“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.

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:

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.

 

Ease

So it seems Nick needs to ease back into full activity. He’s had some pain since beginning to fully weight bear. I checked in with Allison and this is normal. Knowing that Allison said it’s ok seems to have put Nick’s mind at ease. He remembers the third lengthening’s drama and worried about his bone collapsing. Even though that lengthening ended traumatically with him breaking his arm and his leg crashing too it was still super successful. Dr. Standard was abel to save his 6cm and make his leg even straighter than it had been.

In addition to finding out that Nicholas could bear weight on Monday, we also found out that he needs another 8 plate, this one in his femur. We are hoping it can wait till the PRECICE rod comes out but it might need to be sooner. His leg just wants to kick out to the side… again and again and again. If it has to be it’s own surgery so be it.

Above you can see Nicholas’s amazing new bone! He needs to focus on hip strengthening exercises pt wise and Dr. Standard also prescribed gait training so I think we need to have a session at the RIAO so Steven can learn what to do or go back to formal therapy close to home. Nick did well at duPont previously. We will work it out.

Of course I am resisting stressing this leg pain myself although I can’t help but feel like I wont be fully at ease till he is walking pain free. I have to knock that thought out. This is a process and I have to let it be what it is. A friend shared the wonderful quote below yesterday and I am trying to let there be room for whatever feelings are present. To acknowledge them and not try to knock the out, but also not stress them. That’s a process too! I also shared this in the support group and it resonated with others as well. Thanks Scott!

“We think that the point is to pass the test or overcome the problem, but the truth is that things don’t really get solved. They come together and they fall apart. Then they come together again and fall apart again. It’s just like that. The healing comes from letting there be room for all of this to happen: room for grief, for relief, for misery, for joy. (10)”
― Pema Chödrön, When Things Fall Apart: Heartfelt Advice for Hard Times

Nearing the finish line…

So if my memory is correct Nicholas has lengthened 4.2cm’s. The gap is pretty easy to see! He’s near the finish line, though there was a moment of confusion about what the finish line actually is. I think it’s 5cm. Dr. Standards PA thought even legs. We will just go back Monday and get new measurements and go from there.

I got to ask my question about the 8 plate not fixing Nick’s valgus and Chris basically said there are a few ways to fix it but there is time. I am choosing to let that worry go until I can chat with Dr. Standard about it in depth, some time down the road. For now things are going so well, why not focus on that.

Nicholas also had physical therapy to check in on how we are doing at home and the therapist was most impressed. His knee is at 95 degrees. Hip was a little tight but overall all is well and we don’t even necessarily have to go back as long as things keep going well. I think Steven should switch careers and become a physical therapist. He’s doing great, although he credits Nick. PT for lengthening is not an easy thing and it is vital. Mostly I would never suggest families do as we have done but we have and it was certainly harder when Nick was younger. DIY therapy is not for most people and if Nick had not continually improved his ROM we would have absolutely gone back to the pros more regularly.

So maybe one week left of lengthening, maybe Nick will go back to school next Tuesday. Maybe we can get to 5cm complication free… fingers crossed, thoughts and prayers requested, the finish line is near!

Rolling Along

There he goes. Literally rolling along. While Nick is self propelling I am pulling around the large case that contains his remote for lengthening. Calling it a remote makes it sound small. It is not.

Today we started lengthening. Although Nick did get a few mm’s during surgery this is the official start from our end. Nurse Lee delivered the device (which ended up being the old version and not the new… long story) and helped with the first go. I have a really cute photos of the moment but since Nick is in his boxers I wont be sharing it here. Nurse Lee is really one of unsung hero’s of the last decade of our lives. Seriously. I have written about her before but not enough. She has been there for all of Nick’s lengthening’s. In general she teaches parents how to care for their kids in an external fixator and lengthen their limbs. Now also teaches parents how to use a big magnet to lengthen limbs. It’s no small thing to train us to take our kids home and do these things to them. Plus she runs the web chats which have helped families in immeasurable ways.

Nurse Lee is a person I have counted on probably more than should and certainly beyond her job description. I respect and admire her so much! Now back to Nick…. sort of…

Today was doctor appreciation day but we did not actually see the doctor. We saw Chris the PA. With Nick’s third lengthening and monster fixator I was not ok with him not seeing Dr. Standard and frankly neither was he (triple negative… shut up grammar police). It seems we have both grown a lot since then. Today’s appointment with Chris went great. He answered all of our questions and concerns. Right away I asked him about the swelling in Nick’s knee which he said was ok. Nick’s other incisions all looked good. We laughed a little and really had a standard appointment… even without Standard 😉

Our clinic visits will be a crazy lot shorter if we see Chris or Allison. As long as Nick does not ask for Dr. Standard I have decided that I will not. I trust Chris and Allison with Nick’s care. We love seeing Dr. Standard but if things are going smoothly we don’t need to see him. I have talked to another family that does their appointments this way. Nicholas used to ask for Dr. Standard as soon as we walked off the elevator at the RIAO. Times are changing.

So all in all it was a good day in Baltimore. The valet service was a godsend, as it has been in the past. The gentleman who helped us today has been there as long as we have been using the service, which was a comfort. Little details can really make the day easier. Nicks physical therapy evaluation went well. His eight plate is going to make the knee bending harder but it’s really ok. Extending is the focus now and we will keep working on the bending. Since pin care was usually my job and pt was Stevens I might take on some PT. Femoral lengthening requires more work so I think it makes sense to take some on, if Nick will let me. He’s pretty particular about who does what. He jokes that I am out of job without pin sites. Imagine that.

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:

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.

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…

Nick’s Thank You Video

This video, which was recorded last night, may seem silly but its message is heartfelt. Nicks eight plate removal surgery went well. He had his last dose of pain meds Saturday night before bed. Nick is free of metal, except for his braces and even those will come off in less than a month. He’s moving around tentatively but feeling well.

Nick was really calm before surgery and woke up calm. Hopefully we will be back in Baltimore soon for a replacement brace for the trial. Nick said it was helping and once it cracked he said the “push off” it gave him was gone. Good news really.

If I don’t write again, happy holidays and happy new year to all our readers and followers. It is a blessing to have Nicholas in your thoughts and prayers. It is a blessing to know writing this blog has helped others. For the new families, as always, please be encouraged! 13 surgeries down and Nick doesn’t feel like he has had a surgery filled life. He feels grateful! He thanked everyone he could on Friday, even me. “Thank you for bringing me mom”. As if I would have had it any other way.

Friday the 13th

This Friday, the 13th of December, Nicholas will have his 13th surgery. Dr. Standard will remove his current eight plate. It’s outpatient and should be short and sweet. Or as sweet as any surgery can be.

Nick and I are going alone, as we have done many, many times in the past. I worry as he gets older (what I really mean is BIGGER) about going it alone. I can’t even come close to carrying him now. I will just pray we do not need to make any stops on the way home.

Some might suggest Steven take him instead of me but I can’t consider that as an option. I have always been there for surgery. Always taken him into the operating room and helped him go under. It might sound selfish but I know that its also important to Nick to do things as we always have.

The only thing that is really upsetting Nick about this surgery is that there is a basketball program starting at school and he will miss most of it. I don’t think he’ll be running and jumping for a few weeks.

So today begins a crazy week for our family. Today Nick has a party to go to, tomorrow his pre op physical, other family appointments like the dentist and orthodontist, 4 meetings for me in the evenings (including my first as part of the Parent Advisory Council at AI DuPont), an after school excursion to hunt for candy canes at a potato chip factory (and see Santa!), prepping a community dinner for 80+ people (which is Saturday night and my mom’s sauce is the centerpiece), Nicks first concert as part of the school chorus, and of course actual surgery Friday!

This past Thursday Nick and I participated in a web chat with Dr. Standard. Nick offered to answer questions. He didn’t take many but he really enjoyed it. I am always impressed with his knowledge and outlook. He even came up with a new slogan, “Motionless Feet Unite”, for folks with fused ankles like his.

My boy is just living his life and doing what he wants. He knows that not everyone can do that. Each surgery moves Nicholas closer to the light at the end of the tunnel. Although, in many ways, he has always been there. He’s almost always been active and happy. If that’s not the light, I don’t know what is.

Fibular Hemimelia Clinic?

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

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.

Fibular Hemimelia Forever

Tonight Nicholas got an award for most improved swimmer (10 and under). This award was based on improving your times. While Nicholas might not have looked like a winner in terms of meets individually (he was often in last place but altogether the team was the division campions!!!!), he was absolutely a winner in the way that matters most. He worked hard and he improved. I think he had fun too.

I don’t know how much his leg affects swimming, Nicholas once said “Fibular Hemimelia does not matter in the water”. I suspect it does matter some. His foot not flexing must matter. Or I would think so but I am not much of a swimmer so feel free to correct me.

When he started swimming his knee was still clicking. Nick has his second eight plate which will help what is called dynamic valgus. The first few days of swim practice Nicks knee bothered him and his leg was hurting. Of course I had a moment of panic wondering if this was a good idea but within a few days the pain was gone. He has not complained of pain at all since. I am wondering if the swimming increasing his strength maybe gave his knee more support.

In any case Dr. Standard was excited when I told him Nick was on the swim team. It has proved to be a wonderful thing for him and his sister and brother. This is a fh blog so we wont go into the fact that Charlotte would not put her head in the water last summer and now jumps in and is a super confident swimmer and Chris can swim! Before the team he couldn’t.

Before the kids joined the team I didn’t know they could. I thought you had to qualify. I thought they wouldn’t be good enough. I was so excited to find out (from some kind and encouraging neighbors) that how good they were or weren’t didn’t matter. Their team, The Arden Sharks, was remarkable. The head coach Chris was very encouraging but at the same time he did not treat Nicholas any different. That’s hugely important. Nicholas was just another kid on the team. Chris is Nick’s first coach and for that I am grateful. It has been a great first foray into athletics. If it is his only foray into athletics that will be fine with me but he has been talking about trying other things. The confidence he has gained warms my heart.

All the volunteers and assistant coaches were wonderful too. The feeling of support and camaraderie the kids had was great to see. I didn’t think that my kids should do the swim meets at first (I worried they needed to do well and feared they wouldn’t) but coach Chris said they could/should do it. He said that then they would have a time to try to improve. He said it would be good for them. He was right.

Nick wasn’t always in the mood for practice but he never regretted going. This experience was good for learning discipline in general and good sportsmanship. On more than one occasion, when Nick was not interested in going, I’d said “you show up for your team.”

A post or so ago I shared a photo of Nick at a swim meet. The post title was Fibular Hemimelia Wins. I wanted to elaborate on that. I know when someone has a hardship, disease or condition, people like to say they are fighting it or that they are fighters. I may have said that about Nick but thinking about it now I don’t think it’s appropriate.

Fibular hemimelia is part of who Nick is. That will never change. Why fight it. Manage it, work with it, cope in the hard times but don’t make it the enemy. My boy will finish treatment at some point but he may have issues in the future (lots of people without limb defects end up with joint problems). I am happy that his leg with fibular hemimelia has carried him this far! He will be 10 next month!!!

I hate the reference I have seen in web groups a few times that kids who have lengthening and reconstruction are “patients” and kids who have amputations are “athletes” or “just kids” (it came up again this week). We have had some rough seasons surgery wise (last summer was hard but Nick also broke his arm in the middle of it all). I still would not say that in terms of life in general he is a patient. Nicholas doesn’t see the word patient as negative anyway and has actually enjoyed being an inpatient and requested additional days at times. I don’t think the people who have posted this realize how unkind and untrue it is. Maybe kids who have amputations don’t have as many surgeries and maybe they don’t even have fibular hemimelia anymore since they are considered amputees? Nicholas Curley will have fibular hemimelia forever but it is just one part of the amazing person that he is. I couldn’t imagine him any other way.