Meaning and purpose in Huntington’s disease: a longitudinal study of its impact on quality of life

Abstract

Objective Previous work in Huntington’s disease (HD) has shown that a sense of meaning and purpose (M&P) is positively associated with positive affect and well‐being (PAW); however, it was unknown whether HD‐validated patient‐reported outcomes (PROs) influence this association and how M&P impacts PROs in the future. Our study was designed to examine if HD‐validated PROs moderate the relationship between M&P and PAW and to evaluate if baseline M&P predicts 12‐ and 24‐month changes in HD‐validated PROs. Methods This was a longitudinal, multicenter study to develop several PROs (e.g., specific for the physical, emotional, cognitive, and social domains) for people with HD (HDQLIFE). The sample consisted of 322 people with HD (n = 50 prodromal, n = 171 early‐stage manifest, and n = 101 late‐stage manifest HD). A single, multivariate linear mixed‐effects model was performed with PAW as the outcome predicted by main effects for M&P and several moderators (i.e., an HD‐validated PRO) and interactions between M&P and a given PRO. Linear‐mixed models were also used to assess if baseline M&P predicted HD‐validated PROs at 12 and 24 months. Results Higher M&P was positively associated with higher PAW regardless of the magnitude of symptom burden, as represented by HD‐validated PROs, and independent of disease stage. In our primary analysis, baseline M&P predicted increased PAW and decreased depression, anxiety, anger, emotional/behavioral disruptions, and cognitive decline at 12 and 24 months across all disease stages. Interpretation These findings parallel those seen in the oncology population and have implications for adapting and developing psychotherapeutic and palliative HD interventions.

Disparities in Palliative Care Utilization Among Hospitalized People With Huntington Disease: A National Cross-Sectional Study

“…24 Since there are no evidence-based PC models in existence for this population in the outpatient setting, 5,25,26 where most care presides, efforts are, therefore, warranted to adapt and pilot PC models (e.g., meaning-centered) to people with the HD genetic mutation, which could be interchangeable among various environments (e.g., outpatient and inpatient), and stages of illness (prodromal, early, and late). 27 In conclusion, these data are a compelling first step showcasing sociodemographic and psychosocial factors were associated with PC utilization among hospitalized persons with HD. People whose household resides in the bottom income even independent of the hospital's size or location, were negatively associated with PC encounters.…”

“…24 Since there are no evidence-based PC models in existence for this population in the outpatient setting, 5,25,26 where most care presides, efforts are, therefore, warranted to adapt and pilot PC models (e.g., meaning-centered) to people with the HD genetic mutation, which could be interchangeable among various environments (e.g., outpatient and inpatient), and stages of illness (prodromal, early, and late). 27…”

Protocol for EXICODE: the EXIstential health COhort DEnmark—a register and survey study of adult Danes

“…While these tendencies pertaining to the links between health and existential, spiritual or religious aspects and care are relevant for patients with cancer, patients with COPD and cardiac arrest survivors, they may also be relevant to a much broader spectrum of patients. Such tendencies are similarly observed in patients with chronic neurological diseases, for example, in patients with Alzheimer’s disease78 79 and patients suffering from Huntington’s disease, the latter having a relatively rapid decline compared with Alzheimer’s disease and being rarer and ultimately lethal 80…”

“…Such tendencies are similarly observed in patients with Open access chronic neurological diseases, for example, in patients with Alzheimer's disease 78 79 and patients suffering from Huntington's disease, the latter having a relatively rapid decline compared with Alzheimer's disease and being rarer and ultimately lethal. 80 The pathophysiologic processes behind suffering from cancer, cardiac arrest or COPD are very different, resulting in different situations for the patient. Cancer diagnoses have very heterogeneous expressions, cardiac arrest is often a sudden event characterised among other things by the potential of (full) recovery (however, seldom recovery from the underlying cause), while COPD is a slowly progressing disease with no possibility of being cured, and with focus on coping, relief of symptoms and halting of disease progression.…”

“…Such tendencies are similarly observed in patients with chronic neurological diseases, for example, in patients with Alzheimer’s disease 78 79 and patients suffering from Huntington’s disease, the latter having a relatively rapid decline compared with Alzheimer’s disease and being rarer and ultimately lethal. 80 …”