Diagnosis

• MDT: Neurologist, Respiratory Physician, MND specialist, Respiratory Phyiologist & Physiotherapist. Palliative Care specialist and SALT
• Monitor for symptoms of respiratory impairment:
  • Breathlessness
  • Orthopnoea
  • Recurrent chest infections
  • Disturbed sleep
  • Non-refreshing sleep
  • Nightmares
  • Daytime sleepiness
  • Poor concentration and/or
  • Memory
  • Confusion
  • Hallucinations
  • Morning headaches
  • Fatigue
  • Poor appetite
• Signs:
  • Increased respiratory rate
  • Shallow breathing
  • Weak cough[ (PEFR)
  • Weak sniff
  • Abdominal paradox (inward movement of the abdomen during inspiration)
  • Use of accessory muscles of respiration
  • Reduced chest expansion on maximal inspiration

Investigation

• Oxygen saturation
• Forced Vital Capacity. If < 50 % predicted discuss treatment, specialist referral and impact
• Sniff nasal inspiratory pressure. If < 40 cmH2O discuss treatment, specialist referral and impact
• If bulbar palsy then measure O2 sats, but not other function tests if interfaces not available
• Perform ABG is Sats < 94 %, with no lung disease, < 92% with lung disease
• If sleep related problems or Orthopnoea, but Sats > above limits then refer to specialist
• If PaCO2 > 6 kpa refer to specialist to be seen within 1 week

Management

• Offer advice on Non-invasive ventilation at appropriate time, sensitively but soon after diagnosis, and assess carer support and training needs
• Offer NIV is symptoms and signs likely to benefit, or trial only if likely to benefit first from improvement in sleep related symptoms
• Before starting NIV, MDT should consider:
  • Tolerance
  • Risk of ventilator failure
  • How easily may get to hospital
  • Need for humidifier
• Before commencing NIV, MDT to develop care plan
• Planning end of life care: Discuss at commencement or decline of NIV; becoming more dependent on NIV, or when they ask

NICE Source: CG105 Motor neurone disease: non-invasive ventilation. Summary compiled by Dr D P Sheppard MBBS.