Debbie Lewis, Jane Scullion
International Journal of Palliative Nursing, Vol. 18, Iss. 7, 27 Jul 2012, pp 331 - 337
Idiopathic pulmonary fibrosis (IPF) is a progressive, life-threatening interstitial lung disease of unknown aetiology and with limited proven treatment options. As it is predominately a disease of older age, a growing elderly population will increase its incidence. IPF has a poor prognosis, with a median survival of 3-5 years after diagnosis and a 5-year survival rate of 10-15%. Patients may suffer gradual decline but acute and unpredictable episodes of respiratory failure may result in death. Further research is needed to ascertain the worth of potential prognostic indicators such as age, respiratory hospitalisations, percentage of predicted forced vital capacity (FVC), and 24-week change in FVC. Integration of palliative care principles into IPF treatment is essential, including advance care planning, relief of physical and psychological burden, and patient and carer education. It is unknown whether pulmonary rehabilitation is of benefit but it may improve fatigue and functional capacity.