Respiratory care
-
Pompe disease is a rare autosomal recessive disorder caused by α-glucosidase deficiency. Lower airway involvement and management are rare in patients with late-onset Pompe disease. We describe the case of a 16-y-old girl with late-onset Pompe disease who presented with obvious progressive deterioration in respiratory function. ⋯ Following implantation of the stent, the patient's pulmonary function stabilized, and her pulmonary hypertension resolved. The patient continued on enzyme replacement therapy and nighttime CPAP ventilation. This case highlights that lower airway involvement may occur with late-onset Pompe disease and that flexible bronchoscopy can be an effective tool for both diagnosis and management of lower airway collapse in late-onset Pompe disease.
-
Editorial Comment
Therapist-directed protocols designed with health-care reform in mind.
There is no doubt that therapist-directed protocols are an effective way of providing therapy to patients in need of respiratory care. Therapist-directed protocols have been shown to provide the right therapy to the right patients in an effective and efficient manner. The current investigation presents us with an additional reason for promoting the use of therapist-directed protocols by suggesting a reduced readmission rate in the therapist-directed care group. Respiratory therapists must advocate for the continued or expanded use of these therapist-driven protocols as a way of assisting their institutions to potentially reduce COPD readmissions and minimize CMS reimbursement reductions.