It might seem from my one functional foot, two hopeful parents post, that we made our decision right after our first trip to the RIAO. Really we didn’t. That trip eased a lot of our fears about lengthening but we still had reservations. I had my dream and my vision for my son but I did not want my wants to override his needs. He needed to be able to walk and live his life.
One thing I considered was that if I was in a terrible accident and they told me I would have to have a fixator for several months maybe more than once, or they could amputate my leg and save me from lots of surgeries and pain, I would still want to keep my leg. Amputation would be the last resort so long as my leg would be functional. Why should it be any different for my son, because he has a congenital birth defect. I realize that if you amputate early enough you eliminate a lot of the potential psychological damage and phantom limb pain but if there was a way to make Nicholas leg work for him, why not try.
I know many people will disagree and I mean no offense in saying this but I saw amputation as an easier option for us as parents. It would be less disruptive to our lives. Three external fixators and possibly other surgeries are a huge commitment and financially could be pretty costly. Amputation does not actually save money when you consider a lifetime cost of prosthetics and much of that cost would fall on Nicholas himself. At this point in the decision making process we had no idea what our insurance would pay or if they would pay at all. I decided not to even think about money in relation to our decision because I did not want it to be about money. We would make it work. What was best for Nicholas was not going to be a financial decision.
In the end, I think our choice was made out of hope. If kids like Nicholas had been successfully treated, it was not like this was a totally new experimental procedure. Yes Nicholas’ anatomy was unique but there was enough there to work with. In Baltimore I did not ask many questions about how the surgery would be done. It was hard to think about Nicholas’ leg actually being cut open. The how to’s of limb lengthening and reconstruction were not of interest to me then. So long as his surgeon knew how to do it. Why did I need to know what tendon they would be cutting or what nerves they would be releasing. I needed to know that they could do it. I needed to believe it would work for my son.
We could not let our fear of what lengthening would be like for Nicholas, over ride his right to keep his own limb. Steven and I have had moments where we questioned our choice but they have been few and far between. Those were our darkest moments, like when he came out of his first surgery with a tube down his throat, or when he was in pain after this recent surgery. Is there anything harder than seeing your child in pain? I don’t want to think of what is. I still wish with all my heart that I could have just given Nicholas my leg. Really and sincearly I would have it cut off tomorrow if Nicholas would be able to use it. I bet Steven would say the same.
Most days we are not only happy with our choice we celebrate it. We cheer after every visit to Dr. Standard. Looking at x-rays of Nicholas growing leg and healed ankle. Today we will make the oh so familiar two hour drive to Baltimore and be both anxious and hopeful along the way. We have not been there for three weeks. But I am never more at ease and excited than after Dr. Standard tells us everything looks great. I suspect Dr. Standard is as excited for Nicholas as we are. His smile says it all. And so does Nicholas’.
“Perhaps Mommy you love me more than I love you”. Nicholas said to me last night to which I respond “In fact I think you are right”. I can’t imagine that there is any love greater than the love I have for my children. Nicholas is particularly emotional, just like me. We both bawled our eyes out while watching Ice Age. We are very much in tune to each other. I cant be in any kind of mood without him noticing. Like earlier today when he said “Mom are you getting frustrated with us”. I think I know his feelings just as well but sometimes he surprises me.
Nicholas has been in his current fixator for almost four months. I can’t believe he is more than half way through this lengthening. The rhythm and routine of days goes by and we don’t always remember what a big deal this really is. He does have this hunk of metal attached to his leg every moment of every day. I think we are so focused on being positive that we forget that this is hard for him even if he seems fine. A few weeks ago he told Dr. Standard that his knee hurt sometimes and he had not told me that before. Later at home I told him that if his leg hurt he needed to tell me so I could help him. His response was “Mommy it only hurts sometimes and you distract me with your love and hugs”. I was speechless.
You would never think he was in pain at all the way he moves constantly. We went to the local pool with family today and he was in the pool for over 2 hours and I don’t think anyone would know there was a fixator on his leg while it was under water. When we got there I asked Nicholas if he wanted to let the life guard know about his fixator. So he went right over and said “I have an external fixator. It’s for leg lengthening.”
Nicholas wanted to take his cover off. We had never been to a public pool while he was in his fixator and I was worried about peoples reactions. I told him that if he took his cover off people might ask him more questions about his fixator but he was fine with that. He just wanted to swim the same way he swims at home. It was no big deal to him. He did not even notice the few people who stared at his leg or the father that took his toddler daughter away from where we were as soon as he saw the fixator. I cant say for sure if it was on purpose but they never came near us again.
Later in the evening Nicholas started complaining that the big toe on his left foot hurt. He was literally limping up the stairs trying not to touch his big toe to the ground. How he walked that way is beyond me. Balancing on his left heel and his fixator on stairs! When I told Steven about this he asked why I am so worried all the time. I tried to tell him that just because Nicholas can do something, does not mean he should. I think I am getting anxious about something going wrong. The actual lengthening portion of the process is done and he is in the consolidation phase. This is just time for the bone to harden. He already has his 6cm. His ankle has been corrected. So now we wait and I worry. Perhaps thats what happens when you love someone so very much.
When we went to Baltimore for our appointment we knew that it would be a long wait. Based on the research I had done about the RIAO we were prepared to wait six or more hours. I also knew that Dr. Paley had actually studied under Gavril Ilizarov, the guy who discovered the process that could save my boys leg. Google him, it is fascinating. But otherwise we did not know what to expect.
I think I expected Dr. Paley to be an aggressive fast talking type of guy. In the end he was no cowboy in my opinion. He was confidence personified. But Dr. Paley was not the first doctor we saw at the Rubin Institute for Advanced Orthopedics. We saw a doctor who had just started at the RIAO a few months before, Dr. Shawn Standard. We did not know then that he would become Nicholas’ doctor but we liked him from the start. Dr. Standard sat and talked with us for a long while and examined Nicholas. When Dr. Paley came in he examined Nicholas, looked at x-rays and said “this is a functional foot”. The reconstruction of his ankle was the complicated part but Dr. Paley was sure it could be done because he had done it before. Of course there were no promises of a complication free process but a functional foot is what we were hoping for.
I know there is so much more to that first visit and that conversation but it is all a blur. We were probably there for 4 or 5 hours. Dr.Paley said that lengthening and reconstruction would work for Nicholas. He said that there was no reason to amputate Nicholas’ leg and that he had treated children with similar degrees of FH and had success. In fact hundreds of children with fh were treated at his center. Which is pretty impressive considering how rare it is. He too admitted that Nicholas would not necessarily be a track star and that amputation would be one surgery and produce a very functional result. He told us he could do that if that was what we wanted. Lengthening was after all a huge commitment. It would effect our whole family, involving months of appointments, physical therapy and daily care. Nicholas would need at least three lengthening surgeries each likely to be 6 months or more in an external fixator. Another possibility was a surgery to stop the growth in Nicholas’ left leg so his right would not need to be lengthened quite so much but Dr. Paley was not sure if it would be an option for Nicholas with his estimated discrepancy of 8 inches at maturity.
Lengthening would not be an easy road but at the end of that road Nicholas would have not only a functional foot and two legs of equal length, he would also have a foot with sensation. He would feel the sand between his toes. I didn’t really care if Nicholas would not be as fast or as athletic if he had the lengthening. I had an image in my head and heart of Nicholas waking up in the morning and popping out of bed with the legs he was born with.
Were we even saying “googling” back then, when I started researching fh? I can tell you that I looked at each and every result that google came up with back then. But in the fall of 2003 there were only several hundred. Today if you search fibular hemimelia there are 9,702. Clearly I wont be looking at all of those but I wonder if they are any more hopeful than the ones I found 5 years ago. And I didn’t just search fh back then. I searched any related term I could think of. Without much success. Today the first result that comes up is from emedicine and it would tell you straight off the bat that Nicholas’ leg should be amputated because they say the maximum amount a leg should be lengthened or “corrected” as they put it is 7.5-15 cm. Nicholas’ estimated discrepancy was 20 cm.
Those early searching days were frustrating and at times heartbreaking. There were the stats: one in 40,000 births, more common in boys. And the details, occurred during early limb bud formation, bowed tibia possible femur and hip issues and lots of medical terms I wont share. A new name was suggested, Postaxial Hypoplasia of the Lower Extremity, because fh involves so much more than the fibula and it’s absence or shortening. Amputation was the standard treatment. Even for cases much less sever than Nicholas’. Now don’t get me wrong the children who had undergone amputation were living well and had web sites and support groups to show it. Some were paraolympians and all were to be admired.
But I wanted to go online and find success stories involving limb lengthening as well. Instead I found warnings against it and lists of risks and complications. Some doctors refused to do it. There I as with my one month old baby boy, thinking he would either have to have his leg amputated or go through this crazy painful process that some people even likened to torture. Even without the negative stuff I found about lengthening on line, it is still pretty scary sounding when you first hear about it. External fixators are not pretty. Nicholas once called his a “hunk of metal”. Which it is. But it is a hunk of metal that enables him to grow new bone and it keeps his leg safe, so we do appreciate this hunk of metal very much. Just yesterday Nicholas fell down right on his fixator three times and was fine. Most people never really see it. They only see the fun home made covers that conceal it. The covers both keep it clean and keep from shocking people all over town. I think he has 16 pin sites, which means 16 places where there is something sticking out of his leg, sixteen potential sites of potential infection. I should know. I stare at his leg each night to clean it with antibacterial soap and make sure the pin sites are clean. It is a sight I am quite comfortable with now but seeing them online when Nicholas was still a newborn baby was overwhelming to say the least.
During the search and research days I was scared and exhausted. With in a year of Nicholas being born we had bought our first home, been married and I had gone back to school and was working on the weekends. Steven was working two jobs so he was no less tired than me but I was the researcher, appointment maker, question asker, you name it. Steven was just as interested in doing the best we could for Nicholas but he didn’t even have an email account back then and I did take charge from the start. Moms usually do and research was something I was quite familiar with.
All my investigating was done either between classes, during classes I skipped or at night while Nicholas and Steven slept. The library at Saint Francis College was an amazing resource because there I had access to actual medical journals for free! Even though they were hard to get through I had learned enough about research by then to know which parts of the articles I needed to focus on. There were actually studies out there that did not demonize limb lengthening and they were not all written by the supposed cowboy, Dr. Dror Paley. Making our trip to Baltimore would be the next step in our journey.
A few weeks after Nicholas was born we took him to see an orthopedic specialist at NYU and the x-rays began. Nicholas hated them when he was a baby because he hated being held down (not an uncommon feeling among babies I am sure). Currently he chats it up with the techs and wants to know how everything works. Nicholas always smiles when they say “ready” as if they were saying “cheese” . He knows it is only going to be his leg in the x-ray. He just cant help but smile. Now as a baby it was the WORST part of any appointment and it felt like they took forever. I could not go into the room with my boy because I was nursing so I cried listening to him cry. I tried not to and tried to hide it but anyone who knows me knows I really can’t hide my feelings. I cried at a lot of appointments.
After the films were ready and we were back in the exam room we waited for the NYU, all our hopes are hanging on you doctor, to say something. Instead he took out his prescription pad and wrote very slowly (I suppose that is why it was legible) FIBULA HEMIMELIA. This meant nothing to us at the time because neither my husband nor I had heard of it. Of course now I could write a book on fh… or maybe a blog. And now I realize that he spelled it wrong (also not uncommon). I think he spelled it the way he said it, with his classic New York accent he dropped the “R”.
In the next few minutes he explained that Nicholas had no fibula, the smaller of the two bones in the lower portion of his leg and that his tibia was bowed and shortened. Of course his ankle was not formed correctly because the deformity effects the entire limb. Much of this was self evident. Next ,NYU our hopes are now fading doc, told us amputation was the standard treatment and that nine out of ten doctors would suggest this for Nicholas. With amputation he could run track and be an athlete (as if I cared about him running track in this moment. I just wanted my boy to be able to walk). There was another option, limb lengthening and reconstruction which would be at least three long and pain full surgeries and three external fixators. Looking at his foot he explained that what looked like two toes was actually three. Two were syndactaly. Normally he said, with only two toes amputation would be the clear choice but with Nicholas it was hard to say since two were together. This baffled me. Deciding the fate of his whole leg based solely on his number of toes. According to him the big toe and two together equaled 2.5 toes. He told us there was no wrong or right choice. We could take some time to decide what was best for our son and our family. But if we were to choose amputation it would need to be done before Nicholas was eighteen months old to avoid psychological complications and phantom limb pain. We were nearing information overload.
Hearing this diagnosis was one of the hardest moments of my life and I am sure my husband would say the same. Hearing about the options and the choice we would have to make left us shell shocked. I knew from the start that Nicholas would need some kind of surgery. I didn’t think it would be multiple surgeries throughout his childhood or one surgery to remove his leg. NYU doc was confident that everyone else he could refer us to would say amputate. There was however, one doctor in Baltimore, he was well known for limb lengthening advancements and for being “a bit of a cowboy”. But he would be worth seeing. So with Nicholas’ prescription pad diagnosis we went home, made an appointment with the cowboy and then I got to googling.
This is our very own Dr.Standard from the International Center For Limb Lengthening. I am so glad he does these chats. They are a great chance for parents to ask questions and gather information without having to flying to Baltimore!
Direct from the RIAO…
Date : Thursday July 30
Time: 9 PM EDT (Note that this is the time zone for Baltimore, MD or Washington, DC)
Temporary password: lldtest
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If you are reading this chances are you like me have a child who has been diagnosed with Fibular Hemimelia. It is a mouthful isn’t it? When my son Nicholas was born almost 6 years ago I would have to spell it out for people. Now he tells them himself and thankfully they never ask him to spell it. Maybe repeat, but never spell. Nicholas has done a lot of explaining lately because his leg is in an external fixator being lengthened for the 2nd time. If you look limb lengthening and external fixators up on the net you are likely to read some distressing things. Nicholas could disprove all of it. My son could be the poster boy for limb lengthening. He climbs rock walls with his fixator on, swims, hops, dances and plays all day. He takes no pain medication. Simply put, he lives his life and he lives well.
Every two weeks we go to Baltimore to the International Center for Limb Lengthening to get x-rays and see Dr. Standard. Nicholas loves him and I am not sure what he likes better seeing Dr. Standard or visiting the cafeteria. Ok the cafeteria probably wins but I look forward to hearing how well my boy is doing and seeing the new bone growing within the rings, wires and pins that make up his frame. His checkups thankfully have been joyful days.
I wish I knew 6 years ago when I was a scared new mom that Nicholas really would be ok. We did not know until he was born that he had a limb defect. I consider this a blessing because I had an easy pregnancy. Had I known I would have been so stressed and scared. Having him was amazing. Not amazing in the way they portray birth on TV, certainly not pain free and not at all fast. What amazed me was the sight of him. I know I had never seen a more beautiful face. I know many moms say this, but really my son was gorgeous. Not squishy, just beautiful. Right after being born he lifted up his head and looked up at this father. If you know anything about newborn’s then you know he should not have been able to do that. While they were checking his stats and cleaning him up I realized something was going on. Steven told me there was something wrong with his foot. Everyone was really calm so I was too. I delivered him with midwives and they were the best. The first thing they told me was that I did not do anything to make this happen and that he would be ok. I tried my best to believe them. Either way I was in love.
So if you are reading this because you scared like I was, I want to tell you that your baby will be ok. Fibular Hemimelia does not have to define your baby, you as a parent or your life. There are options and choices to make. But for now just enjoy your baby. That was the best advice I ever received and it came long distance from my Mom. We were on the phone and I was stressing wondering what caused this and what to do, and she said “Jen he’s a baby, just enjoy him”. So thats what I did.