Focus sulla Sclerosi Laterale Amiotrofica

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1 Caserta maggio 2012 Focus sulla Sclerosi Laterale Amiotrofica Valutazione del deficit restrittivo neuromuscolare Paolo Banfi

2 Practice Guidelines Clinical practice guidelines are explicit descriptions of how patients should be evaluated and treated. The explicit purpose of guidelines is to improve the quality of care and to assure it by reducing variation in care provided. - review of evidence - consensus of experts

3 Practice Guidelines for ALS (outline) breaking the news to patients and families, symptom management (swallowing difficulties, sialorrhoea and bronchial secretions), nutrition and PEG placements, respiratory insufficiency and mechanical noninvasive or invasive ventilation, management of ineffective cough, decisions around withdrawal of life support treatment and palliative care.

4 Respiratory insufficiency is a major cause of complaints and the major cause of early death in ALS Mean survival time from diagnosis of respiratory insufficiency in ALS is months without ventilatory support With NIV: Additional months? With Trach?: 7-10 y?

5 Respiratory problems in neuromuscular patients Muscular function during INhalation Muscular function during EXhalation Upper airways, bulbar function Ability to cough Hypoventilation Infection Atelectasis Secretion stagnation




9 Supine FVC is possibly more effective than erect FVC in detecting diaphragm weakness and correlates better with symptoms of nocturnal hypoventilation (two Class III studies). Sniff transdiaphragmatic pressure (Pdi) and sniff nasal pressure (SNP) are possibly effective in detecting hypercapnia and nocturnal hypoxemia (two Class III studies). Analysis of Evidence What are the optimal pulmonary tests to detect Respiratory Insufficiency? Nocturnal oximetry and maximal inspiratory pressure (MIP) are possibly more effective in detecting early respiratory insufficiency than erect FVC (two Class III studies).


11 The median survival of ALS patients with baseline FVC <75% was 2.91 years, compared with 4.08 years for patients with a baseline FVC >75% (p<0.001).




15 Test di performance dei muscoli respiratori Contrattilità Endurance Dipendenti dalla volontà: - MIP, MEP - Pdi max Dipendenti dalla volontà: - respirazione contro resistenza - massima ventilazione sostenibile (MVS) - massima ventilazione volontaria (MVV) Indipendenti dalla volontà: - Pdi twich

16 Stages of Respiratory Muscle Dysfunction * Stage VC Symptoms PCO 2, mm Hg SpO 2 PCF, L/s MIC, ml 1 > 50% None < 45 > 95% < 6 > % Dyspnea > 45 < 95% > 3 > < 10% VFBT > 5 min > 3 > < 10% VFBT < 5 min < 3 < 500 PCO 2 = maximum nocturnal end-tidal pco 2 SpO 2 = mean nocturnal SpO 2 VFBT = ventilator-free breathing time. (Bach, Chest. 2002)



19 Amyotroph Lateral Scler Jul 11. Association between low sniff nasal-inspiratory pressure (SNIP) and sleep disordered breathing in amyotrophic lateral sclerosis: Preliminary results. Carrat P, Cassano A, Gadaleta F, Tedone M, Dongiovanni S, Fanfulla F, Resta O. In conclusion, the results of this preliminary study show that SNIP < 60 cmh(2)o might be considered an early predictor of SDB in ALS.


21 85 to 90% of total lung capacity Glottic closure follows for about 0.2 s and sufficient intrathoracic pressures are generated to obtain peak transient expiratory flows PCFs 360 to 1000L/mi Inspiratory Muscle Weakness Expiratory Muscle Weakness Bulbar Muscle Weakness

22 Analysis of Evidence What is the efficacy of targeted respiratory interventions for clearing secretions? Mechanical insufflation/exsufflation (MI-E) is possibly effective for clearing upper airway secretions in patients with ALS who have reduced peak cough flow, although the clinically meaningful difference is unknown (four Class III studies). High frequency chest wall oscillation (HFCWO) is unproven for adjunctive airway secretion management (two Class III studies with conflicting results).

23 Recommendations MI-E may be considered to clear secretions in patients with ALS who have reduced peak cough flow, particularly during an acute chest infection (Level C). There are insufficient data to support or refute HFCWO for clearing airway secretions in patients with ALS (Level U).

24 Symptomatic treatment Bronchial secretions 1. Mucolytics using if sufficient cough 2. Anticholinergic broncodilator and/or furosemide and/or Betareceptor antagonist and/or nebulized clonidine (personal experience) 3. In ALS patient (phenotype spinal) using air stacking and abdominal thrust 4. Cough-Assist

25 23/06/09

26 The most commonly used mechanical insufflator exsufflators today are a) the Cough Assist (JH Emerson Co., Cambridge, MA, USA) and b) the Pegaso (Dima Italia, Bologna, Italy). Circuit


28 SI NO

29 Efficacy of Mechanical Insufflation-Exsufflation in Medically Stable Patients with Amyotrophic Lateral Sclerosis (Sancho J, Chest 2004;125: ) The upper airway CT scans of the three patients showed exsufflation cycle closing of the nasopharynx, with retraction of the uvula, and reduction of the lateral diameter of the pharynx. This narrowing was greatest at the oropharynx. In the patient with PCFMI-E < 2.7 L/s, the maximum reduction of the diameter of the pharynx was 77%; in the patient with bulbar dysfunction and PCFMI-E > 2.7 L/s, it was 60%; and in the patient without bulbar impairment and PCFMI-E > 2.7 L/s, it was 45%.

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31 Symptomatic treatment Sialorrhoea 1. Amytriptiline: there are no formal studies proving its efficacy 2. Atropine drops, 0.5% or 1% 3 or 4 times/day 3. Glycopyrrolate (not in Italy) only experienced in Parkinson s disease not in ALS 4. Transdermal Scopolamine 1.5 mg every 72 H (side effects) 5. Injection with Botulinum toxin (type A or B) into the salivary glands 6. External irradiation of the salivary glands

32 FVC in ALS FVC<80%of predicted and FEV1<80% of predicted with a normal FEV1/FVC ratio indicate the presence of restrictive lung disease. The average decline of VC in ALS is 2.3% per month and follows a linear course (Schiffman & Belsh, 1993). Median survival of patients with ALS with baseline FVC< 75% at diagnosis has been shown to be 2.91 yrs, compared with 4.08 yrs with baseline > 75% (Czaplinski et al., 2006) Because FVC has been shown to predict survival and disease progression in ALS, it is useful as an inclusion or exclusion criteria for clinical trials (Brinkman et al., 1977; Czaplinski et al., 2006) Postoperative risk with PEG increases when FVC declines below 50% of predicted (Kasarskis et al., 1999)

33 Normal VC is 60 to 70 ml/kg of ideal body weight. Patients whose VC drops 30 ml/kg will experience decreased expiratory flow rates, weak cough, impaired secretion clearance, and risk of atelectasis (Bella & Chad, 1998; Mehta, 2006) If a patient's VC falls to 15 ml/kg or 1 L, he or she is considered to be in acute respiratory failure. Continuous ventilation, invasively or noninvasively, is usually required at this point (Mehta, 2006) FVC of < 30% of predicted is used as an admission criterion for hospice care.

34 Dalla parte del Pneumologo 1. Visita Pneumologica Generale 2. Esecuzione di esami strumentali 1. Spirometria (clino ed ortostatismo) 2. SVC (seduto) 3. Emogasanalisi arteriosa 4. Misurazione MIP e MEP (seduto) e SNIP 5. Spirometria durante tosse 6. Saturimetria notturna 3. Accurata anamnesi per identificare possibili sintomi legati ad una alterata qualità del sonno o alla presenza di alterazioni respiratorie in sonno 4. Valutazione del grado di disabilità generale e respiratoria e del coinvolgimento del caregiver mediante autosomministrazione di apposite scale e/o questionari per evidenziare 1. Capacità di mobilità e di spostamento 2. Autonomia e controllo degli sfinteri 3. Comprensione e comunicazione 4. Dispnea 5. Efficacia della tosse 6. Alterazioni della deglutizione 7. Presenza o meno di pseudoscialorrea 8. Presenza di altri sintomi non respiratori 9. Residenza e tipologia di nucleo convivente

35 Follow-up 1. Visita Pneumologica Generale (raccordo anamnestico) 2. Esecuzione esami strumentali: 1. Spirometria forzata (clino e ortostatismo) 2. Emogasanalisi arteriosa 3. Spirometria alla tosse 4. MIP e MEP 5. Saturimetria notturna (Poligrafia notturna) 3. Rivalutazione del grado di disabilità generale raggiunta Nei pazienti adattati alla NIV occorre una verifica del tempo di utilizzo, delle eventuali criticità insorte, un controllo dei materiali e forniture ed un eventuale rinforzo educazionale

36 Laura che stira