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CF treatment

CF is treated intensively to prolong life expectancy and improve quality of life for those living with the condition. There is currently no cure for CF, although research continues and there are treatments that can improve and prolong lives.1

Life expectancy has increased steadily over the past 20 years, and today cystic fibrosis is no longer exclusive to childhood.

Lungs1,3-6

Physiotherapy and exercise to help clear airways and improve lung function

  • Daily airway clearance physiotherapy (often referred to as simply ‘physio’) consists of techniques that help to clear excess mucus from the lungs. This is often aided by equipment such as the Flutter® or Acapella®. Other breathing techniques such as autogenic drainage (AD) also exists. Ask your specialist CF team for more information on alternative physio methods that you may find work better for you
  • Exercise is increasingly being recognised as an essential component of CF treatment, as increasing cardiovascular and muscle strength have been shown to have protective effects against lung function decline. Exercise can also help to clear excess mucus

Antibiotics which can be given in a number of different forms to treat lung infection

  • Inhaled through a machine called a nebuliser
  • Oral tablets or liquids
  • Intravenous (IV) antibiotics may be administered at hospital or at home
  • Antibiotic courses may be in response to an exacerbation or taken constantly at a low level to help prevent or supress or chronic infection

Bronchodilators to help open the airways and make it easier to breathe

  • Bronchodilators administered by inhaler

About inhalers — how to use inhalers in CF?

Side effects of inhaled medicines

  • Steroids to reduce inflammation
  • Hypertension saline which can be inhaled through a nebuliser to help clear mucus. There is evidence that this also helps to fight infection
  • Mucolytics to break down thick mucus and make it easier to move and expel
Digestive system1,3
  • Digestive enzyme replacement
  • Vitamin supplements
  • Nutritional supplements
Mind

It’s ok to need extra support. The burden of treatment and having a progressive disease can be understandably difficult to deal with. Additionally, people with CF may experience body image issues from scarring treatment such as peripherally inserted central catheters (PICCs) and ports for IV treatment, or symptoms such as bloating. If you feel like you need someone to talk to or support with the mental aspects of the condition your specialist team will be able to provide you with support services or recommend other pathways.7

Every person with CF should be in close contact with a specialist CF team at a hospital. Here they will attend regular check-ups, an annual review and inpatient stays if necessary. This can help them to help manage the mental as well as the physical aspects of CF.

Annual review and check-ups

People with CF will have regular check-ups with their specialist team to track lung function and general health. The frequency of these check-ups will depend on their current health status8

Once a year, during an annual review, a person with CF will have more extensive testing and meetings with a specialist team. This team can include a dietician, nurses, physiotherapist, consultants and other healthcare professionals where necessary; such as social workers and psychologists. After the review the treatment plan will be written up and reviewed.8,9

New precision medicines

Recently, drugs have been developed and are beginning to enter the market that can correct some of the underlying problems in CF. These drugs address the underlying condition by targeting the part of the body that is affected by the faulty CF gene, restoring some of the function that was lost. Ivacaftor and lumacaftor/ivacaftor are the most well-known of these. So far, there is evidence that these drugs have a positive impact on lung function and exacerbation rate, potentially proloning lives. However, these treatments may target a very specific CF fault that not everyone with the disease has and so cannot be used in all cases.10

Approval code: RESP-IE-NP-00062

Date of preparation: November 2020

References
  1. Treatment for cystic fibrosis. British Lung Foundation. Available at: https://www.blf.org.uk/support-for-you/cystic-fibrosis/treatment Last accessed: November 2020.
  2. CF Registry Ireland Annual Reports 2018. Available at: https://cfri.ie/annual-reports/ Last accessed: November 2020
  3. Cystic fibrosis treatments and medications. CF Trust. Available at: https://www.cysticfibrosis.org.uk/what-is-cystic-fibrosis/cystic-fibrosis-care/treatments-and-medication#Digestive%20system%20section Last accessed: November 2020.
  4. Physiotherapy treatment in cystic fibrosis: airway clearance techniques. CF Trust. March 2013. Available at: https://www.cysticfibrosis.org.uk/~/media/documents/life-with-cf/publications/factsheets/factsheet-physio-airway-clearance.ashx Last accessed: November 2020
  5. Physiotherapy FAQs. CF Trust. Available at: https://www.cysticfibrosis.org.uk/what-is-cystic-fibrosis/cystic-fibrosis-care/physiotherapy/physiotherapy-faqs#aboutexercise Last accessed: November 2020.
  6. Advances toward the Elucidation of Hypertonic Saline Effects on Pseudomonas aeruginosa from Cystic Fibrosis Patients. Available at: https://journals.plos.org/plosone/article?id=10.1371/journal.pone.0090164 Last accessed: November 2020
  7. Quittner AL, et al. Thorax 2016. Available at: https://thorax.bmj.com/content/thoraxjnl/71/1/26.full.pdf Last accessed: November 2020
  8. CF A model of care for Ireland. Available at: https://www.cfireland.ie/images/uploads/resources/CF-model-of-care-full-PDF_%281%29.pdf Last accessed: November 2020
  9. Cystic fibrosis annual reviews. GOSH. Available at: https://www.gosh.nhs.uk/medical-information-0/procedures-and-treatments/cystic-fibrosis-annual-reviews Last accessed: November 2020.
  10. CF News Today. Available at: https://cysticfibrosisnewstoday.com/2020/08/07/newborn-screening-data-shows-lower-cf-incidence-ireland-than-previously-reported/ Last accessed: November 2020

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