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Inserm. Institut national de la santé et de la recherche médicale. Noninvasive Positive Pressure Ventilation ERS School Courses Hannover - Germany - 2007. Acute and chronic NPPV in children. Brigitte Fauroux Pediatric Pulmonology & Research unit INSERM UMR S-719 Armand Trousseau Hospital

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Acute and chronic nppv in children l.jpg

Inserm

Institut national

de la santé et de la recherche médicale

Noninvasive Positive Pressure Ventilation

ERS School Courses

Hannover - Germany - 2007

Acute and chronic NPPV in children

Brigitte Fauroux

Pediatric Pulmonology & Research unit INSERM UMR S-719

Armand Trousseau Hospital

Paris - France


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Acute and chronic NPPV in children

  • Rationale for NPPV

  • Acute applications of NPPV

    • Indications and outcome

    • Technical requisites

  • Chronic applications of NPPV

    • Indications and long term benefits

    • Technical requisites


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General requisites for NPPV

  • Medical requisites

    • respiratory failure can be improved, reversed, or cured by NPPV

      • hypercapnic respiratory failure (acute + chronic)

      • hypoxemic respiratory failure (acute)

    • patient has a minimal respiratory autonomy

    • NPPV is possible

      • no obstacle in the nose (closure of the mouth)

      • minimum level of co-operation

      • no excessive secretions

  • Technical requisites

    • adequate interface (nasal > face mask)

    • ventilator, settings and alarms adapted for children


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Acute and chronic NPPV in children

  • Rationale for NPPV

  • Acute applications of NPPV

    • Indications and outcome

    • Technical requisites

  • Chronic applications of NPPV

    • Indications and long term benefit

    • Technical requisites


Acute applications of nppv l.jpg
Acute applications of NPPV

Goals :avoid intubation, reverse ARF

reduce mortality, morbidity


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National survey on NPPV in the PICU in France 2006

  • 27/31 do use NPPV: duration of experience < 2 years n=6

  • 2 - 6 years n=13

    • 6 - 10 years n=6

    • > 10 years n=2



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NPPV in children with acute asthma

20 children with acute asthma

2 hours of NPPV (bilevel 10/5)

NPPV was associated with

 respiratory rate

 clinical asthma score

No change in PO2 and PCO2

Thill et al. Pediatr Crit Care Med 2004;5:337


Nppv in bronchiolitis l.jpg
NPPV in bronchiolitis

  • Not new ! : Beasley and Jones BMJ 1981;283:1506

  • Successfull experience (case series)

    • before the ICU: Pirret et al. Intensive Crit Care Nurs 2005;21:314

    • in the PICU: Martinon-Torres et al. Respir Med 2006;100:1458

      • 15 infants received NPPV with heliox

      • H1:  in clinical score, respiratory rate and PaCO2

      • duration of NPPV: 2 – 14 days

      • one patient required intubation


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NPPV unloads the respiratory muscles in cystic fibrosis

PTPdi (cm H2O.s.min-1)

10 patients

mean age 13 years

mean FEV1 25%

p<0.05

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


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NPPV improves alveolar hypoventilation in cystic fibrosis

*p<0.05

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


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NPPV unloads the respiratory muscles in infants with upper airway obstruction

Noninvasive CPAP 10 cm H2O

Spontaneous breathing

Pes (cm H2O)

Pgas (cm H2O)

Pdi (cm H2O)


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NPPV unloads the respiratory muscles in airway obstruction

infants with upper airway obstruction

10 infants with severe upper airway obstructionMean age 11.5 ± 5.4 months, weight 7.2 ± 0.4 kg

Essouri et al. Intensive Care Med 2006;7:329

* p< 0.005


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NPPV in ARF in children airway obstruction

with neuromuscular disorders

Avoid intubation

Reverse ARF

Indication to start long term NPPV

NPPV

3-year old boy, congenital myopathy

Volume targeted NPPV (VT: 220 ml)

Spontaneous breathing (VT: 100 ml)


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NPPV in children with SMA type I airway obstruction

  • 11 SMA type I infants

  • Age at first intubation or episode requiring ventilatory support : 5 – 28 months

  • 48 intubations for 28 episodes of acute respiratory failure :

    • 20 Non-protocol extubations: success 2

    • 28 Protocol extubations: success 23

Bach et al. Chest 2000;117:1100


Nppv in an acute situation hypoxemic respiratory failure l.jpg
NPPV in an acute situation airway obstructionHypoxemic respiratory failure

28 patients, mean age 8 years (4 to 204 months)

pneumonia: n=22

Fortenberry et al. Chest 1995;108:1059


Nppv during hypoxemic respiratory failure 4 children with acute leukemia l.jpg
NPPV during hypoxemic respiratory failure airway obstruction(4 children with acute leukemia)

Piastra et al. Intensive Care Med 2004;30:472


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NPPV in the pediatric intensive care airway obstruction

9

5-yr experience in the PICU

114 children

age 15 days to 17 yrs

Intubation rate (%)

61

114

23

12

9

Essouri et al. Ped Crit Care Med 2006;7:329


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Predictive factors of NPPV success in the pediatric intensive care

*p<0.05

Essouri et al. Ped Crit Care Med 2006;7:329


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Predictive factors of NPPV success in the pediatric intensive care

*p=0.002

*p=0.099

Bernet et al. Ped Crit Care Med 2005;6:660


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NPPV in an acute situation intensive careCardiogenic pulmonary edema

6 years old boy, on heart transplant list

Spontaneous

breathing

PS 10 cm H2O


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NPPV in an acute situation intensive careCardiogenic pulmonary edema

6 years old boy, on heart transplant list


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Acute and chronic NPPV in children intensive care

  • Rationale for NPPV

  • Acute applications of NPPV

    • Indications and outcome

    • Technical requisites

  • Chronic applications of NPPV

    • Indications and long term benefit

    • Technical requisites


Technical requisites for acute nppv l.jpg
Technical requisites for acute NPPV intensive care

Ventilator: ICU ventilator

Interface: adapted for children


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Acute and chronic NPPV in children intensive care

  • Rationale for NPPV

  • Acute applications of NPPV

    • Indications and outcome

    • Technical requisites

  • Chronic applications of NPPV

    • Indicationsand long term benefit

    • Technical requisites


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Chronic applications of NPPV intensive care

Physiopathology of respiratory failure

Respiratory muscles

capacity

Neuromuscular disorders

Ventilatory drive

Ondine’s course

Respiratory load

Cystic fibrosis

COPD

Upper airway obstruction

Alveolar hypoventilation

 PaO2 and  PaCO2


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Mechanical ventilation unloads the respiratory muscles intensive care

Respiratory muscles

Respiratory load

Mechanical

ventilation


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34% intensive care

8%

9%

30%

17%

2%

Long term home NPPV in children (France - 2000)

Number of patients

Fauroux et al, Pediatr Pulmonol 2003;35:119



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Long term NPPV use is associated with an increase in survival in DMD patients

Total Duchenne muscular

dystrophy population in

Denmark 1977-2001

Mortality 4.7  2.6 / 100 years

NPPV users 0.9  43.4 per 100

105

Jeppesen et al. Neuromuscular Disorders 2003;13:804


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NPPV is administered survival in DMD patients

preferentially during sleep

Sleep

Ventilatory

drive

Respiratory

mechanics

Respiratory

muscles

  • central drive

  • chemoreceptor

    sensitivity

V/Q mismatch

 airflow resistance

 FRC

preservation of the diaphragm

 upper airway muscles


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NPPV is associated with an improvement in nocturnal gas exchange in children with neuromuscular diseases (n=40)

SaO2 (%)

Transcutaneous PCO2 (mm Hg)

p=0.03

p=0.03

Simonds et al. Eur Resp J 2000;16:476


Long term nppv use is associated with an improvement in gas exchange in children with nm disorders l.jpg
Long term NPPV use is associated with an improvement in gas exchange in children with NM disorders

30 children

Age 12.3 ± 4.1 yrs

NPPV for 25 ± 13 months

% of total

sleep time

mm Hg

Mellies et al, Eur Respir J 2003;22:631


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NPPV improves nocturnal hypoventilation in CF patients exchange in children with NM disorders

Milross et al. AJRCCM 2001;163:129


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NPPV improves nocturnal exchange in children with NM disorders

hypoventilation in CF patients

Change in PtcPCO2 (mmHg)

from NREM to REM sleep

% of time with SaO2 > 90%

*

**

*

**

*p<0.05

**p<0.01

Milross et al. AJRCCM 2001;163:129


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Improvement of nocturnal gas exchange with NPPV in infants with OSA

12 infants with severe upper airway obstruction

Fauroux et al. AJRCCM 2001:164:1874


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Effectiveness of CPAP therapy in children with OSA with OSA

29 children with OSA

age 2 to 16 years

CPAP: n=13

Bilevel PAP: n=16

p=0.003

Marcus et al. Pediatrics 2006;117:e442


Adherence to cpap therapy in children with osa l.jpg
Adherence to CPAP therapy in children with OSA with OSA

29 children with OSA

Randomly assigned to CPAP or Bilevel PAP

1/3 dropped out < 6 months

Mean nightly use was 5.3 ± 2.5 hours

Parents overestimated CPAP use

Marcus et al. Pediatrics 2006;117:e442


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Long term NPPV use in children with OSAwith NM disorders

  • Effects on lung function

    • IVC  in Duchenne patients (-183 ml)

    • IVC remained stable in other NM diseases

  • MIP

    •  in all patients (40% to 44% predicted, NS)

  • Quality of life

    •  in the 13 patients who were evaluated

  • Lung growth and nutrition ?

Mellies et al, Eur Respir J 2003;22:631


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Efficient respiratory movements are necessary for normal with OSAlung and chest wall growth

Abnormal development of the diaphragm and the intercostal muscles leads to lung hypoplasia in mice

Ackerman et al. PLoS Genetics 2005;1:e10

Inanglou et al. Int J Dev Biol 2003;47:363

Inanlou et al. Dev Dyn 2005;323:43



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Long term NPPV use (1 months) in patients with cystic fibrosis

  • 4 adult CF patients

  • FEV1 11 – 14%

Piper et al. Chest 1992;102:846


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Long term NPPV use in fibrosispatients with severe OSA

  • Correction of sleep disordered breathing

  • Improvement in quality of life (avoids tracheostomy)

  • Allows progressive nutritional autonomy

Fauroux et al. AJRCCM 2001; 164:1874

Essouri et al. Intensive Care Med 2005


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Criteria to initiate NPPV in the chronic setting fibrosis

  • Diurnal hypercapnia (NM patients)

  • After an acute respiratory failure (NM patients)

  • Nocturnal hypoventilation ?

    • Sleep disruption, daytime hypersomnolence, excessive fatigue, morning headaches

    • Polysomnography ++

      • No precise criteria

      • Mean SaO2 ?, % of time spent with a SaO2 < 90% ?

      • Level of transcutaneous or end tidal CO2 ?

      • Sleep fragmentation, apnea - hypopnea index ?


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NPPV for nocturnal hypoventilation fibrosiswith daytime normocapnia

48 patients with

NMD or chest wall disease

age 7– 51 years

all VC < 50%

Ward et al. Thorax 2005;60:1019


Nppv for nocturnal hypoventilation with daytime normocapnia47 l.jpg
NPPV for nocturnal hypoventilation fibrosiswith daytime normocapnia

Ward et al. Thorax 2005;60:1019


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Acute and chronic NPPV in children fibrosis

  • Rationale for NPPV

  • Acute applications of NPPV

    • Indications and outcome

    • Technical requisites

  • Chronic applications of NPPV

    • Indications and long term benefit

    • Technical requisites


Technical requisites for long term home nppv l.jpg
Technical requisites for fibrosislong term (home) NPPV

  • Ventilators designed for pediatric patients

    • appropriate VT, triggers, alarms, battery

  • Appropriate circuits and expiratory valves

  • Interfaces (nasal masks) designed for children

  • Other supportive therapy

    • oxygen therapy

    • suction equipment

    • physiotherapy, assisted cough

    • nutrional support


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Interface adapted fibrosis

for the child


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Conclusion fibrosis

  • NPPV represents a logical therapeutic option in disorders characterised by alveolar hypoventilation

    • neuromuscular disorders

    • dynamic upper airway obstruction

    • cystic fibrosis

    • hypoxic respiratory failure (except ARDS)

    • cardiogenic pulmonary edema ?

  • Future research

    • define the criteria for starting NPPV and the benefit of NPPV in children

      • in the acute and chronic setting

      • according to the underlying disease

    • improve the ventilators and interfaces

    • evaluate the long term benefit of NPPV in children


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Inserm fibrosis

Institut national

de la santé et de la recherche médicale

Acknowledgements

  • Armand Trousseau Hospital

    • Pediatric Pulmonology Department: A Clément

    • ENT Department: N Garabédian

    • Pediatric Intensive Care Unit: S Renolleau

    • Neurophysiology Department: F Renault

  • INSERM U 719

    • M Bonora, F Nicot, S Essouri, M Boulé, A Clément

  • Plastic Surgery and Maxillofacial Department

    • JF Lavis, A Picard, MP Vazquez, R Alzeibak, G Accart

  • Raymond Poincaré Hospital (Physiology Department)

    • F Lofaso

  • Pitié Salpétrière Hospital (Pulmonology and Intensive Care)

    • T Similowski, C Straus

  • Royal Brompton Hospital (Respiratory Muscle Laboratory)

    • MI Polkey, N Hart

  • INSERM 492

    • A Harf †, D Isabey, J Pigeot, B Louis


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