Noninvasive Home Mechanical Ventilation in Adult Myotonic Dystrophy Type 1: A Systematic Review

Introduction: Chronic hypercapnic respiratory failure induces considerable morbidity and mortality in patients with myotonic dystrophy type 1 (DM1). This study systematically reviews the effects of noninvasive home mechanical ventilation (HMV) on gas exchange, quality of life, survival, and compliance in DM1 patients. Methods: A systematic Medline and Embase search was performed (January 1995 to January 2020). Records were screened for eligibility criteria, data were extracted from included studies, and risk of bias was assessed. We present findings mainly using a narrative synthesis. Results: Twenty-eight relevant full-text articles were screened for eligibility criteria. Nine studies were included. Randomized controlled trials were not found. Studies had either an observational (n = 8) or interventional (n = 1) design. In the pooled data analysis, HMV showed to improve mean oxygen saturation with 4.8% and decreased mean carbon dioxide values with 3 mm Hg. Compliance varied widely between studies, from no use to more than 12 h per day. Quality of life was not studied extensively, but some studies reported positive effects of HMV on symptoms of chronic respiratory failure. HMV may improve survival in DM1 patients with chronic hypercapnic respiratory failure. Conclusion: This review shows that HMV can improve gas exchange and relieve symptoms with a possible survival benefit in DM1 patients with chronic hypercapnic respiratory failure. Future studies should focus on developing strategies to optimize the timing of HMV initiation and to promote compliance.

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Non invasive mechanical ventilation in myotonic dystrophy type 1? Hypoventilation versus quality of life perspective

Neuromuscular Disorders ◽

10.1016/j.nmd.2017.11.019 ◽

2018 ◽

Vol 28 (2) ◽

pp. 193

Author(s):

Giuseppe Fiorentino ◽

Antonio M. Esquinas

Keyword(s):

Quality Of Life ◽

Mechanical Ventilation ◽

Myotonic Dystrophy ◽

Invasive Mechanical Ventilation ◽

Myotonic Dystrophy Type 1 ◽

Myotonic Dystrophy Type ◽

Non Invasive

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Non-invasive mechanical ventilation in myotonic dystrophy type 1? Hypoventilation versus quality of life perspective

Neuromuscular Disorders ◽

10.1016/j.nmd.2017.12.004 ◽

2018 ◽

Vol 28 (2) ◽

pp. 194 ◽

Cited By ~ 1

Author(s):

Ghilas Boussaid ◽

Frédéric Lofaso ◽

David Orlikowski ◽

Dante Santos Brasil ◽

Stéphane Bahrami ◽

...

Keyword(s):

Quality Of Life ◽

Mechanical Ventilation ◽

Myotonic Dystrophy ◽

Invasive Mechanical Ventilation ◽

Myotonic Dystrophy Type 1 ◽

Myotonic Dystrophy Type ◽

Non Invasive

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Validation of the Hungarian version of the SRI Questionnaire

BMC Pulmonary Medicine ◽

10.1186/s12890-020-1171-5 ◽

2020 ◽

Vol 20 (1) ◽

Cited By ~ 1

Author(s):

Luca Valko ◽

Szabolcs Baglyas ◽

Laszlo Kunos ◽

Attila Terray-Horvath ◽

Andras Lorx ◽

...

Keyword(s):

Quality Of Life ◽

Mechanical Ventilation ◽

Respiratory Failure ◽

Chronic Respiratory Failure ◽

Life Questionnaire ◽

Home Mechanical Ventilation ◽

Life Tool ◽

Sf 36 ◽

Hungarian Version

Abstract Background Home mechanical ventilation is a reliable treatment for patients suffering from chronic respiratory failure, improving survival and quality of life. Prevalence has been increasing worldwide as a result of evolving technical possibilities, telemedicine and improving national guidelines. Projects to establish a national guideline and registry for patients treated with home mechanical ventilation are currently under way in Hungary and our aim was to validate a quality of life questionnaire suited for evaluation and follow up in this specific patient group. The Severe Respiratory Insufficiency Questionnaire (SRI) is a quality of life tool designed to evaluate patients receiving home mechanical ventilation and has been validated both in patient groups receiving invasive and noninvasive ventilation. Methods The Hungarian version of the SRI was created using the translation-backtranslation method, which was then tested for validity, viability and reliability in a cohort involving patients from three centers, receiving long-term home mechanical ventilation for chronic respiratory failure through an invasive or noninvasive interface. Patient data was collected (demographic data, lung function test, arterial blood gas, ventilation settings) and quality of life was measured with the previously validated SF-36 and newly created Hungarian SRI Questionnaires at two time points. Results One hundred four patients receiving home mechanical ventilation were enrolled. The time to complete the SRI Questionnaire was 8.6 (±3.1) minutes, 69.2% questionnaires were self-administered. Exploratory factor analysis explained 73.8% of the variance of the questionnaire, but resulted in 13 scales. We found correlations between the SRI subscale scores to corresponding scales of the previously validated general quality of life survey SF-36. The Cronbach alpha coefficient was 0.928 for the Summary Scale of the SRI Questionnaire, proving high internal consistency. Reproducibility was high for most scales, resulting in a high overall correlation for the summary score (0.877, p < 0.001). Conclusions The Hungarian version of the SRI Questionnaire is a viable, valid, reliable and reproduceable quality of life tool applicable for patients treated with home mechanical ventilation.

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