Respiratory care is one of the mainstay management issues in children with SMA.
Why children with SMA have respiratory complications
Respiratory muscle function in SMA features very weak intercostal muscles and a relatively stronger diaphragm. The diaphragm is the primary muscle used for breathing in children with SMA type 1 or 2. In the first year of life the chest wall is very complia nt. Thus, the chest wall in children with SMA type 1 or 2 often appears collapsed and bell shaped because of a lack of opposition of the intercostal muscles against the function of the diaphragm the resulting chest wall deformities include a bell – shaped ch est and pectus excavatum. The compromised respiratory muscle function results in:
- Impaired cough resulting in poor clearance of lower airway secretions;
- Hypoventilation during sleep;
- Chest wall and lung underdevelopment; and
- Recurrent infections th at exacerbate muscle weakness and the integrity of the lung parenchyma.
The recommendations have been developed according to the above general principles with the goal of maintaining safety while providing timely, efficient and effective airway management
The following recommendations are for routine care of all SMA patients (2, 3, and 4)
- If child is on routine inhalation therapy that should be continued at the same dosage
- There is no contraindication for the use of inhaled steroid therapy if they are already on it because of the pandemic (3, 4)
- Reliever inhaler therapy should be well stocked up; if spacer is being used then make sure a new or recent one is being used
- In COVID 19 children have what is called happy hypoxia, whe re the oxygen saturation’s may drop unexpectedly, without child showing any features of respiratory distress. (5, 6)
- Know your child’s normal /usual oxygen saturations. Till the pandemic is over monitor the oxygen saturation’s twice daily, even in absence of symptoms and fever (6 – 9)
- In case of fever monitor the oxygen saturations every 4 hours, more frequently if the child has any distress or cough (2, 9)