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

Abstract

INTRODUCTIONAmyotrophic 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. BACKGROUNDALS 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 evidencebased 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.

Associative Increases in Amyotrophic Lateral Sclerosis Survival Duration With Non-invasive Ventilation Initiation and Usage Protocols

“…Many clinics in Europe and Asia begin NIV much earlier, as shown in a recent study examining differences in international standards of care for ALS NIV ( 22 ), but over 20% of international clinics actually begin NIV later, well after functional symptoms commence ( 23 ). Irrespective, it has long been hypothesized that starting NIV earlier than the <50 FVC %predict threshold could be associated with additional benefits ( 24 , 25 ), but there has been a lack of data to illustrate this effect in a large-scale study.…”

Every Second Counts

“…Use of telemedicine and mobile video technologies save travel cost and hospital care, while the patient could stay at home. 35 The concepts of SOC 15,16 as comprehensibility, meaningfulness, and manageability are significant for well-being during illnesses such as ALS. Therefore, nursing staff had a professional role to play in supporting patients in end-of-life situations, which require structured, clear directions and priorities.…”

Using technology to improve access to specialist care in amyotrophic lateral sclerosis: A systematic review

“…Two services in the United States reported that using telemedicine had avoided outpatient visits resulting in patient travel cost savings (41,42). An…”