Long term noninvasive ventilation in children: what’s new in 2015 ?

Brigitte Fauroux, MD PhDPediatric noninvasive ventilation and sleep unit

Necker university hospital, Inserm U 955Paris, France

InsermInstitut nationalde la santé et de la recherche médicale

Noninvasive ventilation in children: what’s new ?

• Epidemiology• Diseases that may require NIV• Interfaces• Compliance• CPAP monitoring• CPAP/NIV initiation

An italian experienceNumber of children treated with NIV since 1993 in a single centre

Pavone et al. Early Human Development 2013;89:S25

McDougall et al. Arch Dis Childh 2013;98:660

Incidence 1995-2009 Prevalence 1995-2009

15 year experience of a pediatric centre (Vancouver)

McDougall et al. Arch Dis Childh 2013;98:660

15 year experience of a pediatric centre (Vancouver)

McDougall et al. Arch Dis Childh 2013;98:66

McDougall et al. Arch Dis Childh 2013;98:660

Noninvasive ventilation in children: what’s new ?

• Epidemiology• Diseases that may require NIV• Interfaces• Compliance• CPAP monitoring• CPAP/NIV initiation

An italian experienceIndications for NIV in a single centre

Pavone et al. Early Human Development 2013;89:S25

Acute group

n=15

Subacute group

n=18

Chronic group

n=43

Age, years 1.2±3.4 6.4±7.2 5.9±7.1

Female/male 7/8 8/10 22/21

Diagnosis Pierre Robin syndromeLaryngomalaciaPolymalformative sdKabuki syndromeCystic fibrosisBPDNeuromuscular disorder

6321111

LaryngomalaciaPrader Willi syndromePierre Robin syndromeBDPCraniostenosisTreacher Collins s dVocal cord palsyDown syndromeMucopolysaccaridosisDuchenne MDLaryngeal massCraniofacial malform.Generalised dystonia AchondroplasiaTracheomalacia

411111111111111

Pierre Robin syndromeDown syndromeMucopolysaccaridosisCharge syndromeLaryngomalaciaNeuromuscular disordersPolymalformative syndromeTreacher Collins syndromeAchondroplasiaPrader Willi syndromeBPDMyhre syndromeSpinal muscular atrophyRett syndromeGoldenhar syndromeIdiopathic OSASHanhart syndromeBeckwith Wiedemann sdLoeys Dietz syndromeOssificant fibrodysplasia

55433332222111111111

76 children started on NIV during 2013-2014

88 patients3 mo – 44 yrs

NIV was started in 16 patients

Neonates hospitalizedn=37

No clinical UAOn=17

Clinical UAOn=20

Severe clinical UAOn=9

Immediate CPAP in the NICU

Moderate clinical UAOn=11

Sleep study with gas exchange

Tracheotomyn=4

CPAPn=5

Abnormal sleep studyCPAP, n=4

Normal sleep studyn=7

Moderate UAO group

Severe UAO group

Mild UAO group

No UAO group

Neonates seen as outpatientsn=7

Neonates with PRS evaluated over one yearn=44

No UAO group

Plastic and Reconstructive Surgery, in press

Pierre Robin syndrome:Necker airway management protocol

• CPAP was started – as a first line therapy in infants with severe UAO– after a (prone position) PG on the following criteria

• AHI >10/h and/or• oxygen desaturation index >15/h and/or • minimal SpO2<90% and/or • maximal PtcCO2>50 mmHg

• CPAP– avoided a tracheotomy in 5/9 patients with severe UAO– normalised sleep in the 4 patients with moderate OSA

Noninvasive ventilation in children: what’s new ?

• Epidemiology• Diseases that may require NIV• Interfaces• Compliance• CPAP monitoring• CPAP/NIV initiation

Interfaces used before 2014

Ramirez et al. Intensive Care Med 2012;38:655

• 14 patients, 2 – 7 yrs• Equivalence between the Pixi mask and the

patient’s previous mask– PSG– adherence (7.4 vs 7.2 h/night)

• Advantages of the Pixi mask– less leaks– improved comfort

J Clin Sleep Med 2014;10:979

Nasal interfaces for infants

Interfaces > 2014

Manufacturer Model

Air Liquide Healthcare

AG Industries

Philips Respironics

ResMed

Fisher and Paykel

Respireo nasal prongsRespireo Soft Baby nasal mask

Nonny pediatric nasal mask

Wisp nasal mask

Quattro air full face mask

Eson, Opus

Noninvasive ventilation in children: what’s new ?

• Epidemiology• Diseases that may require NIV• Interfaces• Compliance• CPAP monitoring• NIV/CPAP initiation

NIV and CPAP adherence according to the interface

Ramirez et al. Sleep Med 2013;14:1290

NIV and CPAP duration� < 3 months� 3-12 months� > 12 months

• 5/8 patients with SDB did not tolerated CPAP

• These patients were deficient in tests of • adaptive behavior• visual-motor integration• achievement

Noninvasive ventilation in children: what’s new ?

• Epidemiology• Diseases that may require NIV• Interfaces• Compliance• CPAP monitoring• NIV/CPAP initiation

PSG during CPAP/NIVNo description of eventsNo explanation on recommended CPAP/NIV changes

Number ofrespiratory events/polygraphy

Consequences of the respiratory events

Nocturnal gas exchange didnot predict PG results

Noninvasive ventilation in children: what’s new ?

• Epidemiology• Diseases that may require NIV• Interfaces• Compliance• CPAP monitoring• NIV/CPAP initiation

CPAP/NIV initiation

• No validated criteria to start CPAP/NIV due to the lack of markers of SDB end-organ morbidity

3 clinical scenario

AcuteImpossibility to

wean from NIV in the ICUN=15

SubacuteAbnormal

nocturnal gas exchange

N=18

ChronicAbnormal P(S)G

N=43

• Minimal SpO2 < 90%• Maximal PtcCO2 > 50 mmHg• % of time with a SpO2 < 90% ≥ 2%• % of time with a PtcCO2 > 50mmHg ≥ 2%• Oxygen desaturation index > 1.4/h

• AHI > 10/h

Number of criteria in the subacute and chronic group

Subacute groupChronic group

Acute group

n=15

Subacute group

n=18

Chronic group

n=43

Age, years 1.2±3.4 6.4±7.2 5.9±7.1

Female/male 7/8 8/10 22/21

Diagnosis Pierre Robin syndromeLaryngomalaciaPolymalformative sdKabuki syndromeCystic fibrosisBPDNeuromuscular disorder

6321111

LaryngomalaciaPrader Willi syndromePierre Robin syndromeBDPCraniostenosisTreacher Collins s dVocal cord palsyDown syndromeMucopolysaccaridosisDuchenne MDLaryngeal massCraniofacial malform.Generalised dystonia AchondroplasiaTracheomalacia

411111111111111

Pierre Robin syndromeDown syndromeMucopolysaccaridosisCharge syndromeLaryngomalaciaNeuromuscular disordersPolymalformative syndromeTreacher Collins syndromeAchondroplasiaPrader Willi syndromeBPDMyhre syndromeSpinal muscular atrophyRett syndromeGoldenhar syndromeIdiopathic OSASHanhart syndromeBeckwith Wiedemann sdLoeys Dietz syndromeOssificant fibrodysplasia

55433332222111111111

76 children started on NIV during 2013-2014

Comparison of nocturnal gas exchange between the

subacute and chronic groupSubacute group

n=18

Chronic group

n=43

Nocturnal gas exchange

Mean SpO2 (%)

Minimal SpO 2 (%)

Time spent with SpO 2 < 90% (%)

Oxygen Desaturation index (number/hr)

Mean PtcCO 2 (mmHg)

Maximal PtcCO 2 (mmHg)

Time spent with PtcCO 2 > 50 mmHg (%)

94.2 ± 2.8

76.8 ± 9.8

7.9 ± 16.6

26.3 ± 24.4

45.8 ± 5.4

53.6 ± 9.4

21.5 ± 34

94.2 ± 2.7

77.3 ± 9.5

8.2 ± 16.0

27.6 ± 25.3

45.7 ± 5.5

53.3 ± 9.4

20.9 ± 32.9

AHI NA 27.2 ± 30

Conclusion

• Epidemiology: � use of NIV• Diseases that may require NIV: expanding• Interfaces: interfaces for infants• Compliance: value of specialised centers• CPAP monitoring: few events• NIV/CPAP initiation: future studies should

look for benefit of CPAP/NIV on end-organ morbidity