Malarvizhi D1, Hariharan S2
1Professor, SRM College of Physiotherapy, SRM Institute of Science and Technology, Faculty of Medical and Health Sciences, Kancheepuram District, Kattankulathur, Chennai 603203, Tamil Nadu, India.
2BPT IV Year, SRM College of Physiotherapy, SRM Institute of Science and Technology, Faculty of Medical and Health Sciences, Kancheepuram District, Kattankulathur, Chennai 603203, Tamil Nadu, India.
*Corresponding Author E-mail: malarvid@srmist.edu.in, hs3034@srmist.edu.in
ABSTRACT:
Background: Individual with Duchenne Muscular Dystrophy are more prone for respiratory dysfunction. And thus the leading cause for death DMD. Objective: To find the effectiveness of inspiratory muscle training using ULTRABREATHE on pulmonary function in Duchenne Muscular Dystrophy. Methodology: The quasi experimental study was done in 5 Duchenne Muscular Dystrophy children as one group of age ranging from 9 to 16 years in wheelchair bound stage and they have been given for an ULTRABREATHE as an inspiratory muscle trainer for duration of six weeks. Outcome Measures: Pulmonary Function Test was analyzed using spirometry.
Results: Statistical analysis shows, the mean and standard deviation value is 1.5380 and .84736 in pretest and the mean and standard deviation is 1.71 and .88752 with respect to tidal volume which shows significant improvement at p value> 0.038, whereas the other components like Duration of Inspiration, Total Lung Volume/Inspiration Time, Total Lung Volume, Inspiratory Capacity, Inspiration Time/Respiration Time, Forced Vital Capacity and Forced Expiratory Volume in One second (FEV1) not showed an significant improvement. Conclusion: The study concluded that the Effectiveness of inspiratory muscle training using ULTRABREATHE on pulmonary function in Duchenne Muscular Dystrophy children showed improvement only in Tidal Volume whereas all other components not showed a significant improvement.
KEYWORDS: Duchenne Muscular Dystrophy, Inspiratory Muscle training, Respiratory Muscle Traininer, Ultrabreathe, Pulmonary Function, Muscular Dystrophy Children.
INTRODUCTION:
Duchenne Muscular Dystrophy (DMD) disease occurs 1 in every 3500 live male births due to recessive X-linked disorder1. Its Manifestation begins between the age of 3- 5 years and the severity greatly witnessed is proximal muscle weakness and pseudohypertrophy of calf muscle.
As the age progresses there is a gradual loss of ambulation, they become wheelchair bound and are expected to live only up to early twenties. The children affected with DMD tends to be obese, have difficulty in maintaining a straight posture or produce any movement and mortality occurs as a result of respiratory failure.
Dystrophin gene is used to synthesize the protein called dystrophin, which plays vital role in contraction of muscles in our body.
The dysfunction in dystrophin synthesis results in continuous succession of damage and repair of muscles which eventually results in the replacement of cancroid tissue in the muscles. This tissue is responsible for gradual muscle atrophy and degeneration and is notably observed at 3–4 years2. The children affected by DMD finds difficulty in walking, getting up from the ground easily and frequent falls may occur. The clinical signs include waddling gait, calf pseudohypertrophy, and increased curvature of lumbar spine on sitting disappears as they sit, abnormal arrangement of teeth, hypertrophied tongue, and retrognathic facial morphology and Gower’s sign3,4. Elevated levels of creatinine kinase and the absence of dystrophin that is identified in muscle biopsy and genetic coding severs as a confirmatory test for DMD5,6. Corticosteroid which has been widely prescribed for the children with DMD has adverse effect child weight and their behavior. Since studies on DMD shows that appropriate physiotherapy treatment initiated before the age of 5 has prolonged the stage of ambulation for 1-3 years, it becomes important to implement physiotherapy as a part of their treatment regime, as it has less side effects and shown to be advantageous over corticosteroids7,8,9. Mortality in DMD patients occurs as a result of Respiratory Failure and cardiac dysfunction which is due to the inability to deliver oxygen to the blood and removal of CO2 from the tissues1. Ventilatory failure in DMD increases due to respiratory load which decreases the chest wall compliance, airway obstruction increases the airway resistance which in turn weakness the respiratory muscle. Due to recurrent aspiration, The respiratory function of lungs fails causing hypoventilation, the pattern of thoracoabdominal gets altered, and they suffered from breathing disorder10,11. The gastro-oesophageal reflux causes a recurrent aspiration of food which may occur either after swallowing or at later stages of deglutition.The prolonged gradual weakness of the respiratory muscles leads to altered thoracic cage mechanisms and this prolongation leads to plastic adaptation, which also occurs due to the fusion of costal joints and thus the chest wall becoming stiff. Prolonged muscle inactivity leads to muscle atrophy, stiffness and degenerative changes in bones and joints. The plastic adaptation of the chest wall leads to kyphoscoliosis which further hinders respiration. The direct consequences of Upper airway (bulbar) musculature in DMD patient is because of swallowing impairments, speech impediments and during inspiration they have ensuing significant airway obstruction. The Work of Breathing increases as the load over the muscles of respiration increases12,13. The respiratory failure in DMD is due to increased Work of Breathing, and the incompetency of respiratory muscles to meet respiratory needs. Hence the affected respiratory muscle has to be improved through strength training and tolerance. Patients with Muscular disease needs a respiratory muscle training device with a resistive load14,15,16.
The goal of the study is to show an effect on the pulmonary function in DMD patients by training the inspiratory muscles. In this study, the inspiratory muscles are trained by an effective inspiratory muscle trainer named Ultrabreathe. It works on the principle of resistance. As the subject inhale, the resistance created makes the respiratory muscle work harder, as they work more stronger and their breathing capacity will increase. It can be gradually adjusted to provide more resistance. It gives easy and convenient means of increasing the strength and the endurance of the respiratory muscles.
The study was conducted with the permission of Departmental Ethical Committee. A quasi-experimental study was done with convenient sampling on 5 wheel-chair depended boys diagnosed with DMD of age between 9 -16 years and their total lung capacity ranges from 0.9 l to 4.2 l were included. And those boys with cardiac abnormalities, lower respiratory tract infections, children under steroid therapy and severe Scoliosis were excluded. Followed by the spirometer analysis of lung volume17 their inspiratory muscles were trained using Ultrabreathe device with minimal resistance set by adjusting the cap. They were instructed to inhale to the maximum for three times a session with rest time of 20-30 seconds in between. The patient is expected to maintain a upright sitting posture throughout the procedure. The mouthpiece of Ultrabreathe were sterilized at the end of each session. At the end of six weeks spirometer analysis were conducted to assess their lung volume. The patient was instructed to stop the exercise if any discomfort is experienced.
The collected data was tabulated and the data was analyzed using International Business Machines (IBM) Statistical Package for The Social Sciences (SPSS) Version 20.0 Software. And Paired sample t test was done to analyse the data.
|
N 5 |
Age |
Frequency |
Percent |
Mean |
Standard deviation |
|
12 |
1 |
20.0 |
14.20 |
1.304 |
|
|
14 |
1 |
20.0 |
|||
|
15 |
3 |
60.0 |
|||
|
TOTAL |
5 |
100.0 |
|||
The Table I, shows that the sample size is 5 and the mean age of DMD children is 14.20.
|
Pulmonary Function Test |
Mean |
n |
Std. Deviation |
t – Value |
p – Value |
|
|
Tidal Volume |
Pre |
1.538 |
5 |
.84736 |
-3.063 |
0.038 S |
|
Post |
1.710 |
5 |
.88752 |
|||
|
Duration of Inspiration |
Pre |
2.058 |
5 |
.21879 |
0.665 |
0.542 NS |
|
Post |
1.922 |
5 |
.58687 |
|||
|
Tidal Volume/ Inspiration time |
Pre |
0.794 |
5 |
.40016 |
-1.163 |
0.309 NS |
|
Post |
1.338 |
5 |
.96567 |
|||
|
Total Lung Volume |
Pre |
2.486 |
5 |
1.42907 |
2.328 |
0.080 NS |
|
Post |
2.112 |
5 |
1.17404 |
|||
|
Inspiratory Capacity |
Pre |
2.346 |
5 |
1.69127 |
0.950 |
0.396 NS |
|
Post |
2.174 |
5 |
1.31933 |
|||
|
Inspiratory Time/ Respiration time |
Pre |
0.550 |
5 |
.21599 |
0.614 |
0.572 NS |
|
Post |
0.486 |
5 |
.04506 |
|||
|
Forced Vital Capacity |
Pre |
1.624 |
5 |
.68061 |
-1.477 |
0.214 NS |
|
Post |
1.740 |
5 |
.82161 |
|||
|
Forced Expiratory volume in one second (FEVI) |
Pre |
1.670 |
5 |
.45563 |
1.084 |
0.339 NS |
|
Post |
1.428 |
5 |
.71601 |
|||
Table II, shows that the mean and standard deviation value is 1.5380 and .84736 in pretest and the mean and standard deviation value is 1.71 and 0.88752 in posttest with respect to Tidal Volume, and the score of t value is -3.063 which is statistically significant at p value> 0.038 value.
While observing the inspiratory muscle training using an Ultrabreathe, The p-value of tidal volume shows the significant result whereas other parameters like duration of inspiration, tidal volume/inspiration time, total lung volume, inspiratory capacity, inspiration time/respiration time, forced vital capacity and forced expiratory volume doesn’t shows any marked significance.
The mean value of posttest of Tidal Volume/Inspiration time is increased when compared with pretest value of tidal volume/Inspiration time is. The mean value of post test of Forced Vital Capacity is increased when compared with pre test value of Forced vital capacity. Hallett S, Ashurst JV (2020) has said that minute ventilation got increased due to physiological demands which caused an increment in oxygenation levels leading to increase in both tidal volume as well as inspiratory rate during exercise18,19,16.
In 6 weeks of inspiratory muscle training, It is shown that there is an significance result in tidal volume. Due to increase in Tidal volume, it can also be expected that there will be a marked increase in minute ventilation which causes an increase in oxygenation. The study result goes in hand with Aldrich and Uhrlass (1987) who proved the effectiveness of the treatment can be bettered by training the respiratory muscles for long duration in DMD and also concluded that inspiratory resistance training coupled with concurrent delivery of intermittent mandatory ventilation increased the effectiveness in patient with DMD20,11.
Orlando E. Flores G (2009) has concluded that the endurance of respiratory muscles in DMD can be significantly increased by training the inspiratory muscle for a period of nine weeks. The observance of peak cough flow on muscle training for a long period makes it certain that endurance and force of respiratory muscles in DMD patients can be increased by undergoing long period of muscle training21.
Inspiratory muscle training with Ultrabreathe for a longer duration in Duchenne Muscular Dystrophy children can also improve the pulmonary function status. A O Frank (2018) stated that specialized sitting can provide appropriate postural support, helping the child to be mobile. In addition to this the spinal complication of Muscular Dystrophy recommends regular monitoring and careful management22.
The children should be maintained in an optimal sitting position in wheel chair while performing the intervention protocol otherwise it may also an reason for not getting significant improvement in all components during the intervention. The study result goes in hand with Theodar Wanke et al., (1994) suggested that scoliosis, straightening of thoracic spine, limitation diaphragmatic and intercostal movements, elevation of diaphragm, recurrent chest function and muscular weakness are the factors which affects the pulmonary function23,13,15.
Rodini et.al., (2012) discussed that Muscular weakness plays the role of reduction factor in inspiratory muscle capacity which allows to expand the chest wall and inflate the lungs. The total lung capacity is ascertained by the balance between elastic recoil of respiratory system and force of the muscle. The total lung capacity of the respiratory muscles grow weaker owing to length tension property and due to decreased lung compliance the respiratory muscles becomes stiffer. Progressive impairment of respiratory muscles, reduces chest wall and pulmonary compliance in addition to this it gets weakened due to mechanical load on it and these results fatigue and respiratory failure24.
There is a no statistical significance is present in this study because of the shorter period of the study and progression of the disease. The reason why Duration of Inspiration, Tidal Volume/Inspiration Time, Total Lung Volume, Inspiratory Capacity, Inspiratory time/ Respiration time, Forced Vital Capacity and Forced Expiratory Volume in one second (FEV1) did not show any changes for Ultrabreathe treatment in DMD children’s may be due to climatic condition, treatment was taken during winter season. Another reason for not showing changes in the components is the treatment given to them only for six weeks. The result may be significant if treatment will be given to them for a long duration of time. This study concluded that inspiratory muscle training using Ultrabreathe shows significant improvement only in tidal volume whereas, there is no significant improvement Duration of Inspiration, Tidal Volume/Inspiration Time, Total Lung Volume, Inspiratory Capacity, Inspiratory time/Respiration time, Forced Vital Capacity and Forced Expiratory Volume in one second (FEV1).
The study concluded that the Effectiveness of Inspiratory Muscle training using Ultrabreathe on pulmonary function in Duchenne Muscular Dystrophy children showed improvement only in Tidal Volume whereas all other components not showed a significant improvement.
The limitation of the study is the study duration was less and smaller sample size was included and the recommendation of the study are the protocol can be given for a prolonged duration and the study needed on both manual technique and inspiratory muscle trainer to show the effect comparatively.
ACKNOWLEDGEMENTS:
The authors acknowledge the Muscular Dystrophy School Authorities, Children’s who participated in the study, Professors and Staff members of SRM College of Physiotherapy and who were helped in the study.
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Received on 16.10.2020 Modified on 10.02.2021
Accepted on 07.05.2021 © RJPT All right reserved
Research J. Pharm. and Tech 2022; 15(1):193-196.
DOI: 10.52711/0974-360X.2022.00031