Amyotrophic Lateral Sclerosis

Andersen, P. M.; Borasio, Gian Domenico; Dengler, Reinhard; Hardiman, Orla; Kollewe, Katja; Leigh, P. Nigel; Pradat, Pierre; Silani, Vincenzo; Tomik, Barbara

doi:10.1002/9780470753279.ch20

Cited by 5 publications

(5 citation statements)

References 101 publications

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“…Palliative care guidelines support the presence of a family member or friend, but emphasise individual assessment [6]. Furthermore, MND specific guidelines stress the need for the patient to be accompanied and for the diagnosis to be delivered with a step-wise approach that takes account of the information requirements of the PwMND and their family [21].…”

Section: Discussionmentioning

confidence: 99%

“…How the diagnosis is communicated has implications not only for future patientprofessional relationships [3], but also for the way individuals adapt to requirements for support throughout their illness [10,12,18]. In palliative care, individualised care in delivering diagnosis and prognosis has long been recognised, with guidelines developed in 2007 [6]; in addition the UK National Institute for Health Care Excellence has developed guidance for assessment and care of PwMND [19], which complements International guidance to aid delivery of the diagnosis [20][21][22][23] but, to date, no similar Australian guidelines have been developed.…”

Section: Introductionmentioning

confidence: 99%

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‘The shock of diagnosis’: Qualitative accounts from people with Motor Neurone Disease reflecting the need for more person-centred care

Aoun

O’Brien

Breen

et al. 2018

Journal of the Neurological Sciences

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The diagnosis of Motor Neurone Disease (MND) is devastating for people with MND (PwMND) and their families. The objective of this study is to describe the experiences of PwMND in receiving the diagnosis in order to inform a more person-centred approach to communicating such bad news. The design was an anonymous postal survey facilitated by all MND associations across Australia (2014-15). Survey questions centred on the SPIKES protocol for communicating bad news; each question contained an area for written responses, which were thematically analysed for content. Two hundred and forty-eight responses were received from people with MND (29% response rate). Four themes emerged: challenges in being diagnosed with MND; the emotions experienced; the good and the bad; and links to further information and support. Receiving such a diagnosis requires preparation, forethought, sensitive and individualised care on the part of the neurologist, including where and how the diagnosis is given; the supports required; and timing, amounts and sources of giving information. The emotional reactions of the neurologist also caused a lasting impression on those receiving the diagnosis. This study could form the basis for best practice programs implementing a more person-centred approach to caring for PwMND right from the diagnosis stage. The focus needs to shift on the person's values, preferences, psychosocial and existential concerns in the context of the underlying disease experience and the manner clinical practice is delivered.

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Section: Discussionmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

‘The shock of diagnosis’: Qualitative accounts from people with Motor Neurone Disease reflecting the need for more person-centred care

Aoun

O’Brien

Breen

et al. 2018

Journal of the Neurological Sciences

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“…Currently, both the AAN and EFNS recommend that Riluzole be initiated as early as possible (Andersen et al, 2005;Miller et al, 2009a). Riluzole is the only FDA-approved medication that has been demonstrated to have a modest effect on survival in ALS (i.e., extended survival for approximately 3 additional months).…”

Section: Medical Intervention and Pharmacologic Treatmentmentioning

confidence: 99%

What is ALS and What is the Philosophy of Care?

Britton

Cleary

Miller

2013

Perspect Swal Swal Dis (Dysph)

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have no financial or nonfinancial relationships related to the content of this article. Amyotrophic lateral sclerosis (ALS) is a rapidly degenerative disease involving upper motor neuron (UMN) and lower motor neuron (LMN) impairments, for which there is currently no cure. It is necessary for speech-language pathologists to understand the underlying neurological pathophysiology and philosophy of care for individuals with ALS in order to facilitate effective assessment and intervention in this population. The authors of this article review the characteristics of ALS, as well as the general philosophy of care for individuals with ALS. The article covers the topics of assessment and intervention within the context of a multidisciplinary ALS clinic; surgical and pharmacological interventions; and monitoring disease progression, including tracking respiratory status. Periodic multidisciplinary assessment aids in appropriate medical and therapeutic interventions, symptom management, and quality of life. Periodic assessment of respiratory function is especially important to aid in timely delivery of appropriate interventions. Amyotrophic lateral sclerosis (ALS) is a degenerative motor neuron disease for which there is currently no cure. The cause of ALS is not known, and survival is, on average, approximately 3 years following symptom onset (Corcia & Gordon, 2012). The purpose of this article is to summarize the characteristics of ALS and related types of motor neuron disease (MND), as well as the current philosophy of care for individuals with ALS. Characteristics of ALS ALS is a rapidly degenerative disease involving motor neurons in the brain, brainstem, and spinal cord. It is commonly known as Lou Gehrig's disease in the United States, motor neuron disease (MND) in Great Britain, and Charot's disease in France. Typically, the first symptom is weakness; about two-thirds of patients first experience spinal muscle weakness in either the upper or lower extremities, while one-quarter of patients have bulbar weakness and initial dysarthria and dysphagia. Respiratory symptoms are rarely a first sign of disease (Gautier et al., 2010), yet breathing difficulties are a serious complication for almost all patients with ALS, regardless of whether primary weakness is spinal or bulbar.

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“…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.…”

Section: Introductionmentioning

confidence: 99%

“…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].…”

Section: Introductionmentioning

confidence: 99%

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

McClellan¹,

Washington²,

Ruff³

et al. 2013

JRRD

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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.

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Amyotrophic Lateral Sclerosis

Cited by 5 publications

References 101 publications

‘The shock of diagnosis’: Qualitative accounts from people with Motor Neurone Disease reflecting the need for more person-centred care

‘The shock of diagnosis’: Qualitative accounts from people with Motor Neurone Disease reflecting the need for more person-centred care

What is ALS and What is the Philosophy of Care?

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

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