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University Medical Centre Groningen The Netherlands

Ventilatory support in chronic non - pulmonary diseases The dutch approach. University Medical Centre Groningen The Netherlands. 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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University Medical Centre Groningen The Netherlands

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  1. Ventilatory support in chronic non- pulmonary diseases The dutch approach University Medical Centre Groningen The Netherlands

  2. Chronic ventilatory support • Who may need it ? • How do we organise it? • Why might it work ? • When should we start ? • What are the effects ?

  3. Respiratory insufficiency Pump insufficiency Lung insufficiency hypoventilation hypoxemia hypercapnia Diffusion disorder V/Q Mismatch shunt

  4. Causes of alveolar hypoventilation Breathing centrecentraal apneu brainstem infarction Central cord/ nerves poliomyelitis / ALS Muscles Duchenne / Becker Thoracic cage Kyphoscoliosis Obesity Lung COPD / CF

  5. 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 ?

  6. Total number of patients Netherlands october 2007

  7. Diagnoses Netherlands october 2007

  8. Home Mechanical ventilation Groningen OSAS/OHS neuromuscular Restriction Lung Duiverman Respir Med. 2006 ;100:56-65

  9. Type of ventilatory support Netherlands october 2007

  10. Where do they live ? Netherlands october 2007

  11. 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 ?

  12. 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

  13. Resetting the CO2 setpoint Dellborg et al. Resp Med 2000;94:1154

  14. 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 ?

  15. When should we start NIV in Duchenne ? Toussaint Chron Respir Dis 2007;4:167

  16. Start when nocturnal hypoventilation occurs Ward Thorax 2005;60:1019

  17. Start in ALS Orthopnea with PImax < 60 % pred. Or symptomatic PaCO2 > 6.0 kPa Bourke Lancet Neurology 2006;5:140-7

  18. 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 ?

  19. Survival Duchenne patients Years 1 2 3 4 5 Meinesz et al. NTVG 2007;151:1803

  20. Survival restrictive disorders Post poliomyelitis Cong. kyphoscoliosis Miscellanous Duiverman Respir Med. 2006 ;100:56-65

  21. Quality of life in Duchenne patients Kohler AJRCCM 2005;172:1032-36

  22. ALS and quality of life Bourke Lancet Neurology 2006;5:140-7

  23. 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

  24. Thoracic-restriction Duiverman Respir Med. 2006 ;100:56-65

  25. Home mechanical ventilation • Organisation in the Netherlands • Type of patients selected for HMV • Effects of HMV • Specific problems with ALS

  26. Start when nocturnal hypoventilation occurs Ward Thorax 2005;60:1019

  27. Growth of ALS on HMV 10% 5%

  28. 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

  29. Case • Physical examination : Speaks loudly/ abdominal breathing / no orthopneu • Bloodgas : pH 7.39–pCO2 6.7-pO210-Bic 30-sat 95

  30. Case What to do ?

  31. Case July 2004 : starts NIPPV placement of PEG Oct 2005: very happy about NIPPV uses NIPPV during daytime very happy with PEG April 2006 uses NIPPV for 24 hrs a day Sept 2006 died

  32. Chronic NIV in ALS • Why ? • When to start ? • How ? • What to do if NIV doesn’t help anymore ?

  33. ALS and survival Bourke. Lancet Neurology 2006;5:140-7

  34. ALS and quality of life Bourke Lancet Neurology 2006;5:140-7

  35. Nutritional state Lo Coco. Neurology 2006;67:761

  36. Where do ALS patients live? Groningen oct 2006

  37. Dutch policy in ALS Was : No, unless ………. Now : Yes, if ….. Kampelmacher NTVG 2004;148:509 Meinesz NTVG 2006;150:449

  38. Chronische beademing bij ALS • Waarom ? • Wanneer ? • Hoe ? • Wat te doen als het niet helpt ?

  39. Wanneer starten ? • Longfunctie achteruitgang (VC ) ? • Aanwijzingen voor hypoventilatie ? • Symptomen ?

  40. Symptomen • Ochtend hoofdpijn • Kortademigheid • Slecht slapen • Niet meer plat kunnen liggen • Afvallen

  41. Studie populatie Bourke Lancet Neurology 2006;5:140-7

  42. 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?

  43. Oorzaak chronisch respiratoir falen Turkington, Thorax 2000;55;417

  44. Obesitas hypoventilatie syndroom Pickwick papers

  45. Obesitas hypoventilatie syndroom Chest wall Resp. muscles ventilatory drive leptin Can’t breathe Won’t breathe Martin and Sanders. Sleep 1995;118:617

  46. O’Donell CP. AJRCCM 1999;159:1484.

  47. 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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