The Lived Experiences of People with Progressive Supranuclear Palsy and Their Caregivers.

Abstract

Progressive supranuclear palsy (PSP) is a neurodegenerative disorder initially characterised by disturbances in gait, balance and posture, with death occurring after several years of progressive physical and cognitive decline. This, along with a low index of suspicion, a high degree of diagnostic uncertainty and no approved treatment options, greatly impacts the lives of patients and caregivers. This research was conducted to (i) gain insight into the clinical and emotional journey of patients with PSP, (ii) assess experiences and perspectives, (iii) understand disease impact and (iv) identify key challenges and unmet needs. A literature search and qualitative interviews with six PSP experts were conducted to map the clinical pathway and identify breakpoints. The pathway was validated by key opinion leaders in seven countries. Qualitative research was conducted over 6months in seven countries with PSP stakeholders (N = 112) to explore the emotional journey. The approach included self-ethnography, 60-min telephone interviews and the completion of 7-day smartphone diaries. The current PSP clinical journey can take many different pathways, with patients cycling through the healthcare system before a correct referral is made and a possible/probable diagnosis received. Breakpoints contribute to delays in accessing appropriate clinical care, a high degree of diagnostic divergence and suboptimal management of the disease. The emotional journey is dominated by negative feelings, although some moments of positivity were noted. The research highlighted a lack of disease understanding amongst all stakeholders and a lack of support for patients/caregivers. The authors make a number of recommendations for care improvements, including longer consultation times, closer collaboration among healthcare professionals and patient organisations, and more varied support and information for patients/caregivers. This work represents a major collaborative effort to understand the lived experience of PSP. The research illustrates that a coordinated effort from all stakeholders is required to address ongoing needs and challenges within PSP.