Big Issue: Living with cystic fibrosis

by on 08/05/12 at 7:00 pm

CYSTIC fibrosis is an inherited condition that affects 1 in every 2,000 babies born in the UK.

It is diagnosed at birth by the screening test done on a “heel prick” of all newborn babies.

CF causes a defect in cells in the lungs, sweat glands and gut, so CF patients have very salty sweat, are less able to digest fat and suffer from chronic lung infections.

There are about 9,000 people with CF in the UK, all of whom are cared for by specialist clinics.

When first recognised in the 1940s, most CF patients died in early childhood. Now all survive to adult life, most to middle age, and a child born today with CF is likely to draw their pension.

Most CF patients have to take enzyme supplements with every meal or snack to allow their bodies to digest fats. this means carrying a pot of tablets with you at all times just in case you fancy a snack.

Without these pills, patients would suffer malnutrition and many struggle to maintain a healthy body weight. Patients also have to take fat-soluble vitamins A, D and E as supplements. some would need other pills to reduce stomach acid, so just eating requires planning.

In hot weather, CF patients can lose excessive salt through sweating and that must be replaced by tablets, something else to pack for holidays or gap year travel.

The main health problem for CF patients is chest infections. they have excessive phlegm and a cough of which they are very conscious.

Clearing secretions regularly by physiotherapy techniques is the most important part of their treatment, but takes time.

If you are in a rush in the morning, an extra 10 to 15 minutes physio time can be a big problem.

Many patients also take regular antibiotics by nebuliser. For some, this twice daily schedule may take 30 to 40 minutes each time.

Exercise is good for us all, but in CF it is a good way to clear your chest and perhaps a bit more fun than routine physio. All patients are encouraged to do regular exercise as part of their regime and many are highly competitive sports people.

All have an annual assessment of fitness.

Clare Williams, the specialist physio in the Gloucestershire Adult CF Service often goes out with patients to gyms and sports clubs to help them arrange a programme to suit them.

Infections in the lungs cause damage to the airways that makes it difficult to clear secretions and allows further infections. A “vicious circle”.

Many CF patients’ lungs become permanently colonised by a bacteria. they may take regular antibiotics to minimise this but often need intravenous antibiotics, two or three times a year. Courses last for two weeks at a time so four to six weeks of your life or more each year are spent having drugs.

Every effort is made to do this at home rather than in hospital and many patients do go to school or work at the same time. Patients or their families are taught how to administer drugs themselves and are visited at home by specialist nurses. For the Gloucestershire Adult CF Service, specialist nurses Zoe Primett and Vicky Aqualina travel the length and breadth of the county.

Many CF patients have had a new lease of life following a lung transplant. Scientists have worked for 30 years on the genetic basis of CF and trials of gene replacement therapy have now started.

The Cystic Fibrosis Trust was set up by families and doctors in the 1950s. It raises funds for medical research into all aspects of CF. its funding of the Gene Therapy Consortium in the UK has been invaluable.

Big Issue: Living with cystic fibrosis

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