The aim of this thesis is to explore fear of choking and fear of falling in people with Huntington's disease (HD) and their caregivers. Dysphagia and falls are common in HD and may lead to fear of... Show moreThe aim of this thesis is to explore fear of choking and fear of falling in people with Huntington's disease (HD) and their caregivers. Dysphagia and falls are common in HD and may lead to fear of choking and fear of falling. However, knowledge about this is mostly lacking, as well as knowledge about the relationship between cognitive and emotional factors and these types of fear. The study confirms that fall prevalence (29% over a 30-day period) and prevalence of dysphagia symptoms (90%) are high. However, a prevalence of about 50% is also reported for fear of choking and fear of falling in people with HD. However, their informal caregivers report the most fear. Severity of dysphagia symptoms was a predictor of fear of choking, and anticipatory awareness of fall risks and gender were found to be predictors of fear of falling. In most individuals with HD, a combination of preventive measures was used.Future research can improve both management of dysphagia and fear of choking and management of falls and fear of falling. Because questioning people with HD is sometimes difficult, due to speech and cognitive difficulties, diagnostic tools may be sought to facilitate this, such as wearable electronic devices. Show less
Huntington’s disease (HD) is a progressive neurodegenerative disorder, which is clinically characterized by motor impairment, cognitive decline, and behavioral symptoms. In this thesis we have... Show moreHuntington’s disease (HD) is a progressive neurodegenerative disorder, which is clinically characterized by motor impairment, cognitive decline, and behavioral symptoms. In this thesis we have investigated measurement properties of the Unified Huntington’s Disease Rating Scale (UHDRS) and the Unified Huntington’s Disease Rating Scale-For Advanced Patients (UHDRS-FAP). These assessment scales have been developed to monitor the presence, severity, and progression of symptoms systematically over time. To measure change of symptoms accurately, reliable and valid scales are essential. Poor interrater reliability was found for all dystonia items of the UHDRS. We concluded that the rating of these items is difficult to interpret, probably as a consequence of the subjective nature of the response options. Future studies are required to explore how the dystonia items can be improved. We have also demonstrated that the motor and cognitive scores of the UHDRS-FAP deteriorated in patients with advanced HD during six months follow-up, while the motor and cognitive scores of the UHDRS did not show any change. This finding suggests that the UHDRS-FAP can detect disease progression in late stage HD, contrary to the UHDRS. Therefore, we recommended the use of the UHDRS-FAP motor and cognitive scores in long-term care facilities to optimize HD care. Show less
