Victoria's Victories with PFFD
Our daughter Victoria was born December 2011 with Congenital Short Femur/PFFD. A rare congenital/birth defect. We were not aware prior to her birth, it was shocking to say the least. I had a complicated pregnancy with (ICP) aka Cholestasis, she was a 'lateterm premie' weighing in at 5lbs 11 oz. We were in love from the moment she was born. Although we weren't expecting a baby with a 'little leg', she's been a blessing to our family. For the past 3.5 years we have been searching for answers, researching, and reaching out for guidance to navigate our way through this journey we are now on with our sweet baby girl.
What is CFD/PFFD? Congenital Femoral Deficiency (also known as PFFD: Proximal Femoral Focal Deficiency) "“ leads to a congenitally short femur with different degrees of a lack of formation of the hip and knee joints. In the mildest cases the hip and knee are properly formed and only the femur is short. In more severe cases the hip is severely deformed, the knee is unstable or has a flexion contracture and the femur is short. In the most severe cases there is either no connection between the femoral head and the femoral shaft or also stiff (limited in its motion). The treatment for cases with a well-formed femur is lengthening. For the more severe deformity, the superhip and superknee procedures are used to reconstruct the there is no hip joint at all and the knee is not only contracted but hip and knee before lengthening. If there is a true pseudarthrosis (false joint) the superhip 2 procedure is used to build a femoral neck and connect the femoral shaft with the femoral head. Finally in the most severe cases Symes amputation and Rotationplasty
We have choosen Dr. Paley for a second opinion. For many reasons, that are stated here at http://paleyinstitute.org/?q=physician-education. He has a 99% sucess rate. Limb lengthening is a long and painful process with many risks. As Victoria's mother, I don't want to gamble on losing her right leg.
20 months old
LEARNING TO WALK!!! YAY!
FIRST X RAY AT 27 days old....
She loves her ice cream! <3
Why the FUNDRAISER? We need to raise funds for Victoria's Limb lengthening surgery with Dr. Dor Paley, Pioneer in Limb Lengthening in September 2015. As well as funds to help with medical expenses and travel/accommodations while we are in Florida for 4-5 months. From the research we have done, we feel that it is important to be proactive, and correct her condition while she's young and before other parts of her body are jeopardized by her gait with a short, bowed and twisted femur. We live on the West Coast (Pacific North West) and Dr. Paley is in WPB Florida. If there were a Dr. closer to us, and had the same experience as Dr. P. We would love to seek care closer. There just isn't a orthopedic surgeon in our area, with the kind of expertice as Dr. P. His patients come from all around the country/world to benifit from his skill and experience.
Please help us get our daughter Victoria to Florida for her surgery with Dr. Paley. He is the BEST of THE best. http://paleyinstitute.org/ Every little bit helps. Any funds that remain after our consultation with Dr. Paley will be put into a fund for the future medical and travel expenses. Our goal amount is just an estimate. But know in the long run expenses will surpass what we are asking for at the moment. From what we estimate the costs associated with such a long stay, the condo rental, airfare, transportation, medicals supplies, medical copays and the cost of living, we are expecting a cost of about $15,000 for the 4-5 month stay.
OUR THANKS TO YOU
THANK-YOU, to family, friends, and the unknown that choose to donate to our cause. Thank you for your generosity, love and prayers. From the bottom of our hearts, THANK YOU!
Her little foot. So tiny, even at 20 months. :)
Waiting for her to wake up from the procedure....
Victoria was born December 2011 with Congenital Short Femur/PFFD. As you may know, we did not know that she would be born with a congenital defect. For the past 4 years we have been searching for answers, reaserching, and reaching out for guidance through this journey we are now on with our sweet girl.
Victoria has been though numerous surgeries including limb lengthening this past fall/winter of 2015/2016 During which time we lived in Florida for 5 months.
After returning home Victoria had a spica cast for 4 weeks. After the spica cast was removed we noticed that her leg was bent and was not extending or flexing. It was bent at a 45 degree angle (at the knee). At that point we were told to start PT and use a special splint. We have done everything recomended with little progress for the past TWO months.
When we met with Doctor Paley 5/10/2016 and had imaging done they found that Victoria has a 9 degree deformity of the knee and a 40 degree deformity within the soft tissue of the knee. The soft tissue deformity can be resolved with aggressive PT by Paleys team.
This week we got news that Victoria NEEDS aggressive physical therapy by Doctor Paleys team for 3-6 weeks.
This means Victoria and I (Kelli) must leave our home in Washington State once again and travel to West Palm Beach Florida to resolve the serious problem with Victoria's leg.
I'm updating Victoria's story here as well as 'YouCaring' because I know some prefer to help out through gofundme instead of you caring.
Thank you for taking the time to read our update. Victoria is in dire need of rehab and we would like to make it back to west palm beach to the Paley institute by June 1st to start aggressive therapy.
Thank you so much for your time!
Thursday we had our bimonthly appointment with Dr. Packer (She handles Dr. Paley's post op patients).
Our day started out with physical therapy before our appointment. That session didn't end well, as Victoria wasn't cooperating and didn't earn her 'treasure chest'. She was also upset because she had to get measured to see how many degrees her leg can bend.
She hates getting measured, but it needs to be done as the Doctors need to know if she is losing, gaining or maintaining flexion and to what degree. Victoria is losing some flexion.
After that we headed to X-ray. Victoria resisted most of the time. Lots of screaming and crying. We had to hold her down for a couple of the views that were needed.
After that we waited for quite some time to be called back. Once again Victoria started to freak out. She knew we were about to see a doctor. She has a lot of fear and anxiety over being in the actual room. She kept trying to escape, using the wheel chair to ram the door repeatedly. Then she started trying to get out of her wheel chair which is dangerous because she still isn't steady without her walker. While trying to keep her safe she was pinching me, trying to bite me, and screaming. I remained calm and tried to reassure her that no one was going to hurt her.
Finally Dr. Packer came in. She asked me if *I* was okay and asked if I would consider stopping lengthening early because she wasn't sure how much more of Victoria's behavior I could handle. The tears started to fall. I told her that we didn't come this far to quit now. She still insisted I at least think about it. She measured Victoria's new bone, which came out to be about 4.3 cm. She checked out Victoria's pin sites. The same pin site that showed signs of infection before was showing signs of infection again, PLUS had some hyper granulation tissue protruding out of one site.
She ordered another round of antibiotics and a medication called Elavil to help with Victoria's behavior issues.
Then PA Luke came in and added another piece to Victoria's fixator so we can continue her daily turns. There was a lot more screaming and crying involved even though it was a painless and quick process.
We have decided that we are NOT quitting the lengthening phase of this process. We have come too far to quit now. 5.5 cm is a very realistic goal and we only have two more weeks until we achieve our goal and stop turning/lengthening. I've decided to cut back on Victoria's pain meds to see if that could be contributing to her behavior issues. I know she is going through A LOT everyday and it's taking a toll on her.
Our next appointment is on the 30th of November with Dr Paley. By then Victoria will have met our goal of 5.5 cm plus some. At that time we will discuss starting the consolidation phase. This is when the new bone consolidates into hard bone.
Victoria continues physical therapy every day at the clinic and at home, daily turns three times a day, pin site care every other day and now fighting off a possible infection.
This little girl shows daily strength and perseverance. She's so strong willed, which is a blessing and a curse. I look forward to the day when this is over so I can have the Victoria I know back. I'm sure she feels the same. This is HARD, but we continue to take it one day at a time.
Thanks for following our journey! We appreciate all the prayers and support!
#victoriasvictories #pffd #shesafighter #limblengthening