the US and a 15 year old girl in the UK, both of whom would be six years older at this point. There is very little information on Meier-Gorlin Syndrome and a lot of the information we can find seem like old studies. So I'm not sure how many cases there are as of 2018 or how long it would take for Emma to be added to these studies, as case #68 (or maybe 75 by now). 

Emma's stats: She was born at 37 weeks and 1 day. Birth length was 15 inches, recent lengths are 19 inches at 7 months and 20 inches now. Birth weight was 3 pounds 3oz (1450 grams). This hospital in Michigan measures in kilos instead of grams and in the past two months she has gone up and down between 4.4k (9.7 pounds) and 5.5k (12.3 pounds!) depending on how much fluid is in her system. IV fluids love to soak into her little body and she can puff up very quickly because of it, so she is often getting doses of Lasix and Albumin after a procedure. We are so excited that we finally got the genetic results since we had been waiting since May when we learned that our daughter was a Little Person. 

Roller Coaster Ride

We have definitely been on a roller coaster lately and right now we're on a loop that goes upside down or perhaps one of those corkscrew parts that go around a few times. Also since last Monday I have been making a lot of beautiful scrapbook cards and pages, mostly featuring pictures of Emma. It's been a great way to pass the time in the hospital, but also a good distraction on sadder days.

At the beginning of last week things were going really well and we had begun the process of getting Emma flown back to Winnie Palmer Hospital in Orlando. However, on Thursday when they did another bronchoscopy and CT scan they saw that she had a 3-4mm hole (or rip or tear) in her trachea/ windpipe tissue, which is so thin already, only estimated to be 1mm! Dr. Green told us that the best hope would that the hole would heal on its own, but if she got a really bad infection in her chest called mediastinitis then they might have to open her back up and possibly take out the 3D splint that is sutured around her trachea. The word "unsurvivable" was used and I began crying. The nurse, Sara, even looked like she was getting teary-eyed from the thought of us losing Emma. They thought the hole was possibly caused by the cuff (tiny balloon at the end of the 41mm trach piece) rubbing against the tissue and slowly creating a hole.

Since they decided on Thursday that the hole should be fine to heal on its own -but probably taking about two months- Michael flew home to Florida on Saturday so he could go back to work on Monday. However, on Sunday they saw on an X-ray that Emma was getting more air in her chest (she just had a few tiny air bubbles before that they were not too concerned about). Dr. Green and his team thought they might have to do that big, possibly unsurvivable surgery the next morning (he has never had to take out one of the 3D splints before), so he suggested that Michael fly back up to Michigan because he knew Michael wanted to be at the hospital for that. He got back in the room with us around 11am on Monday morning. Thankfully by then they had come up with a less evasive plan and were able to put in a tiny pericardial drain in her chest (what I refer to as an air tube). I was thrilled with an easy, helpful solution. It didn't sound like a big deal until one of the doctors asked permission to give Emma chest compressions if needed. We said yes, but she did great and did not have any issues during the procedure.

The plan after getting the air tube yesterday was to just wait and see how she did. They would keep checking to see if she was getting more or less air in her chest. Part of the issue is "how do we prevent the air tube suction from bringing in the same amount of air in from her ventilator?" They turned her PEEP (ventilator pressure amount that keeps her lungs open) down from 13 to 12 in hopes that it would help the trachea relax and then scar tissue would eventually fuse the hole. But that could take a while to work, so they are going to keep checking on it every day.

Emma has been on an antibiotic called Merapenum for most of the past four weeks, but over the weekend her while blood cell count was almost double what it should be, meaning her body was working really had to fight off an infection. The normal amount for her to have is 12,000 and she had 23,000! The Merapenum fights a lot of different bacteria, but not everything, so they added Vancomycin back on and that helped a lot because the next day her white blood cell count was back down to 13,600. Yay!!

Last night we went to sleep peacefully, but were woken up at 4:30am when my phone rang. We had been so happy every morning without receiving a phone call in the middle of the night, but this morning I specifically remember saying "Uh oh" before answering. They said the scheduled X-ray showed that her right lung had collapsed. I guess it was not super unexpected since they did tell us that having too much air in the chest can compress the heart and lungs and cause issues, hence putting the air tube in yesterday. They quickly put in a chest tube (inserted on her right side, close to under her armpit) to help keep that lung open. She has always had more problems with the right lung, which is why she does not do well laying on that side. Before Christmas Dr. Green even talked about maybe removing the top portion of the right lung during her 3D splint surgery, but did not end up needing to do that. I wonder if he will want to remove it now?

When Emma gets crabby anytime she is "bothered" either by the nurses needing to draw blood, or give her the blood clot shot, or her own body issues such as having a dirty diaper, or a lot of gas coming out of her G-tube prolapse, being sweaty, etc. she cries and gets High Peak pressures in her ventilator. The doctors want as less pressure as possible (33 or less is preferable- it often gets up to 70 when she cries), so they are going to keep her sedated today and maybe even for the next few days, as well as adding a low dose of paralytics if needed. Keeping her calm puts less pressure on her trachea and lungs, which should help the hole heal faster.

Those are all of the big updates for now. They put an NG tube in her stomach through her nose over the weekend to help release pressure and gastric fluid so her stomach isn't as distended and pushing against her lungs. I think this was a great idea because that means more fluid and gas is slowly coming out of the tube instead of her often "farting" through the G-tube prolapse, which almost always causes her pain. They have continued to slowly wean the medicines when they can- not every day- but usually give her Tylenol and Versed as needed, which she responds really well to. 

The roller coaster of medical issues translates into a roller coaster of emotional issues. Luckily somehow Michael and I are usually either both viewing things positively or at least one of us is positive. I'm the positive one right now, while Michael is feeling sad about the constant new issues, and we both agree that the more issues that pop up, the harder it is to stay positive. But I absolutely love when people tell us how strong we are and how great of a marriage we have. (Words of Affirmation is my Love Language.) I cried on Thursday night thinking about going the next two months without Michael in Michigan, so I'm glad to have him back here, even though it is on less than desirable circumstances. We make each other laugh a lot, which I think has been so helpful. One night a couple weeks ago when I was in a "blah" mood, he told me to stand next to Emma's crib with him, he put his arm around me and which we were watching our daughter sleep, he said, "This right here is everything you need. On the count of three we are going to laugh." My fake laugh quickly turned into a real laugh and he asked me if I felt better. I did. I actually read something that said your body doesn't know if you think something is really funny or not, but the act of laughing still releases the same endorphins. Michael and I also thank and compliment each other often, and tell the other our favorite things about their personality on a daily basis. I recently wrote two lists about what makes Michael the best husband. One being more current (52 reasons), and one with a list of why I first fell in love with him (20 reasons). I know the list will keep growing too and I look forward to it! 

Recovery Detours

Since last week, Emma is continuing to recover brilliantly from her 3D splint airway surgery which occurred on December 27th. We have not had anything big happen regarding respiratory and we say thank you prayers every morning that we didn't get a bad phone call in the middle of the nights from a nurse or doctor. She is continuing her streak of not passing out- today is Day 24 since her ventilator pressure was turned up which helped her airway stay open, and of course now the splints are doing that for her. With that said, we have encountered "detours" in the recovery process as other issues have jumped in the way here and there. 

She was kept mostly paralyzed and sedated from the 27th to the 30th to help her heal faster and safer without moving around. On the morning of the 28th she peaked an eye open and gave a quick smile before falling back asleep. We were told that her heart rate needed to stay around 125-130 beats per minute because if it went higher that was an indicator that she was trying to wake up while still being paralyzed. That was never an issue though. It has actually been more around 109-115 this week. They put a sticker on her arm with little metal shocks that they could attach tiny alligator clamps to (like jumper cables) in order to gently shock the muscle and see how much more or less paralytics she needed throughout each day. One of the respiratory therapists told me that on her ventilator monitor, which had mostly red and blue waveforms, when there is a yellow wave it means something is triggering the vent to give a breath. They couldn't be sure if it was always Emma triggering it, which would also mean she was waking up under the paralytics, but any time during those four days when there were a few yellow waves, they would do the shock test. 

One of the "detours" was the lack of urine output Emma had, totaling only 2ml or less per hour. She also got puffier and was retaining a lot of IV fluid and post-surgery body fluid in her face and arms. At one point her hand was so puffy that she couldn't close her hand or curl her fingers at all even when she did start waking up. They gave her Lasix and other medicine to help her pee. On days that she had very little output, they used a bladder scanner to make sure that she was not full. It took more than 24 hours to see results from the Lasix, but when her urine was back to good output levels, she stopped pooping. It was more complicated to get her to poop since at that point they had stopped her formula feeds, so they had to give suppositories and an enema instead of regular medicine. Two days later that problem was resolved. 

The second detour involved the arterial line they had placed in her left thigh the evening after the surgery. They needed to place this so they could monitor her blood pressure, but they were able to remove it 48 hours later. It wasn't until they took it out that I saw they had stitched the tube to her leg to stay in. I assumed they could have just used a lot of surgical tape. :(  On December 30th the nurse noticed that Emma's left foot was getting cold and had a purple tint to it so they did an ultrasound of her left thigh where the line had been and found a blood clot. Dang it. They began giving her a Lovenox (mild blood thinner) shot twice a day for that. Next week they will do another ultrasound in her leg to see if the clot has dissolved or if she needs to keep getting the shots. One thing I thought was funny was that the ultrasound tech needed to get a different angle of her leg and asked me if I could bend her leg "froggy style". I replied with a quick, "Nope!" Then explained that her knees only bend backward due to her Genu Recurvatum. Also, because she has been given what was a necessary amount of Lasix, her kidneys have had to work too hard and are now considered "injured". They test her creatinine levels daily and did say they have elevated (from 0.2 when we got to Michigan to 0.8 this morning and 0.7 tonight) which is higher according to her muscle mass. So not ideal, but nowhere near the level of needing dialysis. Unfortunately two of the medicines that Emma is on (Zosyn and Vancomycin) can have an adverse affect on the kidneys, but we were just told by the kidney doctor that they have switched the order of Zosyn to Ceftriaxone starting tonight and her Vancomycin is continuing to be weaned down. 

The third detour has to do with her abdomen distention. We aren't sure if it got distended further when her face and arms got really puffy, but we didn't notice until her face and arms went back to their normal size. According to one of Emma's Orlando nurses, her girth was around 38cm at the beginning of December. Over the past few days it has been between 48-50cm. When she does not have her diaper on for changes, she looks like a pregnant baby. 48cm is the length of her whole little body! They have done a lot of tests and scans and sent cultures to see what could be the cause of why so much fluid is in her abdomen (it's called Ascites). It's not in her stomach or intestines so they can't just give her more Lasix to pee it out. Some days her abdomen has felt harder, which is more concerning, and we can tell that it is stretching her skin because it gets plastic looking, but today it felt pretty squishy at 49cm and she wasn't crying when we pressed on her. We have been told that they are considering doing "a tap" which involves putting a needle in to test the fluid as well as removing some of it. They don't want to have to do this because any foreign object entering the body increases the risk of infection. Plus, it is possible that they could remove some of the fluid, but the body thinks it is supposed to be there and it makes more fluid. 

On Wednesday during rounds they told us that Emma's double lumin PICC line in her right arm had started migrating up towards her neck, which is not what they want since it is supposed to be aimed at the heart. So they had to take it out and place a new one in her left arm. They couldn't take the old one out until all of her medicines had been switched to new IV lines and attached to the lines in her left arm. That night I helped the nurse and the Nurse Practitioner remove the old PICC line. My job was to hold Emma's hand and keep her arm straight and down against the blankets. I also had to wear a mask to cover my nose and mouth. She had her head turned away from me, but I could tell she was crying even though, with her trach, her cries don't really make sound- just mousey squeaks sometimes. I kept thinking, "This is not anything I ever thought I would have to do when I was in the first two trimesters of my pregnancy." But here we are, and all we can do is keep going! This night that her new PICC line was placed we noticed that she had developed a splotchy, goosebumpy rash that covered her cheek, ear, neck, and chest area. Yesterday it also was around her eye and she kept scratching it. One of the Infectious Disease doctors came to examine her and confirmed that he simply thinks it is an allergy rash, but we aren't sure what it is from. Unless they used a different kind of ..something?.. during this PICC line procedure, we don't know what else could have caused it since she has been on the same medicines and didn't have any problems before that. She has also spiked a fever (100-103) about five times over the past few days, so they have sent more cultures, but they keep coming back negative. That is good, but confuses the doctors and nurses to why the fever keeps coming back. They give her antibiotics and/or Tylenol (and Benedryl for the rash when it is more noticeable and itchy at night), and then her temperature goes back to normal later in the day. Overall they are not too concerned because, aside from feeling warm, she is not showing any feverish side affects.

On New Years Eve they took Emma off the paralytics and began weaning her sedation. She began waking up so we were able to do more stimulating things such as holding her hand, talking to her, playing with her hair, and giving her kisses. (I took the Baby New Year pictures before the surgery because I correctly assumed she wouldn't be looking too hot for a midnight photoshoot.) She would be awake for a few minutes, then sleep for an hour, repeating that pattern for the next five days. Today has been her most alert day since before the surgery. Today she was awake for about half of the day, taking naps intermittently. She was able to have Speech Therapy come by, but was asleep when Music Therapy came in. 

They have been doing chest X-rays every morning since the surgery and noticed that the rib fractures that Emma had in September finally finished healing, so they were able to begin percussions on her. This involves taking a foam cup instrument and hitting her on the chest with it. It sounds loud, but the respiratory therapist hit me with it on the arm a few times and I was surprised how light it felt. I'm not sure how such a soft tool helps bring up secretions from the bronchi and lungs, but it works well and they suction her trach after a minute or two of percussions. Emma actually seems to like it a lot and does not make sad faces during those treatments. Sometimes she will even sleep through it. Her PEEP (ventilator pressure) has been turned down from 18 to 16 so far. On Monday Emma has another surgery scheduled for a tracheostomy revision, so they will keep her PEEP at 16 until after that. Dr. Green is hoping we can eventually get it weaned down to 8 or 10.