There's currently no cure for cystic fibrosis, but it's possible to help control the symptoms, prevent or reduce complications, and make the condition easier to live with.
A person with cystic fibrosis will be supported by a team of healthcare professionals at a specialist cystic fibrosis centre. A care plan will be drawn up that's tailored to their individual needs.
A range of treatments may be used and sometimes treatment in hospital will be needed. Regular appointments to monitor the condition will also be recommended.
Some of the main treatments for cystic fibrosis include:
Medications to treat and prevent lung problems
Airway clearance techniques to remove mucus from the lungs
Dietary and nutritional advice
Treatments for associated problems, such as diabetes
Medications for lung problems
People with cystic fibrosis may need to take a number of different medicines to treat and prevent lung problems. These may be swallowed, inhaled or injected.
Some of the main medicines for cystic fibrosis are:
- antibiotics to prevent and treat chest infections
- medicines to make the sticky mucus in the lungs thinner, such as dornase alfa, hypertonic saline and mannitol dry powder
- a medicine called ivacaftor to help reduce the levels of mucus in the body – although this is only suitable for fewer than 1 in every 20 people (4%) with cystic fibrosis
- bronchodilators to widen the airways and help make breathing easier
- steroid medication to treat nasal polyps (small growths inside the nose)
It's also important that people with cystic fibrosis are up to date with all routine vaccinations and have the flu jab each year once they're old enough.
Airway clearance techniques
In addition to medication, special techniques can be used to help keep the lungs and airways clear. A physiotherapist can give you advice.
Some of the main techniques include:
- active cycle of breathing techniques (ACBT) – a technique that involves a sequence of relaxed breathing, followed by deep breathing exercises and then huffing
- autogenic drainage – a series of gentle breathing techniques that clear mucus from the lungs
- modified postural drainage – a technique that involves changing your position to make it easier to remove mucus from your lungs
- airway clearance devices – handheld devices that use vibration and air pressure to help remove mucus from your airways; you may have to pay in the region of £45-60 for one of these devices
Your care team can provide more information on the techniques mentioned above. The Cystic Fibrosis Trust also has information on airway clearance techniques.
Dietary and nutritional advice
For people with cystic fibrosis, getting the right nutrition is vital in helping them develop normally and stopping them becoming frequently ill.
However, the majority of people with cystic fibrosis cannot digest food and absorb nutrients from when they're a baby. Occasionally, the condition can cause the pancreas to become damaged over time, causing the same problem.
A dietitian will advise on what you can do to help avoid malnutrition. They may recommend:
- taking digestive enzyme capsules with all meals and snacks to help with digestion – the number of capsules needed depends on the food being eaten and varies from person to person
- following a special balanced diet that's high in calories (energy), fat and protein
- taking vitamin and mineral supplements
The Cystic Fibrosis Trust has information on eating well with cystic fibrosis. It also has various factsheets, including nutrition advice for adults and children.
In severe cases of cystic fibrosis, when the lungs stop working properly and all medical treatments have failed to help, a lung transplant may be recommended.
A lung transplant is a serious operation that carries risks, but it can greatly improve the length and quality of life for people with severe cystic fibrosis.
The outlook for people with cystic fibrosis is often better than for many transplant patients, as they're often younger and in better general health. It's estimated that up to 9 out of 10 people will survive for at least a year after a transplant.
The Cystic Fibrosis Trust has information on lung transplants in cystic fibrosis.
Treatments for associated problems
People with cystic fibrosis can have a number of other problems that may benefit from treatment.
- exercise can help keep bones and joints healthy, and is recommended for everyone with cystic fibrosis – any sport or exercise is usually good, but if you're not sure about a particular activity, you should ask your physiotherapist for advice
- medicines called bisphosphonates can help treat weak and brittle bones that may occur as a result of cystic fibrosis if the person doesn't get enough vitamin D and calcium (from their diet and/or from supplements)
- insulin medication and a special diet may help someone with diabetes caused by cystic fibrosis control their blood sugar levels
The Cystic Fibrosis Trust has information on cystic fibrosis-related diabetes.