Clinical update

NICE guidance on diagnosing and managing cystic fibrosis

NICE has published new guidance on cystic fibrosis, a progressive and incurable condition suffered by about 11,000 people in the UK that affects the lungs, pancreas, liver and intestine

NICE has published new guidance on cystic fibrosis, a progressive and incurable condition suffered by about 11,000 people in the UK that affects the lungs, pancreas, liver and intestine


A cystic fibrosis patient breathing in through a ventilator while a physiotherapist
assesses her. Picture: Science Photo Library

What’s new

The National Institute for Health and Care Excellence (NICE) published new guidance on the diagnosis and management of cystic fibrosis in October.

Essential facts

Cystic fibrosis is one of the most common genetic conditions, afflicting about 11,000 people in the UK. Affecting the lungs, pancreas, liver and intestine, it is a progressive and incurable condition, although symptoms can be managed with daily treatments that combine medicines, exercise and physiotherapy.

What’s new

In October, the National Institute for Health and Care Excellence (NICE) published new guidance on the diagnosis and management of cystic fibrosis. It specifies how the condition should be monitored and managed, including detailed advice on treating the most common infections associated with the condition. It also includes recommendations for research.

Signs and symptoms

In the UK, all newborn babies are tested for cystic fibrosis when they are seven to ten days old. Symptoms tend to start in early childhood, although they can sometimes develop very soon after birth, or may not be obvious until adulthood. They include recurring chest infections, difficulty putting on weight, frequent wet-sounding coughs, diarrhoea, and occasional wheezing and shortness of breath. There may also be a number of related conditions including diabetes, osteoporosis and liver problems.

Causes and risk factors

While about one in 25 people carries a faulty gene that may cause cystic fibrosis, both parents must have it for there to be a one in four chance their child will have the condition. Cystic fibrosis can have a significant impact on life expectancy, although advances in treatment mean that the outlook has improved considerably in recent years. Currently around half of those with the condition will live beyond 40, while babies born now with cystic fibrosis are expected to live even longer. Lung function is often reduced, with infections often a cause of significant morbidity.

How you can help your patient

Routine monitoring and annual assessments are crucial in providing effective care. Specialist nurses in particular play a key role in providing patients and their families with information about cystic fibrosis, from the point of diagnosis throughout their care. This should include choices about managing their condition, possible or existing complications or comorbidities, and the implications for living independently. They can also signpost to local support and advocacy services, including online support groups.

Expert comment

Nichola_MacDuffNichola MacDuff, advanced clinical nurse specialist, adult cystic fibrosis service, Royal Wolverhampton NHS Trust

‘The new NICE guidelines are important because they are completely independent and based on a robust review of evidence. It’s important to remember that cystic fibrosis is rare – for example, there is roughly just one patient for each GP surgery, sometimes none at all. Hence the need for specialist nurses, who have in-depth knowledge about the condition, to oversee both in-patient and community care.

‘For those nurses who don’t work in this field, the key messages are around infection prevention. Patients coming into emergency departments or acute medical receiving areas should be isolated and protected from contracting infections. Healthcare professionals should also notify the local specialist centre as soon as a patient is admitted, so we can oversee their care.’


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