RESPIRATORY PROBLEMS IN MND RICHARD HARRISON MND RESPIRATORY ASSESSMENT SERVICE LUNG HEALTH UNIVERSITY HOSPITAL OF NORTH TEES

RESPIRATORY PROBLEMS IN MND RICHARD HARRISON MND RESPIRATORY ASSESSMENT SERVICE LUNG HEALTH UNIVERSITY HOSPITAL OF NORTH TEES

A TYPICAL HISTORY: NON BULBAR ONSET Difficulty walking Weak hands and arms Muscle fasciculation Cramps Clumsiness and falls Spasticity BULBAR ONSET 30% Slurred speech Quiet voice Nasal voice Difficulty chewing Swallowing difficulty Regurgitation Choking Drooling Yawning Laryngeal spasms

RESPIRATORY PROBLEMS IN MND BREATHLESSNESS COUGH SPUTUM RETENTION SLEEP DISTURBANCE

BREATHLESSNESS PRE-EXISTING CARDIO-RESPIRATORY DISEASE INCREASED WORK OF BREATHING EXCESSIVE AIRWAY SECRETIONS RESPIRATORY MUSCLE INVOLVEMENT

THE RESPIRATORY MUSCLES INSPIRATORY MUSCLES VENTILATION EXPIRATORY MUSCLES SPUTUM CLEARANCE BULBAR MUSCLES AIRWAY PROTECTION CO-ORDINATION OF SWALLOWING AND BREATHING

DIAPHRAGM WEAKNESS DYSPNOEA ON EXERTION ORTHOPNOEA SUBMERSION DYSPNOEA NOCTURNAL HYPOVENTILATION PARADOXICAL RESPIRATION LYING / STANDING FVC MAX INSP PRESSURE SNIP

SLEEP-RELATED HYPOVENTILATION RESPIRATORY MUSCLE WEAKNESS INCREASED WORK OF BREATHING REM SLEEP ASSOCIATED EVENTS PANIC AND NIGHTMARES INCREASED RISK OF OSA SLEEP HISTORY AROUSALS AND FRAGMENTATION EXCESSIVE DAY TIME SLEEPINESS CARBON DIOXIDE RETENTION AM HEADACHES LETHARGY.

EARLY REM-ASSOCIATED HYPOVENTILATION

SLEEP-RELATED HYPOVENTILATION RESPIRATORY MUSCLE WEAKNESS INCREASED WORK OF BREATHING REM SLEEP ASSOCIATED EVENTS INCREASED RISK OF OSA AND LARYNGOSPASM AROUSALS AND FRAGMENTATION CARBON DIOXIDE RETENTION.

COUGH ASPIRATION INEFFECTIVE SPUTUM CLEARANCE PRE-EXISTING LUNG DISEASE SMOKING

COUGH three steps using the three muscle groups 1. A FULL BREATH - INSPIRATION 2. CLOSE THE VOCAL CORDS BULBAR 3. FORCED EXPIRATION EXPIRATORY MUSCLES DEEP INSPIRATION OF 2-4 L CLOSE GLOTTIS COUGH PEAK FLOW > 360 l/min NEED AT LEAST 160 l / MIN

SPUTUM RETENTION INEFFECTIVE COUGH SPUTUM POOLING HYPERSALIVATION

RED FLAGS DISTURBED BREATHING AND SLEEP RESTLESSNESS NIGHTMARES SWEATING CHOKING AND COUGHING EPISODES PANIC ATTACKS INABILITY TO LIE FLAT MORNING HEADACHES WEAK COUGH DAY TIME EXHAUSTION

RED FLAGS SPUTUM RETENTION CONSTANT BUBBLING WEAK COUGH CHOKING WITH DRINKS

HISTORY EXAMINATION RESPIRATORY RATE ACCESSORY MUSCLES PARADOX OXIMETRY LUNG VOLUMES COUGH PEAK FLOW SNIP, I/E PRESSURES OVERNIGHT OXIMETRY AM BLOOD GASES (pco2 > 6) TRANS-CUTANEOUS C02 ASSESSMENT

LEVELS OF INTERVENTION? VENTILATORY SUPPORT SYMPTOM CONTROL ADVANCED PHYSIOTHERAPY MANUALLY ASSISTED COUGH STACKING MI / E PALLIATIVE CARE

Survival Qol SAQLI Mod bulbar weakness Severe bulbar Mod bulbar Severe bulbar

HOW DOES NIV WORK RESETTING OF RESPIRATORY DRIVE REDUCTION OF BREATHING WORK IMPROVED MUSCLE STRENGTH REDUCTION OF HYPERINFLATION RELIEVES BREATHLESSNESS AND PANIC

HOW DOES NIV WORK (2) AMELIORATES NOCTURNAL HYPOVENTILATION STABILISES GAS EXCHANGE INCREASES CO2 RESPONSIVENESS DE-FRAGMENTS SLEEP IMPROVES DAYTIME SYMPTOMS PLACEBO EFFECT

RESPIRATORY MUSCLE WEAKNESS: THE CONTEXT IN WHICH NIV IS OFFERED LEVEL OF SUPPORT? SLT PHYSIO PALLIATIVE CARE DISEASE TRAJECTORY BULBAR FUNCTION ARM STRENGTH PSYCHO-SOCIAL ISSUES PREFERENCE

When to start NIV in MND (Simonds 2008) Nocturnal hypoventilation Daytime hypercapnia Orthopnoea MIPs <60% predicted Sleep fragmentation and daytime sleepiness Early use: VC < 80% (to aid physio)?

Why don't they accept noninvasive ventilation?: insight into the interpersonal perspectives of patients with motor neurone disease. Ando H 1, Williams C, Angus RM, Thornton EW, Chakrabarti B, Cousins R, Piggin LH, Young CA Br J Health Psychol. 2015 May;20(2):341-59 The threat to the self, the sense of loss of control, and negative views of NIV resulting from anxiety were more important to these patients than prolonging life in its current form

NIV: PRACTICAL PROBLEMS WHEN TO START NIV MASK ISSUES SPUTUM RETENTION MUCODYNE / HYPERTONIC SALINE AZITHROMYCIN COUGH ASSIST BREATH STACKING INFECTIONS HYPER-SALIVATION ATROPINE GLYCOPYRRONIUM BOTOX PANIC ATTACKS LORAZEPAM MORPHINE DISEASE PROGRESSION END OF LIFE ISSUES ADVANCED DIRECTIVES

CONCLUSIONS

MALE Age 71 MALE Age 70 MALE Age 73 MALE Age 65 MALE Age 59 BULBAR SYMPTOMS N Y Y Y Y ORTHOPNOEA Y paradox N Y Y paradox Y paradox SLEEP coma good orthopnoea sweating unrefreshed headaches sfvc lfvc 2.15 2.0 1.7 1.45 2.63 1.43 1.97 0.82 0.57 0.57 MIP 44 19 45 21 unable MEP 55 46 53 31 SNIP 16 44 29 42 21 12 NIV yes no yes no yes (one year) yes

MOTOR NEURONE DISEASE THE EVIDENCE FOR NIV THE IMPORTANCE OF BULBAR SYMPTOMS WHEN TO START? WHEN TO STOP?

Survival times with NIV therapy in patients without bulbar involvement (solid line) and with bulbar involvement (dotted line) on the initiation of treatment. Farrero E. et.al. Chest 2005;127:2132-2138 2005 by American College of Chest Physicians

SYMPTOMS OF RESPIRATORY MUSCLE WEAKNESS BREATHLESSNESS CHOKING IMPAIRED COUGH PANIC ATTACKS DISTURBED SLEEP MORNING HEADACHES DAY TIME FATIGUE

NON-INVASIVE VENTILATION GOOD EVIDENCE BASE SYMPTOMATIC IMPROVEMENT PROLONGS LIFE IN SOME PATIENTS REJECTED BY SOME REQUIRES A SUPPORT NETWORK NOCTURNAL > 24 HOUR WHAT NEXT? QUALITY OF LIFE

NON-INVASIVE VENTILATION GOOD EVIDENCE BASE SYMPTOMATIC IMPROVEMENT PROLONGS LIFE IN SOME PATIENTS REJECTED BY SOME REQUIRES A SUPPORT NETWORK NOCTURNAL > 24 HOUR WHAT NEXT? SURVIVAL

AIRWAY CLEARANCE TECHNIQUES SIMPLE PHYSIOTHERAPY ADVICE BREATH STACKING COUGH ASSIST

NIV: PRACTICAL PROBLEMS WHEN TO START NIV MASK ISSUES SPUTUM RETENTION MUCODYNE / SALINE COUGH ASSIST BREATH STACKING INFECTIONS HYPER-SALIVATION ATROPINE BOTOX PANIC ATTACKS LORAZEPAM MORPHINE DISEASE PROGRESSION END OF LIFE ISSUES ADVANCED DIRECTIVES

NIV OR TRACHEOSTOMY? NIV SIMPLER CARE CHEAPER MORE COMFORTABLE? SPEECH SWALLOWING AND APPEARANCE TRACHEOSTOMY ACCESS TO SECRETIONS BETTER FOR 24 HOUR VENTILATION BETTER SURVIVAL MAJOR CARER BURDEN Resp Med 2008 102 430

THE VITAL CAPACITY FALLS PROGRESSIVELY A VITAL CAPACITY OF < 680 ml PREDICTS DAY TIME HYPERCAPNIA