HOME MECHANICAL VENTILATION: HMV GUIDELINES

1. HOME MECHANICAL VENTILATION: HMV GUIDELINES

2. Committee Members • Doug McKim and Jeremy Road : Co-chairs • Nigel Duguid • Debra Morrison • Colleen O’Connell • Francois Maltais • Fabien Cote • Basil Petrof • Monica Avendano, Steve Abdool and Janet Fraser • Robert Skomro • Karen Rimmer • Methodologist Tom Oliver • Observer Ian MacLusky (Paeds) • Admin. Assistance CTS • Not one have a COI with material to be presented .

3. Committee formed in 2008 • Evolution of technology • Emerging clinical experience • Care gaps across the country, around the world • Absence of CPG • Significant expense • Ethical issues

4. EUROVENT STUDY (ERJ , 2005) • 16 countries • 483 centers • Wide variation in patients on HMV • Invasive v non invasive • “Lung users” < 1 year ; neuromuscular > 6 years

5. CTS HMV Guidelines: Disease Specific Eleven of the areas of most significance for those requiring Prolonged Assisted Ventilation (PAV) and addressed by the guidelines were as follows: • * Airway Clearance in Ventilator Assisted Individuals • * Transition to Home • Amyotrophic Lateral Sclerosis • Central Hypoventilation Syndrome • Stable COPD • Kyphoscoliosis • Obesity Hypoventilation Syndrome • Spinal Cord Injury • Duchenne Muscular Dystrophy • Muscular Dystrophies other than Duchenne and Myopathies • * Ethical Considerations • First CPG on HMV to adapt this process

6. Methodology • Guideline development • AGREE II , GRADE/Grading recommendations table from the ACCP report, Judging the Strength of Recommendations Consensus-building process, external review • Literature Search Strategy • MEDLINE (OVID: 1980 through June 2010), EMBASE OVID: (1980 through June 2010), HealthStar (1980 through June 2010), the Cochrane Library (OVID; Issue 1, 2009), the Canadian Medical Association InfoBase, and the National Guideline Clearinghouse • Study selection criteria • outcomes of interest: survival, pulmonary function, sleep parameters, airway clearance techniques, VAI and caregiver quality of life, transition to home, ethical considerations

7. Guideline Development Process • Grading system ( GRADE , Guyatt et al Chest 2006) • Quality of evidence : A,B or C • Strength of recommendations 1 or 2 • Consensus building meetings • Established key research questions for the future. • External review process: international experts, Canadian partners including Spinal Cord Network, ALS and Muscular Dystrophy Societies and CRTS.

8. External Reviewers Comments • Strong support for the document • AGREE II scores were high • 2 concerns: procedure for updating guideline and opinions of patients were not sought • Some wanted more information on “how to” • Some suggestions re : recommendations and levels of evidence .

9. Guideline Development Process • Compendium (source document). • Executive summary includes • Synopsis of section • Conclusions • Recommendations with levels of evidence

10. Collaboration with LTMV Canadian Community of Excellence Steering Group • Initial meeting of working group in 2010 • CIHR funded program, April 2011. “Understanding LTMV in Canada: A Programmatic Approach” . PI : Louise Rose.

11. Dissemination and Implementation • Publication in 2011 • Target groups with shared interest • Collaboration with LTMV group • Research opportunities

12. Central Hypoventilation Syndrome (CHS) • Rare condition • Decreased respiratory drive • Varied etiology

13. Etiology of CHS

14. In pediatric population 4 retrospective chart reviews (n=6-196) • In adults mostly case reports on acquired causes of CHS

15. In pediatric population with Congenital Central Hypoventilation Syndrome ( CCHS) 90% have the PHOX2B mutation • Late onset , LO-CCHS in adults can be associated with the PHOX2B mutation as well, less severe than pediatric presentation • First degree relatives should be screened as carriers can be affected

16. Recommendations • 1. The diagnosis of CHS is best established by polysomnography supplemented with transcutaneous CO2 or early morning ABGs. (GRADE 1C) • 2. Once the diagnosis of CHS is established, it is strongly recommended that acquired causes should be excluded by MRI of the brainstem. (GRADE 1C) • 3. Patients with CHS and no known cause should undergo genetic screening for the PHOX2B gene mutation. (GRADE 1C) • 4. For patients confirmed to have PHOX2B mutation, first degree relatives should be screened for hypoventilation. (GRADE 1C) • 5. CHS patients who require only nocturnal ventilatory support may be managed by NIV with a back up rate or diaphragmatic pacing. (GRADE 1C) • 6. Severe CHS mainly seen in C-CHS requires continuous ventilatory support but daytime diaphragmatic pacing can markedly improve mobility. (GRADE 1C)

17. COPD • RCT ’s of > 3 months duration selected , 7 found. • Clinical practice guidelines from CTS,ATS/ERS and GOLD consulted • 2 systematic reviews • Outcomes : HRQL, Dyspnea, Exercise Capacity, Sleep, Hospitalization , Mortality.

21. Dyspnea- weak evidence • HRQL-conflicting results • Exercise capacity-2 trials showed increase in 6MWD 53 and 13m,one no change • Sleep-conflicting results • Mortality-2 trials no benefit and 1 a benefit (HR 0.63 , 0.4-0.99)

22. Difficult studies to perform ,ill patients with high drop out rates and non adherence to therapy rates of 15-40%. • In Eurovent study COPD is common indication and use increasing in some countries.

23. Recommendations • The use of long-term NIV cannot be widely recommended in patients with stable COPD. (GRADE 1B) • Long-term NIV in COPD should only be considered on an individual basis. One subgroup of patients with COPD in which long-term NIV could be considered are patients with severe hypercapnia (e.g. PaCO2 greater than 55 mmHG) experiencing repeated episodes of acute hypercapnic respiratory failure that require in-hospital ventilatory support. However, definitive proof of efficacy of long-term NIV in these patients will need to await further studies. (GRADE 2C) • The overlap syndrome (concomitant COPD and obstructive sleep apnea syndrome) should be differentiated from chronic respiratory failure that is solely due to advanced COPD. (GRADE 1B)

24. Duchenne Muscular Dystrophy (DMD) • Studies selected included outcomes of : survival, HRQL, hospitalizations, PFTs and ABGs. • 11 observational studies (n=10-42) • 8 retrospective chart reviews (n=15-243) • 2 small RCTs (n= 60)

25. Absence of dystrophin • Decline in VC 8-12% per year • VC < 40% or MIP < 30 cm H20,at risk for hypoventilation • Diurnal hypercapnia = high one year mortality and reduced HRQL • FVC<1L. 5 year survival is 8%.

26. Largest RCT Rafael et al (Lancet 1994,343:1600) • Does early NIV improve survival (n= 35 in each arm) • Mortality > in NIV ( 8v2 ) study stopped • Problems : infections managed at home, groups not controlled for cardiac disease, infrequent follow up and airway clearance techniques were sub optimal.

27. Vianello et al (Chest 1994,105:445) • 10 DMD patients with daytime hypercapnia ,followed for 2 years matched by VC and comorbidity. • 5 refused NIV 4 died, mean survival 9.7 months • NIV group 100 % survived at 2 years

28. Danish experience • 243 cases (retrospective chart review) • 1988-2002 vent use increased from 0.9-43% • Mortality rate 3.8 v 9.8% in vent users • 2,4,6 year survival 93,91 and 81% in vent users

29. Japanese experience • 157 DMD • Mean age at death 31 yrs in vented group v 20 yrs .

30. ABGs improve : from pCO2 60 to 45 mmHg on NIV • HRQL improves

31. Recommendations for Monitoring • Carefully question and educate patients to report symptoms consistent with hypoventilation, including disturbed sleep, excessive daytime sleepiness, headache and weight loss. (GRADE 1B) • Measure VC, MIP, MEP, peak cough flow, and awake oxyhemoglobin saturation by pulse oximetry at least yearly; if VC < 40% predicted, also monitor awake CO2 tension by non-invasive methods or blood gas analysis. (GRADE 1B) • Perform an evaluation of respiration during sleep if there are symptoms consistent with nocturnal hypoventilation or other forms of sleep-disordered breathing (1B). In the absence of such symptoms, periodic screening for sleep-disordered breathing should also be considered once FEV1 or FVC < 40% predicted. (GRADE 2B)

32. Recommendations for Treatment • Offer nocturnal NIV to patients with diurnal hypercapnia (daytime arterial pCO2 > 45 mmHg), or when there is documented nocturnal hypercapnia and the presence of symptoms consistent with hypoventilation. (GRADE 1B) • Institution of NIV during sleep should also be strongly considered in patients demonstrating a major degree of nocturnal gas exchange disturbance, even if asymptomatic. (GRADE 1C) • When bilevel ventilation is used, back up respiratory rates are recommended during sleep while on NIV to reduce the work of breathing associated with spontaneous breath initiation (Grade 1C)

33. Individualize the decision about the transition from nocturnal NIV to daytime ventilation by carefully evaluating patient factors (symptoms, bulbar involvement, patient preference, etc.) and available resources. In patients requiring daytime ventilation, strongly consider NIV as an alternative to invasive tracheostomy. (GRADE 1B) • Lung volume recruitment maneuvers should be introduced with declining vital capacity. (see Airway Clearance section) (GRADE 1C) • Methods to assist secretion clearance should be initiated when peak cough flow is less than 270 l/min.(see Airway Clearance section) (GRADE 1C)

34. Thank you