Prune Belly Syndrome: Elijah’s Grand Garage Fundraiser

The incidence of prune belly syndrome is about 1 in 40,000 births; 95% of cases occur in males. In the past, it was suggested that the abdominal wall was deformed by pressure from a distended bladder due to bladder outlet obstruction in utero. Currently, it is thought that prune belly syndrome is a multisystem disease complex which derives from a primary defect in mesodermal development at about 8 weeks gestation.
The major prognostic factor is the degree of dilation of the urinary tract; 20% of patients are stillborn, 30% die of renal failure or urosepsis within the first two years of life, and the remaining 50% have varying degrees of urinary pathology.

I was born in renal failure with grade 5 bilateral reflux. I have a lower urinary tract obstruction which prevents me from passing a normal stream of urine and at 4 days old my bladder began draining through my urachus, the urachus is normally obliterated during embryonic development, transforming into a solid cord. Mine did not and for 6 months this was my only method of emptying my bladder. Then one day it closed over. After that I have suffered periods of up to 5 days unable to pass urine and this strain has now meant one of my kidney’s has completely failed.

I constantly suffer UTI’s (urinary tract infections) and probably as a result of some of the medicines I get to treat them have lost my hearing.

To date I have not been offered any surgery to repair any of my urinary problems and now with the help of my Mum and the wonderful support networks overseas I am hoping to beat the odds and not be one of the statistics!