RESPIRATIONSCENTER VEST Århus Universitetshospital - Skejby

Ole Nørregaard

Januar 2013

Respiratory Center West

Respiratory Center East

Respiratory Center South

SKEJBY SYGEHUS

Respirationscenter Vest

har højt specialiseret funktion for • diagnostik, • behandling og • opfølgning af patienter med kronisk respirationsinsufficiens i bredeste forstand, herunder søvnrelaterede sygdomme.

Disposition

• RCV's historiske udvikling (incl. vækst)

• Epidemiologi

• Patofysiologi

• Diagnostik

• Hvornår henvisning til RCV ?

• Behandling – Ventilation

• Non-invasiv

• Invasiv (organisering, hjælperoplæring)

Historie:

• 1952 Polioepidemien

• 1954 Etablering af center på Blegdamshospitalet

• 1978 Flyttes til Rigshospitalet

• 1990 Sundhedsstyrelsens vejledning vedr. visitation og sygehusbehandling af patienter med kronisk respirationsinsufficiens.

• 1991 Respirationscentrene – RCV-RCØ

Historie: • 1991 RCV oprettes som en del af int.afd.N, ÅKH med 1-4 sengepladser • 1998 RCV etableres som selvstændigt afsnit med 4-7

sengepladser og ambulatorium • 2010 Sundhedsstyrelsens specialeudmelding for det

anæstesiologiske speciale definerer området som en højt specialiseret funktion.

• 1.6.11 RCV Skejby åbner med 8 sengepladser og udvidet ambulant funktion.

Afdelingen:

• 8 senge

– Enestuer med plads til hjælper/pårørende

• Søvnambulatorium

• Ambulatorium

• 24 timers

hotline funktion

Personale på RCV:

RCV

4 Overlæger

32 Syge-plejersker

5 SoSu-assistenter

Service-medarbejder

3 Sekretærer

Respirations

teamet –

3 socilal

rådgivere

Samarbejdspartnere Kompleks logistik kræver et bredt samarbejde

• Lægfolk (patienter, pårørende, hjælpere) • Leverandører af teknisk udstyr • Medicotekniske afdelinger • Sociale myndigheder • Patientforeninger • Kliniske afdelinger/praktiserende

læger/speciallæger • Respirationcenter Øst • Udenlandske centre

Patientkategorier: • Neuromuskulære sygdomme • Thoraxdeformiteter • Tetraplegi • Adipositasbetinget hypoventilation

• KOL • Cystisk fibrose

• Børn med syndromer og kroniske lungesygdomme (BPD)

• Søvnudløste respirationsforstyrrelser (SDB) • Søvnforstyrrelser

Duchenne’s muscular dystrophy --- National data

Start of the respiratory centers in Denmark

Patientudvikling (RCV)

0

500

1000

1500

2000

2500

2000 2001 2002 2003 2004 2005 2006 2007 2008 2009 2010 2011

Home mechanical ventilation in Denmark (5 mill inhibitants)

• 240 ventilated via trachesostomy

• 1035 ventilated via mmask

• 81 % ventilated via NIV

• Respiratory Centre West (55% of the population): 20 % of ventilated individuals are children

Age distribution (percent)

0%

20%

40%

60%

80%

100%

ALL

Austria

Belgium

Den

mark

Finlan

dFra

nceG

ermany

Gree

ceIre

land

ItalyN

etherlan

ds

Norw

ayPolan

dPortug

alSp

ainSw

eden

UK

66 years +66 years +

26 – 65 yrs26 – 65 yrs

17 – 25 yrs17 – 25 yrs

16 or less16 or less

RESPIRATORY PHYSIOLOGY AND PATHOPHYSIOLOGY

Søvnudløste ventilationsændringer

SØVN

RESPIRATORISK KONTROL • Kemoreceptor følsomhed • Cortical input • Respiratoriske motorneuroner

RESPIRATORISK MUSKELFUNKTION •Intercostal •Diafragma •Accesesoriske

LUNGE-MEKANIK • Luftvejsmodstand • FRC • V/Q match

Raw - patophysiology

• Congenital malformations (laryngomalacia, epiglottic anomalies, tonsils, membraneous obstruction, vascular ring etc)

• tumor mediastini

• sekretions, foreign bodies

• age 1-3 years

KARAKTERISTIKA

• Små dimensioner => luftvejsmodstand

• adenoide vegetationer & tonsiller

• compliant chest wall => paradoks respiration => energitab(wasted ventilation)

• horisontale costae

• immature muskler

• FRC => vulnerabel for hypoxæmi

KARAKTERISTIKA

• Alveolær ventilation:FRC hos små børn = 5.0:1.0, hos voksne 1.5:1.0

• Apnøer kraftigt REM-associerede

• hypoxæmisk respiratorisk respons svækkede hos små børn

• med alderen ofte aftagende compliance af thorax => øgning af det respiratoriske arbejde

KARAKTERISTIKA

• Neuromuskulær sygdom er oftest associeret med HYPERKAPNISK respirationsinsufficiens (i modsætning til hypoxæmisk)

Pediatric characteristics

• FRC very small (unmodified 15 % of TLC, 40 % modified)

• Modified with

– Expiratory breake

– High respiratory frequency

– Maintanence of muscular tone

• Periodic closure of the airways during tidal breathing

diagnostics

• Pulmonary function tests

• Pulse oxymetry

• cardio-respiratory monitoring (CRM)(flow, thoraco-abdominal movements, SaO2, CO2)

• polysomnography (PSG)(= CRM + sleep stages)

• SYMPTOMS

Cardiorespiratorisk monitorering (CRM)

Airflow

Chest- and abdominal movements

SaO2

tcCO2 EKG

CRM

Why PSG ??

• Document prescence of vulnerable (REM) sleep

• PSG determines diagnosis

• PSG can possibly identify differential diagnosis

• PSG can contribute to prognosis

• Evaluate severity

• Contributes to the evaluation of perioperative risc

• Determines base line for follow-up comparison

Polysomnografi(PSG)

Airflow

Chest- and abdominal movements

SaO2

tcCO2 EKG

EEG

Leg movements

EOG

EMG

Polysomnografi

HENVISNING TIL RESPIRATIONSCENTER

HVORNÅR ??

Physiological criteria

• Vital capacitet < 15 ml/kg

• PCEF < 2-3 l/sec (180 l/min) (Bach)

• PaCO2 > 6.0 kPa (45 mmHg)

• PaO2 < 9.3 kPa (70 mmHg)

• SaO2 < 97 % (on room air) Chest

1998;113:289S-344S

Indications for NIPPV (neuromuscular, restrictive a.o.)

• Symptoms (fatigue, dyspnea etc.)

• PaCO2 > 45 mmHg (6 kPa)

• Nocturnal desat. < 88% for 5 consecutive minutes

• Pimax < 60 cm H2O or FVC < 50% predicted

Chest, 1999;116:521

Referral of children

• signs and/or symptoms of nocturnal hypoventilation (NH) during the night

• daytime symptoms of NH

• failure to thrive

Referral of children

• IVC < 60 % (=> SBD, < 40% => noct hypovent)

• MIP < 4.0 kPa (=> SDB, < 2.5 => noct hypovent)

• CPF < 270 l/min

• Daytime PaCO2 > 45 mmHg (=> noct hypovent)

TREATMENT

Problems with NIPPV in children with neuromuscular disease

• Impaired ability to trigger in child =>

• child-ventilator dyssynchrony =>

• increased work of breathing

• discomfort, potentially =>

• poor compliance (treatment) (and thus)

• lack of effect

Choose the right interface and put it in the right position

Choose the right ventilator

• Trigger pressure (sensitivity, inspiratory and expiratory)

• Time delay

• Flow rise time

• Durability, noise, simpliticity in setting etc

Is it complicated

• Yes

• No

• Clinical mode

– VE , SaO2 , respiratory rate, patient comfort

• Scientific/invasive mode

– Clinical + Pes, Pga

Thorax

Abdomen

BiPAP

Figure 3

Long term/chronic setting

Simonds, Thorax 1998;53:949

• Method: record review

• N = 14 (DMD, cong myopathy, myoton dyst)

• Age 7.7 yrs (1.5 – 16)

• Treatment: BiPAP (reg tm)

– Settings: ?

– Duration: 30 mos (6 – 84)

– h/day: ?

Results

• Hospitalization:

– 41.7 days/y before treatment -> 10.5 days/y

– Number of hosp stays: 3.8/y - > 0.7/y

– PICU days: 10.2/y -> 2.3/y

• Annual direct cost og health care/patient

– $ 55.129 -> 14.914

• 30 patients aged 12.4 + 4.1 yrs

• IPAP/EPAP: 13.9 (8-19)/4.4 (3-8) cm H2O

• Ventilated for 25.3 (8-60) mos

• Questionnaire

• 24 + NIV, 11 no NIV

• Ventilation > 36 mos

Fauroux, ICM 2005

Adverse effects of long term non-invasive ventilation

Retrograde position of maxillar teeth

Figure 5

• Appetite improved in 7 of 12

• Dyspnea disappeared in 8 of 11

• Swallowing improved in 6 of 7

Cough assist/ in-exufflator

INVASIV VENTILATION

WHEN ?

WHEN ?

• NIPPV is insufficient to oxygenate and/or ventilate the ventilator assisted individual (VAI) satisfactorily

• VAI is unable to be weaned

• VAI with no spontaneous respiration

• VAI with (advanced) bulbar insufficiency or other upper airway impairment

PERCUTANEOUS DILATION

Ventilator – invasive treatment

SUMMARY

• NIV & TIV works in children in the – Acute setting

– Chronic setting

• Perform appropiate diagnosis

• Use appropiate equipment

• Use appropiate ventilator settings

• Adverse effects should be monitored – In particular facial malformation

• Trained staff very important

Referral of children

• signs and/or symptoms of nocturnal hypoventilation (NH) during the night

• daytime symptoms of NH

• failure to thrive

Referral of children

• IVC < 60 % (=> SBD, < 40% => noct hypovent)

• MIP < 4.0 kPa (=> SDB, < 2.5 => noct hypovent)

• CPF < 270 l/min

• Daytime PaCO2 > 45 mmHg (=> noct hypovent)

Mistanke om en evt henvisning til RCV er relevant ?

• Ring

– 78451350/78451340

• Skriv

– olenorre@rm.dk