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Human A1,2, Corten L2, Jelsma J2, Morrow BM3
1Sefako Makgatho Health Sciences University, Physiotherapy Department, Garankuwa, South Africa, 2University of Cape Town, Health and Rehabilitation Sciences (Division Physiotherapy), Observatory, South Africa, 3University of Cape Town, Paediatrics and Child Health Department, Rondebosch, South Africa
Background: Progressive respiratory muscle weakness and ineffective cough contributes to morbidity and mortality in children with neuromuscular diseases (NMD). Inspiratory muscle training (IMT) aims to preserve or improve respiratory muscle strength, optimise ventilation, improve cough ability, reduce morbidity, delay the onset of respiratory failure and ultimately improve health-related quality of life. The use of IMT in this subpopulation remains controversial, and there is currently insufficient evidence to guide clinical practice.
Purpose: To determine the safety and efficacy of IMT on clinical outcomes (hospital admissions, adverse events, upper and lower limb function, upper limb coordination, spirometry, inspiratory muscle strength and peak cough flow (PCF)).
Methods: In a cross-over study design, participants were randomly allocated to two groups: Group 1 trained twice daily for three months (30 breaths with a tapered flow threshold device), after which they stopped training and were monitored monthly, for three months. Resistance was set at 30% of the patient's inspiratory muscle strength (Pimax) and adapted every two weeks during intervention. Group 2 did not train for the first three months after which they crossed over to the same IMT intervention for three months. Post-intervention monitoring was continued for a further three months.
Results: Preliminary results of the first 10 participants (n=9 male; median (IQR) age 12.3 (10.1-14.3) years) are presented. Six participants were non-ambulant; none of the participants received daytime ventilation, whilst one received nocturnal noninvasive ventilation. Participants had baseline median (IQR) lower limb function score (Vignos scale) of 7.5/10 (3-9). Upper limb function was more preserved, with median Brooke scale score of 1.5/6 (1-3) and upper limb coordination was high with Motor Function Measure (MFM) score of 28/30 (24-29).
One patient was hospitalised twice during the intervention period for an intercurrent respiratory tract infection. No adverse events related to IMT were reported. There were no significant changes in lower limb function, upper limb function and coordination scores, or spirometry following the IMT intervention. However, median (IQR) Pimax and PCF improved by 11.5 (-4.0 to 26.0) cmH2O and 30 (-10 to 80) L/min in the intervention group, compared to change of -5 (-12.0 to 3.0) cmH2O (p = 0.04) and -30 (-40 to -10)L/min (p = 0.02) respectively during the control period. Order assignment had no effect on results. Patient satisfaction with IMT based on a 10 point scale was extremely high, with a median (IQR) of 10 (8-10).
Conclusion(s): The use of a three month IMT programme in children with NMD improved inspiratory muscle strength and cough flow, with very high patient satisfaction.
Implications: Inspiratory muscle training appears safe and well-tolerated, with improved, clinically relevant outcomes. Larger randomised controlled trials are recommended to confirm these findings and determine the long-term outcome of IMT on morbidity and mortality.
Keywords: Inspiratory muscle training, neuromuscular diseases, children
Funding acknowledgements: URC Equipment Grant (Western Cape); Sefako Makgatho Health Sciences University Research Development Grant; South African Society of Physiotherapy.
Purpose: To determine the safety and efficacy of IMT on clinical outcomes (hospital admissions, adverse events, upper and lower limb function, upper limb coordination, spirometry, inspiratory muscle strength and peak cough flow (PCF)).
Methods: In a cross-over study design, participants were randomly allocated to two groups: Group 1 trained twice daily for three months (30 breaths with a tapered flow threshold device), after which they stopped training and were monitored monthly, for three months. Resistance was set at 30% of the patient's inspiratory muscle strength (Pimax) and adapted every two weeks during intervention. Group 2 did not train for the first three months after which they crossed over to the same IMT intervention for three months. Post-intervention monitoring was continued for a further three months.
Results: Preliminary results of the first 10 participants (n=9 male; median (IQR) age 12.3 (10.1-14.3) years) are presented. Six participants were non-ambulant; none of the participants received daytime ventilation, whilst one received nocturnal noninvasive ventilation. Participants had baseline median (IQR) lower limb function score (Vignos scale) of 7.5/10 (3-9). Upper limb function was more preserved, with median Brooke scale score of 1.5/6 (1-3) and upper limb coordination was high with Motor Function Measure (MFM) score of 28/30 (24-29).
One patient was hospitalised twice during the intervention period for an intercurrent respiratory tract infection. No adverse events related to IMT were reported. There were no significant changes in lower limb function, upper limb function and coordination scores, or spirometry following the IMT intervention. However, median (IQR) Pimax and PCF improved by 11.5 (-4.0 to 26.0) cmH2O and 30 (-10 to 80) L/min in the intervention group, compared to change of -5 (-12.0 to 3.0) cmH2O (p = 0.04) and -30 (-40 to -10)L/min (p = 0.02) respectively during the control period. Order assignment had no effect on results. Patient satisfaction with IMT based on a 10 point scale was extremely high, with a median (IQR) of 10 (8-10).
Conclusion(s): The use of a three month IMT programme in children with NMD improved inspiratory muscle strength and cough flow, with very high patient satisfaction.
Implications: Inspiratory muscle training appears safe and well-tolerated, with improved, clinically relevant outcomes. Larger randomised controlled trials are recommended to confirm these findings and determine the long-term outcome of IMT on morbidity and mortality.
Keywords: Inspiratory muscle training, neuromuscular diseases, children
Funding acknowledgements: URC Equipment Grant (Western Cape); Sefako Makgatho Health Sciences University Research Development Grant; South African Society of Physiotherapy.
Topic: Paediatrics; Cardiorespiratory
Ethics approval required: Yes
Institution: University of Cape Town
Ethics committee: Human Research Ethics Committee
Ethics number: 513/2015
All authors, affiliations and abstracts have been published as submitted.
