On our way home

So, we are on our way home. She still had about 30ml out of the drain in he back so we left it in and I'll take it out on Saturday. Avery is very self conscious about people seeing the drain.

It's really hard to update this on an iPhone! We are headed to Omaha today and then on to Denver tomorrow. Avery has 2 birthday parties to make it to this weekend.

The people at the RMcD house were really nice. The director and the house director were great. Avery loved Laurie, the house director and Laurie was great with Avery. Gave her little jobs and also homework! A loved it all. We are anxious to head back next spring/summer for the revision, or at least to stay at the house!

Chat later!

Avery;s drainage from the drain in her back put out 30ml in 24 hrs. We texted Dr. Siebert tonight and he said he would take out the drain tomorrow. We asked him if he was OK with us hitting the road once he pulled the drain via text. We didn't hear back in about 15 minutes so we texted back and told him if he wanted us to stay, we could and immediately we heard back from him saying he'll take the drain out Thursday to hopefully avoid any probelms. While we absolutely want to do what is best for Avery but it is kind of a bummer that we don't get to leave tomorrow. 

Don't know what we are doing tomorrow. Maybe get the A-meister a hair cut and pick up some birthday gifts for the two birthday parties Avery was invited to this weekend.

KU basketball starts in a couple minutes.

oh yeah!

On Sunday we also went to a magic show for kids on campus. The girls laughed and giggled! It was so much fun watching them together. We also made a target run to get hangers, kitchen scissors and a griddle as gifts for the house, bc we had no hangers when we got here, we needed a griddle for pancakes and grilled cheese and we also needed scissors. We got some silly looks but hey, we discovered a need!

The people who work here at the RMcD house are so nice. This community seems to be so generous. I wonder if it's like this in Denver. Avery has gotten so many toys and today she helped the House director sort through old games. There is a little girl who is 2 whose brother is at the childrens hospital that hangs out here at the house. Avery really likes to play with her. She looks just like one of my friend's 2 year old.

pics

I am trying to figure out the best way to post pics so they are coming...at some point. :)

Tuesday

Busy busy weekend. We went out to dinner Saturday night with my uncle, Rob and his wife Jeanne who drove over from Jackson, WI which is north of Milwaukee. It was great to see them and Avery just adores them! Plus, Paige and my mom haven't seen them since April, so that was nice too. We went to a nice little Italian restaurant. The food was yummy. I have a funny story to tell but you have to be careful what you say to the girls b/c they may get very embaassed. The girls needed to go to the restroom and it was a small restaurant and there was the 2 of them so I sent them on their own. About 5 minutes later we hear SCREAMING from the bathroom. The whole restaurant heard it and another couple got up and ran over at the same time Greg and I did. I had a vision of something really bad that happened to Avery's surgical site, blood everywhere, large hematoma, something. When we got back there, a woman who worked there opened the door. Both girls were in tears and hysterical because they couldn't get the door open and they thought they were going to be locked in the restroom forever. The door wasn't locked, they just didn't turn it far enuf to get it open. Imagine a clock face, door closed handle at 9 o'clock, to open it, the handle had to got to about 5 o'clock. Big hugs solved all and miraculously, the server brought over pizza dough for them to play with.

Sunday we had breakfast at IHOP and said farewell to Rob and Jeanne. Then, we went the Capital b/c the girls really wanted to see it and go on the tour. After the tour, (Avery adopted the nice gal giving the tour) we went to a restaurant called Ella's Diner. They have a carousel in the warm months but that is closed now. The atmosphere is very kid oriented. After dinner, we took Buggy and Paige to the airport. There were tears and it was hard to say goodbye to the Paigey-poo. Avery fell asleep at 730 that night.

Monday we hung around the house. Greg and I both got a work out in and then we went to Avery's follow up appt with Dr. Siebert. He was running way behind so we waited an hr in the waiting room. But, as luck would have it, a woman came up to us and introduced herself. She asked if Avery had PRS and had the free-flap microvascular surgery.  She told us she had the surgery about 2 years ago. She is 35 years old and noticed changes in her face at age 7 and progression stopped at age 15. I do have to say, she looks pretty darn good! She is having a revision tomorrow with Dr. Siebert. She comes to WI from Israel!  It took us about 1.5 hrs to get all the bolsters and sutures out. There was lots of screaming and negotiating. "Just a minute", "I want to look again", "I want to choose". OMG! I told her that Dr. Siebert was nicer than me as I was ready to papoose her! I should've given her some Ativan! Why didn't I think of that yesterday?!?! She did not however, get the drain out of her back. Still draining too much. So we are still in Madison. I am going to text him the 24hr output tonight at about 6 and he will let us know if she gets it removed Wed or Thurs. Hopefully Wed so we can get back in time for a birthday party on Friday but we'll do whatever he thinks is best.

We went to dinner at a fancy pants restaurant right across from the Capital last night. Avery had her best adult like behavior. We happened to be sitting right next to a few surgeons. Greg and I surmise that they were transplant surgeons, recruiting. They spoke of hearts, livers, kidneys and a transplant service line. I felt like I was at work!!

We plan to go to the Children's museum today and I don't know our plans for tonight. Avery is digging not having all the sutures in and there is no more syrup stuck on the one by her mouth.

Chat later!

Saturday

Hello! Sorry, yesterday was very busy with the discharge and Paige and Buggy coming to town.

They landed a little early but we got them picked up and back to the RMcD house. We hung around and Avery showed Paige around the house. We made an outing to the State Capitol building. We thought everyone could learn a little. After driving around trying to find a close parking spot so Avery wouldn't walk in the cold. All the close spots were only one hour parking and our tour of the Capital would take longer than one hour. Greg ultimately dropped us off close to the capital and then he parked the car. Buggy, Paige, Avery and I waited about 5 minutes for Greg and about 2 minutes after Greg got there, the fire alarm went off and we had to evacaute! So, we got to see the rotunda of the Wisconsin State Capital Building.

After that excursion, we took Buggy and Paige to the hospital to show them where Avery stayed. We saw Meagan the Child Life Specialist and Avery gave her a big hug. Paige and Buggy saw the floor where Avery stayed but no rooms b/c all the rooms were full! Paige also got to see the Gift shop.

We went out to dinner at the Great Dane Brewery and I don't even remember the last time the girls got along like they did yesterday!
Today we chilled in the morning and the girls played in the playroom and art room. Eventually we all got ready and went to the Vilas Zoo. It's a free Zoo here in Madison. It's smaller than the Denver zoo but quite nice. We saw a polar bear, grizzley bear, a buffalo and a badger. They had several indoor exhibits which I guess you must need if you're in Wisconsin!!! Lots of people asked Avery today what she had on her face and this made her very uncomfortable and sad. :( Overall, we had fun though!

We are going out to dinner with my uncle who came into Madison from Jackson, WI which is outside of Milwaukee.  Avery LOVES Uncle Rob!!

Thursday - poat op day 3

Wow! What a great day! Avery was awake and playful and laughing and smiling! Her left eye opened more today. She took another shower and got dressed in her own clothes. Her IV didn't work anymore so it came out. We walked down to the cafeteria for lunch and then again for ice cream. There is a place called Babcock Hall (I'm pretty sure) that makes its own ice cream here at UW and they sell it 24hrs a day at the hospital. A few people recommended it including my pen pal so we had to go check it out.

They were still doing every 2 hr pulse checks and every 4 hrs vitals. Dr. Siebert came to see us after his case today and chatted with us for 30-45 minutes! What a great guy! Her eye is a little cloudy which he says is swelling and it will go away for sure by Monday. She has a tiny skin tear from tape that is being covered with triple antibiotic ointment, her steristrips along her back incision are intact and the drain continues to drain and will hopefully come out Monday. The drainage is still relatively minimal and is less bloody. A is being discharged tomorrow!

Paige and my mom come up tomorrow and their plane lands around 130p I think. Avery is super excited to see her sister. Hopefully the weather is a little nicer this weekend when they are in town so we can wander around the city a bit.

Not a lot to report really.  She is doing very well and is pretty much back to herself today. She still doesn't like to see herself but she isn't having the reaction of crying and running away when she does. Greg is staying at the hospital tonight and I get to sleep in the tempurpedic bed at the RMcD house. Here's to a great night's sleep!

2nd day post-op - Wednesday

Sorry it's so late today. Been busy bc little A has been much more awake today. Just a couple short naps. She slept much better last night, only really waking for once to go potty. We were woken up by the plastics team at 0715. 

The drain in her head was removed today as was the bandage from her back. She screamed loudly things like "I do not like this at all!" but she held very very still. The drain from her back incision stays in until next Monday when they will remove the bolsters/sutures from her face. She got moved to the regular floor  (out of the ICU) this evening and it was very scary for her. I think she is just being bombarded with changes and she was comfortable in the other room. Now we are settled and she seems a little better. She was so unhappy moving i told her I would stay with her again instead of Daddy. Dad's getting great ZZZZZZZZs. Oh well, I'm a sucker. ;) Anything for my Little A.

She was able to take a shower this afternoon which made her feels so much better. Got the betadine and blood out of her hair and they are washing her jammies here at the hospital so she'll have nice clean home PJs to sleep in again tonight.

It snowed today and was dreary. The Ronald McDonald House has a membership for the YMCA for guests. Both Greg and I were able to get away for a bit today.  While I was gone, Daddy was able to get A to smile and laugh.  That was great to see and hear.

We don't know yet if we're out of the hospital tomorrow or Friday. Dr. Siebert told us that she's "home free" bc he has never lost a graft after 48hrs post-op. Cross our fingers. She is no longer being monitored, no IV medications and they are doing every 2 hour pulse checks. I asked him what we frequently ask of us and our patients "what are we doing here that I can't do at the RMcD House?" Dr. Siebert anticipates that we'll wean to every 4 hour checks tomorrow morning and he leaves to go to NYC tomorrow night. I don't want to rush things but...... We'll see if we go "home" tomorrow evening or Friday.

Overall, she is doing leaps and bounds better than yesterday. Yea! She's such a sweetie pie.

1st night out

Little A had a "good night" as told by the nurses and docs. To me, she was a bit restless but pretty good. She had a mild reaction to the morphine so she got tylenol twice and benadryl once. IV benadryl knocks kids OUT, she fell right asleep after being rally agitated at 3am. They did pulse checks of the artery and vein at the anastomosis site (right in front of her ear) every hour. Those dopplers are really loud at 11p, 12a, 1a, 2a, you get the picture.

This morning she got the foley catheter out and has walked to the potty twice. She was sure she couldn't and that her legs didn't work. But, to her surprise, she did it! She looked at herself in the mirror and was upset, understandabley so. She has 12 little popcorn sutures (little yellow gauzes) along her eyebrow, down her nose and chin and along her jawline. The one right by her eye and nose are annoying her. She doesn't have any sensation on the left side of her face due to the graft and it must tingle around her eye b/c she keeps complaining about her eye "hurting."

They are still doing hourly pulse checks and may space out to every 2 hrs this evening. Most likely she will hang in the PICU overnight only because of the frequent pulse checks. Hopefully she'll be able to eat solids this eve, though she isn't too terrible interested right now. They also like to keep the room temp at 74 degrees to make sure she stays more vasodilated to keep good flow through the vessels. Poor little punkin also has a couple pressure sores on her heels that are bothering her. :(

Its raining and dreary outside today. Good day to hang out inside and watch Rocky.

Recovery

Avery got out of the OR at about 315p which was about 7 1/2 hours. She did well. She looks pretty beat up but looks "good". We were able to see other pics of kids post-op so we were a bit prepared. She has a good pulse at the graft site and that's what we want. She doesn't seem to be in a lot of pain but the little yellow bolsters (popcorn sutures) are already bugging her and she's a little cranky pants about it. She also doesn't get to drink or eat tonight just in case she has to go back to the OR emergently. She is really thirsty. She got about an 1/8th of a dose of morphine which gave her a rash but she is sleeping quietly now.

She is in the PICU now because they are checking pulses every 1 hr and she should move to the floor tomorrow when they space pulse checks out to every 2-4 hrs. The drain from her face should come out on Wednesday and the one on her back will stay in until Monday next week when the sutures come out.  Hopefully she will only be in the hospital for 4-5 days. It would be nice if she's out when Paige and Buggy get here.

Greg and I got the chance to educate the PICU fellow and resident on PRS. Of course, they had never heard of it.

Now, off to relax and watch the Eagles/Bears game. More later!

Thoughts

So, this is really hard to deal with because there just isn't much info on this crazy "syndrome". I have come to believe that everything out there on the net is hardly correct. It doesn't primarily affect girls necessarily, or kids aged 5-15 and doesn't seem to burn out. I am part of a yahoo group and I think there are more adults than parents of kids. Also, it does seem to recur, possibly in pregnancy. And, it begins at later stages in life too, like pregnancy or even in people's 40s, 50s, and even 60s. Bottom line, it's so rare, no one really knows much of anything about it.

Day of surgery

Well, 5am is early. We got up early this morning to get ready to go to the hospital. i have been sleeping with little A and she felt hot this morning. That worried me a bit. we came on in and she had a low grade fever of 99.7 when the nursing assistant took her temp. Hmmm. She checked with the nurse who paged the anesthesiologist and Dr. Siebert, the surgeon. Dr. Sasse ("Do you think he's sassy?" asked Avery b/c I call her sassy all the time) the anesthesiologist listened to her and her lungs are clear though she has a cough. A cough that seems post-nasal but has increased over the past several days. It was decided to go ahead with the surgery. I walked back to the OR with her after she got some Versed. Heh heh, she was a little drunk. She really wanted to get up and walk. :) They only let one parent go into the OR with the kids. Daddy gave her a big hug and we rolled away. We got into the OR (which is bigger than our CVORs) and she reluctantly climbed on the bed. She was scared. She chose bubble gum gas to be put to sleep with. She fell asleep in about 3-4 minutes.

At about 9 am we got an update - all electronic through a paging system, that they had started the procedure and Avery was doing fine. Then again at 10am "Still working. Avery is doing fine." Greg and I are just hanging out. Hey, only 6 hours to go! Big Sigh.

Well, we made it to Madison. A long drive (>1000 miles) over two days and lots of movies! Avery did well until about the last two hours of each day then she was antsy and wanted to know when we would be there. We went to dinner at a Mexican restaurant last night and ate at the bar. She thought that was cool because she "has never eaten up here before."

The RMcD house is nice. Tempurpedic beds...nice. She loves the art room and play room. The kitchen is stocked and we were able to make pancakes this am just like at home. Greg and I both went for runs (which is so much easier at sea level, btw) while she was happy as a lark playing. She is excited to have speghetti O's for lunch!

Uh oh, Avery doesn't want me to tell anyone. Gotta sign off for now.

We spent the night in Omaha and Avery got to swim an have waffles! We are getting ready to hop back in the car to drive to Madison. Avery gets very bored in the car. How can you blame her? Too bad she doesn't nap anymore, that would pass the time!

So,we are on our way. Avery is scared and excited. It goes back and forth. She definately likes all the gifts she's been getting. Thanks everyone for the well wishes, kind words and gifts for Avery.

Address

American Family Children's Hospital
1675 Highland Ave.
Madison, WI 53792
General Information:
(608) 890-KIDS (5437)


Ronald McDonald House Charities of Madison, Inc.
2716 Marshall Court
Madison, WI  53705
Phone: 608-232-4660
Fax: 608-232-4670
http://www.rmhcmadison.org/

How it all started

It all started about a year ago. I was playing with Avery in my arms at my mom's house on a Thursday night. I asked to blow up her cheeks or as she likes to call it "make a bubble." Well, it was bigger on the right side than the left. My mind went to facial nerve damage b/c that's CNVII and we have to learn that in school, you know, Bell's Palsey or something. Yikes! nerve damage? That's no good! I called the doc the next day and I couldn't get in till the next Wed "because it's been going a while." Well, her crooked grin had been "going on awhile", maybe 6 months? A year? We had to look back at pictures but everyone in the family knew Little A had a cute crooked grin - it made her, her. While I thought this was an under reaction to a possible facial nerve injury or other neurological problem, I tried to be the mom and not the pediatric nurse practitioner.

We saw the pediatrician on Wednesday the following week. She looked at her and could tell what we were talking about. She brought in a colleague to look at Avery, to which Avery responded, "Stop looking at my face." They noted "atrophy" specifically but didn't know what to do with that information. A concern for them was a brain tumor so we were scheduled for an MRI at Children's.  That was scheduled for two days later, on that Friday. Her MRI was completed and we heard before the weekend that it was negative - no tumors or anything else that wasn't supposed to be there. The pedi then wanted Avery to see a Neurologist which was also approved to be done at Childrens. I called to schedule the appt. With the help of an NP in Neuro that one of my friends knows and me dropping the fact that I work there, we were able to get into the clinic the following Wednesday. While the weekend seemed like eternity, I knew we were SO lucky to get in that quickly! A wait for a neuro appt can be weeks to months. Over the weekend Greg and I googled hemifacial atrophy which just kept leading us back to this super rare autoimmune disease called Parry Romberg Syndrome. It couldn't be that though, because there's only like 600 people in the world with that so there is NO WAY my kid could have it. That is just too far out there. NO WAY.

The following Wednesday morning we took Avery to see Dr. Moe. He is a super Neurologist who has been around forever. His PA did a routine neuro exam that was normal. Then he came in and looked at her while she was on my lap. He said "hemifacial atrophy" and cocked his head a little to the side and said "parry romberg". He told us a little bit about it, of which I can't remember most of what he said and recommended we see rheumatology and genetics.

With this overwhelming news, Greg took Avery back to school and he went back to work. Another friend of mine knows the rheumatologist at Children's well, so we traipsed over to her clinic for a quick sidebar. She looked at all of the pictures I had gathered and she took one look and confirmed it. She told me about a geneticist at Children's who had been doing some research on PRS. Again, we were able to get in to see her in just a couple weeks. In the meantime, all my colleagues and friends racked their brains and asked friends of friends about this crazy rare disorder.

When we saw the geneticist, they took a thorough history and then we chatted for a long time. We were told that there is nothing you can do about this disorder. It affects girls more than boy, ages 5-10, it is autoimmune. It will burn out in 5-10 years and at that time, maybe we can do something. We received very vague information and really nothing more than we did when we scoured the internet. Not encouraging feedback at all.

Also online, it is described as "linear scleroderma" which is specifically on the face. It's only PRS if it's on the face. With the info, two people mentioned to me to see a Dermatologist. This too was approved by Kaiser (Phew! As much as I bitch about KP from a provider standpoint, they have been nothing but great with this adventure).  We saw a great Derm guy at Childrens that had worked with us with the transplant team. He had not worked with a PRS pt but did know of a treatment that there had been a little experience with that was different than methotrexate. It was UVA1 light therapy. And, hey! Some kind, well to do family, had donated a UVA1 lamp to the University of Colorado b/c their child needed the therapy. Not for PRS but for something else. We were so lucky, well, if you can call it that. The plan was to do oral steroids and light therapy. Light therapy 4 days a week for 6 weeks at University hospital in Aurora. And a 3 month taper of prednisone.

This was the plan a week before we were off to Mexico with our close friends the Stutz's for one week to get some sun. We headed to Mexico and didn't use sunscreen on the left side of her face to get a jump on the treatment.  Haha, just kidding we put sunscreen on her.  When we came back, we dove head first with the help of grandmas and grandpas taking Little A to light therapy 4 times a week 35-40 minutes away from where we all live. And we proceeded through the winter with this plan.  She was weaned off of the steroids in ~Feb and we followed up with the Dermatologist (who conveniently moved over to the KP office right next to our house) in March. All looked good. No progression! Yippee! It worked. The Derm didn't think it would progress, it had stopped!.

Or so we thought. Greg continued to go out on the Internet to do searches on PRS. In April, he ran across the CNN article on Christine Honeycutt in Charlotte, NC. This was fascinating and reassuring. It was a surgery which, for all practical purposes, STOPS progression of PRS and repairs the damage done. Greg called Christine's mom and chatted for a good amount of time. I then talked to her that night for 2 hours. At that time, Avery hadn't progressed further, but to have this in our back pocket was reassuring. And they are in Charlotte, which is where we going to have a 4 hour layover on the way to the my brother's wedding in the Dominican Republic just a few weeks later. We met Vicki in the airport in Charlotte and she showed us pictures and talked to us about this crazy disorder. But, I still wasn't sold. I didn't want my kid to have such an invasive surgery if it had stopped. She was adorable just the she was. She was my baby with a crooked smile.

In June, we noticed more progression. We had briefly consulted with Dr. Siebert in April/May and sent him pictures for him to review. When we took pics the weekend of July 4th, we could tell there was a difference. We sent them to Dr. Siebert who also noted worsening. I was now convinced that this is what we had to do. We set up an appt to meet Dr. Siebert (after multiple telephone calls) in early September. We flew up to Wisconsin to meet with him. We talked about the surgery more indepth and we made an appointment for surgery.

And, that, brings us to today.....

More on PRS

 - http://therombergsconnection.com/
 - http://www.prsresource.com/
 - http://abcnews.go.com/Health/surgery-saves-face-girl-parry-romberg-syndrome/story?id=13021455 - we got to meet Christine's mom and this is how we found out about the surgeon who is doing Avery's surgery
 - http://www.faces-cranio.org/Disord/parryromberg.htm

Avery's Adventure

Hi everyone. I have been asked by several people if I am going to start a blog to keep friends and family apprised of Avery's upcoming surgery and trip to Madison WI. Well, here it is. I have never done one of these before so I can't guarantee how much will be here.

So, Avery has a really rare condition called Parry Romberg syndrome. It is a condition that causes the tissue and bones in her face to "atrophy" and waste away. We are headed to Madison WI in about 10 days to have surgery to repair the damage already done and hopefully stop all further progression.

Greg, Avery and I are driving to Madison. We leaving on Fri Nov 4th and driving about 1/2 way to Omaha and then on to Madison the following day. Surgery is scheduled for Mon 11/7. It is a 6-8 hr surgery and she will be in the PICU for at least the first night. We anticipate an ~5 day stay in the hospital. Then she will have a follow up appt at 7 days post-op. we are unsure how long we will be in WI. Paige is staying home with family & the dogs while we are up there. She does get to come visit Avery the weekend of Nov 11th with Buggy (my mom).

That's the quickie scoop for now since I'm just trying this out and getting it started.

DD