Magazine Article :Dying Young: living with cystic fibrosis

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                             Health Media Group Features

                                 April 11, 2003

HEADLINE: Dying young: living with cystic fibrosis

BYLINE: Vivienne

HIGHLIGHT:

Cystic fibrosis is the UK's most common life-threatening inherited

disease and

kills three young people every week. Nicky West, 28, explains how she

lives with

the condition.

    " The harsh reality is that, short of a medical miracle, I will almost

certainly die within the next five years, " says Nicky. " I will never grow

old.

It's unlikely that I will ever make it to my mid-30s. "

    The average life expectancy for people with cystic fibrosis is 31.

Most

sufferers die as a result of lung damage. Nicky was born with the

condition but

not diagnosed until she was six after it was noticed she couldn't digest

her

food. Now 28 and living in London, she has to structure her life around

the

treatment demands of the life-limiting condition.

    Faulty genes

    Cystic fibrosis (CF) affects more than 7,500 babies, children and

young

adults in the UK, and five babies are born with the condition every week.

    A person has cystic fibrosis if they inherit the faulty CF gene from

both

parents. About 1 in 25 people, or 2.3 million in the UK, are carriers of

the

gene, but have no symptoms of the disease. Children whose parents are

both

carriers have a one-in-four chance of inheriting the condition.

    CF is a " multi-system " disease. This means it affects many of the

body's

organs, although most symptoms are concentrated in the lungs and the gut.

    In a healthy person, there is a constant flow of mucus over the

surfaces of

the airways in the lungs, which keeps them free of debris and bacteria.

In a

person with CF, this mucus is excessively sticky and instead of

performing its

role properly, it provides an ideal environment for bacterial growth -

making

people with CF more at risk from chest infections and pneumonia.

    Just over half of people with CF are also unable to digest fat, which

means

they have difficulty putting on weight, and suffer from abdominal pain

and

constipation. Other symptoms include an enlarged liver and spleen,

fertility

problems and small growths or polyps in the nose.

    A tough regime

    Living with CF requires a complicated treatment regime. Nicky cannot

get out

of bed in the morning until she has used her nebuliser for 10 minutes.

This is a

small portable machine that she uses to inhale a drug to open up her

airways.

After this, Nicky performs about an hour-and-a-half of physiotherapy

exercises

in order to bring up the excess mucus.

    " Then I have to make sure I eat, " she says. " Because we can't digest

fat, we

have to make sure we eat regular meals and a lot of food, so I have to

fit that

in.

    Nicky has to use her nebuliser again after she's had a shower and

will be on

the machine six or seven times a day. There will be another hour of

physiotherapy in the late afternoon and, in addition to this daily

regime, she

has to undergo a course of intravenous antibiotics in hospital every six

weeks.

    For Nicky, living with CF has meant making some dramatic lifestyle

changes.

Although her school and university years were fairly trouble free - Nicky

excelled at sports - working in the City for four years began to take its

toll

on her health.

    " I was working quite long hours, had to socialise quite a bit with my

job so

everything got a lot worse, " she says. " I actually gave up my job because

I was

advised that if I carried on the way I was, I probably wouldn't be here

for much

longer. "

    Nicky stopped working four years ago and now focuses on looking after

herself and enjoying life. She spends much of her time singing jazz and

blues,

which as well as being a pastime she loves, also provides good therapy

for her

lungs. She also works for the Cystic Fibrosis Trust, helping out with its

public

relations pork.

    Finding a cure

    The trust raises money to fund CF research, both to find a cure for

the

condition and to develop ways to improve symptom control. The trust's

chief

executive Rosie says the main focus is on gene therapy - to

replace the

faulty CF gene with a properly functioning one.

    This area of research is looking extremely promising, says Rosie, and

significant developments are likely to emerge in the next four or five

years.

    " We also give a great deal of support and indeed funding to the

clinical

care of those with cystic fibrosis via the NHS to ensure that the NHS can

look

after them better than it would be able to do with just its own

resources, " she

says.

    On top of this, the trust offers direct support to families in the

form of

advice, information, welfare grants and help in accessing state benefits.

    Accentuate the positive

    Although facing a life that is tragically likely to be cut short,

Nicky is

philosophical about her condition.

    " I wouldn't say I've been very depressed because of the CF; I would

say

there have been times when I've thought, Oh my goodness, what's this all

about?'

but I've always tried to turn it into a positive, " she says.

    She also gets a lot of support from the friends she has made through

the CF

community.

    " I just find they give me huge strength because they know exactly

what I'm

going through, " she says. " They're just trying to live normal lives as

well but

they're very positive. "

    April 5-13 is Cystic Fibrosis Awareness Week.

    Further information:

Becki

YOUR FAVORITE LilGooberGirl

YOUNGLUNG EMAIL SUPPORT LIST

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Pediatric Interstitial Lung Disease Society

http://groups.yahoo.com/group/InterstitialLung_Kids/