Shizzy Cardiac Echo Results

That's right, shizzy. People who know me know that I have my own vocabulary at times. Can I point out the difference between this picture and the one on the top of this blog? Kate has become a totally different person since we started this journey. I can't believe we have been doing this for so long, and yet I can barely remember life without Marfans.

 I honestly thought we would walk in there and they would say that Kate had grown, she is now 5 6", and that her aorta had stayed the same size, or by some miracle had a smaller Z score because her body was now so much bigger and it had only grown a little. This happened last year, so I wasn't completely bonkers for thinking this.

Unfortunately, I was way off. A little lesson first. A Z score is basically measuring the dilation of the aorta. Her last Z score was 3.4 which was one year ago almost exactly. Her new Z score is 3.8. A change of .4 in one year. Definitely not the results we wanted. We asked a few questions and the doctor said at a Z score of 5, surgery is indicated. So I said we have until she reaches 5, and he shook his head and said no, we start talking surgery at a Z score of 4.5.

When Kate was first diagnosed, we knew this surgery was a possibility, but wasn't a for sure thing. And I had hoped IF it had to happen, it would happen later in life. Now we are just trying to postpone the inevitable. Very hard to hear, very hard to keep it together with Kate sitting right there. She was asked if she understood and she said that she did.

To slow down the progression we are going to add a medication to her current medication that lowers her blood pressure. One side effect is depression. That is the only one that I will really be aware of. Kate is such a happy and upbeat kid, I'd hate anything to change that. This new medication will help lower her heart rate, but we need to lower it only enough so she is still within normal heart rate parameters. She is wearing a heart monitor for 24 hours to get a baseline heart rate, then she will start a low dose of the new medication. We then go back in a few weeks and recheck her heart rate and increase medication as needed. This will likely happen 3 or 4 times until we get the correct dosage. Her positive on this, at least she doesn't have to wear the new uncomfortable brace for 24 hours.

We asked about her PE class at school and her doctor is convinced that the benefits outweigh the risks. We then had a several minute conversation about what Kate can and cannot do again. I think she gets it, but it's hard when all of your friends take off running and you cannot. So, that is kind of stinks. Least of my worries at the moment though.

Here's what is coming up. X-rays and doctor visit to see if new brace is working on November 3rd. Appointment with her cardiologist on November 5th to check heart rate. A visit to her eye doctor on November 19th to check on her lenses. We might have to throw in a weekend at Disneyland. Serious group therapy right there. Prayers are always welcome.

EKG and Cardiac Echo

 Kate and I went to her yearly EKG and cardiac echo on wednesday. The EKG is a breeze, in and out in 5 minutes complete with sticky sticker removals.

On the way into the hospital this time Kate and I were talking about how we have driven this road so many times it feels like we could drive with our eyes closed. Well, I could. For some reason the accident came up and I retold her the story of where I parked that night and how I ran across the street and burst into the ER and started banging on the window and yelling that my daughter was here somewhere.  She said they probably thought I was crazy. Haha. I told her that's probably why they gave me a person who didn't leave my side for the first 3 hours.

 But then Kate told me some things about the accident I didn't know. She said she fell asleep at the accident site for part of the time, that she didn't remember talking to me on the phone there at all. She also said that she woke up in the ambulance and was really fighting the paramedics when they tried to start an IV. She hates needles.

She remembers me coming into the ER room she was in, but she thinks she slept for a time because she didn't remember leaving the ambulance. None of these memories seem to her upset her, unless she tells me about the needles. Amazingly brave kiddo.

Back to our tests, we sat in the second waiting room for a while reading and playing games on our phones together. It sobers you up fast seeing all of the other children and parents in there. There's that story or saying about if everyone put all of their challenges on a table, people would still reach down and take their own troubles back. I still think that is true. Don't get me wrong, I'd give anything to take Marfans away from Kate. I still find myself going through those stages of grief, I mostly live in bargaining. Saying in my head and sometimes aloud all of the things I would give up or go through for Kate to not have these health issues. God hasn't taken me up on any of them yet, but I keep trying. 

 Time for the echo exam finally. Tangled was playing, there's always a movie on which is nice as the test took around 40 minutes. I used to try and figure out what I am looking at, but I only see obvious things and I convince myself I see things that likely don't exist. It is pretty cool to see all four chambers and valves seemingly doing their jobs perfectly.

The test took long enough that I thought Kate had dozed off. You can barely see in the last picture, but her eyes were closed. I asked her about it after the test and she said she nearly fell asleep but she thought it might change her heart rate and wasn't sure she should let herself. Smarty pants kid. We left the test absolutely starving so we grabbed a pizza on the way home.

New Back Brace

 Kate and I went in on Monday this week to get her new brace fitted. It doesn't seem to go as high as the last one, but it goes down over both hips where the last one only went down one side. Kate has two different curves to her spine, an S shape. Her old brace put pressure on both curves going in opposite directions. The new brace is only putting pressure on the lower, larger curve with the hope that the smaller curve above will straighten some by default.

Because it's a new brace with new pressure points, Kate gets to start the brace wearing adventure all over again. It hurts to wear so she is wearing it mostly at night at first, oblivion is nice if you can get away with it, then she will start wearing during the day slowly. Eventually it will be on for 20 hours a day.

She ended up choosing the same pattern for this brace as her last one. She is actually becoming a little fashionista as of late and I think this design is as close to white without being boring as they come. The straps are black this time which might put a damper on some outfits.

As per usual, Kate is a trooper. She never ceases to say something positive about her situation. Her brace gives her fantastic posture, or so she tells me. Love this girl.

Back Update

Getting measured

 For the last couple of months Kate has been all too happy to take her back brace off any chance she could. It is supposed to be worn 20 hours a day. There's always an excuse, it's too hot being the most popular. I noticed that it isn't fitting like it used to and took her into to checked. Sure enough she has grown almost 6 inches and her back brace isn't sitting on her hips like it should be.

On the bright side, because that's what we look for, the technician who measured her back thinks that the curvature of her spine hasn't changed for the worse and possibly is even improved. Only x-rays will be able to determine if he is correct.

The plan is to get a new brace, which takes about three weeks to make, then wear it for a few weeks. After that we go back into see her doctor at Rady Children's and get more x-rays. End of plan there. Everything hinges on that.

Getting fitted for a new brace is quite the party. First she gets two "dresses" made from stretchy material. They then stick a long rubber looking ruler between the two "dresses" so after the cast is made it can be cut off and leave a dress on Kate afterwards. Then he measured and drew lines all over Kate's outer dress and proceeded to apply casting material, the same used when you break a bone. Lots of experience with this stuff. Kate is a giggly girl and had a hard time holding still during the casting process. This girl makes me laugh. When he was cutting off the cast, the sound off the cast getting cut off sobered her up pretty quickly. She looked at me and declared that we are stopping and getting a treat on the way home. Something tangy, which we did. Now we wait.

One other thing, Kate just started middle school and we are all in a mess trying to get her out of going to PE. She can do a few things, but so very few it is not worth her dressing. Personal opinion. Also, she is a stubborn thing. She knows she shouldn't do certain activities, but she's still a kid and likes to prove that she can still do things even if she shouldn't. I cannot imagine the peer pressure in middle school to fit in, and how is she to do that in a PE class where she will be automatically singled out? Several emails and phone calls between the school and a district representative and we are still waiting.

Knowing that Kate cannot do some of the activities that other kids participate in, we gave her a week of golf lessons that she quite enjoyed. She is also taking regular archery classes. Of course the classes are sometimes held in a shop that is plastered with pictures of all the dead animals the people there have shot with arrows. Kate is very much not a fan. The first time we went, she gestured with her head to a wall of photo's and rolled her eyes. Man I love that kid.  

On goes the casting material

Casting

Life Update

 A few weeks ago I took the kids to Utah for South Weber Days, it's a family tradition. On the plane Kate was sitting next to me, she was tired and had her head on my shoulder and her arm draped over my lap. It was chilly, it always is on airplanes, and Kate was sharing my jacket as a blanket. I looked down at her long, graceful arm, letting my eyes follow to her hand. Longest fingers ever. Her wrists are even long. I don't know if others see it, if they notice her long limbs. I know they notice her height, people comment on it all of the time. Especially when they find out she is only 11. She will be 12 in October. But still. It used to break my heart a little when someone commented on how tall she is, but she is so happy to be tall at the moment, I try to take it in stride. I pray her growing with slow down, even stop. I don't tell her that I pray for this, but I do. Constantly. Not only so she doesn't have these things that might set her apart from others, but so her heart and back can remain steady, no more enlarging, no more scoliosis questions. 

I have this little thing for books, some people might know this about me. One thing I like to do in Utah is take my kids to a bunch of second hand book stores and see what treasures we will find. This trip I took my two nieces with me. The blond is the same grade in school as Kate. The brunette is a couple years younger. But the difference in height is staggering. Granted, my side of the family was not gifted with height in any way, shape, or form. I try to convince myself that the difference is really because I come from a family who is vertically challenged.

Kate is as enthusiastic as ever with life. She has been over a year not eating any meat other than fish. She did go full vegetarian for about 6 months, but the call of fish was too strong and she went back to being a fish girl.  She and Noah have had some animated conversations about animal cruelty and the health benefits of not eating meat. She darn near went total vegan, but I told her if she wanted to do that I would take her shopping, but she would be in charge of all of her meals and would have to chart how she was meeting her nutritional standards. Not sure if that felt like too much work, but she decided against it. I have to give her props, she makes up her mind about something and sticks to it. Amazing little kiddo.

Beautiful Teeth, Tall Girl

I am completely and totally biased when I ask this, but have you ever seen such a beautiful smile?  This is actually from December of last year, 2014.  On the bright side, I haven't had a lot to post the last six or so months. That is usually a good sign. For the most part things have been remaining steady. But, it's now entering the fall and that is the time of year we go back to all of her doctors to make sure we are doing well.

Along with braces coming off, Kate has also surpassed both Abby and I and height. She is absolutely thrilled about this, and Abby cannot stand it. Haha. So, currently in order of height we are Noah, Kate, Marci, and then Abby. I guess you can see why she hates it.

Spring Break at Universal Studios CA

Passing Up Mom

Backs and Braces. Fractures and Casts

First Back X-ray

Lots to catch up on, first of all we had the 3 week x-ray of Kate's back to see if the brace is helping at all. This is with her wearing the brace. That is in this first picture. We were told that the brace was doing the job we wanted it to and to return again in 6 weeks or so and take an x-ray without the brace on and see how things look.

In the meantime, we have been getting used to wearing this brace. The bottom of it pokes out of her shirt, although luckily at this age, her friends are all really cool about it. Kate is a pretty remarkable girl, hopefully her contagious good spirits will carry through times when kids turn into dirt bags you want to add kids names to a hit list. Yup, that's a warning. I'll take you out, or some other nonsense, if you hurt my baby girl.

Just to make life a little more exciting, I got a call on my way into work that Kate had fallen at school and her wrist hurt, but she didn't think it was serious. Kate never cried or anything. I had Kate's dad look at it and he didn't think it looked swollen so he sent her off to a play date. I got home from work about 7 pm and looked at her wrist and instantly knew something was wrong. So, urgent care closed at 8 so we jumped in the car and made a mad dash. Sure enough, Kate had a fracture all the way through her radius right below her growth plate. They gave her a splint and orders to be seen at Rady Children's at the beginning of the next week. They asked if we had ever been there or know anything about the orthopedics there and Kate looked at me and rolled her eyes. She laughed when the doctor stepped out and said 'hello, like I live at Rady Children's'. This was Thursday night, October 30th. I took her trick or treating with her friends on Friday night.

Urgent Care

Kate already had an appointment on Tuesday the 4th for her back check up to do x-rays without her brace, so they just added the wrist for good measure. We took the x-ray we had taken at urgent care so she only had her spine x-rays done. She really didn't want a cast, but of course, a cast we got. She has a great sense of humor about it though, she had already had  red, blue, and green casts and all that was left was purple, pink and black. I refused to let her have black, no reason in particular. And Kate refuses to wear pink, I may have screwed up my girls in this regard. So, purple it is, and we decided that IF there's a next cast, we will start over at red again.

Her back pictures looked good and we go back in 4 months to do another x-ray. Until then, she wears the brace 20 hours a day. Honestly, sometimes her back hurts now when she's not in the brace so she usually wears it most of the time.

Her cast on her wrist will stay on until December 1st. Which isn't horrible, except she was supposed to go swimming with the otters this weekend. Argh, postponing that was a drag. The facility was very sweet about it though. Probably going in January. We are back to washing hair in the sink, wrapping her wrist in plastic for a bath, just all of it.

You can see Kate getting her cast on and getting her back pictures taken in the two pictures. By the time we got back to the car Kate was kind of sad, who can blame her, right?  The smile came when I told her we would ditch school the rest of the day and go to eat at a vegetarian Chinese food place she's been wanting to try. It wasn't bad, actually. And Kate cleared her plate of orange 'chicken' so I'd say it was a success.

 I took Kate and Noah to the park today and watched her climb all over with her back brace hanging out and her wrist in a cast, and a smile on her face. Sometimes I find myself thinking about her health, about my health, about how much I miss Abby, who is doing fantastically in France, and I find myself getting tears in my eyes, lump in my throat. I can turn it off pretty fast, lots of practice. I turned it off again today. The kids and I got in the car to come home and proceeded to drive into the garage. Until I realized I wasn't moving very fast and it dawned on me that I had boxes of books that are going to be picked up tomorrow by UPS going to a client. Holy freak. I got out and saw that four boxes were squeezed, squished, and totally stuck under the front of my vehicle. It was a laugh or cry moment. Noah and Kate got out and saw the disaster and looked at me, waiting to see the reaction. I looked at them both and just said 'bummer'. We eventually got a knife out and literally cut the boxes open and took out each book. Not one book was damaged, which was crazy, because the boxes were in unbelievable shape. As Noah helped me get new boxes and weigh the books again, we were talking about life and things we deal with, and he said that if someone were to look at my life, they would think it was kind of bad, not very fun. But, day to day, he thought we were doing pretty good and I seemed happy. That was good to hear. And it's the truth, moment to moment, day to day, life is great. 

 ***Have to add what happened last night, part of why life is really OK even when it's not. So when we were cutting the boxes open, laying on the driveway, heads under my vehicle, Noah and Kate were discussing the imminent damage were were going to find in box number 2 because it just looked gnarly, no way the books will be alright. They sounded so concerned, I just thought it was cute. I looked at them and said 'this is hilarious'. They both looked at me like I had gone totally bonkers, Haha. So I said, 'well, not right this minute, but someday we are going to be sitting around and it will be totally funny when we start talking about that time Mom ran over her books'.  Late last night I was sitting on the couch repairing some antique books, these came to me damaged, I swear, I had no hand in it. Anyway, Noah came up to me and started talking to me about the Captain America that was on the TV and then turns to me and says, 'remember that time you ran over all your books? That was hilarious'. See what I mean! How can life not be great with kids like mine. :)