Lung Disease/Sleep Apnea

Individuals with MPS I may have a condition called obstructive sleep apnea. Obstructive sleep apnea is defined as temporary breathing interruptions during sleep that occur when the airway in the neck becomes blocked as muscles in the airway relax. The risk of the airway becoming blocked is increased by some of the physical effects of MPS I, including a short neck, a narrow airway, and enlarged tonsils and adenoids.

Sleep apnea can be improved in some individuals by opening the airway with nighttime CPAP (continuous positive airway pressure) or BiPAP (bi-level positive airway pressure) treatments. In cases where CPAP or BiPAP are not effective or appropriate, a tracheostomy may be used.

A tracheostomy (tray-kee-oss-ta-mee; also called an artificial airway or “trach”) is a surgically created opening through the neck into the trachea, or the windpipe. A tube is usually placed through the opening into the trachea. This tube is referred to as a tracheostomy tube or a trach tube. The function of the tube is to open an airway and to remove secretions from the lungs.