Moving beyond stigma - are concurrent palliative care and management of pulmonary arterial hypertension irreconcilable or future best practice?

Abstract

Pulmonary arterial hypertension (PAH) is aserious life-threatening condition. Althoughmedian survival was estimated at 2.8 years(1), recent studies suggest median survival insome patients may approach 9 years (2). Nev-ertheless, symptoms such as dyspnea, painand depression, among others have a negativeimpact on patient’s overall quality of life(QOL). This perspective aims to explore fac-tors that may account for a lack of palliativecare (PC) utilisation in PAH.The philosophy of PC is often misunder-stood by patients with PAH and their provid-ers, as PC is commonly mistaken for hospicecare, although the two are distinct (3). Pallia-tive care involves active total care of patientswhen diseases are not fully responsive tocurative therapy. PC seeks to achieve the bestQOL for patients by increasing comfort anddignity for as long as patients have to live.This often involves identifying and treatingproblematic symptoms and attempting tounderstand a patient’s goals of care in anongoing fashion, in the face of a life-limitingillness. However, PC does not have restric-tions regarding a specific prognosis or limita-tions in which therapy can be offered. Thisfreedom allows PC to be introduced earlier inthe disease trajectory, whereas hospice carerequires a terminal diagnosis and an averagelife expectancy of 6 months or less.Palliative care has benefit to patients withlife-limiting illnesses, as evidenced by recentstudies in advanced lung cancer which dem-onstrate that PC can improve patient symp-tom burden and QOL without worseningmortality (4). Beyond malignancy, routine PCinvolvement has been suggested in advancedheart failure to maximise QOL and empowerpatient decision-making (5,6). While progno-sis with PAH may be better than somepatients with heart failure, significant symp-tom burden and diminished QOL related toadvanced PAH, or the pharmacotherapy totreat PAH, are well-described (3,7–9). How-ever, attempts at utilising PC to mitigatesymptom burden may be met with fear orresistance.Palliative care involvement still allowspatients to receive concurrent PAH-directedtherapies, which is a commonly encounteredmisconception. As PAH is often treated by amultidisciplinary team, routine PC collabora-tion may be valuable with identifying andtreating symptoms. As a relative dearth ofliterature exists regarding PAH and PC, wepropose three potential barriers for a paucityof collaboration.