Ok Here's the Plan

So this is what we learned from our visit with Dr. Paley.  It started with an x-ray and then a consultation.  He basically told us the basics about PFFD or what he now calls CFD (congenital femoral deficiency).  Its delayed calcification of the femur and hip bones.  In Nash's case it is unilateral or only on one side.   He classified  Nash's severity as a 1b.  On a scale of 1-4 , 1a being the best and 4 the worst or hardest to fix, that was good news.  He said his knee was normal and his ankle was good, too.  That was also good news because sometimes those can be deformed as well.   He wasn't sure if he has an ACL on his right side but he will know more when he does the first surgery.  That will be the "Super hip" surgery he will get when he is 2 yrs old.  It is an extremely complicated procedure where he basically reconstructs the hip, the joint, and top of the femur.  He has to work with all the nerves, ligaments, bone and muscle.  There is an incision from his hip to his knee.  Its a lengthy surgery and Nash will be in the hospital for 3 days and we will need to be in Florida for 2 weeks.  He will be in a cast from his hip down with a bar between his legs for 6 weeks.  He won't be able to crawl or walk, just scoot.  The cast will be removable so that will be nice for bathtime!  If all goes well he will be ready for his first lengthening a year after the hip surgery.




Doctor's office in
West Palm Beach, Florida

 

So that brings us to the lengthening process.  Dr. Paley said that if we didn't do anything and just let Nash grow naturally, he would have a 24cm length discrepancy.  That would be about a 10 inch difference in his legs.  Before this surgery, most people were advised to amputate and use a prosthetic.  This is just another reason we are so thankful for Dr. Paley.  He is giving our child and many others a chance to live a normal life.  The process starts with a surgery to cut the femur in half and insert pins into the bone.  These pins are connected to an external fixator where you turn the screws about  5 times a day to lengthen the bone.  You can gain about 1mm a day.  Nash will have to have phisical therapy for 2 hours a day for the first 4 months of this process.  That means we will be in Florida for the first 4 months.  This part is painful.  Not only are you stretching the bone, skin, and ligaments with the fixator, you are stretching the muscle with the PT as well.  I am not looking forward to this :(   Our goal is to get 8cm out of each lengthening.  It is possible but doesn't always happen.  It depends on each person and how their body reacts. 

After we get the length, we will get to go home!  However, he will keep the fixator on for another 4 months while the new bone hardens.  So basically he will have this device on for 8 months and we will get 8cm of growth.  One month for each cm of growth.  Nash will have to do this 3 times in his growing years or until he's 12 or 13.  One lengthening every 4 years.  That's the plan.

In the meantime, we will be getting him some fancy shoes with a lift on the right side.  Hopefully soon because he is ready to take off!  We have a friend named Madi in Des Moines who has CFD, too. She was nice enough to let us borrow her shoes to practice.  Nash was NOT a fan.  Hopefully he will like his better.  I think he was just mad because they were girly shoes :) Daddy says were gettin him some Jordans!



Nash with Dr. Paley




Nash practicing with Madi's shoe

 Hopefully this all makes sense.  We don't mind when people ask questions, we actullay like it because we want everyone to understand as much as possible.  Thanks for taking the time to read this and we appreciate your love and support!  I will update with pictures when we get his shoes :)