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Neuromuscular disorders: Respiratory assessment and ventilatory management.
In patients with Amiotrophic Lateral Sclerosis
Joan Escarrabill MDMaster Plan of Respiratory Diseases (PDMAR)
Institut d’Estudis de la Salut
Barcelona
Stressa, April 4th 2009
2
ALS
Degeneration and death of motorneurons• Upper Motor Neuron
– within brain/spinal cord
• Lower Motor Neurons – leaves brain (stem)/spinal cord.
Incidence 1-2.5 / 100,000.
The life span of ALS patients is 3 to 5 years after they are diagnose.
3
ALS: Prognosis
Difficult to predict in an individual patient
3-4 yrs 50%
> 5 yrs 20%
> 10 yrs 10%
> 20 yrs Occasional
Life expectancy
4
ALS: 3 key words
Precocious Team
Package
5
ALS: 3 key words
Precocious Team
Package
6
Clinical presentation
Asymmetric Weakness (most common)
Onset single limb or bulbar
Local spread then regional spread Bulbar, cervical, thoracic, lumbosacral
Fasciculations
Acute respiratory failure
7
ALSClinical Signs and Symptoms
Clumsy hand Hoarse voice (dysarthria) Shoulder dysfunction Weak foot (foot drop) Difficulty walking (spastic gait) Exercise intolerance Fasciculation Respiratory insufficiency Cognitive impairment / emotional lability
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ALS: Natural History
End of life
Diagnosis
Symptoms
Non Invasive Ventilation
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ALS: Natural History
End of life
Diagnosis
Symptoms
Early indication of NIV
Natural History
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ALS: Natural History
End of life
Diagnosis
Symptoms
Natural History
Palliative NIV
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ALS: Natural History
End of life
Diagnosis
Symptoms
Natural History
Palliative NIV
Early indication of NIV
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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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Muscle strength vs Vital capacity
M. Estenne, 1991
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Lung function
VC (lying, and sitting/standing)
20%
Sniff-Nasal Inspiratory force
Nocturnal pulsioxymetry
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Eur Respir J 2008; 31: 93–98
Many patients with neuromuscular disease find the PImax manoeuvre difficult to perform
-70 cmH2O in males or ,-60 cmH2O in females is unlikely to be associated with inspiratory muscle weakness
Sniff-Nasal Inspiratory force (SNIP)
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Am J Respir Crit Care Med 2005;171:269–274
Kaplan-Meier survival curves based on the SNIF categories
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Lung & bulbar function:Signs and symptoms
Weight loss > 10%
Dysarthria
Dysphagia
Syalorrhea
Aspiration
Intolerance to supine position
Ineffective coughLung function
Bulbar function
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ALS: 3 key words
Precocious Team
Package
19
Effective team It has a range of individuals who contribute in different
ways. Clear goals. Everyone understands the tasks they have to do. Coordinator There is a supportive, informal atmosphere. Comfortable with disagreement. A lot of discussion (Group members listen to each other) Feel free to criticise Learns from experience.
www.kent.ac.uk/careers/sk/teamwork.htm
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The team produces more than the individual contributions of members.
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Patient care team
Wagner. BMJ 2000;320:569-72.
R. Casas & P Romeu (1897)
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Aiken L. NEJM 2003;348:164-6
Holistic vision
Better care related to coordination
Increasing role of non-physcian health professionals.
23
Mitsumoto H & Rabkin JG. JAMA. 2007;298:207-216
Care in multidisciplinary clinics is
associated with enhanced quality
of life by alleviating symptoms
and may extend survival
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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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www.has-sante.fr/
2003 17 Reference centers
CoodinationWorking groupsLocal organization
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59%
41%
Ile de France Non ILD
Evalutaion of ALS reference centers
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Survival of Irish ALS patients
One year mortality wasdecreased by 29.7%
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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Effect of referral bias
Sorenson EJ et al. Neurology 2007;68:600-602
132 subjectsTertiary center3 years.
Survival
p = 0.007
referral population
local
population
29 months 18 months
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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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Home care organized through reference centers has many limitations
Complex organization, Distance, Response to emergencies
In most cases the reference center coordinates care but it can not assume direct care
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Bias in the care of patients
Accessibility
Low rate of interventions
Distance
Referral Bias
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ALS: Multidisciplinary Approach to Care
Neurologist Clinical/research nurse Dietician Speech/swallowing therapist Family/caregivers Psychologists General Practitioner Community nurse
Respiratory therapist Occupational therapist Social worker GI physician Support organizations Homehealth/hospice Pulmonologist Volunteer Helpers
Adapted from Leigh PN. J Neurol Neurosurg Psychiatry 2003;74(Suppl IV):iv32–iv47
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Conclusions:
3 key words
Precocious Team
Package
35
ALS: Management
weakness fatigue nutrition dysphagia feeding tube dysarthria communication
spasticity cramps pain depression anxiety breathing end-of -life
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Lancet Neurol 2006;5:140-7
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Are NIV trials necessaries in ALS with non-bulbar impairement?
Servera E. Sancho S. Lancet Neurol 2006;5:140-7
Non-bulbar patients in control group
Stop studies according the results
Assessment efficacy of NIV
Pressure vs volume ventilators
Secretion management
It’s mandatory to evaluate therapy “package”
Ethical issues
Technical issues
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“Therapy package” in ALS
Mobility Swallowing &
Speaking
Coping with changes
Breathingchanges
Symptoms
Caregiver
Adapted from www.alsa.org/
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Coping with changes = Accessibility to the team Symptoms control Non invasive ventilation (tracheo?) Mechanical assisted cough Daily living activities aids Communication devices Percutaneous endoscopic gastrostomy Caregiver support End-of-life issues
“Therapy package” in ALS
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Chest 2000;118:1390-6
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Chest 2002;122:92-98
NIV Mechanical assisted cough Oximetry as feedback
Delay the need for tracheotomy
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Chest 2004;125:1400-5
PCFMIC > 4 L/sMI-E not generate greater PCF than manually assisted coughing
PCFMIC < 2.7 L/s Dynamic collapse of the upper airway during the exsufflation
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Lung function
Sancho J. Chest 2004;125:1400-5
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Caregivers’ perception of the impact of ALS on the patients, The duration of ALS, The coping strategies employed, Satisfaction with the support services available
The QoL of caregivers is a complex interaction between:
Neurology 2006;66:1211–1217
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www.patientslikeme.com/
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Sweden 1965-2004
6642 patients
40 years
3 years
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Mitsumoto H & Rabkin JG. JAMA. 2007;298:207-216
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ALS: 3 key words
Precocious Team
Package
Especially in regard to lung function
Respiratory & Non-respiratory
Since the beginning of the disease
