Miracle Work

Back at Hanger. I am in the lobby and Nick is in the gym. I don’t want to follow him around all day and I want to get some work done. He’s in the thick of his Miracle Work.

You might wonder what I mean by miracle work. Well for one I think the miracle is that there is something that will work. That someone (Ryan) created it. The miracle is being here having access to this device. Limb salvage isn’t new, though it is becoming more common. What happens after the salvage is less studied and deserves much more research.

Most of what I will share  I learned form Ryan and Jared yesterday. When I described the ExoSym as like Nick getting a prosthetic without having to lose his leg, I really had no idea how right I was. The ExoSym will bypass the part of Nicks leg that does not work. While this is a bypass, it is not a shortcut. This means the rest of Nick’s body will have to work harder to accommodate it. The brace itself will help some without putting in the work but it wont be a Miracle, and it would cause Nick to suffer in the long term if he tried to let the brace do the job alone. The Miracle Work must be done in order to avoid this and get the best result. It is already helping but the full effect will take about six months of work.

Nick started yesterday with some exercises that showed him how to engage the muscles he will need to work to have his miracle. Here is a video:

This work and the information shared reminded of Reenee letting Nick know that he is the master of his universe. He is the one who will make this miracle work. He’s not opposed to, nor deterred by the work one bit. Me, I am exhausted watching him but so proud. So exceptionally proud and so grateful to be here getting him what he needs.

 

The Case for the Brace

So Jen from Cigna helped the case for the brace move up to Tina from Cigna who will bring it up to Dawn and after Dawn it’s just Amtrak left to work on…. This sounds confounding but it is progress!

The reason at the moment for all this is that the code for the brace ends in 999. 999 means miscellaneous and any 999 code over $500 must go through higher levels for approval and have additional supporting documentation.

It does seem fairly reasonable to want documentation before shelling out over $10,000. I did point out to Tina that this is way less than the cost of another surgery. $10,000 would not even likely cover one day’s hospital stay post op.

In the mean time while we wait for approval Nick and I are going to Hanger! We fly into Seattle on Sunday, visit Hanger Monday and Tuesday and fly home after midnight. This trip is just for Nick to get fitted. This is the first step and we will have to fly back and stay for a week when the brace is created but this gives insurance more time to authorize it and Nick is just dying to get moving on this.

We are blessed beyond measure to be welcomed by a friend in Seattle. Remember how I wrote that Jen’s are awesome… Well of course her name is Jen! I have only met her in person one time but she is one of the kindest souls and I really can’t express how grateful I feel. It reminds me that we have support and things work out.

This is happening.

Mission Possible

I owe you all an update on Nicholas and our ExoSym mission. The paper work has been submitted. His case manager from our insurance company now check’s in with me weekly to see if there is any progress. This week she said:

“Please don’t stop reaching out to me. You don’t have to fight for this. I will do that for you.”

Did I mention her name is Jen? Jen’s are fucking awesome.

These are words I never ever expected to hear from our insurance company. Really, you will fight to get your company to pay for this expensive brace for my son? Thank you. God bless you. You are my new BFF. Sorry old BFF’s. Jen at Cigna is a my girl now. Sorry Steven I am leaving you for Jen from Cigna. She hasn’t actually done anything yet but I love her because she is on our side.

Speaking of women on our side.  I took Nicholas to a new doctor about a week ago although this doctor is not new to us. I have known, respected and admired her for years. Her name is Dr. Reid Nichols and she is at AI duPont… basically in our back yard! She’s treated complex limb deformities for years including fibular hemimelia and at least one of her patients is part of our FH family. She’s brilliant and thinks outside the box. She will do whatever it takes. She treats all kinds of conditions so I feel like she has a really broad knowledge base as well. She is a kick ass doctor.

While we were at AI we also had Nick’s brace adjusted and met with an amazing gal from PT and who made some suggestions for the brace adjustment that blew me away. Dr. Nichols also had real time solutions for some of Nick’s issues. A small thing from the pharmacy for toe pain. Kinesio tape for his knee. The whole AI experience was awesome. To be fair I know AI. I am on the Family Advisory Council and spent some time as the parent rep on the Ethics Committee. I already loved the place and I felt totally confident in Dr. Nichols and I believe Nick did too.

I can not wait to get my boy to Washington to Hanger! I am so grateful to Dr. Nichols for being on our team. She always has been really but now she is in the trenches with us! She has actually met someone who has the ExoSym and connected with the creator of the brace. She is going to apply her whatever it takes motto to my kids situation and in turn I will do whatever it takes to make sure this brace gets to more kids who need it. My mission does not end with my son, though it surely beings there and is my current focus and forever first focus!

This mission is possible. This brace finally feels within reach for Nicholas. Hopefully my next update will include a date for our trip to Hanger.

 

Faith Sans Fibula

Lately I have had some moments of fear and frustration trying to figure out how to get Nicholas this life changing brace that requires not one but two trips to Washington and still has uncertainty about whether insurance will pay for it. However I decided he would have it no matter what.  

Thirteen years ago when Steven and I were trying to decide if he should keep his leg, I took finances off the table as a decision making factor. We would make it work. I was not going to let money decide if my son could keep his leg or not. I don’t know if that sounds foolish or immature… maybe it was a matter of faith. I knew we could work that part out. I didn’t know if Sinai would be covered by insurance. I didn’t know if we would have to spend months in Baltimore, like so very many families do. I didn’t know what any of it would be like really. 

What I did know is that I could not decide to amputate my sons limb if it was functional. I am surely more open minded on that front now and I do think amputation can be the best option for many kids. Its not just a last resort. But in Nick’s case, for 12 years his leg certainly functioned as needed. Then last fall he had to start using crutches to walk because of pain and the words symes (amputation) came out of his docs mouth somewhat causally when discussing options. To be clear he didn’t think Nick was there yet and he presented other options but for me there really has been no going back from that moment.

It has made me question the viability of Nick’s foot like never before. Dr. Standard came up with a surgical plan which had about 5 parts and most of that any reader of this blog knows. Nick needed that surgery for various reasons and it was a success except for the fact that he still has pain in his foot (and knee at times). Pain that keeps him from being active. Pain that even happened when he was swimming. He was supposed to be free this summer and it has been anything but.

So again I questioned the viability of Nick’s foot. I refuse to give my son pain medication so he can be active. So he has spent a lot of this summer, way more than I would have liked off his feet and it made me think about the option we did not choose more than ever before. I still think it’s crazy that we even had to make that choice. I don’t regret the choice we made for our beautiful baby but his case of fibular hemimelia is not mild. His foot deformity was not solved with the first super ankle or subsequent corrections. He has had more than double the number of surgeries we were told he would need. That number means nothing to Nick and he has said so. Nick said he would gladly have surgery every few years to keep his foot functional. For him avoiding surgery is not part of the decision… speaking of decisions…

There is a huge difference between today and thirteen years ago. Today the choice is not mine and Stevens. Today the choice belongs to Nicholas. He wants to keep his foot. I have to make that happen. Thats what moms do.

The Exo SYM is where I have placed my hope. Since it has worked so ridiculously well for others I believe my faith is well placed. I can’t do it alone and I wont have to. As stressful as trying to figure it all out has been I have faith. Just like 13 years ago I knew if we made the choice we felt was right the rest would work out. Money will not, and will never decide if my son keeps his leg.

And so as the universe seems to provide what’s needed (or God or love or whatever you like to call the force that so many of us feel in our lives) I have had friends near and far reach out….

“Start a funding page and I’m in.”

“I might be able to help you.”

“I might have friend who can help you.”

“If I can help let me know.”

“I too will help.”

“I have a relative who may be able to help.”

“Go fund me. There are tons of us who would like to see Nick not in pain.”

“Benefit Concert?”

“I have your back.”

“Nick will have what he needs, I promise.”

Do you know what this does to fear and frustration? It neutralizes it. Whether or not these offers are needed, though I feel fairly certain they are. Whether or not they materialize for whatever reason they all create this avalanche of of kindness I feel washing over us. They remind me that as hard as it can seem and as dark as the world itself can get in moments (my Facebook friends know I have had some lately thanks to a political rant I shared), I know, know, know, know, know that Kindness is Everything, that Love Wins that we will make this happen for Nicholas. We have so much love and support behind us and beside us and holding us up. This is why my moments of frustration are moments! Just moments.

Our faith requires no fibula.

“Cant Stop Wont Stop Keeping My Foot”

“Can’t stop wont stop keeping my foot” and “Can’t stop wont stop growing”.  Those are Nick’s quotes on our way out of Sinai on Thursday after learning his predicted height jumped to 6’6″ and Dr. Standard’s plan for his foot.

For a kid who also learned he would be having surgery Nicholas left pretty happy. I think a lot of his happy was about his foot. I didn’t feel particularly confident in Nick’s foot even though the new insert has helped, he’s actually still had a fair amount of pain and knowing he’s going to be an even bigger guy than we thought just didn’t inspire confidence on my part.

I have to say I did feel differently after hearing Dr. Standards plan which is basically a whole lot of arthrodesis or fusing. It made sense to me when Dr. Standard said it all and I wish I could remember in more detail but I remember he and Nicholas were talking about doing a triple and I think that referenced fusing it in three places.

This surgery will actually have 5 potential separate events going on.

  1. Take out Precice rod.
  2. Put an 8 plate in his femur and adjust the 8 plate currently in his tibia.
  3. Epiphysiodesis on his left femur… the boy has agreed to be shortened some!
  4. Check out his knee and try to figure out what is causing the popping and clicking and pain. If he needs a new ligament he will not do the foot surgery this time. OR if his knee can be put off he will do the foot surgery because Nick feels this is the priority.
  5. Arthrodesis as described above.

Wow. That is a lot isn’t it? Still it’s not all big stuff and Nick wants it done now. The timing of the epiphysiodesis is vital so I don’t think we can wait on that. The precice rod is ready to come out. Nicks still having issues with knock knee so he needs the additional 8 plate. His current 8 plate is bothering him so it needs to be looked at and the general knee and ankle pain are the things Nick most wants addressed.

The good new though is that Nick might not need any more leg lengthening! He might just end up with a 2-3 cm difference and since he has to wear his brace and insert most of the time already that could easily make up the difference. Dr. Standard said we can wait and see and not plan to lengthen again till he is done growing anyway.

On our way to Nicks appointment we used the notes app on my phone to write out questions and then when we got to into a room at RIAO Nick wrote them down on the examining table paper so we would not forget. My plan was to really help him take the lead question wise even if some questions were mine. I don’t know how good I was at letting him lead but it felt like a great visit communication wise.

My most pressing question was his current heigh prediction and current predicted difference at maturity. Right now his discrepancy prediction is 5.8cm. It’s interesting that his height prediction jumped quite a bit but his difference did not. We think it means his right leg has been growing really well post lengthening. Sometimes lengthening can stimulate growth.

My boy is a great grower all around. I’d even say he’s an expert grower. Below you can see our hands traced on top of each other as closely as we could. Even though it’s a little off you can see Nick’s hands are a good deal bigger than mine.

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We did other drawing as well. Nick’s really into architecture and design although he still plans to be a chef and restauranteur. I love hearing him talk about his various interests and dreams. He’s currently doing a Young Actors Workshop in our community  for his second time and we planned surgery around his performance.

We also planned surgery for my spring break from school. I am much more concerned about Nick’s academics this year compared to last year so I have written to the person in our school district who handles 504 plans and have requested one for Nicholas. If I don’t receive a reply in a day or so I will call. I am not stopping till he has one. We only have two months to get things in order and of course there are a lot of logistics to figure out but I know we can do it.

While we were waiting at RIAO a young gal came over to ask Nick about the Precice and what it was like. He said the worst part of surgery was these supplements:

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Sorry to the parents whose kids will start taking these soon! I don’t mean to be a bummer!!!! 

It was kind of funny that he had this big surgery and months long process of leg lengthening and this was the worst of it. To be fair one of the 3 daily pills was like the worst big vitamin you can imagine. Drinking a can of seltzer to take them helped but apparently they made a lasting impression.  I am sure they helped with his amazing bone formation and who knows maybe they contributed to his “can’t stop wont stop growing” overall.

 

Our Best Days

Nicholas did not trick or treat last night. Nicholas did not dress up. But Nicholas did walk our dog and that made me pretty happy. I was happy that he could. Walking sans crutches is still feeling fabulous and looking fabulous (to me, Nick is having some leg pain which we assume is from not bearing weight for about 5 weeks).

Trick or treating was rough last year. He was waiting for a new shoe insert and brace (I think that was why he was in pain). Fall seems to be a rough season for his foot (which some theories are brewing on for another post). This year he just didn’t want to. At first I was upset and tried hard to encourage him but then I quit because I realized he really just didn’t want to do it.

Holidays I also realized can be little pressure cookers of stress! We want them to fun and fabulous. How many trick or treating Halloween’s are there in ones life time? Oh the pressure!!!!

This morning I realized, while reading a post on the fibular hemimelia support group that the holidays are not my favorite days. Sometimes I feel like they should be but if I am being honest, they really are not. Another mom was writing about her son having a hard time and not being able to trick or treat. I knew that feeling. I knew the emotions she was having so well and commented:

Nicks had some rough trick or treating years. He didn’t do it this year. He just refused but he’s 12. I could over analyze and wonder if his rough Halloweens have made him less into it but I think he just doesn’t care about it that much. I feel like holidays are a little like summer where I try hard at times to have as much fun as everyone else and make the most of it but my favorite days and our best days as a family are not the holidays anyway! All the pressure for it to be great and memorable isn’t really conductive to joy. For me…

I continued a bit more but I don’t want to share personal information about someone else and their child here. I hoped what I wrote helped this mother see that she is not alone. That these feelings are normal and that our kids really don’t seem to focus on the hard times. Nick does not talk about his hard time trick or treating last year or any other year.

To be honest I was kind of grouchy yesterday. I think I was upset about Nick not wanting to trick or treat, maybe it’s the bittersweet nature of him growing up. I also felt pressure to make it fun, be happy, it’s a holiday… etc. Well then the dog didn’t want to wear her costume, I didn’t know where the kids trick or treat bags were, getting everyone dressed stressed me, we were running late, Steven was not with us, Bess fell within the first 5 minutes…. I could go on. I wish I could stop this kind of spiral when it’s happening but I just smiled and wished I was off somewhere drinking a glass of wine!

A few hugs from neighbors and seeing friends out and about helped but I just prefer regular days. Holiday’s stress me. It’s fine and I don’t think it’s a unique thing to feel this way. Acceptance usually brings some easing of the stress so maybe next year will be better for me. It’s interesting to me though that I can be grouchy and grateful at the same time. I am so grateful for kind neighbors and my children’s happiness.

It was a beautiful night regardless of whether it was the best for not.

New Brace, Old Questions

IMG_5570I am so excited to report that Nick not only has his new brace but he is also wearing two shoes that are the same size thanks to the brace and shoe insert/filler. It was exciting and strange to see this for the first time on Tuesday. By next week Nick will be back to playing football and living his life without limitations. I am so grateful for that.

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I promised families in our facebook group a post on the brace and that is coming. I am not sure it is going to be the answer for all fibular hemimelia kids because some kids have ankle motion and likely wont need it. Yesterday though a question sparked an over 60 comment discussion and that is on my mind today. The poster asked if anyone had faced amputation as an option after having done many surgeries for reconstruction. These discussions always spur further questions and one thing I end up coming back to is that the skill of your surgeon or your child’s surgeon will determine the outcome of the reconstruction option more than anything else. With physical therapy and parent compliance following close behind and being absolutely vital as well.

When Nick was a newborn we took him to a doctor in NYC, Dr. Al Grant. He was familiar with and had treated fibular hemimelia. We were lucky to get an accurate diagnosis and layout of treatment options right away. Dr. Grant suggested we go to Baltimore to get a second opinion from Dr. Paley. Grant was on the fence regarding what would be best for Nick. He subscribed to the idea that 2 toes meant amputate and 3  meant reconstruct. Nick has 3 toes but it looks like 2 because two are together.

The RIAO blew us away from the start. We got to see Dr. Standard, who had arrived there a few months earlier and Dr. Paley. At the end of the consult we asked Dr. Paley if Dr. Grant could do what he could for Nicholas. His response “I can’t tell you what he can do I can only tell you what I can do”, was an honest one and it reminds me of my constantly suggesting families in our group take their kids to an expert, at least for a consult and for treatment if they can. That’s what I know.

I cant say what any other doc can do, though I have read things on the group and received priveate messages and emails from families that recount some unfortunate examples of doctors trying to do what they can’t. I can share with confidence what Dr. Standard has done for Nick and so many other kids that I have seen for myself. Nicks fibular hemimelia is on the severe end of the spectrum and I am sure that Dr. Standard’s skill set and experience are the reason Nick has had the wonderful outcomes he has had. When things have not gone according to plan Dr. Standard was able to handle it. He makes it look easy but I know that’s not always the case. He knows fibular hemimelia. The newest way he knows fibular hemimelia is collaborating to create this brace for Nick and other fh kids with stiff/fused ankles. Years of treatment has helped him see what these kids need long term.

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We didn’t plan on Nick needing a brace but I still consider this a wonderful outcome. The brace enables him to do what he wants and he still gets to wake up each day and stand on his own two feet. Lengthening and reconstruction is evolving just as prosthesis evolve for kids who have the amputation option. Nick’s brace is made with the same carbon fiber technology that some prosthesis are made of. I am so grateful technology seems to be moving fast enough to keep ahead of what my boy needs!

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