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Relationship between respiratory function and need for NIV in childhood SMA
Nitin Kapur1,2, Sean Deegan1, Ankit Parakh1
1Department of Respiratory Medicine, Children's Health Queensland, South Brisbane, Australia.
Pediatric Pulmonology
|July 23, 2019
Summary
Noninvasive ventilation (NIV) is common in children with spinal muscular atrophy (SMA), with respiratory function (RF) abnormalities predicting its need. Even with normal RF, sleep-disordered breathing (SDB) can occur, highlighting the importance of monitoring.
Area of Science:
- Pediatric Pulmonology
- Neuromuscular Disorders
- Respiratory Medicine
Background:
- Spinal muscular atrophy (SMA) is characterized by progressive respiratory muscle weakness.
- Respiratory function (RF) in children with SMA, particularly those utilizing noninvasive ventilation (NIV), is not well-documented.
Purpose of the Study:
- To characterize RF in pediatric SMA patients.
- To compare RF between SMA patients who use NIV and those who do not.
Main Methods:
- A cross-sectional study involving 25 children with SMA.
- Assessment included polysomnography (PSG), spirometry, forced oscillation technique (FOT), lung clearance index (LCI), sniff nasal inspiratory pressures, peak cough flow, and maximal inspiratory/expiratory pressures.
- NIV use and indications were recorded.
Main Results:
- Forty percent (10/25) of children with SMA required NIV, primarily for sleep-disordered breathing (SDB) or lower respiratory tract infection (LRTI).
- Children on NIV were older and exhibited significantly more abnormal forced vital capacity (FVC) z-scores and LCI compared to those not on NIV.
- Abnormal FVC z-scores and LCI were associated with higher maximum transcutaneous carbon dioxide levels during PSG.
Conclusions:
- Noninvasive ventilation (NIV) is frequently used in pediatric SMA patients.
- Normal respiratory function does not rule out SDB in this population.
- Children with diminished FVC and elevated LCI are at higher risk for requiring NIV, especially during or after an LRTI.
Keywords:
neuromuscular disordersnoninvasive ventilationrespiratory functionsleep-disordered breathingMore Related Videos
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