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NMD children on long tem ventilation support:

Monitoring and transition

Dr. Renato Cutrera

Head of Respiratory Unit – Sleep & PLTV Lab.

Academic Department of Pediatrics

Bambino Gesù Childre’s Hospital - Rome – Italy

renato.cutrera@opbg.net

R. Cutrera 2016 - cutrera@opbg.net

Stages of Hypercapnic Respiratory Failure

• Khatwa UA et Al. Indian J Pediatr 2015

R. Cutrera 2016 - cutrera@opbg.net

JO Benditt, PEDIATRICS Volume 123, Supp 4, 2009

Sleep RF (REM)Respiratory muscles atonyDiaphfragm weakness Upper airway muscles weakness (OSAS)Tachypnea

Respiratory Failure (RF) progression in NMDs patients

Sleep RF (NREM/REM)

Awake and Sleep RF

OSA (Obstructive Sleep Apnea)

CA (Central Sleep Apnea)

Hypoxemia Hypercapnia

Riduzione

R. Cutrera 2016 - cutrera@opbg.net

R. Cutrera 2016 - cutrera@opbg.net

R. Cutrera 2016 - cutrera@opbg.net

Diagnosis of Hypoventilation e SDB in NMD children 1/2

R. Cutrera 2016 - cutrera@opbg.net

Diagnosis of Hypoventilation e SDB in NMD children 2/2

NOCTURNAL PULSOXIMETRY

Since oxygen desaturations are a major determinet of adverseneurocognitive and cardiovascular consequences, pulse oximetryshould be included in the monitoring of NIV or CPAP efficacy

Janssens JP, Borel JC, Pépìn JL; SomnoNIV Group. Thorax 2011

R. Cutrera 2016 - cutrera@opbg.net

BUT WHAT COULD WE MISS?

The aim of the study was to assess if overnight pulse oximetry and daytime blood gases are sufficiently accurate to detect nocturnal hypoventilation in children receiving long-term noninvasive respiratory support.

21 of the 50 patients (42%) on long-term noninvasiverespiratory support presented nocturnal hypercapnia, without nocturnal hypoxemia.

Daytime capillary arterialized carbon dioxide levels

were normal in 18 of these 21 patients.

NOCTURNAL MONITORING OF pCO2

R. Cutrera 2016 - cutrera@opbg.net

Interpretation of oxygen saturation measured by pulse oximetry (SpO2) patterns in patients using non-invasive ventilation (NIV).

Jean-Paul Janssens et al. Thorax 2011;66:438-

445

NOCTURNAL PULSOXIMETRY IN NIV

R. Cutrera 2016 - cutrera@opbg.net

• Continuous PtcCo2 recordings show good agreement with arterial measurements

• limitation is the requirement for periodic recalibration and changes of membrane in order to ensure sufficient precision of transcutaneous measurements

• Importantly, the good agreement between PtcCo2 and PaCo2 measurements is preserved when patients are treated by continuous positive airway pressure (CPAP)

• More recent devices combining PtcCo2 and Spo2 earlobe sensors and validated in acute care and chronic clinical settings are designed for continuous recording over 8 h periods without requiring recalibration and are feasible for routine use

NOCTURNAL MONITORING OF PCO2

R. Cutrera 2016 - cutrera@opbg.net

PetCO2 PtcCO2

NOCTURNAL MONITORING OF pCO2

R. Cutrera 2016 - cutrera@opbg.net

Transcutaneous measurement of carbon dioxide

(PtcCo2) during night in a ventilated patient.

Jean-Paul Janssens et al. Thorax 2011;66:438-445

Note normalisation of Ptcco2 (A) during periods

of wakefulness versus (B) hypoventilation

related to continuous major leaks during sleep

R. Cutrera 2016 - cutrera@opbg.net

NOCTURNAL PULSOXIMETRY + CO2 MONITORING IN NIV

NOCTURNAL HYPERCAPNIA WITHOUT HYPOXEMIA

R. Cutrera 2016 - cutrera@opbg.net

S/T IPAP 12 cmH20,

EPAP 4 cmH20,

FR 16 atti/minuto

Duchenne patient

NOCTURNAL PULSOXIMETRY + CO2 MONITORING DURING SETTING NIV

R. Cutrera 2016 - cutrera@opbg.net

S/T IPAP 14

cmH20, EPAP 4

cmH20,

FR 18 a/min

Duchenne patient

NOCTURNAL PULSOXIMETRY + CO2 MONITORING DURING SETTING NIV

R. Cutrera 2016 - cutrera@opbg.net

SUGGESTED ALGORITHM FOR MONITORING NON INVASIVE VENTILATION (NIV) DURING NIV

Jean-Paul Janssens et al. Thorax 2011;66:438-445

LIMITATIONS OF PULSE OXIMETRY AND PtcCO2

• LACK OF SPECIFICITY IN INDENTIFING RESIDUAL SLEEP DISORDERED BREATHING

• LACK OF SENSITIVITY IF OXYGEN SUPPLEMENTATION• EVENTS WITHOUT DESATURATION ARE NOT TAKEN INTO

ACCOUNT (MICROAROUSALS)

R. Cutrera 2016 - cutrera@opbg.net

RW

N1

N2

N3

Cn.A

Ob.A

Mx.A

Hyp

Uns

RERA

+5

+5

+5

+5

+5

+5

Parametri Valori

Tempo Totale di Sonno (h) 8:54.5

SaO2 media (%) 95

SaO2 minima (%) 75

SaO2 < 90 % (% tempo) 3.5

Indice di desaturazione >4% 1.3

Frequenza media del polso (bpm) 84

MOAHI (Indice di apnea/ipopnea ostruttiva+mista) (N°/h) 13.9

Indice di Apnea Centrale 0.0

POLISOMNOGRAPHY

R. Cutrera 2016 - cutrera@opbg.net

• OBSTRUCTIVE APNEA duration of 2 breaths associated with the presence of respiratory effort throughout the entire period of absent airflow.

• CENTRAL APNEA absent inspiratory, and at least one of the following: The event lasts 20 seconds or longer. The event lasts at least the duration of two breaths during baseline breathing and is associated with an arousal or ≥ 3% oxygen desaturation.

• MISTA at least the duration of 2 breaths during baseline breathing and is associated with absent respiratory effort during one portion of the event and the presence of inspiratory effort in another portion

• HYPOPNEA: 30% drop in flow; for the minimum duration of 2 breaths with either ≥ 3% oxygen desaturation or arousal

• RERA respiratory effort related arousals (2 breaths, increasing respiratory effort, flattening of the nasal pressure or PAP device flow waveform, snoring, or an elevation in the end-tidal PCO2 leading to arousal.

R. Cutrera 2016 - cutrera@opbg.net

The AHI obtained from RP was strongly correlated to the corresponding PSG AHI (r = 0.91)

However, Bland Altman analysis showed that in-lab RP underestimated the AHI, this underestimation was due to missed hypopneas causing arousals without desaturation

R. Cutrera 2016 - cutrera@opbg.net

PSG IN TIME CONSUMING, EXPENSIVE AND TECHNICALLY DEMANDING

Abbreviated testing modality (PolyGraphy, Oximetry, PtcCO2)

PSG ID GOLD STANDARD

CONCLUSIONS MONITORING

- to monitoring NIV

- precise monitoring of treatment efficacy and characterization of respiratory events

R. Cutrera 2016 - cutrera@opbg.net

Medicina di Transizione

“The purposeful, planned and timely transition from child and family-centered pediatric health care to patient-centered adult-oriented health care.”

Society for Adolescent Medicine, 1993

• The goal of transition is to optimize the quality of life and future potential of young patients. The elements of the transition preparation includes

• (1) a written transition policy,

• (2) a registry for identification and tracking of patients,

• (3) planning tools (transition readiness assessment, portable medical summary and transition action plan), and

• (4) timely and organized transfer to adult health care providers

TRANSITION

R. Cutrera 2016 - cutrera@opbg.net

Children and Youth with Special Health Care Needs (CYSHCN)

• Asthma

• ADHD

• Diabetes mellitus

• Sickle cell disease

• Cerebral Palsy

• Cystic fibrosis

• Chronic kidney disease

• Inflammatory bowel disease

• Congenital heart disease

• Childhood cancer survivors

• Solid-organ transplant recipients

• Spina bifida

• Down syndrome

• HIV-AIDS

• Genetic and neuromuscular disorders

Progresso Medico e Tecnologico

MAGGIORE SOPRAVVIVENZA dei neonati prematuri bambini affetti da malattie altrimenti letali

aumento delle patologie croniche e nuova tipologia assistenziale con bisogni complessi necessità di risposte integrate multispecialistiche ed interistituzionali

Children with Medical ComplexityCurr Probl Pediatr Adolesc Health Care 2012;42:113-119

Pediatrics 2011;127;529

- Malattia Multisistemica

- Grave Compromissione

Neurologica con Deficit

Funzionali

- Pazienti Oncologici

-con Deficit Funzionali

Multisistemici

- Dipendenza da Presidi

Medici/Tecnologici per le

attività quotidiane

CSHCNCMC

“Childrenwith Medical

Complexity”

CMC vs CSHCN• maggiore fragilità clinica • maggiore necessità di

cure intensive

Special Article

Children With Medical Complexity: An Emerging Population for Clinical and Research Initiatives

1. Eyal Cohen, MD, MSc, FRCP(C)a,b,c, Dennis Z. Kuo, MD, MHSd,Rishi Agrawal, MD, MPHe,f, Jay G. Berry, MD,

MPHg,Santi K. M. Bhagat, MD, MPHh,Tamara D. Simon, MD, MSPHi,Rajendu Srivastava, MD, MPH, RPC(C)j

ABSTRACT

Children with medical complexity (CMC) have medical fragility and intensive care needs that are not easily met by

existing health care models. CMC may have a congenital or acquired multisystem disease, a severe neurologic

condition with marked functional impairment, and/or technology dependence for activities of daily living. Although

these children are at risk of poor health and family outcomes, there are few well-characterized clinical initiatives and

research efforts devoted to improving their care. In this article, we present a definitional framework of CMC that

consists of substantial family-identified service needs, characteristic chronic and severe conditions, functional

limitations, and high health care use. We explore the diversity of existing care models and apply the principles of the

chronic care model to address the clinical needs of CMC. Finally, we suggest a research agenda that uses a uniform

definition to accurately describe the population and to evaluate outcomes from the perspectives of the child, the family,

R. Cutrera, 2016 - cutrera@opbg.net

Chatwin M, Tan HL, Bush A, Rosenthal M, Simonds AK . Long Term Non-Invasive Ventilation in Children: Impact on Survival and Transition to Adult Care PLOS ONE 10(5): 2015

use of NIV would be associated with increasing numbers of children transitioning to adult services.

R. Cutrera 2016 - cutrera@opbg.net

Chatwin M, Tan HL, Bush A, Rosenthal M, Simonds AK . Long Term Non-Invasive Ventilation in Children: Impact on Survival and Transition to Adult Care PLOS ONE 10(5): 2015

R. Cutrera 2016 - cutrera@opbg.net

R. Cutrera 2016 - cutrera@opbg.net

R. Cutrera 2016 - cutrera@opbg.net

R. Cutrera 2016 - cutrera@opbg.net

The majority of respondents, 78.1% (25/32), reported that they do not utilize a standard protocol for transition while 41.4% (12/29) have no process in place. No program surveyed uses a designated transition leader. Referral to an adult pulmonologist within the same health system occurs more frequently than referral to private practice. Forty-three percent are not satisfied with involvement from the adult pulmonology care team. Coordination of care with other specialty services such as adult otolaryngology is provided by 31% of respondents. Of respondents, 13.8% assessed "readiness to transition" to adult pulmonary for RTD patients. Pediatric pulmonary providers are not satisfied with their current practices or involvement from the adult team, and only 24% track the transition process until the first visit with the adult pulmonologist.

R. Cutrera 2016 - cutrera@opbg.net

Monitoring Conclusions

1. Awake ABG analysis is often used to start and titrate the ventilator2. Pulse Oxmetry can detect hypoxemia and cluster of desaturations3. PtcCO2 and Pulse Oximetry detect hypoventilation and can be usefull to titrate

ventilators4. Cardio Respiratory Polygraphy + PtcCO2 detect hypoventilation and moderate

and severe OSAS5. Full Polysonnography + PtcCO2 detect hypoventilation and all respiratory events

(arousals)

1. CMC transition from childhhod to adulthood medicine can be very difficult and potentially dagerous

2. Lack of specific programmes is very common even in USA3. A lot of work has to be done (at least in my country)

Transition Conclusions

Thanks for your invitation