This is Trinity, my very energetic seven year old daughter. She was born six weeks early weighing 2lbs. 3oz. with a rare birth defect known as P.F.F.D. or Proximal Femoral Focal Deficiency. Some of you may know what this is, but if you find yourself thinking copy --> paste --> Google, don't feel bad because I was in the same boat the first time I heard these words.
P.F.F.D. basically means she was born with a short leg. The femur bone in her left leg is less than half the length of the one in her right leg. You can go HERE to read more in depth on this. Along with P.F.F.D. she was also born with Fibular Hemimelia. The Fibula in her left leg never developed at all.
As you can see in the photo above, the femur in her left leg has a significant difference in length. You can also see that the missing fibula causes her ankle to kick out without its support.
During her 32 day stay in NICU at the Children's Hospital in Kentucky due to her size, none of the doctors could explain why she was born this way. No one seemed to know anything. All they could tell us was that it wasn't gentetic and that it wasn't anything I had done. By the time she left NICU we had an appointment at Shriner's to meet with a team of doctors to discuss our options. I really had no idea what to expect but I can say that the options we were given had never crossed my mind.
1. Amputation from the hip down.
We were given these two options because she did not appear to have a hip joint or a kneecap. They didn't expect us to make a decision right away but advised us to do so before her first Christmas. As we were leaving Shriner's I thought I would breakdown right there in the parking lot. How could I cut off my precious little girls leg? And the Van Ness Rotation...well...I couldn't even think of that as an option.
The Van Ness Rotation is where they remove the femur completely and move the lower part of the leg up and turn it backwards. The ankle is supposed to act as a knee. A prosthetic is worn to make up the rest of the length difference.
Thinking there were no other options I attempted to discuss our options with my husband on the way home that day. As soon as I began he stopped me. He refused to believe that this was our only choice. He planned on searching the Internet for more information and seeking out a second opinion, a third, and forth...
He found five hospitals across the US that he wanted to visit. The first of the five was the Children's Hospital in Ohio. I tried not to get my hopes up as we headed off for that first appointment. When the doctor finally come into the room after examining the x-rays I expected to hear the same thing we heard at Shriner's. Much to my surprise, I was wrong.
The doctor agreed that Trinity's length was severe and that there didn't appear to be a hip joint or a kneecap, BUT she did notice that there was a small gap between the top of her femur and where her hip joint should have been. She wanted to wait a few months to see if there might be something there that just needed a little more time to calcify.
After six months we returned for more x-rays. When the doctor came in with a beautiful smile I could hardly contain my excitement. She brought up the x-rays on her computer and showed us a tiny white area where her hip joint should be.
"I do believe that is a hip joint." She had said.
She hadn't wanted to get our hopes up but she told us that if she was right, lengthening might be an option for her. She did stress the word might. Even with a hip joint there was still the possibility that we couldn't lengthen. There are for classes for P.F.F.D. with D being the worst. Trinity was classified as a C, meaning lengthening could be possible but unlikely in most cases.
For almost two years we traveled back and forth for x-rays. Each visit building my hope up just a little higher. Everything was going well. She did have a hip joint as well as a knee cap, both just needed more time to calcify so that they could be picked up on x-rays. To make up the difference in length during this time she wore an AFO prosthesis with a pipe and prosthetic foot off the bottom.
Then...Her doctor transferred to a hospital in Utah and we were to start seeing her partner. We were sad to see her go but we were still hopeful and willing to work with her team member. As we waited for the day we were to meet her new doctor we found ourselves back at Shriner's. I'd made an appointment to get a prosthetic made there. After we arrived for the appointment we were informed that we could not come there for a prosthetic without meeting with the doctors. We agreed to an evaluation so that would could get the prosthetic. The evaluation turned out to be a nightmare.
After taking x-rays, four doctors came to our room. I'm not sure of the purpose of three of those doctors since they simply stood in the background without a single word as the other doctor proceeded to tell us that we were making a bad decision. He told us we were ruining her quality of life and that our efforts to save her leg were a waste. I tried to bring to light the fact that they wanted to amputate her leg based on the fact that she had no hip joint or kneecap and if we had allowed them, we would have never known they had been wrong. He refused to listen to me and was firm in his belief that we were making the wrong choice. After I informed him that we already seeing doctors in Ohio he let us know that he was good friends with the doctor we were being switched to and would contact him.
After our visit with the doctor at Shriner's I was a little anxious about meeting with her new doctor and what he might say, and I was right to be. He was very short with us and he made very little eye contact as he was speaking with us. He told us that her other doctor had only been getting our hopes up and that there was nothing that could be done to save her leg. He asked to see her walk on her prosthetic and barely gave her time to stand after putting it on before he walked off.
Everyone of us were angry and devastated. Shortly after he left the room the nurse suggested we meet with the entire team of doctors for an evaluation. We agreed but were not so eager to return. As we waited for the evaluation day to arrive we made the decision to seek out a different doctor no matter what they had to say.
The evaluation was short and we were informed that we would not know their conclusions for about 6 to 8 weeks. Even when the report came in the mail stating that Trinity was in fact a candidate for lengthening, we were firm on our decision to seek out a new doctor after the way we had been treated.
For a while our search for somewhere different was relentless, but after a few months it began to fade. By this time she was getting older and we both knew that if amputation was going to be our only option we needed to make a decision soon. We both wanted the transition for her to be as easy as possible.
Then...
I found Lexxi's mommy on MySpace. After browsing through images online I stumbled across a baby girl with the same leg condition as Trinity. I sent a message and waited for a reply. It didn't take long and her mother was more than happy to talk with me. For several days we exchanged messages. Her daughter had already begun the process of lengthening and she shared with me her doctors name and where he was located.
Armed with fresh excitement and new hope I made an appointment. In November of 2008 we met with Dr. Standard in Baltimore Maryland at the Rubin Institute. I was extremely nervous. Before we left we had decided that if amputation was our option again, we would go ahead with it before she got any older. To be quite honest, I expected that to be his response.
So...when he came into the room after examining her x-rays and said, "We can fix this." I nearly leaped out of my chair to kiss him. He also told us that if we had come to him in the beginning he would have told us the same thing the doctors at Shriner's did. Without a hip joint or a kneecap lengthening just doesn't work. Waiting, in Trinity's case, had actually been a blessing in disguise.
The funny thing is...
I feel like we were given signs throughout our journey that brought us to Baltimore. A lady in the waiting room in Shriner's had told us about a relative that was seeing specialist in Baltimore. Our neighbors told us of a long time friend that was taking her son to a specialist at the Rubin Institute in Maryland. When I searched online I often come across the name Dr. Paley, who happened to work with children like Trinity in Baltimore.
He found five hospitals across the US that he wanted to visit. The first of the five was the Children's Hospital in Ohio. I tried not to get my hopes up as we headed off for that first appointment. When the doctor finally come into the room after examining the x-rays I expected to hear the same thing we heard at Shriner's. Much to my surprise, I was wrong.
The doctor agreed that Trinity's length was severe and that there didn't appear to be a hip joint or a kneecap, BUT she did notice that there was a small gap between the top of her femur and where her hip joint should have been. She wanted to wait a few months to see if there might be something there that just needed a little more time to calcify.
After six months we returned for more x-rays. When the doctor came in with a beautiful smile I could hardly contain my excitement. She brought up the x-rays on her computer and showed us a tiny white area where her hip joint should be.
"I do believe that is a hip joint." She had said.
She hadn't wanted to get our hopes up but she told us that if she was right, lengthening might be an option for her. She did stress the word might. Even with a hip joint there was still the possibility that we couldn't lengthen. There are for classes for P.F.F.D. with D being the worst. Trinity was classified as a C, meaning lengthening could be possible but unlikely in most cases.
For almost two years we traveled back and forth for x-rays. Each visit building my hope up just a little higher. Everything was going well. She did have a hip joint as well as a knee cap, both just needed more time to calcify so that they could be picked up on x-rays. To make up the difference in length during this time she wore an AFO prosthesis with a pipe and prosthetic foot off the bottom.
Then...Her doctor transferred to a hospital in Utah and we were to start seeing her partner. We were sad to see her go but we were still hopeful and willing to work with her team member. As we waited for the day we were to meet her new doctor we found ourselves back at Shriner's. I'd made an appointment to get a prosthetic made there. After we arrived for the appointment we were informed that we could not come there for a prosthetic without meeting with the doctors. We agreed to an evaluation so that would could get the prosthetic. The evaluation turned out to be a nightmare.
After taking x-rays, four doctors came to our room. I'm not sure of the purpose of three of those doctors since they simply stood in the background without a single word as the other doctor proceeded to tell us that we were making a bad decision. He told us we were ruining her quality of life and that our efforts to save her leg were a waste. I tried to bring to light the fact that they wanted to amputate her leg based on the fact that she had no hip joint or kneecap and if we had allowed them, we would have never known they had been wrong. He refused to listen to me and was firm in his belief that we were making the wrong choice. After I informed him that we already seeing doctors in Ohio he let us know that he was good friends with the doctor we were being switched to and would contact him.
After our visit with the doctor at Shriner's I was a little anxious about meeting with her new doctor and what he might say, and I was right to be. He was very short with us and he made very little eye contact as he was speaking with us. He told us that her other doctor had only been getting our hopes up and that there was nothing that could be done to save her leg. He asked to see her walk on her prosthetic and barely gave her time to stand after putting it on before he walked off.
Everyone of us were angry and devastated. Shortly after he left the room the nurse suggested we meet with the entire team of doctors for an evaluation. We agreed but were not so eager to return. As we waited for the evaluation day to arrive we made the decision to seek out a different doctor no matter what they had to say.
The evaluation was short and we were informed that we would not know their conclusions for about 6 to 8 weeks. Even when the report came in the mail stating that Trinity was in fact a candidate for lengthening, we were firm on our decision to seek out a new doctor after the way we had been treated.
For a while our search for somewhere different was relentless, but after a few months it began to fade. By this time she was getting older and we both knew that if amputation was going to be our only option we needed to make a decision soon. We both wanted the transition for her to be as easy as possible.
Then...
I found Lexxi's mommy on MySpace. After browsing through images online I stumbled across a baby girl with the same leg condition as Trinity. I sent a message and waited for a reply. It didn't take long and her mother was more than happy to talk with me. For several days we exchanged messages. Her daughter had already begun the process of lengthening and she shared with me her doctors name and where he was located.
Armed with fresh excitement and new hope I made an appointment. In November of 2008 we met with Dr. Standard in Baltimore Maryland at the Rubin Institute. I was extremely nervous. Before we left we had decided that if amputation was our option again, we would go ahead with it before she got any older. To be quite honest, I expected that to be his response.
So...when he came into the room after examining her x-rays and said, "We can fix this." I nearly leaped out of my chair to kiss him. He also told us that if we had come to him in the beginning he would have told us the same thing the doctors at Shriner's did. Without a hip joint or a kneecap lengthening just doesn't work. Waiting, in Trinity's case, had actually been a blessing in disguise.
The funny thing is...
I feel like we were given signs throughout our journey that brought us to Baltimore. A lady in the waiting room in Shriner's had told us about a relative that was seeing specialist in Baltimore. Our neighbors told us of a long time friend that was taking her son to a specialist at the Rubin Institute in Maryland. When I searched online I often come across the name Dr. Paley, who happened to work with children like Trinity in Baltimore.
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