Exploring and supporting everyday life with a rare dementia - understanding symptoms, developing strategies and sharing stories

这项研究的目的：这项研究将揭示和交流患有后皮质萎缩症（PCA）的人每天在这种衰弱的情况下的经历。PCA是一种罕见的主要影响视力的痴呆症，通常发病年龄较小。它会导致严重的问题，看不清东西是什么和在哪里，并导致各种日常活动的严重困难，包括阅读、开车和穿衣。我的博士研究表明，患有PCA的人面临着一个复杂而紧张的诊断过程，因为症状不寻常，许多健康专业人员对这种情况不熟悉。有了这个奖学金，我将把这些经验传达给相关的健康专业观众，以确保他们能够更好地注意到并支持那些患有任何与痴呆症相关的视觉障碍的人。我的博士也强调，诊断后，缺乏关于如何管理PCA症状的指导，因此，个人和家庭真的很有创造力和反应迅速，开始了一个持续解决问题的过程，不断适应渐进的症状，以帮助PCA患者尽可能保持独立，并参与对他们重要的活动。这个奖学金将使我能够巩固和分享这些丰富的知识，与其他个人和家庭进行诊断的PCA，以及健康和社会护理专业人员支持他们。这不仅会帮助PCA患者更独立地生活，改善健康状况，还会灌输一种自信和联系感，提醒他们他们是同龄人中的一员，所有人都在经历类似的挑战。我的博士研究包括深入访谈和全天观察患有PCA和更典型的记忆型阿尔茨海默病（AD）的人的家庭，在此期间，我收集了视频和音频记录，健康和功能的测量，以及包括心率和活动水平在内的生理数据。这样的数据组合和数据量以前从未在不同痴呆症患者的家中收集过，与来自不同学科（如言语和语言治疗、生理学）的研究人员合作，有许多进一步数据分析的可能性，这个奖学金将给我机会去追求。重要性：对家中的人进行访问使这项研究对痴呆症患者来说非常容易和包容，因为他们在一个最舒适、最熟悉、最能干的环境中进行观察。另一个重要的下一步是不仅分享我在博士研究中发现的东西，还要分享我在研究过程中学到的东西，鼓励其他人在家里对痴呆症患者进行研究，以确保未来的研究结果能够代表不同痴呆症患者及其家人的真实生活并与之相关。从更大的角度来看，分享我的发现和进一步的数据分析是很重要的，因为阿尔茨海默病（AD）经常被误认为是一种只影响记忆的疾病。人们对痴呆症相关的视觉障碍了解甚少，往往被忽视，这可能是因为在更典型的记忆主导型阿尔茨海默病中，视觉症状往往出现在疾病的后期，此时人们不太能够清楚地解释和回忆起他们的症状和任何感知挑战。这对如何在现场提供护理具有实际意义，例如，患有晚期AD的人可能被认为“忘记”了他们的一杯茶，而实际上他们可能无法感知它。更多地分享与痴呆症相关的视力损害的影响以及人们如何适应和调整，对服务发展具有重大意义，从长远来看，有可能最大限度地提高受影响者的独立性、应对能力和福祉。

What this fellowship will achieve:This fellowship will reveal and communicate what people living with posterior cortical atrophy (PCA) experience living with this debilitating condition day-to-day. PCA is a rare dementia mostly affecting vision which usually has a young age of onset. It leads to significant problems with seeing what and where things are and causes profound difficulties with all sorts of daily activities including reading, driving and dressing.My PhD research has shown that people living with PCA face a convoluted and stressful diagnostic journey, as the symptoms are unusual and many health professionals are unfamiliar with the condition. With this fellowship I will communicate these experiences to the relevant health professional audiences to ensure they are better equipped to notice and support those living with any kind of dementia-related visual impairment. My PhD also highlighted that post-diagnosis, there is a lack of guidance about how to manage PCA symptoms and as a result, individuals and families are really creative and responsive, embarking on a process of ongoing problem-solving, continually adapting to the progressive symptoms in order to help the person with PCA to stay as independent as possible and engaged in the activities that matter to them. This fellowship will allow me to consolidate and share this wealth of knowledge with other individuals and families navigating a diagnosis of PCA, as well as the health and social care professionals supporting them. This will not only help people with PCA to live more independently and improve wellbeing, but will also instil a sense of confidence and connection in reminding them they are among a community of peers, all keeping going through similar challenges.My PhD research involved in-depth interviews and all-day observations in the homes of people living with PCA and more typical, memory-led Alzheimer's disease (AD), during which I collected video and audio recordings, measures of wellbeing and functioning, and physiological data including heartrate and activity levels. This combination and volume of data hasn't before been collected in the homes of people with different dementias and there are numerous possibilities for further data analysis in collaboration with researchers from a range disciplines (e.g. speech and language therapy, physiology), which this fellowship will give me the opportunity to pursue.Why it is important:Visiting people at home made this study very accessible and inclusive for people with dementia as they were observed in an environment in which they were most comfortable, familiar and able. Another important next step will be to share not only what I found in my PhD research but also what I learned while doing it, to encourage others to do research at home with people living with dementia, to make sure that future findings are representative of and relevant to the real lives of people living with different dementias and their families. Looking at the bigger picture, the sharing of my findings and further data analysis this fellowship will allow me to do is important because Alzheimer's disease (AD) is often misperceived as a disease that solely affects memory. Dementia-related visual impairment is poorly understood and often neglected, perhaps because in more typical memory-led AD, visual symptoms often occur at the later stages of the condition, at a time when people are less able to clearly explain and recall their symptoms and any perceptual challenges. This has real implications for how care is delivered on the ground, e.g. a person with later stage AD may be thought to have "forgotten" their cup of tea when they may actually be unable to perceive it. Sharing more about the impacts of dementia-related visual impairment and how people adapt and adjust, has significant implications for service development, and in the longer term, potential to maximise the independence, coping and wellbeing of those affected.