A world where all children with cystic fibrosis are given the opportunity to benefit from good care, enjoy their childhood and look forward to their future as adults.
Cystic Fibrosis (CF) is caused by a single faulty gene that controls the movement of salt in the body. It is the UK’s most common life-threatening inherited disease and affects more than 8,000 people. Over two million people in the UK carry the faulty gene, causing CF – around 1 in 25 of the population. If two carriers of the faulty gene have a child, the baby has a 1 in 4 chance of having Cystic Fibrosis.
Cystic Fibrosis 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. Hence physiotherapy for the lungs and breathing passages and dietary advice have a major role to play in the healthcare of a child with CF. Symptoms of CF can include a troublesome cough, repeated chest infections, prolonged diarrhoea and poor weight gain.
Average life expectancy in the UK is around 31 years in 2008 although improvements in treatments mean a baby born today could expect to live for longer.
Testing to identify CF gene carriers and for early diagnosis of CF in a baby is widely practised as follows.
A simple mouthwash test can be taken to tell if you are a carrier. This is important if a relative has CF or is a known carrier. It is very important to have the test if your partner is a known carrier.
This test is used early in pregnancy to tell whether a baby has Cystic Fibrosis. It is usually offered to mothers who are recognised as being at a high risk of having a child with Cystic Fibrosis.
Other tests for Cystic Fibrosis are the sweat test (people with CF have more salt in their sweat, which can be detected) or a genetic test, which is a swab taken by gently rubbing the inside of the cheek to check for the faulty CF gene.
In many countries – in Africa, India, ex-Soviet bloc (such as Rumania, Lithuania and Ukraine) – the rate of child mortality from Cystic Fibrosis is unnecessarily high. Early, accurate diagnosis and access to the most modern treatments – drugs, enzymes, diet, and physiotherapy – are critical to good prognosis but are often lacking.
In contrast, CF services in Britain are well developed and many clinicians and other key staff working in the field of CF are willing to volunteer their services to help address problems overseas.
Against this background CHI sees a future in which Cystic Fibrosis is no longer debilitating and continuously life-threatening. We offer hope that all children inheriting the Cystic Fibrosis gene should have a reasonable opportunity to enjoy normal, fulfilling lives into mature adulthood.
Until then, all children with Cystic Fibrosis, everywhere, should be correctly diagnosed early and they, their parents, and their doctors will be armed with the appropriate support and self-determination for the disease to be treated and for the child to be full of hope and free from suffering.
CHI believes that it should not get involved if money is all that is required and that its role is to contribute in more practical long term ways. Nor does it believe it should prescribe solutions. Working in partnership is essential. CHI will not ignore any plea for help but may have to direct such requests elsewhere.