We started our relationship with the Paley Institute when Evan was less than 1 year old. It started with an exploratory email to Dr. Paley while he was lecturing in Europe. He responded to our email within 45 minutes and said he would review Evan’s x-rays and an MRI as soon as he returned home. He then followed through by calling us at 11pm on a Friday night and spent well over an hour with us explaining calmly and confidently that he had a plan for our son that he had already used to successfully treat many other children. This was such a relief as the other doctors we had spoken to told us that Evan would probably need to amputate his left leg.
Evan has Congenital Femoral Deficiency (CFD/PFFD) – type 1B in his left femur. His femoral head was severely deformed and he was born with most of the cruciate ligaments in his left knee missing. Dr. Paley performed the SUPERHip and SUPERKnee surgeries on Evan in December 2010. During the summer of 2012, Evan had leg lengthening surgery and achieved 8 cm of bone growth in just 91 days. Evan will likely need to repeat the leg lengthening process at ages eight and twelve.
Parents that are evaluating options for children with CFD/PFFD should understand that the surgeries to correct this condition, and the physical therapy that follows, are very challenging. Evan required approximately 6–7 months of PT and a great deal of emotional support following his SUPERHip and SUPERKnee procedures before he gained the strength and confidence to play at full speed with other children. By comparison, the lengthening surgery was far less traumatic than the hip and knee reconstructive surgeries, but the 3 month lengthening process, and the 2 month consolidation phase that followed, were by far and away the biggest challenge our family has ever faced. Managing multiple daily PT sessions, daily pin site cleaning, and the emotions of a small child living through the treatment requires an incredible amount of focus, dedication, and endurance.
While nobody that goes through the lengthening process enjoys it, the children and their families all figure out how to get through it, and in the end the results are amazing and totally worth the sacrifice. In fact, although the process is grueling, there is something truly inspirational about being around the kids and their families that are going through the lengthening process. We were constantly humbled by Evan’s incredible resilience, ability to smile through it all, and his ability at such a young age to comprehend the process and truly take great pride in working towards his 8 cm lengthening goal. Once the process was finished we were overwhelmingly proud of our son, his bravery, and the incredible step forward he took in securing a brighter future for himself that would not have been possible had we not pursued treatment.
Evan has been truly blessed to be treated by a master surgeon, surrounded by highly responsive physician assistants (PAs) and orthopedic technologists who always made themselves available for us even at night, weekends, and on holidays. Dr. Paley’s supporting staff is incredibly dedicated and has created a specialized environment that is custom-made for patients with Evan’s needs. Perhaps the best examples of this were the daily appointments that Evan had with the Paley PT staff and his personal pool therapist. Not only was Evan’s physical therapist uniquely qualified to assist a child with his rare medical issues, she developed such a close and trusting bond with Evan that she became an extension of our family for the summer. The same was true for Evan’s pool therapist who constantly found new ways to have fun in the pool while also dramatically enhancing Evan’s flexibility and range-of-motion. It is also extremely helpful to be surrounded by other similarly situated kids and families going through the same process. The emotional support of comparing notes and getting together socially are what kept us sane through the process.
Although Evan is only half way done with his journey, we are already incredibly grateful to Dr. Paley and his amazing staff for what they have done for our son. While most doctors would have pursued amputation, Dr. Paley has successfully delivered on 3 extremely complex surgeries and treatments that are allowing our son to live a normal, physically active, and happy life full of potential and promise.
Scott and Jennifer
Victoria, Minnesota
