MND respiratory management

Question 1

describe the pathophysiology of respiratory insufficiency in MND

Answer 1

reduced number of motor units within the respiratory muscles,

interruption of the descending input from the respiratory centre,

impaired response to hypoxia and hypercapnia,

bulbar dysfunction contributes to respiratory insuffiency

Question 2

what are the clinical markers of respiratory insufficiency in MND?

Answer 2

orthopnea,

nocturnal respiratory insufficiency,

reduced exercise tolerance,

ineffective cough,

recurrent chest infections,

chronic/acute respiratory failure

Question 3

what occurs as a result of hyperventilation?

Answer 3

lack of ability to maintain adequate blood gases,

increased co2 (hypercapnia) and lowering of oxygen (hypoxemia) ,

pathological levels co2>6kpa o2 <10kpa

Question 4

what respiratory feature is a sign of MND?

Answer 4

weak cough / reduced peak cough flow

Question 5

what are the consequences of a weak cough?

Answer 5

secretion stagnation,

lung collapse (atelectasis),

recurrent chest infections,

loss of overall pulmonary compliance

Question 6

what does assisted ventilation do?

Answer 6

alleviates dyspnea,

ameliorates consequences of hypoventilation,

increases lifespam,

improves QOL,

Question 7

what two types of ventilation are available?

Answer 7

NIV (non invasive ventilation): self sufficient, low hazard.

TV: (tracheal ventilation): high hazard, high need for support

Question 8

describe the evidence for NIV survival

Answer 8

bourke et al 2006 showed that NIV lived on average 300 days longer than TV (check this)

Question 9

what are the 5 cough augmentation techniques available?

Answer 9

manual chest physio,

forced expiratory techniques,

manually assisted coughing,

breath stacking technique,

cough assist

Question 10

malnutrition in MND is

Answer 10

common

associated with worsening survival,

increases as diseases progress

Question 11

what are the causes of malnutrition in MND

Answer 11

muscle wasting,

swallowing problems,

physical inability to prepare food,

inability to feed,

increased energy use,

chronic lung infections,

poor appetite

Question 12

what are the consequences of malnutrition in MND

Answer 12

loss of muscle function,

weak immune system,

reduced breathing strength,

skin breakdown,

reduced survival