Communication of the diagnosis and information relating to ALS should be tailored to the needs of the patient and their family. Not all patients will want detailed information about prognosis straight away. Patients should be asked about their preferences for involving their family members and/or other caregivers. Both verbal and written information about symptoms, management (especially nutritional and respiratory support), and specific treatments should be provided.[11]Shoesmith C, Abrahao A, Benstead T, et al. Canadian best practice recommendations for the management of amyotrophic lateral sclerosis. CMAJ. 2020 Nov 16;192(46):E1453-68.
https://www.cmaj.ca/content/192/46/E1453
http://www.ncbi.nlm.nih.gov/pubmed/33199452?tool=bestpractice.com
[36]National Institute for Health and Care Excellence. Motor neurone disease: assessment and management. Jul 2019 [internet publication].
https://www.nice.org.uk/guidance/ng42
[63]van Eenennaam RM, Kruithof WJ, van Es MA, et al. Discussing personalized prognosis in amyotrophic lateral sclerosis: development of a communication guide. BMC Neurol. 2020 Dec 14;20(1):446.
https://bmcneurol.biomedcentral.com/articles/10.1186/s12883-020-02004-8
http://www.ncbi.nlm.nih.gov/pubmed/33308184?tool=bestpractice.com
[72]Khan A, Frazer-Green L, Amin R, et al. Respiratory management of patients with neuromuscular weakness: an American College of Chest Physicians clinical practice guideline and expert panel report. Chest. 2023 Mar 13 [Epub ahead of print].
https://journal.chestnet.org/article/S0012-3692(23)00353-7/fulltext
http://www.ncbi.nlm.nih.gov/pubmed/36921894?tool=bestpractice.com
All patients with ALS should be offered genetic testing, and both pretest and post-test counseling. Pretest counseling should cover personalized risk assessments and the range of possible outcomes, and prepare the patient for possible personal, psychological, and economic impacts of testing on themselves and family members. Post-test counseling allows the patient to discuss their result and the implications for them and their family.[24]Roggenbuck J, Eubank BHF, Wright J, et al. Evidence-based consensus guidelines for ALS genetic testing and counseling. Ann Clin Transl Neurol. 2023 Nov;10(11):2074-91.
https://www.ncbi.nlm.nih.gov/pmc/articles/PMC10646996
http://www.ncbi.nlm.nih.gov/pubmed/37691292?tool=bestpractice.com
Advance directives and wishes for end-of-life care should be discussed with the patient and family/caregivers as early as possible (but only when they are ready to do so), and long before hospice care is needed. Ongoing discussion of advanced care planning should be part of regular follow-up.[11]Shoesmith C, Abrahao A, Benstead T, et al. Canadian best practice recommendations for the management of amyotrophic lateral sclerosis. CMAJ. 2020 Nov 16;192(46):E1453-68.
https://www.cmaj.ca/content/192/46/E1453
http://www.ncbi.nlm.nih.gov/pubmed/33199452?tool=bestpractice.com
[36]National Institute for Health and Care Excellence. Motor neurone disease: assessment and management. Jul 2019 [internet publication].
https://www.nice.org.uk/guidance/ng42
[62]Taylor LP, Besbris JM, Graf WD, et al. Clinical guidance in neuropalliative care: an AAN position statement. Neurology. 2022 Mar 8;98(10):409-16.
https://n.neurology.org/content/98/10/409
http://www.ncbi.nlm.nih.gov/pubmed/35256519?tool=bestpractice.com
Patients who have feeding tubes and their caregivers should be instructed in their use: for example, how to prevent clogging.[129]Farrag K, Shastri YM, Beilenhoff U, et al. Percutaneous endoscopic gastrostomy (PEG): a practical approach for long term management. BMJ. 2019 Jan 22;364:k5311.
http://www.ncbi.nlm.nih.gov/pubmed/30670385?tool=bestpractice.com
The patient, and their family members and other caregivers, should be advised to seek immediate medical evaluation if the patient shows symptoms of severe respiratory dysfunction, especially when associated with fever.
Online information from recommended sources may be helpful for patients and families.
MDA (Muscular Dystrophy Association): ALS division
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