Cystic Fibrosis, Photos

GI problems, Cystic Fibrosis and Gratitude Friday

For today’s gratitude Friday post I want to show gratitude for CF suffers, family members of people with CF and CF charities that put their stories out there on the internet so that other people who are affected by Cystic Fibrosis can gain hope, encouragement and support.

So many times as a parent to a child with CF I have felt powerless, I have felt hopeless and I have felt lost and alone. And so many times I have come across other peoples stories, information and encouragement online that has just helped me through it or made me realise that we are not alone in this!

One such post I discovered a few moments ago! As you may know Joe (2 1/2 yrs old with CF) seems to be suffering with problems around food, reflux and digestion, and has done since birth- loads of GI issues really. These issues never really seem to be discussed that much. I felt lost and confused and felt really helpless, like nobody else was having these problems, like I was making it up!

And now the CF Trust and Dr Jon King have gone and put up the following blog post and raised the issue of GI problems in people with CF. It’s just come at the right time for me and my family. As I read it I felt like crying- “yes! That’s what my Joe feels like! That’s whats happening to him!” Even though it’s not solved anything it’s made me realise that we are not alone in this and that it is a big issue for a lot of people out there with CF. At last I know I’m not going Crazy!

Please take the time to read the blog. You can read it below or it’s on the CF Trust website blog which you can find here.

And here is a key to explain what some of the terminology means in the blog:

CF- Cystic Fibrosis

GI- gastrointestinal

pantoprazole, ranitidine and domperidone- anti reflux medication

pH manometry- test to see how acidic the fluid in your oesophagus is.

Creon- artificial pancreatic enzyme replacement- helps the body to break down and digest fat.

osteopenia- a condition where bone density is lower than normal

Osteoporosis- a medical condition in which the bones become brittle and fragile from loss of tissue, typically as a result of hormonal changes, or deficiency of calcium or vitamin D.

Distal intestinal obstructive syndrome- blockage of the intestines by bulky stool

steatorrhoea- the excretion of abnormal quantities of fat with the faeces owing to reduced absorption of fat by the intestine.

NAC- mucus thinning medication

movicol- laxative

gatsrograffin- medication used so that X-rays can be taken of the GI Tract

klean prep- bowel cleansing agent

picolax- laxative



More Than Lungs – Digestion & CF

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 steatorrheic 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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