Scoliosis Sucks!

Because Kate is limited in what she can do physically, she is now doing archery. She really enjoys it and is doing well at it.

Kate should have been wearing her brace for her scoliosis but she went on strike in January and started refusing to wear it. She would put it on at night only to rip it off and leave it on the floor by morning. At first it was just here and there, but it gradually became straight up absolutely no. She found her voice and let us know she was done. It just hurt too much and she basically said she would rather have surgery than wear it any longer.

Looks like she gets her wish. She went to her back doctor last week and it turns out she is still getting worse. AND apparently the brace only had like a 10% success rate in preventing surgery anyway. Very annoying to find out after the many painful days and nights in her brace. Her new number in her lower back in 42, up from 38 I believe, last fall. They normally do surgery when the number hits 50. In addition to the sideways curve, it is also curving in toward her breastbone, which likely impacts her heart and lung function. Yay, right?!

The surgery to correct it would be a fusion of her back, likely almost the entire length of the vertebra. The downsides would be that she would not have any further growth there, and she would lose flexibility. The upside is that she would not have pain, the risk of further complications from further bending would be eliminated, and she would get to stop wearing the brace. Kate is thrilled about this part. The surgery is not without it's risks. There could be nerve damage, and her cardiologist would have to consider how it will affect her heart. There aren't really other options so we have to move forward with the surgery. She will still be able to do archery, but there are other activities that she will  not be able be limited by lack of flexibility.

Her doctor wants her to wait another 6 months before having the surgery. One reason is just to recheck her back and make sure her x-rays are truly what they say they are or to see if the bending stops on it's own. It's very unlikely, but fusion is permanent so it's better to be certain. We will probably not wait too long as her back may continue to get worse with makes more straightening they have to do in surgery. We will probably wait until the end of summer since the surgery requires a week or so in the hospital and another three weeks at home before she can resume normal activity. There are tests to be run before she can have surgery. She needs to have a pulmonary test, her regular EKG and cardiac echo, we need to talk to her hip doctor, etc. Everyone who has been involved with her care, with exception of her eye surgeon, will have to be consulted with and involved in the surgery decision and care. First of these appointment is June 3rd with her EKG.

I won't lie, been a rough start to the year between this news and my dumb Meniere's. Here's hoping the rest of the year is awesome, the surgery goes well, the pain ends, and heart stays stable. Insert animal sacrifice and multiple prayers here.... make that a fruit,veggies, and plant sacrifice. Kate is a pescetarian, no animals should be harmed, even in jest.

 

Expander is Out!

Kate got the dreaded expander out of her mouth today. She had to hold my hand the whole time. She is now being fitted for a retainer she will wear every night. She chose one with a ying and yang design on it. So very Kate. We got a donuts to celebrate, turned up the music and sang a song all the way home.

So she is now 5 7" which is towering over over her 5 4" mother and her 5 3" older sister. Noah is still the tallest here. It has been a while since I updated which is a good sign. She spent a few days with cousins from Utah at Disneyland in January and went swimming with some cousins the first of April in Escondido. February and March were full of school and life as usual. I was out all of February and most of March, dumb Meniere's Disease.

Quick update on Noah and Abby. Noah made the JV tennis team and loves it! He and Abby are still doing robotics and have traveled to Utah to a competition. Abby and I went to Japan for her graduation gift last week. Tokyo, Tokyo Disneyland, and DisneySea. Fantastic experience.

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.