Living with VACTERL Association


My Story – Submitted by: Gerald

I was born in 1957 with VACTERL Association. I have a Klippel-Feil in both my cervical spine and lumbar spine (L1-L4 congenitally fused), a syrinx in lower cervical spine, urinary tract fistulas, imperforate anus, and a horseshoe kidney. I have a history of distension, pseudo-obstruction (for which I have been hopitalized and NG’d several times), two obstructions due to adhesions, and these were surgically repaired. I also have a syrinx in my lower cervical spine which is stable. I was also born with an imperforate anus and required a pull-thru at birth. I had laminectomies in both my cervical and lumbar spines (L1-L4 is congenitally fused with hemi-vertibrae, scolioses, severe stenosis in the foramen and central canal). I have a horseshoe kidney but do not have any renal issues that I am aware of, though I am prone to UTI’s. I am largely incontinent and have severe motility problems and am sometimes unable to pass stool for a week at a time. I am usually always distended. I am often incontinent, having to use the bathroom in excess of 15 to 20 times per day. (I swing between loose stools and constipation). I am unable to work b/c of my bowel issues. If I am not having loose stools I have some control. If the stools are loose I have little or no control. I experience peristalsis which causes me to evacuate involuntarily. I have been hospitalized many, many times for pseudo-obstruction and severe gastritis. My sigmoid colon has swollen completely shut on several occasions, and usually when this happens my WBC shoots up to above 20,000. The number of times I have repeated this ordeal has made life hell on earth.

I am writing for three reasons. First, I wnat to learn more about my particular incontinence issues. Secondly, I would like to help or participate in any studies that are ongoing. I have unfortunately, not contributed much in my life and would like to give back to the world. I would like to help spread the message that “birth defects” are forever, that one afflicted with them lives with the reality that they will never experience even for one day, what life might have been like without them. I certainly advocate and endorce the whole “baby your baby” concept. Finally, I wonder how rare of a creature I am. Are there lots of survivers of VATER and analplasty from the 50’s. Am I lucky to have survived? How do they fare? Are they able to cope and lead successful lives?

People tell me I am strong to have survived but I don’t see it this way. I see it as luck of the draw and the will to survive is universal is it not? I see myself as a product of modernity and this provides a kind of fascination…to have survived because of the necromancy of science and medicine and I am glad to have survived to see these odd days of miracles come to pass. It’s been a weird ride.

Webmaster’s Note: VACTERL association is a disorder that affects many body systems. VACTERL stands for vertebral defects, anal atresia, cardiac defects, tracheo-esophageal fistula, renal anomalies, and limb abnormalities. People diagnosed with VACTERL association typically have at least three of these characteristic features. Affected individuals may have additional abnormalities that are not among the characteristic features of VACTERL association. Source: Accessed 19 April 2016.

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