Quality of life in primary progressive aphasia: What do we know and what can we do next?

Background: A quality of life approach to managing communication and neurodegenerative conditions is increasingly encouraged by professional bodies and healthcare models. Clinical services for primary progressive aphasia (PPA) continue to expand and the research base continues to grow; however, there is little research specifically investigating quality of life in PPA. Aims: This critical review aimed to determine the available knowledge about quality of life in PPA and gaps in that knowledge, and to consider how PPA researchers might best address those gaps. Main contribution: Two unpublished single case series studies and one published case study were identified that directly investigated aspects of quality of life in PPA. These suggested that quality of life is heterogenous and determined by a range of factors, supporting a person-centred approach to care. The current literature also includes five accounts of living with PPA authored by individuals with PPA or their carers, discussing factors that influenced these individuals' quality of life. Seventeen empirical investigations of mood were identified, suggesting that approximately 40% of individuals with PPA develop depression at some point. Finally, several recent studies have noted the importance of promoting quality of life in PPA and/or have utilised quality of life outcome measures; however, most lack a clear conceptualisation of the construct of quality of life, and how this construct is related to the instruments and methods chosen to measure it. Conclusions: Literature on quality of life in PPA is beginning to emerge, although little is available in indexed databases. More research is needed that better conceptualises the construct of quality of life, critically appraises the way quality of life is assessed in PPA, builds on findings from the current small samples, and involves individuals with PPA in formulating research directions and research tools that will enable discussion about their quality of life.