cáceres (nov 07) final
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ALS:
Tolerance and survival predictors
ALS:
Tolerance and survival predictors
Cáceres, 10 de Noviembre de 2007
Dr. Joan Escarrabill Hospital Universitari de Bellvitge
L’Hospitalet (Barcelona)jescarra@csub.scs.es
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Agenda
Predictors of tolerance
NIV and survival
Survival and care organization
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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
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Predictors
PEG
Care organization
Bulbar involvement at onset
NIV
Secretions magement
Nihilism
Autonomy
Management of acute problems
Age at onset
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Limitations of the studies
Long-term studies:– Different diagnostic criteria– Incomplete data
Retrospective studies
Data from patients that “knock to the door”: not population-based studies
The role of PEG and NIV are, in general, underconsidered
No biomarkers
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Amyotrophic lateral sclerosis
Onset of symptoms
Firstvisit
Onset NIV
Medicaldelay
Unclearfirst symptom
PrecociousNIV
PalliativeEnd-of-life
NIV
Studies are difficult to compare
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Amyotrophic lateral sclerosis
Onset of symptoms
Firstvisit
Onset NIV
Medicaldelay
Unclearfirst symptom
PalliativeEnd-of-life
NIV
PrecociousNIV
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Amyotrophic lateral sclerosis
Onset of symptoms
Firstvisit
Onset NIV
Medicaldelay
Unclearfirst symptom
PrecociousNIV
PalliativeEnd-of-life
NIV
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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
Includes assessment of swallowing, speech, and respiratory function, and both strength and function of upper and lower extremity musculature
Appel V. Ann Neurol. 1987;22:328-33.
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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 Swallowing4
3
21
0
4
3
21
0
Cedarbaum JM. J Neurol Sci 1999;169:13-21
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J Neurol Neurosurg Psychiatry 2004;75:1753-55
Scottish ALS Register
n=1226
Medical nihilism ?
Riluzole & PEG use increases
Patient autonomy
Less aggressive therapy
2.4 months
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ALS: Acute chest infection
Servera E. J Neurol Sci 2003;209:111-3
65 yrs old man
Daytime Mouth pieceNasal mask for nocturnal use
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Secretions management
MI-E
Ventilador+30 +TAT
Ventilador+TAT
Air stacking+TAT
TATSPON + m
ot
SPON
1100
1000
900
800
700
600
500
400
300
200
100
0
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٭
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†
††
†
‡
‡‡
• P
CF
l/m
n
Courtesy of Cristina Senent MD (H. Sant Joan - Alacant)& Jesus González MD (Hôpital Pitié-Salpetière - Paris)
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Bulbar impairment
BulbarNon-Bulbar
Bourke SC. Lancet Neurol 2006;5:140-7
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Survival in ALS patients with bulbar involvement
Farrero et al. Chest 2007;127:2132-8
NIV Tolerance
NIV intolerance
NIV Tolerance
NIV intolerance
Hypercapnia
Normocapnia
NIV Tolerance
NIV intolerance
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Agenda
Predictors of tolerance
NIV and survival
Survival and care organization
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Survival on HMVLaub M & Midgren B. Respir Med 2007;101:1074-8
n=1526
ALS
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Indication of NIV
Melo J. J Neurol Sci 1999;169:114-7
n=2357 patients
15% on NIV
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Lancet Neurol 2006;5:140-7
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Lancet Neurol 2006;5:140-7
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Lancet Neurol 2006;5:140-7
Time SAQLI symptoms domain maintained above 75% of prerandomization assessment
BulbarNon-Bulbar
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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”
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Chest 2007;132:62-9
Median survival time 37 months
Lenght of hospital stay55 days (7-124)
91% home discharge
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Agenda
Predictors of tolerance
NIV and survival
Survival and care organization
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Zoccolella S et al. J Neurol 2007;254:1107-12
No improvements in survival: Low rate of interventions?
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Survival of Irish ALS patients
One year mortality wasdecreased by 29.7%
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Survival of Irish ALS patients with bulbar onset
Prognosis of bulbar onset patients was extended by 9.6 months
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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 neurologistssaw all ALS patients
Living further from ALS clinicMore disabledIncreased ageBulbar onsetShorter duration of illness
Hutchinson M. J Neurol Neurosurg Psychiatry 2004;75:1208-12
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J Neurol Neurosurg Psychiatry 2006;77:948-50
Tertiary center
Neurology clinic
1080 days
775 days
The median survival from onsetwas 10 months longer
in ALS centers
4 yrs youngerPEG & NIV more oftenLess hospital admissions
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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
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www.has-sante.fr/
2003 17 Reference centers
CoodinationWorking groupsLocal organization
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Local organization
Visite de “retourn d’annonce”
Care planningSocial supportCall center
2-3 weeks after diagnostic
Hôpital Pitié SalpetièreParis
Multidisciplinary teamDay hospitalRespiratory unit
Median survival on HMV: 18 months
J. González-Bermejo, MD
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Escarrabill J. Arch Bronconeumol 2007;43:527-9
Patient-centered care: accessibility vs performance
Reference centers: benefits and limits
Improve skills & knowledge
DistanceUnnecessary referals
+
-
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Conclusions
Nihilism is bad
It seems that patients don’t like agressive treatments
Integrated care is a good option
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