Respiratory Management of ALS: Emerging Need (2009 AAN Practice Parameter) Lee Guion, MA RRT

1. Respiratory Management of ALS: Emerging Need(2009 AAN Practice Parameter)Lee Guion, MA RRT Forbes Norris MDA/ALS Research Center, San Francisco, CA GuionL@sutterhealth.org

2. Have You Seen Me?

3. 63 year old woman admitted to ED Change in mentation, poorly responsive Respiratory insufficiency following sedation for routine out-patient colonoscopy. Past medical history: -- cigarette smoking (20 pack years. Quit 15 years ago.); hypertension; and type II diabetes, both well controlled. Reports losing fine motor movement in right hand, with increasing difficulty writing and brushing teeth. Ambulates with walker. Diagnosed with mild CVA. Intubated and ventilated for airway protection. Unable to wean from MV. MIP < 20 cmH2O; Vt 200 mL, RR 36 on t-piece trials. No active respiratory disease process. After one week, tracheotomy performed and moved to long-term ventilator facility. Have you seen this?

4. Have You Seen Me?

5. Have You Seen Me?

6. 54 year old man admitted to med/surg unit after seeking treatment in urgent care for increasing shortness of breath and inability to mobilize airway secretions. Chest radiograph = LLL infiltrate, poor lung expansion, depressed diaphragm. No history of smoking, asthma, occupational exposure to lung irritants Prescribed aerosolized bronchodilator therapy and CPT. Diagnosed with foot drop and peripheral neuropathy 6 months prior. Impaired mobility. SpO2 = 85%. Administered supplemental oxygen 4 LPM to keep SpO2 > 95% (per order set). Initial relief of dyspnea. RT called to bedside 4 hours later when patient somnolent and unresponsive. Intubated and ventilated. Unable to wean from mechanical ventilation (MV). Eventually trached and sent home on MV. Have you seen this?

7. Have You Seen Me?

8. 42 year old woman brought to ED by family members due to inability to swallow, 40 pound weigh loss over a year. Excessive saliva (drooling). Cachetic, mute, but without complaints of shortness of breath. Diagnosis one year prior = progressive bulbar palsy. Admitted for hydration and nutrition therapy via feeding tube. Consented to PEG. Following this procedure patient developed respiratory discomfort & hypoventilation. SpO2 86%. Supplemental oxygen administered. Improvement in symptoms. Transferred to MICU. Developed acute respiratory failure 6 hours later. PCO2 78, pH 7.28, HCO3- 30 (acute RF imposed upon chronic RF). Intubated, ventilated. Expired 3 hours later. Have you seen this?

9. Have You Seen Me?

10. People with ALS (PALS)At various stages of the disease Lower extremity weakness (legs and feet) Upper extremity weakness (arm, hands) Bulbar muscle weakness (loss of speech, swallowing)

11. What do these scenarios have in common? • Unexpected decline in respiratory status • Undetected compensated chronic respiratory failure • Emergency intubation • Failure to wean from mechanical ventilation

12. What do these scenarios have in common? Unrecognized, and therefore, poorly managed neuromuscular disease (ALS)

13. Factors precipitating acute respiratory failure in ALS • sedation/respiratory suppressant medication; • supine position, lung volume displacement, loss of accessory muscle use; • acute respiratory infection with chronic respiratory insufficiency; • surgical procedure, post-op pain, splinting; • ineffective cough;

14. Factors precipitating acute respiratory failure in ALS • loss of upper airway function masking lower airway dysfunction; • poor oral intake masking respiratory hypermetabolic state; • chronic, silent aspiration.

15. Perfect storm for ventilatory failure in ALS: • Misdiagnosis when neurological symptoms first appear • Not referred to a neuromuscular clinic for evaluation and management • Slow disease progression • Gradual respiratory compensation for diaphragm, intercostals, and abdominal muscle weakness • Lack of subjective symptoms due to loss of mobility, acclimation, denial • Hospital medical personnel unfamiliar with neuromuscular diseases (ALS) and treatment guidelines

16. Definition of ALS: • Amyotrophic lateral sclerosis (Lou Gehrig’s disease) • Relentlessly progressive paralyzing disease of the motor neurons. • Most patients die within 2 to 5 years of symptom onset. • Focus of treatment is symptom management.

17. Symptom onset: • Limb onset (unilateral, upper or lower) • Bulbar muscle onset (speech or swallowing) • Diaphragm onset (rare) • Slowly progressing primary lateral sclerosis (PLS), progressive muscle atrophy (PMA), and bulbar palsy can progress to ALS • All patients will develop lung muscle weakness and paralysis • Majority of patients die of respiratory failure

18. ALS: not a pulmonary disease but a ventilation pump disease • Alveoli and lung parychema are healthy • Loss of innervation of muscles of inhalation and exhalation • CO2 retention and oxygen desaturation due to hypoventilation • Rapid/shallow breathing and increased caloric expenditure • Lack of ventilatory reserves/risk of acute respiratory decompensation

19. Pulmonary infection risk: • Poor glottic function • Decreased muscle tone in upper airway • Risk of aspiration • Decreased lung expansion • Weakened abdominal muscles • Weak, ineffective cough

20. Practice Guidelines Evidence-based statements to assist practitioner and patient make smart decisions about health care 2009 American Academy of Neurology Practice Parameter for Care of ALS

21. AAN Guidelines Are Evidence-based Consensus-based Evidence-based ?

22. Rating the Evidence Weak Strong Randomized Masked Trial SingleCase Report Class I Class II Class IIIClass IV Level A  B  C  U

23. Most Important Findings 10 Class I, 13 Class II, 73 Class III Role of multidisciplinary ALS clinics Riluzole - safety and modest efficacy Respiratory assistive devices (NIV) Nutrition and feeding tubes (PEG) Treatment for drooling Screening for thinking trouble and personality change Telling the diagnosis, treating symptoms, and end-of-life issues - insufficient evidence

24. Analysis of Evidence, Conclusions, Recommendations What is the effect of a feeding tube (PEG) on stabilizing weight and prolonging life? Conclusion: Nutrition via PEG is probably effective in stabilizing body weight (two Class II, seven Class III studies) and in prolonging life (two Class II studies). Recommendation: In patients with ALS losing weight, PEG should be considered to stabilize body weight (Level B) and prolong life (Level B).

25. Analysis of Evidence, Conclusions, Recommendations Does noninvasive ventilation (NIV) at night lengthen life? Conclusions: NIV is probably effective in prolonging life (one Class I, three Class III studies). NIV is probably effective in slowing the rate of breathing decline (one Class I, one Class III study). Recommendation: NIV should be considered to treat respiratory insufficiency in ALS, both to lengthen life and to slow the rate of breathing decline (Level B).

26. Analysis of Evidence, Conclusions, Recommendations How does NIV affect quality of life (QOL)? Conclusions: NIV is possibly effective in raising Quality of life for patients with ALS who have breathing trouble (five Class III studies). Recommendations: NIV may be considered to enhance quality of life in patients with breathing difficulty (Level C).

27. Summary More high-quality studies have been reported leading to more confident recommendations regarding the value of multidisciplinary clinics, riluzole, NIV and PEG. The ALS patient CARE database was developed with the hope of tracking outcomes to raise the standard of care for PALS. Data obtained from the ALS CARE program have shown that many therapies for ALS are underutilized (especially PEG and NIV). Thus an evidence-based practice parameter may over time change practice. The broader use of clinics and treatments is a top priority.

28. Recommendations • Get to know ALS (PALS and the disease) • Think outside the respiratory disease box during patient assessment • Beware of the order set for O2 administration • Participate in patient care rounds and contribute to discussion

29. Recommendations • Contact and utilize RCPs in neurorespiratory specialties • Join the AARC Neuromuscular Roundtable (post questions on line) • Attend our post presentation meeting (Sunday, Dec 6th, 5:10 to 6:10 in Room 101)

30. Have You Seen Me?