Introduction
Idiopathic pulmonary fibrosis (IPF) is a typically life-shortening condition whose debilitating symptoms include activity-limiting dyspnoea, nagging cough and fatigue.1 At the time they are diagnosed with IPF, patients (and their informal caregivers) are forced to confront the ‘devastation’ and begin thinking about a life with this disease.2
Most patients with IPF will require supplemental oxygen (O2) at some point after diagnosis. Although O2 is prescribed in the hopes of preventing long-term consequences of hypoxaemia and improving symptoms, it poses a threat to already-impaired quality of life (QOL) in these patients2: many feel stigmatised when they are seen in public wearing a nasal cannula, and using O2 robs patients of their ability to live as carefree and independently as they would like.3 4 Understandably, patients view the need for O2 as a marker of disease progression—an unwanted milestone in the course of IPF that, like being told the diagnosis, can cause their worlds to ‘crash for a second time.’5 Compared with patients with IPF who do not need O2, those who need it report worse QOL in multiple life domains, including emotional well-being, social participation and independence.6 Patients with IPF are resilient: in clinic, I frequently hear patients with IPF say things like, ‘I can deal with this… if it just doesn’t get any worse’. But, it so often does—and they deal with it anyway and figure out how to move on.
The closest thing to a formal staging system for IPF is the GAP index (a combination of gender, age and physiological variables), which can be generally informative in discussions of prognosis.7 But patients with IPF also want information on what life is going to look like with IPF—how disease progression is likely to affect them in their daily lives and what things they should be doing or thinking about along the way. Informal caregivers want similar information about the patient. They also want to know how their roles will change as disease severity increases, and they need to know that helpful resources are accessible to them.
I believe care of patients with IPF requires an ongoing conversation (with them and their caregivers) woven through serial visits over the course of the disease. This conversation forms the backbone of the palliative care these patients need—unfortunately, the term ‘palliative care’ has become synonymous with end-of-life care, and perhaps more appropriate terms to use are ‘comprehensive care’ or ‘quality of life (QOL) care’. Regardless, the conversation should, among other topics personalised to a given patient, include an assessment of disease status, an appraisal of the tolerance of and response to therapeutic manoeuvres (pharmacological and/or non-pharmacological), an evaluation of the patient’s and the caregiver’s physical and emotional well-being, assurance that necessary supportive resources are in place, and time for questions. As with any patient with a potentially life-shortening condition, this longitudinal conversation must involve appropriately timed talk around advanced care planning and, most importantly, patients’ wishes for end-of-life intervention and care.5 8 9 Like other topics in the weave, these are best brought up early, before the pressures and chaos of abrupt decline mandate. Here, practitioners have the challenging duty of (1) ascertaining what information patients and caregivers are ready to receive, (2) recognising that patients’ and caregivers’ levels of acceptance—or as Overgaard and colleagues called it, ‘reactional stage’5—may differ, and thus (3) carefully tailoring information delivery to the recipient while considering their readiness to receive it.
I view four major events in the course of IPF as meriting special care and attention and forming a framework for the disease-long conversation. The first is when the diagnosis is given. Because of the potential impact of O2,2–6 the final three events centre on it: when O2 is prescribed for use with exertion; when O2 is needed at rest; when high-flow O2 is required (ie, when a portable oxygen concentrator or other lightweight, portable, O2 conserving device does not meet a patient’s oxygen demands).
I initially regarded these events as stages because, to some degree, they often correspond with disease severity. But on reflection, they are much more: for patients and caregivers, both individually and for them as a team, these events are times of transition, when each is particularly vulnerable, when their QOL is most threatened, and they are forced to adapt to a new normal. More than perhaps any other times in the course of IPF, these are the occasions when patients and caregivers need practitioners as partners—accessible, ready to give information, offer support, bolster realistic hope and attend to their emotional well-being and QOL. For practitioners, these events can serve as touchpoints—special opportunities to intervene when we have the chance to more fully understand patients’ and caregivers’ preferences, values and goals through comprehensive needs assessments. Whether done systematically using a tool specifically developed for this purpose10–12 or spontaneously in the natural flow of a visit, the assessments are critical to QOL care. Because patients' and caregivers' needs change over time, the touchpoints can serve as both reminders and opportunities for the assessments to occur.