Fowler's Syndrome

It struck me today that I have written a few posts about living with a catheter/chronic illness but I had yet to write anything about Fowler's syndrome. So I decided that I would rectify this just now, especially seen as we are having a huge drive in raising awareness for the condition.

I'm no stranger to bladder and kidney problems or indeed health problems in general, but Fowler's Syndrome, in particular, has had a huge impact on my day to day life. There are all the usual challenges of living with a chronic illness/catheter but because it is quite rare and only first diagnosed in 1985, very few doctors have heard of it. This makes getting the quality care Fowler's girls need and deserve much harder.

So what is Fowler's? Well, it is a condition that causes problems with the bladder muscles, specifically the urethral sphincter. These muscles usually work to keep you continent and relax to allow you to pass urine. But in girls with Fowler's Syndrome, the urethral sphincter will not relax and we end up in urinary retention. We then need to find someway of emptying the bladder. This can be through intermittent self-catheterisation or an indwelling catheter (urethral or supra-pubic). Some girls have had a mitrofanoff stoma created that they can catheterise through and some girls have needed their bladder completely removed and now live with a urostomy bag (similar to a colostomy bag for the bowels) There is no cure for Fowler's Syndrome, although sacral nerve stimulation (a bladder pace-maker) can sometimes restore near normal function. (To find out more about the how the urinary system works or more about the different forms of catheterisation, click here to read my first introductory post)

So, I can't pee. Not a big deal, right? This is what the literature (what very little there is) would have you believe. But speak to any girl with Fowler's Syndrome and you'll find out just how debilitating the illness can be.

There are many other symptoms such as painful bladder spasms, which can leave us doubled over in pain, and overwhelming fatigue. Infections are possibly one of the biggest problems we face. Stale urine from retention can cause infections but the various catheter techniques also greatly increase the risk of infections. Infections can be incredibly painful and make us feel incredibly ill, especially if they spread to the kidneys. They are often hard to get rid of due to antibiotic resistance and can sometimes lead to very severe infections needing hospitalisation. Some infections can be life threatening if they cause sepsis. Many girls now struggle with antibiotic resistant infections and many of us have lost kidney function. I think in the last year I have maybe only had 15 weeks antibiotic free, I've had roughly 12 hospital admissions and I've had sepsis twice.

Day to day life with Fowler's can be a huge struggle. As well as the symptoms above, the medication we take, whether it be antibiotics,strong painkillers or antispasmodics, can cause an array of side-effects. Strong antibiotics can lead to nausea and vomiting (if the infection hasn't already caused it) or a form of colitis, causing severe diarrhoea. Each form of catheterisation has its own set of difficulties from intermittent catheters getting stuck, to blockages, to supra-pubic site infections.

As mentioned above, another huge obstacle we Fowler's girls face is the fact that many doctors and nurses have not heard of the condition. It can lead to massive delays in receiving a proper diagnosis and huge problems receiving the treatment we need. A young woman in urinary retention is pretty rare, even when taking into account the other causes such as MS, spina biffida and such. When we Fowler's girls present to places such as A and E we can leave doctors a little baffled and sometimes they don't believe us. Its not uncommon for a Doctor to suggest a trial without catheter to a girl with Fowler's, even if she has had numerous tests and previous trials that show she cannot pee! When we complain that intermittent self-catheterisation is difficult and very painful it falls on deaf ears as it isn't painful and difficult in many other causes of chronic urinary retention. On a similar note, doctors did not believe me when I said I was in severe pain with my urethral catheter. They insisted that although it might be uncomfortable that it should not be causing severe pain. They refused for months to insert a suprapubic catheter or refer me for sacral nerve stimulation, leaving me bed bound and feeling completely hopeless.

This issue is amplified due to the inaccuracy of the literature available about the condition. When first confronted with Fowler's syndrome, doctors will sometimes turn to this literature to gain an understanding of the condition and read that, yes, we cannot pee and yes, we get a lot of infections. But I feel the resources that currently exist do not accurately describe just how debilitating the condition is. For that reason, doctors reading this watered down material will often not believe we are in as much pain as we say we are. This too makes getting the right treatment harder.

This is why we are desperately trying to raise awareness. Life with Fowler's is hard enough without having to fight for the quality care we need and deserve. Recently some of the Fowler's girls, myself included have started a campaign to raise awareness. We made videos and have collectively reached over 20 thousand people on social media. My friend Tia's blog has reached over 6 thousand people. And thanks to STV2 and Jennifer Reoch, the presenter who very kindly pitched our campaign to her producer, we are about to make a massive step forward. Tia and I will be interviewed on the 10th of July on the Live at Five show!

What can you do to help?

We are extremely grateful to anyone who takes the time to read or watch. We are even more grateful to anyone who shares our posts.

Please help us out by sharing this blog post and by telling everyone you know to tune in to STV2 on Monday the 10th July at 5. (The show will also be available on STV player).