How is cystic fibrosis treated?

On this page read about the different ways cystic fibrosis is treated.

Sadly, there’s no cure for cystic fibrosis (CF), but treatments can help control symptoms, prevent complications, and make the condition easier to live with. Treatments have come a long way in the last few years, and that research is ongoing to make sure everyone with CF can live a long and full life.

People with CF are cared for by a team of specialist health care professionals. There are a range of different treatments available, which will be based on each person’s individual needs and form an individual care plan.

Children with CF usually see their treatment team in an outpatient clinic once every two months. Sometimes these appointments will be virtual using video, or on a phone call. At each visit, the team will:

• check your child’s symptoms
• assess their growth
• check for signs of a lung infection
• consider your child’s medications.

They may see their CF team more often or stay in hospital for treatment just after their diagnosis or if they’re unwell.

Airway clearance

A physiotherapist can show you or your child airway clearance techniques (a type of chest physiotherapy) to help clear the airways. These techniques loosen the mucus in their lungs so it can be coughed up more easily. This can lower the risk of lung infections and improve lung function. Most people with CF need to do airway clearance twice a day but some need to do it more often.

The physiotherapist will help to decide on the best way to clear the airways. There are many different techniques and devices that can be used. Physical activity and exercise also help your child to clear mucus and stay healthy.

The best airway clearance techniques for you or your child will depend on their age and what works for them. It can help to read aloud to your child or to have the TV, an audiobook or some music on while doing their airway clearance. Make it close time with your child and part of their normal routine, so they don’t see it as a chore. You can even ‘gamify’ it with an app, different techniques, or using exercise! Cystic Fibrosis Trust has some useful ideas to get started, such as Project Fizzyo, PlayPhysio, or these videos to make exercise fun.

Mucolytics

Some people with cystic fibrosis will benefit from medicines called mucolytics. These make mucus thinner and easier to clear. Examples are nebulised rhDNase (Pulmozyme™) and hypertonic saline (salty water).

CFTR modulator drugs

Most people with CF can benefit from medicines called CFTR modulators. These can correct or can greatly improve problems with the CFTR protein that are caused by a fault in the CFTR gene that causes CF. CFTR modulators are dramatically improving the outlook for many people with CF.

But not everyone with CF can benefit from modulators. There are many reasons for this. Ask your CF team whether CFTR modulators are an option.

Treating chest infections

If you have CF, you may have frequent chest infections.

Chest infections from bacteria are treated using antibiotics. These can be given by mouth or into a vein. They may also be breathed in through a device called a nebuliser. A nebuliser makes the medicine into a mist that is breathed in through a face mask. Some people with CF take antibiotics long-term. Doctors sometimes recommend this to reduce the chance of new infections.

People with CF are at risk of developing chronic infection, meaning it does not go away even after antibiotic treatment.

Sometimes, people with CF can get pulmonary exacerbations during which their lung symptoms and breathing become much worse. If the symptoms get very bad, they may need to go to hospital for treatment or further tests.

Nutrition and diet

A CF team will include a dietitian to give you advice about food. People with CF should eat a normal healthy diet, although some may need to alter the amount they eat. Talk to your dietician for advice.

People with CF may not take in enough energy or nutrients from their food. Your child may be prescribed vitamin supplements and high-energy nutritional supplements to help. People with CF have regular blood tests to check vitamin levels and will occasionally be prescribed other supplements such as vitamin D or iron if these are low.

Pancreatic enzyme replacement therapy (PERT)

The pancreas is an organ that makes digestive juices (enzymes). These are released into the gut to digest food. CF can sometimes stop the pancreas from working properly, which stops these enzymes from reaching the gut. If this happens, enzyme supplements can help people with CF to digest food. It’s very important that you check how many tablets to take with which foods. Speak to your dietician for advice.

Lung transplant

Some people with CF may benefit from a lung transplant to replace their damaged lungs with healthy lungs. This is only done when all other forms of treatment have been tried and are no longer effective. You can read more about transplants on the Cystic Fibrosis Trust website.

You can get more information on treatments for cystic fibrosis from the Cystic Fibrosis Trust.

Treating the complications of CF

If your CF has caused complications (such as CF-related diabetes, liver disease, osteoporosis or male infertility) you’ll need additional check-ups and treatment. Your GP may be able to provide these, or you might be referred to a specialist.

When your child is 12 years old, their treatment team should start discussing with you and your child the process of moving their care from child to adult services. Involve your child in these discussions. Children with CF usually transition to adult care when they are 16 – 18 years old. You can read more about the transition of care on the Cystic Fibrosis Trust website.

Each person with cystic fibrosis is different. Your child may be happy talking to their friends, teachers and other peers about their condition, or they might prefer to keep it private. If you’re concerned about how your child is adapting to life with CF, talk to their doctor or nurse.

Life with cystic fibrosis can be challenging, but there are resources and information out there to help. You can read more about life with CF on the Cystic Fibrosis website.

According to the 2020 UK Cystic Fibrosis Registry Report, half of the people born with CF today are expected to live to at least 50. As new treatments become available the outlook for people with CF may continue to improve. Indeed, it’s likely that CFTR modulators will improve life expectancy for people with CF.

Sadly, there is no cure for CF, but treatments can greatly help to control symptoms, reduce complications, and help people manage their condition. There are also steps you can take to help manage CF.