Guest Editorial: Early and innovative symptomatic care to improve quality of life of ALS patients at Cleveland VA ALS Center

Abstract

INTRODUCTION Amyotrophic lateral sclerosis (ALS) is a neurodegenerative disease of unknown etiology. However, there is good evidence to validate the theory that military service predisposes the individual to the future development of the disease [1]. As a direct result of these data, in 2008 ALS became a presumptively compensable illness for all veterans with 90 days or more of continuously active service in the military. This decision led to an influx of ALS patients into the Department of Veterans Affairs (VA) healthcare system and the development of a nationwide plan of care [2]. The implementation of this plan at the Cleveland VA Spinal Cord Injury (SCI) Division has been challenging. However, it has offered the opportunity to review current practice parameters that dictate the care of ALS patients in a new context. Using these parameters and considering the strengths and weakness of the VA healthcare system, we have made significant improvements to current practice and applied novel technology to care for ALS patients. Herein we describe these innovations and the foundation we have established to provide high quality ALS care. BACKGROUND ALS is a terminal neurodegenerative disorder. The management of patients with ALS centers almost entirely around symptomatic care. Although many studies have examined the role of particular interventions in prolonging survival after diagnosis or decreasing the rate of decline in physical ability, perhaps the most important studies guiding intervention have investigated quality of life (QoL) over the course of the disease. In 1999, the American Academy of Neurology (AAN) published the first set of formal practice parameters based on a review of the literature [3]. These evidence- based practice parameters were revised in 2009 [4] and included important updates on how care is administered, as well as recommendations regarding the use of the first disease-modifying agent available. The European Federation of Neurological Societies (EFNS) published parallel recommendations in 2005 [5], with revised and updated practice guidelines published in 2012 [6]. A third publication in 2007 also summarized current evidence-based recommendations for care of ALS patients [7]. These three sets of recommendations are nearly identical and in many instances lack precise details in terms of when and how to implement care. This reveals a need for further studies to answer basic questions regarding interventions, such as when a percutaneous endoscopic gastrostomy (PEG) tube should be placed in a patient or how early respiratory support should be provided. This information would help standardize practice parameters and allow for a higher level of symptomatic care in patients with ALS. INNOVATIVE CARE IN VA SYSTEM Early Noninvasive Positive Pressure Ventilation for ALS Patients The majority of patients with ALS will die from respiratory failure [8]. Furthermore, during the course of the disease, respiratory function correlates with QoL and patient survival [9-11]. The survival benefits from noninvasive positive pressure ventilation (NIPPV) supersede those obtained from riluzole or PEG tube insertion [12]. It follows, logically, that aggressive management of respiratory compromise should be part of a comprehensive plan not simply to prolong life (because some patients prefer not to have their life prolonged) but also as a mechanism to improve QoL. The latter goal, which is more consistent with our mission as an ALS center, is supported by several well-designed studies [12-13]. These studies suggest that NIPPV improves QoL by improving a patient's energy, concentration, and sleep quality, decreasing daytime somnolence and physical fatigue. NIPPV also improves lung compliance [14] and decreases the rate of decline in vital capacity [15-16]. Despite this evidence, the ALS Clinical Assessment Research and Education database has documented underutilization of NIPPV by patients with ALS [17]. …