ABSTRACT
INTRODUCTION: The Swallowing Quality of Life instrument (SWAL-QOL) is a patient-reported outcome measure of swallowing-related quality of life (SR-QoL). Its psychometric properties in oculopharyngeal muscular dystrophy (OPMD) are not known. METHODS: We administered the SWAL-QOL to U.S. OPMD Registry participants. We described SR-QoL profiles and assessed reliability and validity. RESULTS: The mean composite score in 113 individuals with OPMD was 54.4 ± 20.7, indicating moderate impairment. Severe impairments were observed in eating duration, burden, and fatigue scales. Internal consistency reliability of all scales was found to be satisfactory, and 9 of 10 scales demonstrated adequate test-retest reliability. Data confirmed 86% of hypotheses, supporting construct validity. The SWAL-QOL limitations in OPMD include: floor/ceiling effects in 7 of 10 scales and low specificity of sleep, fatigue, and communication scales for dysphagia. CONCLUSIONS: SR-QoL is reduced in OPMD. Given several limitations of the SWAL-QOL, development of an improved dysphagia-specific QoL instrument for OPMD is warranted. Muscle Nerve 56: 28-35, 2017.
Subject(s)
Deglutition Disorders/etiology , Deglutition Disorders/psychology , Muscular Dystrophy, Oculopharyngeal/complications , Psychometrics , Quality of Life , Aged , Cross-Sectional Studies , Female , Humans , Male , Middle Aged , Reproducibility of Results , Retrospective Studies , Severity of Illness Index , United StatesABSTRACT
INTRODUCTION: Despite multiple studies reporting marked benefit of botulinum toxin (BTX) for treatment of cricopharyngeal dysphagia, little is known about its safety for this indication. We examined the safety of cricopharyngeal BTX for dysphagia in oculopharyngeal muscular dystrophy (OPMD). METHODS: We reviewed records of patients with OPMD who received cricopharyngeal BTX. RESULTS: Twenty-four patients underwent 66 procedures. Overall adverse event frequency was 44%. The most common adverse events were dysphonia (24%) and worsened dysphagia (14%). Logistic regression demonstrated that dose was a significant predictor of worsened dysphagia (P = 0.036) and of the composite event of dysphonia or worsened dysphagia (P = 0.009). There was a nonsignificant trend for dose as a predictor of dysphonia (P = 0.073). 59% of procedures were associated with symptomatic improvement. CONCLUSIONS: While BTX appears to be beneficial for treatment of dysphagia in OPMD, caution is warranted when injecting the cricopharyngeus muscle due to dose-related risk of dysphonia or worsened dysphagia.
