Symptom management in ALS. David Walk, M.D. Medical Director ALS Certified Center of Excellence University of Minnesota Health

Transcription

1 Symptom management in ALS David Walk, M.D. Medical Director ALS Certified Center of Excellence University of Minnesota Health

2 Outline of today s points Overview Disease state Clinic team Assessment Functional status Ventilatory status Symptom management

3 ALS: overview

4 ALS: disease overview About 20,000 people living with ALS in the US Average age of onset about 60 About 60% of patients are men

5 ALS: Disease overview motor neuron disease Upper motor neuron signs Spasticity Hyperreflexia Clumsiness UMN pattern weakness Strained, strangled speech Lower motor neuron signs Flaccid tone Atrophy Weakness Fasciculations Breathy, hypophonic speech

6 ALS: disease overview

7 ALS: Disease overview Ravits, La Sapda, Neurology 2009

8 Cognitive and behavioral impairment: Edinburgh Cognitive Assessment domains Abrahams et al, Amyotr Lat Scler and Frontotemporal Dementia 2014;15:9-14.

9 ALS: a whole-brain disorder? Based on Brettschneider et al, Ann Neurol 2013;74:20-38.

10 ALS symptoms UMN symptoms: clumsiness, spasticity LMN symptoms: weakness, dyspnea, fasciculations Bulbar symptoms Dysarthria, sialorrhea, dysphagia, PBA Cognitive and behavioral symptoms Dysexecutive syndrome Language disorders Behavioral change

11 Multidimensional aspect of clinical presentation Bulbar-limb Cog-Behav UMN-LMN

12 American Academy of Neurology ALS Quality Measures 1. Multidisciplinary care plan 2. Disease-modifying pharmacotherapy discussed 3. Cognitive and behavioral impairment screening 4. Symptomatic therapy offered 5. Respiratory insufficiency addressed 6. NIV offered 7. Screening for dysphagia, weight loss, and impaired nutrition 8. Nutritional support offered 9. Communication support referral 10.End of life planning assistance 11.ALS falls querying

13 ALS multidisciplinary clinic team training Nurse MD MD PT OT SLP RD RT MSW PhD GC clinic role Care coordination neurologist pulmonologist Gait, falls, wheeled mobility, functional assessment ADLs, functional and cognitive assessment Swallowing, speech, functional and cognitive assessment Nutritional management PFTs and respiratory education Counseling, social work, help with advanced directives Counseling Genetic counseling, MDA database MDA health care services coordinator ALS Association representative

14 Clinical assessment

15 Assessment in the clinical setting: ALSFRS-R

16 ALSFRS-R as a guide to progression and care needs

17 Assessment in the clinical setting: ventilatory parameters

18 ALS: symptom management

19 Major categories of today s discussion of symptom management Bulbar and nutrition Ventilation, sleep, and energy Muscle symptoms Cognitive, behavioral, mood Pain Social and existential Not covered here: disability management: ADLs, mobility, communication

20 Pseudobulbar affect Dextromethorphan/Quinidine (Nuedexta) Amitriptyline Occasionally SSRIs

21 Sialorrhea Anticholinergics Glycopyrrolate: short acting, well-tolerated, titratable Amitriptyline: long-acting, can help with sleep, mood, and pain Atropine drops: go where the problem is Scopolamine: set it and forget it, but limited tolerance and AEs Hyoscyamine Botulinum toxin to parotids, submandibular glands No systemic AEs 3 month DUA Risk of exacerbating dysphagia Radiation therapy No systemic AEs Permanent fix

22 Phlegm, mucus and upper airway symptoms Possible cause Reflux Post-prandial cough due to silent aspiration Tracheobronchial secretions Excess saliva Post-nasal secretions Laryngospasm Pneumonia management Upright after meals, omeprazole Dysphagia diet, gastrostomy Manually assisted cough, cough assist, lung volume recruitment, guafenisin Saliva management ENT assessment SLP management (training) Urgent medical evaluation Not always easy to diagnose or treat!

23 Dysphagia Assessments Bedside assessment VFSS EAT-10 Interventions Dysphagia diet Gastrostomy

24 Gastrostomy Green light: FVC > 50% Yellow light: FVC 30-50% Red light: FVC < 30% RIG probably safest if no contraindications

25 Total daily energy expenditure estimates based upon H-B + ALSFRS-6 (only one approach to estimating caloric needs) BMR calculation for men BMR = 66 + ( 13.7 x weight in kg ) + ( 5 x height in cm ) - ( 6.76 x age in years ) BMR calculation for women BMR = ( 9.6 x weight in kg ) + ( 1.8 x height in cm ) - ( 4.7 x age in years ) TDEE = BMR from H-B equation + (55.96 x ALSFRS-6 score) -168 From Rup Tandan, M.D.

26 Ventilation, sleep, and energy Parameters for NIV FVC <50%, MIP < 60, or symptoms of noc ventilatory dysfunction Supine FVC, nasal insp pressure (sniff) used when applicable SaO2 < 88% rarely relevant in ALS NIV is sometimes poorly tolerated in bulbar-onset disease Try different masks, nasal pillows, daytime trials Secretion management (see above phlegm, mucus, and saliva) Poor sleep hygiene Energy conservation Exhausted after toileting, shower, dressing! Use the power w/c

27 Spasticity Stretching Oral baclofen Tizanidine Dantrolene Intrathecal baclofen Botulinum toxin

28 Cramps Vitamin E Magnesium oxide Mexiletine Hot Shot Quinine

29 Mood, cognitive, and behavioral problems Symptom management Depression: counseling SSRI Anxiety: SSRI benzodiazepines (caution) Behavioral: Family strategies Quetiapine Low-dose olanzapine Safety management CPT Driver s evaluation Less practical in ALS because of rate of progression Behavioral health evaluation

30 ALS Cognitive Behavioral Screen (ALS CBS) Woolley et al, Amyotroph Lat Scler 2010; 11: Susan Woolley

31 Pain Immobility musculoskeletal pain Adhesive capsulitis Neuropathic pain is uncommon

32 Social and existential considerations Social considerations Isolation Loss of life roles Changed relationships Am I a burden Existential considerations Life goals achieved? Quality of life? Sources of pleasure (physical/cognitive) and self-worth?

33 Multidisciplinary care in ALS Problem Management Expert team member Ventilatory impairment Non-invasive ventilation Respiratory therapist Tracheostomywithmechanical ventilation Pulmonologist Weak cough Manually assisted cough Long volume recruitment Mechanically assisted cough (insufflator/exsufflator) Respiratory therapist Dysarthria Augmentativecommunicationdevices Speech/language pathologist Dysphagia Sialorrhea Dietary modifications Gastrostomy Anticholinergic medications Botulinum toxin to salivary glands Radiation therapy to salivary glands Speech/language pathologist Neurologist Weight loss High-calorie, high fat diet Estimating caloric needs based upon activity Registered dietician Impaired ADLs Adaptations, equipment Occupational therapist Impaired mobility and falls Gait aids, wheelchair, power chair Physical therapist Spasticity Stretching, oral baclofen, tizanidine, dantrolene, and intrathecal baclofen Neurologist or Physical Medicine & Rehabiliation specialist Pain Physical therapy, medications Neurologist, physical therapist, pain medicine specialist Anxiety,depression, and changing life roles Counseling,medications Psychologist, Neurologist Pseudobulbar affect dextromethorphan/quinidine(nuedexta ), amitriptyline Neurologist Cognitiveandbehavioral impairment, dementia Screening instruments, diagnostic instruments, safety evaluations Occupational therapist, neuropsychologist Life planning Financial counseling, advanceddirective, POLST Social worker, attorney, neurologist Clinical research Discussion, on-line resources Neurologist, research coordinator, patient advocacy group representative

34 Thank you