Also known as Eagle-Barrett syndrome, prune belly syndrome is characterized by three main features:
1. Lack of abdominal muscles
2. Urinary tract anomalies (such as a very large bladder)
3. Bilateral cryptorchidism (two undescended testicles)
The incidence of prune belly syndrome is about 1 in 40,000 births although I am only the 5th documented case in New Zealand; 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 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 ruptured through my urachus (belly button), 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 have been hospitalized dozens of times for pneumonia as my mega sized bladder crushed my lungs before birth and with no muscles a little cough or runny nose can quickly become life threatening. 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. I do not speak and because I can't walk due to my muscles I lack the time looking at people's faces to have learned to lip read properly yet.
In the city where I live we have no paediatric renal specialists and my hospital have no plans to send me to Auckland's Starship hospital to see their renal team until I am in end stage renal failure (requiring dialysis and transplant). 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 I am hoping to beat the odds and not be one of the statistics! In less than 3 months I will be turning 2...something that only about half of kids born with Prune Belly Syndrome get to celebrate.
Aside from a birthday party mum has a few dreams for me:
1. to learn to walk eventually unaided but in the mean time with the help of a gait trainer/walking frame, this needs some added support as my main method of communication is still NZ sign language so I need my hands free.
2. to learn to talk, again we need speech and language therapy
He is 22 months old!
He likes playing with his Thomas the Tank Engine!
Next year he will start Preschool!
His favorite color is Red!
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