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Disease 3 > Neuromuscular disease > Flashcards

Neuromuscular disease Flashcards

1
Q

What are the 4 levels of pathologic injury in NMD?

A

Upper motor neuron
Lower motor neuron
Neuromuscular junction
Muscle

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2
Q

What is the normal response to oxygen desaturation vs the normal response to a decrease in PaCO2?

A

SpO2 = Linear, 1% decrease in sat = 1 L/m increase in minute ventilation
PaCO2 = Steep linear, 1 mmHg increase in PaCO2 = 2.5-3 L/m increase in MV

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3
Q

Describe how patients with NMD respond to changes in O2 sat and PaO2

A

Exhibit a significantly decreased response

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4
Q

Describe how mouth occlusion pressure is tested

A

Mouth occlusion pressure is tested by measuring the negative pressure produced during the first 100 milliseconds of inhalation with complete airway occlusion

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5
Q

How can respiratory drive be demonstrated?

A

Mouth occlusion pressure

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6
Q

What nerves innervate the muscles of expiration?

A

Lumbar
Intercostal

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6
Q

Why is mouth occlusion pressure an effective measure of respiratory drive?

A

It is measured so early in the breath that it cannot be voluntarily interfered with
Requires only a fraction of total muscle strength so it remains viable even in severely compromised patients

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7
Q

What nerves innervate the upper airways?

A

Glossopharyngeal
Vagus
Spinal accessory
Hypoglossal

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8
Q

What nerves innervate the muscles of inspiration?

A

Phrenic
Cervical
Intercostal
Spinal accessory

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9
Q

When does hypercapnia develop in relation to NIF score?

A

Once NIF declines to less than 30% of predicted, hypercapnia develops

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9
Q

What might a patient with NMD complain of besides dyspnea?

A

Fatigue
Poor sleep
Dyspnea on exertion

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10
Q

Why might a patient with a NMD develop sleep apnea?

A

Damage or disorders of the nerves may be more readily apparent at night when conscious control of breathing is absent and cannot compensate

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11
Q

Why can a patient with NMD develop decreased lung compliance?

A

Microatelectasis from reduced respiratory muscle strength \
Increases alveolar surface tension from low lung volumes
Sedentary lifestyle contributing to low tidal breaths

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12
Q

Why can the chest wall of a patient with NMD become stiff?

A

Chest wall structures such as tendons ligaments and costosternal articulations can grow stiff due to lack of physical movement from large breaths

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12
Q

What should be measured routinely in NMD patients in order to track the progression of the disease process?

A

Maximum inspiratory pressure
Maximum expiratory pressure

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13
Q

How can transdiaphragmatic pressure be measured?

A

Catheter with a balloon goes to mid esophagus (Pes), catheter with a balloon goes to (Pga) and the pressure difference is recorded to give us transdiaphragmatic pressure

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14
Q

In patient with diaphragmatic weakness, which position would be provide for a better FVC?

A

Upright

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15
Q

In patients with spinal cord injuries, which position would be better for providing a greater FVC?

A

Supine

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16
Q

What is the normal Pi max in men and women?

A

Men = -105 - -124 cm H2O
Women = -91 - –71 cm H2O

17
Q

What is measured by measuring transdiaphragmatic pressure?

A

Diaphragmatic strength

18
Q

What is the normal Pe max in men and women?

A

Men = 233 - 140 cm H2O
Women = 216 - 89 cm H2O

19
Q

Define hemorrhagic stroke

A

Blood vessel weakened by aneurysm or arteriovenous malformation ruptures and spills blood into the brain
20% of all strokes

20
Q

Define an ischemic stroke

A

80% of all strokes
Blood clot or plaque blocks off oxygen and nutrition to part of brain downstream of blockage

21
Q

What is occurring in multiple sclerosis?

A

Demyelination of the central nervous system

22
Q

What kind of respiratory problems could be caused by a stroke?

A

Loss of upper airway coordination resulting in aspiration
Loss of respiratory control resulting in either cheyne stokes breathing, apneustic breathing or ataxic breathing

23
Q

An injury to what part of the spine will likely resulting in the need for chronic respiratory support?

A

C1-C3

24
Q

An injury to what part of the spine will result in the preservation of diaphragmatic function?

A

C5-C6

25
Q

What risks do patients with parkinson’s disease face?

A

Ventilatory failure
Upper airway obstruction
Aspiration

26
Q

How does MS affect the respiratory system?

A

Respiratory muscle weakness
Bulbar dysfunction
Abnormalities in respiratory control

27
Q

What upper airway difficulties do individuals with amyotrophic lateral sclerosis face?

A

Dysarthria
Layngospasm
Dysphagia
Risk for aspiration

28
Q

Why is counseling for patients with ALS important?

A

Prepares them for what to expect as their disease progresses, allows for decisions regarding their care to be made ahead of time and allows them to understand options for ventilatory support

29
Q

Where is the bacteria that causes tetanus found?

A

Found in the guts of many animals and is widely found in soil

29
Q

Describe what is happening in a patient with Duchenne muscular dystrophy

A

Defective gene on X chromosome
Gene is responsible for production of dystrophin
Lack of dystrophin results in damaged muscles that lose functionality

30
Q

How does tetanus affect muscle control?

A

Bacteria secrete a toxin that inactivates inhibitory neurotransmission resulting in the muscle being unable to “turn off”

31
Q

Why do patients with tetanus need to be sedated and mechanically ventilated?

A

Sedation controls the muscle spasms but inhibits breathing
Patients may spend weeks on vent and be trached

32
Q

What kind of training can be performed by patients with NMD to train their respiratory muscles?

A

Devices that set a resistive load for inhalation
Studies do not show improved outcomes

33
Q

What peak flow indicates a need for assisted cough?

A

Less than 160 L/m

34
Q

Describe a quad cough

A

Basically a manual cough assist

35
Q

What maneuvers can patients perform in order to remain independent from mechanical support for short periods of time or build up enough volume for a sufficient cough?

A

Breath stacking
Manual resuscitator
Frog breathing/glossopharyngeal breathing

36
Q

What is another option for assisting patients cough?

A

Mechanical insufflator-exsufflator
Provides positive pressure during inhalation, negative pressure during exhalation to aid cough

37
Q

What factors favor NIV for NMD patients?

A

Awake and cooperative patient
Good control of airway
Has minimal secretions
Maintains hemodynamic stability

38
Q

What factors favor invasive ventilation in NMD patients?

A

Copious secretions
Poor airway control
Doesnt tolerate NIV
Sufferes from impaired cognition
Maintains unstable hemodynamics

39
Q

How should patients with spinal injuries be ventilated?

A

Ventilated with large tidal volumes, 10-15 ml/kg

40
Q

What are the benefits associated with diaphragmatic pacing?

A

Allows some patients to come off of ventilator

41
Q

What are the downsides of diaphragmatic pacing?

A

Associated with increased risk of mortality in ALS patients
Expensive
Potential for sudden hardware failure
Possible for upper airway to become obstructed after tracheotomy closes
Can induce diaphragmatic fatigue

Disease 3 (9 decks)

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