amaya at lake

amaya at lake

Tuesday, December 7, 2010

A matter of perspective

     I realize that Amaya could have something worse than PFFD, in fact, she could have a worse case of it as well. We could be like many other parents having to decide wether to amputate or having to face multiple surgeries. She has, as several doctors have told us, a best case scenario for someone with PFFD.  So even though I wish she didn't have to endure the pain, the stares, the rude comments, the surgeries, and all the discomforts that go along with this. I am thankful that it is what it is.  It could be worse, and I am glad that it isn't. Very glad.

Friday, December 3, 2010

Information on PFFD

     I realize that many of you do not know what PFFD is. We didn't until Amaya was diagnosed, and still are learning as we go along.
     Here is some basic information;
PFFD can affect one or both legs
it can affect the femur as well as the tibula or fibula
PFFD affects the hip, femur and knee
there are four basic types varying in severity, Amaya has one of the least severe cases.

     This is just some basic information to help understand what it is and how it affects Amaya. You can always ask us any questions you may have and we'll try to answer them as best as we can.
     Here are two helpful websites www.pffd.org and  www.limblengtheningdoc.org  Both have lots of helpful information about what PFFD is and how it's treated. You can also google PFFD and find all sorts of information.

Where to Begin...

     By this point most of you know that Amaya has PFFD (proximal femoral focal deficiency) it is also known as CFD (congenital femoral deficiency).  We discovered she had it shortly after birth.  In fact the first day she was born we heard a "pop" while her uncle held her. We weren't quite sure what to think of it.  After several follow up visits with nurses, doctors and an orthopedic nurse we finally got in to see a specialist.  An orthopedic surgeon at Kaiser in Fontana named Dr. Hurley.  After several ultrasounds she believed that Amaya had a dislocated hip (possible hip dysplasia) and sent us home with a brace.  Once Amaya  got a bit older and she was able to see more clearly in x-ray's what was going on we found out the whole scope of the situation.  Dr. Hurley told us that Amaya had PFFD (we had never heard of it before then) and that she would need several surgeries throughout her life beginning as early as age one.  Amaya's left femur is 4 cm shorter than the right one and grows at a slightly slower percentage.
     We decided to get a second opinion and got in to see Dr. Kishan at Loma Linda Children's Hospital. He confirmed the diagnosis, but had a more "laid back" approach to treatment.  He took a wait and see approach, and only saw Amaya once a year.  Amaya continued to wear a one inch lift on her shoe and then began to wear a neoprene  knee brace to help stabilize her knee. Often children with PFFD don't have the ligaments in the knee.  Because of this Amaya's knee dislocates frequently.  After a year Dr. Kishan suggested a sturdier knee brace to help keep the knee aligned. He was afraid of the damage that her knee would suffer from constant dislocating.  He suggested she have ligaments implanted at age ten and then later do a lengthening surgery or possibly stunt the growth of the right leg to allow the left leg to "catch up" or a combination of both procedures.  Amaya is currently wearing this knee brace as well as her one inch shoe lift.     
     In the summer we received a referral letter from her primary doctor to go see Dr. Nelson at Loma Linda, Dr. Kishan was leaving Loma Linda. We met Dr. Nelson, an orthopedic surgeon, who had been living in Santo Domingo, Dominican Republic for the past five years. He was serving as the medical director of the Cure International children's hospital.  He was very familiar with Amaya's condition and immediately began doing some calculations. He predicted the leg discrepancy to be closer to 8cm (about 5 inches) once she was done growing.  He recommended we do a superhip, superknee surgery then follow up with a lengthening surgery.  He came to Loma Linda to work as a faculty and surgeon. We were impressed with his knowledge, confidence and plan for Amaya.  He was very happy with her bone structure and was quite confident that he could correct her leg in two surgeries. 
     We met with Dr. Nelson in October and he recommended scheduling the surgery as soon as possible as recovery takes about three months and since his surgeries are booked three months in advanced. The soonest appointment we could get was January.  He recommends doing the lengthening six months after the superhip, superknee. But, as of now we are not sure when that will take place.
So, that is where we stand for now.