Myotonic dystrophy type 1 Neuromuscular disorder
Conditions
Interventions
None listed
Sponsors
MUMC
Eligibility
Inclusion criteria
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
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
Design outcomes
Primary
| Measure | Time frame |
|---|---|
| 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
| Measure | Time frame |
|---|---|
| Secondary objectives are to define possible determinants for development of sleep disordered breathing (disease severity, disease duration, age, weight, lung function parameters). | — |
Contacts
Public ContactBettine Vosse
Maastricht University Medical Center
Outcome results
None listed