Noninvasive breathing support for SMA

Overview
Some people with spinal muscular atrophy (SMA) require breathing assistance. The type of ventilation equipment or device used depends on the severity of the SMA symptoms.

Noninvasive ventilation is one type of support that can aid breathing using an air-tight mask placed over the nose, or nose and mouth. The mask is connected to a machine that delivers continuous air flow to the lungs.

Noninvasive ventilation is used to prevent hypoventilation (weak or shallow breathing) while sleeping or during illness. Children with the most severe types of SMA may also need breathing support 24 hours a day.

Adults with SMA type 3 may need NIV as they age, due to an increased risk of obstructive sleep apnea and hypoventilation.

What does it involve?
With bi-level positive airway pressure (BiPAP), the amount of air volume and pressure delivered alternates as the person inhales and exhales. BiPAP delivers air even when someone is in deep sleep and not breathing adequately.

Mechanical ventilators may also be used non-invasively with a mask. They offer more options and settings than BiPAP and allow for better breathing control when someone is asleep or awake. Air can be delivered through a mask that covers the nose or nose and mouth. Different types of mechanical ventilators can be carried over the shoulder or placed on a wheelchair.

A sipper vent is a NIV device that allows a person sitting in a wheelchair to take a breath through a small mouthpiece positioned like a microphone in front of the face. The sipper vent is connected to a ventilator machine that provides a boost of air for talking and breathing at the same time.

Intended outcomes
The short-term goal of NIV is to provide symptom relief and easier breathing. The long-term goal is to improve sleep and quality of life, and prolong survival without surgical intervention. NIV may improve chest wall deformity and lung development in children with SMA type 2.

Results
Studies are inconclusive on the benefits of using assisted breathing devices in children. However, some clinical studies observed reduction in sleep disturbances, night sweating, and morning headaches in children with nocturnal breathing issues. NIV may also improve appetite and concentration.

In a small study of 22 children with SMA, researchers observed that NIV decreased frequency and severity of respiratory infections. NIV may also improve quality of life for children with SMA as well as prolong their life expectancies. In SMA type 1, the use of NIV has been debated.

Constraints
Although many children tolerate the nose mask, it may not work well for every child or family. Common problems include skin irritation, pressure sores, poor fit, or the child rejects the mask. A pulmonologist-respiratory team can help you find a solution for your child.

For more details, visit:
1. Breathing Basics – Cure SMA
2. Special Considerations in the Respiratory Management of Spinal Muscular Atrophy ーJournal of the American Academy of Pediatrics
3. Consensus Statement for Standard of Care in Spinal Muscular Atrophy 一 Journal of Child Neurology
4. Spinal muscular atrophy--noninvasive ventilatory support in pediatrics ー Revista Portuguesa de Pneumologia