Outcome of goal-directed non-invasive ventilation and mechanical insufflation/exsufflation in spinal muscular atrophy type I

  title={Outcome of goal-directed non-invasive ventilation and mechanical insufflation/exsufflation in spinal muscular atrophy type I},
  author={Michelle Chatwin and Andrew Bush and Anita Simonds},
  journal={Archives of Disease in Childhood},
  pages={426 - 432}
Background There are widely discrepant views on the respiratory management of infants with spinal muscular atrophy (SMA) type I. Typically, management is palliative. Design A descriptive study of interventions and investigations is reported that were offered to a cohort of 13 children with SMA type I referred to our centre. Interventions and investigations included sleep studies, provision of non-invasive positive pressure ventilation (NIPPV) for ventilatory support/dependency and for… 
Spinal Muscular Atrophy Type 1: Avoidance of Hospitalization by Respiratory Muscle Support
A protocol including high span bilevel positive airway pressure along with MAC to expel airway secretions and normalize oxyhemoglobin saturation can be used by trained caregivers to avoid episodes of acute respiratory failure and hospitalization for children with spinal muscular atrophy type 1 under 3 yrs of age.
Long-Term Mechanical Insufflation-Exsufflation Cough Assistance in Neuromuscular Disease: Patterns of Use and Lessons for Application
Greater exsufflation pressures than insufflation pressures, together with a shorterinsufflation time than exsufflated time, were used and were used to promote increased cough peak flow (CPF) in conjunction with a cough.
Mechanical Insufflation-Exsufflation as Bronchial Hygiene Technique in Critical Care Patients: Systematic Review
This study aimed to systematically review the outcomes enabled by the respiratory therapy using the mechanical insufflation-exsufflation in critically ill patients admitted to the intensive care unit through a systematic literature review.
Non-invasive Ventilation in Children With Neuromuscular Disease
Non-invasive ventilation (NIV) is used to assist or “replace” the weakened respiratory muscles in order to correct alveolar hypoventilation by maintaining a sufficient tidal volume and minute ventilation in children with neuromuscular diseases.
Home Mechanical Ventilation: An Overview.
  • A. Simonds
  • Medicine, Engineering
    Annals of the American Thoracic Society
  • 2016
The combination of noninvasive ventilation and cough-assist devices has reduced the indications for tracheotomy ventilation in some situations and has also prolonged survival.
Noninvasive Ventilation in Palliative Care and Ethical Dilemma
Significant difference exists between validated indications for noninvasive ventilation use in children and current real life practice and a structured professional frameshift should be available for support and ethical guidance in order to provide confidence to patients, families and all the involved caregivers.
Management of bronchial secretions with Free Aspire in children with cerebral palsy: impact on clinical outcomes and healthcare resources
The findings show that Free Aspire for bronchial secretion clearance in cerebral palsy patients with limited capacity to collaborate is safe and effective in reducing the impact of respiratory exacerbations in terms of number of PCP visits, days spent in hospital, and days of antibiotic therapy; its regular use maintains this effect in time.


Spinal muscular atrophy type 1: A noninvasive respiratory management approach.
Although intercurrent chest colds may necessitate periods of hospitalization and intubation, tracheostomy can be avoided throughout early childhood for some children with SMA type 1.
Noninvasive Ventilation in Children with Spinal Muscular Atrophy Types 1 and 2
NIV improved sleep breathing parameters and thoracoabdominal coordination during sleep in SMA types 1 and 2 and phase-angle improvement correlated with bilevel PAP pressures.
Thoracoabdominal pattern of breathing in neuromuscular disorders.
The quantitative assessment of TAPB enhances the ability to estimate pulmonary function in neuromuscular disorders, and the efficiency of mechanical ventilation.
Use of the mechanical in-exsufflator in pediatric patients with neuromuscular disease and impaired cough.
In 90% of the study population, the use of an MI-E was safe, well-tolerated, and effective in preventing pulmonary complications.
Implementation of “the consensus statement for the standard of care in spinal muscular atrophy” when applied to infants with severe type 1 SMA in the UK
It is vital to share information and formulate an anticipatory care plan with the infant's parents from the point of diagnosis, and there are many forms of active respiratory management which can help maintain the well-being of infants with severe type 1 SMA.
Respiratory support in spinal muscular atrophy type I: a survey of physician practices and attitudes.
A wide variation in physician practice is found regarding the mechanical ventilation of patients with SMA type I, and it is suggested that physician training and attitudes affect recommendations regarding mechanical ventilation and ultimately family decision making.
Spinal muscular atrophy type 1: Management and outcomes
Our objectives were to describe survival, hospitalization, speech, and outcomes related to respirator needs for spinal muscular atrophy type 1 (SMA1) patients, using noninvasive or tracheostomy
Respiratory capacity course in patients with infantile spinal muscular atrophy.
This data shows the progressively worsening course of restrictive respiratory insufficiency in patients with SMA, and the importance of early respiratory management to limit pulmonary complications and improve the quality of life for these patients.