What is CF technically (apart from being a gigantic pain in the arse)?

In 2018, the Cystic Fibrosis Trust described CF as 'a faulty gene that disrupts the movement of salt & water in the body's cells making the mucus in people with CF thicker and stickier.'

SO the issue occurs pretty much wherever you might find a degree of mucus in the body. Those of us with CF suffer predominantly with our lungs & digestive system but it also affects organs such as: the pancreas, 'reproductive bits', kidneys, liver & causes further complications with bones, joints, hearing, heart, skin, sinuses (depending on who you ask).

Respiratory

For myself personally, my lungs are the main problem. Typically, the lungs gradually fill up with this sticky mucus I mentioned previously (some people have described it as drowning on the inside, which is a statement I can unfortunately relate to). This build up of mucus reduces the lung capacity which eventually makes breathing a literal battle, consequently making any kind of activity difficult (less than ideal for anyone). All of this mucus provides the perfect breeding ground for bacteria to grow & thrive (I'm usually carrying at least two infections at any one point), these infections cause nasty exacerbations (basically a flare up) of fevers, sickness, weight loss & extreme fatigue. The lungs eventually become very damaged & scarred through these exacerbations, reducing their ability to recover & remain optimal. The most favourable treatment option from our multi-disciplinary team is a hospital admissions, two weeks of IV antibiotics & lots of physiotherapy in an effort to move the mucus up & out. The IV's are usually administered in two ways, either through a PICC line (an access point from the arm into the artery) or a portacath like mine (a permanent access point underneath the skin). I had my first hospital admission when I was 12 (I'm nearly 20 now), & since then I have been admitted 17 times, the shortest was one night (for Hemoptysis; coughing up blood - no lie it looked like I'd been fighting in a zombie apocalypse) and my longest was four weeks (fighting a nasty infection).

PICC Line

Portacath

CF often ultimately leads to a double lung transplant and/or premature death - sorry to those of you expecting a happy ending HOWEVER, saying this there is hope as new drugs are becoming available that work literal wonders for our bodies (fingers crossed the NHS get hold of them soon🤞🏼😁)!!

Digestive System

The digestive system issues begin with the pancreas; the pancreas provides the enzymes that are used to digest food in the intestines. However, unsurprisingly the pancreas is yet another organ that is often affected by the faulty gene (shocker right?), due to this, some of us have very limited enzymes at hand so instead we have tablets who very kindly do the digestion job for us. These must be taken with any food & drink that contains fat, protein or carbs (so basically everything but fruit & veg), I currently have 15-20 of these a day with my meals. Complications that can occur are difficulty to maintain/gain a healthy weight which in some cases require a feeding tube to ensure extra nutrients are absorbed (I used to struggle massively with my weight as a child & the doctors did discuss the possibility of a tube for me, but fortunately it never got to that point). Another issue is the risk of severe constipation, not constipation as in haha I can't push my poo out today but a blockage in the digestive system which is not only extremely painful leaving you looking like you're nine months pregnant but if serious enough can lead to death, the very last resort to fix this is invasive surgery.

Feeding Tube

Pancreas

As I mentioned previously, the pancreas has its own issues. Not only creating pretty useless enzymes but also in some cases having issues with production of insulin, resulting in CF related Diabetes, this 'hybrid' shares characteristics of both type 1 and type 2 Diabetes requiring additional medications and management. It is estimated that just over a third of adults with CF have CFrD, usually developing in adolescence years.

I do not have the official diagnosis but was being investigated (for obvious reasons this is currently on hold) for potentially being in the early stages of developing it as I do struggle with low blood sugars occasionally which affect my ability to function well. My particular CF centre has funding to use the 'Libre' devices for screening purposes. This device allows the user to scan their levels through an app, I personally found it helpful as it could be used as often as deemed necessary, and also saves having to faff with needles etc.

Libre Device

Reproduction

Reproduction is an issue of CF which isn't often talked about; this isn't to say that those with CF don't have healthy sex lives (sorry mum & dad) but can be overcome with assistance. Needless to say it affects men & women differently as follows:

Men - the 'sperm canals' are often completely blocked or none existent meaning the semen never received the sperm in the first place making conception impossible. 97-98% of men are infertile.

Women - the uterus, fallopian tubes etc have a far thicker lining than the average person, this can make conception a challenge as the eggs & sperm are unable to travel as easily as they ordinarily would.

Kidneys

Kidneys are not necessarily affected directly through CF but they often get a bit of a hammering from all the medications we are on, requiring frequent blood tests to ensure there is nothing too dramatic going on. In February 2017, it had been roughly a year since my last course of IV's but unfortunately I suddenly became very ill which required quite strong antibiotics, this knocked my kidneys for six to such a degree that I suffered with near acute kidney failure, I was on litres & litres of liquids for days to try & rehydrate my body (which did work in the end).

Hearing

The same can be said for hearing, some antibiotics known as Aminoglycosides have a common side effect that reduces hearing, if the treatment is continued without intervention it can cause complete irreversible deafness, although this is extremely rare. I have been wearing hearing aids for roughly two years (I think).

So in conclusion, the above is a brief overview of the common affects CF can have on the human body but as I said before, this condition is so diverse that you'll receive a range of answers depending on who you talk to. Thank you again for reading my second piece of writing (so exciting!!), I do hope you've found it interesting & have learnt something. Until the next extract my friends 🥰