Hello friends! Reflecting back on 2012 it is hard to look beyond further than the past 3.5 months. So much has happened since our last post. I thought I would be updating more than I have. Honestly at the end of the day I am drained and have not been able to find the words to describe all that Hayden has been going through from day to day. It most certainly has been the hardest thing we have ever faced in our lifetime.
Shawn and I started this blog to keep our family and friends updated on Hayden’s journey. We also have opened up our lives to strangers….families that are going through similar situations or will be embarking on this journey in their future. Before beginning any of this, before any of Hayden’s surgeries, we searched the Internet for others who had already been down this road. As parents we wanted to inform ourselves, get a glimpse of what was to come. We were so grateful to those who allowed us in and shared their stories with us. We found ourselves celebrating inch by inch with these amazing children. We would cry tears of joy as we watched these kids get both feet on the ground. We were relieved to see these children doing normal things and see their smiling faces with fixator on their legs. What I did not realize at the time is that you really only pick up the camera when your child is smiling and feeling well. While we have posted many pictures of Hayden smiling ear to ear and looking happy as ever this does not clearly depict what the past few months have been like for her or us for that matter. I want to give an honest account of what this journey has been like.
So now I speak to the families who have found our blog in search of what is to come for their own child. I must emphasize that what I share with you is our own story. Each child that goes through lengthening is different. What we have experienced may not be what another experiences in his/her own lengthening process. We thought we came prepared and thought we had an idea of what this was going to be like. We most certainly did not. In the very beginning of our search to decide how and who will treat our daughter we were given a very simple and straight forward plan. It sounded so easy…hip and knee surgery, 2 lengthenings, possibly fusion of a growth plate and vuala two even legs. It is not so simple; there is a lot of pain, tears, and possible complications within that plan. There have been many times at the end of our long days that Shawn and I have looked at each other and said we had no idea how hard this would be.
When you have a child with PFFD you are faced with extraordinarily difficult decisions, to lengthen or not to lengthen and in some cases to amputate or not to amputate. It’s a decision that you make on behalf of your child, a decision that you must live with and will question many times. “Is this the right choice, is this what she would choose for herself once an adult.” Seeing the most important thing in your life endure and suffer so much because of a decision you have made comes with a heavy weight to bear. I do believe we have made the right decision for Hayden, but I don't believe that lengthening is the right thing for every child but, in Hayden‘s case it is. If you choose to lengthen you must understand the commitment that goes with it. It is something that was not explained to us. This has been and will continue to be a full time job. The therapy is beyond intense and excruciating for your child. I have literally seen grown men yelling and crying when doing the same stretches we have to do to Hayden. When you walk into the therapy gym you can hear kids screaming and yelling from different rooms. Your child does not have to go through this once a day... but 2-3 times a day. You, the parent are responsible for doing these painful stretches to your child at home every single night and twice on days the therapy department is not open. If you are making the decision to have the lengthening done on your child then you must understand the commitment you the parent have to make… therapy twice a day, 7 days a week for a minimum of 8 months. We still do not know how long Hayden will be required to do therapy once the fixator is removed.
Pin care is another painful part of this process. We do pin care daily. It takes us about an hour to bath and do pin care. In the beginning it easily took us 1 ½ hours to do this. Hayden hates it. It has gotten a bit better but she is still terrified of it and, at times, will scream and cry out while we are doing it.
I had mentioned complications. We are dealing with our own complications right now and I will get into that shortly. Honestly it is rare to see any child get through this process without experiencing their own complications. You hope your child will be the lucky one but they just seem inevitable. Complications that we have seen in the patients since our time here…. Infections, nerve pain, hip dislocation, pre-consolidation, loosening pins to name a few. It is a very hard, hard road. These kids are amazingly brave, resilient, and strong. I admire each and every one of them. Hayden is my hero. At the age of 4 she has gone through more than I have come close to enduring in my 35 years. I love her and I want this all to be a distant memory. I want her to wake up without pain, not to be afraid of “what are they going to do to me next”. I want her to get back to being a kid again. I know we are getting there, just wish we were there already.
WHERE WE'RE AT TODAY
Alright, now to give you an update on our precious little girl. I think I will start with where we are at now and go backwards. Hayden had a great day today. She was as happy and silly as ever! We had a follow up on Jan. 3rd (we have x-rays and follow ups every 2 weeks). Hayden’s right leg is longer than her left!!!!!! In the beginning she had a 62mm discrepancy. Her x-ray today showed that she has lengthened 73mm. Our goal has been to lengthen 80mm. This is the most Dr. Paley will lengthen on her due to complications if you lengthen beyond that. There have been plenty of times in the past couple months we did not think it was possible to reach this goal. But here we are… the end is in sight. We will likely be going home in a couple of weeks!!! We have been waiting for this day for so long but now that it is here I have mixed feelings. I am feeling very nervous about leaving all of our support here. If there are any concerns or problems we have immediate answers and intervention here. We will not have that at home. I am also feeling sad that I will not get to spend all my time with Shawn and Hayden. We have grown so close during our stay here.
We originally were doing 4 turns per day= 1 mm. Hayden had been experiencing severe nerve pain in her foot so Dr. Paley had us go to 2 turns a day=1/2 mm each day. It made a huge difference for Hayden. She does still experience some nerve pain in her foot but nothing like before. We hoped the nerve pain would be the only complication we would run into but unfortunately it was not. We are currently dealing with some of the pins loosening. It has been explained to us that it is her body trying to reject the fixator in a sense. On the x-ray above you can see that there is what they call a “halo” around the pins just above the knee. The bone has been eaten away causing dead space and cavities around the pins. Why is this a problem? It has caused much more pain for Hayden especially with the already painful stretches we have to do to her. The pins move around, tethering through muscles, tendons, nerves, and the bone. It also is a breeding ground for infections which we have been fighting for the past couple months. Hayden does not experience a little pin site infection. Her infections are deep in the bone causing her entire leg to swell, severe pain, and loss of mobility. As soon as she comes off the antibiotics the infections comes back with a vengeance. As meticulous as we are with pin care it does not matter, bacteria seeps down into these cavities in the bone causing the ongoing infection. The fixator is colonized with bacteria and we will now remain on the antibiotics until the fixator is removed in May. Not something I want to do but she is a different child when on the antibiotics….happy, energetic, and just as silly as ever. We were worried Hayden was going to require surgery to replace the loosened pins. This is something we did not want to see happen. We did not want Hayden to be put under again and go through the post surgical pain. It is hard to see the scarring she has already... and even harder thinking about adding to the existing ones with a new set of pin tracks. Speaking of the scarring, she will not only have the pin hole scars, but also the tracks caused by the pins pushing through the skin as we slowly lengthen. As she lengthens the pins drag through all of the muscles and tissue of her thigh. So the length that one gets is the length of the scars left behind which are very deep. The pins are much larger than you might think. They are about the diameter of a pencil (6 mm). Hayden has ten in total. We are told that there can be scar revision down the road by a plastic surgeon but that is something that we would let Hayden decide if she wants once we have finished with all of her lengthenings. Fortunately the actual lengthening process itself does not cause her pain. It's just the side affects of that process that causes all the pain. I still think she is the most BEAUTIFUL girl in the world scars and all. Sorry to get off subject I just remember wondering about the scarring before we started the lengthening and wanted to share with those who may be curious themselves. Back to how we are addressing the loosening of the pins. We were relieved and happy to learn that there was an alternative to surgery. Hayden received an IV infusion of a medication named Zometa. It is frequently used for bone cancer and osteoporosis patients. The bone has a life cycle that simply put consists of a bone building phase and a bone destruction phase. This medication inhibits the bone destruction phase allowing more time for the bone to build and hopefully fill in the dead spaces around the pins and prevent further destruction around the pin sites. It is a slow process and we will not see any changes on the x-ray for about one month. We are keeping our fingers crossed this treatment is successful. Surgery is not out of the question so please pray this infusion works for her. Thankfully the most recent x-ray showed that it was not getting worse so that is a good sign. Unfortunately with this infusion come the bad side effects such as flu like symptoms, bone and joint pain. We had to do the infusion the day before her 4th birthday. Needless to say her birthday is what Hayden would call a “rough one”L. She had back and leg pain and on top of it all the damn infection came back in her leg….her entire leg was swollen and she was in significant pain. Those infections pull the rug from under the poor girl. Thankfully she responds well to the antibiotics and was feeling much better a couple days later.
4th BIRTHDAY AND HOLIDAYS
We celebrated Thanksgiving at the Quantum House. The Breakers resort catered a very nice Turkey dinner with all the fixings. We had some friends from the Quantum House come over afterwards for drinks and to let the kids play.
We were happy to have my mom and dad come visit for a few days in December. Hayden was so excited to have her Mimi and Papa here. Unfortunately her leg became very infected during their stay and it was probably one of the worst periods for her during our stay here. I know it was heartbreaking to see their little angel in so much pain. I had tried to prepare them for what it was going to be like with the stretches but there is really know way to prepare one for what they are like. It gave them a whole new appreciation for how hard this has been for Hayden and her mommy and daddy. Aside from the rough week we were so happy to have them here.
We had a wonderful Christmas here in sunny WPB. It was a low key holiday…just the three of us. It will likely be one of the most memorable Christmas’s we ever share together. Thankfully Hayden was feeling really well after just getting over yet another bad infection. She was spoiled with gifts to say the least. Santa Claus was also very good to her for being so brave and strong this year! A very sincere thank you to all of you who have sent cards and gifts to Hayden during our time here, it brightens her day and she loves getting the mail. The drawings from her little friends back home literally make her entire day. We had decided long before Christmas that we were going to let Hayden have a day free from all things she hates…pin care and physical therapy. It was the first time in three months she did not have to do these things. She was excited to say the least when we told her Christmas morning she was not going to have to do these things. It was probably the best gift we gave her. Her spirits were so high not worrying or anticipating PT or PC. She was not the only one excited. Shawn and I had been looking forward to not having to see her go through these things for weeks. I do not have the words to describe how nice it was for everyone to just have a normal care free day like we once did. We took for granted those kinds of days. 
We had a belated birthday celebration on the 31st for Hayden. We had some friends over to celebrate Hayden’s Birthday and bring in the New Year who are also Paley patients. Hayden had a blast. The other girls are 4 and 5 years old and the sweetest things ever. It was pretty cute to see all three girls hobbling around and getting hung up on each other’s fixators. Nothing was slowing them down. It was nice spending the evening with their parents as well. We all share a strong common bond having shared and been through such similar experiences.
PT , STRETCHES, ETC
Physical therapy is hands down the hardest part of this entire process. The stretches are excruciating and even more so for Hayden now that her pins have loosened. We worked so hard to keep her at 90 degrees on her bends but no longer can push that far because of the loosening pins. Our therapist is cautious with her stretches as the bone is not as strong as it was due to all the bone that has been “eaten away”. We now only push to 75-80 degrees. I think she still has the range of motion to push to 90 degrees but it is too risky to do. I cringe every time I have to do her stretches... afraid that I will literally break her bone. Our therapist assures me that she will get the range of motion back once the fixator is removed. Right now one of the main focuses is getting her extension (straightening the leg). The muscles and tendons are stretched to the limits right now. Her leg always wants to rest in a flexed position (slightly bent). With every mm we lengthen the bone the muscles and tendons only lengthen 1/3 of that. So we have to try to make up the difference with her stretches. It is a constant game of catch up and gets more difficult the longer you go. The muscles never are as long as the bone while lengthening. Her muscles are so tight... and have been for the last couple months. It was explained to me by the therapist that the muscles “get confused and do not know how to work” from the aggressive lengthening we are doing to them. In a sense they have to relearn this new length and relearn how to work properly. There are times that she literally cannot do the kicks in therapy because her muscles simply lock up. Much of our exercises are now passive range of motion. We have been told that this is normal and once we stop lengthening her muscles will have time to catch up and re learn how to work. Hayden has to wear her knee bar throughout the day. This is a lock that we place on her fixator when she goes to bed and now many hours throughout the day. It literally locks her leg to zero degrees (perfectly straight). This can be extremely painful for her. It essentially puts her in a prolonged stretch for hours at a time. Once she wears it for a while she does get use to it and does not mind it. It is a catch 22…you can compromise the flextion/bends by focusing on the extension. The extension is the hardest to get back, so that is where our focus will remain.
Hayden has lost so much weight… about 18% of her body weight. I have been concerned about this all along but it has recently caught the attention of the Paley staff. We had been told that all the kids that go through lengthening lose a lot of weight but Hayden just did not have the weight to lose to begin with. She has never been much of an eater but the last couple months it has been next to impossible to get her to eat anything. I had been worried about how she looked and her overall health for quite some time. As a nurse I am use to keeping an eye on labs, it’s a bit of a security blanket I guess. I spoke with one of the PA’s and he agreed it would be a good idea to get some basic labs on her. He also urged me to get her off the Roxicet which is a narcotic she had been taking for the pain in hopes that her appetite may improve. The medication is known to suppress the appetite as well as cause sleep disturbances. Hayden was waking up 4+ times a night making it a long night for all of us. We were not sure how she would tolerate coming off the Roxicet since at the time she was dealing with this ongoing infection we have been dealing with. Her liver enzymes came back elevated… likely from Tylenol products (roxicet contains Tylenol) and also she had come down with hand foot and mouth disease at the time so you may see liver enzymes elevated from viruses as well. Regardless what the cause, we had to stop all Tylenol in addition to another medication they had put her on to try to get her to gain weight. Thankfully her liver enzymes are back down and she is sleeping much better. As for her appetite it remains about the same. She is a tiny little thing. These days if she is willing to eat cake or a cookie for breakfast that’s what she gets. J
Before coming here I thought we would get therapy out of the way in the morning and the rest of the day would be ours. I envisioned going to the beach every day. We have been to the beach a total of three times since being here. I think I have mentioned in past posts how busy we are. Hayden and I usually leave at 9:30 am and do not get back until 2:30 pm. Hayden has a couple hours to play or nap and then we have to do our home PT which consists of 20 minutes of heat on the leg and then 45 minutes of exercises and stretches. We had no idea we would be doing so much ourselves. There is not much offered to the parents to prepare them or inform them of what this process entails. I put together an outline of a handbook or guide to walk parents through this process... something I have done at my work in the past. I met with some of Paley’s people and they were very enthusiastic and receptive about what I had put together. It is something that needs to be offered to the parents. We are responsible for every aspect of our child’s care, it is so involved and the process is so long. My hope is that something will be put together to make things easier for the patients and their families.
Late entry: Today was a FABULOUS day!
We are trying to take full advantage of the beautiful WPB weather before we have to go home in a couple weeks. We had a delicious lunch at a water front Tiki bar and grill and spent the rest of the day at the ocean. We all had so much fun together playing in the sun and beautiful water. It makes a world of difference when Hayden is feeling well. When Hayden is happy we all are happy and today was a great day. Looking back on the past couple months Hayden was very sick with her infections almost every other week. It really prevented us from enjoying all the things West Palm Beach has to offer. I wish we would have been put on the antibiotics indefinitely a couple months ago. It has made such a difference for Hayden; she is back to herself most all of the time. It is amazing how well she gets around when feeling well. We went the entire day with only one dose of Motrin. Wish we could have had more days like this. Hoping we get a few more of them in before we head home to chilly St. Louis. J
