Investigation of the Effects of Functional Inspiratory Muscle Training Applied With Tele-Rehabilitation on Respiratory and Functional Parameters and Quality of Life in Children With Duchenne Muscular Dystrophy: Randomized Controlled Study

Izmir Katip Celebi University40 enrolled

Overview

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.

Study Type

INTERVENTIONAL

Allocation

RANDOMIZED

Purpose

TREATMENT

Masking

TRIPLE

Enrollment

Conditions

Duchenne Muscular Dystrophy

Interventions

Respiratory Muscle Training

Eligibility

Sex: MALEMin age: 5 YearsMax age: 17 Years

Medical Language ↔ Plain English

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

Outcomes

Primary Outcomes

Respiratory Muscle Strenght Test

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.

Time frame: eight week

Respiratory Function Test

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.

Time frame: eight week

Secondary Outcomes

Modified Medical Research Council (mMRC) Dyspnea Scale

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.

Time frame: eight weeks

Six-Minute Walk Test

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.