Or also known as Proximal Femoral Focal Deficiency. This is a page that is about my son Julian that has PFFD and Fibular Hemimelia. This is our trials and our joys regarding this.
HOW WE FOUND OUT
The first couple of posts were originally on www.pffd.org. It is a website for those who either have this condition or those who know of people who have this condition. Julian is now 13 months and is growing faster than a weed and finally walking around some. He will be walking on his own soon. He is our only child for the moment and we are thrilled that he is here with us.
We had gone to my OB for a regular ultrasound here in Town Lake in October 2005. My entire pregnancy was going well, as well as can be anyways when you are throwing up all the time in the beginning. We had the ultrasound and the Dr. of the day came in and told us that everything looked good except for his legs. They looked a bit shorter than what they should be. They were going to send us to a perinatologist by the hospital that I had Julian in Marietta. We weren't to worried at that point, But after we had plenty of doctor's give me an ultrasound they couldn't tell us to much until Julian was born. That way they could see what the problem was instead of trying to read an ultrasound and guess, but they had a pretty good idea it as PFFD. They didn't know however that he would have Fibula Hemmomila.
We were also sent to see Raymond Morrissey. He was a leading doctor in the field of PFFD, from what I understood. He has now retired and our orthepedic surgeon is Dr. Michael Schmitz. Across the hall is the Orthotics and Prosthetics office where Collen Coulter O'Berry works. She is Julian's physical therapist and we love her as much as the rest of our doctor's.
By the time Julian was born in March of 2006 we thought that we were ready for anything. Except for my c-section because Julian refused to turn. He was breech. What luck... My nurses didn't know that we knew about his legs so when they told us that his legs were a little short we confirmed that we knew that. Then they said "oh, good. So you know then that he has three toes on his right foot?" I was surprised at that. Then they showed us. His little foot was so narrow and long, but hand only three toes. It was so cute. The prints of his feet they gave us at the hospital is fabulous.
And now he is growing and exploring. He is a strong toddler and is pretty much ready for anything. And an attitude to go with it if he doesn't get his own way.
JULIAN IS TURNING 3!!!
His birthday is in March and he is growing into such a little boy!!! Active as anything and is constantly telling me to "Check it out!" Whatever "it" is...
I worry that as he grows the kids that are his age will start to pick on him. I know that there isn't really anything I can do about especially if I am not there. We were in church a few Sunday's ago and he was in his Nursery class and was bitten on the side of his face by a kid in his class. I saw the kid later after I had taken care of Julian and wiped his tears and gave him a hug. The kid I am sure is a normal height for his age, but just showed me how short Julian is compared to the rest of his class. He is as tall as the one year olds in church.
He has been fitted with new AFO's for the past few years but I think we are getting to the stage where we will have to start making decisions. I just don't know when yet. We will be seeing Dr. Schmitz here at the Atlanta Children's Hospital on the 11th of March. We have been in a wait and see situation ever since he was born.
We will see how it goes.
JULIAN IS ALMOST FOUR...
and is a busy boy. Nothing seems to deter him until his mommy tells him no. We saw Dr. Michael Schmitz last in December. We are still in the "wait and see" mode. Dr. Schmitz had decided that he doesn't want to do anything while Julian is still able to move about and function without any issues. He tells us that he wishes more people could see Julian and the way that he is because he seems to be a unique case. We are told that if you just look at his x-rays that you would assume that he isn't walking and wouldn't be able to walk considering that he really doesn't have ankles or knees, not like we have anyways. But then you look out into the hallway and there he is running up and down it trying to chase his little sister, who doesn't have any issues. We were told that Julian would be a good candidate for leg lengthening in his future. What would happen to Julian is that they would lengthen one leg and stop the growth on the other to be able to match them to be equal lengths. But what happens below his knees is still up in the air. He won't be able to wear a shoe lift for the rest of his life because of the difference in the lengths below his knees. A shoe lift wouldn't be stable. We have found however that when Julian walks long distances with or without his shoes and braces that his back starts to hurt. We constantly have his shoes on until his feet get sore with his braces. I am so grateful for our physical therapist, Colleen O'Berry, and our orthotic guy, Ed Barber. They are awesome.
I just wanted to give an update. I know that we are coming soon to decisions that we as his parents will have to make in his best interest. That is going to be a hard time because we don't know what his best interests would be as a young man. We have to think of now and in the future. How will our decision affect him now and in the future. Such a responsibility.
So we will see what happens. We don't go back until August of 2010 to see Dr. Schmitz again.
JULIAN IS FIVE -
We had gone to see Julian's orthopedic surgeon in March. He said to come back in a year. We are still in the same place that we were in the last time that we saw Dr. Schmitz. In a way this is a good thing since that means that Julian is doing well as he is.
We will see what happens next year.
JULIAN BEING CAST
The following are pictures of Julian being cast for his braces. He is cast every time that he gets a new pair and adjustments are made in between should he start getting red spots for blisters. In the beginning he hated to get measured and cast for braces and I would have to hold him on my towel covered lap to keep him from squirming. Now he just goes in and helps out by making sure that his legs are stretched out and pants are out of the way.
JULIAN JUST TURNED EIGHT AND THERE ARE BIG CHANGES HAPPENING....
There has been so much going on that I had forgotten to update this page. So to start at the beginning.. In October of 2013 we had a visit with our Orthopedic surgeon Dr. Michael Schmitz. It was the dreaded appointment. Julian had been having problems for awhile. His back, feet and legs were hurting almost constantly. The wear and tear on his shoes was extraordinary. He had a lift on his right leg and it would drag when he walked. It got to the point where it was really heavy and the lift couldn't go any higher because then it wouldn't be stable enough for him to continue walking. Dr. Schmitz said it was time. We needed to decide if we were going to amputate now or later. There was no rush at this point but Julian was just getting top heavy. His lower body just couldn't handle the weight any longer. (The kid is built like a small planet. His upper body strength is amazing.)
It took a lot of prayer to confirm that it was definitely time to do with the amputation. We had thought that maybe lengthening would be good in our case but that would just be lengthening his femurs but wouldn't allow his lower half to handle the weight of his upper half. He needed something that would allow him to grow up and still give him the strength and stability needed to handle his weight. So we decided on having both of his feet amputated. It is called the Symes amputation. It is the amputation of the feet through the ankle. He has his tibia but no longer has any of the bones that would be his feet. The doctor kept the heel pad which would wrap up to be attached to the front of the leg. It gives him the ability to walk on his legs like stilts and still have tough skin and muscle to be able to do so.
The surgery was scheduled for January 7th of 2014. We wanted to be prepared and we wanted to prepare Julian. We didn't hide anything from him, we didn't evade any questions that he had. We were very open and honest with him. But we have been like that from the very beginning. Never shielding him from what the doctors had to say to what might happen in his future. So Julian took this in his stride. My husband and I were not quite to that point yet. There was a lot of grieving and still is sometimes. As an adult it is easier to see the long range consequences of these actions. I think kids don't think that way. They roll with the punches and get up and keep going. We as adults have to think about everything and then worry about it all too. It is very tiring.
In preparation for the surgery I made sure that I had all the necessary things in place to keep Julian going with his education. I have to say the school that Julian attended was very helpful. He was able to have Homebound Study. That is where a teacher from the school will come to the house and work with the child to keep him on track. Ms. Diaz was fabulous to work with!
So the big day rolled around, I had my mom watching my oldest daughter because the day of the surgery was the day that school started again from Christmas break. My other daughter was with family so all my kids were taken care of. So of course, on the day of the surgery the temperature is 5 degrees outside. We had an arctic blast hit us that day and every time we had a follow up apt it was cold. The warmer nice weather were always on the weekends. Go figure.
The surgery was scheduled at Children's Hospital of Atlanta/ Scottish Rite off of Meridan Mark Parkway in Atlanta. The staff was fabulous. We got there and was registered pretty quickly and in our pre-op room without any difficulties. Then it was the waiting for all the required staff to come and get the his medical history and anything else that they needed from us. Julian was nervous but you couldn't really tell. He was completely wrapped up in his DS. About 20 minutes before the surgery the nurse came in with a liquid that would help him relax. They told us that depending on the child he might get a little goofy. We were actually disappointed, Julian didn't react that way at all. He was a bit more talkative than he had before but that would have been normal for him if he hadn't been going through this. I think that tells you how nervous he was. Then the next nurse came in, we said our loves and kisses and he was rolled off to surgery. I cried at that time. It was hard to watch him being wheeled away to have his feet taken from him. Despite crying I had to remind myself that not only is he a strong boy but that there are worse things in the world that just having no feet. That is a thought that I constantly keep in my mind as we go through these...challenges.
We waited for about four hours before we were able to see him again. The surgery itself took around three hours and then once it was over recovery was another hour. Dr. Schmitz came and told us that it all went well. Because of the deformity on his right foot that was the most difficult to complete. He was really glad of how it went. So while Julian was recovering we moved into the hospital room where we would be staying overnight. It was a tight and cramped room. And we had a lot of family visit that evening. I am so glad that we had a lot of family showing their support but on the other hand there were to many people in that tiny room. At one point I made everyone get out because the nurses needed room to check on Julian and do the things that they needed to do to care for him. Some were not happy about that. But you know what? Who cares. If you are in the way then I am going to tell you to get out of the way so that the nurses can do their job. That is just how it is.
We ended up only staying one night in the hospital. I thought that was kinda short myself. I figured that we would have been there at least two nights but Dr. Schmitz said it is easier to recover in a familiar environment and the hospital is full of sick people. I understand that! Dr. Schmitz had wrapped up Julian really well so that there were no changing of the bandages until we saw him again a week later.
Before we left the hospital the staff wanted to make sure that he was off the morphine and could handle eating regular food. Which was not a problem. You know life is different in the hospital, where you are hooked up to the IV and have drugs at the call of a nurse. So much easier. But once you have left the hospital that is it. Not so easy. Definitely not as easy with Julian. His medication started wearing off on the way home. We were halfway home and he was crying because of the pain. We had to go to Wal-Mart to pick up the Lortab that would ease his pain. It was one hectic and tiring night for all of us. As we moved on through the week we ended up working on a four hour schedule of Lortab and Ibuprofen and Valium. The night is always harder to handle...but we didn't know that there would be muscle spasms. The Valium helps to control that. He was in so much pain that weekend that I had to call the doctor on call and she sent in a script of Valium. That helped immensely. Thinking about it now it didn't seem like such a long time, but while in the midst of it the time was forever and with very little sleep.
So for the two weeks after the surgery Julian was watching movies, TV and or playing is video games almost constantly. It was a great distraction for him when he was in pain and couldn't take any more medication yet but when the he is trying to convince me that in order to ease his pain he should play video games is when I knew that he was getting better we started to ease up on all the game playing. That was really hard. Julian is such a gamer. He loves his video games and I make sure to limit him so that he has other interests to occupy him. Thank fully he also loves to read. Getting him to cut back after the surgery and after he started to feel better was the fight of a lifetime. I was so glad when he went back to school!
Sometime during the second week and third week of being home Ms. Diaz started to come over to help with his studying. I am so grateful to her that she was able to do that for Julian. I appreciate it to no end. Then the week following that he went back to school. YAY!!
It has been a wild ride and we are not done yet. His left foot has healed quite nicely. It looks really good and clean. We are hoping that we will be able to get his left leg cast and get the ball rolling for legs! His right foot however, is giving us problems. Some how he managed to get bacteria under his scab that was on his right foot. He didn't have any of the normal signs of infection like fever, heat or redness around the wound but he did get a really stinky roadkill kind of smell. So Julian had to go back for a second surgery where Dr. Schmitz had to remove the scab that was doing so well in order to reach the bad flesh that was making a stink. Unfortunately, that couldn't have been done in the office because they needed to get under that scab and Julian would have had to be under for that to happen. So March 5th is when my husband noticed the smell. I contacted the Dr. office that evening and they contacted me the next day for me to see the Physicians Assistant Mr. Ed Wilson. We saw him on Thursday the 6th and Julian had the second surgery on the 7th. We were doing fine until last Thursday the 13th when the smell started again. We managed to get an appointment with Mr. Wilson for the 14th where he cleaned up the bad spot and I keep putting wet to dry dressings on the wound. Wet to dry is where you take gauze and get it wet with Saline solutions. Squeeze out the excess and place the wet gauze on and into the wound. Then wrap with clean gauze and then with ace bandages. They get changed out twice a day. I am not sure how it works but when you take off the dry dressing the gauze will pull off the yucky stuff that you are trying to clean up. It cleans out the wound that way. I am also told that now that the scab is off healing will be quicker than if the scab had stayed on. We wanted the scab to stay on so that the body could heal itself naturally, but when there is a smell coming from the wound it needs to be taken care of as soon as possible.
So that is where we are at the moment.
JULIAN AND HIS BIG NEWS
So, If you have read the prior pages then you know that we are having issues with his right foot. It still smells but not as bad. Thankfully. But we did get some good news. Last Wednesday his left leg was cast. Meaning in two weeks Julian is getting his first prosthetic leg. Yay!!! It is about time. I thought that we were never going to get to that point. Unfortunately, because of the right leg we still have to hold off on that one. But at least he will be up and walking or hobbling along with a new leg and crutches until his right foot heals.
He did tell me the other night that he is excited about getting his new leg because he then will be able to walk on the walls. I am not quite sure where he got that idea from but I didn't have the heart to pop that dream. I figure that he will figure it out when there aren't any suction cups on the bottom of the leg.
UPDATE - 5/2014
Julian has been cast for his right leg. Finally!!