rationale and benefits of niv in lung...

Rationale and benefits of NIV in

lung diseasesBrigitte Fauroux

Pediatric noninvasive ventilation and sleep unitResearch unit INSERM U 955

Necker university Hospital, Paris, France

InsermInstitut nationalde la santé et de la recherche médicale

Rationale and benefits of NIVin lung diseases

• Introduction• Cystic fibrosis• Bronchiolitis obliterans• Bronchopulmonary dysplasia• Interstitial lung disease

Two types of chronic respiratory failure

Abnormalities of the respiratory mechanics

� PaCO2 � PaO2

� Respiratoryload

Respiratory control

� Respiratory muscle capacity

NM diseases

Lung and airwaydiseases

Abnormalities of thealveolar-capillary barrier

Chronic lung diseases in children

• Obstructive lung diseases– Cystic fibrosis– BO– BPD

• Interstitial lung disease

Alveolar hypoventilation=

Hypercapnia & hypoxemia

Hypoxemia

Two types of respiratory treatments

Abnormalities of the respiratory mechanics

Abnormalities of thealveolar-capillary barrier

� Respiratoryload

Respiratory control

� Respiratory muscle capacity

Mechanicalventilation

O2

Respiratory diseasesexaggerate during sleep

Sleep

Ventilatory drive

Respiratory muscles

Respiratory mechanics

� Central drive� Chemoreceptor

sensitivity

Preservation of the activity of the diaphragm� Intercostal and upper airway muscles

Ventilation/perfusion mismatch� Airflow resistance� Functional residualcapacity

• If you can not measure it, you can not improve it.

William Thomson (1824 - 1907) or « Lord Kelvin »

physician, founder of the thermodynamics

Measurement of the work of breathing

100

150

200

250

300

350

400

450

500

550

600

650

PT

Pes

/min

(cm

H2O

.s.m

in-1

)

10 15 20 25 30 35 40 45 50

r = 0.-55; p =0.001

Hart et al. AJRCCM 2002;166:61

Respiratory mechanics incystic fibrosis

5

10

15

20

25

30

35

40

45

RR

(b

pm)

10 15 20 25 30 35 40 45 50

r = 0.38; p =0.03

.20

.25

.30

.35

.40

.45

.50

.55

.60

.65

.70

VT

(L

)

10 15 20 25 30 35 40 45 50

r = 0.37; p = 0.04

5

6

7

8

9

10

11

12

PaO

2 (K

Pa)

10 15 20 25 30 35 40 45 50

r = 0.76; p < 0.0001

4.5

5

5.5

6

6.5

7

7.5

cPaC

O2 (

Kp

a)

10 15 20 25 30 35 40 45 50

% Pred FEV1

r = 0.70; p < 0.0001

VEMS %

VEMS %

PTPes

RR

VT

PaO2

PaCO2

Noninvasive ventilation unloads the respiratory muscles in CF

86 88 90 92 94 96 seconds

-25 -20 -15 -10 -5.00.05.010

cmH

2O

Poe

so

-25 -20 -15 -10 -5.00.05.010

cmH

2O

Pga

s

-1.0

-0.5

0.0

0.51.0

l/s

Déb

it pa

t

-5.0

0.0

5.0

10

cmH

2O

P p

at

72 74 76 78 80 82 seconds

-40 -35 -30 -25 -20 -15 -10 -5.0

cmH

2O

Poe

so

-30

-20

-10 0.0

cmH

2O

Pga

s

-1.0

-0.5

0.0

0.5

1.0

l/s

Déb

it pa

t

0.0

5.0

10

15

cmH

2O

P p

at

Spontaneous breathing Pressure support (14 cmH2O)

Pes

Pgas

Flow

Paw

NIV decreasesthe work of breathing in CF

Fauroux et al. Crit Care Med 2001;29:2097

8 children with CF, mean age 13 years, mean FEV1 25%

NIV improves gas exchange in CF


NIV improves (daytime and) nocturnal gas exchange

Milross et al. AJRCCM 2001;163:129

10 adult CF patients3 nights: air, O2, NIV

NIV prevents oxygendesaturation and

respiratory muscle fatigue during chest physiotherapy

Fauroux et al. Pediatrics 1999:103:e32

NIV is associated with a decrease in dyspnea in CF


The level of inspiratorypressure should be sufficient

Fauroux et al. Eur Respir J 2004;24:624� Mean level 16 cmH2O

Expiratory pressure should be low

Hart et al. AJRCCM 2002;166:61

13 children, FEV1 35-79%

Benefits of NIV in cystic fibrosis

Proven benefits• Improvement in gas

exchange• Improvement in the

tolerance of chest physiotherapy

• Decrease in dyspnea• Improvement in exercise

tolerance• Stabilisation of the

decline in lung function

Expected benefits• Increase in survival• Increase in sleep quality• Increase in quality of life• Increase in weigth• Decrease/correction of

PHT• Improved outcome of

lung transplantation

Spontaneous breathing 12/4 cmH2O 13/5 cmH2O

Poe

s (c

mH

2O)

-60

-40

-20

0

Pga

s (c

mH

2O)

-2

0

2

4

6

8

10

12

14

16

Paw

(cm

H2O

)

0

2

4

6

8

10

12

Time (sec)

0 1 2 3 4 5

Pdi

(cm

H2O

)

-10

0

10

20

30

40

-60

-40

-20

0

-2

0

2

4

6

8

10

12

14

16

0

2

4

6

8

10

12

Time (sec)

0 1 2 3 4 5-10

0

10

20

30

40

-60

-40

-20

0

-2

0

2

4

6

8

10

12

14

16

0

2

4

6

8

10

12

-10

0

10

20

30

40

0 1 2 3 4 5

-60

-40

-20

-10

Time (sec)

Spontaneous breathing Clinical CPAP Physiological CPAP

SB CPAP clin CPAP Physio CPAP Physio + 1

Sw

ing

Exp

irato

ry P

gas

(cm

H2O

)

0

2

4

6

8

10

12

14

16

UAOBPD

Expiratory abdominal activity

SB CPAP clin CPAP PhysioCPAP Physio + 1

Sw

ing

Poe

s (c

mH

2O)

0

10

20

30

40

50

60

70

UAOBPD

SB CPAP clin CPAP Physio CPAP Physio + 1S

win

g P

di (

cmH

2O)

0

10

20

30

40

50

UAOBPD

Decrease in the respiratory effortwith CPAP

Khirani et al. Crit Care 2013;17:R167

No benefit of NIV in ILD13 year old girl with « end-stage » ILD

PoesocmH2O

PgascmH2O

AirflowL/sec

PawcmH2O

PdicmH2O

240.00000 245.00000seconds

-30.0

-15.0

0.0

15.0

cmH

2O

Poe

so

-20.0

-15.0

-10.0

-5.0cm

H2O

Pga

s

-2.0

-1.0

0.0

1.0

2.0

l/s

Déb

it pa

t

-20.0-10.0

0.0

10.020.0

cmH

2O

P p

at

-30.0

-15.0

0.0

15.0

30.0

cmH

2O

Pdi

135.00000 140.00000

-30.0

-15.0

0.0

15.0

cmH

2O

-35.0

-30.0

-25.0

-20.0

cmH

2O

-1.0

-0.5

0.0

0.5

1.0

l/s

0.0

5.0

10.0

15.0

cmH

2O

-30.0

-15.0

0.0

15.0

cmH

2O

140.0seconds

Spontaneousbreathing Nasal CPAP 6 cmH2O Nasal bilevel PAP 12/6cm H2O

OSAS in common inadults with interstitial lung

disease

Troy et al. World J Clin Cases 2014;2:828

OSAS is common in ILD

Lancaster et al. Chest 2009;136:772

AHI predicts survival in ILD

Kolilekas et al. J Clin Sleep Med 2013;9:593

Marie, 10 years, ILD

13 year old girl witha mutation of NKX2-1

Polysomnography

Normal sleep quality andstructureNo apneas9 hypopneas/h70% are obstructive

Mean SpO2 92%Minimal SpO2 86%Desaturation index 10/h11% of time with SpO2<90%

Mean PtcCO2 35 mmHgMaximal PtcCO2 40 mmHg

Conclusion

• Physiological studies show that there is a rationale for NIV in pediatric lung diseases– CF, BO, selected BPD, ILD ?

• Necessity of more systematic – sleep studies– physiological measurements

• Future studies should aim at defining:– patients who could benefit most from NIV– initiation criteria for NIV– clinical benefits from NIV

rationale and benefits of niv in lung...

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