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Respiratory

Respiratory weakness can develop at any stage of disease progression and may cause dyspnoea (shortness of breath), fatigue, impaired quality of life and somnolence.

Respiratory weakness is best addressed by:

  • referral to a specialist respiratory physician should take place early in the disease course and regular assessment initiated
  • management of symptoms, which can affect how other symptoms of MND are managed.

The diagnosis and management of respiratory weakness is critical because most deaths from MND are due to respiratory failure (Berlowitz et al. 2016).

The person living with MND, together with their family, should be guided through a decision-making process related to management of respiratory symptoms and their future care. 

Practice points

  • Domiciliary NIV therapy has been an important advance for patients with MND.
  • Cough strength may be impaired in MND; a suitable cough augmentation technique can help such patients.
  • Clinicians should pay careful attention to nutritional support in patients with MND.
  • Pharmacological management of symptoms may include anticholinergics for excess saliva and mucolytics, for example, carbocisteine, to reduce secretion viscosity.
  • Tracheostomy ventilation is an option for patients who wish to live but who cannot use NIV.
  • Palliative care needs, end-of-life issues and stopping respiratory support should be discussed regularly with the patient and the family.


MND Australia 2017

Clinical features 

  • early in the course of the disease nocturnal hypoventilation may not be manifest by obvious shortness of breath but more by headaches or general tiredness
  • later, more marked shortness of breath may appear but this tends to be at the time when the person living with MND has severe generalised weakness which may affect the throat and limb muscles - the symptoms at that time may include:
    • breathlessness on exertion, sometimes just the exertion of speaking or eating
    • sleep disturbance, anxiety and panic
    • orthopnoea - breathless lying flat
    • quiet voice and fewer words per breath
    • weakened cough and sneeze
    • hypoventilation is worse during sleep. Upper airway may also be partially obstructed due to bulbar and laryngeal muscle weakness
    • increasing blood C02 levels result in headaches, nausea and tiredness, especially on waking
    • hypoxia, especially if there is coexisting lung disease

Management

Regular respiratory assessments, including sleep studies, by a respiratory physician are indicated in most persons living with MND.
  • these should be initiated at a relatively early stage and their timing depends upon the level of abnormality detected
  • where nocturnal hyperventilation is detected it may be appropriate for the respiratory physician to initiate and prescribe non-invasive positive pressure ventilation (NIPPV)


Respiratory management algorithm

(Miller and others 2009a)

Reproduced with the kind permission of AAN Enterprises, Inc., 2009, LIPPINCOTT WILLIAMS & WILKINS 

MillerandOthers2009aRespiratoryAlgorithm_20100606095616_Resized_20100606095824.jpg
 
KEY

PFT = pulmonary function tests
PCEF = peak cough expiratory flow
NIV = noninvasive ventilation
SNP = sniff nasal pressure
MIP = maximal inspiratory pressure
FVC = forced vital capacity (supine or erect)
Abnl.nocturnal oximetry = pO2 <4% from baseline.
*Symptoms suggestive of nocturnal hypoventilation: frequent arousals, morning headaches, excessive daytime sleepiness, vivid dreams.
+ If NIV is not tolerated or accepted in the setting of advancing respiratory compromise, consider invasive ventilation or referral to hospice.

 

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