My experience of living with nonfluent/agrammatic variant primary progressive aphasia: Challenges, compensatory strategies and adaptations

Background Primary progressive aphasia (PPA) is a rare neurodegenerative brain disorder characterized by declining language ability. There is currently no way to reverse or slow the course of the progressive brain degeneration, nor is there a cure for PPA. Throughout the course of the disease, any treatment must therefore be palliative in nature and should be designed to manage symptoms and improve the quality of life of the affected person. There is little information in the medical literature about strategies to make meaningful improvements to the quality of life of people with PPA written from the perspective of those living with this condition. Aims I have a clinical diagnosis of the nonfluent/agrammatic variant of PPA (nfvPPA), supported by imaging. In this report I discuss my experience of the progressive loss of language and communication skills, and detail the challenges I have been facing. I also describe how my quality of life has been enhanced by the early initiation of treatment focusing on communication strategies targeted to my specific impairments and designed to support my individual interests and goals. Methods & Procedures I was fortunate to obtain an early diagnosis from a cognitive neurologist experienced with PPA. From the onset of my language difficulties, I have received excellent personalized care from a multidisciplinary medical team including speech-language pathologists, a cognitive neurologist and other doctors. Main Contributions My life during the early stage of nfvPPA has been enriched by personalized care focused on supporting the particular activities, interests and goals that are most important and meaningful to me. As my disease has progressed, I have benefited from an evolving range of strategies and adaptations targeted to the specific deficits in the areas of speaking, writing and reading that I have been facing at any given time. In addition, I have adopted methods to enhance the benefit of these language-directed strategies. And I have been employing evidence-based approaches that improve general brain health and thereby indirectly support my language. Conclusions & Implications My experience represents a model for the personalized care of people in the early stage of nfvPPA. WHAT THIS PAPER ADDS What is already known on the subject There is minimal information in the medical literature describing the subjective experience of a person with PPA. There is little information in the medical literature about strategies to make meaningful improvements to the quality of life of people in the early stage of PPA. What this paper adds to existing knowledge I have a clinical diagnosis of nfvPPA, supported by imaging. In this paper I give a first-person account of my experience of the progressive loss of language and communication skills, and I detail the challenges I have been facing. I describe how my quality of life during the early stage of nfvPPA has been enhanced by an evolving range of strategies and adaptations tailored to my speech and language deficits as they have arisen. These compensatory strategies have focused on supporting the particular activities, interests and goals that are most important and meaningful to me. What are the potential or actual clinical implications of this work? The description of my subjective experience of the progressive loss of language and communication skills offers insight for speech-language pathologists, neurologists and other professionals involved in the clinical care of people in the early stage of nfvPPA. My experience represents a model for the personalized clinical care of people in the early stage of this disorder.