On the Brightside

Kate had an appointment on Monday with her hips doctor, new X-rays were taken and lo and behold her hips look alright. The angle on the back film was making it look as though she had protrusio again. So that is a huge, huge, relief. But, the doctor did say that the lines between her femur head and pelvic socket weren't very thick, meaning it might need intervention sooner than he thought. Right now we are hoping for sometime in her 40's. That used to be 50's or 60's. You know what, I'll take it. With her aorta enlarged and her scoliosis so bad, I can put hips on the back burner for a while.

The rest of it...expander gets put back in her mouth next Tuesday. She see's her eye doctor at Shiley Eye Center next Thursday. This coming Friday she gets her new back brace to see if we get a bigger improvement on her scoliosis. X-rays for that in four weeks.

It's officially alright to tell me to hang in there, all that gooey crap. But please don't feel obligated, I don't actually want to hear it. I'm just saying I might not turn Hulk on you now as opposed to a few days ago. It's a start people.

Scoliosis and Worse

So yesterday started with being sad about Kate and her orthodontics appointment the day before but it picked up a little bit when we saw this beautiful rainbow on the way into her scoliosis appointment. But then we had to go inside, and killed the whole thing.

Kate has a new back brace and we went in to have X-rays with and without the brace on to see how it was working. She is an absolute fan of those little paper shorts she gets to wear every time we go to get X-rays. Luckily she got a gown to covers those beauties. This is a serious X-ray machine. We did shots with and without the back brace on. Then we went to our little room and waited.

Kate is reading constantly, but it's on her phone lately and drives me crazy. Why I think a book is better, I don't know. Weird Marci-ism I suppose. I brought Goose Girl by Shannon Hale to tempt her with and we got in three pages before they opened the door and came inside.

Dr Newton, two residents, and the nurse. Team effort apparently. He does a physical exam and has Kate bend over and measures her back while he tells me that her back looks really good on the outside for what is happening on the inside. I'm like, excuse me? Her back has gotten worse, like leaps and bounds worse.

Last March when she had her last X-rays taken, her top curve in her back measured 27 and lower curve 25 without her brace on. Now, her back is 36 and 37. That's 8 months to jump 10. She also grew almost 3 inches during that time.

We need to go back to Scope and get the brace fixed with some padding or something to get us a better result. With the brace on the number go from 36/37 to 32/32. It needs to be better than that.

So at this point I am having that inner panic thing going on, I really thought her back was at least holding steady, but now this. The two resident doctors are looking Kate over and playing with her brace and I am literally freaking out inside.

 My first reaction in a crisis is to make a plan and to figure out what the future possibilities are. So I asked Dr. Newton what our numbers have to be before the brace is no longer the best option, hate to throw the word surgery out around Kate, and he said 50. At our current rate, that could be around a year from now.

I'm letting that sink in and realize he's about to leave and I haven't asked him about Kate's left knee that she told me hurt her the day before. I know it's not his "area" but when I had his 2 minutes, I figured ask away. He had Kate lay down and put her leg through a series of range of motion tests and she did fine. Dr. Newton was mid sentence telling me it was probably nothing when he stops and says 'wait a minute' and takes the X-ray papers out of my hands and looks them over.

And this is the moment things fell apart for me. Recap, Kate has had bilateral hip surgery for protrusio, a condition where the femur heads start to push their way into the pelvic cavity. She had both femurs broken, turned, and bone taken off the sides to strengthen the inside of the pelvic floor to stop this from happening.

Left hip, you can see it on the X-ray, her femur head is pushing into her pelvic bone. You know that lump you get in your throat when you're about to burst out in tears but know you really can't? Hit me something fierce. A mix of fighting that lump and pure anger. The words going through my head are of the very angry, colorful variety and my thoughts were of my punching bag that I haven't used in forever.  Dr. Newton was telling me to follow up with Dr. Wenger and that we will see him again in 4 weeks and I am just nodding, digging my fingernails into my other hand just trying to hold it together.

We take our papers and go to check out and make our next appointments with Dr. Newton and Dr. Wenger. Kate is sitting on a chair and the lady checking us out took forever. My eyes are watering and I am struggling the whole stinking time. Of course it was taking forever. Finally, I got Kate and we started walking down the hall to the elevator. I asked Kate if she knew what Dr. Newton saw on the X-ray and she blurted it out like nothing, 'Oh my femur head is going into my pelvic bone again, on the bright side, if I have to have another surgery, at least I'll get out of going to school and gym class.' This kid.

At the end of the hall was a restroom so I left Kate outside the door with her book and I went in and let it go. I cried hard for about 2 minutes. Then I cleaned up and walked out like everything was just dandy. Kate was dandy. We were down the stairs and almost to the car when I burst into tears again, saying I'm so sorry, I'm so sorry, because there is this guilt, this horrible, crushing pain in my chest because I must have done something wrong when I was pregnant with Kate.  Kate came and gave me a hug, patting my back, and telling me over and over again that it's going to be OK. She finally said, 'If  I'm OK, you should be OK, Mom.'

I'm still off and on tears today, but at least I waited until now to write this or it would also be filled with all those fun words and phrases I had flying through my head yesterday.  Cardiologist tomorrow to check heart rate from new medicine and change dose as needed. Dr. Wenger and hip check up on Monday now. Orthodontist on Tuesday. Oh, my yearly yup I 'm still going deaf hearing test is on the 16th.

I get it, I really do. All those inspirational quotes about pulling through, hanging in there, blah, blah, blah, I don't want to hear it today. Maybe tomorrow or next week. But today, I deserve an angry, pissed off, hurt and sad day. I'm taking it. Sorry mom, I know you hate that word. Love you.

Orthodontic Update

As I have mentioned in previous posts, people with Marfan Syndrome can have narrow palates and issues with their bites. Kate has already had an expander in for a few months, she then had braces to help straighten a few teeth and they look great. But, there's always a but it seems, a week after she got her braces off last December, her orthodontist decided that he had been through enough of this life and took his own. Shocking and sad to say the least. How did this affect Kate? Well, his office tried to sell and hire new doctors for several months and Kate was seen twice by two random people. Her orthodontic care had a major lapse in care. Completely my fault. I should have been more on top of getting her seen at a new office.

We went to a new guy on Monday, really nice and just happens to deaf in his right ear also. There are more of us out there than you realize, certainly more than I did. The news he gave us was not great. Whether she has grown and changed things or the expander was not left in long enough, we don't know for sure, but we do know she needs an expander in again. 6 months this time and followed by a type of retainer to make sure it sticks this time. Eventually she will need braces as well. Joy, right. Kate still has 6 baby teeth on the bottom and 3 on top.

All I kept thinking was you've got to be kidding me. It's like starting from scratch and I hate, hate, hate to put Kate through all that again. I don't think I have ever thought the word crap over and over again so many times. They put in spacers and next week we get bands on her teeth, and the following week we get the expander in. Here's the kicker. Walking out to the car I noticed Kate was limping. She said her left leg had been hurting off and on the last two weeks. The pain was around the bottom and back side of her knee. I figured we are seeing Dr. Newton, scoliosis doctor, tomorrow and I would just mention the knee and see what he thought. Was not prepared in any way for what he found.

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