Oral Feeding in Huntington’s Disease: A Guideline Document for Speech and Language Therapists

Abstract

SUMMARY Speech and language therapy has an important role in the management of Huntington’s disease (HD). Swallowing difficulties affect most individuals with HD. Throughout the disease process these difficulties require management with timely and effective therapeutic intervention. Currently there are no European guidelines for the assessment and management of swallowing impairments in HD. The European Huntington’s Disease Network (EHDN) Standards of Care Speech and Language Therapy Working Group has brought together expert speech and language therapists from across Europe to produce guidelines to improve the management of swallowing disorders for individuals with Huntington’s disease. The guidelines were developed with the aim of promoting timely and appropriate assessment of the swallowing process and focused management throughout all stages of the disease. Literature was systematically searched in an attempt to ensure that the recommendations are based on sound evidence. Where evidence was lacking, specific guidance is based on expert consensus. The provision of care varies widely between countries in Europe and the implementation of those guidelines should improve the quality of care delivered to individuals with HD.

Swallowing endoscopy findings in Huntington's disease: a case report

“…Clinical assessment provides information on the presence, nature, and severity of dysphagia 4,10 , but supplementary exams through objective assessment of swallowing are needed for its diagnosis 8 .…”

“…Also, the literature reports the presence of these signs only in advanced stages of the disease. In addition to the progression of the disease, other aspects that should be considered and that can increase the risk of tracheal aspiration are also related to the amount of pharyngeal residue 4 . There are no studies in the literature describing oropharyngeal dysphagia by the progression of the disease in this population due to the particular manifestations of each individual.…”

“…They verified the swallowing of individuals using videofluoroscopy, and identified postural changes, inadequate movement of the tongue, multiple swallowing, pharyngeal residue after swallowing, and residues in the valleculae and pyriform sinuses 6,7 . The presence of penetration and/or laryngotracheal aspiration was observed only in the advanced stages of the disease 4 . In this paper we used Fiberoptic Endoscopic Evaluation of Swallowing (FEES) 8 , which is an excellent method to analyze the pharyngeal phase of swallowing, identifying posterior oral spillage, pharyngeal residue, penetration, and aspiration.…”

“…In the descriptions of this disease in the literature, there are studies on swallowing which investigate the characteristics of the oral and pharyngeal phases using videofluoroscopy (VFD) and fiberoptic endoscopic evaluation of swallowing (FEES), as well as the risks of aspiration associated with bronchopulmonary infections, airway obstruction, dehydration, and malnutrition 4,5 .…”

A Standard of Care for Huntington’s Disease: A Patient and Family Perspective

“…The green shoots of change in the profile of HD over the last three decades, which have brought about care homes like the excellent one that Michael Wooldridge runs, have also brought challenges; not least the [1][2][3][4][5][6][7] could affect the HD community in more ways than the obvious provision of accumulated knowledge to the health services.…”