Children with neuromuscular diseases are group of relatively common diagnosis that includes SMA, DMD, ALS, congenital myopathy with a prevalence of about 1 in 3000 globally 1 and 26% of the respiratory impairment in India 2.

Spinal Muscular Atrophy (SMA) is an autosomal recessive neurodegenerative disorder characterized by progressive muscle wasting due to motor neuron degeneration, secondary to mutations in the survival motor neuron 1 (SMN1) gene 3. Muscle weakness and poor muscle coordination can affect any part of the upper airway and cause breathing problems such as reduced lung function, hypoventilation, and weak cough, difficulty clearing mucus from the airways, upper airway obstruction and sleep apnoea 4.

Respiratory impairment is predominant with variable severity of chest wall distortion, impaired airway clearance and cough that worsens later in childhood 5. Common respiratory consequences such as hypoventilation, upper airway obstruction, aspiration lung disease, secretion retention, lower airway infection finally to respiratory failure in children with neuromuscular disease 6.

Secretion retention is the major burden of lung disease due to a weak cough typically noted in children with neuromuscular disease. Airway clearance can eliminate secretions to improve survival and prevent unexpected hospital admissions 7.

Commonly practiced chest physiotherapy techniques such as Percussions, vibrations, positioning, adapted postural drainage, breathing exercises and manually assisted cough were effective airway clearance techniques. Traditional chest physiotherapy techniques are clinically tiring procedure for children and also precipitate episodic oxygen desaturation in children with SMA that demands lesser therapy duration 8.

Children with neuromuscular disease have a weak cough that limits their ability to get retained secretions; airway clearance therapies such as a cough assist device can help to maintain bronchial hygiene 1.

Mechanical Insufflation-Exsufflation device is a non-invasive procedure and effective airway clearance modality to simulate physiological cough. Positive air pressure (insufflation) is delivered to obtain a large volume of air within the lungs; quickly reversing the flow of air by shifting to negative air pressure (exsufflation) 9. High expiratory flow “Asymmetric pressure values” helps mobilize retained secretions from deeper lung zones 10.

Visual Analogue Scale (VAS) determines user rated comfort during MI:E therapy with cut-off points were 0–3 not/slightly, 4–6 moderately and 7–10 very uncomfortable. (B.hov et al 2024) “Asymmetric pressure” values i.e. exsufflation pressure (Pe) greater than insufflation pressure (Pi) has better airway clearance efficiency in children with NMD but the effect of airway clearance using MI:E therapy with chest physiotherapy throughout hospitalisation course during respiratory distress were not reported 11. This study intends to find out the clinical effectiveness of MI:E therapy as an effective airway clearance on respiratory distress during hospital stay in children with NMD.

This 5 years old child diagnosed to have SMA – type 2 by gene testing with birth history of triplet pregnancy delivered at 31 weeks of gestation by LSCS admitted at NICU for 16 days with initial diagnosis of Respiratory Distress Syndrome (RDS), child was on disease modifying therapy SPINRAZA at 4 years of age.

Spinal muscular atrophy is the most common sequalae in children with neuromuscular diseases that worsens respiratory functioning remarkably. Quality of life is at most important factor of consideration by intervening early primary and secondary complications that may prolong the disease state. Poor functional and productive cough is compromised in children with neuromuscular diseases which results in retention of secretions over the deeper lung zones. Children with SMA type 2 are hospitalised with the respiratory distress and risk of aspiration pneumonia, this frames a major life-threatening reason for medical evaluation and management. Peak cough flow is the clinical outcome to decide the severity risk of aspiration pneumonia chances; children with NMD averages critical value lesser than 180 L/min. Medical management should strongly consider PEG tube insertion, effective airway clearance strategies to prevent further respiratory infections in children with NMD. Cough augmentation is crucial for poor and non-functional cough; children with NMD has difficulties in coughing effectively as their respiratory muscle strength are very weak and retention of secretions over the deeper lung zones this demands the need of MI:E therapy. MI:E therapy is the effective airway clearance modality that mobilises secretions from peripheral airways to the central airways to prevent aspiration and lung infections. Miguel et al 2012 concluded that inclusion of MI-E therapy reduces reintubation rates with consequent reduction in postextubation ICU length of stay also efficient in improving the efficacy of non-invasive ventilation. Similarly in this case report intensive chest physiotherapy combined with M:IE therapy had significant changes in maintaining bronchial hygiene thereby reducing the respiratory distress gradually from PICU admission day 5 to day 8 to wean off from ventilation support. MI:E therapy along with BiPAP support was clinically effective for this child to maintain hemodynamic stability and physiological parameters within normal limits to facilitate early discharge from the hospital that was anticipated to be prolonged (Table 2). Involvement of parents during the MI:E therapy was supportive to reduce the apprehension of the child and also to gain better clinical outcome. Visual analogue scores were ranging 1-2 “very slightly uncomfortable” when MI:E therapy was given by parents during hospital stay (Table 1) ensured that MI:E therapy is very comfortable to applied as a part of home care settings.

In Children with Neuro Muscular diseases, chest physiotherapy with MI:E therapy have an excellent clinical effectiveness to reduce respiratory distress; also to decrease the duration of shifting out transitions from intensive care unit to ward thereby prepare plan for early hospital discharge.