Inspiratory Muscle Training in Myotonic Dystrophy type 1: a pilot study.

Completed

Conditions: 10028396
Myotonic Dystrophy type 1

Registration Number: NL-OMON50107

Lead Sponsor: longziekten / centrum voor thuisbeademing

Brief Summary: Not available

Detailed Description: Not available

Recruitment & Eligibility

Status: Completed

Sex: Not specified

Target Recruitment: 10

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

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, decompensatio cordis
-known heart failure, defined as left ventricular ejection fraction of < 50% of
predicted.

Study & Design

Study Type: Observational non invasive

Study Design: Not specified

Primary Outcome Measures

Name	Time	Method
<p>Difference in inspiratory muscle strength and endurance capacity after 12 weeks<br /><br>IMT program compared with baseline. </p><br>

Secondary Outcome Measures

Name	Time	Method
<p>Additional effects of IMT will be investigated on symptoms (using<br /><br>questionnaires about cough, dyspnea, fatigue, quality of life), longvolumes<br /><br>using spirometry, gas-exchange during the night.<br /><br>Also effects on the diaphragm will be measured using ultrasound and EMG. </p><br>