Cystic Fibrosis

What is cystic fibrosis?

Cystic fibrosis is an inherited disease caused by a faulty gene. This gene controls the movement of salt and water in and out of your cells, so the lungs and digestive system become clogged with mucus, making it hard to breathe and digest food.

More than two million people in the UK carry the faulty gene – around 1 in 25 of the population.

There is currently no cure for CF but many treatments are available to manage it, including physiotherapy, exercise, medication and nutrition. Each week five babies are born with CF, and two people die.

More than half of the CF population in the UK will live past 41, and improved care and treatments mean that a baby born today is expected to live even longer.

Physiotherapy

For people with cystic fibrosis, physiotherapy helps clear the thick, sticky mucus from the lungs.

Parents are taught how to do physiotherapy with their child by the physiotherapist in the CF clinic. Adults with CF can learn to carry out their own physiotherapy.

Why is chest physiotherapy important?

Chest physiotherapy helps prevent thick, sticky mucus in the lungs from blocking the airways, which can reduce infection and prevent lung damage.

What physiotherapy techniques are there?

There are lots of different airway clearance techniques, and a specialist CF physiotherapist will conduct an individual assessment and give advice on the most appropriate technique to use, as well as the length and frequency of treatment sessions.

The technique used may change as you get older, or as the effects of your disease changes. The amount of mucus you clear will also vary, sometimes even from day to day.

Some techniques require no equipment, and focus on specific breathing exercises:

Active Cycle of Breathing Techniques (ACBT)
Autogenic Drainage
Other techniques employ a device to aid the clearance of mucus, using positive pressure to hold open the airways, and some also create vibrations within the airways:
Positive Expiratory Pressure (PEP)
Oscillating Positive Expiratory Pressure – e.g. Flutter®, Acapella®

Some techniques involve large pieces of equipment which can be expensive, and may therefore only be available in hospital:

High frequency chest wall oscillation (HFCWO) – an electric air compressor connects to an inflatable jacket (vest) to vibrate your chest

How much physiotherapy do you need?
The length of treatment sessions varies according to your needs. Daily physiotherapy is usually required, and if you have a chest infection you may need to increase the amount of airway clearance you do.

If there are few or no lung secretions, treatment sessions may only need to last 10-15 minutes, but if there are many then it could take as long as 45-60 minutes.

The number of treatment sessions will also vary. Most people do two a day when all is well, rising to four a day when necessary. If no secretions are present, some people with CF will only need physiotherapy once a day.

Your physiotherapist can advise you how much physiotherapy is needed.

When should physiotherapy start?

Physiotherapy should start from the time of diagnosis.

Who should do physiotherapy?

At first, the adults who care for the child with CF should deliver the physiotherapy. In time relatives or friends should also learn, so that no one person becomes indispensable.

Breathing exercises can be introduced from the age of two or three in the form of a game. From around the age of nine, most children can start doing part of their physiotherapy for themselves.

Most teenagers and adults become completely independent, and only need help if they have increased mucus secretions.

Useful Contacts:

Cystic Fibrosis Trust

Clinics are held in Morriston Hospital Paediatric Outpatients

Contact: Mari Powell (Senior Paediatric Physiotherapist) 01792 530742 or mobile no: 07772098575