Ava's Diagnosis

When we heard the first orthopedist tell us that they suspected that Ava had 'avascular necrosis of the hip', our minds reeled.  Anything with NECROSIS in the title is scary stuff.  That means something in your body is dying.  And that can't be good news.

So, what is the first thing any 21st Century mom does when she hears that her child may have a disorder/disease/condition?  Head directly to Google!  It didn't matter that everyone tells you NOT to do that.  It's instinct now.  But google can be a very scary place, especially for a parent who has just been told that her child has something wrong with them.

My first few searches had me confused - as this disease does not seem to have any cause, nor any one defined treatment.  It depends greatly on multiple factors but the greatest is the age of the child and the severity of the necrosis and "stage" of the disease.  But after wading through all the technical terms, I learned that the disease is called Legg-Calve-Perthes Disease.  Not because it affects the legs or the calves at all.  Rather, it is named for Arthur Legg, Jacques Calve and Georg Perthes, all orthopedic surgeons.

This is a very good, brief explanation: http://nonf.org/perthesbrochure/perthes-brochure.htm

So, basically, interrupted blood flow to the head of the femur bone causes the bone to start degenerating.  The continued pressure on the hip joint causes the femoral head to flatten and disintegrate, causing pain and decreased mobility.  That's why Ava was limping for the past 4 months and complaining of pain in her upper leg for the past month or two.

Historically, treatments have NOT been very child-friendly - even though this is a childhood disease. Here is an ABC Report on Perthes Disease done by Cameron Mathison who had this disease when he was a child.  He had to wear a horrible looking brace/cast for 4 years!

Unfortunately, there are some hospitals who are still treating Perthes this same way - bracing or casting children for YEARS as the femoral head regenerates.  If a child is diagnosed young, they sometimes take a 'wait and see' approach and have the child use crutches or walkers or wheelchairs until they decide whether or not surgery is a good option.

Ava is considered 'old' in the area of bone growth and development.  Yep, she's 'old' at only 8 years of age!! So, a wait-and-see approach was never really an option for her.  And, honestly, I can't imagine restricting her that way for an extended period of time anyway.

We were set to have a follow up appointment at Johns Hopkins with the orthopedic surgeon who mentioned Perthes, but in my online research, I found a couple support groups on Facebook where there was a good bit of chatter about a newer approach to treating Perthes Disease without incapacitating children for years on end.  Two doctors on the East Coast pioneered this new treatment plan, one in Florida named Dr. Paley and one in Baltimore, MD named Dr. Standard.  I immediately started a web search of Dr. Standard and his plan.  I was so excited to see that someone so close to us (5 hours away was way better than across the country!) seemed to be 'the man' to go to.  The feedback online was great.  His patient satisfaction was high, people raved about his bedside manner, and his skills seemed to be incredible.  I found this video which pretty much sealed the deal for me and had me calling his office that day for a consult appointment.



When I called his office, I was told by the receptionist that his first available appointment was July 16th.  I was crushed.  I had my heart set on meeting this doctor and having his expert opinion on how we should proceed with treatment.  I went on the forum to lament with others about the situation and lo and behold, the first person who responded was a NURSE from DR. STANDARD'S OFFICE!  I was shocked. She messaged me, was so sweet, gave me her personal phone number and told me that she would have his secretary call me the next day and they would fit me in the following Thursday.  How is THAT for customer service??  Thank Goodness for Facebook!

So, we met Dr. Standard the next week and he confirmed the diagnosis, did an exam of Ava and then talked to us about our options.  He explained that he and his colleague, Dr. Paley in FL, developed this treatment plan because they have children and they couldn't imagine immobilizing a child the way these other treatments do - for the better part of their childhoods.  So, his plan allows the child to be mobile and put about 50% of their weight on the injured hip during treatment.  It also speeds up the process of the disease considerably.

Here's the basic facts:  The bone is going to die and then regenerate no matter what you do.  If you leave it alone (with casts, braces, walking or not), that will happen.  The danger to doing nothing is that as the bone regenerates, it may not grow back to it's original round shape, fitting perfectly in the hip socket.  If it is misshapen or doesn't fit properly, it will cause pain and arthritis and often leads to total hip replacement surgeries - many times at a very young age.  Like, teenage years!

So, the plan for Ava that Dr. Standard advocates is surgical.  They will first go in and drill holes in the femoral head to encourage the bone to break up more quickly.  Then, they will take her stem cells (from her hip bone, I believe) and insert them into the femoral head to stimulate regrowth and make the regeneration process faster.  This way, instead of taking 2-3 years for the bone to die and regenerate, it will take approximately FOUR MONTHS.  (Isn't that amazing!?)  There are a few other pieces to the surgery that we are not completely sure he's going to do with Ava, but one is a tenotomy, where cuts are made in the tendon of the leg to release pressure and help with mobility.  Then, they will surgically apply an external fixator onto Ava's hip and leg.  Pins will be put into the leg and they will stick out through the skin onto the fixator.  It reminds me a little of a halo that people have to wear after breaking their neck - but put on the leg/hip instead.  This fixator will pull the femur bone away from the hip so that she can continue to be somewhat mobile (walking with a walker - crutches eventually if she's coordinated enough) to encourage mobility and flexibility, but also to avoid the head becoming deformed or flattened as it regrows.  She will have to have that on for the full four months of regrowth and then there will be another surgery in October to remove the fixator.  After that, she'll have about a month in a brace and then she should (God Willing!) be slowly released to more and more activity until she's back to normal activity only 8 months after the first surgery.  So, the goal is full mobility as normal in 8 months instead of years of treatment.  That is why we are going with Dr. Standard and his plan.

(I *think* I have most of those details correct.  I am still learning about the disease and treatment plan details, so bear with me if I have something mixed up) Here is a photo sent to me by another family of their son who had this same surgery with Dr. Standard in Dec. 2012.  This is the external fixator.

It is somewhat scary to think about having this big thing drilled into my little girl, but after meeting Dr. Standard and taking a tour of the children's wing of Sinai Hospital and the physical therapy department at the Rubin Institute for Advanced Orthopedics, we are confident in their plan, their facility and the entire staff.  I have literally heard nothing but positive reviews from everyone who has been treated there - not just for this disease, but other orthopedic conditions.  Every person seems to absolutely love Dr. Standard and his staff and they all rave about how they were treated, how their child was taken care of, and every detail.  Dr. Standard explained everything to Ava, drew her a picture of her bone and explained what was happening and what they would be doing.

Funny story, as he was explaining the bone dying and all that, Ava totally interrupted him and said, "So, what are we gonna do to fix it?"  She is SO my child.  LOL!

We are anticipating that Ava may have a more difficult physical therapy recovery due to an underlying skeletal issue that we have not yet been able to diagnose.  We have been to the Genetics area of John's Hopkins twice now and they are trying to figure out what, exactly, is going on with her bone and joint structure. She already has decreased mobility and flexibility in all of her joints - including the hip joints, so we are a tiny bit fearful that it may affect how well the treatment works and how well she does with PT.  We were told about 4 years ago that Ava has femoral anteversion, but Dr. Standard said that he is not sure that is actually the case.  He'll be able to do a better exam of her mobility when she is under anesthesia and will have some answers for us about this issue after her first surgery.

We will be in Baltimore for about 2 weeks (depending on how well she does with PT and when they release her to come home and continue therapy at CHKD (or another center yet to be determined). She will be able to swim (Hallelujah!) this summer, but will have to use a walker or wheelchair to get around until the external fixator (heretofore referred to as the 'ex fix' like all the cool kids) is surgically removed sometime around October.

I will be updating this blog as we go forward on this journey, so stay tuned for all the details.  We are set for surgery on June 13th.  The scheduling department said, "I hope you aren't superstitious.  It is Friday the 13th."  I said, "I laugh in the face of fear".....we'll see how I do when they wheel my baby girl into surgery with that.  Ha!

Thank you to everyone for your continued prayers and good thoughts for Ava.  And for those of you who donated through my GoFundMe page, I cannot even begin to tell you how much it means to me. We truly appreciate it!