Fused and Fancy Free

I am about to loose any street cred I had regarding knowledge of fibular hemimelia. Seriously. Maybe just stop reading now. Why come here if I don’t know basic information such as…

Nick’s ankle is fused.

I didn’t know that.

My only excuse is that I swear Dr. Standard never used the word fused to describe Nick. Confused yet? I certainly am.

Dr. S basically said Nick’s ankle is very typical for his type of fibular hemimelia. “His type” hmmm… I know it’s type 3 and I know there are subtypes but I have no idea which of those it is. I knew it was the type that needed the super ankle surgery. I knew that there were bone cuts and all kinds of things done in that surgery that I never cared to ask for details on. I have never claimed to understand that stuff. Thank goodness! 

What I do know is that I usually referred to it as on the severe side. That was the type according to me. Severe or maybe I said moderate to severe. Because he’s done so well and we had options. In a way severe sounds like the worst. Nick isn’t the worst it could be. He had bones in his foot to work with.

Anyway the fact that I had to ask Dr. Standard if Nick’s ankle is considered fused felt really dumb. I write about fh here, I support other families through email and the Facebook group. They come to me for information. Like I know what I am talking about, meanwhile I missed this bit of info about my boy. 

We have always focused more on what the result of what was being done would be. The last surgery Dr. Standard put Nick’s foot in a better position and he straightened out his tibia which had a bit of a bend still and lengthened to the point of Nick being even! Thats what that mega fixator did. That’s what I know. But I am thinking I missed something (since I didn’t think Nicks ankle was always considered fused or was that word just not used?).

It also doesn’t matter. Nick has what he has. It is what it is. His fused ankle is working for him. He has a stiff but stable joint. I knew that already. He has no pain. He walks and runs and swims. He talks about trying new things. He does not feel limited by this fused ankle. Fused sounds like a bad word at times in the fh groups… “It might have to be fused”, “Better to amputate than be fused”, and so on but it’s not a failure and not a bad word and just because I am using it to describe Nick now doesn’t actually change anything. 

Except now I can tell parents who fear fusion that it’s just fine! Nick is fused and fancy free. Nicholas will likely tell me tomorrow “I bet fused and fancy free sounded cooler in your head than it did out loud”. And I will tell him it doesn’t matter cause I don’t care about cool and since my street cred is gone already, I’ve got nothing to loose.

 

17 centimeters

Adding up the length from each surgery Nicholas has gained 17cm through leg lengthening. I think he still has 7 left but that can all be done internally. This was the biggest surgery and it is over. Dr.Standard removed Nick’s fixator and put a rod in his tibia this morning! Like most of his past surgeries this has gone pretty smoothly. I did not really cry in the operating room when I left him. Usually the walk back to the waiting area is so hard but I asked the fellow who walked me back what his plans were because I knew his fellowship was ending. He told me that his next fellowship would be in trauma. I said “Trauma really? This is traumatic enough for me”. I guess I must have been ok if I was making jokes!

Since at our last appointment we didn’t have a set plan for removal I really didn’t ask many questions about the rod and for whatever reason I did not realize Nick would need to stay overnight. It has actually worked out so well! At least here at Sinai. I hope the kids are doing ok in PA with Grandam and Grandpa. They sounded great when we called them (they sang happy birthday to me) but I hate to be gone longer than we said we would be. I am so grateful that they are with my inlaws, otherwise Nick and I would have been alone and not had an awesome birthday dinner and movie night here in room 3122.

Nicholas has not been in pain at all. I did ask for his pain meds to be given because I didn’t want his blood pressure going up like last time. Still he seems tired but ok. He is so happy to be fixator free. He was even so calm this morning. He didn’t have happy juice or anything before surgery and had an anesthesiologist today that he had not had before but he was a really kind man. There were plenty of other familiar faces throughout the day. It is still surprises me how familiar this place is and how comfortable we are here. It’s nothing you expect in life but hay it is what it is!

When Dr.Standard came out to tell us how everything went he explained why Nicholas would need to stay over night. It’s really just the antibiotics because of the rod. He has a protocol he follows and I would not question his methods. That conversation we have each time when surgery is done but Nick is not really out yet is such relief. Once I see Dr. Standard and he says everything went great I finally exhale! We always talk a little about whats next but this time whats next should be really far away. Nick can have the rod in for years if it does not cause any problems.

Dr. Standard has been Nicholas’s doctor through this whole journey, each surgery has been at Sinai, each time we have family and friends helping along the way and every outcome has been excellent. Every day I am grateful. Without the love and support of so many people this really would not be possible. Nicks right leg may be longer or he’s finally even. That is amazing! If he had had no surgery his right leg would already be almost 8 inches shorter than his left! That seems crazy but thanks to 17 hard earned centimeters Nicholas has a far different reality!

Summers of Surgeries

Some days I really can’t be positive. So I keep quiet. It was a rainy day anyway. No need to venture out into the world which is good because I happen to be pretty annoyed at the world. I am just tired of Nick’s summers being so surgery filled. Last year on June 17th (his baby sisters first birthday) it was the knee. This year on June 22nd (my birthday) it will be fixator removal. I could add previous surgeries to this list but you get the picture.

As much as this is a surgery to be celebrated it is hard because we have no idea how he will feel after it. He might now want to walk for a while. The rod could cause him pain. Who knows! I just want Nick to be able to relax and just be a kid for a while. A really long while!

I also sometimes realize how much it impacts the other kids. We wont be going to amusement parks until Nick can go on rides. Would it be more fair to take the other kids and leave Nick behind? I don’t think so. Is it the end of the world not to go to amusement parks? No but when I think of how few times we really have gone it is kinda sad. There are other things I don’t do because it is hard with 4 kids (and really it’s just hard cause one is in a fixator and one is about to turn 2). The kids also have fifth disease and the rash from it seems to be gone until they are out in the sun and then it is red and itchy for a while after.

I guess we’re home bound and a little stir crazy. I took them to Target yesterday and the best part was that I could get them all in one cart. People found the sight of us quite amusing. Big kids, little kid, baby and stuff all in one cart. I counted pushing it around as a workout for me.

I think the moving factor is changing this summer too. We’re in a kind of limbo and I am tired of it. So really I am just complaining which is a waste of time and energy considering the kids are asleep and I could be asleep. Plus I realize I have much to be grateful for. I just wish this summer was easier for my boy and I guess the rest of us too.

Fibular Hemimelia Before and After

I’ve been asked to provide some before and after photos of Nicholas. That’s kind of hard to do since at the moment he is in a kind of before situation. Being that he’s currently undergoing treatment. It was even hard to find great photos from between previous surgeries. It really reminds me of the fact that between surgeries we don’t pay a whole lot of attention to his leg.

I guess that is truer at some times than others. The fall was hard with Nick having random pain for the first time. Between surgeries one thing that always reminds us of fibular hemimelia is shoe shopping! That seems like it will never be easy. And getting shoe lifts, which either seems super easy or a real pain. Once we find someone who does a quality lift at an affordable price we pray they will stay in business.

Apparently though Nick does not need a lift right now! He’s walking with the walker but still reluctant to put weight on his right foot. I worry he is going hurt his left foot with all the work it’s doing! Still he’s not terribly active right now and now that the infection is under control we’ve got to encourage him more (I guess the swollen red infection pictures are a kind of before. I should take some nice afters to go with them). Steven is away and told Nick that if he takes a few steps each day he’ll take him to McD’s when he comes back.

This time when this fixator comes off I will be sure to take lots of awesome after photos. I hope it will be a few years before Nick will need another surgery. Though the way my boy grows it is hard to imagine going too long without another lengthening! Hopefully I will get to posting some more before and after pics on this blog later today! For now check out the new header collage I created this morning between toddler temper tantrums, preschooler melt downs and eight year old whining (that’s what Nick has been doing whenever we have to go some place without his dad-evator (as in Dad as elevator)! Considering how little attention I had to give it I think the header is fabulous!

Fixator-less Pinsite Infection… not so much

We had a bit of a scare recently. Nicholas has a random high fever and headache for about 4 days. When we took him to the doctor we also mentioned an itchy red spot on his leg. The doc noticed it was right on an old pinsite. Nicholas always had headache’s with pinsite infections. He had no other symptoms so his doc thought it could be an infection in the old pin site. Apparently infections can lay dormant or something! It also happened to be a pinsite that had quite a few infections when he had his fixator on.

Luckily this diagnosis happened on a Thursday! Chat Day!! I was able to explain it all to Dr. Standard that night. And the strangest part at the time was the fact that by the time I was on chat that night the red spot was gone! Old pinsite scar looked like an old pinsite scar and nothing more. Dr. Standard mentioned some possible things that could be going on (including bone infection) and said he could see Nicholas in the morning. Nick had an appointment Monday anyway but I was relieved to get to take him in sooner.

At this point Nick and I enjoy these little trips. We get so much time to chat just the two of us. It is a 2 hour drive from our house in PA to Sinai in Baltimore. It is all so familiar to me… the curves of the road, the river, the exits with good eats and clean restrooms. I try not to stop on the way down though.

Friday is not  a usual clinic day for Dr. Standard but it still was not much of a wait for us. He must have come between surgeries. Thankfully Nick’s x-ray’s looked GREAT. His bone looked prefect! (his eight plate is still doing it’s thing as well) I did not realize just how worried I was till I felt the relief wash over me when Dr. S came in and said all was well. Now we know it was a virus since my 3 other kids had it too but at the time it was just Nick, who hardly gets sick and it was scary!

At the appointment Nick was scheduled for on Monday we were to schedule Nicks knee surgery so we did. Dr. S said we could do it in June or September. We were trying to make sure Nick has no limitations during our August family vacation!! We chose June since I would rather not interfere with school if we don’t have to. So on June 17th (Nick’s baby sisters 1st birthday), Nicholas will have his knee surgery. June 10th was an option but we’ll be at Jellystone Park with Dr. S and other Sinai families that weekend! Somehow we were able to plan around all our little vacations! Dr. Standard’s new secretary Ann (not so new actually but we had not met since Barbara was still Dr. S’s secretary when Nick last had surgery and we loved Barbara) came by to tell us what dates were available. Summer is so busy at the RIAO I think we were lucky to get Nick scheduled for June.

So now the countdown begins.

I would love to write out all the details but really I did not ask for them. I trust Dr. Standard and that he knows what to do. I know he is getting a ligament from a donor. I have previously described the surgery as similar to an acl replacement. Nick’s knee is loose (or loosey goosey depending on your particular lingo). It moves in ways it should not and while he has no troubles now there is the potential from big trouble. Nick’s knee could get stuck in the wrong position which would require some much more serious surgery.

I guess this is small compared to a fixator but it is still surgery, a few days in the hospital and 6 weeks recovery! Nick has art camp in June but I am thinking a sitting activity will be just the thing for him. He’s taken abstract art and clay this year and he loves it! I know he’ll be upset not to be able to go swimming for a few weeks but we’ll make the  most of the time we have and not put him in situations where he is watching other kids do something he can’t. That worked well when he was in the fixator. We did not go to amusement parks and such because come on, fixator and roller coaster just don’t seem like a natural pairing!

I know he’ll be ok. His knee will be stronger and I will really feel that he is over the hump in terms of surgeries. More than 1/2 way to the light at the end of the tunnel. The light being 2 legs, same length, with stable joints, carrying Nicholas into his future. We’re getting there!

No Fibula, No Problem

On the limb differences chat at the moment and someone was looking for something to put on a t-shirt to fundraise for fh and it hit me ‘No Fibula, No Problem’. That’s the reality for Nicholas. He has no fibula on the right side and ok I guess it is a problem in that it requires treatment but he is still living and loving life without it.

First grade is awesome so far. Nicholas loves his teacher and his principal even told me tonight how impressed she is with how his leg is doing. He also stops to chat with her in the hall way. Not sure how many kids do that.

Nicholas has had some pain behind his knee but Dr. S has suggested we do some stretches at night. So I am not really worried about it so long as it stops. Otherwise we will be on our way back to Baltimore. We wont be going back till October unless something happens or this knee business continues.

Some seriously sad news… our local shoe hospital has closed it’s doors. This was an old fashion shoe guy who also happened to sell roasted peanuts but the important part was his lifts were awesome and cheap. Now we are paying over $100 for one shoe!!! Thank goodness Nicholas his happy with one pair.

I have not been on the fh support site or the fh Facebook group as much as I would like but school starting has made life so busy and the days go even faster than ever. I am going to put it on my calendar as a reminder to check the groups. Being there for others is so important to me. As a mom it means so much me to be able to use our experience in service to families new to this world of fh. The chats are just one awesome way to do it and of course asking questions real-time is great but the parent interaction and support is wonderful. And I love reading about the kids who are having surgery and doing do well, when I can remember chatting with their parents when they were babies, or receiving emails about newborns with a new diagnosis of fh. When I first came in contact with families with fixators it was life changing. Knowing that we were not alone meant more than I can say. By the way, if your reading this because your child has a diagnosis of fh, you’re not alone either! Come to chats, email, join groups, do whatever you can to get the support and answers you need.

Back to Baltimore

Heading down for the 8 plate insertion… small outpatient surgery… small outpatient surgery. Thats what I keep telling myself because it’s really the truth but it’s the fact that it’s a surgery that gets me. Anesthesia is a scary thing but I figure, in the rational part of my brain, that this being Nicholas 6th time going under I can reasonably assume he will not have any anesthesia problems.

He is truly crazy excited to go down there. No stress on his part. I am grateful that through all of this he is still such a positive and happy boy. This is really small compared to all the other surgeries but maybe my pregnancy is making me more on edge… well at least I have an excuse.

Big Blue Cast

Friday night: We are home and Nicholas is in a huge blue cast that goes above his knee. He is resting and playing computer games, which may be our solution to forcing him to take it easy! He is on pain meds for now and a little grumpy. One minute he is thrilled when he remembers that the fixator is off, the next he is mad because we wont let him walk.  The surgery was so quick (eight plate not needed at this time). Taking him back to the operating room was as hard as it always is. Dr. Standard had the great idea to tell him that he could pretend the mask they put on to make him sleepy was a pilot’s mask. So as he was laying on the operating table and he had that moment where he seemed uneasy I talked to him about his jet flying to Brooklyn to go trick or treating with his cousins and that made him happy.  Walking away with him lying on that table is so hard. I really was not worried about Dr. Standard’s part of the surgery at all. I knew his leg would be fine but I always worry about the anesthesia. Even though this was his 5th surgery I still worry the  same way.

It really is such a relief to have the frame off and yet I find myself on the edge of tears. I think it is the stress of the whole thing coming out. I also feel like I got hit by a truck. My whole body is exhausted.

Thinking about Nicholas spending a month in that giant cast is daunting. I really can’t lift him now and that will make it so much harder. Plus we have too many stairs in this house!

Today:  Nicholas is in school and insisted on going back on Monday. In fact he was mad on Saturday not to get to go to school even though we explained that no one was at school. He is doing really well in the cast, walking with the walker for the most part. Some scooting and sometimes he walks by himself when he does not realize he is doing it. We sent him to school with the wheelchair in case he needed it.  I don’t think he needs it but we shall see. His teacher has been amazing through all of this and I feel like she really looks out for him and really I am only upset to leave him at school the moment I have to walk away. Not sure why I am still sad to leave him there. I prefer Steven to take him to school to avoid the feeling all together.

“I’m okay. It’s just an external fixator for leg lengthening.”

Today we got back from a great little vacation.  We were upstate NY for a few days and it was perfect.  We swam, relaxed with family and ate great food that I did not have to cook!  Nicholas did really well.  He is still not walking like he was before the removal of the foot plate but he is trying.  He wants to walk but gets tired because he still is not fully weight bearing on his right leg so he limps. He really is self conscious about it. He told Steven that he did not want his cousin Kentie to see him limping and that he was embarrassed that he is not walking. We did our best to reassure him but clearly it is bothering him.

Swimming was great and was a great break from walking and the playground, which is where he spent good deal of time too.  He climbed all over the place without any trouble and met lots of new kids and answered lots of questions about his fixator.  At one point someone asked him about the cover he had on it and he thought the woman as asking him to show her what was under the cover so he told her “I like to keep that private”.  It was cute really.

Our only real drama was that a set screw came off of one of his half pins.  The previous night Nicholas had banged his leg really hard on the bed frame and even bled around one of his wires.  Nothing appeared to be wrong at the time  and I am not sure if this had anything to do with the set screw but Steven fond the set screw off in the morning.  I would never have known what it was had I found it so I am so glad it was Steven. I asked him to call Dr. Standard because he could explain it much better since he actually knew what it was.  This explanation is really inadequate too! Dr. Standard returned our call and left a message saying that Seven should put it back in and to call  him back if there was any trouble. Thank goodness the place were were staying had an extensive work shop where Steven found an Allen wrench and secured the half pin.

Crisis was averted and we really had a lovely time.  We had to come home a bit early for Nicholas’ kindergarten open house.  New situations do make me nervous for him so it was important to me that we be there.  Worrying that someone will say something that will hurt his feelings occupies me more than it should.  I wanted him to get to meet some kids before the first day since we really don’t know anyone else starting kindergarten this year. When we got there I found out that his teacher did not even know about his fixator. I could not believe she had not been given that information after all the people I talked to and paper work I filled out. It irritated me that I had to explain it all to her when we were there for Nicholas to meet her, meet some kids and see his classroom.  He walked away and introduced himself to a shy little boy while I explained things to his teacher (I am sure this explaining has gotten boring for him to hear).  She was very nice and I am sure everything will be fine but I will still worry.

Many of the kids played on the playground after the open house and Nicholas was no exception. Although I was the only parent who was standing within earshot of the kids.  I knew someone would ask him about the fixator, plus I wanted to be close by in case he fell or something.  When he was asked about it I did  not hear what the question was but I heard Nicholas say “I’m okay.  It’s just an external fixator for leg lengthening” then they went back to playing and it never came up again.  Now I just have to believe it myself.

The Same

Someone once said to me that they thought it was great that I treated Nicholas “the same”. I can’t remember what I said for sure but I remember thinking, ‘how else should I treat him’.  From the day he was born I felt, with certainty, that everyone would take their cue from me, so I needed to set the tone.

But it’s not that simple.  It hit me on Sunday when our minister, Barry Stopfel, was doing a sermon that largely was about differences (It was about more than differences but this is what I took from it on Sunday).  Our differences, whatever they may be shape our lives.  Our experience effects our perspective and how we see the world. Do we spend so much time worrying about seeing everyone as the same, that we miss who they are in the process. Fibular hemimelia is a part of my boy, maybe hard to miss while he is in his fixator or in shoes and long pants but it is still there because it has changed his life inextricably.

I have tried so hard not to make fibular hemimelia who he is. Nicholas only leaned the word a few months ago and I think I should have told him sooner.  It almost seemed like things made more sense to him when he had a word for it. In the past when he asked why his leg and foot were different, I would say ” because that is how you was made in my belly”, or “that is how god made you in my belly”.  I was so worried about him being defined by his difference without realizing that maybe he has been refined by it.

Fibular hemimelia certainly shaped me as a parent, from day one.  First off I was grateful that his health was excellent because once one thing goes wrong, you begin to realize anything can go wrong and it is easier to be grateful for what you have.  Secondly, I wanted to make sure to give him the best and strongest foundation of love at home, hoping he would then be better able to handle whatever happened when he was out in the world.  I was sure that if we accepted him, just as he was, then he would accept himself too.  And I believe he does. Nicholas does not hide his leg.  He will gladly tell anyone about it.  Fixator and all he is comfortable with himself.

Nicholas thinks we are all original in some way. According to him, I am original because I have a five head instead of a forehead (or because I am child size by his estimation). Wayne at church is original because he has a one hundred head (picture a handsome man who also happens to not have so much hair). Steven is original because he is lacking in the booty department, and so on. Is it the same as having fibular hemimelia? Not really, but to a six year old life is so much simpler (Nicholas asked on the fourth of July while talking about patriotism “why cant we just love everyone in the whole world”).

Simple? It should be.  Accepting someone does not mean looking past or forgetting parts of them, which do make up who they are. It isn’t treating them the same as everyone else.  Why do we need to be the same or be treated the same? None of us are the same.

I am so proud of my son for his strength and courage.  Is he the same as all the other six year old boys? No.  He has been through things most children could not imagine.  He has compassion and courage beyond many adults I know. That is a part of who he is.  Maybe it’s not because of fibular hemimelia and maybe it is.  Who’s to say?

I am teaching Nicholas what I believe to be the same about everyone, although he has likely grasped the concept already, that we are all the same in worth and dignity and you can love everyone, even if it’s not the same kind of love, without ever having to forget or look past any part of who they are. Whether it be a smaller leg, a larger forehead or a booty-less back side.