New Leg Same Life

I have not had much to write about since returning from Washington with Nick and his Exo Sym. I don’t think either one of us is used to his leg not limiting him just yet. He has had a couple instances where his knee has hyper extended but Ryan says he needs to develop the muscles in his leg in order to control that because controlling it via the brace will interfere with every day activity. Nick and Chris will both be seeing Dr. Nichols in a few weeks so we will discuss it further with her then.

Other than the knee pain he’s had some soreness in his right thigh but I am pretty sure that’s the good kind… like his leg saying “hey you are using me so effectively that I am sore”.

This week we attended a meeting of the Scholars Gild which is part of the Arden Club. Here is what is was about:

“Everyday life activities with a physical disability can be difficult. The November Scholars Gild discussion will be lead by Dr. Michele Lobo, assistant professor in the Department of Physical Therapy at the University of Delaware. Dr. Lobo will discuss how advances in fashion/apparel studies, engineering, and rehabilitation can be used to design clothing that improves independence, function, and quality of life for people with disabilities, particularly for those with physical disabilities. She will highlight innovations from her Super Suits FUNctional Fashion and Wearable Technology Program at the University of Delaware. Those innovations range from lower-tech adaptive clothing designs through exoskeletal garments and smart garments that allow users to control gadgets in their environment.
Bio
Dr. Michele Lobo received her Masters in Physical Therapy from Hahnemann University and her Movement Science PhD from the University of Delaware. She has been a visiting researcher at The Free University, Amsterdam, and the University of California, Berkeley. Her research focuses on developing and testing early interventions and devices to maximize participation, play, and learning. Her NIH- and DOE-funded group has members from rehabilitation, developmental psychology, engineering, and fashion and recently developed the first exoskeletal garment for rehabilitation.  Her current work includes a multi-site early intervention study and developing and testing a range of low- to high-tech clothing aimed at increasing independence and function.”

Interestingly Nick’s physical therapist Reenee was listed as a contributor. It is fun when worlds connect. What was also fun was Nick’s willingness to talk to the group about his Exo Sym and his experience. He never stops impressing me!

What also struck me about this talk was how awesome it is that there are people in the world so devoted to helping others achieve and get what they need. Dr. Lobo was quite impressive but her caring is what really touched me. We have encountered many people like this on Nick’s Fibular Hemimelia Journey and for that I am so grateful!

Maybe I will try to write about them now that there is much less to say about Nicholas. His leg works. He’s still Nick but Nick without limits. Once he absorbs this I might have some sort of activity post to share but truth be told I will be perfectly happy if he never does anything new with his leg other than live his life and walk though his days without pain. He’s more than his old leg or new leg or any leg or any part of him. We all are.

*** Special thanks to Steven Threefoot for letting me know about the Scholars Gild event! We live in a remarkable community and Steven represents the best of it for sure.

9 Seconds

This morning at Hanger Nick and I were feeling pretty cheerful. He was still sore from the previous days miracle work but he was ready for more and he was excited to receive the knee portion of his ExoSym.

By the end of the morning he did this:

And I think it speaks for itself. It wasn’t easy. He’s feeling sore but he’s aware of the difference between soreness and pain. He’s found the sweet spot as he calls for using the ExoSym effectively when stepping.

I really want to thank every person at Hanger for making this possible. I love that Nick gets to work out in a gym with others with the device. I am in awe of everyone there and in awe of the brace itself. It is, in the words of Beth, exosymply amazing!!

 

Pain in the Ass

Nicholas and I are back in Washington. We arrived last night and according to him had a bit of a harrowing drive from the airport to our dear friends home in the rain. I guess he’s not too wrong. Driving in the dark, in less than familiar surroundings, is a little hard for me. Add rain and… harrowing? Maybe so.

The rest of our journey was easy peasy. Lots of time for chatting and reflection. While going through security at JFK to pass the time I asked Nick if he had his ExoSym already and he could go anywhere where would he want to go and he said “home” and I asked “why” and he said “to take a nap”. So we have established that he was tired.

So I amended my question and said the nap would come first at any destination and he still said “home”. And now I was frustrated.

Me: “You’ve got to be kidding me”

Nick: “I want to go home, nap and go for a walk in the Arden Woods.”

Ahhh… this make sense to me. Something he can’t really do now or at least not do comfortably. This I get and this touched my heart. I want to do the same exact thing when we get home.

Today he started using his ExoSym and it was a pain in the ass… Yes a pain in the ass and that seemed like good news to me right away. Nick was using some muscles much more than he had before. The brace and the process are both simpler and more complex than I thought. I don’t have the brain power left to share all we learned today but I will. I promise. For now just know that while it was a pain in the ass it was not a pain anywhere else and Nick is doing remarkably well with his functional training which will go on for quite some time. He wont experience the full effect of the device for possibly 6 months. There will be a lot of work involved. I call it Miracle Work and I will elaborate next time.

For now: Happy Halloween!

14900320_10209191163869141_7027061662061084174_n

Foot Prints On My Mind

fullsizerender-7

A few weeks ago Nicholas had a gait analysis at A.I. duPont. It was actually a pretty fun Friday for him and his siblings. I can’t share pics because Nick in short shorts is not meant for the internet but the whole thing was so interesting. I suggested they rent the place out for parties. The gentleman who did the study let the sibs try out some fun stuff. I never think going anywhere in a hospital with four children will be easy but it was easy and funny and pretty painless for Nick except he lost some hair getting sticky things taken off after. All the circles on the figure above represent a sticky thing. It all comes back to bandages and tape. #whatFHmomsknow

The image above was given to us on our way out. The complete results of the study take weeks. I so love science and data and as impressed as I was with the whole thing I was not so happy to see this image.

Nick is still bearing weight primarily in the same place he did before he had 3 screws put in his foot.

This study was done after we went to Gig Harbor to get Nicholas fitted for the ExoSym. I am glad we had done so already because I feel this points to the fact that we need non surgical solutions now. The surgical ones just are not cutting it, despite a lot of cutting! Ha! Nick found that quite funny.

I am trying to take the insurance bit of the ExoSym process in stride. Cigna has not completed a preauthorization yet and may not ever. We just have to go and see what happens. This is potentially super nerve wracking as this brace will cost over $10,000. It’s really not just a brace though. It’s Nick having a prosthetic and keeping his foot. It also has a knee component that will be vital. I am understanding this issues Nick’s knee has more and more as Nick works with Reenee his physical therapist extraordinaire.

Yet again I find myself in a situation in which I have had to commit not to let money decide my sons fate. I really wont. It’s not fair to him. On Halloween my boy will get his ExoSym and he will create a whole new set of foot prints and follow the trail blazed by Zak Green and hopefully many kids with fibular hemimelia to come.

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.

Breakthrough

Nicholas is having some breakthrough pain in his foot. It has reminded me of a conversation I had with Allison, Dr. Standard’s PA, in the fall about what we could do about Nick’s foot and she mentioned how painful foot surgery it. She was right. She’s a lot like Dr. Standard; calm, kind, smart and pretty much right about stuff. I was going to write “right about shit” but Nick does not like profanity. He’s not likely to read this so maybe shit is ok to share here.

Shit as a topic brings me back to the pain he is having. We had spread his pain medicine dose to every 6 hour instead of 4. Then down to 5 because of pain and now we are going back to 4 to see if the pain goes away. If it does not I will email Allison to let her know Nick’s pain is getting worse instead of better. If it does get better at least we know it’s spreading the doses that made the difference and things are not worse than they were. I am also wondering if we should go to 1 pill every 4 hours instead of 2 every 6. Or one and a half ever 4.

If things get better at 4 we might still spread the doses a bit if the pain is manageable. Nick actually thinks the pain is spasms so we are giving him valium as well. I can’t help but want him to be less medicated if possible but I certainly wont let him suffer. Some intermittent pain is not equal to suffering IMO.  I am encouraging Nick to use distraction as much as possible. Right now his little bro is helping with that.

This seems to sound more confusing than it is. I had just written to Nick’s teachers to say I he could start doing school work next week. Seeing as he was less medicated I thought he had enough mental clarity to do so. If he’s back to 2 pills every 4 hours I don’t think it will work.

It really does not pay to stress school work right now, Nick’s teachers are not pushing and really taking their cue from us.

The other breakthrough of the moment is that I am taking a bit of a break from Facebook. I am not deleting my account or anything but I took it off my phone and wont be checking with regularity. This blogs updates will still post there so you can still get updates on Nick there if that’s how you do. My email and cell number are listed on my account so actual friends can reach out if they so desire. With changes to my school schedule coming up I really need to reconsider how I use my time and I also want to be more mindful in terms of how I connect with people. Simply put this breakthrough is about more than taking a break but I will leave it at that.

“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.

IMG_20160107_151712 (1)

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:

fbc1aa6b-8020-4bec-9876-12339a58b03c_285_0
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.

 

There is no good leg.

Elizabeth Gilbert recently said or wrote (I can’t remember which since I try to absorb everything she puts out in the world) that she is trying to stop being judgmental… to give up righteous indignation (not a direct quote in any way but it’s the jist).

I’ve been trying to do the same. But I am failing and this post is proof.

There is a procedure called an epiphysiodesis which basically is used to stop the growth of the longer leg in kids who have fibular hemimelia (it’s also used for other conditions). From what I learned at RIAO ages ago it’s safe, reliable and a great option to have. The International Center for Limb Lengthening describes it as a “small, minimally invasive, uncomplicated procedure”. It’s outpatient and for some kids it can eliminate an entire many months long lengthening.

In Nick’s case, so far, he is still refusing it. He wants to be whatever height he is meant to be. We want to honor the choices he makes about his body.

If it were me, I’d be all over this option. I would not hesitate. Not for a second. If Nick were willing, I wonder if it would have happened already. The timing of the procedure is very important. You can use the multiplier app to figure it out.

The thing that upsets me is that some parents wont consider if for their child because they “don’t want to mess with the good leg”. Calling the longer leg, without fh, “the good leg” in and of itself feels wrong to me. For one leg to be good by default it means the other is bad. I can imagine how Nicholas might feel if we had referred to one leg as good. His leg with fibular hemimelia has been through a lot, I’d argue it has worked harder than his leg without fibular hemimelia ever will. That’s a good leg in my mind.

Like anyone else’s legs, I think the simplest way to label the legs of kids who have fibular hemimelia is “right” or “left”.

And the aversion to doing a surgery on one leg in order to eliminate multiple surgeries on the other is poor logic, in my opinion.  Although I do understand the fear that drives such a belief. It’s also really not my business what choices anyone else makes for their child. I just happen to read a whole lot about it in the support group and sometimes share my views if opinions are requested.

Maybe the labels good and bad seem pretty benign to you. Maybe I overthink these things… I don’t know. I’ve seen other things this week which I judged pretty harshly. Such as a parent sharing a photo of a baby in a fixator calling it the “ugly truth” of fibular hemimelia treatment. It seems to me that this parent is trying to illustrate that fh treatment will not be an easy road. However I think the choice of words is frankly ugly and inaccurate. My sons leg grew 17 centimeters with the help of three taylor spacial frames. There is nothing ugly about that. It wasn’t always an easy journey but it needs no dramatizing and the last thing I would ever call it is ugly. Some of his fixator pics are so darn cute!

Nicholas' baby fixator (taylor spacial frame about a month after surgery March 2005
Nicholas’ baby fixator (taylor spacial frame about a month after surgery March 2005

I have been keenly aware from the start of this process that as his mom, Nick wold take his cue from me at the start (we reverse places pretty often on that one these days). Nick does not feel like a victim of fibular hemimelia. Yes we are still figuring out his foot pain but he’s living his life just fine in the mean time. He had some foot pain on Sunday after a lot of activity but both feet hurt… mine did too. There are no good feet or bad feet.

We’re pretty lucky to have feet. Whether two toed, or five, braced or free, carbon fiber or flesh… we are fortunate. Don’t judge a foot by it’s fixator…  or a leg by it’s scars… or a parent for their fear… or a mom for her judgy post on judgment… try not to. I’ll keep trying too. It doesn’t get any more good than that.