CDM 4 Exam 2 Review ALS I What is ALS Article 1 A Amyotrophic lateral sclerosis a neurodegenerative disease characterized by a loss of motor neurons in the spinal cord brainstem and motor cortex 1 Cause of the disease is unknown 2 Disease is incurable however there are a number of important therapies available 3 Relentlessly progressive paralyzing disease most patients die within 2 5 years of onset 4 Mainstay of treatments are symptomatic treatment and palliative care B AAN evidence based practice parameters for managing patients with ALS C Only one drug available riluzole 50 mg BID safe and effective for slowing disease progression to a modest degree in ALS SHOULD BE OFFERED 1 Lithium carbonate there is inadequate data on the effectiveness of Lithium carbonate insufficient data at this time to support or refute treatment with lithium carbonate in ALS pts D Nutrition in ALS factors that restrict adequate nutrition develop insidiously and progressively worsen functional consequences are dysphagia swallowing dysfunction choking aspiration weight loss and dehydration 1 Strategies to maintain oral nutritional Intake altering food consistency using nutritional supplements PEG percutaneous endoscopic gastronomy or equivalent device may be needed as an alternative route for delivering nutrition PEG does NOT eliminate oral feeding but is an alternative method for administering medication and fluid as well as stabilizing weight 2 Enteral nutrition administered via PEG is probably effective in stabilizing body weight body mass index PEG is probably effective in prolonging survival in ALS PEG SHOULD be considered for prolonging survival in pts with ALS 3 Creatine 5 10 g daily is ineffective in slowing the rate of progression or in improving survival in ALS DO NOT USE 4 Vitamin E 5 000 mg day plus riluzole is probably ineffective in improving survival or functional outcomes Vitamin E 1000 mg day plus riluzole was marginally effective in slowing the progression of ALS from milder to more sever ALS health states using a single measure DO NOT use high dose Low does permits no recommendation E Respiratory management Critical because moth deaths from ALS are due to respiratory failure 1 Nocturmal oximetry and MIP maximal inspiratory pressure are possibly more effective in detecting early respiratory insufficiency than erect FVC forced vital capacity consider Nocturmal oximetry may be considered to detect hypoventilation 2 Supine FVC erect FVC in detecting diaphragm weakness and correlates better with symptoms of nocturnal hypoventilation Consider FVC and MIP plus erect FVC for respiratory monitoring 3 SNP stiff nasal pressure may be considered to detect hypercapnia and nocturnal hypoxemia possibly effective 4 NIV noninvasive ventilation may be considered to enhance QOL in ALS with respiratory insufficiency possibly effective may be considered at the earliest sign of nocturnal hypoventilation or respiratory insufficiency in order to improve compliance with NIV in patients with ALS 5 TIV tracheostomy invasive ventilation may be considered to preserve QOL in patients with ALS who want long term ventilatory support possibly effective but greater caregiver burden 6 MIE mechanical insufflation exsufflation may be considered to clear secretions in patients with ALS who have reduced peak cough flow particularly during an acute chest infection possibly effective 1 II Breaking the news support Article 2 7 Insufficient data to support or refute HFCWO high frequency chest wall oscillation for clearing airway secretions in patients with ALS 8 Medications with mucolytics like guifenisen N acetylcystine BBlocker nebulized saline or an anticholinergic bronchodilator like ipratropium are widely use however no controlled studies exist in ALS A Not enough evidence to support or refute any specific method of disclosing an ALS diagnosis 1 Useful strategies have been developed for disclosing a diagnosis of cancer that may be used B Specialized multidisciplinary clinic referral should be considered for patients with ALS to optimize health care delivery and prolong survival and may be considered to enhance QOL C Sialorrhea drooling from ALS is embarrassing and associated with aspiration pneumonia 1 Anticholinergic medications are used 1st line to reduce sialorrhea although effectiveness is unproven ALS 2 Botulinium toxin has been effective in controlled trials to treat drooling in parkinsons and D Pseudobulbar affect excessive laughing crying or involuntary emotional expression disorder 1 Dextromethorphan Quinidine 30mgDM 30 mg Q BID if approved by the FDA with tolerable ADE should be considered to treat the symptoms of pseudobulbar affect E Fatigue is common in patients taking riluzole 1 Once the risks of fatigue vs modest survival benefits of riluzole have been discussed withholding the drug may be considered F Cramps insufficient data to support or refute any specific intervention for treatment of cramps in ALS studies of gabapentin vitamin E and riluzole for treating cramps were all negative G Spasticity might improve gait and relieve painful spasms insufficient data to support or refute exercise or medication for treating spasticity in ALS 1 In MS cerebral palsy BZDs baclofen dantrolene and tizanidine are effective in reducing spasticity related symptoms H Depression Anxiety insomnia Insufficient data to support or refute specific treatments in depression anxiety and insomnia 1 There is a consensus among experts that depression SHOULD be treated however I Cognitive and behavioral impairment a significant proportion of patients with ALS demonstrate cognitive impairment and some have dementia Screening for these should be considered 1 Screening tests of executive function may be considered to detect cognitive impairment in patients with ALS prior to confirmation with formal neuropsychological evaluation 2 CAM Mini cog MoMCA MMSE BMSE CDR FAST 3 4 Impact of impairment is unknown Insufficient data to support or refute treatment NIV may be used J Communication Insufficient data to support or refute treatment to optimize communication Strategies used include alphabet board computerized systems morse code utilization of the anal sphincter and infared eye movements K Palliative care lots of pain and a strong desire for physician0assisted suicide in late stage ALS insufficient data on exact treatment Insufficient data to support or refute hospice spiritial care or advance directives 1 What is the optimal method of