We arrived in West Palm eager and ready for our appointment with Dr.Paley. When we arrived in the morning, Rich joked that we were like rockstars walking in as everyone shouted, "Norah! Norah!" like they already knew her and immediately came over to us with warm welcomes and hugs. He had been communicating with them since the week we brought her home so there was already a friendship there which made things a little easier. I on the other hand went in a bit nervous, a little queasy and no surprise....a bit protective of my baby. I had managed to hold it together pretty darn well until I caught a glimpse of the first of many patients we would see that day and lost my cool. Interestingly enough, he didn't have fixators on his legs like Norah would. He had them on both arms. Shortly after, we met a woman from Florida with them on her lower legs, then a little boy from Kansas who had just undergone the Super Ankle Surgery (quickly became great pals with my girl) and a young girl from Poland with one on her right leg. Needless to say, I felt like I was diving right in....there was no sitting on the edge of the steps and easing into the water nonsense. It was all in the minute we walked through that door.
We were surprised to see there was no helicopter pad or red carpet and Dr.Paley didn't come through a big wave of fog or anything.....just a normal doctor's office. :) Rich and I both had psyched ourselves up as many other parents seem to do as well and then you arrive and it's like, "okay, no big deal, just another hospital." I know at some point another family will read this and believe me, that statement will bring you comfort. When you just go in, fill out the usual paperwork, do your x-rays and wait for the Big Man, it feels normal. Like there's nothing special or scary about it. And trust me, while you're there, the more normal it feels, the easier it will be for you to not throw up your breakfast in the nearest trash can.
Norah and I played with little J.T. from Kansas while waiting. She had a BLAST with this kid. They were literally playing Tug-O-War over her piano, so cute!
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| Norah and fellow Paley patient "J.T." playing in the waiting room. |
After several hours of x-rays, playtime and waiting, we were pleased to see him by 2 o'clock. Like everyone has told us the last 9 months. "Worth the wait." He came in and gave us a warm welcome, shook our hands and got right down to business. Now I'm going to get scientific....
Her official diagnosis is Congenital Femoral Deficiency (C.F.D.) Type 1A and Fibular Hemimelia. Type1A is the best case you can have so that's great news. Her left femur (upper thigh bone) is shorter than her right as well as her lower Fibula and Tibia (lower leg bones) are shorter also. Her leg length discrepancy is anticipated to be 17.8cm or 7-8 inches at full skeletal maturity. This doesn't include the foot so maybe a tad more. When she begins to stand we can get a more accurate number. This means that if we do nothing her left leg would be 7-8 inches shorter than her right, prohibiting her from putting both feet on the ground.
Her treatment plan works in two phases. Preparation Phase and Lengthening Phase. Preparation would mean any surgery on any joints prior to leg lengthening to ensure all are strong and stabile. Lengthening would be the actual leg lengthening process which would most likely be accomplished in 2 lengthenings. (2 is awesome news) We were really hoping to avoid a Hip Surgery mainly because that occurs at age 2 and just the thought of a 2 year old under anesthesia makes me sick to my stomach, however that most likely won't be avoided. The good news about that though is he didn't see any glaring evidence that she will need the Super Hip Surgery which is more complex. She would be doing the Pelvic Osteotomy. This surgery from a patient standpoint is pretty much the same but from a technical/surgical standpoint, it is less involved. So thumbs up on no SuperHip and thumbs down on Pelvic Osteotomy. Her knee looked good, although she was born without an ACL or PCL. This is a minor detail in this case though. Her ankle is rotated so it is quite possible we would need an Ankle surgery as well. We didn't dive into details on that because it so far down the road.
At age 3, we would begin lengthening. For girls, half of the length discrepancy appears in the first 3 years. So this means by age 3, her left leg would already be roughly 3-4 inches shorter than her right. Then, from 3 to say 10 to 13 year old age the last 3-4 inches would be visible at a much slower pace. So after the 1st lengthening she would most likely gain 3 inches from that and it would appear as though her legs were almost the same length. Then over the next 7 years, the remaining 3-4 inch discrepancy would be visible and we would do our final lengthening at age 10. Lengthening takes 8 months. So after her prep surgeries and 1st lengthening she would have some "time off" until about age 10 where we would complete her last surgery/lengthening. What does that mean?
OUR LITTLE GIRL WILL HAVE BOTH FEET ON THE GROUND!
She will run, play, dance and do all the things other kids do. Even while undergoing this process,she will be able to swim and play in her fixators. In fact, you see alot of CFD patients swimming alot because it keeps the pins clean. Below are some photos "Before and After" and other kids coping with CFD.
| Before | After |
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http://paleyinstitute.org/?q=intro-to-limb-lengthening (This link explains the lengthening process very clearly if you have questions as to how they actually lengthen her leg)
With that said, it became pretty clear to us on the way home that this process was the right decision for her. Amputation is just crazy talk and a Prosthetic Leg was a consideration of ours for a while she but unnecessary. If she was going to spend her entire childhood in a surgical room, maybe we would consider that. I know there are other extreme cases where those options are the best route but in Norah's case, it just doesn't make sense for us. If we can give her the ability to walk with her own two legs ,that is the best thing we can do. Most of this will happen at such a young age that she wont even remember it. Sure she will remember her fixators at age 5 or 6 because she will be wearing them well into her teen years, but she wont remember the surgery itself.
For now, the treatment plan is a shoe lift and ankle brace until we begin the process. When Norah begins to stand on her own and begins cruising furniture then we will have special shoes designed to help her walk. The brace will help keep her ankle from twisting and rolling due to the platform. They will look a little bit like this:
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| This is Rory. Following her story has helped us SO much. You can read more about her at roryjane.com |
Jennifer, the Patient Coodinator took these pictures during our visit. She was super friendly!
All in all, we were pretty relieved when we left. To have a plan, answers and meet the man who will perform her surgeries was comforting. Rich reminded me of something on the way home. "God doesn't make mistakes", he said. He reminded me of that and that he has entrusted her with us and chosen us to be her parents. He knew we could handle this and that we would do something amazing with it. Who knows what the mission is or what she will do,but I believe it will be something remarkable, simply amazing. The less I focus on "this happening to her" and the more I focus on the blessing and how this will forever change her in the most humbling and beautiful way, the better Mom I am to her. I don't want to be angry about it. It is so exhausting being so angry and so wonderful when I just appreciate how beautiful she is.
My husband and I will be launching an INCREDIBLE fundraiser for The Paley Foundation very soon. As some of you know, he accomplished his greatest fishing achievement so far: Qualifying for the BASSMASTER CLASSIC 2014. This was a huge game changer for our family. You can support this cause by betting on my husband! You can donate an amount of your choice per pound of fish caught at The Bassmaster Classic 2014 or donate directly to the foundation once we post it. We will be posting website details and information soon and the fundraiser will be alot of fun! STAY TUNED!
As always, thank you all so much for your continued support through this. It means alot to us.
