Quality of life and palliative care need in multiple system atrophy and progressive supranuclear palsy : a pilot study using quantitative and qualitative methods

Abstract

Background Multiple System Atrophy (MSA) and Progressive Supranuclear Palsy (PSP) are atypical Parkinsonian disorders which are rapidly progressive. The impact that Parkinsonian disorders has on quality of life (QoL) is increasingly understood, though less work has been done in MSA and PSP compared to Parkinson’s disease. The role of Palliative Care in enhancing QoL is beginning to be translated into clinical practice though fewer studies have been done in MSA and PSP compared with Parkinson’s disease. Methods A cross-sectional study of 23 MSA patients and 24 PSP patients was carried out, assessing aspects of QoL, depression, palliative symptoms and clinical milestones such as dysarthria. Carers for each disease type were assessed in terms of carer strain and QoL. A range of QoL measures including subjective, disease-specific and general QoL scores were taken with the aim of achieving a holistic impression of QoL and symptom burden. A subset of participants were selected for interviews to obtain personal perspectives of living with these conditions. The interviews were evaluated using thematic analysis, to gain a still fuller, richer picture of the implications of these diseases on QoL for patients and carers. The use of both quantitative and qualitative methods was intended to complement each other, with the recognition that QoL is a complex and subjective concept and cannot be encompassed using a single type of assessment. Results Using multiple linear regression, QoL was predicted for by depression and palliative need in MSA and PSP, with severity having an influence in PSP only. Carer mental well-being and patient depression influenced different aspects of carer QoL. Issues with legs was the highest-rated symptom in both groups and there was no significant difference in palliative need between MSA and PSP. Subjective QoL using the SEIQoL-DW score produced diverse domains which people felt influenced their QoL. The most commonly nominated were ‘family’ and ‘partner’; some domains in common were discussed in interviews. ii The overarching themes in interviews were connection to others, transitions (including adjustment) and seeking support, from peers, palliative care services and sourcing expertise for these rare conditions. Conclusion MSA and PSP have a profound effect on QoL, seen using a range of QoL scores. Depression and symptoms frequently managed in palliative care, predict for patient disease-specific QoL, though severity seems to have a greater impact on QoL in PSP compared with MSA. Carer QoL is impacted by patient depression and by carers’ own mental well-being. This work emphasises that QoL in progressive neurological disorders is heterogeneous and individual. Patients and their carers would likely benefit from an individualised, palliative approach supporting patients through the course of their disease, maximising QoL to enhance the experience of living with a progressive disease.