Myotonic Dystrophy type 1
Conditions
Interventions
Sponsors
Eligibility
Inclusion criteria
Inclusion criteria: In order to be eligible to participate in this study, a subject must meet all of the following criteria: - Diagnosed with DM1 (based on DNA features). - Age = 18 years old. - Reduced PImax and vital capacity (both <80% of predicted). - Able to provide feedback.
Exclusion criteria
Exclusion criteria: A potential subject who meets any of the following criteria will be excluded from participation in this study: - Need or indication for HMV (daytime hypercapnia, pCO2 = 6.0 kPa) - Inability to adequately use a POWERbreathe, for example air leak during maneuvers. - Inability to communicate or answer questionnaires. - Vital capacity <25% of predicted. - Clinically unstable, for example pneumonia
Design outcomes
Primary
| Measure | Time frame |
|---|---|
| inspiratory muscle strenght and inspiratory endurance capacity after 12 weeks of training. | — |
Secondary
| Measure | Time frame |
|---|---|
| Symptoms (using questionnaires about cough, dyspnea, quality of life, fatigue), lung volumes, gas-exchange during the nigth, effects on diaphragm using ultrasound and EMG. | — |
Contacts
UMCG