Lucky 22 Centimeters

On our way out of Sinai on Monday Nick was pretty happy. We had just learned from Dr. Standard that the lengthening portion of this surgery is done. I had expected it to be. I had marked May18th as the last day in our lengthening register. Doing 4, .25 mm lengthenings per day made it pretty easy to calculate but it could not be official without an x-ray and the ok of Dr.Standard. 5cm of lengthening achieved with the PRECICE. There it is.

A filtered picture of Nicks leg to help hi-light where the new bone is filling in.
A filtered picture of Nicks leg to help hi-light where the new bone is filling in.

Back to leaving on Monday, while expressing his joy Nicholas said “Mom these people are miracle workers. I want to make sure that these things don’t get forgotten and there are people to keep them going”. So I asked if he wanted to be a doctor or a physical therapist so he could make sure they continue but I could tell by the look on his face it’s not an option (and I know he wants to be a chef), so I reminded him that there was a fellow in the room today, as always, because they are always teaching others so these techniques can continue and spread further. Nicholas said that was a huge relief. He cares about the future and other kids having access to what he has had. He feels lucky. That’s the magic I could not have imagined when he was a newborn but my child is lucky.

Nicholas has gained 22 centimeters through leg lengthening (so far). 22 is my lucky number but I think it’s lucky enough to be shared. For along time I thought Nick only needed 20cm’s but predictions change. Not usually quite so much though!

I could not have imagined almost 12 years ago that he would have come this far by this time. Nick’s legs are pretty much even now (again)! This journey has not been what I expected in almost any way. I thought I would have to work so hard to help Nicholas feel good about himself because of this big limb difference. I thought I had to make everything ok. I thought wrong.

He’s always been ok. He has always been his own sparkling self. That was always my biggest fear, not what would become of his leg but what would become of his heart. How would he feel about himself. Now I know he is ok, no matter what happens to his leg.

So many fibular hemimelia families are at the beginning of the journey, wondering what will be, trying to make the right choices. I’ve often said you just have to make the choice and then make it right. Making it right means finding the best possible doctor and team to live that choice out with your family. No one else can make that choice for you. I don’t know how Nicholas could have come this far without the ICLL team and I am grateful to all of them but it’s Dr. Standard that has me in awe still. It’s Dr. Standard who can ease my fears like no one else. He knows my kids leg inside out and he knows fibular hemimelia inside out. That’s everything. I said thank you on Monday but it never feels like enough.

So what can we do with our gratitude? We can fundraise for Save-A-Limb! Want to help click here! If this blog has helped you or inspired you, please give. If you have ever felt inspired by Nicholas, please give. If you want to help others walk, give! We joined an awesome team this year with our friends from Mexico. I can’t wait to them on October 10th!! Its the 10 year anniversary of Save-A-Limb and we are so exited. Nick will be riding! Please consider joining us for what will be an amazing day. You can give or register here. Please also consider sharing and encouraging others to give. Save-A_Limb has helped Nick get what he has needed. We will be forever grateful. Nicholas has had the best care in the world and for that we feel so lucky!

Surgery Perspective

I accidentally out-ed myself as Santa. Nick needs a new charger for his computer that “Santa” brought for him and I said I would order it. He offered to bring me the computer so I could order the right one and I said no that I could just check the order. His “gotcha” expression said it all. I could not even speak. I was shocked at myself. When I could speak I said “Did I just do what I think I did?” Nicholas said no, and that he had been questioning for a long time. Something about this still felt so sad to me. I didn’t necessarily think he believed but we all behaved as if he did. Something about keeping up the farce kept it “real”.

Nicholas is growing up. Literally up, up, up but he is also changing in ways I can’t see but I can certainly feel. It is already an emotional week with surgery coming up on the 24th. This Santa business just put me over the emotional edge! I just need a good cry so badly! Nick is also visiting middle school on Wednesday. He has to do an interview for a specific program, which he is really hoping to get into it. It is interesting to have him want something and for me to really not be able to make it happen. Ahh… parenting! Nick, like my other three children, is a joy to parent but parenting comes with such a broad range of experiences and emotions. Minute to minute they change. This is life. I get it. Surgery is the kind of thing that puts it further into perspective. That is a kind of gift in and of itself.

Last night Nicholas also came to me to talk about surgery fears. He knows it is expected to have fears. We talked about the usual discomforts and things that are out of our control. He was happy to learn that he can weight bear some right away. He was worried about getting around within our house.

Nicholas has an amazing perspective for an eleven year old. He decided on the timing of this surgery and I am realizing more and more that he is a part of the team in a new way now. We have to included him in all decisions regarding his care. Being part of the Ethics Committee at our local children’s hospital reminded me of this recently. It’s really easy to think that because he is not eighteen he is not the decision maker but that is just not true and most certainly not ethical.

So Nick is expressing his surgery worries. I have shared some of mine. The rest of the household seems be a little on edge as well. Nick’s siblings worry about him. Their lives are impacted too. Meeting everyone’s needs through it all really has the potential to stress me but I am focusing on one day at a time. Today I had an 8am meeting and later I have a doctor’s appointment but I have dinner in the crock-pot already (Corned Beef…Happy Saint Patrick’s Day!!). If I had more time on my hands I would prep crock-pot meals and freeze for post surgery but I have schoolwork to keep me busy this week. Graduate school and surgery are not the easiest mix but I am taking that one-day at a time too. This weekend I plan to do as much of next weeks work as I possibly can!

I have also accepted the fact that this is simply not going to be an easy season. Limb lengthening is a huge deal. It just is. Even with everything going well it will take over our lives. We will find a new normal for a while. This is ok. We are so lucky that Nicholas has an amazing team at Sinai to care for him; we are lucky that Grandma and Grandpa can come and care for the crew at home, we are also lucky to be so close to Sinai so Steven can go back and forth some.

We are lucky. Surgery has helped me see that. It is a perspective I mostly wouldn’t trade that for anything but of course there are times when I wish Nick didn’t have to go through any of it. No matter how beautifully he does. No matter how freaking awesome he truly is through it all. There is a part of me that just wants to protect him from everything. I think thats just part of being a mom.

The Anatomy of a Success Story

This is an emotional topic. I have been criticized for writing about it repeatedly but you cant participate in the support groups with out facing this question again and again. It’s one of the reasons we need the support groups.

Amputation or Lengthening

One argument that drives me bananas is that many kids who go the lengthening route end up choosing amputation for themselves because it doesn’t work out. Many? Really? I just don’t think that’s an accurate statement.

I would say that almost ANY kid that goes through several surgeries and ends up choosing to amputate creates a story you will hear about it. Media loves that kind of story. It’s tragic and triumphant. Now consider a boy who had 13 surgeries and was doing great and just keeps going even though it was hard sometimes, he stays the course. Would that be newsworthy? One reason I have this blog is so parents making this choice can read about a kid like Nicholas. There are many more Nick’s than kids choosing to amputate, that’s a fact. It’s just not news.

Needing surgery and a brace to get to the level of function you or I have is not a reason to give it up my child’s limb. Nick’s ankle bones were fused, some obviously not even present, his leg was bent, shortened, missing toes, I could go on. But it was his and it works. Nick having had many surgeries, and needing more is not discouraging to us. It is part of his story, part of who he is. He is strong and beautiful and doing great. Sorry if that’s not the kind of success story people care to hear about (boy keeps leg… life goes on).

You would never know what Nicholas has been through by looking at him. Even Dr. Standard said that after watching the video of him running, however, Nick would be proud to tell you! So the fact that people would never know isn’t important to me. I am raising a child who wants people to know who he is. I’ve read this argument from amputation parents but seriously imagine summer and my kid and yours in shorts… guess what? Everyone knows. Prosthetic leg or leg full of scars are both pretty visible. Make it ok for your kid. That’s what matters. What other people think and know does not matter.

I am not saying lengthening is better or amputation is worse. What I believe 100% is that every case, every child, deserves to see doctors who know how to, and have had success in reconstruction before deciding it can’t be done!!! It saddens me to read about cases just like Nick where doctors told parents reconstruction was impossible. Maybe due to a larger than 20 cm projected difference or fused ankle bones. In the end thats about as inaccurate as you can get. It’s possible. I see it. Choosing amputation has to be a really difficult choice, but own your choice. Make a fact based choice. Don’t say ‘many kids who keep their legs choose to amputate them’. It’s just not true. I am not saying it never happens but suggesting many do, indicates a high percentage. That’s not reality.

There are reasons to amputate that are outside the realm of the medical facts and there are medical reasons outside the orthopedic facts. I respect parent’s who have to make that choice too but if you can’t own it don’t scapegoat and say lengthening doesn’t work and those kids choose to remove their legs.

In our lives success looks like Nick playing basketball after school. Nick joining the swim team. Nick running to meet the bus. Nick does not need people not to be able to tell. Nick does not need to be on the news to feel like he is a brave boy with a big success story.

Look at a specific doctors cases. How much fh has he or she treated and how? How successful has he been with either option? You wouldn’t let the chevy trained mechanic deem your honda a lemon would you? You’d go to a honda trained mechanic to have your car evaluated. Believe me I do not enjoy comparing our children’s legs to motor-vehicles but its an analogy that has been used before because it fits.

The irony is that if Nick’s leg was no longer working for him, I believe Dr. Standard would be the first person to suggest we consider amputation. I know that what matters to him is that Nick be able to live his life with mobility, doing what he wants to do. He is teaming up to create this brace Nick has got because he is looking at the big, long term picture. It’s not just about what surgery he can do. It’s about Nicholas. Dr. Standard is obviously a very successful surgeon but I don’t think he measures himself against others or by public accolades. For him it’s about the kids and I can’t help but suspect that for him success is watching a video of Nick running down the hall with joy.

 

the anatomy is there

the doctor has tweaked it

in the operating room

again and again

with metal and wire

and saws and drills

outside looking torturous

inside miraculous

through each and every step

nothing worth doing is easy

it’s a success story

the grand finale

life going on

step after step after step

and then he takes off

 

 

 

 

 

What if…

A conversation on the Facebook support group, regarding choice of surgeon and limits of insurance, had me thinking about when we first went to the RIAO. Nicks first orthopedic doc at NYU, Dr. Al Grant, suggested we see what Dr. Paley had to say. Its the only name he offered for a second opinion. He said every other doctor he knew would recommend amputation. Dr. Grant was in the middle regarding amputation versus lengthening and he warned us that Dr. Paley was “a bit of a cowboy”.

The night before our appointment in Baltimore Dr. Grant called to tell us he believed lengthening was the right choice for Nick. We realize he had just been at a conference with Dr. Paley and made his decision based on Paley’s information and experience. We counted that against Dr. Grant. He had said he could do the surgeries and could hold our hand through the whole process. He was really interested in treating Nick and I think he was being honest. Since he didn’t have as many lengthening patients. I think he realized that sending us to the RIAO for a second opinion meant there was a good chance we wouldn’t be coming back.

We met Dr. Standard at Nicks first RIAO visit. He spent a lot of time with us and we liked him right from the start. However we were there for Dr. Paley. He had been on Oprah for goodness sake! Anyone who knows me, knows I love Oprah (haha). By the time Paley left the RIAO a few years later, Nick was beyond established as Dr. Standards patient. That happened at the start due to his availability but in the end I am sure, was just as it was meant to be.

I have tried to consider what we would have done if the RIAO didn’t accept our insurance. I think at first we would have tried to get insurance that would have worked. As it was I avoided thinking about the money and didn’t research much about the cost of surgery. I just figured we would do it and deal with the consequences later. We ended up selling our home in Brooklyn and buying a much cheaper home in central pa, paying for a cobra plan and taking a year off work to care for Nick. Selling the house enabled us to do that and I know we were super lucky. In fact I felt like it was fate that we had bought my grandmothers house and then sold it to pay for Nicks care. I loved that tiny old house and the neighborhood it was in. I don’t know that I was totally honest when telling people why we were selling and moving but the truth is it was all for Nick.

The day before Nicks surgery we got word it was being canceled because we didn’t have insurance. I think because we changed to the cobra plan something was off. Once they realized that we had the cobra plan it was on but it was a few moments of crazy stress.

So in the end I haven’t answered my question as to what I would have done. Dr. Grant certainly knew FH. He was basing his, in the middle opinion, on the old adage of 3 toes lengthen, 2 or less amputate, which was already outdated by then according to the RIAO. I understand the logic. You need a certain amount of foot for the foot to be worth saving. The toes just aren’t the bones that determine success.

If we had chosen Dr. Grant we would certainly had not left NY. Fibular hemimelia put our lives on such a different course. I believed I was making the best decisions I could for Nick. It’s hard to imagine things any other way but back then it was us and Nick. Now with 3 more kids I think they would have factored in the decision too, had they been around. It would have been harder to pick up and move. Surgery complicates life so very much. Right now it’s all they know and I don’t think they’ve ever felt put aside for the sake of Nicks leg. I hope it stays that way.

In the end it’s not really possible to know what the right thing for another family is. Asking what if regarding our own past isn’t really relatable. My advice is usually to become as well educated on options as possible and then follow your instincts. Becoming well educated on options goes beyond just amputation versus lengthening.

Ten years later I can’t answer the “what if”. I can only be grateful to be where we are. Nick has a basketball game after school today. He wasn’t feeling great this morning but when I told him he had a game today he said, “My team needs me”, and got on with getting ready.

On the fence…

Nick is on the fence about fencing. During class last Monday night his foot and knee hurt. He really needs to be careful how he positions his foot to protect his knee and I am not sure he is able to do that. Right now we are waiting on a new insert/brace that Dr. Standard and Anil Behave PT Director at the RIAO have teamed up to create. Actually Nick will be testing at least 2 creations that we hope will help Nick with his athletic pursuits. This is only a trial but if it is successful it could help so many people. Anil told me that at a recent orthopedic conference he said “Children who have amputations receive a $10,000 prosthesis, while children who keep their leg receive a $50 insert” (thats a paraphrase really but I think the messages is clear).

I shared the following article, The Miracle Manufacturers: Limb Salvage and the IDEO Team when it first appeared and even messaged the creator of the Intrepid Dynamic Exoskeletal Orthosis. I also sent it to Dr. Standard. This article really made me think about what Nick might need long term. The IDEO helped soldiers go back to war. Previously they were in pain and some even sought amputations for limbs that had been salvaged. The IDEO was a game changer. I hope the orthotic Dr. Standard and Anil are creating will be a game changer for FH kids the way the IDEO is for the soldiers. There is no guarantee

Needing some kind of assistance isn’t something that worries me. Nicks foot is unique, his fibular hemimelia on the severe end. When it comes to his foot, surgical interventions are done. His foot handles every day life just fine. At home he walks barefoot all the time and has no pain. Running and high impact activities are not always so easy.

This endeavor makes us love Dr. Standard even more, which I am sure you all thought was not possible. The reason is the fact that he is more than willing to think outside the surgical box, in terms of solutions. He thinks about what life will be like for Nicholas and other children who have limb saving treatment. Not just function now, but long term. That may sound like common sense to you but I am telling you, not all doctors are looking at the big picture.

Nick’s leg will never perform as the average leg would. Some kids with fibular hemimelia have less severe cases and wont need additional help. My kid will and Dr. S is on it.

Fibular Hemimelia Forever Part Two: Fencing, Functional Panic and Famous Nick!

Image

These photos were taken last night. Nick and I had a great time at his school, at a sort of reading pajama party. Check out the huge cup of Jolly Ranchers. Nick won it by estimating how many were in it and being the closest to the actual number. He guessed 110 and there were 116. You might also notice the walker in the photo. Foot and ankle pain… sigh.  Might as well start at the beginning.

Monday Nicholas had his first fencing class. Meanwhile I had my most crappy feeling parent moment ever! Ever! I did not talk to the coach before the class about Nick’s leg. For some reason it never occurred to me. I also did not talk to Nick about only doing what his comfortable for him. Did you know that fencing requires a ton of fancy footwork? I didn’t.

Essentially Nick tried to do everything including standing on his toes and other things that he can not possibly do with a fused ankle. The end result was pain. Pain that had him calling me from school on Tuesday. Pain that had him calling me from school yesterday (that’s when I brought the walker in to him).

My heart really hurts right now because I feel like, had talked to Nick and his coach, this could have been avoided. Then I feel bad for feeling guilty, because this is not about me, and I shouldn’t dwell on my own feelings. A wise friend shared this tidbit, unrelated to Nick’s leg but it fits “Guilt is for a jury of your peers, it has no place in parenting.” I love it and I try to believe it. Feeling bad, and then feeling bad for feeling bad sounds ridiculous, but it happens!

I didn’t just sit around feeling like a bad parent. I emailed Dr. Standards Physician Assistant, Allison, and here is part of her response:

I talked to Dr. Standard and he said rest is going to be the best treatment for right now to get him over this flare-up.  He can use the walker, and if you feel like it is getting too painful we can put him in a full contact cast to rest the foot for about a week.  In the future, you can try some of the new accommodative orthotics that Dr. Standard talked with you about.

When fencing he should try to lead with his left foot.  It will require some adjustment if he is right handed but it alleviate some of the pressure on his fibular hemimelia leg.

I also left a message for Ann, Dr. Standard’s administrative assistant, because by the time I had to bring Nick the walker I was starting to feel that familiar panic in my heart. It’s a relatively calm panic. I would call it functional panic. Still it’s awful. I felt awful thinking of Nick being in pain, slowed down by the walker, stressed about his leg. His foot had it’s final correction. It’s all good now isn’t it?

Well it’s not. Didn’t I post Fibular Hemimelia Forever not too long ago? This is part of managing fh. This is a flare up. Flare ups can and will happen. Nick’s foot and ankle are complex. I know I knew things could come up. I didn’t think it would be so soon. We will have to be more cautious. Although it is encouraging that Dr. Standard did not say not to fence, and Nick still wants to try. His coach says he can do it, and he can adapt to work with his FH.

Nicks fencing coach seemed really supportive and that helps a lot. The plan is to try to keep going, although Nick says he really wants to swim. I don’t know how doing both would be schedule wise. We did intent to do a weekly swim lesson at the YMCA but maybe he should do more than that. The schedule is really a potential issue though. Four kids, various activities, and school in the mix… our calendar feels pretty full already.  I will not be one of those parents who over schedules their kids, running around from activity to activity every day. Well, with four children, I may not be able to avoid the running around part.

One more thing about school… I was worried Nick would be embarrassed or upset to have to use the walker. Not so much! Yesterday he came home and said:

Three weeks in [to school] and I am already famous.

That’s my boy!

*** Regarding fencing, I will be posting soon about the Paralympics and a world class fencer with fh!***