Treatment - Cystic fibrosis

There's no cure for cystic fibrosis.

But there are treatments that can help to:

• control symptoms
• prevent or reduce complications
• make the condition easier to live with

You will need regular appointments to check the condition. A care plan will be set up based on your needs.

A team of healthcare professionals will treat you. This may be in a hospital.

Medicines for lung problems

You may need to take different medicines to treat and prevent lung problems. These may be swallowed, inhaled or injected.

Medicines for lung problems include:

• antibiotics – to prevent and treat chest infections
• drugs to make the mucus in the lungs thinner and easier to cough up
• drugs to help reduce the levels of mucus in the body
• bronchodilators – to widen the airways and make breathing easier
• steroids – to treat small growths inside the nose (nasal polyps)

Medicines to make the mucus in the lungs thinner include:

• dornase alfa
• hypertonic saline
• mannitol dry powder

Medicine to help reduce the levels of mucus in the body include:

• ivacaftor (Kalydeco)
• ivacaftor (Kalydeco) taken with lumacaftor (Orkambi)

Orkambi is only available on compassionate grounds. People must fulfil several criteria set by the manufacturer in order to use it.

If you have cystic fibrosis you need to have your routine vaccinations up-to-date. You should get the flu vaccine every year from when you are old enough to get it.

Children with cystic fibrosis should get all their normal childhood vaccinations.

Exercise

Staying active can help clear mucus from the lungs. This includes any kind of physical activity, such as running, swimming or football. Exercise also improves physical strength and overall health.

A physiotherapist can help set an exercise and activity plan for you.

Airway clearance methods

A physiotherapist can also teach you ways to help keep your lungs and airways clear of mucus.

These include:

• active cycle of breathing techniques (ACBT) – a cycle of deep breathing, huffing, coughing and relaxed breathing
• autogenic drainage – this is a series of gentle controlled breathing techniques which clear mucus from the lungs
• handheld airway clearance devices – these use breathing techniques, vibration and air pressure to help remove mucus from the airways.

A positive expiratory pressure (PEP) is a type of airway clearance device.

Diet and nutritional advice

The build up of mucus can make it difficult to digest food and absorb nutrients. This is why healthy eating is important.

The pancreas often does not work properly because of cystic fibrosis. This makes it even harder to digest food. A dietitian will tell you how to take in extra calories and nutrients to avoid malnutrition.

They may recommend a high-calorie diet. You may have to take vitamin and mineral supplements. Taking digestive enzyme capsules with food will help with digestion.

Lung transplants

If you have severe cystic fibrosis you may need a lung transplant.

A lung transplant is a serious operation. It carries risks. But it can improve the length and quality of your life.