Volunteer, Department of Medicine, Division of Gerontology, Geriatrics, and Palliative Care, The University of Alabama at Birmingham, Birmingham, AL, USA.
Int J Lang Commun Disord. 2023 Mar;58(2):542-554. doi: 10.1111/1460-6984.12798. Epub 2022 Oct 14.
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.
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.
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 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.
原发性进行性失语症（PPA）是一种罕见的神经退行性脑疾病，其特征是语言能力逐渐下降。目前，尚无逆转或减缓进行性脑退化的方法，也没有治愈 PPA 的方法。在疾病的整个过程中，任何治疗都必须是姑息性的，旨在管理症状并提高受影响者的生活质量。医学文献中几乎没有关于从患有这种疾病的人的角度出发，对 PPA 患者的生活质量进行有意义的改善的策略的信息。
我被临床诊断为非流利/语法障碍变异型原发性进行性失语症（nfvPPA），并得到了影像学的支持。在本报告中，我讨论了我经历的语言和沟通能力逐渐丧失，并详细介绍了我所面临的挑战。我还描述了我的生活质量如何通过早期针对我的特定障碍的沟通策略的治疗得到提高，这些策略旨在支持我的个人兴趣和目标。
我很幸运，在一位经验丰富的 PPA 认知神经科医生那里获得了早期诊断。从我的语言困难开始，我就从一个包括言语语言病理学家、认知神经科医生和其他医生在内的多学科医疗团队那里获得了出色的个性化护理。
在 nfvPPA 的早期阶段，我个人化的护理集中在支持对我来说最重要和最有意义的特定活动、兴趣和目标，使我的生活变得更加丰富。随着疾病的进展，我从针对我在任何特定时间面临的说话、写作和阅读方面的特定缺陷的一系列不断发展的策略和适应中受益。此外，我还采用了一些方法来提高这些语言导向策略的效益。我还采用了基于证据的方法来改善整体大脑健康，从而间接地支持我的语言。
我的经历代表了 nfvPPA 早期患者个性化护理的模式。
关于该主题已有的知识：
医学文献中关于描述 PPA 患者主观体验的信息很少。
医学文献中关于在 PPA 早期阶段提高生活质量的策略的信息很少。
本文增加了哪些新的知识：
我被临床诊断为 nfvPPA，影像学支持。在本文中，我以第一人称的方式描述了我经历的语言和沟通能力逐渐丧失的过程，并详细介绍了我所面临的挑战。
我描述了在 nfvPPA 的早期阶段，我的生活质量是如何通过一系列针对我的言语和语言缺陷的策略和适应来提高的，这些策略和适应是随着缺陷的出现而出现的。
这些补偿策略集中在支持对我来说最重要和最有意义的特定活动、兴趣和目标上。
这些策略和适应是如何影响我的生活的：
对言语语言病理学家、神经学家和其他参与 nfvPPA 早期患者临床护理的专业人员的描述，为他们提供了关于我逐渐丧失语言和沟通能力的主观体验的见解。
我的经历为这种疾病的早期患者的个性化临床护理提供了一个模式。
