There are some strange quirks that come as a result of having the life-threatening illness cystic fibrosis (CF) that make it a rather unique and curious condition. It’s not just about the lungs…
I’ve noticed during my life-time that for the majority of CF sufferers with a serious form of the condition, our more distinctive features are our small build and youthful demeanour. Most adults with CF are not that tall, are slim in build, youngish looking and reasonably non-threatening – I doubt there are many nightclub bouncers with the condition!
Here’s the science…
In people with CF, thickened secretions block the normal flow of the digestive juices from the pancreas, so digestive enzymes can’t get into the intestines. Without these enzymes, the intestines cannot properly digest food and the sufferer does not get the nutrition they need to grow normally and gain weight. Most of us have to take enzyme pills to digest our food.
The Peter Pan Effect
Now I’m 40 years old, there’s just one thing that doesn’t fit with reaching this milestone – I don’t look that old nor do I have any middle-aged bulk. Indeed, very few people who meet me at work or socially would ever believe my age.
Just occasionally life can imitate art, with the art in this case being the short book by F. Scott Fitzgerald: ‘The Curious Case of Benjamin Button’ and the recent Brad Pitt film of the same name.
How a 27 page novel can be made into a two and a half hour film is for Hollywoodto answer to; but the main premise behind both the film and the book is of a person who ages in reverse starting off as a crumbly old pensioner and aging backwards and ending up as a baby.
I’m not going to state that people with CF are aging in reverse BUT CF does seem to keep me and other sufferers looking eternally young, almost as if we were never meant to look older than our young life expectancy. This condition seems to lock us in and keep us small in stature and youthful. It’s as if our heavy drugs regime acts as a form of formaldehyde – somehow preserving us in a child-like state.
There are people all over the world desperate to discover the secret of eternal youth – well it appears that having CF is one of those options but trust me it’s better to enjoy growing old gracefully and avoid something as cruel and debilitating as this condition!
I do have other evidence that I’m aging in reverse – I’ve recently re-discovered a taste for cider and my favourite 80’s clothes are fashionable again. What a shame that 80’s music doesn’t follow suit…
Having said all this, I do have some grey and silver hair which I embrace. For most of my life, I never thought I’d live long enough to actually have hair that greyed – I must be getting on!
In case you were wondering whether I was looking to draw a complete parallel with the Benjamin Button film – I don’t claim to look like Brad Pitt – I’m more like the chef Heston Blumenthal when I have my glasses on!
Other oddities of a life with CF
CF also affects the sweat glands with a lot of salt being lost through sweat, which disrupts the delicate balance of minerals in my body. In essence, I sweat a lot, it’s very salty and unfortunately it’s mainly through my face. So you’ll often spot me on busy and hot tubes, bars, meeting rooms, sport pitches wiping my brow furiously as the human version of the Niagara Falls erupts! Not terribly pleasant and at times very frustrating and embarrassing; although if the sea runs out of salt, environmentalists know who to turn to…
One of the prevailing physical signs of CF is ‘digital clubbing’ (not something from the Ministry of Sound), where the tips of fingers or toes have an unusually large amount of excess tissue. This results in the nail bed taking on a curved appearance. Some CF patients acknowledge this trait saying their fingertips look like the character “E.T.” or even that their fingers have a certain “gecko-like” quality.
You’ll have to trust me when I say that we are not actually lizards with a human skin as in the TV series “V”.
A knock-on effect of a life-time of medication is that nearly half of my drugs regime is needed to counter the side effects of my antibiotics and 20 years of steroids has led to the development of my recent Type 1 diabetes and Osteoporosis.
We may be small in build, appear age-less, have amphibian qualities, sweat like demons and if you shook us we would rattle from our daily pill intake… but we are giants in character. Dealing with the constant grind of CF – the drugs regime, physiotherapy, nebulisers, IV therapies, coughing etc. is not for the faint-hearted and needs huge amounts of guts and determination.
Half the battle is being able to have a sense of humour about our little quirks!
I will keep you posted on my life affirming moments, trials and tribulations as and when they happen. Please keep reading and sharing my blog and sign up if you have not already done so.
Yours cup half full
A dear friend of mine, Darren Smith, is taking part in the forthcoming London Triathlon and Great South Run and is raising funds for CF. If you wish to donate to make him go faster, please click.
* Cystic Fibrosis is one of theUK’s most common life threatening inherited diseases, affecting over 8,500 people. The condition affects the internal organs, especially the lungs and digestive system, by clogging them with thick sticky mucus. This makes it hard to breathe and digest food. Each week, five babies are born with the condition, however, each week, three young lives are also lost to it. There is currently no cure for CF. However, existing gene therapy trials in the UK are bringing people with the illness closer to a form of cure but CF is not that well known and would benefit from more public donations.
For more information and to find out more view the CF Trust Website