Thursday, April 12, 2007

Legg-Perthes and us....

A good friend asked me the other day if I remembered that I had a blog.  I laughed.  As it turns out, I've tried to blog 20 times in the last couple of months...but the words are slow in coming.

Strange how easy it is to cry to someone in person and how difficult it is to put those words down in a coherent fashion here...but I'll give it a go...there is always the draft button .

Some of you know that my Beanie was diagnosed with Legg-Perthes back at the beginning of  March.  Some of you have been waiting anxiously for word on what the specialists said.  And some of you have been out of the loop all together.

Let me see if I can answer some of these questions.

First of all the diagnosis.   (Oh and Beanie will be 8 at the beginning of May...that may be important)

In February, Beanie started limping.  It wasn't anything big at a matter of fact I wasn't absolutely sure that she wasn't playing.  She COULD walk normally if I told her to, but she couldn't seem to run.   When she did there was this weird cadence to it...normally a kid running sounds like da da da da da da as the right and left hit evenly...she sounded like da    DA da  DA da  DA...she was really slow on that left leg and not spaced evenly, almost like she had to THINK to place it on the ground and she was hitting it really hard.

This went on for about a I said, I wasn't sure it was anything and she didn't complain of ANY pain...swore that nothing hurt.  But after about a week I took her to our chiropractor. 

He checked her feet, knees, hips etc.  The whole thing was out of whack...her feet were almost an inch different (not normal) he adjusted her left hip.  She got up and while better still limped.  Over the next couple of days he checked her and adjusted her hip, her lower back and her knee.  She was better but not all the way. 

We waited a little longer to see if she just needed to heal a little and then about a week later (March 5, 2007) went to the pediatrician.  This was a new pediatrician to us...but it was pretty much love at first visit.  She is wonderful and kind and helpful and I'm so glad that I found her.  During the visit we talked through how this thing had started and what we had done so far.  She found that the left hip had very limited outward rotation...if you think about sitting cross-legged...that is what we are talking about here.  Basically Beanie could sit cross legged on the right with no problems...but the left couldn't travel very far that direction and even with the dr pushing it...the hip didn't roll.

So directly from the pediatrician's office we went to Baylor Hospital and got blood work and an X-Ray.  Bean was SO brave.  The X-Ray didn't faze her...but that blood test...whew...she cried big crocodile tears the whole way through but she didn't move a muscle!!!  She didn't even bruise...I personally have teeny tiny veins and I never sit there crying and they still ALWAYS pop them...apparently I still don't sit still enough...I was very impressed.

Tuesday, the pediatrician called and said that the blood test came back perfect...there was no elevated white other things that might have crossed my mind.  Praise the Lord.

Wednesday, the pediatrician called again.  It seemed that the X-Ray showed an abnormality in the left femoral head consistent with Legg-Perthes Disease.  I was stunned.

Legg-Perthes Disease is an interesting condition.  For some reason (I can't find anyone who will even hazard a guess) the blood flow to the femoral head is interrupted for some period of time.  During that time the bone that should have been receiving the blood dies.  At some point blood flow will be restored...but the dead bone is still dead and will go through the fracture or breaking down stage, slough off and then re-build.  Through this process the femoral head is distorted...sometimes becoming jagged and then flattening out.  So instead of fitting properly in the socket of the hip, and rolling like it gets stuck and it slips out and generally doesn't move like it should.  That is why the limp...that is also why her feet were off by an inch.

I know that many of you will run off and google this condition as soon as you are done reading...I'm betting some of you have already opened a new window.  What you will find is a SLEW of information...some very old...some very scary...suffice to say during the week and a half of waiting to go get the MRI done...I read a lot of them.

We did get an MRI done on a took an hour and a half...Bean had to be sedated.  She made it through wonderfully...she didn't even cry when she woke up which is pretty common with the drug that they used (Sivo).  And then we waited again.

Finally on Monday, my pediatrician had the results and through some medical jargon it was confirmed that indeed we were looking at Legg-Perthes and we must get to an orthopedic soon.

Problem was, we didn't know any.  My pediatrician called all the orthopedics on my insurance...none of them would take her...the case was too specialized.  So she called a friend who was an orthopedic and he said..."She has to go to Scottish Rite".  Now Scottish Rite is the very best hospital (in the state) for orthopedic problems in kids.  It is WHAT THEY DO!!!  But it sounds like you have to be referred there by someone who knows someone OR a mason.  Guess what...her friend referred us.

During the doctor's call to me, Scottish Rite called and set up our appointment.  It was for April Monday before last. 

Well...they looked her over...checked her X-rays...did some more...and said that it's a hard decision.  If she were 8 and had gotten the disease after she was 8 it would be more clear cut.  Over 8 they often do better with surgery at the outset simply because they have fewer years to grow and reshape the femoral head.  Because she got the disease at 7 and she has a young bone age is uncertain if surgery would be the ONLY option to a good outcome.

They would still LIKE to do surgery.  The surgery that they suggest is the Osteotomy (varus - which means turn in).  This surgery would cut the bone that attaches the femoral head to the femur and then turn that femoral head deeper into the hip socket.  They would attach a plate to that cut section to strengthen it.  The socket would help the femoral head to regrow properly...kind of like squeezing toothpaste into a cup, how it would form the shape of the cup...with the femoral head that deep into the socket the bone would be more likely to grow in the proper shape for the socket.

As far as recovery from the surgery, I'm unsure of the exact casting involved but they did say it was an 8-12 week recovery...from what I can tell online most of these involve a cast from armpits to upper thighs...but I'm not sure of actual flexibility because the drs did say they would need to teach her how to use crutches or walker etc.  So that is a question that needs answering.

Approximately 1 year after the original surgery, they would need to go in to remove the stabilizing plate so that perhaps her bones would straighten out on their own.  The drs were pretty vague in this area.  He said that there would be a limp from the osteotomy, however if the limp was unacceptable at the end of the disease...when her growth plates close then they would consider the reverse surgery - Osteotomy (valgus - or turn out) which would involve the initial surgery and the plate removal again a year after that.

Here is the problem at face value...the disease process itself (bone death, fracture stage, sloughing and regrowth) is 2-4 years...Sarah is not quite 8, at worst she would be almost 12 when the bone is fully regrown in whatever shape it's going to be.  They would not reverse the osteotomy until her growth plates close which is approximately 2 years after menstruation...if she follows her mother which they seem to do, I was 14 at menstruation which would say my growth plates didn't close until I was around 16...while there are a lot of "IF"s here...that would be around 8 years of limp for a maybe 4 year disease.

The other problem is that even THEY are not sure that the surgery is absolutely necessary for a good outcome for 2 reasons.  #1) even at almost 8, with a 5 yo bone age, she probably still has a lot of growing to do and at BEST it puts her on the line of maybe yes/maybe no on the surgery itself.  #2) Even with the MRI the doctors STILL do not know how bad this is going to get.

Something else about Legg-Perthes...there are different designations if you will...there are A, B and C.  With "A" just a little bit of the bone dies - these have the best outcome.  "B" more of the bone dies but not 50% these usually still have a good chance of good outcome with either surgery or not if they are young a matter of fact had she been 1 year younger surgery probably wouldn't have even been on the table.  "C" 50% or over dies.  These have the worst chance of a good outcome either way because so much of the bone dies and has to regrow, there is a much better chance that the bone will grow back malformed.  In the case of a C, the osteotomy gives the best chance at a good outcome, because there isn't any other way to keep that bone in the hip socket...there is not enough for it to hold on to.

With Beanie...even with the MRI...the only thing they are SURE of is that it will NOT be an A...we are in the B stage at this point.  But they don't know if she's on the up side or the down side of the illness...she was not to the fracture stage yet so until that happens, we don't know.

Dave and I have NOT made our final decision yet.  We are really struggling with it.  We are talking about making a MAJOR decision based on a gamble...roll the dice...she is on the upside, she is careful, watches out for her activity level, takes anti-inflamatories and in 4 years she's a normal kid again...roll them again...she is on the downside and we do surgery and in 4 years we know the femoral head has regrown round and in 3-4 more years she faces surgery again to get rid of the limp that made a round femoral head possible.

Or she's on the upside and we do surgery now and her femoral head is fine because it always was going to be but she's stuck with a limp until she's 16 anyway and then a surgery that puts her on her back for 8-12 weeks as a teenager.  Or she's on the downside and we DON'T do surgery and find that it's harder to fix later with this surgery so it's a bigger deal with hip reconstruction or replacement and all the concerns involved in those decisions.

I will tell you we are leaning toward the wait and see approach.  If we do NOTHING (and if you know us you know that doesn't actually mean "nothing") Scottish Rite will X-Ray her every 3-4 months to keep track of the progress of the disease.  They would like to do the surgery NOW...within the next 2 months...We would like to wait the 3 months and see where we are.

Right now, our plan until we make the decision or get the surgery or get the X-Ray is to keep her safe.  We are working with 2 chiropractors (one a good friend, the other our professional chiro) who have been helping us with physical therapy exercises and checking those hips.  She is taking a lot of epsom salt baths for stiffness and an herbal anti-inflammatory or ibuprofen depending on the time of day or stiffness. 

We've been blessed that other than 1 week she has been pretty pain free...that week was VERY bad.  But at least for today...she is okay pain wise. 

And there you have it.  That is where we've been and where we are headed.  Please keep us in your prayers.