Summary of the Clinical Practice Guideline

Article Citation

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

Hamilton, A., Heemskerk, A. -W., et al. (2012).
Degenerative Neurological and Neuromuscular Disease, 2(1), 45-53.
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European Huntington's Disease Network Standards of Care Speech and Language Therapist Group

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Article Details

Description

This guideline provides recommendations for speech-language pathologists on the assessment and treatment of swallowing disorders in individuals with Huntington's disease.

Recommendations from This Guideline

What are Recommendations?

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Assessment

During mid-stage Huntington's disease, "assessment and re-assessment should be a regular feature ... and will include bedside clinical evaluation, imaging and biofeedback assessments as appropriate" (p. 50).

Keywords: Timing, Assessment of Clinical Features (e.g. Gag Reflex), Imaging Assessment (Not Specified), Huntington’s Disease

Instrumental assessment (e.g., videofluoroscopy or fiberoptic endoscopic evaluation of swallowing) may or may not be used depending on the clinical judgment of the speech-language pathologist. Although cervical auscultation and pulse oximetry have been used to evaluate dysphagia in other neurological populations, evidence is inconsistent on the reliability of these methods. 

Keywords: Endoscopy, Fluoroscopy, Cervical Auscultation, Huntington’s Disease, Pulse Oximetry

The following information should be collected during dysphagia assessment for individuals with Huntington's disease:
  • medical history, including current medications; 
  • feeding/swallowing and dietary history;
  • patient-specific information;
  • "oro-facial examination, including cranial nerve assessment;
  • generalized motor skills, including co-ordination, posture and tone;
  • respiratory status; 
  • secretion management; 
  • oral hygiene and dental health;
  • effects of emotional status, mood and behavior;
  • cognitive status/factors; 
  • alertness levels;
  • communication status;
  • nutrition and hydration;  [and]
  • ability to participate and co-operate" (p. 48)

Keywords: Huntington’s Disease

Treatment

During end-stage Huntington's disease (HD), the following management approaches are recommended:
  • maintenance of oral hygiene
  • continued use of exercises/strategies for swallow facilitation or stimulation, dietary modifications, compensatory swallowing techniques, postural changes, and safe swallow maneuvers, with regular review 
  • caregiver education, specifically including feeding techniques, "food placement, dietary modifications, positioning and postures, management of behavioral and environmental factors, [and] management of choking" (p. 51)
  • monitoring of weight, nutrition, hydration, aspiration and pneumonia; if there are changes in risk in such areas, initiation of multidisciplinary discussion and decision-making on alternative management (in accordance with the wishes of the individual with HD, "if this discussion has taken place with the individual with HD at an earlier stage when the ability to understand the options and the implications was clear" [p. 51])

Keywords: Timing, Dysphagia, Huntington's disease, Adults, Diet Modification, Education/Training (includes Oral Hygiene), Huntington’s Disease, Oral Motor Treatments (includes Shaker Exercises), Postural Techniques/Maneuvers, Sensory Stimulation

During mid-stage Huntington's disease (HD), the following management approaches are recommended:
  • Providing safe swallow recommendations/education in multiple formats (verbal and written), and "limitations should be explicit" (p. 50); safe swallow recommendations or "eating and drinking plan may also be presented in picture/accessible information format to facilitate the eating/swallowing routine" (p. 50).
  • Training caregivers.
  • Maintaining or improving oro-motor function which may include "breathing exercises, oral and chewing exercises and individualizing swallow sequence, for example, cough postswallow" (p. 50).
  • Tailoring treatment plan to the individual's cognition, alertness, behaviors, and receptive abilities.
  • Environmental modification and adaptive equipment.
  • Diet modification via food/liquid texture/consistency/temperature/viscosity, with "ongoing systematic assessment in order to increase the safety and efficiency of the swallow" (p. 50).
  • Modifying placement of bolus.
  • Postural changes, working jointly with physical therapy, to facilitate safe swallow, to include chin tuck positioning for airway protection.
  • Discussing and decision-making with the speech-language pathologist (as part of the multidisciplinary team, including the individual with HD and his/her family) regarding non-oral means of nutrition and hydration, "while the patient's cognitive abilities allow full participation" (p. 50-51). 

Keywords: Provider (SLP/Caregiver), Dysphagia, Huntington's disease, Adults, Diet Modification, Education/Training (includes Oral Hygiene), Environmental Modification, Huntington’s Disease, Oral Motor Treatments (includes Shaker Exercises), Postural Techniques/Maneuvers, Sensory Stimulation

In the early stages of Huntington's disease (HD), "therapeutic interventions in the form of oral/respiratory/phonatory exercises may ... be introduced if appropriate" (p. 49). Recommended early-stage HD dysphagia management approaches, each of which should be prioritized according to the judgment of the speech-language pathologist, include:
  • increasing awareness and self-monitoring of swallowing;
  • providing education on how mood changes, anxiety, depression or stress may affect swallowing or appetite;
  • providing education on potentially difficult foods/textures;
  • addressing anxiety/embarrassment of choking/drooling/messiness while eating; and
  • strategies such as environmental modification, energy conservation before mealtimes, and/or postural changes.

Keywords: Timing, Education/Training (includes Oral Hygiene), Environmental Modification, Huntington’s Disease, Postural Techniques/Maneuvers

Service Delivery

During end-stage Huntington's disease (HD), the following management approaches are recommended:
  • maintenance of oral hygiene
  • continued use of exercises/strategies for swallow facilitation or stimulation, dietary modifications, compensatory swallowing techniques, postural changes, and safe swallow maneuvers, with regular review 
  • caregiver education, specifically including feeding techniques, "food placement, dietary modifications, positioning and postures, management of behavioral and environmental factors, [and] management of choking" (p. 51)
  • monitoring of weight, nutrition, hydration, aspiration and pneumonia; if there are changes in risk in such areas, initiation of multidisciplinary discussion and decision-making on alternative management (in accordance with the wishes of the individual with HD, "if this discussion has taken place with the individual with HD at an earlier stage when the ability to understand the options and the implications was clear" [p. 51])

Keywords: Timing, Dysphagia, Huntington's disease, Adults, Diet Modification, Education/Training (includes Oral Hygiene), Huntington’s Disease, Oral Motor Treatments (includes Shaker Exercises), Postural Techniques/Maneuvers, Sensory Stimulation

During mid-stage Huntington's disease (HD), the following management approaches are recommended:
  • Providing safe swallow recommendations/education in multiple formats (verbal and written), and "limitations should be explicit" (p. 50); safe swallow recommendations or "eating and drinking plan may also be presented in picture/accessible information format to facilitate the eating/swallowing routine" (p. 50).
  • Training caregivers.
  • Maintaining or improving oro-motor function which may include "breathing exercises, oral and chewing exercises and individualizing swallow sequence, for example, cough postswallow" (p. 50).
  • Tailoring treatment plan to the individual's cognition, alertness, behaviors, and receptive abilities.
  • Environmental modification and adaptive equipment.
  • Diet modification via food/liquid texture/consistency/temperature/viscosity, with "ongoing systematic assessment in order to increase the safety and efficiency of the swallow" (p. 50).
  • Modifying placement of bolus.
  • Postural changes, working jointly with physical therapy, to facilitate safe swallow, to include chin tuck positioning for airway protection.
  • Discussing and decision-making with the speech-language pathologist (as part of the multidisciplinary team, including the individual with HD and his/her family) regarding non-oral means of nutrition and hydration, "while the patient's cognitive abilities allow full participation" (p. 50-51). 

Keywords: Provider (SLP/Caregiver), Dysphagia, Huntington's disease, Adults, Diet Modification, Education/Training (includes Oral Hygiene), Environmental Modification, Huntington’s Disease, Oral Motor Treatments (includes Shaker Exercises), Postural Techniques/Maneuvers, Sensory Stimulation

During mid-stage Huntington's disease, "assessment and re-assessment should be a regular feature ... and will include bedside clinical evaluation, imaging and biofeedback assessments as appropriate" (p. 50).

Keywords: Timing, Assessment of Clinical Features (e.g. Gag Reflex), Imaging Assessment (Not Specified), Huntington’s Disease

In the early stages of Huntington's disease (HD), "therapeutic interventions in the form of oral/respiratory/phonatory exercises may ... be introduced if appropriate" (p. 49). Recommended early-stage HD dysphagia management approaches, each of which should be prioritized according to the judgment of the speech-language pathologist, include:
  • increasing awareness and self-monitoring of swallowing;
  • providing education on how mood changes, anxiety, depression or stress may affect swallowing or appetite;
  • providing education on potentially difficult foods/textures;
  • addressing anxiety/embarrassment of choking/drooling/messiness while eating; and
  • strategies such as environmental modification, energy conservation before mealtimes, and/or postural changes.

Keywords: Timing, Education/Training (includes Oral Hygiene), Environmental Modification, Huntington’s Disease, Postural Techniques/Maneuvers

Early referral to speech-language pathology should be provided for individuals with Huntington's disease, given multifactorial nature of dysphagia management (e.g., considering cognitive, behavioral changes), the need for baseline data, and the need to provide patient education. Referral should be discussed with individuals identified as at risk for Huntington's disease, in order to provide education.

Keywords: Timing, Huntington’s Disease

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Assessment

During mid-stage Huntington's disease, "assessment and re-assessment should be a regular feature ... and will include bedside clinical evaluation, imaging and biofeedback assessments as appropriate" (p. 50).

Keywords: Timing, Assessment of Clinical Features (e.g. Gag Reflex), Imaging Assessment (Not Specified), Huntington’s Disease

Instrumental assessment (e.g., videofluoroscopy or fiberoptic endoscopic evaluation of swallowing) may or may not be used depending on the clinical judgment of the speech-language pathologist. Although cervical auscultation and pulse oximetry have been used to evaluate dysphagia in other neurological populations, evidence is inconsistent on the reliability of these methods. 

Keywords: Endoscopy, Fluoroscopy, Cervical Auscultation, Huntington’s Disease, Pulse Oximetry

The following information should be collected during dysphagia assessment for individuals with Huntington's disease:
  • medical history, including current medications; 
  • feeding/swallowing and dietary history;
  • patient-specific information;
  • "oro-facial examination, including cranial nerve assessment;
  • generalized motor skills, including co-ordination, posture and tone;
  • respiratory status; 
  • secretion management; 
  • oral hygiene and dental health;
  • effects of emotional status, mood and behavior;
  • cognitive status/factors; 
  • alertness levels;
  • communication status;
  • nutrition and hydration;  [and]
  • ability to participate and co-operate" (p. 48)

Keywords: Huntington’s Disease

Treatment

During end-stage Huntington's disease (HD), the following management approaches are recommended:
  • maintenance of oral hygiene
  • continued use of exercises/strategies for swallow facilitation or stimulation, dietary modifications, compensatory swallowing techniques, postural changes, and safe swallow maneuvers, with regular review 
  • caregiver education, specifically including feeding techniques, "food placement, dietary modifications, positioning and postures, management of behavioral and environmental factors, [and] management of choking" (p. 51)
  • monitoring of weight, nutrition, hydration, aspiration and pneumonia; if there are changes in risk in such areas, initiation of multidisciplinary discussion and decision-making on alternative management (in accordance with the wishes of the individual with HD, "if this discussion has taken place with the individual with HD at an earlier stage when the ability to understand the options and the implications was clear" [p. 51])

Keywords: Timing, Dysphagia, Huntington's disease, Adults, Diet Modification, Education/Training (includes Oral Hygiene), Huntington’s Disease, Oral Motor Treatments (includes Shaker Exercises), Postural Techniques/Maneuvers, Sensory Stimulation

During mid-stage Huntington's disease (HD), the following management approaches are recommended:
  • Providing safe swallow recommendations/education in multiple formats (verbal and written), and "limitations should be explicit" (p. 50); safe swallow recommendations or "eating and drinking plan may also be presented in picture/accessible information format to facilitate the eating/swallowing routine" (p. 50).
  • Training caregivers.
  • Maintaining or improving oro-motor function which may include "breathing exercises, oral and chewing exercises and individualizing swallow sequence, for example, cough postswallow" (p. 50).
  • Tailoring treatment plan to the individual's cognition, alertness, behaviors, and receptive abilities.
  • Environmental modification and adaptive equipment.
  • Diet modification via food/liquid texture/consistency/temperature/viscosity, with "ongoing systematic assessment in order to increase the safety and efficiency of the swallow" (p. 50).
  • Modifying placement of bolus.
  • Postural changes, working jointly with physical therapy, to facilitate safe swallow, to include chin tuck positioning for airway protection.
  • Discussing and decision-making with the speech-language pathologist (as part of the multidisciplinary team, including the individual with HD and his/her family) regarding non-oral means of nutrition and hydration, "while the patient's cognitive abilities allow full participation" (p. 50-51). 

Keywords: Provider (SLP/Caregiver), Dysphagia, Huntington's disease, Adults, Diet Modification, Education/Training (includes Oral Hygiene), Environmental Modification, Huntington’s Disease, Oral Motor Treatments (includes Shaker Exercises), Postural Techniques/Maneuvers, Sensory Stimulation

In the early stages of Huntington's disease (HD), "therapeutic interventions in the form of oral/respiratory/phonatory exercises may ... be introduced if appropriate" (p. 49). Recommended early-stage HD dysphagia management approaches, each of which should be prioritized according to the judgment of the speech-language pathologist, include:
  • increasing awareness and self-monitoring of swallowing;
  • providing education on how mood changes, anxiety, depression or stress may affect swallowing or appetite;
  • providing education on potentially difficult foods/textures;
  • addressing anxiety/embarrassment of choking/drooling/messiness while eating; and
  • strategies such as environmental modification, energy conservation before mealtimes, and/or postural changes.

Keywords: Timing, Education/Training (includes Oral Hygiene), Environmental Modification, Huntington’s Disease, Postural Techniques/Maneuvers

Service Delivery

During end-stage Huntington's disease (HD), the following management approaches are recommended:
  • maintenance of oral hygiene
  • continued use of exercises/strategies for swallow facilitation or stimulation, dietary modifications, compensatory swallowing techniques, postural changes, and safe swallow maneuvers, with regular review 
  • caregiver education, specifically including feeding techniques, "food placement, dietary modifications, positioning and postures, management of behavioral and environmental factors, [and] management of choking" (p. 51)
  • monitoring of weight, nutrition, hydration, aspiration and pneumonia; if there are changes in risk in such areas, initiation of multidisciplinary discussion and decision-making on alternative management (in accordance with the wishes of the individual with HD, "if this discussion has taken place with the individual with HD at an earlier stage when the ability to understand the options and the implications was clear" [p. 51])

Keywords: Timing, Dysphagia, Huntington's disease, Adults, Diet Modification, Education/Training (includes Oral Hygiene), Huntington’s Disease, Oral Motor Treatments (includes Shaker Exercises), Postural Techniques/Maneuvers, Sensory Stimulation

During mid-stage Huntington's disease (HD), the following management approaches are recommended:
  • Providing safe swallow recommendations/education in multiple formats (verbal and written), and "limitations should be explicit" (p. 50); safe swallow recommendations or "eating and drinking plan may also be presented in picture/accessible information format to facilitate the eating/swallowing routine" (p. 50).
  • Training caregivers.
  • Maintaining or improving oro-motor function which may include "breathing exercises, oral and chewing exercises and individualizing swallow sequence, for example, cough postswallow" (p. 50).
  • Tailoring treatment plan to the individual's cognition, alertness, behaviors, and receptive abilities.
  • Environmental modification and adaptive equipment.
  • Diet modification via food/liquid texture/consistency/temperature/viscosity, with "ongoing systematic assessment in order to increase the safety and efficiency of the swallow" (p. 50).
  • Modifying placement of bolus.
  • Postural changes, working jointly with physical therapy, to facilitate safe swallow, to include chin tuck positioning for airway protection.
  • Discussing and decision-making with the speech-language pathologist (as part of the multidisciplinary team, including the individual with HD and his/her family) regarding non-oral means of nutrition and hydration, "while the patient's cognitive abilities allow full participation" (p. 50-51). 

Keywords: Provider (SLP/Caregiver), Dysphagia, Huntington's disease, Adults, Diet Modification, Education/Training (includes Oral Hygiene), Environmental Modification, Huntington’s Disease, Oral Motor Treatments (includes Shaker Exercises), Postural Techniques/Maneuvers, Sensory Stimulation

During mid-stage Huntington's disease, "assessment and re-assessment should be a regular feature ... and will include bedside clinical evaluation, imaging and biofeedback assessments as appropriate" (p. 50).

Keywords: Timing, Assessment of Clinical Features (e.g. Gag Reflex), Imaging Assessment (Not Specified), Huntington’s Disease

In the early stages of Huntington's disease (HD), "therapeutic interventions in the form of oral/respiratory/phonatory exercises may ... be introduced if appropriate" (p. 49). Recommended early-stage HD dysphagia management approaches, each of which should be prioritized according to the judgment of the speech-language pathologist, include:
  • increasing awareness and self-monitoring of swallowing;
  • providing education on how mood changes, anxiety, depression or stress may affect swallowing or appetite;
  • providing education on potentially difficult foods/textures;
  • addressing anxiety/embarrassment of choking/drooling/messiness while eating; and
  • strategies such as environmental modification, energy conservation before mealtimes, and/or postural changes.

Keywords: Timing, Education/Training (includes Oral Hygiene), Environmental Modification, Huntington’s Disease, Postural Techniques/Maneuvers

Early referral to speech-language pathology should be provided for individuals with Huntington's disease, given multifactorial nature of dysphagia management (e.g., considering cognitive, behavioral changes), the need for baseline data, and the need to provide patient education. Referral should be discussed with individuals identified as at risk for Huntington's disease, in order to provide education.

Keywords: Timing, Huntington’s Disease

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