There is no cure for cystic fibrosis so people with CF must do treatments each day that aim to keep them well. Chest physiotherapy and taking pancreatic enzymes and medications are important aspects of their treatment.
All people with CF will need to do some type of airway clearance technique (ACT), often referred to as chest physiotherapy, to help loosen the thick, sticky mucus that builds up in their lungs and to help minimise the number of chest infections they develop.
Both children and adults with CF should be receiving care from a multi-disciplinary team, including a physiotherapist, who provides ACT training and on-going support. Younger children need help from adults to manage their daily physio, but as they get older they learn how to manage on their own.
Every day, usually twice a day, a person with CF needs to do chest physio to help keep their lungs clear of mucus. This can take between 20–40 minutes each time but it can be longer if they're unwell. Chest physio is usually done in the morning, for example before school or work, and before bed.
For younger children, physio is usually done by parents or caregivers and is a session of percussion (hard tapping) or cupping on their chest. By aged 6 or 7, many children learn to do some of their physio themselves with a positive expiratory pressure (PEP) device. Adults still need to watch their child to make sure they’re doing it correctly.
A salty solution, called hypertonic saline, is often inhaled via a nebuliser before or during chest physio to help thin the mucus which makes it easier to cough up.
Regular exercise is also a great way to help lungs healthy, so children and adults with CF are encouraged to find ways to keep active.
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Most people with CF need to take pancreatic enzyme capsules with most of their food to help absorb nutrients, especially fats, to gain and maintain weight. The number of enzyme capsules people need to take depends on their age, food they are eating and how much CF affects their pancreas.
If people with CF don’t take pancreatic enzymes when they eat it can cause stomach pains, wind or diarrhoea. Missing a couple of doses doesn’t cause any long-term consequences, but over time with more and more missed doses they will struggle to put on weight or start to lose weight.
Find out more about nutritionOther medications, such as antibiotics, bronchodilators and vitamin supplements are often used in the treatment of CF. Some medications are used as a preventative while others are used to treat specific problems, such as antibiotics for a chest infection.
If you are a parent of a child with CF or you have CF yourself and would like help or advice with any of your treatments, please get in touch with your CF field worker or speak with your CF nurse.
Contact your CF field worker