Duchenne Muscular Dystrophy
Conditions
Keywords
respiratory muscles, tele rehabilitation
Brief summary
Dushenne muscular dystrophy (DMD), which is characterized by the deficiency of dystrophin protein, prevents the muscle from maintaining its normal activity, causing progressive damage to the heart, respiratory muscles and skeletal muscles. When studies on patients with DMD are examined in the literature, very few studies are found investigating the effectiveness of pure respiratory muscle training, while no studies are found investigating the effectiveness of functional respiratory muscle training.
Interventions
OTHERRespiratory Muscle Training
Respiratory Muscle Training with Telerehabilitation
Sponsors
Izmir Katip Celebi University
Study design
Allocation
RANDOMIZED
Intervention model
FACTORIAL
Primary purpose
TREATMENT
Masking
TRIPLE (Subject, Investigator, Outcomes Assessor)
Eligibility
Sex/Gender
MALE
Age
5 Years to 17 Years
Healthy volunteers
No
Inclusion criteria
1. Being between the ages of 5-17 2. Ambulate 3. Having a diagnosis of DMD based on the presence of clinical symptoms, genetic testing or muscle biopsy results 4. Having the ability to perform respiratory function tests 5. Being able to cooperate with the physiotherapist
Exclusion criteria
1. Having severe upper extremity contracture or serious systemic disease that would prevent our evaluation 2. Having a respiratory tract infection in the last 3 months 3. Having had an injury or surgery on both upper extremities in the last 6 months
Design outcomes
Primary
| Measure | Time frame | Description |
|---|---|---|
| Respiratory Muscle Strenght Test | eight week | Maximal inspiratory pressure (MIP) and maximal expiratory pressure (MEP) measurements were used to assess respiratory muscles. MIP and MEP represent the intraoral pressures measured under closed airway conditions during maximal inspiration and expiration, respectively. In this study, these measurements were performed using an intraoral pressure measurement device (Pony FX, COSMED Inc., Rome, Italy) to determine respiratory muscle strength. During the test, for MIP measurement, participants were instructed to perform a maximal inspiration for 1-3 seconds following a maximal expiration while the airway was occluded with a valve. For MEP measurement, after a maximal inspiration, the airway was occluded and participants were asked to perform a maximal expiration for 1-3 seconds. Among the three trials conducted for each parameter, the highest value was recorded. |
| Respiratory Function Test | eight week | The measurements were performed using a portable spirometer. In the spirometric assessment, forced expiratory volume in one second (FEV₁), forced vital capacity (FVC), the FEV₁/FVC ratio, and peak expiratory flow (PEF) parameters were examined, and these values were recorded as percentages of the predicted values according to age, height, body weight, and sex. |
Secondary
| Measure | Time frame | Description |
|---|---|---|
| Modified Medical Research Council (mMRC) Dyspnea Scale | eight weeks | This scale is a categorical instrument scored from 0 to 4 that assesses the level of breathlessness experienced by patients during daily life activities. Participants responded by selecting one of the five statements that best reflected the severity of their dyspnea. |
| Six-Minute Walk Test | eight weeks | The six-minute walk test, frequently used to evaluate physical functional capacity and endurance in neuromuscular diseases, is a submaximal test that measures the walking distance covered by patients over six minutes on a hard surface within a 30-meter corridor. Participants were instructed to walk at their own pace, with rest permitted during the six-minute period, and the total distance walked was recorded in meters. |
Other
| Measure | Time frame | Description |
|---|---|---|
| Brooke Scale | Baseline | Upper extremity functions were scored on a scale ranging from 1 (able to abduct arms in a full circle) to 6 (no functional use of the hands). |
| Vignos Scale | Baseline | This scale was developed to classify lower extremity functions. Lower extremity functions were scored from 1 (able to walk and climb stairs without assistance) to 10 (confined to bed). |
Countries
Turkey (Türkiye)
Outcome results
None listed