On 8–9
February, the Trust will be hosting a research ‘sandpit’ exploring issues
related to digestion in cystic fibrosis, a meeting bringing together experts
from within and beyond the world of cystic fibrosis. Here Dr Jon King shares his
experience of the often overlooked impact of cystic fibrosis on digestion.
A few months ago, the Cystic Fibrosis Trust
approached me to be a part of a research meeting (sandpit) to discuss possible
research avenues for gastrointestinal (GI) problems in cystic fibrosis (CF).
The Trust is exceptionally good at viewing CF
and its care holistically, and I realised what an immense impact GI problems
have on my daily life – dare I say it, rather more so than my lungs! During a
committee conference call, we joked that perhaps CF is actually a condition of
the GI tract with some lung involvement!
Of course when we think of CF, we think about
our lungs. When we fear the future, we worry about downward trends in
spirometry and worry about the need for transplants. We think about those times
when we have been caught out in public; short of breath, coughing and
spluttering, feeling quite awkwardly alien to everyone else around us. This is
CF… But is it?
Until 18 months ago, almost every morning for
over 10 years, I threw up acid and bile due to morning reflux. My face would
contort with the griping burning pain of my reflux between meals or whilst
eating certain foods. Triple therapy (pantoprazole, ranitidine and domperidone)
made no difference and when I had a pH manometry the surgeon did not believe
that I was complying with the drugs because the results were so extreme. I have
since had two operations on my stomach, the first failed, the second with the
addition of a metal mesh has worked well.
Reflux is annoying and can be really painful,
even leading to surgery; but to paraphrase Monty Python, apart from reflux,
what does the GI system do to us?
Well, what about Creon? Look at yourself. You’re
out with your friends, having a meal and you pull out a container and take more
tablets in one go than anyone round that table probably takes in a year. And
you have to do that almost every time you eat or drink. That is quite an impact
on our daily lives. Now that we are being deprived of 40,000s, I am on at least
70 25,000 capsules a day - it does allow me to perform my party trick of 24
tablets in one go though!
How about insulin? CF-related diabetes requires
careful blood-sugar monitoring and insulin regimes, and yet we rarely really
think about it; we deal with it as an adjunct to everything else, like taking a
vitamin tablet.
Which brings me conveniently on to the small
bowel GI effect of CF: fat-soluble vitamin malabsorption. What about the long-term
effect of the half a dozen or so more tablets (vitamins A+D, vitamin E and a
calcium tablet) for that? We have regular dexa scans to monitor bone density
because with the additive effect of regular steroid use, we have a high risk of
developing osteopaenia and eventually osteoporosis.
Alas, we are only at the upper GI system – we still
have the large bowel to tackle yet. Distal intestinal obstructive syndrome
(DIOS): excruciating pain requiring hospital admission for pain relief and
rehydration, sometimes an operation.
And what about steatorrhoea? I don’t know about
the readers but I spend an awful long part of my day in the toilet. How many
times have you been stood over a toilet wiping for three, four or five times
longer than you were ever sat on it?! And how many times have you had to flush
the loo so many times that someone has looked at you strangely as you return to
the living room? Do you even dare repeat this ritual in a public loo? I commend
you if you do – you are braver than me! I live in a small flat with my
girlfriend. I spent all my savings refurbishing but ran out of money before I
could afford the en suite door – to say that this can be a mood killer is an
understatement!
And our bowels can go completely the opposite
way with severe constipation, thanks to a perfect combination of sticky, fatty
steatorrhoeic stools and the thick mucus lining our guts. I once went 15 days
without going to the toilet at all. There can be advantages – I use all this
time on the toilet catching up with social media and replying to emails.
The constipation also affects our appetite intake,
which is essential for our calorie counts, and can even lead to regular
throwing up as the food has nowhere to go. Some readers may know that I cycled
to Barcelona last summer to raise money for the Cystic Fibrosis Trust. For five
days in a row I was so constipated that I was throwing up all my meals every
day - not a great way to tackle hundreds of kilometres of cycling.
So how do we deal with this severe constipation?
Yet more medication. Maybe four or more sachets of movicol a day and an equal
amount of NAC. Perhaps the occasional day on the toilet after a gatsrograffin
or picolax or Klean prep clear out. These are medications strong enough to
clear the bowel out for surgical or camera test procedures and yet we use them
merely to return to regular bowel movements!
But still, apart from painful reflux, stomach
operations, dozens of tablets a day, insulin regimes, malabsorption of vital
vitamins, high risk of osteoporosis, occasional DIOS, irregular bowel habits,
embarrassing steatorrhoea, sachets of laxatives a day and occasionally needing
to set a day or two aside for a full bowel clear out, what does the GI system
do to us?!
What oddities have you found with digestion and
cystic fibrosis? We can help those coming to the research sandpit really get to
grips with the subject – share your experiences with me, @JonKingBYD, on Twitter
with the hashtag #DigestCF.
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