Many of you that know us have heard me talk about Mattie’s health a good bit. Those of you that are new to our world will get to hear all about it. It is actually kind of an issue for me. I probably talk about it too much. Part of me just feels the need to educate people about KFS, and a lot of you just get used for therapy. 😛
Klippel-Feil Syndrome is a rare disorder characterized by the congenital fusion of any 2 of the 7 cervical (neck) vertebrae. It is caused by a failure in the normal segmentation or division of the cervical vertebrae during the early weeks of fetal development. The most common signs of the disorder are short neck, low hairline at the back of the head, and restricted mobility of the upper spine. Associated abnormalities may include scoliosis (curvature of the spine), spina bifida (a birth defect of the spine), anomalies of the kidneys and the ribs, cleft palate, respiratory problems, and heart malformations. The disorder also may be associated with abnormalities of the head and face, skeleton, sex organs, muscles, brain and spinal cord, arms, legs, and fingers.
Mattie has had a total of 10 surgeries to date. She has had coccyx removal/reshape, tubes in her ears three times, a tendon transfer and muscles moved in her right hand, a spinal fusion from her occipital bone to cervical vertebrae 6, she also wore a halo for almost 7 months with the fusion surgery, she had implants put into the urethras to help with kidney reflux, and had a BAHA hearing implant.
She is a pretty amazing kid and puts up with a lot. I often wonder if her positive spirit comes from having to deal with so much for so long, or if she would have been like this with out kfs. She has what we call Orange and Gray days. If it is an orange day Mattie can be found wearing orange or pink from head to toe and smiling ear to ear. Orange days consist of no pain and hold lots and lots of joy. Gray days usually mean she is tired or that something hurts. Steve and I coined them gray days based on the lovely color of gray that she turns on these days. It is kind of like someone took that orange light of hers and turned it off.
Please feel free to ask me any questions you have about the syndrome. It is not something that we try to keep private. I would much rather people have the real information than to make assumptions that may or may not be true.