I had a Canuck Parent, Johnny, who has a wee son with Exstrophy comment on a previous post and I thought I’d follow up on some of the trials & tribulations he & his son may be going through at the moment and what could be some of the challenges & joys later on in life as an Exstrophy Patient.
I am originally from Canada, and I was born in the 1970s with my magnificently deficient urinary tract, back in the days when parents were not given mental/psychological support and both parents & children were expected to just get on with things. Thankfully things are better.
So to the parent who commented, there are some resources, which I know that most pediatric units still do not share readily with parents. If you are in Southern Ontario/Toronto there is the Hospital for Sick Children – that’s where I had my first 3 surgeries. My first Urologist was a Dr Robert Jeffs – a pioneer in pediatric urological repair, who went on to found & run the Pediatric Urology Unit at John Hopkins and was the mentor for Dr Gearhart (who all the American parents rave about). Sick Kids has a Social Worker Team who provide support to the parents and kids, helping them navigate some of the more obscure government grants available, getting us in touch with suppliers and finding any psychological support required. So if you are in the Southern Ontario region, try giving Sick Kids a ring.
So what are some of the challenges with Exstrophy? I cannot really comment on the male experience, except for what I have heard during Patient Conferences. Females tend to have it a little easier, as our reproductive organs are not enmeshed with our urinary tract like they are for males (urethra is not only for urine, but also ejaculation, it’s all so interconnected for males). I’ve made young men who suffer intense pain when they get erect, or when they do get erect their penises curve in a less than normal fashion. There are surgeries which can repair this or at least make it more bearable. And from my understanding (though someone feel free to correct me on this) the penis tends to be a bit shorter than the norm.
For females, because of the initial surgery – Closure – sometimes there’s an incision that goes from the perineum all the way up to the abdomen, bisecting the clitoris and the labia. Because the goal of the initial surgery is to get everything back into the proper position there isn’t really much thought given that the young female patient may want to experience coitus later on in life. So we invariably require some surgery later on in life to help loosen the vaginal opening. And because the way our pelvic bones are positioned, we tend to suffer from vaginal and uterine prolapse, even though we haven’t had children. I’ve had surgery, 10yrs ago at Sunnybrook Health Sciences Centre, to not only get a mitrofanoff but also to hoist my uterus back up into position – so far so good, everything is staying place.
So the tribulations with Exstrophy, and any other birth defect, centres around body image mostly. Unlike other birth defects – whether it’s a hole in the heart, club foot, etc – because Exstrophy is primarily the urinary tract it’s still part of that social taboo of bodily fluids — that age old “why is your child not potty trained”? “why do you keep peein’g yourself?” “that’s disgusting, ewww” – I’ve heard it all and it does wear you down, especially when you’re a child. It’s one of the last taboos, but thankfully there have been strides in that area – because society in generally has become more tolerant and accepting of differences.
Whilst growing up I hated my body. I hated all the scars. I hated that I had to wear diapers. I hated that I had to know where all the toilets were within a km of wherever I was. I knew the location of every single publicly available toilet near every subway station from home to school, in case I had to nip off quickly to get to a toilet. I hated that my antibiotics constantly gave me the runs, and that they affected my moods – some would make me sleepy, others antsy, some made me hallucinate, and others just made me ache. Even when I lose a lot of weight, because of all the surgeries my abdominal muscles are shot – my abdomen will always stick out, even if I get down to 50kg. I didn’t have a boyfriend until I was about 24, which is a long time for any young person before they experience their first relationship. If anything, I learnt how to value my friendships precisely because I was so lonely.
Because of all my surgeries I am not suppose to have kids. Makes dating difficult, as trying to find someone who is okay with their potential life partner not being able to have kids is extremely difficult.
But today, I have a husband, who is incredibly supportive and loves the fact that there will not be any Tiny Beans. He, himself, is healthy with no birth defects and no true health issues.
So to the father who had commented, yes, there are difficult times and there will be some joyful times. Your son will find love and it will be with someone who is incredibly special, supportive and kind. It happens, it just takes a while.