Polly's pages (aka 'Donna Williams')

Ever the arty Autie

Babysitting the bowel in Vascular Ehlers Danlos Syndrome IV


Hanging in The Balance by Donna Williams My father died from bowel cancer in his late 50s. I survived breast cancer in my late 40s and of course this means I’m a reasonable candidate sooner or later for possible bowel cancer to give me a run for my money. As such I had to have a colonoscopy every year to ensure I kept this bogie man away.

I did this once, and there were no complications, but after that I shied away from them for 3 years, winging it with the fecal smear screening instead and saying that was good enough for me. In 2012 I was diagnosed with EDS IV, also called Vascular Ehlers Danlos Syndrome or VEDS. It comes with aneurysms and organ rupture and so far four of my relatives have rupture (two uterine, one brain aneurysms, one spleen) but I haven’t.

After this diagnosis and learning I had a 12% chance of spontaneous bowel rupture, I decided colonoscopies were not for me. Instead I opted for CRT colonography. This had a minimal risk of puncturing the bowel and I could do it once every three years. I was told they found no cancer but that I had diverticular disease. Common in people over 60 and really no biggie for most them. Diverticular disease involves thinning out of parts of the bowel causing pockets rather like a failed baloon which has lost its integrity. But in people with EDSIV who have only 20% of the bowel integrity of those without EDSIV, it is these thinned out parts of the bowel which then risk spontaneous rupture and whose bowel ruptures will be in their 50s. I’m 52, rather ancient in EDS IV terms.

Those likely to ultimately have bowel rupture commonly have abdominal adhesions from spontaneous tears in the peritoneum. My ruptured cousin and I both had abdominal surgery where the surgeon was perplexed at the level of abdominal adhesions and both of us were asked if we were certain we hadn’t had previous abdominal surgeries.

Those likely to rupture have scars which stretch and sometimes re-open. One of my mastectomy scars did this. The tidy scar is now stretched at the join to over an inch wide of scar tissue so its easy for me to imagine that if my bowel ruptured any reconnection of thin, flimsy bowel tissue would ultimately also have a reasonable chance or tearing open again.

Diverticular disease is big red flag for those with EDSIV. But even with EDSIV and a history of abdominal adhesions and scars that stretch, it would take pressure in the bowel to cause any of those thinned out pockets to burst.

I have dealt with chronic constipation all my life and it comes with the autonomic challenges of EDS. And there are times, particularly this past year, that this causes tight pulling, colic like sensations in parts of my bowel and it literally feels like there’s a tight, pulled balloon that is at risk of going ‘pop’. And when I have these episodes I don’t know what to say or do. There’s no real point saying or doing anything.

If I tell a gastroenterologist they would either tell me they have no experience with EDSIV (which occurs in 1 in 25,000 people) or say they can’t tell me anything without doing invasive viewing of my bowel which risks causing puncturing in people with EDSIV.

If I did rupture there is poor chance surgeons could successfully reconnect my bowel and have that reconnection last and function. If I got to a hospital following a rupture (there is one less than a block from me but one is generally dead within 15 minutes of bowel rupture) they could remove the whole bowel following rupture and give me life with a colostomy bag. But I’m already living with respiratory failure and can’t sleep without a machine that now does my breathing for me, and one can only handle so much medicalisation before it just doesn’t feel ‘worth it’. So I wouldn’t even bother going to the hospital.

I know that death from bowel rupture would be quick, that the blood loss would quickly lead to sleep and that for me that means my failed respiratory drive will mean I quickly stop breathing with no struggle or distress as my heart then stops, my brain then dies and I’m gone.

And bowel cancer? What would be the point of any operation for bowel cancer? They would not be able to successfully reconnect my bowel and I wouldn’t want to live with babysitting both a failed respiratory drive and a colostomy bag for the rest of my life. So any diagnosis of bowel cancer and I’d cut to the chase, tidy up lose ends, drink some well stewed lemon balm tea (its a muscle relaxant that makes you sleepy), warm up some sake until I’m well and truly too drunk to care and fall asleep without my bilevel ventilation and in around 15 minutes I’d be gone (I presently only take over from the machine for a few breaths every 30min-2.5hrs.

And so what do I do with doctors, their sense of responsibility for their patients, the idea that 52 is too young to ‘quit’ or stop caring too much about staying alive? I think that only the person living with this stuff actually really understands the situation and that its ok to make one’s own rules for the situation. I’m going to continue to babysit my bowel, to help it with exercise and magnesium for peristalsis, with salt for the autonomics, with rhubarb and beetroot and xylitol and psyllium for the constipation, and let the cards fall where they may.

I know there are bogie men waiting in the wings… the inevitability of one day snoozing without mechanical ventilation, bowel rupture, breast cancer metastases, development of bowel cancer… but I can not see any point in concerning myself with worrying too much about any of these.

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