University Medical Centre Groningen The Netherlands
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Transcript of University Medical Centre Groningen The Netherlands
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University Medical Centre Groningen
The Netherlands
Ventilatory support in chronic non- pulmonary diseases
The dutch approach
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Chronic ventilatory support
• Who may need it ?
• How do we organise it?
• Why might it work ?
• When should we start ?
• What are the effects ?
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Respiratory insufficiency
Pump insufficiency Lung insufficiency
hypoxemia
shunt
Diffusion disorder V/Q Mismatch
hypercapnia
hypoventilation
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Causes of alveolar hypoventilation
Breathing centre centraal apneubrainstem infarction
Central cord/ nerves poliomyelitis / ALS
Muscles Duchenne / Becker
Thoracic cage KyphoscoliosisObesity
Lung COPD / CF
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Home Mechanical ventilation
• Who may need it ?
• How do we organise it in the Netherlands ?
• Why might it work ?
• When should we start ?
• What are the effects ?
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Total number of patients
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1991 1996 2001 2006 2007
Netherlands october 2007
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Diagnoses
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Thoracic cage Neuromusc Lung Apnea
2001200520062007
Netherlands october 2007
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Restriction
neuromuscular
OSAS/OHS
Lung
Home Mechanical ventilation Groningen
Duiverman Respir Med. 2006 ;100:56-65
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Type of ventilatory support
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Utrecht Maastricht Rotterdam Groningen
TIPPVNIPPV
Netherlands october 2007
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Where do they live ?
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Home Supervhome
Nursinghome
rest
2001200520062007
Netherlands october 2007
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Home Mechanical ventilation
• Who may need it ?
• How do we organise it in the Netherlands?
• Why does it work ?
• When should we start ?
• What are the effects ?
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Why does NIV work ?
• Resting the muscles
• Improve the compliance of rib the cage
• Improving sleep efficiency
• Resetting of CO2 setpoint
Mehta S and Hill NS. AJRCCM 2001;163:540
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Dellborg et al. Resp Med 2000;94:1154
Resetting the CO2 setpoint
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Home Mechanical ventilation
• Who may need it ?
• How do we organise it in the Netherlands?
• Why does it work ?
• When should we start ?
• What are the effects ?
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When should we start NIV in Duchenne ?
Toussaint Chron Respir Dis 2007;4:167
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Start when nocturnal hypoventilation occurs
Ward Thorax 2005;60:1019
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Start in ALS
Orthopnea with PImax < 60 % pred.
Or symptomatic PaCO2 > 6.0 kPa
Bourke Lancet Neurology 2006;5:140-7
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Home Mechanical ventilation
• Who may need it ?
• How do we organise it in the Netherlands?
• Why does it work ?
• When should we start ?
• What are the effects ?
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0
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1 2 3 4 5
Survival Duchenne patients
Years
Meinesz et al. NTVG 2007;151:1803
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Survival restrictive disorders
Duiverman Respir Med. 2006 ;100:56-65
Post poliomyelitis
Miscellanous
Cong. kyphoscoliosis
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Quality of life in Duchenne patients
Kohler AJRCCM 2005;172:1032-36
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ALS and quality of life
Bourke Lancet Neurology 2006;5:140-7
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Conclusions
• Home mechanical ventilation (HMV) can be set up effectively in restrictive (non pulmonary) lung diseases
• Non invasive ventilation is the preferable treatment
• Monitor the patients closely to start HMV when necessary
• HMV increases survival and quality of life
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Thoracic-restriction
n acute Age VC PaCO2
Kypho 60 42% 54(13) 1.1(0.5) 7.7(1.8)
Polio 27 48% 49(16) 1.2(0.6) 7.4(1.4)
Misc. 19 53% 63(10) 1.1(0.4) 8.0(2.2)
Duiverman Respir Med. 2006 ;100:56-65
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Home mechanical ventilation
• Organisation in the Netherlands
– Type of patients selected for HMV
– Effects of HMV
• Specific problems with ALS
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Start when nocturnal hypoventilation occurs
Ward Thorax 2005;60:1019
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Growth of ALS on HMV
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2001 2007
ALSTotaal
5% 10%
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Case
Man 67 years
History 2004 april : ALS
2004 juni
tired / shortness of breath / works 4 for hours on 3 days / sleeps well / no problems to lie flat / no headache / lost 8 kg in 4 months
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Case
• Physical examination :
Speaks loudly/ abdominal breathing / no orthopneu
• Bloodgas : pH 7.39–pCO2 6.7-pO210-Bic 30-sat 95
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Case
What to do ?
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Case
July 2004 : starts NIPPV placement of PEG
Oct 2005: very happy about NIPPVuses NIPPV during daytimevery happy with PEG
April 2006 uses NIPPV for 24 hrs a day
Sept 2006 died
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Chronic NIV in ALS
• Why ?
• When to start ?
• How ?
• What to do if NIV doesn’t help anymore ?
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ALS and survival
Bourke. Lancet Neurology 2006;5:140-7
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ALS and quality of life
Bourke Lancet Neurology 2006;5:140-7
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Nutritional state
Lo Coco. Neurology 2006;67:761
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Where do ALS patients live?
home
nursing home
Groningen oct 2006
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Dutch policy in ALS
Was : No, unless ……….
Now : Yes, if …..
Kampelmacher NTVG 2004;148:509
Meinesz NTVG 2006;150:449
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Chronische beademing bij ALS
• Waarom ?
• Wanneer ?
• Hoe ?
• Wat te doen als het niet helpt ?
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Wanneer starten ?
• Longfunctie achteruitgang (VC ) ?
• Aanwijzingen voor hypoventilatie ?
• Symptomen ?
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Symptomen
• Ochtend hoofdpijn
• Kortademigheid
• Slecht slapen
• Niet meer plat kunnen liggen
• Afvallen
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Studie populatie
N=41
Orthopneu N=38
Symptomatische Hypercapnie
N=3
PaCO2 < 6.0 N=20
PaCO2 > 6.0 N=18
Bourke Lancet Neurology 2006;5:140-7
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Alveolaire hypoventilatie
• Bij wie kan het ontstaan ?
• Hoe kun je het behandelen ?
• Wanneer is er indicatie voor chronische beademing ?
• Waarom werkt chronische beademing ?
• Wat zijn de effecten van chronische beademing?
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Oorzaak chronisch respiratoir falen
Load Drive Capacity
NMD =
TR =
Lung =
Central hypoventilation
= =
Turkington, Thorax 2000;55;417
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Pickwick papers
Obesitas hypoventilatie syndroom
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Obesitas hypoventilatie syndroom
Chest wall
Resp. muscles
Can’t breathe Won’t breathe
ventilatory drive
leptin
Martin and Sanders. Sleep 1995;118:617
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O’Donell CP. AJRCCM 1999;159:1484.
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Alveolaire hypoventilatie
• Bij wie kan het ontstaan ?
• Hoe kun je het behandelen ?
• Wanneer is er indicatie voor chronische beademing ?
• Waarom werkt chronische beademing ?
• Wat zijn de effecten van chronische beademing ?
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Restriction
neuromuscular
OSAS/OHS
Lung
Centrum voor thuisbeademing Groningen
Duiverman Respir Med 2006;100:56-65
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Wie behandelen wij in Nederland?
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spierzenuw long thoraxwand apneu overig
Centra voor thuisbeademing oktober 2005
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Hoe worden zij behandeld?
non invasief
invasief
Centra voor thuisbeademing oktober 2005
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Waar worden zij behandeld?
thuis
woonvorm
verpleeghuis
elders
Centra voor thuisbeademing oktober 2005
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Alveolaire hypoventilatie
• Bij wie kan het ontstaan ?
• Hoe kun je het behandelen ?
• Wanneer is er indicatie voor chronische beademing ?
• Waarom werkt chronische beademing ?
• Wat zijn de effecten van chronische beademing ?
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De rationale van chronische beademing
• Uitrusten van de ademhalingsspieren
• Verbeteren van de compliance
• Verlagen van het setpoint voor CO2
Mehta S and Hill NS. AJRCCM 2001;163:540
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Masa Jimenez JF. Chest 1995;107:382
Uitrusten van de ademhalingsspieren
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Annane ERJ 1999;13:157-163
Chronische beademing en ventilatoire respons
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Mellies ERJ 2003;22:631
NIPPV in NMD
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NIPPV in NMD
Mellies ERJ 2003;22:631
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Alveolaire hypoventilatie
• Bij wie kan het ontstaan ?
• Hoe kun je het behandelen ?
• Wanneer is er indicatie voor chronische beademing ?
• Waarom werkt chronische beademing ?
• Wat zijn de effecten van chronische beademing ?
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ALS en survival
Bourke. Lancet Neurology 2006;5:140-7
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Buyse et al .Eur Respir J. 2003;22:525-8
Kyphoscoliose en survival
NIPPV
Zuurstof
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Kwaliteit van leven in M. Duchenne
Kohler AJRCCM 2005;172:1032-36
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Conclusies • Alveolaire hypoventilatie veroorzaakt door extra-pulmonale
oorzaken is goed behandelbaar
• Chronische positieve drukbeademing is een geaccepteerde en goed te implementeren behandeling
• Chronische beademing is effectief en leidt veelal tot een betere survival en toegenomen kwaliteit van leven
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Beademen of niet beademen
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Casus
• Vrouw 70 jaar • Vg. poliomyelitis als kind kyphoscoliose
• 11 / 2000 : dyspneu spoedopvang – A: al mnd. in stoel , kan niets meer – LO : adipositas, Ag li , dik warm been re – X : deformiteit thorax, sluiering LOK,
hoogstand li-hemidiafragma – BGA : 7.31-9.3-6.4-54-79 (+ 2 l/min)
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Casus
• R / afdeling : Augmentin / vernevelen / O2
• Suffer, PaCO2 12 kPa
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Casus
Wat te doen ?
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Casus
• Intensive care : geïntubeerd
• 14/12 : gedetubeerd
• afdeling : 4/1 ontslagen
• afspraak met familie : geen re-intubatie
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Casus
• 20/2 : totale resp. Insufficiëntie – BGA : 7.29 - 15.4 - 4.5 - 54 - 65 (geen O2)
• Discussie tussen intensivist en longarts over het te volgen beleid
• Wat te doen ?
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Casus
• Geïntubeerd
• op 23/2 overgeplaatst naar AZG
• wegens overvulling lasix iv 12 kg • 2/3 gedetubeerd start NIPPV
• geen succes, PaCO2 , intubatie
• 6/3 tracheostoma, start TIPPV
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Casus
• Ontwennen, uiteindelijk alleen ‘s nachts
• Mobiliseren
• ontslag (18/4)
– TIPPV alleen s’nachts – overdag zuurstof (1 l/min)
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Casus
Oktober 2001 : polikliniek CTB
– erg goed , 2 keer kuurtje
– tijdens opname 25 kg afgevallen
– VC 1.4 L
– BGA : 7.37-6.5-11-27-96 (O2 1/2 l/min)
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Casus
November 2002 : polikliniek CTB
– klinisch goed
– VC 1.5 l
– BGA : 7.38 - 6.6 - 9.5 - 29 - 95 ( - O2 )
– alleen nog inhalatie medicatie– TIPPV s’nachts (8 uur)
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Casus
Maart 2005 : polikliniek CTB
– klinisch goed
– VC 1.5 l
– BGA : 7.38 - 6.6 - 9.5 - 29 - 95 ( - O2 )
– TIPPV s’nachts ( 8 uur)– Overzetten op NIPPV
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Casus
1 november 2005 : wederom opname IC
Totale respiratoire insufficiëntie
PaCO2 11 kPa
Geïntubeerd
KNO legt opnieuw stoma aan
Plan herstart TIPPV 4/2006: pH 7.35-pCO2 7.1–pO2 12.4- Bic27-sat 97 (+1l/min)
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Wanneer starten ?
1) PaCo2 > 7.3 kPa overdag of s’nachts
2) PaCo2 6.7-7.3 overdag of s’nachts
en klachten van :ochtendhoofdpijn, onrustige slaap,
vermoeidheid, slaperigheid, gewichtsverlies
Goldberg et al.Chest 1999;116:521
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NIPPV en starre thorax
Baseline 12 months
PaO2 6.4(1.2) 8.9(1.3)
PaCO2 7.8(1.0) 5.9(0.6)
SaO2 73(9) 91(5)
PAPmean 33(10) 25(6)
Schonhofer. Thorax 2001; 56:524
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Criner et al. Chest 1999;116:667
NIPPV en functionele score
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Schonhofer. ERJ 1997;10:218
ADL en PaCO2
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Survival UMCG
Duiverman et al. Respir Med 2006;100:56-65
Post poliomyelitis
mengvorm
Cong. kyphoscoliosis
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Conclusie
• Ademhalingsondersteuning bij patienten met een starre thorax is effectief
• de overleving na de start van ademhalingsondersteuning is goed
• Starten bij combinatie van hypercapnie en klachten
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Casus 2
Man, 55 jaar
• VG / Adipositas / hypertensie
• A / Dyspnoea, veelvuldig exacerbaties, gaat niet goed, moe
• Med / Foradil, Combivent, Flixotide Spirinolacton, Burinex, ascal, renitec
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Casus (2)
Man, 55 jaar
• LO / RR 135/85, gewicht 135 kg, BMI 34zacht ademgeruis, verder g.b.
• LF / FEV1 1.3 L (3.7) / VC 2.7 L (4.8)Tiff 30% / TLC 98 % pred.TLCO / VA 85 %pred.
BGA 7.35- 7.8- 7.4- 35- 87% (- O2)
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Casus 2
Man, 55 jaar
• Conclusie ?
• Wat te doen ?
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Casus (2)
Na 1 jaar NIPPV en daarna succesvolle revalidatie
– voelt zich stukken beter (“in andere wereld”)
– kan verder lopen
– BGA : 7.39-5.6-10.3-26-95 ( - O2)
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Wanneer starten ?
PaCO2 > 8.0 kPa overdag of s’nachts
en klachten van nachtelijke hypoventilatie
en ≥ 2 opnames/jaar wegens respiratoir falen
Goldberg et al. Chest 1999;116:521
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Wie behandelen wij in Nederland?
0
100
200
300
400
500
600
700
spierzenuw long thoraxwand apneu overig
Centra voor thuisbeademing oktober 2005
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Randomised controlled trials
Psych.Dysp.
12/46.70.85Casanova2000
=10/27.30.68Gay 1996
QOLGasex. Sleep
18/27.40.86Meecham Jones 1995
Psych. 15/26.50.54Strumpf 1991
EffectenBIpapPaCO2FEV1Studie
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Outcome Sample Treatment effect
Mean
Treatment effect
95 % CI
FVC, L 33/33 -0.01 -0.14 , 0.13
Pimax, cm H2O 24/24 6.2 0.2 , 12.2
Pemax, cm H2O 24/24 18.4 -11.8 , 48.6
PaO2, mmHg 33/33 0.0 -3.8 , 3.9
PaCO2, mmHg 33/33 -1.5 -4.5 , 1.5
6-MWD, m 12/11 27.5 -26.8 , 81.8
Sleep eff., % 13/11 -4.0% -14.7 , 6.7
Wijkstra. Chest 2003;124:337-43
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Uncontrolled trials
BGA 12/48.10.90Jones
BGA 10/28.60.70Sivasothy
QOLBGA
18/27.80.86Perrin
BGA 15/280.53Elliot
EffectenBIpapPaCO2FEV1Studie
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Nachtelijke hypoventilatie
Meecham Jones et al.1995:152:538-544
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Prevalentie van hypoventilatie in COPD
O’Donoghue et al.ERJ 2003;21:977
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Chu et al. Thorax 2004;59-1020
Kans op heropname na NIPPV
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Chu et al. Thorax 2004;59-1020
SURVIVAL na NIPPV
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Chronische beademing en COPD
Nachtelijke ademhalingsondersteuning bij
patienten met respiratoire insufficiëntie
t.g.v. een stabiel ernstig COPD heeft
geen invloed op het verloop en de
prognose
Ketenzorg COPD NVALT/CBO
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COPD en chronische NIPPV
1) NIPPV en revalidatie ( RECOVER) loopt sinds 2004
2) NIPPV na acuut respiratoir falen gaat mogelijk lopen in 2007
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Casus 3
67 jarige manVG : 2003 Zenkers divertikel
4/2004 ALS
6/2004 A: moe/luchttekort/ werkt nog 3 halve dagen
/ slaapt goed / kan nog plat liggen/ geen hoofd pijn / 8 kg afgevallen in 4 mnd.
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Casus 3
• Lich. Ond :
Praat duidelijk, kan plat liggen, wel buikademhaling
• Verder onderzoek
BGA : pH 7.39–pCO2 6.7-pO210-Bic 30-sat 95
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Casus 3
Wat nu ?
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Casus 3
7/2004 : start NIPPV
plaatsing PEG sonde
10/2005: nog steeds tevreden over NIPPV
ook overdag NIPPV
zeer blij met PEG
4/2006 bijna gehele dag NIPPV
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ALS en survival
Bourke. Lancet Neurology 2006;5:140-7
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ALS en kwaliteit van leven
Bourke Lancet Neurology 2006;5:140-7
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Inclusie criteria
Orthopneu met PImax < 60 % pred.
Of symtomatische PaCO > 6.0 kPa
Bourke Lancet Neurology 2006;5:140-7
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Studie populatie
N=41
Orthopneu N=38
Symtomatische Hypercapnie
N=3
paCO2 < 6.0 N=20
PaCO2 > 6.0 N=18
Bourke Lancet Neurology 2006;5:140-7
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Miller et al. Neurology 1999;52:1311
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1e gesprek TIPPV/NIPPVdoor neuroloog
Patiënt wijst het niet af
Patiënt wil geen NIPPV
Vitale capaciteit < 80%
2e gesprek neuroloog of revalidatie arts
Patiënt wil eventueel wel NIPPV/TIPPV
Verwijzing CTB
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Beleid CTB bij ALS
Was : Nee, tenzij……….
Momenteel : Ja, mits
Kampelmacher NTVG 2004;148:509
Meinesz NTVG 2006;150:449
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Non invasieve beademing in ALS
• Retrospectieve studie (n=122)
• BiPAP bij symptomen en/of
– FVC < 50% predicted of– FVC daling > 15% in 3 mnd
Kleopa et al . J Neurol Sc 1999;164:82
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Non invasive ventilation in ALS
539Riluzole
241415PEG
85 (13)98 (23)83 (18)FVC
17/3512/2014/24Bul/limb
64 (11)63 (13)60(13)age
no BiPAPBiPAP < 4u/dgBiPAP > 4 u/dg
Kleopa et al . J Neurol Sc 1999;164:82
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SURVIVAL
postpolio
myopathy
kyphoscoliose
Sequelae tbc
COPDbronchiectasis
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Schonhofer. ERJ 1997;10:218
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Restrictie
neuromusculair
OSAS/OHS
Long
Thuisbeademing Groningen
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Buyse et al .Eur Respir J. 2003;22:525-8
ZUURSTOF of BEADEMEN
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Thorax-restrictie
n acuut Leeftijd VC PaCO2
Kypho 60 42% 54(13) 1.1(0.5) 7.7(1.8)
Polio 27 48% 49(16) 1.2(0.6) 7.4(1.4)
meng 19 53% 63(10) 1.1(0.4) 8.0(2.2)
Duiverman Respir Med 2006; 100:56-65
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Janssens et al. Chest. 2003;123:67-79
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Survival restrictieve longfunctie stoornis
Duiverman Respir Med 2006;100:56-65
Post poliomyelitis
Miscellanous
Cong. kyphoscoliosis
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Indicatie voor beademing
Reden percentage
Hypercapnie overdag 3 8.1
Nachtelijke hypoventilatie 11 29.7
Gewichtsverlies 3 8.1
Ochtend hoofdpijn 1 2.7
Rec. infecties 6 16.2
Meerdere 9 24.3
Rest 4 10.8
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Verloop longfunctie
Baseline (n=28)
18 mnd (n=18)
36 mnd (n=11)
VC 1.0 (0.4-2.7) 0.9(0.3-3.0) 0.7(0.2-1.7))
PaCO2,max
(kPa)`s nachts
7.5(5.6-10.2)
PaCO2,max
(kPa) overdag
7.0(4.2-11.0) 5.9(4.2-7.8)
PaO2,max
(kPa) overdag
10.7(6.9-18) 12 (8.7-12)
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Doodsoorzaak
aantal
tracheabloeding 3Tracheamalacie 1Cardiomyopathy 3Pleura-empyeem 1Post-anoxischeencefalopathie na OK
1
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Toekomst NIPPV/TIPPV
Middelen
• Airstacking
• Mondstuk-beademing
• Frogbreathing
• In/exsufflator
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Peak cough flows
0
1
2
3
4
5
6
7
8
9
10
UPCF stack assisted exsufflator
L/sec
Bach, Chest 1993;104:1553
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OHS OSAS COPD
PaCO2, mmHg 49 7 38 4 42 6
Tsat< 90%, % 52 32 9 18 20 26
Ppa, mmHg 23 10 15 5 20 6
Kessler et al. Chest 2001;120:369
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Alveolaire hypoventilatie
• Hypoventilatie veroorzaakt altijd een verhoogde PaCO2
PaCO2 = (VCO2 / VA ) X K
• Hypoxaemie kan worden opgeheven door zuurstof toe te dienen met een masker
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Thoracic-restriction
n acute Age VC PaCO2
Kypho 60 42% 54(13) 1.1(0.5) 7.7(1.8)
Polio 27 48% 49(16) 1.2(0.6) 7.4(1.4)
Misc. 19 53% 63(10) 1.1(0.4) 8.0(2.2)
Duiverman et al. Respir Med 2005
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Drukgestuurde NIV en slaap
• Wat betekent NIV voor Nederland ?
• Waarom is NIV effectief?
• Wat zijn de effecten van NIV bij– langzaam progressieve vorm ( M.Duchenne)
– snel progressieve vorm (ALS)
• Wanneer beginnen met NIV?
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Totaal aantal patiënten
0
200
400
600
800
1000
1200
1400
1600
1991 1996 2001 2006 2007
Nederland 1-10-2007
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Diagnose groep
0
100
200
300
400
500
600
700
800
Thoraxwand Neuromusc long apneu
2001200520062007
Nederland 1-10-2007
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Type beademing 2007
0
100
200
300
400
500
600
700
Utrecht Maastricht Rotterdam Groningen
TIPPVNIPPV
Nederland 1-10-2007
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Drukgestuurde NIV en slaap
• Wat betekent NIV voor Nederland ?
• Wat is de rationale van NIV ?
• Wat zijn de effecten van NIV bij– langzaam progressieve vorm (M.Duchenne)
– snel progressieve vorm (ALS)
• Wanneer beginnen met NIV?
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Masa Jimenez JF. Chest 1995;107:382
Uitrusten van de ademhalingsspieren
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Dellborg et al. Resp Med 2000;94:1154
Ventilatoire respons
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Verbeterde slaapefficientie
0
50
100
150
200
250
300
350
400
TST RDI S1-2 REM SE SaO2<90
Baseline
NIPPV
Barbé Chest 1996;110 : 1179
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Drukgestuurde NIV en slaap
• Wat is NIV?
• Wat is de rationale van NIV?
• Wat zijn de effecten van NIV bij– langzaam progressieve vorm ( M.Duchenne)
– snel progressieve vorm (ALS)
• Wanneer beginnen met NIV?
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NIPPV in NMD
Mellies ERJ 2003;22:631
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Mellies ERJ 2003;22:631
NIPPV in NMD
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NIV en gaswisseling
Mellies ERJ 2003;22:631
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0
10
20
30
40
50
60
70
80
90
100
1 2 3 4 5
Survival curve M. Duchenne
Jaren
Meinesz et al. NTVG 2007;151:1803
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Improving sleep effiency
0
10
20
30
40
50
60
RDI-REM Arousal S1-2 S3-4 REM
Baseline
NIPPV
Mellies Eur Respir J 2003;22:631
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Drukgestuurde NIV en slaap
• Wat is NIV?
• Wat is de rationale van NIV?
• Wat zijn de effecten van NIV bij– langzaam progressieve vorm ( M.Duchenne)
– snel progressieve vorm (ALS)
• Wanneer beginnen met NIV?
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Studie populatie
N=41
Orthopneu N=38
Symtomatische Hypercapnie
N=3
paCO2 < 6.0 N=20
PaCO2 > 6.0 N=18
Bourke Lancet Neurology 2006;5:140-7
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ALS en survival
Bourke. Lancet Neurology 2006;5:140-7
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ALS en kwaliteit van leven
Bourke Lancet Neurology 2006;5:140-7
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Drukgestuurde NIV en slaap
• Wat is NIV?
• Wat is de rationale van NIV ?
• Wat zijn de effecten van NIV bij– langzaam progressieve vorm ( M.Duchenne)
– snel progressieve vorm (ALS)
• Wanneer beginnen met NIV?
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Start NIV in ALS
Orthopneu met PImax < 60 % pred.
Of symtomatische PaCO2 > 6.0 kPa
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Conclusies • Drukgestuurde NIV wordt steeds vaker
gebruikt
• NIV is bij NMA zeer effectief t.a.v.– Verbeterde slaap– Verbeterde gaswisseling – Verbeterde kwaliteit van leven– Verbeterde survival
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Conclusies
• Bij Duchenne kan NIV al effectief zijn indien gestart wordt bij nachteljke hypoventilatie al of niet met klachten
• Bij ALS moet NIV in vroeg stadium overwogen worden om op het juiste moment te kunnen starten
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Verandering in type NIV
Janssens Chest 2003;123:67
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Annane ERJ 1999;13:157-163
Ventilatoire respons
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Home mechanical ventilation
• Organisation in the Netherlands
– Type of patients selected for HMV
– Effects of HMV
• Specific problems with ALS