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Dysphagia-related quality of life in oculopharyngeal muscular dystrophy: Psychometric properties of the SWAL-QOL instrument.

ABSTRACT: INTRODUCTIONThe 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.METHODSWe administered the SWAL-QOL to U.S. OPMD Registry participants. We described SR-QoL profiles and assessed reliability and validity.RESULTSThe 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.CONCLUSIONSSR-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.

SUBMITTER: Youssof S

PROVIDER: S-EPMC5397376 | biostudies-literature | 2017 Jul

REPOSITORIES: biostudies-literature

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Dysphagia-related quality of life in oculopharyngeal muscular dystrophy: Psychometric properties of the SWAL-QOL instrument.

Youssof Sarah S Romero-Clark Carol C Warner Teddy T Plowman Emily E

Muscle & nerve 20170212 1

<h4>Introduction</h4>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.<h4>Methods</h4>We administered the SWAL-QOL to U.S. OPMD Registry participants. We described SR-QoL profiles and assessed reliability and validity.<h4>Results</h4>The mean composite score in 113 individuals with OPMD was 54.4 ± 20.7, indicating moderat ...[more]

PMID: 27759888

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Project description:BACKGROUND:Although dysphagia represents a hallmark manifestation of oculopharyngeal muscular dystrophy (OPMD), limited knowledge exists regarding the underlying nature of oropharyngeal swallowing impairments in this patient population. We aimed to delineate global pharyngeal dysphagia profiles in OPMD and identify the prevalence and physiologic associations of impairments in swallowing safety and efficiency. METHODS:Twenty-two individuals with OPMD completed a videofluoroscopic swallowing evaluation. Blinded raters completed validated scales of global dysphagia (dynamic imaging grade of swallowing toxicity, DIGEST), efficiency (normalized residue ratio scale, NRRS), and safety (penetration-aspiration scale, PAS). Degree of laryngeal vestibule closure and aspiration events were described. Descriptives and chi-squared analyses were conducted with alpha set at P < .05. KEY RESULTS:One hundred and thirty-four swallowing trials were analyzed. DIGEST scores revealed that 96% (n = 21) of participants demonstrated pharyngeal dysphagia (score >1). Presence of a cricopharyngeal bar was noted in 10 individuals. The predominant swallowing categorization across patients was safe and inefficient (51%) followed by unsafe and inefficient (32%). 77.3% demonstrated vallecular residue (NRRSv>0.07) and 90.1% piriform sinus residue (NRRSp > .20). 33% (n = 54) of swallows were unsafe (PAS>3) with 45 episodes of penetration and 9 episodes of aspiration. Aspiration occurred during the swallow in 100% of identified occurrences. Incomplete epiglottic inversion was associated with airway compromise and postswallow residue (P < .05). CONCLUSIONS & INFERENCES:These findings highlight the high prevalence of oropharyngeal swallowing impairments in both swallowing efficiency and safety. A high proportion of physiologic impairments in epiglottic inversion and laryngeal vestibule closure were noted that related to functional impairments in swallow safety and inefficiency.

Project description:IntroductionPressure ulcer-specific patient-reported outcome (PRO) instruments should be used to inform patient care and provide a strong evidence base for interventions aimed at preventing pressure ulcers. The aim was to carry out a comprehensive evaluation of the psychometric properties of a PRO instrument designed to assess symptoms and functional outcomes in patients at high-risk of developing pressure ulcers, the PU-QOL-P instrument.MethodsWe modified the original PU-QOL instrument to be suitable for patients at high risk of pressure ulcer development based on feedback from patients, specialist nurses and PRO methodologists. The modified PU-QOL-P instrument was administered to a sub-set of patients participating in the PRESSURE 2 trial. Patients completed PU-QOL-P and SF12 instruments at baseline, weeks 1 and 3, and 30 days post-treatment. We undertook psychometric evaluation of the modified PU-QOL-P to test scale targeting, scaling assumptions, reliability, validity and responsiveness.ResultsThe analysis sample consisted of 617 patients that completed both instruments at baseline. We found that the PU-QOL-P instrument, consisting of nine PU-specific outcomes: three symptom and six function scales, meets established criteria for reliability, construct validity, and responsiveness. Internal consistency reliability was high with all scale Cronbach alpha > 0.795 (range 0.795-0.970). The factor analysis mostly supported the six-function scale structure. Scaling assumptions were satisfied; all item-total correlations above 0.30. Convergent validity was confirmed by significant correlations between hypothesized scales as expected. PU-QOL-P scales were responsive to change: mean scale scores from baseline to 30 days post-treatment were statistically significant for all scales apart the daily activities scale (effect sizes ranged from moderate to high). As expected, worse symptoms and functioning was observed in patients who had a category 1 or 2 PU compared to patients who did not have a PU.ConclusionsThe PU-QOL-P provides a standardised method for assessing pressure ulcer-specific symptoms and functional outcomes for quantifying the benefits of associated interventions from the patient's perspective. It can be used in research with adults at risk of pressure ulcer development in all UK healthcare settings.

Project description:Small non-coding microRNAs (miRNAs) are involved in the regulation of mRNA stability. Their features, including high stability and secretion to biofluids, make them attractive as potential biomarkers for diverse pathologies. This is the first study reporting miRNA as potential biomarkers for oculopharyngeal muscular dystrophy (OPMD), an adult-onset myopathy. We hypothesized that miRNA that is differentially expressed in affected muscles from OPMD patients is secreted to biofluids and those miRNAs could be used as biomarkers for OPMD. We first identified candidate miRNAs from OPMD-affected muscles and from muscles from an OPMD mouse model using RNA sequencing. We then compared the OPMD-deregulated miRNAs to the literature and, subsequently, we selected a few candidates for expression studies in serum and saliva biofluids using qRT-PCR. We identified 126 miRNAs OPMD-deregulated in human muscles, but 36 deregulated miRNAs in mice only (pFDR < 0.05). Only 15 OPMD-deregulated miRNAs overlapped between the in humans and mouse studies. The majority of the OPMD-deregulated miRNAs showed opposite deregulation direction compared with known muscular dystrophies miRNAs (myoMirs), which are associated. In contrast, similar dysregulation direction was found for 13 miRNAs that are common between OPMD and aging muscles. A significant age-association (p < 0.05) was found for 17 OPMD-deregulated miRNAs (13.4%), whereas in controls, only six miRNAs (1.4%) showed a significant age-association, suggesting that miRNA expression in OPMD is highly age-associated. miRNA expression in biofluids revealed that OPMD-associated deregulation in saliva was similar to that in muscles, but not in serum. The same as in muscle, miRNA expression levels in saliva were also found to be associated with age (p < 0.05). Moreover, the majority of OPMD-miRNAs were found to be associated with dysphagia as an initial symptom. We suggest that levels of specific miRNAs in saliva can mark muscle degeneration in general and dysphagia in OPMD.

Dataset Information

Dysphagia-related quality of life in oculopharyngeal muscular dystrophy: Psychometric properties of the SWAL-QOL instrument.

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Dysphagia-related quality of life in oculopharyngeal muscular dystrophy: Psychometric properties of the SWAL-QOL instrument.

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