Table 6 Guidance statements for CPAP, NIPPV, oxygen supplementation and hypercapnia monitoring
From: Recommendations for the management of MPS VI: systematic evidence- and consensus-based guidance
Statement | Percentage consensus |
|---|---|
CPAP therapy is recommended for patients with MPS VI who display the presence of obstructive sleep apnoea (OSA) which persists after tonsillectomy and/or adenoidectomy Evidence Grade: B (extrapolations from level 1 studies) | 100% |
NIPPV therapy is recommended for patients with MPS VI who display nocturnal hypoventilation and are unresponsive to CPAP, or display daytime hypoventilation with increased PaCO2 and/or serum HCO3 levels Evidence Grade: B (extrapolations from level 1 studies) | 94% |
Oxygen supplementation is recommended for patients with MPS VI who display sleep apnoea with nocturnal hypoxemia and who do not tolerate CPAP or NIPPV masks Evidence Grade: B (extrapolations from level 1/3/4 studies) | 83% |
Patients with MPS VI should be monitored for development of hypercapnia after starting oxygen therapy with measurement of PaCO2 and/or serum HCO3 Evidence Grade: D (level 5, expert clinical opinion) | 97% |