Idiopathic pulmonary fibrosis (IPF) is a debilitating condition with life expectancy of two to five years from diagnosis. Treatment strategies for IPF are disappointingly limited and pirfenidone is currently the only licensed drug that has been shown to reduce the decline in forced vital capacity (FVC) at six months. We demonstrate our experience in prescribing pirfenidone in a single centre observational study of forty patients involved in a named patient programme (NPP) from September 2011 to January 2013. We demonstrate that improved adherence and compliance can be achieved by specialist nurse and clinician review, support and education of the patient. Twenty three of 40 (58%) patients experienced predominantly gastrointestinal adverse effects. Importantly we have enhanced patient adherence and compliance from an initial discontinuation rate of six patients (15%) at the beginning of the study to a zero discontinuation rate in the subsequent ten months. This study shows that in the real world pirfenidone is well tolerated and with expert regular specialist review adherence can be optimised and improved.
Keywords: Idiopathic pulmonary fibrosis; Interstitial lung disease; Treatment.
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