Knox’s adventures with PFFD/CFD
Hello, this is Knox, a little boy who was born with PFFD/CFD as well as fibular hemimelia. As his parents, we created this blog in part to help other families who are raising or expecting children with limb deficiencies. We learned of Knox’s little leg during our 20-week ultrasound, and finding and reading other parents’ blogs was a huge source of comfort and information for us. We also wanted to start this blog to keep friends and family up to date on Knox’s progress. Over the past 5 years, Knox has mostly been a very healthy, very happy little guy. He has used a prosthesis to run, jump and play like any other kid.
However, that is about to change for awhile, as Knox is scheduled to undergo rotationplasty, a major limb surgery, in March of 2019.
What is PFFD/CFD?
Proximal femoral focal deficiency (PFFD), also known as Congenital Femoral Deficiency (CFD), is a rare, non-hereditary birth defect that affects the femur and often the pelvis, particularly the hip joint. The severity and particulars of the condition vary a lot from person to person. Knox is considered on the severe side, as he has hardly any femur in his right leg, causing it to hang at about knee-height of his left leg.
His hip on that side is also very underdeveloped. It is flat, and he doesn’t really have a femoral head on his femur. In other words, his hip has no ball and socket. Because of his short femur, his knee is located very close to where his hip should be, and that is where most of his movement comes from.
Knox also has fibular hemimelia (missing fibula), which is common with PFFD. Many people with fibular hemimelia don’t have a fully formed foot with five toes, but Knox does. His ankle is also surprisingly stable and flexible for his condition. Since he was 10 months old, he has used a “foot-on-foot” prosthesis to walk, run, snowboard, ride a bike and do pretty much everything else other kids can do.