WHAT IS CYSTIC FIBROSIS
(CF) ?
How do you get it?
Well you can’t catch it, that’s for sure…..
Cystic Fibrosis is an inherited genetic disorder. This means that if two
people come together who happen to carry the CF gene, there is then a
1 in 4 chance that the baby they conceive will be born with CF. This also
means there is a 3 in 4 chance that baby will be born without it. 1 in
25 people in the UK carry the CF gene but the only way you will know if
you are a carrier is by getting tested.
The Lungs
People with Cystic Fibrosis (PWCF) are born with a distressing inherited
disease which affects many organs. The main problem tends to occur
in the lungs. All lungs secrete mucus but in CF lungs the mucus
is abnormally thick and this then becomes infected. Recurrent infections
lead to scarring of the lungs and scarring is permanent damage, which
means those bits of lung no longer work. The symptoms we suffer from as
the lung damage progresses, due to constant chest infections, are severe
coughing and breathlessness. We need regular physiotherapy to clear
the build up of mucus as well as regular antibiotic treatment to keep
infection under control.
Sadly all of us will eventually require
lengthy hospital stays, needing
constant injections of drugs 
Lung damage inevitably worsens and as a result people can suffer with constant bleeding, collapsed lungs and chest pain. When lung function deteriorates much further we often require extra oxygen to help us breathe and as the lungs become very weak in later stages a ventilator is used to assist the lungs to do their job.
| The Pancreas The second problem occurs in the pancreas. In people with CF the pancreas does not secrete the enzymes needed to digest food properly and so digestive enzymes are taken in tablet form, whenever they eat. It can be very difficult to keep weight on due to poor fat digestion and often supplements need to be added to the diet to keep up a reasonable calorie intake. Not only can it be physically difficult to put on weight for some people with CF but often they have the added complication of constant chest infections that leave them with no appetite and so they don’t feel like eating in the first place. Think about trying to eat a huge Sunday lunch when you have raging flu and then apply that to every meal time, every day! |
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Sometimes people also suffer with CF related conditions
such as as liver disease or a type of diabetes. Males with CF often suffer
with fertility problems. See the ‘Useful stuff’ page.
Genes
No two people with CF will have the exact same symptoms. This is
because each person inherits a specific gene type which dictates how CF
will affect that person. Whereas some people with CF will suffer
severe chest infections from childhood, others won’t suffer with
these problems until much later in their life. The gene also dictates
the kind of problems you will have with the pancreas and whether you will
suffer with complications such as liver disease.
| Some common misconceptions about
CF . . . Friends for Life are a group of people (young, and not so young) - not children! . . . Medical research has extended our life expectancy, meaning that people with Cystic Fibrosis are more likely to die later, in their twenties, thirties and sometimes forties, rather than in childhood. However this unfortunately means that often their condition becomes more critical not long after gaining their independence. |
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Gene Therapy . . . This does not offer
a cure for us!
Despite media hype, the promise of Gene Therapy will not repair damage
already established. Although a breakthrough for young children with CF,
the cure will not come in time for many older patients including every
one you see on this website!
CF is a well-hidden condition! . . .
For one thing people with CF look completely normal; in fact quite a few
of us are very nice looking! People have often commented that there
is a CF look; pale but beautiful, one positive of a malfunctioning gene
(if you believe it)! We have also learned to be exceptionally good
at hiding our condition. You may not be aware that you are in regular
contact with someone who may one day be in need of a life-saving transplant,
or indeed waiting for one right now.
In summary, we're just like you !
People with CF are neighbours, friends, lovers, work colleagues, parents
and employers, the majority of whom are striving to live a life that most
take for granted. We are dealing with daily trials including intensive
physiotherapy, chronic pain, and essential medications which can amount
to hundreds of tablets a day as well as nebulisers and intravenous antibiotics.
Despite these barriers we are trying to live a normal life.