ALS:  Tolerance and survival predictors Cáceres, 10 de Noviembre de 2007 Dr. Joan Escarrabill   Hospital Universitari de B...
Agenda <ul><li>Predictors of tolerance </li></ul><ul><li>NIV and survival </li></ul><ul><li>Survival and care organization...
ALS: some figures Forbes RB et al. J Neurol Neurosurg Psychiatry 2004;75:1753-55 Kimura F et al. Neurology 2006;66:265-7 M...
Predictors PEG Care organization Bulbar involvement at onset NIV Secretions magement Nihilism Autonomy Management of acute...
Limitations of the studies <ul><li>Long-term studies: </li></ul><ul><ul><li>Different diagnostic criteria </li></ul></ul><...
Amyotrophic lateral sclerosis Onset of symptoms First visit Onset  NIV Medical delay Unclear first symptom Precocious NIV ...
Amyotrophic lateral sclerosis Onset of symptoms First visit Onset  NIV Medical delay Unclear first symptom Palliative End-...
Amyotrophic lateral sclerosis Onset of symptoms First visit Onset  NIV Medical delay Unclear first symptom Precocious NIV ...
J Neurol 2006;253:1428-36 Better survival Younger age Limb onset Longer delay before first examination Higher initial FVC ...
Neurology 2005;64:38-43 Symptom duration at baseline visit  was a significant predictor of survival Shorter duration = Hig...
J Neurol Neurosurg Psychiatry 2004;75:1753-55 Scottish ALS Register n=1226 2.4 months Medical nihilism ? Riluzole & PEG us...
ALS: Acute chest infection Servera E. J Neurol Sci 2003;209:111-3 65 yrs old man Daytime Mouth piece Nasal mask for noctur...
Secretions management MI-E Ventilador+30 +TAT Ventilador+TAT Air stacking+TAT TAT SPON + mot SPON 1100 1000 900 800 700 60...
Bulbar impairment Bulbar Non-Bulbar Bourke SC. Lancet Neurol 2006;5:140-7
Survival in ALS patients with bulbar involvement Farrero et al. Chest 2007;127:2132-8 NIV Tolerance NIV intolerance NIV To...
Agenda <ul><li>Predictors of tolerance </li></ul><ul><li>NIV and survival </li></ul><ul><li>Survival and care organization...
Survival on HMV Laub M & Midgren B. Respir Med 2007;101:1074-8 n=1526 ALS
Indication of NIV Melo J. J Neurol Sci 1999;169:114-7 n=2357 patients 15% on NIV
Lancet Neurol 2006;5:140-7
Lancet Neurol 2006;5:140-7
Lancet Neurol 2006;5:140-7 Time SAQLI symptoms domain maintained above 75% of prerandomization assessment Bulbar Non-Bulbar
Are NIV trials necessaries in ALS with non-bulbar impairement? Servera E. Sancho S. Lancet Neurol 2006;5:140-7 Ethical iss...
Chest 2007;132:62-9 Median survival time  37 months Lenght of hospital stay 55 days (7-124) 91%  home discharge
Agenda <ul><li>Predictors of tolerance </li></ul><ul><li>NIV and survival </li></ul><ul><li>Survival and care organization...
Zoccolella S et al. J Neurol 2007;254:1107-12 No improvements in survival:  Low rate of interventions?
Survival of Irish ALS patients One year mortality was decreased by  29.7%
Survival of Irish ALS patients with bulbar onset Prognosis of bulbar onset patients  was extended by 9.6 months
ALS patients who received their care at a multidisciplinary clinic  had a better prognosis Recruitment bias ALS clinic  tr...
J Neurol Neurosurg Psychiatry 2006;77:948-50 Tertiary center Neurology clinic 1080 days 775 days The median survival from ...
Chest 2007;127:2132-8 Early systematic respiratory evaluation  is necessary to improve the results Of HMV in ALS Survival ...
www.has-sante.fr/ 2003 17 Reference centers Coodination Working groups Local organization
Local organization Hôpital Pitié Salpetière Paris Multidisciplinary team Day hospital Respiratory unit Median survival on ...
Reference centers:  benefits and limits Improve skills & knowledge Distance Unnecessary referals + - Escarrabill J. Arch B...
Conclusions Nihilism is bad It seems that patients don’t like  agressive treatments Integrated care is a good option
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CáCeres (Nov 07) Final

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V symposium on non-invasive ventilation. Cáceres 10 nov 2007

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CáCeres (Nov 07) Final

  1. 1. ALS: Tolerance and survival predictors Cáceres, 10 de Noviembre de 2007 Dr. Joan Escarrabill Hospital Universitari de Bellvitge L’Hospitalet (Barcelona) [email_address]
  2. 2. Agenda <ul><li>Predictors of tolerance </li></ul><ul><li>NIV and survival </li></ul><ul><li>Survival and care organization </li></ul>
  3. 3. ALS: some figures Forbes RB et al. J Neurol Neurosurg Psychiatry 2004;75:1753-55 Kimura F et al. Neurology 2006;66:265-7 Mean age at onset 65 yrs From onset to diagnosis 15 months Czaplinski A et al. J Neurol 2006;253:1428-36 Diagnostic Overall median survival 2-4 yrs Five years survival 11% Survival > 8 years 4% Survival
  4. 4. Predictors PEG Care organization Bulbar involvement at onset NIV Secretions magement Nihilism Autonomy Management of acute problems Age at onset
  5. 5. Limitations of the studies <ul><li>Long-term studies: </li></ul><ul><ul><li>Different diagnostic criteria </li></ul></ul><ul><ul><li>Incomplete data </li></ul></ul><ul><li>Retrospective studies </li></ul><ul><li>Data from patients that “knock to the door”: not population-based studies </li></ul><ul><li>The role of PEG and NIV are, in general, underconsidered </li></ul><ul><li>No biomarkers </li></ul>
  6. 6. Amyotrophic lateral sclerosis Onset of symptoms First visit Onset NIV Medical delay Unclear first symptom Precocious NIV Palliative End-of-life NIV Studies are difficult to compare
  7. 7. Amyotrophic lateral sclerosis Onset of symptoms First visit Onset NIV Medical delay Unclear first symptom Palliative End-of-life NIV Precocious NIV
  8. 8. Amyotrophic lateral sclerosis Onset of symptoms First visit Onset NIV Medical delay Unclear first symptom Precocious NIV Palliative End-of-life NIV
  9. 9. J Neurol 2006;253:1428-36 Better survival Younger age Limb onset Longer delay before first examination Higher initial FVC = Longer survival Lower initial AALS score <ul><ul><li>Includes assessment of swallowing , speech , and respiratory function , and both strength and function of upper and lower extremity musculature </li></ul></ul>Appel V. Ann Neurol. 1987;22:328-33.
  10. 10. Neurology 2005;64:38-43 Symptom duration at baseline visit was a significant predictor of survival Shorter duration = Higher mortality Symptom duration at first visit related to rate of disease progression ALSFRS-R Physical function in daily living activities Salivation Swallowing 4 3 2 1 0 4 3 2 1 0 Cedarbaum JM. J Neurol Sci 1999;169:13-21
  11. 11. J Neurol Neurosurg Psychiatry 2004;75:1753-55 Scottish ALS Register n=1226 2.4 months Medical nihilism ? Riluzole & PEG use increases Patient autonomy Less aggressive therapy
  12. 12. ALS: Acute chest infection Servera E. J Neurol Sci 2003;209:111-3 65 yrs old man Daytime Mouth piece Nasal mask for nocturnal use
  13. 13. Secretions management MI-E Ventilador+30 +TAT Ventilador+TAT Air stacking+TAT TAT SPON + mot SPON 1100 1000 900 800 700 600 500 400 300 200 100 0 ٭ ٭ ٭ ٭ ٭ † † † † ‡ ‡ ‡ • PCF l/mn Courtesy of Cristina Senent MD (H. Sant Joan - Alacant) & Jesus González MD (Hôpital Pitié-Salpetière - Paris)
  14. 14. Bulbar impairment Bulbar Non-Bulbar Bourke SC. Lancet Neurol 2006;5:140-7
  15. 15. Survival in ALS patients with bulbar involvement Farrero et al. Chest 2007;127:2132-8 NIV Tolerance NIV intolerance NIV Tolerance NIV intolerance NIV Tolerance NIV intolerance Hypercapnia Normocapnia
  16. 16. Agenda <ul><li>Predictors of tolerance </li></ul><ul><li>NIV and survival </li></ul><ul><li>Survival and care organization </li></ul>
  17. 17. Survival on HMV Laub M & Midgren B. Respir Med 2007;101:1074-8 n=1526 ALS
  18. 18. Indication of NIV Melo J. J Neurol Sci 1999;169:114-7 n=2357 patients 15% on NIV
  19. 19. Lancet Neurol 2006;5:140-7
  20. 20. Lancet Neurol 2006;5:140-7
  21. 21. Lancet Neurol 2006;5:140-7 Time SAQLI symptoms domain maintained above 75% of prerandomization assessment Bulbar Non-Bulbar
  22. 22. Are NIV trials necessaries in ALS with non-bulbar impairement? Servera E. Sancho S. Lancet Neurol 2006;5:140-7 Ethical issues Non-bulbar patients in control group Stop studies according the results Technical issues Assessment effects of NIV Pressure vs volume ventilators Secretion management It’s mandatory to evaluate therapy “package”
  23. 23. Chest 2007;132:62-9 Median survival time 37 months Lenght of hospital stay 55 days (7-124) 91% home discharge
  24. 24. Agenda <ul><li>Predictors of tolerance </li></ul><ul><li>NIV and survival </li></ul><ul><li>Survival and care organization </li></ul>
  25. 25. Zoccolella S et al. J Neurol 2007;254:1107-12 No improvements in survival: Low rate of interventions?
  26. 26. Survival of Irish ALS patients One year mortality was decreased by 29.7%
  27. 27. Survival of Irish ALS patients with bulbar onset Prognosis of bulbar onset patients was extended by 9.6 months
  28. 28. ALS patients who received their care at a multidisciplinary clinic had a better prognosis Recruitment bias ALS clinic treated a group of fitter ALS patients General neurologists saw all ALS patients Living further from ALS clinic More disabled Increased age Bulbar onset Shorter duration of illness Hutchinson M. J Neurol Neurosurg Psychiatry 2004;75:1208-12
  29. 29. J Neurol Neurosurg Psychiatry 2006;77:948-50 Tertiary center Neurology clinic 1080 days 775 days The median survival from onset was 10 months longer in ALS centers 4 yrs younger PEG & NIV more often Less hospital admissions
  30. 30. Chest 2007;127:2132-8 Early systematic respiratory evaluation is necessary to improve the results Of HMV in ALS Survival in patients without bulbar involvement Protocol Pre-Protocol
  31. 31. www.has-sante.fr/ 2003 17 Reference centers Coodination Working groups Local organization
  32. 32. Local organization Hôpital Pitié Salpetière Paris Multidisciplinary team Day hospital Respiratory unit Median survival on HMV: 18 months J. González-Bermejo, MD Visite de “ retourn d’annonce” Care planning Social support Call center 2-3 weeks after diagnostic
  33. 33. Reference centers: benefits and limits Improve skills & knowledge Distance Unnecessary referals + - Escarrabill J. Arch Bronconeumol 2007;43:527-9 Patient-centered care : accessibility vs performance
  34. 34. Conclusions Nihilism is bad It seems that patients don’t like agressive treatments Integrated care is a good option

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