Treatment

Treatments for cystic fibrosis

There's no cure for cystic fibrosis, but a range of treatments can help control the symptoms, prevent or reduce complications, and make the condition easier to live with.

Regular appointments to monitor the condition are needed and a care plan will be set up based on the person's needs.

People with cystic fibrosis are treated by a team of healthcare professionals. Sometimes the condition will require treatment in hospital.

Medicines for lung problems

People with cystic fibrosis 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
• a combination of 3 medicines (Kaftrio) to treat the root cause of cystic fibrosis in people age 12 and over
• medicines to make the mucus in the lungs thinner and easier to cough up – for example, dornase alfa, hypertonic saline and mannitol dry powder
• medicine to help reduce the levels of mucus in the body – for example, ivacaftor taken on its own (Kalydeco) or in combination with lumacaftor (Orkambi, but this is only available on compassionate grounds if people fulfil several criteria set by the manufacturer)
• bronchodilators to widen the airways and make breathing easier
• steroid medicine to treat small growths inside the nose (nasal polyps)

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.

Exercise

Any kind of physical activity, like running, swimming or football, can help clear mucus from the lungs and improve physical strength and overall health.

A physiotherapist can advise on the right exercises and activities for each individual.

Airway clearance techniques

A physiotherapist can also teach techniques to help keep the lungs and airways clear.

These include:

• the active cycle of breathing techniques (ACBT) – a cycle of deep breathing, huffing, coughing and relaxed breathing to move mucus
• autogenic drainage – a series of gentle controlled breathing techniques that clear mucus from the lungs
• airway clearance devices – handheld devices that use breathing techniques, vibration and air pressure to help remove mucus from the airways (for example, a positive expiratory pressure, or PEP, device)

The Cystic Fibrosis Trust has more information on airway clearance techniques and physiotherapy.

Dietary and nutritional advice

Eating well is important for people with cystic fibrosis because the mucus can make it difficult to digest food and absorb nutrients.

The pancreas often doesn't work properly, making it even harder to digest food.

A dietitian will advise on how to take in extra calories and nutrients to avoid malnutrition.

They may recommend a high-calorie diet, vitamin and mineral supplements, and taking digestive enzyme capsules with food to help with digestion.

The Cystic Fibrosis Trust has more information on nutrition and eating well.

Lung transplants

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 Cystic Fibrosis Trust: information on lung transplants in cystic fibrosis.