Respiratory Care in Neuromuscular Diseases

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1 Sestri Levante, 19 ottobre 2012 Respiratory Care in Neuromuscular Diseases Respiratory Care in ALS Paolo Banfi MD Pulmonologist Department of Neuromuscular Disease Fondazione Don Gnocchi Milan - Italy

2 Charcot

3 PHYSIOLOGY IN ALS MUSCULAR WEAKNESS Bulbar muscles Expiratory muscles Inspiratory muscles Obstructive apnea Bronchoaspiration Ineffective cough Alveolar Hypoventilation Microatelectasis EARLY DETECTION

4 ALS: different phenotypes for different approach 1.Classic (Charcot s) phenotype 2.Bulbar phenotype 3. Flail arm phenotype 4.Flail leg phenotype 5.Pyramidal phenotype (predominant upper motor neuron ALS) 6.Respiratory phenotype 7.Pure lower motor neuron 8. Pure upper motor neuron

5 Flail arm phenotype

6 Flail leg phenotype


8 funzionalità respiratoria % Tempo

9 DIAGNOSIS Symptoms and signs Assessment of respiratory muscles weakness Evaluation of nocturnal hypoventilation Assessment of diurnal hypoventilation NON INVASIVE SIMPLE ACCESIBLE REPEATABLE

10 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)

11 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.

12 Riduzione della FVC nella SLA NIV NIV H 24


14 Nocturnal SaO2 and TcCO2 in control and NIV groups Mean nocturnal SaO2 % Time TcCO2 > 6.5 kpa 5 20 SaO2 % Percent time TcCO2 > 6.5 kpa: p=0.005 p= Group 1 Control Group 2 NIV Months Group 1 Control Group 2 NIV p=0.031 p=0.049 Months Significant reduction in time TcCO2 > 6.5 kpa and increase in mean SaO2 in NIV group

15 NIV criteria: Symptoms of sleep-disordered breathing such as morning headache, daytime fatigue or dyspnea, and 1 of the following: (1) PaCO2 level of >45 mm Hg; (2) nocturnal In the oximetry absence of demonstrating hypercapnoea, oxygen saturation at 88% for measurement 5 consecutive of HCO3 minutes; may be very usefull and a value >30 mmol/l is a criterion of hypoventilation. (3) maximal inspiratory pressure of <60 cm H2O; or (4) forced vital capacity of <50% (Lyall predicted. RA, Brain 2001)

16 Mechanical ventilation for amyotrophic lateral sclerosis/motor neuron disease (Review) Radunovic A, Annane D, Jewitt K, Mustfa N Analysis of Evidence Does NIV improve respiratory function or increase survival? Conclusions: NIV is probably effective in prolonging survival (one Class I, three Class III studies). NIV is probably effective in slowing the rate of FVC decline (one Class I, one Class III study). Recommendation: NIV should be considered to treat respiratory insufficiency in ALS, both to lengthen survival and to slow the rate of FVC decline (Level B).

17 Mechanical ventilation for amyotrophic lateral sclerosis/motor neuron disease (Review) Radunovic A, Annane D, Jewitt K, Mustfa N Analysis of Evidence How do invasive ventilation and NIV affect QOL? Conclusions: NIV is possibly effective in raising QOL for patients with ALS who have respiratory insufficiency (five Class III studies). Tracheostomy invasive ventilation (TIV) is possibly effective in preserving QOL for patients with ALS, but possibly with a greater burden for their caregivers (two Class III studies). Recommendations: NIV may be considered to enhance QOL in patients with ALS who have respiratory insufficiency (Level C). TIV may be considered to preserve QOL in patients with ALS who want long-term ventilatory support. (Level C).

18 Effect of NIV on sleep quality Mellies U et al ERJ 2003;22:631-4



21 Non invasive ventilation and ALS 25 Moderate to substantial improvement of QoL and survival.. in spite of disease progression..indicating that treatment did NOT prolong suffering ALS-FRS 10 Pre NIV Bourke, S.C., et al., Noninvasive ventilation in ALS: indications and effect on quality of life. Neurology, (2): p


23 Conclusioni: NIV può esser usata come alternativa alla TPPV. I pazienti con adeguata funzione della muscolatura bulbare possono utilizzare la MAC come prevenzione di distress respiratorio. about 20% of ALS patients can use NPPV up to a continous level to prolong survival for an additional 14 to 17 months, and in some cases for > 7 years..


25 Mechanical ventilation for amyotrophic lateral sclerosis/motor neuron disease (Review) Radunovic A, Annane D, Jewitt K, Mustfa N Analysis of Evidence What factors influence acceptance of invasive ventilation and NIV? Conclusions: Nocturnal oximetry is possibly effective in detecting early respiratory insufficiency and the early use of NIV possibly increases compliance (two Class III studies). Bulbar involvement and executive dysfunction possibly lower compliance with NIV (two Class III studies). Recommendation: NIV may be considered at the earliest sign of nocturnal hypoventilation or respiratory insufficiency in order to improve compliance with NIV in patients with ALS (Level C).

26 Non-invasive mechanical ventilation (NIMV) in patients with amyotrophic lateral sclerosis (ALS): our experience. Banfi P 1 *, Roma E 1, Robbiani A 2, Malnis D 2, Aliprandi G 3, Tonni M 4 and Silani V 5. 1 Pulmonary Division-A.O.Mellini-Chiari,Italy; 2Home Care Dept.-ASL Provincia di Milano-Magenta,Italy;3Lung Function Unit-Ancelle delle Carità, Domus Salutis-Brescia,Italy; 4Univ.Brescia Physiotherapy school,brescia,italy; 5Dept. Neurology, Neuroscience -Univ. Milan Med. School, IRCCS Ist. Auxologico, Milan, Italy.

27 BASELINE 6 MONTHS 12 MONTHS 18 MONTHS 24 MONTHS 30 MONTHS 36 MONTHS Survival FVC% 82 77,8 70,9 61,8 54, ,8 po2 s.r pco2 s.r po2 vent pco2 vent SpO2 mean T90% Pat. In NIMV NIMV h/day 1,5 6,8 11,2 12,6 15,2 22,8 We have not ABG at 30 and 36 months in supine spontaneous breathing because all the patients was in NIV all time. Four patients had an acute respiratory distress between 12th and 18th month, 5 patients between 24th and 30th was tracheotomized and 4 death, between 30th and 36th again 2 tracheotomized and 9 death: they wasn t considered in the statistical evaluation.


29 H 24 NIV

30 Daylight NIV Ventilated few minutes Non ventilated few minutes

31 INVASIVE VENTILATION Improves survival Easier management of secretions Effectiveness independent of bulbar dysfunction Quality of life. (totally locked-in state, TLS) Greatly increased costs Increased family and carer burden Ethical issues

32 Performing the tracheostomy Prefer an elective surgical procedure rather than the percutaneous technique in order to: Minimize the risk of malposition (lateral stoma) Remove a piece of the tracheal wall Manage a "good" skin hole The objective is to facilitate recannulation


34 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/min Inspiratory Muscle Weakness Expiratory Muscle Weakness Bulbar Muscle Weakness


36 For patients with progressive neuromuscular diseases, ARF most often occurs during otherwise benign upper respiratory tract infections (URTIs). Bach JR. Conventional approaches to managing neuromuscular ventilatory failure. In: Bach JR, ed. Pulmonary Rehabilitation: the obstructive and paralytic conditions. Philadelphia: Hanly & Belfus, 1996; During these episodes, already severe pulmonary dysfunction is further compromised by bronchial mucus plugging and by further weakening and fatigue of inspiratory and expiratory muscles Mier-Jedrzejowicz A, Brophy C, Green M. Respiratory muscle weakness during upper respiratory tract infections. Am Rev Respir Dis 1988; 138:5-7

37 Causes of Acute Respiratory Failure in Neuromuscular Patients Admitted to Hospital Common Acute bronchitis/upper respiratory tracts infections Pneumonia Atelectasis Heart failure

38 CPF cough peak flows collected using a peak flow meter CPF normal values range from 400 to 1200 liters/min for adults Soudon P, Steens M, Toussaint M: Desobstruction tracheobronchique chez les patients restrictifs majeurs paralises. Respir Care 1999;3:1 25

39 Mechanical ventilation for amyotrophic lateral sclerosis/motor neuron disease (Review) Radunovic A, Annane D, Jewitt K, Mustfa N Analysis of Evidence Conclusions: 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).

40 Mechanical ventilation for amyotrophic lateral sclerosis/motor neuron disease (Review) Radunovic A, Annane D, Jewitt K, Mustfa N Clinical Context

41 The most commonly used mechanical insufflator exsufflators


43 NO

44 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%.

45 Oropharynx CT scan of a bulbar ALS patient with PCFMI-E < 2.7 L/s. Left, A: baseline. Right, B: during the exsufflation cycle Oropharynx CT scan of a nonbulbar ALS patient with PCFMI-E > 2.7 L/s. Left, A: baseline. Right, B: during the exsufflation cycle.


47 Neurologo Pneumologo Palliativista Fisiatra Fisioterapista Associazioni Infermiere professionale Nutrizionista 118 Tecnico di ergonomia Psicologo Logopedista Foniatra simultaneous care : approccio olistico alla SLA

48 Caratteristiche dei Programmi di Assistenza Domiciliare Multidisciplinarità Alto livello di preparazione Medico/Infermieristica Supervisione di infermieri dedicati Continuità assistenziale Supervisione di un medico specialista Adeguata copertura finanziaria Buona interazione con le Società di Servizio Ottimo link tra Ospedale-Territorio Buona interazione tra MMG e Operatori domiciliari Adeguata presa in carico degli aspetti psicosociali Presa in carico delle problematiche di fine vita ma ora c è la spending review!

49 Consulta Ministeriale sulle Malattie NeuroMuscolari (D.M ) PERCORSO ASSISTENZIALE OSPEDALE-TERRITORIO Assistenza domiciliare multidisciplinare Individuazione di progetti finalizzati a realizzare o potenziare percorsi assistenziali domiciliari che consentano una presa in carico globale della persona affetta da SLA e dei suoi familiari Definizione del Piano Assistenziale Individuale (PAI) da parte dei CdR in relazione all analisi del complesso delle funzionalità e dei bisogni assistenziali da trasmettere all ASL di appartenenza e al MMG del paziente. Precoce attivazione, in accordo con la persona affetta da SLA o di suo familiare, l Unità di Valutazione Multidimensionale (UVM) del territorio/asl e MMG della Persona affetta da SLA per una corretta e tempestiva presa in carico territoriale.

50 Assistenza domiciliare L UVM deve promuovere l integrazione tra ospedale, territorio, servizi e figure professionali e promuovere risposte più efficaci per i bisogni dei pazienti. Lo staff delle UVM è costituito dalle seguenti figure professionali: il Direttore Sanitario o un suo delegato; il case manager; un Dirigente medico dell Area intensiva e/o respiratoria; un Dirigente medico dell Area distrettuale/o cure primarie, intermedie, della protesica ed assistenza domiciliare; un Assistente sociale; un rappresentante dell Area amministrativa.

51 Assistenza domiciliare Compiti specifici dell Unità di Valutazione Multidimensionale: la formulazione del PAI anche su indicazione dei CdR; la facilitazione dei percorsi di accesso ai diversi punti della rete assistenziale di volta in volta coinvolti e a presidi, protesi ed ausili: per quest ultimo aspetto si ritiene utile la creazione di Portali on line che possano mettere in diretta relazione CdR, UVM, ASL (ufficio protesi); l interfaccia più semplice, agile e meno burocratica possibile tra la persona con SLA e i suoi familiari e gli uffici della Pubblica Amministrazione; il raccordo tra CdR e Medico di Medicina Generale.

52 Assistenza domiciliare Il PAI sarà costituito da un documento scritto e prevede i seguenti elementi: dati identificativi del Paziente esito della valutazione multidimensionale con esplicitazione dei bisogni assistenziali definizione degli obiettivi di assistenza e delle azioni da intraprendere cronoprogramma degli interventi elenco dei servizi attivati e attribuzione delle responsabilità per gli specifici segmenti assistenziali indicatori per il monitoraggio e la valutazione dell assistenza.

53 Assistenza domiciliare Interventi al domicilio sulla persona con SLA, la ASL deve essere in grado di assicurare le seguenti figure professionali: MMG; neurologo; anestesista/pneumologo; nutrizionista; fisiatra; gastroenterologo; logopedista; fisioterapista motorio e respiratorio; terapista occupazionale; psicologo.

54 Assistenza domiciliare Collaborazione con il CdR al quale sono affidati le ospedalizzazioni per la gestione della fasi critiche: 1) adattamento alla ventilazione meccanica non invasiva 2) predisposizione di PEG o RIG, adattamento alla NE totale, ed eventuale adattamento a minima alimentazione idonea per os 3) esecuzione di tracheostomia e adattamento a ventilazione meccanica per via tracheostomica 4) eventuali problemi acuti non gestibili a domicilio (polmonite con instabilità degli scambi gassosi; sepsi severa; cardiopatia ischemica; problemi della PEG non gestibili a domicilio) 5) eventuali accessi in DH per: verifica dell adattamento a ventilazione meccanica; sostituzioni di sondino PEG o cannula tracheostomica; esami diagnostici complessi.

55 Comunicazione di rete nell ambito del percorso assistenziale della persona affetta da SLA CdR II livello PRI CdR I livello PAI ASL ADI UVM MMG

56 Quali modelli di cure?

57 Le criticità nell assistenza al malato SLA Comunicazione ancora oggi inadeguata della diagnosi Assenza di presa in cura globale Assenza di sostegno psicologico al nucleo familiare Assenza di corsie preferenziali Assenza di assistenza pre tracheostomia Infermieri e operatori non qualificati Assenza di cure domiciliari qualificate Assenza di controllo sulla qualità dell assistenza domiciliare Difficile gestione del malato in ospedale Scarsa presenza dei medici di base Fonte AISLA

58 LIVELLI DI ASSISTENZA DEI PAZIENTI AFFETTI DA MALATTIA NEUROMUSCOLARE (Dott. M. Corbo, Dott. P. Banfi, Dott. C. Lunetta, Dott. M. Melazzini) LIVELLO 1:Paziente con deficit stenico modesto (al bilancio muscolare valore medio di MRC 4) e/o PEG LIVELLO 2:Paziente con deficit stenico intermedio (al bilancio muscolare valore medio di MRC 3 e/o PEG e/o NIV per un periodo < 12 ore (prevalentemente notturna) LIVELLO 3:Paziente con tetraparesi (al bilancio muscolare valore medio di MRC compreso tra 2 e 3) e/o PEG e/o NIV per un periodo compreso tra ore die LIVELLO 4:Paziente con tetraplegia (al bilancio muscolare valore medio di MRC compreso tra 0 e 1) e/o PEG e/o NIV per un periodo > 16 ore die o ventilazione assistita in tracheostomia Legenda: MRC: Medical Research Council. Aids to the examination of the peripheral nervous system, Memorandum no. 45, Her Majesty's Stationery Office, London, 1981


60 Laura che stira (Duccio Guidotti)