1095 Treatment with Trilogy AVAPS AE Mode in Pompe Disease with Chronic Respiratory Failure Unresponsive to AVAPS ST Mode. (27th April 2018)

• Record Type: Journal Article
• Authors: Rajagopal, Anita; Bodkin, Cynthia
• Is Part Of: Sleep. Volume 41(2018)Supplement 1
• Page Start: A407
• Page End: A407
• Publication Date: 2018-04-27
• Subjects: Sleep -- Physiological aspects -- Periodicals; Sleep disorders -- Periodicals; Sommeil -- Aspect physiologique -- Périodiques; Sommeil, Troubles du -- Périodiques; Sleep disorders; Sleep -- Physiological aspects; Sleep -- physiological aspects; Sleep Wake Disorders; Psychophysiology; Electronic journals; Periodicals; 616.8498
• DOI: 10.1093/sleep/zsy063.1094
• Languages: English
• ISSNs: 0161-8105

Abstract

Abstract: Introduction: Pompe disease is a genetic disorder that involves the enzyme alpha-glucosidase which breaks down complex sugars in the body. Patients with late-onset disease experience progressive muscle weakness, including the muscles that control breathing. As the disorder progresses, managing respiratory failure can be challenging. We present a case of Pompe disease in a patient who failed treatment with average volume assured pressure support (AVAPS) spontaneous-timed (ST) mode but responded well to Trilogy AVAPS Auto EPAP (AE) mode. Report of Case: A 60-year-old patient with Pompe disease developed proximal weakness over 10 years. She was hospitalized with severe hypercarbic respiratory failure. After requesting no intubation, she required non-invasive ventilation. She was transitioned to Trilogy with oxygen at night and oxygen alone during the day. A titration study was performed on BiPAP AVAP-ST®. The study showed a cumulative AHI of 2.0/hr with oxygen nadir 81.0%. At a PAP pressure of EPAP 6cmH20, IPAP 10-25cmH20, back-up rate 10bpm, sleep disordered breathing was improved with oxygen saturations in the 90s. However, respiratory rate was still irregular and sleep was still fragmented. Patient's respiratory rate while awake was 40-44bmp. During sleep, she had 10–40 second period with a respiratory rate 40bmp alternating with 10–60 seconds of a rate of 10-12bpm, which is felt to be the back-up rate. A repeat titration study was performed on Trilogy AVAPS, same settings as above, with ST and AE modes. On AE mode, the patient had a cumulative AHI of 0.0/hr with oxygen nadir 88.0%. Sleep disordered breathing resolved. Conclusion: In patients with chronic respiratory failure from genetic disorders, managing treatment can be challenging. We have illustrated that in patients who fail treatment with AVAPS ST mode, use of the Trilogy AVAPS AE mode can be successful, perhaps due to the sensitivity of the Trilogy being superior to the AVAP.