Myotonic Dystrophy type 1
Conditions
Interventions
None listed
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
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
Design outcomes
Primary
| Measure | Time frame |
|---|---|
| Difference in inspiratory muscle strength and endurance capacity after 12 weeks IMT program compared with baseline. | — |
Secondary
| Measure | Time frame |
|---|---|
| Additional effects of IMT will be investigated on symptoms (using questionnaires about cough, dyspnea, fatigue, quality of life), longvolumes using spirometry, gas-exchange during the night. Also effects on the diaphragm will be measured using ultrasound and EMG. | — |
Countries
Netherlands