Jamie's Page
From: Her family - as remembered by her Aunt Jo
To: Jamie Nicole Miller
Born: November 24, 1995
Battle Creek, Michigan
JAMIE ARRIVES...
Two years ago, God put one of his little angels into our care here on Earth. Jamie Nicole Miller was born on November 24, 1995 to Dianne and Ron Miller and her 4 year old sister Samantha, healthy and screaming. Within minutes of her birth though, we would learn that not all was normal. Her right leg was shorter than her left and she was immediately taken to X-ray, to see if there was anything further. An orthopedic surgeon was called, and we waited, some crying, some consoling, for the doctor to come tell us what was wrong with our beautiful little addition to our family. He would introduce us to what is more easily called PFFD (proximal femur focal deficiency). Those four little letters would alter our family forever.
The orthopedic surgeon, Dr. Garcia, explained that the X-rays showed a short femur bone in her right leg and a missing fibula in her lower right leg. He informed us it was not uncommon with PFFD, that there be two bone deficiencies. Their main concern, at first, was the development of the hip joint. This would determine the procedures she would go through in the future. He told us this looked hopeful and to take Jamie home. He recommended taking her to the University of Michigan Children's Hospital for further evaluation.
A DISAPPOINTING TRIP TO ANN ARBOR...
An appointment was scheduled for Jamie with Dr. Hensinger, an orthopedic surgeon, at the University of Michigan. This first meeting at U of M would be an evaluation of Jamie's case based on the X-rays that were done following her birth. Dianne, Ron, Jamie and Ron's sister Rhonda traveled one and a half hours to Ann Arbor for a fifteen minute meeting with Dr. Hensinger. Without any explanation of the whys or hows of Jamie's condition, the doctor informed them that the best they could hope for would be a foot amputation and lower leg prosthesis. Dr. Hensinger wanted Jamie back in January for follow-up X-rays to check the growth of her femur. The group came home with their dreams for Jamie's future shredded. It was time to regroup. Cutting off her little foot was not going to be the only option for Jamie.
TWO MORE TRIPS TO ANN ARBOR...
The new X-rays didn't show much of anything new than her first set of X-rays. Dr. Hensinger wanted her to come back at 5 months to be fitted for a corrective shoe. With the absence of her fibula in her lower leg, her little foot lay to the right. The corrective shoe would help her ankle and foot to develop as correctly as was possible in her case. On March 11, 1996, Jamie went back to U of M for a prescription for her corrective shoe. They set her an appointment with a prosthetic group back in Battle Creek, called Wrights & Filippis, Inc. A week later, Jamie was at Wrights & Filippis. Her foot was casted, which she absolutely hated. When it was dry, they used a drill to remove it. This, it turns out, was a happier experience. The vibration tickled and she giggled through the whole thing.
An ugly pink plastic shoe was made from the cast and it fastened with velcro. She would spend the next few months wearing it.
WHATEVER IS NORMAL...
Jamie spent the next months much the same as any baby. Changing her diaper was about the only physical challenge we had. She was a good baby. The mental challenges were a little harder to deal with. Being a very close family, we helped Dianne and Ron as best as we could, none of us with any knowledge of what PFFD would entail in the future. We all knew that something could be done, and we tried not to let Dianne and Ron forget it. In one of our soon to be frequent discussions, I told my sister that God never gives us more than we can bear, and she would repeat this to herself in the months to come.
Jamie learned to crawl around on all fours, showing no problem in learning to do so. We called her our little bear cub, because she went around on her hands, knee of her long leg and foot of her short leg. Soon she was pulling herself up on everything, her little leg dangling, which was no longer a shock to family and friends. It was Jamie. The time to call for her first prosthesis appointment was at hand. Dianne and Ron weren't ready for this, but we encouraged and assured them it was time, and yes, we even nudged them when they stalled.
JAMIE'S FIRST PROSTHESIS...
The appointment was made, and her first prosthesis was made. This was where Jamie learned that men and women in white coats weren't her friends, even though they were. X-ray machines and white coats were an immediate tear turn on for Jamie from here on out. However, she made it through every appointment with some comforting from mom and dad, who in turn needed comforting from the family. We were always ready. To everyone's surprise, at 10 months, Jamie was walking on her new leg. The doctors and her prosthesis team were so proud of her. We knew then that Jamie would have the perseverance to live with her deficiency.
As she grew, the prosthesis had to grow with her. Unfortunately, this did not happen fast enough for Jamie. One day in June, while on vacation at Grandpa Bilek's in New Jersey, Jamie refused to have her prosthesis put on. She had been wearing it for over 9 months without a complaint. She cried every time we tried to put it on her to the point of making herself sick. Something was definitely not right, and we did not push the issue until we could get her home and to the doctor. By the time we had returned from New Jersey, pulling or touching her leg had started to bother her too. Dianne decided to take her to the emergency room at our local hospital. Jamie's Uncle Steve, Rhonda's husband, did her X-rays. How lucky to have a radiologist in the family. The X-rays ended up showing nothing. Dr. Hensinger, from U of M, recommends a bone scan.
A SCARY VISIT TO THE HOSPITAL...
She was scheduled for a bone scan. The doctors feared that she may have a bone fracture or that the hip joint was causing her pain when she put weight on her leg. The bone scan should show us what was wrong. At this point in time, the bone scan had to have been the scariest thing Jamie had gone through since she was born. She was about 19 months old and fully aware of good and bad. The bone scan was bad. Dianne and Jamie were on their own for the beginning of this appointment. The radioactive dye had to be introduced into Jamie's body an hour before the bone scan. Uncle Steve to the rescue again. Steve helped hold Jamie so the nurse could insert the port for the dye into her little arm. Not fun. The dye is injected and Dianne and Jamie wait the hour out in the hospital. Jamie had to be sedated next, because the bone scan requires perfect stillness. Our sister Teresa(Aunt Tee Tee), arrives in time for this. They gave Jamie a suppository. This lasted a whole two minutes and it was right back out. They put it back in. It stayed in this time, but it did not sedate her. Teresa walked up and down the halls for an hour trying to get her to fall asleep. She stayed asleep until Teresa laid her down. The minute Jamie lost contact with Aunt Tee Tee, she was wide awake. They tried another suppository. By now, she had had enough to tranquilize a baby elephant. Dianne resorts to her old trick of driving Jamie around the block to get her to sleep. After about a hundred times around, Jamie finally fell asleep. Dianne carried her back to X-ray, but the minute Dianne laid her down, she was awake again. The radiologist couldn't do his job until that stubborn little girl was out, and he was almost to the point of having an anesthesiologist come in. They gave her one half more of a suppository. By now, Aunt Rhonda was there. They laid her down on the table and as Aunt Tee Tee and mommy held her hands, Aunt Rhonda rubbed her head and talked to her. The next step was getting her in place for the scan. The bed of the scanner raised her up towards the scanner. She was still awake and aware that this wasn't good. Finally, she was to the point of exhaustion and still enough to try the first scan. She twitched. The radiologist decided to try to immobilize her the old fashioned way. Using towels and masking tape, yes, masking tape, they bound her legs to keep her from moving. The adults in the room cringed, knowing she's awake for this. Consoling her was a group effort after that. Thankfully, it only lasted until that last half of a suppository kicked in and she finally went to sleep.
HER SECOND PROSTHESIS...
The bone scan showed there were no bone fractures, so this left the solution to be too much pressure on her hip. She was fitted with a prosthesis that would help support her hip better. She was about 20 months old when they added a clear plastic top to her old prosthesis that molded to her upper leg and hip area. It was strapped around the waist and it was awkward. She didn't like it. She wouldn't wear it, and forcing her could be detrimental to her future with prostheses. We encouraged her and enticed her. Her cousin Devin had turned one in July of 1997, around the time she got her new prosthesis and he was walking around and having a ball. She watched, she wanted to go, but she wouldn't use that new leg. Slowly, we got her to wear it, but she wouldn't walk alone. She would say "hand, hand", and you would have to help her. Finally, one day, she and I were playing in the kitchen. She had asked to have her leg on, but she still wasn't walking alone with it. We were in the middle of the floor having a popsicle, when she decided she needed to throw the paper away. Holding my finger to stand up and steady herself, I let go. Without thinking, she took off for the trash can and came back to me, her eyes wide. I hugged her and told her I knew she could do it and could we show mommy and daddy. With a yes and tears in my eyes, I called Dianne and Ron to watch what Jamie could do. Her dad came in the kitchen, and with a steadying hold, she let go of my finger and walked to her dad. That was a triumphant day. She would overcome all obstacles. I knew it.
LONG TERM SOLUTIONS...
Now was the time to start thinking long term about Jamie's leg. We were told about Shriner's Children's Hospital through a friend, and were set to call them about a sponsor, when one day at the dentist's office where she works, my sister was discussing Jamie's situation with a coworker and a patient overheard. She told Dianne that her husband was a Shriner and that she would talk to him about Jamie. Dianne got a call the next day from Col. Finley Phipps, retired. He wanted to sponsor Jamie. He would make the necessary calls to Shriner's and when they could get her looked at, they would call with an appointment. She went to the Shriner's Temple in Kalamazoo, MI to be evaluated. They were told she would go to Shriner's Hospital in Chicago and that they would be notified within 6 to 8 weeks from Shriner's for the date and time. The time stretched out, and we ran into some clerical snags with Jamie's paperwork, but we were persistent and finally got the go ahead for Jamie to go to Chicago. She was scheduled to meet with a pediatric orthopedic surgeon at Shriner's Hospital-Chicago in November, right around her second birthday.
AN ENLIGHTENING VISIT...
Dianne, Ron, Jamie and Ron's sister Rhonda went. Rhonda was the family's ears, taking in everything. She brought a mini-recorder and would tape the whole meeting. They met with Dr. John Lubicky, chief of surgery. He was pleased with Jamie's abilities and accomplishments. After looking at her records, he informed them that leg lengthening, the option we were counting on, was not one of Jamie's options. The femur was too small and the hip ball hadn't developed sufficiently. His alternative was tibial rotation, surgery involving turning the lower leg around and fusing it to the knee and femur. This would create a stable upper leg, and a lower leg prosthesis would then be used. He suggested in Jamie's case that the foot be left intact as the basis for her lower leg prosthesis, and that her ankle would become a natural knee joint. Some of the time, the surgery involves amputation of the foot at the ankle and an artificial knee joint be used with the prosthesis. These are the decisions they would face. Surgery. Not what they came to hear. Dr. Lubicky said it should be done before her third birthday. He told them to take her home and think about it. He wanted them to call after the first of the year if they decided on the surgery so they could get her scheduled.
THE SEARCH FOR KNOWLEDGE...
After all this, we decided we needed to know more. My friend Kim had internet access and volunteered to get online and surf the net for anything on PFFD. She's the one who found the PFFD VSG. I decided to research this further. I got on the Internet with my boyfriend "Uncle Ron", and we printed everything we could find on PFFD. Then we went deeper, and printed everything we could find on tibial rotation, the Van Nes procedure. As scary as it sounds, I think we are all in agreement that it is the best solution for Jamie. We don't want to hold her back with any of our decisions. The need for education was great. The PFFD support group supplied that information. I'm eternally grateful to the creators of this group. It has helped my sister and brother-in-law and the extended family tremendously. We are not alone. There are "survivors" doing great things with their lives. We needed to know she was going to be O.K.. My sister would appreciate some one on one with the parents and children who have gone through this. They do not have a computer linked to the Internet, so you e-mail me at jlbilek6@aol.com. I will print anything sent and pass it along to my sister and brother-in-law. We could make arrangements for contact from there. Any encouragement and information will be received with love and open minds. Thank you for your time. As with every patient of this surgery, this story is to be continued. Her tibial rotation surgery will be scheduled before November, 1998, and I'm sure they would love any advice to help a 2 ½ year old cope with the months to come. Updates will follow.
Jamie's Aunt Jo
Joann Bilek
Joann can be contacted at jlbilek6@aol.com