Differential Response to non-invasive ventilation in myotonic dystrophy
Recruiting
- Conditions
- Myotonic dystrophy type 1Neuromuscular disorder
- Registration Number
- NL-OMON23487
- Lead Sponsor
- MUMC
- Brief Summary
Not available
- Detailed Description
Not available
Recruitment & Eligibility
- Status
- Recruiting
- Sex
- Not specified
- Target Recruitment
- 80
Inclusion Criteria
diagnosis myotonic dystrophy type 1
- age >18 years
- daytime pCO2 >6.0 kPa and complaints of alveolar hypoventilation OR daytime pCO2 >7.0 kPa without complaints of alveolar hypoventilation
- the general criteria regarding home mechanical ventilation ('dutch veldnorm 'Chronische beademing' versie 1.0, 2012) are met
Exclusion Criteria
- previous episode of non-invasive home mechanical ventilation or CPAP (continuous positive airway pressure) therapy in de last 5 years
- other medical condition leading to hypercapnia
- severe heart failure, NYHA IV
- instable angina pectoris
Study & Design
- Study Type
- Observational non invasive
- Study Design
- Not specified
- Primary Outcome Measures
Name Time Method The primary objective is to investigate the effect of NIV on different factors including gas exchange, lung function, daily functioning, fatigue and sleepiness, sleep quality, quality of life and compliance to the therapy.
- Secondary Outcome Measures
Name Time Method Secondary objectives are to define possible determinants for development of sleep disordered breathing (disease severity, disease duration, age, weight, lung function parameters).