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Effects of 1‐month withdrawal of ventilatory support in hypercapnic myotonic dystrophy type 1
Author(s) -
O'Donoghue Fergal J.,
Borel JeanChristian,
Dauvilliers Yves,
Levy Patrick,
Tamisier Renaud,
Pépin JeanLouis
Publication year - 2017
Publication title -
respirology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 1.857
H-Index - 85
eISSN - 1440-1843
pISSN - 1323-7799
DOI - 10.1111/resp.13068
Subject(s) - medicine , anesthesia , epworth sleepiness scale , polysomnography , arterial blood , ventilation (architecture) , myotonic dystrophy , respiratory minute volume , blood pressure , respiratory system , apnea , mechanical engineering , engineering
ABSTRACT Background and objective The benefits of domiciliary non‐invasive ventilation ( NIV ) in myotonic dystrophy type 1 ( DM1 ) are unclear. We sought to determine the effects of elective discontinuation of ventilatory support for 1 month in DM1 patients receiving NIV for chronic hypercapnic respiratory failure. Methods At baseline, 12 patients underwent polysomnography, and assessment of subjective (Epworth Sleepiness Scale) and objective (Oxford Sleep Resistance Test) sleepiness, fatigue (Fatigue Severity Scale), respiratory function including muscle strength, arterial blood gas ( ABG ), hypercapnic ventilatory response ( HCVR ), Blood Pressure, peripheral arterial tonometry ( PAT ) and pulse wave velocity ( PWV ). They also completed the SF36 . Testing was repeated (Visit 2) 1 month after elective cessation of NIV and again (Visit 3) 1 month after NIV reintroduction. Results No changes were seen in SF36 , sleepiness or fatigue, respiratory function, muscle strength nor HCVR . Likewise, there were no changes in Blood Pressure , PAT or PWV . Mean nocturnal SpO 2 deteriorated off NIV and improved on resumption (mean ± SD = 95.02 ± 1.90%, 92.23 ± 3.61% and 95.08 ± 2.28%, P = 0.006 change Visit 1 to Visit 2, 0.009 Visit 2 to Visit 3). Daytime PaCO 2 (arterial partial pressure of carbon dioxide) was 43.13 ± 4.20 mm Hg , 46.28 ± 2.25 mm Hg and 43.87 ± 2.85 mm Hg , P = 0.056 and 0.017 over the same intervals. Conclusion DM1 patients derive little benefit in symptoms or quality of life from NIV . Nocturnal and diurnal ventilatory functions deteriorate slightly off NIV for 1 month, but this does not appear to be due to changes in HCVR or respiratory function. HCVR changes may be of primary CNS origin given stability on or off NIV .