Her 2nd Limb Lengthening

It's early winter of 2019.  Katelyn has had such a wonderful 2.5 years from her last lengthening. She did have a small outpatient surgery last year to remove the screw in her 8 plate but it was fairly simple in terms of surgeries.  I'm so thankful that we've had very little procedures since she was born.  I think this is surgery number 5 in the 6 years of her life and while that does sound like a lot quite a few of them are grouped together so there are many years in between where her FH doesn't come into affect her life.  When she was born and we were given the really difficult choice as to keep her leg or amputate, some of the agony that we had was if we would be subjecting her to a childhood of surgeries if we chose to keep her natural leg.  If you are a new parent of a child with FH that has just found this blog online I can tell you that so far, (albeit we're only 6 years into this) it's been worth it to keep her leg.  The length difference doesn't set her back at all and she's really grown to love her little leg.  There's never been any anxst over her difference until this point and I don't regret for a moment keeping her leg.

Skiing All Day- Age 6- 10.5cm difference 

Nothing Stops Her!

Which brings us to today.  Next week, Wednesday, January 30th, Katelyn is set for her next leg lengthening. This time it's on her femur (thigh bone) as that bone is also short.  It wasn't short at birth but has progressed to a point that it needs intervention.  Here is a recent x-ray.  She's not standing on a block that is the full height of her discrepancy but if she were you could see plainly that her left femur is shorter than her right.  The valgus knee positioning (knock knee) has returned which is disappointing but all part of dealing with a progressive condition. The 8 plate that is in her knee now will be screwed down again during the lengthening surgery which will push her knee out to a straighter alignment as she grows.  This will also give her a little bit of added length as the bones come back into proper alignment.

At it's basic form, this lengthening is the same principle as her first lengthening.  During surgery the doctor does a horizontal cut in the bone and through a series of weeks and bedrest the bone is separated multiple times a day and the body continues to regenerate marrow in the gap that is created.

But how is the gap created in her bone?

The process and technology has advanced in just a few short years and now looks a bit different.

 *If you are new to our lives within the past few years I'd suggest you look up her first lengthening on the blog so that you will understand some of the technical terms.*

In order to avoid the use of the external fixator (because it had serious downsides, open wounds, heavy medications involved, scars, emotional trauma etc) new technology was developed over the last few years to do the bone lengthening from inside of the bone itself.  Or the creation of an internal fixator.  A small rod was invented to be placed inside the bone and then through an external receiver the rod is activated  and it expands, thus opening the break that was created in surgery.  The body then regenerates marrow in the gap that the rod keeps creating.  This procedure has only been limited to patients who are almost fully grown as their growth plate is damaged when the rod is implanted. This is where our doctor is willing to take it a step further.  With the consent of the parents he implants the rod alongside of the bone with it being screwed into the top and bottom of the femur so that it doesn't damage the growth plate.  And it's actually working!  The doc in Minnesota has done it 15 times and the doc here has done it twice.  Katelyn will be the 3rd patient for the Ohio doctor to receive this type of implant at such a young age.  Here's a peek inside the little leg during lengthening.

This x-ray was taken towards the end of lengthening and you can see the new growth being formed in between the expanded break.  The best part is this is all inside of the leg so there is no external fixator or cage with screws that need to be turned etc. in order to open the break. It all happens inside of her body with the help of the parents who use the activator to communicate with the rod.  Every 2 hours for 30 days, during waking hours.  We'll also be doing physical therapy at home and in the office to stretch the soft tissue as it is not lengthened surgically, so it just has to stretch to meet the new growth.  After the 30 days of lengthening comes the consolidation phase (bone hardening) in which there is very little weightbearing.  I believe this phase is 8 weeks...and weightbearing is increased by increments as well as re-learning to walk.  It's a long marathon but our hope is that if we start this process now she'll be back to normal by spring and ready for full play outside once the weather has turned and school is out in May.

She's handing this all in stride.  She is fully aware of this surgery  as it was too big of a process not make her aware.  Last week was her last gymnastics class which she loved and we'll see if she wants to return next fall.  Most of what she loved about gymnastics was time with her cousins but if she wants to return in the fall we'll support that fully.

Triplet cousins at gymnastics

We're hoping to have her return to school in 4-5 weeks once the every 2 hour care is over and she's comfortable moving about in a wheelchair.  Here's a quick clip of how she told her class.  The whole presentation was about a 15 minutes on why her leg is little and why she'll be gone for a few weeks.  I'm so grateful that at this age they are so loving and accepting.  Their biggest concern was who will get to deliver her mail at home and who will get to push her wheelchair when she returns.  Gotta love this 1st grade class and a wonderful teacher who God knew she needed this year.  It's such a huge testament to how God works even through the smallest details of our lives.  

And that's all for now.  There will be the removal of the hardware one day too.  It will come either when her body tells us it needs to go or at best next year if we are lucky.  One hurdle at a time though. For now we're concentrating on growing this little leg and the days ahead.