Best supportive care for idiopathic pulmonary fibrosis: current gaps and future directions

Abstract

Best supportive care (BSC) is generally defined as all the interventions and the multiprofessional approach aimed to improve and optimise quality of life (QoL) in patients affected by progressive diseases. In this sense, it excludes and might be complementary to other interventions directly targeting the disease. BSC improves survival in patients with different types of cancer. Patients with idiopathic pulmonary fibrosis (IPF) experience a vast range of symptoms during the natural history of the disease and might have a beneficial effect of BSC interventions. This review highlights the current evidence on interventions targeting QoL and gaps for the clinical assessment of BSC in the treatment of IPF patients. Very few interventions to improve QoL or improve symptom control are currently supported by well-designed studies. Sound methodology is paramount in evaluating BSC in IPF, as well as the use of validated tools to measure QoL and symptom control in this specific group of patients.

Patient reported distress can aid clinical decision making in idiopathic pulmonary fibrosis: analysis of the PROFILE cohort

“…Previous studies using alternate patient reported outcomes to measure quality of life in IPF have similarly noted weak to moderate correlations with lung function measures, including the MRC dyspnoea scale, ATAQ-IPF and CAT [25-27]. Imperfect correlations indicate that lung function alone cannot account for all differences in patient reported outcomes, in combination they offer tremendous clinical value [28]. However, the MRC dyspnoea scale is one dimensional, ATAQ-IPF remains extensive at 75 items and CAT was developed for COPD, which at 8 items is convenient but may not have the sensitivity to capture IPF traits [29].…”

Comprehensive Care in Pulmonary Fibrosis

“…The best supportive care would be the combination of interventions in a holistic multidisciplinary therapeutic approach that looks for improving wellbeing and QoL in patients suffering progressive and devastating illnesses, considering the different needs related to the disease, the personal or cultural believing and the physical and psychosocial state 31,65 . The best care in IPF and other pulmonary fibrotic diseases should be patient-centered and not only include an optimal anti-fibrotic approach but also any intervention that reduces the burden of the disease, helps to control drug-related adverse events, and enhances the physical and mental state of the patient, aimed at improving the QoL.…”

“…Talking about the end of life with the patient and caregivers remain a challenge in IPF and other lung fibrotic diseases; however it is useful to enable patients and families to make decisions in line with their values and preferences 16,65 . Although the integration of palliative care into non-malignant life-threatening disease is not routine, family members of patients that received care at home with hospice services were more likely to report a less harmful dying experience.…”

Palliative care for patients with pulmonary fibrosis: symptom relief is essential

“…Once symptom burden is accurately assessed, there are a number of pharmacological and nonpharmacological therapies available to reduce symptom burden [6,10,11]. These include regular counselling, anxiolytic and anti-depressive medications, low-dose narcotics, pulmonary rehabilitation, supplemental oxygen therapy, and early referral to palliative care to alleviate symptom burden and address advance care planning, with the ultimate goal of improving quality of life [3,12,13].…”