Bay Area Pediatric Pulmonary Medical Corporation - BAPP - Post-Operative Care of Children with Neuromuscular Disease

RECOMMENDATIONS FOR POST-OPERATIVE CARE OF CHILDREN WITH NEUROMUSCULAR DISEASE FOLLOWING SURGERY (OR THE INTUBATED CHILD WITH NEUROMUSCULAR DISEASE)

For the intubated patient, perform the following q 4 hours:

1.Intrapulmonary percussive ventilation followed by
2.Cough Assist machine set at inspiratory pressure +30 for I second, expiratory pressure - 30 for 1 second and 2 second pause, 4 sets of 5 breaths, followed by
3.Endotracheal tube suctioning.

The Cough Assist machine can be used as often as every 10 minutes followed by endotracheal suctioning to help remove lower airway secretions.

Extubate when the patient is:
1.afebrile
2.not requiring supplemental 02
3.CXR is without atelectasis or infiltrates
4.off respiratory depressants
5.airway suctioning is 1 time/hour or less

Extubate to continuous nasal mask ventilation and no supplemental 02 such as BiPAP with settings: IPAP 14-17 and EPAP 3-6 using the spontaneous timed mode with a backup rate to match their respiratory rate.

Use oximetry to guide the use of expiratory aids, postural drainage, and CPT.
Following extubation perform the following q. 4 hours:

1.Chest physiotherapy, either manual or IPV, followed by
2.Cough Assist machine, set at inspiratorv pressure +40 for 1 second, expiratory pressure - 40 for 1 second and 2 second pause, 4 sets of 5 breaths, followed by
3.Postural drainage (trendelenberg) up to 15 minutes as tolerated, followed by
4.Cough Assist machine, 4 sets of 5 breaths.

The Cough Assist machine can be used as often as every 10 minutes to clear lower airway secretions. Use if oxygen saturation drops to <94% acutely.

Wean from BiPAP during the day as tolerated. Goal is to use BiPAP per nasal mask while sleeping only. Wean airway clearance regimen above as airway secretions decrease working toward a 4x/day. schedule or less.

Nutritional Management during concomitant illness/catabolic states is critical! Do not allow fasting> 6 hours in SMA type 1 subjects, or > 8 to 12 hours in SMA type 2 subjects or other neuromuscular patients. SMA patients have a secondary fatty acid oxidation defect and virtually all have reflux, delayed gastric emptying and autonomic gastrointestinal dysfunction which worsens with illness: recommend immediate institution of continuous feeds via NJ, plus/minus supplementation with PPN or TPN. Include approximately 1.5 gms/kg amino acids per 24 hour period and 10-15% intralipid.