One of the greatest joys in life is having a baby! Our third child, Jesse Alexander Rause, was born May 27, 1995. That same tremendous joy which we felt when his two older brothers were born was here again! It is a joy that is absolutely wonderful. It can not be explained. This time it was a little bit more special, after overcoming the two miscarriages we had also experienced.
Four hours after birth, after holding and kissing and hugging our new little baby, a nurse discovered what we and the doctors had not detected. Our "perfectly healthy" baby had a serious birth defect - one leg was extremely shorter than the other. Words cannot explain the feeling.
After a specialist was brought in and did some research the defect was given a name: PFFD (proximal femoral focal deficiency). Very few people we spoke with knew much about PFFD in 1995. We studied in medical libraries and surfed what there was of the internet back then, but found very little. Basically, it means that the femur bone (the bone between hip and knee, also the longest bone in the body), is short and deformed at the connection to the hip. Jesse's femur was ½ the size of his other leg. If we did nothing at all, his left leg would be 12 inches shorter than his right leg when full grown.
We spent a year searching for answers, traveling to various doctors. The first doctor we went to see told us matter-of-factly that there was NOTHING we could do. He automatically assumed that we would amputate. He said there was no hope of saving the leg and amputation of the foot would be required for him to use a prosthetic device (artificial extension) in order to lead a 'normal' life. His little foot was perfect - how could we even consider cutting off a perfectly good foot so a prosthesis could fit better on a stump? It made no sense. Who could say what medical and technological advances would be achieved years from now. We won't be going back to that guy.
Our second doctor at UNC told us that there was only a very small chance of lengthening the femur, depending upon Jesse's hip socket formation. However, this would give him a longer leg but render him virtually unable to walk. He prepared us for the probability of amputation. We were not satisfied and kept on searching.
We had hope before visiting our third doctor at Duke because we heard good reports of his lengthenings. But he told us in Jesse's case he would recommend amputation. We said "thanks, but we'll keep looking."
The fourth doctor visit was scheduled at Shriners in South Carolina. But after some research and talking to others who had been to them, we concluded that Shriners was great with burn cases but they hardly ever considered lengthening and mostly did amputations. So we cancelled that trip.
By doctor number 5 our determination was wearing thin. But this doctor was the "Foremost lengthening specialist in the world". He taught lengthening at the famous DuPont Institute in Delaware. He lectured in Europe and Asia. Our hope focused on him. We waited over 5 hours past our scheduled appointment to see him. Our hope crashed and burned in flames. For the first time I stopped and considered amputation. He was very good. He almost talked us into it, describing how it would be MUCH better for Jesse in the long run. "Do it now while he's young, he'll never miss it." As we drove back to North Carolina feeling numb, we tried to build up a little bit more hope.
It had been a full year after Jesse's birth. We had yet one last lead to follow up on. Just before our trip to DuPont, we heard about one more doctor that we should visit. A mother of another child like Jesse had told us if this doctor couldn't help us then no one could. With almost all of our optimism gone, we made an appointment to visit Dr. Paley in Baltimore. The rest is history.
We met with Dr. Dror Paley in Baltimore Maryland, at Kernan Hospital, part of the University of Maryland. He spent over an hour with us talking to us and explaining PFFD, but truly we heard nothing after the first few minutes when he said "I am sure I can help Jesse and I can lengthen his leg". We broke down and cried in front of him. We knew at last that Jesse would be able to function normally with his own leg! What a wonderful feeling it is to be given hope! We cried all the way home.
All of our friends and relatives share the same anger about the doctors who told us to amputate. They've told us to write letters, tell the media, get the news out. And we should. There are people out there who might get talked into doing something they don't want, something that can't be undone.
Jesse is 4 ½ now and currently has undergone two major surgeries to correct some deformities in his hip, pelvic bone and knee, which must be done before starting the lengthening process. He will have surgery January 25th 2000 to begin his first lengthening procedure, which will last 9 months to a year. The whole procedure is not an easy process by any means and it takes a tremendous toll on our family, friends and Jesse. The lengthening is an incredible thing. The femur bone is broken after pins are screwed into the bone. The pins protrude out of the leg and attach to several external rings that circle the leg. Threaded rods attach the rings to each other, forming a sort of metal cage. Every day we are required to turn screws on the rods which in turn push the rings, and therefore the bones, apart ever so slightly (1 millimeter a day) and the human body regenerates bone to fill in the gap. What an amazing thing. We have met teens who have gained 14 inches. This takes several procedures over many years. We expect to gain 3 to 4 inches of length per procedure.
Jesse will require at least 2 more procedures by the time he turns 14. What his leg lacks in inches God has given him in spirit. If you've met Jesse you know that nothing stops him. He keeps up with his older brothers while running (hopping) using only one leg. His left shoe's sole is 6 inches high. He has to wear a brace to support his ankle from bending. But he still learned to pedal his two-wheel bicycle with both legs. Until now he only rode his tricycle while pedaling with only one leg. When his brothers play baseball or basketball he runs out there trying to do the same. We don't worry about him much. We know he'll be ok. It'll be a long hard road. But he's been given the character and the disposition to get him through anything.
The following link has great information and links telling all about PFFD:
Jesse's Surgery Update!