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Long-Term Noninvasive Ventilation in the Geneva Lake Area: Indications, Prevalence, and Modalities

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Long-Term Noninvasive Ventilation in the Geneva Lake Area:

Indications, Prevalence, and Modalities

CANTERO, Chloé, et al.

Abstract

Noninvasive ventilation (NIV) is standard of care for chronic hypercapnic respiratory failure, but indications, devices, and ventilatory modes are in constant evolution.

CANTERO, Chloé, et al . Long-Term Noninvasive Ventilation in the Geneva Lake Area:

Indications, Prevalence, and Modalities. Chest , 2020, vol. 158, no. 1, p. 279-291

DOI : 10.1016/j.chest.2020.02.064 PMID : 32243941

Available at:

http://archive-ouverte.unige.ch/unige:144320

Disclaimer: layout of this document may differ from the published version.

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Online Supplement

Long-Term Noninvasive Ventilation in the Geneva Lake Area

Indications, Prevalence, and Modalities

Chloé Cantero, MD; Dan Adler, MD; Patrick Pasquina; Christophe Uldry, MD;

Bernard Egger, MD; Maura Prella, MD; Alain B. Younossian, MD; Paola M. Soccal, MD;

Jean‑Louis Pépin, MD; and Jean‑Paul Janssens, MD

CHEST 2020; 158(1):279-291

(3)

e-Figure 1. Comorbidities and their frequency in 489 patients under home non-invasive ventilation

0 50 100 150 200 250 300 350 400

Treatment with opiates Cerebro-vascular disease Asthma Pulmonary hypertension Chronic heart failure Type II Diabetes Dyslipidemia Anxiety and/or depressive disorder Obesity

Systemic hypertension

334 (68%)

271 (55%)

220 (45%)

209 (43%)

149 (30%)

106 (22%)

86 (18%)

63 (13%)

26 (5%)

17 (3%)

(4)

e-Figure 2. Distribution of daytime PaCO2 (in kPa) without NIV in 371 subjects for whom arterial blood gases without ventilator were available. X axis: cumulative number of cases. Y axis: PaCO2.

0 1 2 3 4 5 6 7 8 9 10 11

1 10 19 28 37 46 55 64 73 82 91 100 109 118 127 136 145 154 163 172 181 190 199 208 217 226 235 244 253 262 271 280 289 298 307 316 325 334 343 352 361 370

n=91 > 6.5 kPa (24.4%)

n=57 > 7.0 kPa (15.3%)

n=158 > 6.0 kPa (42.5%)

Daytime PaCO2(kPa); n=371 values

D ay ti m e Pa C O

2

(k Pa )

Cumulative number of cases

(5)

e-Figure 3. A (left) and B (right). Apnea-hypopnea Index (left) and average daily use of NIV (right) provided by ventilator software, according to interface used.

Facial Nasal

prongs

Nasal Facial Nasal

prongs

Nasal

A ve ra ge d ai ly u se o f N IV ( m in )

A p n e a -h yp o p n e a in d ex p ro vi d e b y ve n ti la to r so ft w ar e (e ve n ts /h o u r)

Facial masks

Nasal pillows

Nasal masks

Facial masks

Nasal pillows

Nasal

masks

(6)

e-Table 1. Details in changes of diagnostic groups under NIV between 2000 (Reference 3) and 2018 (present study).

Diagnostic group 2000 2018 Ratio 2018/2000

COPD 43 190 4.4

OHS 49 127 2.6

NMD 24 77 3.2

RLD 17 22 1.3

KYPH 17 29 1.7

SRBD 4 44 11.0

Total 154 489 3.2

Comment on e-Table 1:

The largest numerical increases in utilization of NIV occurred in the COPD, OHS and NMD groups. Possible contributing factors are: 1/ changes in epidemiology, with an increase in OHS patients as in most Western countries (although changes are modest in Switzerland); 2/ increased awareness of SRBD in the obese population in the primary care setting; 3/ better scientific evidence for the benefits of NIV in COPD and OHS; 4/ improved care for certain NMDs, as for ALS, with implementation of multidisciplinary management groups in both university centers. Patients with RLD and KYPH remained relatively stable. The increase in SRBD patients treated by NIV is secondary to the constant increase in this population (with presently 19’350 CPAP users and 500 patients under ASV in the same area) and represents a very small proportion of the total SRBD population.

COPD: Chronic obstructive pulmonary disease; OHS: Obesity hypoventilation syndrome; NMD:

Neuromuscular disorders; RLD: Restrictive lung disorders; SRBD: sleep-related breathing disorders. See

“Patients and methods” section for definitions of diagnostic groups

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e-Table 2. Detailed diagnoses of patients with neuromuscular disorders (n=79; 16% of total population), other restrictive disorders (n=22, 4% of total) and sleep-related breathing disorders (SRDB; n=42, 9% of total).

Neuromuscular disorders 79

Myopathies 32 (40)

Congenital muscular dystrophies 14

o Duchenne’s muscular dystrophy 6

o Facioscapulohumeral muscular dystrophy (Landouzy-Dejerine) 2

o Other 6

Congenital myopathies 4 (5)

o Nemaline myopathy 2

o Desmin myopathy 2

Myotonic muscular dystrophies 13 (16)

o Steinert’s myotonic muscular dystrophy 9

o Pompe’s disease 3

o Other 1

Amyotrophic lateral sclerosis (ALS) 15 (19)

Phrenic nerve paralysis (unilateral and/or bilateral) 14 (18)

Brainstem or cervical cord injury 6 (8)

Spinal muscular atrophy (SMA) 5 (6)

o Type I 2

o Type II 3

Demyelinating diseases (multiple sclerosis) 4 (5)

Myasthenia gravis 3 (4)

Missing data 1

Other restrictive disorders 22

Post-polio syndrome 9 (41)

Sequellae of thoracic surgery 5 (23)

End-stage pulmonary fibrosis 5 (23)

Bronchiectasis 2 (9)

Sleep related breathing disorders 42

Obstructive sleep apnea syndrome 18

Emergent central sleep apnea syndrome 14

Central sleep apnea syndrome (CSA) 10

o Drug induced CSA 6

o Idiopathic CSA 2

o CSA associated with chronic heart failure 1

o CSA associated with neurological disorders 1

Values expressed as n (%) unless specified otherwise.

(8)

e-Table 3. List of ventilator devices used in the Geneva Lake area.

Ventilator devices All patients

(n=489)

ResMed 388 (79)

S9 VPAP ST 177 (36)

LUMIS 150 115 (23)

STELLAR 100 ou 150 59 (12)

VPAP IV 18 (4)

Astral 150 7 (1)

S9 V-AUTO 7 (1)

AirCurve 10 Vauto 2

VPAP III 2

S8 Auto 25 1

Philips Respironics 89 (18)

BiPAP A40 58 (12)

BiPAP Synchrony II 11 (2)

BiPAP A30 6 (1)

BiPAP System One AVAPS 5 (1)

BiPAP Synchrony II AVAPS 4 (1)

Trilogy 100 3

BiPAP Auto M 1

BiPAP System One ST 1

Lowenstein Medical 11 (2)

PRISMA 30 ST 5 (1)

PRISMA ST 4 (1)

SOMNOvent Auto-ST 1

Ventimotion 1

Air Liquide Medical Systems 1 (1)

Monnal T50 1

Values expressed as n (% of total) unless specified otherwise.

(9)

e-Table 4. Ventilator settings according to modes.

All patients COPD Overlap

Syndrome NMD OHS Kyphoscoliosis Other

RLD SRBD

Number of subjects 489 (100) 135 (28) 55 (11) 79 (16) 127 (26) 29 (6) 22 (4) 42 (9)

Bi-level ventilators, ST mode (n=407)

IPAP (cmH2O)2 18 (16; 21) 18 (16; 20) 20 (18; 22) 15 (13; 18) 21 (18; 24) 17 (16; 20) 18 (16; 20) 17 (15; 18)

EPAP (cmH2O)2 7 (5; 10) 6 (5; 7) 8 (7; 10) 5 (4; 7) 10 (7; 11) 6 (5; 8) 6 (5; 7) 10 (7; 12)

BURR (cycles/min) 3 14 (12; 17) 14 (12; 16) 16 (14; 18) 14 (12; 16) 14 (12; 18) 14 (14; 16) 15 (13; 17) 14 (12; 16) Rise time (msec)4 150 (150; 200) 150 (100; 200) 150 (100; 200) 200 (150; 250) 200 (150; 250) 200 (100; 225) 200 (150; 275) 200 (125; 200) TIMIN (sec)5 0.6 (0.5; 0.8) 0.5 (0.3; 0.6) 0.6 (0.5; 0.8) 0.7 (0.6; 0.8) 0.7 (0.5; 0.8) 0.7 (0.6; 0.8) 0.7 (0.5; 0.7) 0.5 (0.3; 0.7) TIMAX (sec) 5 1.4 (1.2; 1.6) 1.4 (1.1; 1.6) 1.3 (1.2; 1.5) 1.5 (1.2; 1.6) 1.5 (1.2; 1.7) 1.5 (1.3; 1.6) 1.5 (1.3; 1.8) 1.5 (1.3; 2) Bi-level ventilators, volume-targeted modes (n=49)

ResMed devices 29 (6) 12 (9) 4 (7)* 4 (5) * 8 (6) 1 (3) * 0 (0) 0 (0)

Targeted VA (L/min)1 5.2 (5.2; 5.2) 5.2 (5.2; 5.2) - - 5.2 (5.2; 5.5) - - -

Targeted RR (cycles/min) 2 15 (13; 15) 15 (13.5; 15) - - 14 (13; 15) - - -

Minimal PS (cmH2O) 10 (9; 13) 11.5 (10; 14) - - 8 (7.8; 9.3) - - -

Maximal PS (cmH2O) 11 (10; 14) 11.5 (10; 14) - - 9 (8; 10.3) - - -

Minimal EPAP (cmH2O) 6 (4; 10) 6 (4; 8) - - 11.5 (9.5; 12.3) - - -

Maximal EPAP (cmH2O) 10 (8; 14) 10 (7.5; 10.3) - - 15 (14; 16) - - -

Philips Respironicsdevices 20 (4) 5 (4) 3 (5) * 2 (2) * 10 (13) 0 (0) 0 (0) 0 (0)

Targeted VT (ml) 500 (500; 555) 570 (550; 600) - - 500 (500; 500) - - -

Targeted VT/body weight(ml/kg) 5 (4.5; 5.9) 5.1 (5; 6) - - 4.7 (4.2; 5) - - -

Maximal pressure (cmH2O) 24 (22; 27) 25 (24; 30) - - 22 (21; 26) - - -

BURR (cycles/min) 14 (12; 14) 12 (12; 13) - - 14 (14; 14) - - -

Minimal PS (cmH2O) 10 (10; 14) 10 (9; 10) - - 10 (9; 13) - - -

Maximal PS (cmH2O) 12 (10; 16) 13 (12; 20) - - 12 (10; 14) - - -

Minimal EPAP (cmH2O) 8 (6; 8) 8 (6; 8) - - 8 (8; 8) - - -

Maximal EPAP (cmH2O) 12 (10; 12) 12 (12; 12) 12 (11; 12)

(10)

Values listed are the most recent values obtained.

Values expressed as median (IQR) or n (%) unless specified otherwise.

COPD: Chronic obstructive pulmonary disease; OHS: Obesity hypoventilation syndrome; NMD: Neuromuscular disorders; RLD: Restrictive lung disorders; SRBD: sleep-related breathing disorders; ST: Spontaneous-timed mode; IPAP: Inspiratory positive airway pressure; EPAP: Expiratory positive airway pressure; TIMIN: : Minimal inspiratory time; TIMAX: Maximal inspiratory time; VA: Alveolar volume; VT: Tidal volume; RR: Respiratory rate; BURR: Back-up respiratory rate; PS: Pressure support.

*: values not reported because of limited number of patients.

Results are presented separately according to manufacturer of device because settings differ.

Missing data: 1: n=1; 2: n=2; 3: n=3; 4: n=48; 5: n=23

e-Table 5. Data downloaded from ventilator software.

All patients COPD Overlap

Syndrome

NMD OHS Kyphoscoliosis Other

RLD

SRBD Missing

data

Number of subjects 489 (100) 135 (28) 55 (11) 79 (16) 127 (26) 29 (6) 22 (4) 42 (9)

Bi-level positive pressure ventilators, ResMed (n=381; 78% of total) and Lowenstein Medical devices (n=11, 2% of total)

Median leaks (L/min) 1.2 (0; 7.2) 0.5 (0; 7.2) 0 (0; 6.2) 3 (0; 7.3) 1.2 (0; 7.1) 5 (1.1; 17.1) 0 (0; 4.8) 0 (0; 2.4) 31 Median leaks 95th centile

(L/min) 14.4 (3; 30) 16.4 (1.2;

32.6) 7.5 (2.1; 26.3) 16.8 (6; 31.1) 16.8 (4.8;

27.3) 24.3 (5; 35.1) 2.4 (0; 26.4) 10.2 (1.8; 25.2) 27 Median VT (ml) 480 (380;

620)

520 (400; 630) 525 (400; 640) 420 (360; 530) 500 (400; 660) 320 (275; 440) 460 (380; 480) 460 (380; 520) 33 Median VT/weight (ml/kg) 5.7 (4.3; 7.5) 6.8 (5.6; 9.4) 5.3 (4.6; 7.1) 5.7 (4.7; 8.2) 4.5 (3.4; 5.7) 5.9 (4.6; 7.3) 4.9 (3.9; 6.1) 5.1 (3.8; 6.2) 33 Median VE (L/min) 8.6 (6.7; 10.4) 9.3 (7.3; 10.6) 9.1 (8.3; 10.6) 7.3 (6; 8.5) 8.8 (6.9; 11) 5.6 (5; 7.7) 9 (7.5; 10.8) 7.8 (5.9; 9.8) 37

(11)

Bi-level positive pressure ventilators, Philips Respironics devices (n=87; 18% of total)

Mean leaks (L/min) 51 (39; 66) 43 (35; 57) 61 (53; 67) 51 (47; 66) 52 (45; 68) 37 (33; 58) 63 (55; 65) 50 (38; 73) 7 Median VT (ml) 504 (415;

613)

623 (496; 716) 543 (500; 685) 386 (322; 445) 463 (442; 615) 414 (262; 595) 414 (384; 531) 525 (509; 538) 8 Median VT/weight (ml/kg) 5.7 (4.6; 7.2) 7.5 (5.3; 9.1) 5.9 (5.1; 7.8) 6.4 (5.3; 7.7) 4.5 (3.8; 4.8) 6.1 (5.6; 7.1) 7.2 (6.7; 7.7) 6.8 (6.1; 7.0) 8 Median VE (L/min) 8.4 (6.5; 10.6) 11 (8; 12.6) 10.2 (8.5; 11.7) 6.3 (5.2; 7.1) 9.1 (7.3; 10) 6.6 (4.3; 9) 8.7 (6.9; 11.2) 7.7 (7.2; 9.1) 9 Median RR (cycles/min) 17(16; 19) 17 (16; 19) 17 (17; 19) 18 (16; 19) 16 (15; 18) 17 (15; 19) 18 (16; 21) 15 (14; 17) 8

[RR-BURR] (cycles/min) 2.3 (0.9; 4.7) 3.3 (1.9; 4.8) 3.9 (1.3; 5.6) 2.1(1.2; 4.3) 2.2 (0.9; 3.6) 2.9 (2.4; 4.8) 4.3 (1.1; 7.6) 1 (0.3; 4.8) 8 Triggered inspirations (%) 54 (28; 75) 64 (47; 74) 58 (53; 93) 40 (25; 49) 49 (39; 76) 68 (51; 73) 26 (7; 69) 48 (14; 78) 8 AHI (n/hour) 3.4 (1.8; 9.1) 3.3 (1.5; 3.9) 2.6 (1.7; 3.3) 3.8 (3;7.9) 6.7 (1.8; 10) 3.5 (2.6; 7.1) 1.9 (1.8; 2.4) 9.3 (8.5; 10.6) 23 Mean daily use (min/day) 425 (336;

542) 464 (370; 567) 503 (377; 584) 415 (347; 488) 422 (326; 534) 491 (428; 519) 579 (473; 595) 322 (287; 347) 7

Values listed are the most recent values obtained.

Values expressed as median (IQR) or n (%) unless specified otherwise.

Results are presented separately according to manufacturer of device because software and data presentation differ.

COPD: Chronic obstructive pulmonary disease; OHS: Obesity hypoventilation syndrome; NMD: Neuromuscular disorders; RLD: Restrictive lung disorders; SRBD: sleep-related breathing disorders; VT: tidal volume; VE: Total minute ventilation; AHI: Apnea-hypopnea index; cpm: Cycles per minute. [RR-BURR]: Difference between spontaneous respiratory rate and back- up respiratory rate.

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e-Table 6. Settings for multimodal ventilators.

All patients Neuromuscular

disorders Kyphoscoliosis Other restrictive disorders n=489

(100%) n=79 (16%) n=29 (6%) n=22 (4%)

VAC mode 5 (1) 2 (2) 1 (3) 2 (9)

Tidal volume (ml) 750 (630;

780) 490 (420; 688) - 705 (668; 743)

Tidal volume/weight (ml/kg) 15 (14.5;

15.9) 17.1 (16.5;

17.7) - 12.2 (10.8; 13.6)

Pressure control mode 4 (1) 4 (5 - -

IPAP (cmH2O)* 18 (17; 19) 18 (17; 19) - -

EPAP (cmH2O) 0 (0; 1) 0 (0; 1) - -

Back-up respiratory rate

(cycles/min) 6.5 (0; 14) 6.5 (0; 14) - -

Values listed are the most recent values obtained.

Values expressed as median (IQR) or n (%) unless specified otherwise.

COPD, Overlap syndrome, OHS and SRBD groups did not use multimodal ventilators.

IPAP: inspiratory positive airway pressure; EPAP: expiratory positive airway pressure.

*missing data=1.

(13)

e-Table 7. Multivariable analysis of average daily use vs. relevant parameters.

n = 434 (55: missing data) Beta (95%CI) p value

Intercept 347

Gender

o Female -

o Male 8 (-29, 46) 0.664

Implementation of NIV

o Outpatient setting -

o Hospital setting 21 (-34, 76) 0.457

Diagnoses

o COPD -

o Neuromuscular disorders 8 (-52, 68) 0.789

o Overlap syndrome -27 (-92, 37) 0.402

o SRBD -86 (-168, -4) 0.040

o Obesity hypoventilation syndrome -96 (-154, -38) 0.001

o Other restrictive lung diseases (includes Kyphoscoliosis) -33 (-103, 37) 0.352 Type of mask

o Facial -

o Nasal -8 (-56, 39) 0.729

o Nasal pillows 22 (-50, 93) 0.551

Prior use of CPAP

o No -

o Yes 3 (-43, 50) 0.884

Comorbidities Metabolic syndrome

o No -

o Yes 27 (-21, 75) 0.263

Obesity

o No -

o Yes -25 (-70, 21) 0.283

Chronic heart failure

o No -

o Yes 21 (-22, 64) 0.335

Cerebrovascular disease

o No -

o Yes -52 (-133, 29) 0.207

Anxiety and/or depressive disorder

o No -

o Yes 6 (-32, 44) 0.764

Treatment with opiates

o No -

o Yes -141 (-243, -39) 0.007

Time spent under NIV (months)

[0,12) -

[12,36) 106 (56, 156) <0.001

[36,72) 144 (91, 198) <0.001

[72,341] 165 (111, 219) <0.001

Age (years)

[0,60) -

[60,70) 3 (-52, 58) 0.911

[70,80) -13 (-65, 38) 0.612

[80,95] -19 (-81, 42) 0.537

Values expressed as median (IQR) or n (%) unless specified otherwise.

(14)

Comments on medical insurance system and reimbursement in Switzerland:

The medical insurance system in Switzerland is defined by a federal law (LAMal, implemented in 1994). According to this law, having a medical insurance is mandatory for any person living in Switzerland. When insurance premiums are not affordable, a financial support is provided by the Canton (region). Medical insurances (which are all private institutions) must reimburse treatments and medical equipment according to a predefined and regularly up-dated list of medical interventions and devices (LiMA). Prices are also defined yearly at a national level.

Home ventilators are usually rented to the patients (and can thus be updated or changed when necessary). They belong either to the medical insurance company, to a health-care provider or to the manufacturer. Approximately 40% of patients under NIV are covered by a reinsurance company (SVK: Schweizerischer Verband für Gemeinschaftsaufgaben der Krankenversicher, www.svk.org) which covers also costly treatments such as transplantation or hemodialysis. Long-term NIV can be prescribed by any pulmonologist with a post-graduate training validated in Switzerland. No specific training other than that received during the years of medical specialization is required.

When a treatment by NIV is initiated, the prescribing physician must submit a request to the medical insurance specifying 1/ the diagnosis (i.e.: COPD, or ALS..); 2/ the indication (i.e.: hypercapnic respiratory failure, severe orthopnea); 3/ basic physiological data (FEV1, FVC, ABG with and without NIV); and 4/ a short medical report. The medical expert of the insurance then decides to accept or not the reimbursement. Once the indication has been accepted, the medical insurance will cover expenses related to rental (or acquisition) of NIV device, and annexes (humidifier, interfaces...) up to a pre-defined maximal sum per year.

A group of experts of the Swiss Society of Pulmonology establishes and publishes recommendations for the long- term use of NIV, and the follow-up of these patients. These recommendations also specify details such as when a second ventilator is required, and what type of ventilator is required for specific situations (i.e life-support devices).

Although day-time discontinuous home care is reimbursed (i.e. several home visits of a nurse for basic care, nutrition

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