Development of a Palliative Care Approach for Primary Progressive Aphasia: My Experience as a Person Living With This Rare Disorder.

Abstract

Frontotemporal disorders are a group of rare young-onset dementias for which there is no cure, nor is there any way to slow the underlying progressive brain degeneration. To date those affected have typically received very little, if any, follow-up care after diagnosis, particularly in the early stages of their disease. I have received a clinical diagnosis, supported by imaging, of primary progressive aphasia, a form of frontotemporal degeneration characterized in the initial phase by progressive impairment of language ability. From the onset, I have been fortunate to receive excellent ongoing palliative care from a multidisciplinary team, some of whom had never previously seen anyone with this disorder. My quality of life has been enhanced by an evolving range of creative strategies and adaptations targeted to my deficits as they have arisen. In this paper, I discuss my experience of the process underlying this personalized plan, which serves as a paradigm for the development of novel palliative care approaches for people living with rare disorders, both neurodegenerative diseases and other conditions.