Ronald McDonald House Charities

   At the end of November 2017 when we started talking about possibly going to Michigan for Emma's airway surgery, Michael was nervous about the procedure while I had a million unrelated questions. I wanted to know where we would stay, who would take care of our dog, how we would pay the bills that I normally write checks for if we weren't getting our mail there, and would I have enough time off from work available? These worries were solved to our relief when Michael's mom drove down to get our dog and take care of her at their house. There is this new thing called The Internet, so that's how we are able to pay those bills, and yes, I had enough time off available from work for at least 3 months. When I was trying to figure these things out in November I hoped that there would be a Ronald McDonald House close to the hospital in Ann Arbor, just like there is one next to Arnold Palmer in Orlando. One night I looked that up and -hooray- there is!

   They actually have two houses. The Mott House is inside of the Mott Children's Hospital, on the same floor as the PICU and PCTU (Pediatric Intensive Care Unit and Pediatric Cardio Thoracic Unit). It has a couple washers and dryers, a living room and kitchen combo, and 12 small bedrooms, each with one twin bed, a recliner chair, and a bathroom. The main house is across the street and has 29 bigger rooms (some with two twin beds, some with three). According to the website it said that the patient's case worker is the one who arranges where the family stays, so the next morning and for the rest of the time before we flew up to Michigan in December, we kept calling our case worker in Orlando to make sure we would be able to stay there while in Ann Arbor. If not, I heard that sometimes hospitals will give hotel vouchers, but I did not want to have to pay to stay in a hotel for 8+ weeks. Luckily it all worked out, as everything usually does.

   We checked into the Mott House about an hour after we arrived on December 11th and got our kisses from baby Emma. We got a room key (like a hotel key card) that got us into the Mott House and our specific room. The first night we both fell asleep in the small bed, but when I woke up in the middle of the night I saw Michael sleeping in the chair, and when I woke up in the morning he was on the floor! Here are pictures from the Mott House:

    We were very excited when we were told five days later that they had a room available across the street in the main house, because then we both got our own bed. (One of the nurses made a call to check for availability after we joked/ complained about the tight quarters at the Mott House.) I quickly suggested that we scoot the beds together, since the office manager said that was allowed, but Michael enjoyed having his own space. So we slept like married couples did back in the 1950's, at least how they did on TV!! One of my favorite parts about living in the main house is that we get a mailbox, so we were able to give out the address and have received several wonderful letters and presents between Christmas and Valentine's Day.

View of the Main House from Emma's first hospital room.

   The kitchen in the main house is very large and has 4 stoves, 4 microwaves, 6 fridge/freezers, and a big pantry with lockers. Each room gets a locker for dry goods. We keep bagels and soup in there, but lately have been keeping most of our food in Emma's hospital room since that is where we are for a majority of the day between 10am and 10pm. Over the weekend one of Emma's primary nurses even bought us groceries. There sure is something to say about Northern Hospitality!! There is a calendar on the bulletin board above the mail slots at the Ronald McDonald House that tells everyone who (which organization volunteers) will be at the house to make dinner for everyone, along with what kinds of food will be provided. Occasionally it gets cancelled without much notice if there is a lot of snow, so everyone has to eat macaroni and cheese or cereal or anything else they happened to get themselves from the grocery store. Thursdays are always pizza days because it is provided for free in the Conference Room on the top floor of the Children's Hospital, courtesy of Delta Airlines.

   One week towards the beginning of our time here, two employees from a yoga studio made dinner and then provided a complimentary yoga session. I was the only one that attended, but I enjoyed it. I was hopeful when I asked if they did free yoga every Monday, but unfortunately they said no. We have been in Michigan for two months now and they have not come back yet. Last week the office put a sign out saying they were doing an errand run to Walmart for two hours and they could take six people, so Michael and I signed up. When the afternoon of the errand came around, we were the only ones who signed up! So the super nice volunteer driver said she would take us anywhere we wanted to go. We were able to go to Target and JoAnn Fabrics instead, and that was great because usually we have to pay for an Uber if we need to get groceries since there is not a grocery store within walking distance. The closest option is CVS and Walgreens in Downtown Ann Arbor, which we do walk to often.

   Unrelated to the Ronald McDonald House, I love that on Mondays in the Mott Children's Hospital, a lady from The Giving Library comes around with a cart of books and we get to pick one out for Emma to keep. Sometimes we miss her and they pick a book for us, but the past four weeks we have been there. The recent books we got were Miss Maple's Seeds by Eliza Wheeler, The Day the Crayons Quit by Drew Daywalt, You're All Kinds of Wonderful by Nancy Tillman, and a few weeks ago Michael picked a big book that has six stories in it including The Wizard of Oz, which is obviously the most important one. ;)

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.