How is cystic fibrosis treated?
Children with cystic fibrosis are cared for by a team of specialist health care professionals, based at a children’s CF centre. They’ll have a care plan designed for their individual needs.
Although there is currently no cure for CF, the available treatments help to reduce symptoms and prevent lung disease.
On this page:
- Chest physiotherapy
- New developments in CF treatment
- Treating chest infections
- Nutrition and diet
- Pancreatic enzyme replacement therapy (PERT)
- Lung transplant
- Will my child need to go to hospital?
- What is the life expectancy of a person with cystic fibrosis?
Chest physiotherapy (airway clearance) helps clear your child’s airways by shifting the mucus that has built up in their lungs. This is usually performed twice a day.
There are many different techniques and devices that can be used to clear your child’s airways. These vary depending on their age and what works for them. The physiotherapist will help decide on the best way to clear the airways.
Physical activity and exercise are important for general health and to help clear mucus.
Some children benefit from medicines called mucolytics. These help make the secretions less thick and easier to clear.
New developments in CF treatment
New medicines called CFTR modulator drugs are now being developed for some types of cystic fibrosis. These work differently from other CF treatments. They correct the problem that stops children with CF controlling the balance of salt and water between cells.
These drugs will not help all children with CF. They are only effective in children with particular genetic problems causing CF. At this time, they are not yet all available on the NHS.
Children with cystic fibrosis have frequent chest infections. These may sometimes be called exacerbations.
Chest infections are treated using antibiotics. These can be given by mouth or into a vein. They may also be breathed in (inhaled) through a nebuliser. Antibiotics may be started if:
- your child has symptoms of a chest infection
- an infection is found in the airway mucus sample collected when they are seen in clinic.
Some children with CF take antibiotics regularly all the time, if recommended by their doctors.
Children with cystic fibrosis should have a normal healthy diet.
If they are not growing as they should, they may need more calories in their diet.
It’s common for children with cystic fibrosis not to absorb vitamins properly, so your child may be prescribed vitamin supplements.
Cystic fibrosis can stop the pancreas working properly. This means that the body doesn’t have the enzymes it needs to digest food properly.
If this happens, your child will need to take enzyme supplements to help them digest food.
Children with very poor lung function may sometimes benefit from a lung transplant. This is very rare in children. Most children will not need this.
Children with CF are usually seen in the out-patient clinic by the multi-disciplinary team (MDT) every 2 months. At each visit their symptoms and growth will be assessed. They will also be screened for chest infections and will have their medications reviewed. Children will be seen more often just after diagnosis or if they are unwell.
If your child gets a lung infection, they will need antibiotics. Sometimes these need to be given intravenously – that means putting the antibiotics directly into the vein. Your child may be admitted to hospital for this.
Sometimes children with cystic fibrosis have something called a pulmonary exacerbation or CF lung attack. This is when the symptoms relating to their lungs and breathing become much worse. If the symptoms get very bad your child may need to go into hospital for treatment or specialist investigations.
In hospital, people with CF will be kept away from each other, to prevent infection.
Cystic fibrosis shortens life expectancy, but better treatment and management is helping people with CF to live longer.
According to the UK Cystic Fibrosis Registry, half of all people with cystic fibrosis alive in 2017 were likely to live to at least 47. Treatments are getting better all the time. It’s thought that children born with cystic fibrosis today are likely to live longer, to at least 50.