A Neurogenic Bladder Due to Marfan Syndrome


My Story – Submitted by: Carl

I first noticed urinary leakage in January, 1985, and simply changed my pjs and underwear. However, within weeks, I found myself changing my underwear several times each day, and staining my bed sheets. After my first accident, I panicked, went to my PCP, and was referred to the first of a series of urologists, none of whom took my situation seriously. The urinary pain began almost simultaneously with the onset of the incontinence, though the spasms were infrequent and not really painful; they were just bad enough to get my attention. I joined the Simon Foundation and through it discovered folks, whom I later discovered to be AB/DL, who patiently taught me much of what I would need to know to get through life as an incontinent. Though I am not one of their number, I owe them much.

The pain slowly grew in frequency and intensity through the later 1980s, and I was shuttled from urologist to urologist. They tried me on Ditropan and Detrol, which gave me intense dry-mouth, so that I inadvertently drank far more fluid than was advisable, with the resulting accidents. One doctor told his nurse to teach me to self-cath; however, my sphincters clamped shut, making catheter insertion impossible. Some of my most memorable accidents during these years involved external catheters coming unglued, once in my boss’s car. In 1990, my cousin called me and told me that Marfan’s Syndrome, a genetic disease, ran in our family, and that I should be tested. When I tested positive, my current urologist read the report, told me that it made sense, because I was dealing with a neurogenic bladder. He said I should keep up the good work, and sent me home.

Meanwhile, the pain was slowly growing in frequency and intensity. By June, 1998, my current urologist expressed exasperation with my complaints that Tylenol was no longer working. He put me under anesthesia and infused four ounces of saline into my bladder, whereupon it went into a “violent spasm” (his words) and squirted the fluid out at him. He had never seen a human bladder behave that way before, did not know what to do, and charged me $45.00 to tell me that he was sending me on to a urological service at the University of Pittsburgh. The university urologist, one Michael B. Chancellor, was the first doctor who took me, the pain I was enduring, and my incontinence, seriously.

At first Dr. Chancellor injected Botox into my urinary sphincters, to disable them. However, the injections wore off after successively diminishing lengths of time. By spring, 1999, he had put me on morphine, which was the only thing that gave me relief from disablingly painful bladder spasms. I spent my days curled in a fetal position on my bed, bracing for the next bladder spasm, which, by May, 1999, were coming 4-6 times per hour, around the clock, and praying for my next dose of morphine. Relief came in August, 1999, when Dr. Chancellor infused RTX into my bladder to destroy the offending nerve endings, and surgically destroyed my urinary sphincters. I am totally incontinent; however, the pain and the threat of reflux damage to my kidneys are ended, so I do not intend to complain.

Slightly off-topic: I also have Meniere’s Disease. Meniere’s is a disease of the auditory and balance mechanisms. I am bi-lateral, and need two hearing aids in order to function in public. Meniere’s patients experience nausea, balance and walking difficulties, nystagmus (an eyesight anomaly), dizziness, vertigo, and tinnitus (ringing in the ear(s)), and collapsing and falling, which sometimes include unconsciousness. Meniere’s has hospitalized me twice, the nausea and vomiting dropping my weight down to 110 lbs., which, for me, was the brink of starvation. I had to re-learn to stand and walk after one two-week hospitalization. Meniere’s has enormously complicated the task of incontinence management. Trying to wash and change when I’m walking like a drunk because my world is spinning violently; I know that the images my eyes are sending to my brain are not to be believed, and I must hang onto anything solid enough to support me in order to move from place to place, is about as much challenge as I care to face. The silver lining here is that Meniere’s is episodic, and I do have remissions.

If I live long enough, someday I will give the staff of a nursing home more work than they care to undertake. I dare not finish without thanking my wife, who has stood by me, even when I’ve stained our bed linens and our house and car upholstery. She watched Dr. Chancellor work on me. She took care of me when I was too far gone to care for myself, and she hired caregivers when she was unable to assist me. She drove me to doctor’s appointments when morphine would have kept me home, and she believed me when a succession of doctors told me that I had nothing to worry about.


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