Safety and Tolerability of Systemic Beta-2-Adrenergic Agonist[Albuterol] as Pharmacological Therapy in Non-Invasive Ventilation[ NIV ]-supported Amyotrophic Lateral Sclerosis[ALS]Patients with Chronic Respiratory Failure (P4.079)

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OBJECTIVE: Clinical audit of use of systemic albuterol in NIV-supported ALS patients at an ALS Multidisciplinary Clinic. BACKGROUND: NIV decreases the rate of respiratory function decline in ALS patients[Kleopa,1999;Bourke,2006]. Few clinical trials have addressed improving respiratory function or extending time before NIV daytime-dependency in these patients[Yi,2012]. Beta-2-adrenergic agonists[albuterol/clenbuterol]have increased vital capacity and neck strength short-term in ALS and spinal muscular atrophy patients[Brooks,2000; Soraru,2006, Querin,2013]. DESIGN/METHODS: Retrospective audit of albuterol utilization in NIV-supported ALS patients from 2010-2013. Vital signs[pulse rate, respiratory rate, blood pressure, vital capacity[%predicted-Knudsen(adult), ALSFRS-R individual or combined bulbar-and respiratory-subscores]were reviewed in 108 NIV-supported ALS patients[55M;52F]at start of NIV or at start of albuterol on NIV and then at 3 and 6 months subsequently. Albuterol was started at median systemic dose[tablet/liquid]of 6 + 3[SD] mg/day in NIV-supported ALS patients with difficulty maintaining breathing capacity associated with bulbar and respiratory dysfunction. RESULTS: Albuterol was tolerated for 6 months in 49/52 [31M;18F]NIV-supported ALS patients but not in 3 patients[1M;2F][94.2% tolerability]with no further VC reduction for 3 months. More M patients(P=0.0262)with VC below 70% predicted [VC=61.0% predicted(95%CI=53.9-68.2]were given albuterol compared with NIV-supported ALS patients[23M;32F]not receiving albuterol[VC=77.3%(95%CI=70.7-83.9)]. ALSFRS-R respiratory-, but not bulbar-sub-score was significantly[P=0.0006] decreased in albuterol-treated[6.4(95%CI=5.4-7.3)}compared with patients not receiving albuterol[9.2(95%CI=8.0-10.6)]. Pulse rate increased 10% from baseline but the population variability of this change within the normal pulse rate range was not significant. VC remained unchanged at 3 months[VC=57.7%(95%CI=49.8-65.5)]but declined at 6 months on albuterol[VC=50.0%(95%CI=35.8-58.5)]. CONCLUSIONS: Systemic albuterol is 94.2% tolerable when used in NIV-supported ALS patients. M NIV-supported ALS patients more commonly required albuterol. VC was maintained at 3 months without change but not at 6 months in albuterol-treated NIV-supported ALS patients. Further studies are required on pharmacologically enhancing the treatment of NIV-supported ALS patients. Study Supported by: Carolinas ALS Research Fund/Pinstripes Fund/Carolinas Garden of Hope/Carolinas Healthcare Foundation

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