#STANDARDIZED

I am not a hash-tagger. I could probably count on one hand how many I’ve used them over the last few years since they have become a thing. However, I may be becoming a convert. Chandler, one of our RIAO friends, created this one and you can check her out here. Dr. Standard really has the perfect moniker for such things.

Now back to he who has been #standardized, over and over again… Nicholas is doing really well post op. Super Leg surgery was on Wednesday. Today he is being weaned off his epidural and so far so good. He’s eating some, drinking well, sleeping a lot and generally handling this experience like the champion he is.

As for what Super Leg means, in Nicks case it involved a lot more knee work than I thought. Dr. Standard too a piece of Nick’s IT band and made him an ACL, he tightened the ligament he gave Nick in his first super knee surgery and he cut off a flap that was a meniscal tear and was probably causing Nicks popping an pain.

Nick also got a 2nd eight plate to correct his valgus so he has 2 now.

The Precice rod was removed.

Nicholas ankle and foot got the super treatment as well. His heel was brought down so he no longer has the rocker bottom and his ankle was fused.

On the left he had staples and a screw put in to stop the growth of his tibia. These things are removable if his growth trajectory changes and nothing was done to his femur but that can be at the age of 14. So we can watch his growth and see what happens.

Today Nick’s post op splint was removed and a cast put on. Allison did that and it went so much smoother than I thought it would. Usually removing anything is stressful but it went great. Nick has a temporary brace for his knee but a more specific one should be coming this afternoon. Dr. Standard does not want him to bend his right knee for the first 2 weeks post op and then he will start bending gradually. For Nick’s left leg we have already received exercises and started them yesterday with the help of PT. Inpatient PT and OT help so much through this process. They are so understanding and really got Nicholas going despite his reluctance. This time he didn’t resist too much though and he realized pretty quickly that for his left leg moving and bending felt kind of good.

After taking some steps and getting into the reclining chair also known as my bed for the last few days, Nick is asleep. If all goes well he could be released tomorrow. He’s had his sushi. He’s up and moving. As long as his pain stays managed we should be good to go tomorrow. Super leg inpatient stay almost complete.  #superleg, #nofibulanoproblem, #standardized, #tiredbutsupergratefulmothersigningofftotakeanap

“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.

 

Not so much to report…

Literally. Nicholas is doing great. We are stretching out time between doses of pain medicine. I only feel a little zombie like from the sleep disturbance. Siblings are loving having Nick home and loving playing with his balloons from our sweet fibular hemimelia friend Zoey and her family! Seriously balloons are joyful things! The siblings also received some gifts while Nick was away which they were so excited about and I was so grateful that they were thought of. Little things like that help them feel special too which is important. Last night we showed the kids Nick’s bandages so that they could understand better whats going on. Chris was feeling really sad and jealous that Nicholas got a new video game and was laying around playing while he had to get ready for bed. I think showing him helped. I would show all of you but some are on Nick’s backside. Gotta keep some business private.

I am a little worried about how physical therapy will go on Monday. Maintaining range of motion through lengthening is vital but Nick also had an eight plate inserted and eight plate surgeries usually meant weeks of Nick not wanting to bend his knee. We’ll see what they say Monday. He’s also complaining that his brace is uncomfortable. Not much to be done about that. So far Nick is still swollen around his knee and elsewhere. His right thigh (fibular hemimelia side is similar size to his left which is not the norm). When the swelling around his knee goes down I think it will be easier to bend.

Well Chris is crying about lunch. This will be an easy fix so I better hop to it. Thanks for the continued thoughts and prayers. Nick LOVES comments on his video so if you can please comment on that post.

PRECICE Preparations

Today Nick had his pre op visit at The Rubin Institute for Advanced Orthopedics (RIAO to the regular readers). Nick had X-rays and photos taken of his leg. We signed consents and answered questions so he could be included in a study on the PRECICE (All caps is the companies thing not mine). We had the chance to connect with all the usual suspects and people we adore such as Ann, Marilyn & Lee. Of course we met with Dr. Standard and Chris his Physician Assistant, and then some Fibular Hemimelia families. In particular we met a family from Ohio who stopped us in the hall to say this blog was the reason they were there! It is WONDERFUL to see the is blog serving it’s first purpose, which is to provide hope for families.

Some Surgery Updates: Nick’s current discrepancy is 5.8cm. We will be aiming for 5cm of length, which will take an estimated 67 days (lengthening 1/4 mm, three times per day). This is more than we thought. I had a feeling things would change as Nick has been growing so much! His predicted femoral difference is 3.6 cm but it’s ok to over-lengthen the right femur. Then Nick will need to do an internal tibia lengthening at age 15-16 of 5.5cm. That means he has 10.5cm left to go as of today. He has lengthened 17cm through his previous surgeries, therefor Nick would have had a discrepancy of 27.5cm at maturity if we had done nothing. That is almost 12 inches.

And knock knee is back. So Nick will get an eight plate or peanut plate in his knee. This is his third one. Talk about ‘chasing the valgus’! That was a surprise too!

Pain management might be treated a little different this time but I will post about that tomorrow and about Nick needing to get an IV before he is under anesthesia. Long story but it sounds like it is for the best.

Next stop Pre-Op Education with Nurse Lee. We received our Teal Book (we have three Purple Book’s from Nick’s external fixator days) and we got to check out this:

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That is the magnet we will use to lengthen Nicks’ leg. Also known as the ERC 2P (according to the manual.  It is the newest version. There are only three in the United States and we will get one. Nick enjoys feeling like guinea pig although I don’t see him as a guinea pig at this point. Just the fact that there are only three of these is neat and there are some differences between this version and the last. This one has a camera in it so we can see that we are positioning it correctly on Nick’s leg. He will have a mark on his leg that we will put the magnet over. The magnet is surprisingly heavy. I am hoping to have Nick do a video about it. I really don’t feel capable of explaining right now. I am still absorbing the details.

Then we got to meet a gal who has the PRECICE in right now and her mom. I have messaged with her mom over the last few weeks and that has been so helpful to me. Connecting with someone who is in the thick of it is huge!

Our next top was lunch and the grocery store to pick up some snacks to share. Then off to Hackerman Patz House where we got to meet Noah and and his dad (from the Fibular Hemimelia and Limb Lengthening Awareness group). Noah is having surgery this week too. This was pretty prefect. The boys played ping pong and video games and the grown ups chatted.

Then we got to meet Kinsey and her family! What a day!!! Connecting and chatting was great. It always is.

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Nick is wearing shorts on his head.

Below is Nicholas talking to his siblings on the phone. Saying goodbye to the kids was so hard this morning. I know they will be fine it’s just hard! Saying goodbye is the moment when everything gets real! Here we go…20150323_193809

Fibular Hemimelia Clinic?

Nicholas had a great check up at the RIAO today. Here’s the inside scoop:

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By the looks of it Nick’s eight plate is doing exactly what it needs to. It may be hard to see but it is correcting the valgus quite nicely. It still amazes me that essentially Dr. Standard uses that little thing to guide the growth of Nicks bone to correct the knock knee which is a classic issue with fh. Valgus has become a familiar word to a ridiculous extent.

The plate will probably need to come out around Christmas/New Years. Not looking forward to another surgery but its quick and out patient and frankly I will be glad to have Nick free of metal, at least for a long while.

I also added close ups of Nick’s knees. His ligament addition from 2 years ago is still holding up great. I have recently read about parents and doctors being against using cadaver donations. Personally I would not hesitate to let Dr. Standard do it again. It has worked fine for Nicholas.

Right now Nicholas has a length difference of less than 3cm. Probably closer to 2cm which is pretty great. No lift for now still just the insert which has a lift on it of a centimeter and a half I think or maybe two. This is the stuff I should remember but don’t.

Maybe it’s because we get very caught up socializing at the RIAO. We chatted with 4 fibular hemimelia families today. It felt like a fibular hemimelia clinic. Well I chatted much more than Nick but he chatted some. We saw a family we had met when Nick was getting his first shoe in NYC so long ago. We met a family from the Facebook group and a family of RIAO regulars that we have seen several times at events like Save A Limb and camping with Dr. Standard. We also met a 10 year old who seemed pretty similar to Nicholas. Nick talked to him (not about fh) while I talked to his mom. There is nothing like discussing this journey with someone who understands! I cant believe I got to do it more than once today.

I think Nicholas will benefit from that type of connection as he gets older. I am already thinking of ways to connect the kids. The Fibular Hemimelia and Limb Lengthening Awareness Facebook group has been awesome for parents. I think the kids may need their own thing.

So that was our day. All good news. Visited the cafeteria. Lots of laughs. As illustrated in the pic below. Nicholas was showing how much he has grown by pretending to be small which of course is unnecessary but made us laugh.

Batman was at the RIAO today but Nick does not like guys in costumes so we avoided him. We don’t need super heroes with capes and cars, our rescues and our own little miracles come care of Dr. Standard.

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Legs!

Monday was Nick’s post op appointment with Dr. Standard. The op being the 8 plate insertion back in January. It felt like we had not been there in forever, however through web chats, following other families, and having the fh potluck at hp (Hackerman Patz) in March, we didn’t exactly feel out of touch. 

Here’s the pic that really says a thousand words… To me it reads like a song. A very cheerful tune. Maybe an Irish jig.

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You can see the 8 plate in his knee, you can see that there is a length difference (Just 2 centimeters!!!) and you can see that my boy has really long legs. 

That’s what strikes me. Two long and straight legs. Dr. Standard said that everything looks great. In fact I had a bit of a surprise. Years ago we had talked about Dr. Standard doing a “final correction” to Nick’s ankle. This surgery would involve a diagonal cut through Nick’s tibia which could not be done till the growth plates at the bottom of the tibia were closed. So this was a surgery that would be done at the end to get Nick’s foot into the right position. The right position being directly under his leg. 

Well it turns out Nick’s foot it is in the right position or as right as it needs to be. I guess we’ve had so much going on at the last few visits I hadn’t really talked to Dr. S about the long term stuff. Now the long term stuff is just lengthening (internal lengthening by the way!!!!!). And of course 8 plate removal will have to happen. 

So this means Nick is really done with external fixators. Done. No more. Bye Bye! I was pretty excited discussing it with him in the cafeteria after his appointment and Nick said “You never know Mom. Don’t get your hopes up.” That sounds kind of negative but he really didn’t say it in a negative way. Just sort of ‘hay you never know’.  Of course he’s right and yet… 

It feels like a victory to me. Look at those legs. Nick will be 10 in August. He’s getting closer and closer to my height. We can share t-shirts now and if I were slimmer I bet we could share pants too. He is growing and thriving and running and playing and that’s the dream coming true every day. 

I will post soon about the fibular hemimelia potluck (another one is coming soon), Nick’s foot (because it’s all about a functional foot in the end), and the five fibular hemimelia families we saw at the RIAO on Monday! Yes FIVE in one day. There may have been others for all we know.

No Fibula, No Problem, No Restrictions

As of yesterday Nicholas is free. He danced and jumped today to celebrate. The reason he did not dance and jump yesterday was that I did not remind him that he was officially free. I thought of it many times yesterday but didn’t tell him. I was scared.

I am sure there is no reason to be. Everything looked great post surgery. Nick has not had pain. It really was a super easy recovery. Shockingly easy in fact. Nick’s last eight plate insertion caused inflammation and a lot of pain but this one has been fine (which is how eight plate surgeries usually are).

So he had the big seven screw plate taken out, little eight plate put in, adhesions taken care of and was on pain meds for only a few days. He’s been antsy to be active for weeks now. I told him I’d race him when he was restriction free but thankfully it’s a little icy outside so I am off the hook. At least for a while.

Now my only complaint is that the boy is having trouble falling asleep. Mostly I think because he does not want to go to sleep. Here’s a pic of him around 11pm a few nights ago. That’s late for him and you can see it in his eyes. Image

Nick does not want to leave Sinai.

Another surgery done. Yesterday Dr. Standard and company put an eight plate in Nicks’s knee, took out the 7 screw plate from the summer and took care of some adhesions that were causing pain for Nick from time to time. I was super happy Nick had nurse Harriet with him. The sight of her is such a comfort to me and Chris Fisher was in surgery with him as well.

Having so many familiar and trusted people taking care of my boy is wonderful. Chris walked me out of the or when I left Nick asleep. It was nice that he did because I might have cried had I walked alone. Chatting a little was a nice distraction. We talked about how violent orthopedic surgery actually is and the fact that the equipment used is similar to what could be found out in our tool shed. Nick use to ask for details on what Dr. Standard was going to do. I never asked for too much info but I am sometimes curious. I imagine it being loud in the or with drills and such going. I guess it’s pretty gross to consider so I’ll stop now.

We are having a pretty nice morning. I am kind of leaning Nick’s way and not wanting to go home. Except I really, really miss my other three children. Other than that we could stay. We’ve got nurses making sure Nick is comfortable and ok and a lovely lunch menu to choose from and it’s quiet and there’s cable and we even slept pretty well last night.

Maybe we are too good at making the most of it? Still we’ve got to go. Nicholas does not need to stay. His pain is under control and his leg looks good (according to Brian one of Dr. Standards new residents). Brian is new to us but may not be terribly new. Hard to tell. He was great with Nick and we had an interesting chat about zombies which has given me a whole new perspective on them and the fact that they are not as altogether impossible as I had thought they were. Despite his sharing that unfortunate zombie info with me I liked him a lot.

Nicholas had asked Dr. Standard Thursday if he would have to do physical therapy and walk right away and Dr. Standard said no so we’re good to go and Nick was so relieved. Dr. S said Nick can continue to do things his own way at his own pace. It is great that Nick has that control and Dr. S knows him enough to know he’ll get moving when he’s ready.

We and a brief visit from a family with an adorable little 15month old in his first fixator. It was nice to get to chat with them. We had met over the summer during the arm break/leg healing phase. I remember so well when Nick was a baby and we were here for his first surgery. Nothing since has ever been as hard as that. I hope that talking to Nick and I helped some. It’s hard to be at the beginning but that little boy was so beautiful and eager to get down and move around! I am sure he’ll do great and god willing his mom and dad will look back like I do and feel that the hardest part is behind them too. The light at the end of the tunnel gets brighter with each surgery behind us and thankfully today it doesn’t feel like an oncoming train (thanks Gretchen for that reference from so many years ago on the yahoo group it’s so fitting I never forget it or the support you and so many others gave me).

“Mom, lets go talk about my surgery fears.”

Thats what Nick and I just did. We sat in my bed and I asked him what his fears were. He said the surgery itself and cause he wakes up with surgeons he does not recognize. He asked to see Dr. Standard after surgery this time but I am pretty sure Dr. S goes from operating room to operating room and cant exactly sit by Nick and wait for him to wake up ; ) Thats my job.

So without being able to take this fear away I asked Nick what he thinks we could do to help with fear and he said “think of ways to make the most of it!“. We came up with 10!

  • We get to be together.
  • Video games at the hospital!
  • Getting to eat and drink whatever we want. (He has no idea that there are drinks I would want that I cant actually have at the hospital).
  • The awesome hospital rooms. Spacious! Massive TV! Giant sleep chair for moms!
  • Hackerman Patz!! Getting to stay across the street from Sinai the night before surgery is awesome and Nick loves it there.
  • The snack vending machine at Hackerman Patz.
  • Video games at Hackerman Patz.
  • Getting to sleep as much as we want in the hospital (he has no idea that this is not at all true).
  • Getting to play Minecraft with Charlotte even though we will be far away.
  • Having his new kindle fire to use at the hospital and the hospitals wireless internet. I’ve been grateful for that one for a long time myself.

So that’s todays list. By the end of the list making Nicholas was tickling me and laughing and smiling and I was reminded of how insanely lucky I am to be the mother of this most amazing boy!!!!

Teeter Totter Knee or Dynamic Valgus

For the last few weeks Nicholas has had a bit of knee trouble. It started with popping and clicking and sometimes it didn’t want to unbend. This happened a lot for about a week and then has been intermittent since. I was going to take him for an xray closer to home but Thursday I was helping out at the RIAO web chat and I figured I’d ask Dr. Standard about it. My instinct was that he would want to see him. Nick’s knee is unique, which I guess you could say for a lot of FH kids, but Dr. Standard knows it inside and out ; )

I was right and we went to Baltimore yesterday. I was worried that the problem was the ligament Dr. Standard put in in June of 2011. Dr. Standard had the same worries but it turns that is ok. Nick has a teeter totter knee or what’s known as dynamic valgus and he’ll need an eight plate put in to correct it. This was a relief compared to the problem being the ligament so it was good news in a way but then again your kid needing surgery is never actually good news. Yes I am grateful it is smaller, yes I am so grateful it is fixable and yet today I am kind of down about it. It’s actually 2 more surgeries.

Nick was relieved to hear eight plate. He wiped his brow in an expression of relief when he heard it was a small surgery and not happening right now. Can you imagine a kid reacting that way. My boy is really amazing and I know when the time comes he will likely take it in stride but after all he went though in the last year I will pay close attention to his emotions and reactions. It is a lot for a little (or not so little) boy to go through.

So surgery will likely happen by spring and it will have to be sooner if it starts to hurt him. Right now he’s saying it does not hurt but nearly trips him and is annoying. We might opt for January to get it over with in winter. Plus he will likely have the 7 screw plate in his tibia removed when the 8 plate is put in and that will mean weeks of downtime too. Honestly I am glad the plate will be removed. I know it was ok to be there but it is a potential problem in my mind and if it’s not needed I’d rather it be gone.

Teeter Totter Knee

Yesterday was also Christopher’s 5th birthday. We made it a great day despite spending it on Nick’s knee mostly. Chris was excited to get to have lunch in the cafeteria and cake by the water fountain in the atrium in the lobby. I also took them to build a bear at the hospital. It was fun to watch the kids choose their animals and just be happy. Because we bought four I was able to buy $50 in gift cards for $25. Nick asked what we would do with it and I said we’d talk to Marilyn about giving it to kids who will be in Baltimore for the holidays for surgery. When we were in the parking lot he saw a kid in a fixator and he wanted to run after the boy and give him the gift cards. I probably should have let him but we were just getting into the car and with all four kids and Nick would have really had to run to catch up to him.

Going to the RIAO with the four kids myself was not a hard as it could have been. Thankfully we were put in a room pretty quick. It kind of turned into insanity after that. Playing cards strewn across the room, paper from the exam table ripped and thrown, singing, dancing, yelling and general excitement. A doctor who was not working with Dr. Standard peeked in and I said “I bet you’re glad you’re not coming in here”. He just stared and ultimately I think he was entranced with Elizabeth’s singing and dancing (2 year olds are quite entertaining at least to me). As soon as she saw him watching she stopped but it was really a fun moment. When Dr. Standard came in he wasn’t phased by the chaos.

Nick also does not need a shoe lift. His difference is a little more than a centimeter or maybe it was a centimeter and a half. We found that out at the end of the visit so my brain was fried by then. I thought it meant that Nick finally slowed down growing but Dr. Standard said his leg might just be keeping up better at the moment. Sometimes the lengthening stimulates growth and hopefully thats what happening. No lift is a pretty big deal! And makes a nice happy ending to this post.