In Amyotrophic Lateral Sclerosis, dysphagia has a high incidence. With deterioration of swallowing function, percutaneous endoscopic gastrostomy (PEG) tube is recommended to ensure sufficient and safe oral intake. Dysphagia and PEG placement alter quality of life (QoL). However, QoL and attitudes toward PEG remain largely unexplored. The purpose of this study is to monitor the swallowing function in relationship to QoL and attitudes toward PEG tube insertion and feeding.
18 years of age or older
diagnosis of ALS
follow-up at University Medical Centre Ljubljana, Division of Neurophysiology, every 3 months
be able to visit the study site for in-person procedures every 3 months
co-existing illness or disorder that could influence the swallowing function independently of the ALS diagnosis
clinically significant cognitive deterioration or dementia at enrollment, as determined by the ALS study neurologist
signs of probable cognitive deterioration or dementia at enrollment
The study will include adult ALS patients able to visit the study site for in-person procedures every 3 months, and their close relatives.
Cohort
Prospective
Iowa Oral Performance Instrument (IOPI)
Diagnostic testIOPI measures the peak pressure of the tongue strength and endurance using a disposable bulb placed on a hard palate behind the front teeth or at the base of the tongue. The participants will be asked to press the bulb with their tongue with maximum pressure, three times. To measure the endurance, the participants will be asked to hold the pressure for as long as they can.
Mann Assessment of Swallowing Ability (MASA)
Diagnostic testMASA is a standard clinical swallowing assessment protocol including general patient examination, on-the-outside visible signs of oral preparatory and transportation phase and pharyngeal phase of swallowing function.
Fiberoptic Endoscopic Evaluation of Swallowing
Diagnostic testThe subjects will ingest up to 10 boluses ranging in thickness and volume (from thin to extremely-thick and a cookie).
Respiratory function measurements
Diagnostic testRespiratory function measurements will include forced vital capacity (FVC), maximum inspiratory pressure (MIP), and maximum expiratory pressure (MEP), all expressed in percentage of predicted values. Conventional methods of testing will be applied.
Penetration-Aspiration Scale (PAS) change
PAS is an 8-point scale used to determine swallowing safety. Levels 1 and 2 are considered safe, levels \>3 indicate penetration or aspiration and are considered unsafe.
Yale Residue Severity Rating Scale (YRSRS) change
YRSRS is a validated measure of swallowing efficiency using an image-based assessment to determine post-swallow pharyngeal residue: location and severity. It is assessed using a 5-point ordinal scale (from 1=0 % residue to 5=50 % residue).
Iowa Oral Performance Instrument (IOPI) change
IOPI is a device which measures tongue strength (in kilopascals; kPa) and endurance (in seconds) while the participant presses the bulb with the front or the base of their tongue to the hard palate.
Mann Assessment of Swallowing Ability (MASA) change
MASA is a standard clinical swallowing assessment protocol administered by the speech-language therapist. Total score ranges from 38-200. Lower score indicates higher impairment.
ALS Functional Rating Scale-Revised (ALSFRS-R) change
ALSFRS-R is a 12-tem ALS-specific assessment of global functioning using a five-point scale (0=not able, 4= normal ability), with 48 points representing normal function.
Functional Oral Intake Scale (FOIS) change
FOIS is a validated 7-point scale demonstrating an individual's ability of daily oral food intake (1=no oral intake, 7=total oral intake with no restrictions).
Dysphagia Outcome and Severity Scale (DOSS) change
DOSS is a 7-point scale that rates the functional severity of dysphagia based on objective assessment and provides information on diet recommendations, independence level and type of nutrition necessary (level 7=normal swallowing function, level 1=severe dysphagia).
BMI change
Body mass index (in kg/m\^2)
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University Rehabilitation Institute, Republic of Slovenia
Lead sponsor
University Medical Centre Ljubljana
Collaborator
