Improving clinical management and quality of life in mid to late stage Huntington’s disease: How research can inform clinical practice and vice versa

 Caroline A. Fisher, Anahita Brown, Katherine Sewell and Bronwyn Moorhouse

Thematic Overview

Utilising the research literature is an important part of clinical practice as implemented interventions should be evidence-based, wherever possible. However, in some areas a strong research evidence base may be lacking. Under these circumstances there is often much that can be achieved through clinical research to improve knowledge and evaluate practice and outcomes. Thorough practice evaluation and clinical audit data can be used to formulate new management guidelines to improve both patient care and quality of life.

Content Overview

This presentation will focus on the driving factors behind, and results of, five papers recently published by the multidisciplinary research team from the Brain Disorders Program at Austin Health. The research focussed on two areas of concern in the care of patients with mid-to-late Huntington’s disease: aggression and the long-term use of modified diets. The clinical implications of this research will be discussed, including how the results can be used to inform and improve clinical practice into the future.

Background

Huntington’s disease (HD) is an autosomal dominant genetic disorder. The three defining features are a movement disorder, psychiatric symptoms and cognitive impairment. The onset of HD symptoms generally occurs in mid to late adulthood, then progress over a 10 to 20 year period to be eventually fatal. In addition to psychiatric disturbance, behaviour problems are also common in HD, particularly aggression. Despite previous studies indicating that rates of aggression are high, little was previously known about causative or antecedent contributors to aggressive behaviour in HD sufferers. There was also little published empirical data regarding treatment methods for this problematic behaviour. A second issue of clinical concern in this population is the prescription of the long-term use of modified (pureed) diets, necessitated by swallowing problems as a result of the progressive movement disorder. Concerns included the sensory deprivation of ingesting a pureed diet for many years, disconnection from food in its standard form, taste fatigue and reduced dietary enjoyment.

Methods

Members of the clinical team (Occupational Therapy, Speech Pathology and Neuropsychology) on the Brain Disorders Unit (BDU) thoroughly reviewed the research literature in the areas of aggression and the use of long-term modified diets in HD. These reviews then formed the basis for a series of clinical research initiatives aimed at improving knowledge and patient care. Varying research methodologies were utilised, as appropriate, including systematic review, practice analysis, empirical clinical case studies, and a large scale clinical data audit.

Results and Outcomes

This series of papers was instrumental in improving knowledge about aggression in mid-to-late stage HD sufferers. It revealed that rates of aggression in this population are very high (90% in the inpatient BDU sample), and that both verbal and physical aggression are common. It further revealed that personal care is the most common antecedent trigger for aggressive behaviour, but that there is a degree of inter-individual heterogeneity of triggers for aggressive behaviour. Additional outcomes indicated that sensory sensitivities may contribute to behaviour disturbance and that individual sensory modulation programs can be effective for reducing aggression, particularly when combined with behaviour modification plans. Multiple treatment recommendations to reduce distress and rates of aggression were developed as a result of this research. These included modifications to personal care routines such as taking extra care to ensure the HD sufferer feels comfortable, unrushed and forewarned of upcoming tasks during personal care, conducting sensory processing assessments and utilising sensory modulation equipment and techniques, such as the use of individualised comfort items (soft pillow, blankets) and/or distraction (click-clack balls, hand-held massagers, playing music to the client) during personal care and other activities, and the use of behaviour assessments and individualised behaviour support plans to target known antecedents for aggression in individual clients.

 

The research also revealed the paucity of studies into the long-term use of modified diets in any population, not just HD. The clinical practice analyses undertaken indicated that it was possible to make changes to clinical practice to improve the gastronomical and social experience of mid-to-late stage HD sufferers, via improving the texture of food, the range of food choices available for clients and participation in food preparation and meal groups.