Living well with IPF

At the end of this section you should:

  • Understand some of the reasons why early diagnosis may be difficult .
  • Be able to describe ways that may help someone with IPF to keep well.
  • Be able to use interventions which  help breathlessness.
  • Think about the need for anticipatory care in IPF.


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Diagnosis: Diagnosis is very difficult. It is often late in the course of disease as symptoms may be confused with normal ageing. Symptoms are similar to other lung conditions, such as COPD.

Anti-fibrotic medicines: Pirfenidone or Nintedanib. These are relatively new drugs which can slow down the damage to lungs. Patients need to be counselled before starting and monitored  whilst taking them. The support of specialist team is essential. New therapies are being developed.

Symptom management:

  • Signs:
    • Unexplained weight loss.
    • Finger clubbing.
    • Breathlessness on exertion, progressively worsening over time.
    • Breath sounds: fine crackles mostly at lung bases, sounds like sound Velcro.
  • Symptoms:
    • Progressive breathlessness during activity.
    • Dry cough.
    • Fatigue.
    • Aching muscles and joints.

Pulmonary rehabilitation: "Evidence has shown that patients with IPF benefit from the peer support and help with breathlessness management in PR.” 1

Family /carer involvement: Encourage family to accompany to appointments and to PR.

Oxygen assessment: The aim of 02 assessment is to make sure people have access to oxygen at the right time with the right dose to make breathing easier for them.

Anticipatory care looking ahead: Prognosis can be very uncertain in IPF and condition may change rapidly with little warning. 

Multidisciplinary team management: There is evidence that multidisciplinary diagnosis and management increases quality of life and also prolongs life.2


  1. Raghu G, Collard HR, Egan JJ, et al. An official ATS/ERS/JRS/ALAT statement: idiopathic pulmonary fibrosis: evidence-based guidelines for diagnosis and management. Am J Respir Crit Care Med 2011; 183: 788–824 DOI:
  2. Lok SS. Interstitial lung disease clinics for the management of idiopathic pulmonary fibrosis: a potential advantage to patients. Greater Manchester Lung Fibrosis Consortium. J Heart Lung Transplant 1999; 18: 884–890.