Life Experience With Sprengles Deformity

I did post earlier to someone else's question and then decided to write this. I was diagnosed with sprengles at birth, there were many concerns that I wouldn't survive the first week. I did, but that was the easy part. Mine is on the right, but also includes the doubles s curves on my spine as well as deformity of the thoracic cage itself. Basically, if you wanted to insert a tube in my lung on the right side, you do not need a rib spreader, they are far enough apart with room to spare, the left side is too close together to be almost fused in some areas. I did have surgery at the age of 7, there wasn't any choice really. I didn't have any real rotation and wearing tank tops or undershirts like that were not an option because of the height of my shoulder. They did find a fair amount of muscle tissue hanging in a sheet like fashion on my shoulder when they did the operation, sooo they opened me up from neck to tail in order to try and put things into a somewhat normal order. This is said with a good sense of humor, I have learned to have a great one. After the healing and relearning of things, my parents said "ok, doc says your good, get to being a kid now." That worked for a while, then adulthood hit me, got married had children, was doing ok sort of until my mid thirties. That was when it was discovered that I also had a tethered spinal cord in the lumbar region, first surgeon said it should only take 4 hrs in o.r. then a couple days in hospital. that I should be ok after that. yeah I wish, try 16hrs and prying the darn thing off. not once but then an additional 3 times with the same kind of hours. after the 4th surgery the peds neuro told me and my parents that it appears as if this is the result of the deformity as well and that any more surgery should only be thought about if I lose the use of certain internal things. Now 15 yrs later, they have found that the spontaneous fusion in my neck has went to the extreme in making one solid bone from c7 to t4, and c6 slipped a little. I still have a great sense of dark humor about it. I have to, because they say that even a little accident of 15 miles an hour can turn me into a quad. My mom asked me once if I would have been better off not being born. first I told her, I don't think she raised her hand and said I want a child like this. and second no, someone had to be me. Who better then someone who will not give up or in. Then I thanked her for being my mother
ravensfare ravensfare
2 Responses Jan 13, 2013

You sound a wonderful person and thank you for your story,

Hello, I was born with Sprengels deformity (left shoulder) KFS and congenital scoliosis. I have had three surgeries and an attempt at a fourth but woke up on the table,so needless to say surgery was cancelled. I have had two children even though i was told it was a risk managed to carry and deliver both naturally. Life for me has been free of pain and discomfort although as I am reading people's comments I see am very lucky regarding the pain or my lack of it. Very glad to see that there is some info finally on Sprengels and Klepel File Syndrome

there is a facebook page that has about 115 people with varying degrees of sprengles and kfs, sometimes they go together. They are a great bunch of people, you just have to ask to be invited they are a private group. The upside is they are from all over the world and are trying to find out as much as they can about these deformities.