Neuromuscular and Other Diseases of The Chest Wall

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Neuromuscular and Other

Diseases of the Chest Wall

 Pathophysiology and PFT


 Clinical Signs and Symptoms
What is neuromuscular weakness?
• Neuromuscular diseases affects the function
of muscles in your body. These conditions are
usually due to problems with the nerves not
sending information to your muscles and/or
the muscles not working correctly.
Neuromuscular Diseases Causing
Respiratory Failure
• Cerebral Cortex
– Stroke, tumor, traumatic brain injury
• Brainstem
– Drugs, hemorrhage, anoxia, polio, multiple sclerosis
• Spinal Cord
– Trauma, tumor, tetanus, myelitis
• Motor Nerves
– ALS, GBS
• Neuromuscular Junction
– Drugs, MG, neurotoxins
• Myopathies
– Duchenne’s muscular dystrophy, acid maltase deficiency, pompe disease,
myotonic dystrophy
Chest Wall Diseases
• Scoliosis
• Kyphosis
• Poland Syndrome
• Pectus Excavatum
• Pectus Carinatum
• Tumors in the chest wall
• Flail chest
What kind of sleep problems should I look
for if I have a neuromuscular condition?
• 3 problems that can affect breathing with
neuromuscular weakness while sleeping are:
– Nocturnal hypoventilation
– Obstructive sleep apnea
– Central sleep apnea
• A patient can have any or all of these
problems depending on the type of
neuromuscular disease he has.
Chest Wall Malformations
Pectus Excavatum Pectus Carinatum
Cobb angle is an orthopedic measurement used
in vertebral bending disorders. 10 degrees of
deviation from straight before scoliosis is defined.
20 degrees will require back braces while 40
degrees requires surgery

Flail Chest Scoliosis


Kyphosis Poland Syndrome
Pulmonary Consequences of Neuromuscular
Disease
• Hyperventilation (initial sign)
• Central apnea
• Lung hypoventilation
• Pulmonary hypertension
Maintenance of Normal Ventilation

1) Regulation of Respiratory Drive


2) Control of Ventilation
3) Cough and other airway reflexes
Consequences of Neuromuscular Disease

1) Dysregulation of respiratory drive or rate


(inability to ventilate)
2) Loss of strength or control of the mechanics
of breathing (aspiration risk)
3) Loss of strength or control of the muscles
responsible for airway protection and cough
(inability to protect airways from the
pathogens, irritants and foreign aspiration)
Lung Volumes and Capacities
1) Tidal Volume
– Volume of air inhaled and exhaled in a quiet breathing
– Normal: 6-8ml/kg
– It falls when there is a restriction/obstruction.
2) Inspiratory Reserve Volume
– Maximum volume of air which can be inspired after a normal tidal inspiration
from end inspiration.
3) Expiratory Reserve Volume
– Maximum volume of air which can be expired after a normal tidal expiration
from end expiration.
4) Inspiratory Capacity
– Maximum volume of air which can be inspired after a normal tidal expiration.
5) Expiratory Reserve Volume
– Volume of gas that can be exhaled form end inspiratory
level during tidal breathing
6) Residual Volume
– Amount of air remaining in the lungs after forceful
expiration
7) Fractional Residual Capacity
– Amount of air remains in the lungs after a quiet
respiration
8) Vital Capacity
– Maximum volume expired after a maximum inspiration
Lung Volumes and Capacities
Inspiratory Reserve
Volume (3100ml) Inspiratory
Capacity (3600ml) Vital Capacity
(4800ml)
Total Lung Capacity
(6000ml)
Tidal Volume
(500ml)

Expiratory Reserve
Volume (1200ml) Fractional Residual
Capacity (2400ml)

Residual Volume Residual Volume


(1200ml) (1200ml)
PFT Parameters
• Height
• Weight
• Age
• Gender
• Race
• Pack Years (if smoker)
Risks For PFT
• Dizziness during the test
• Feeling short of breath
• Coughing
• Asthma attack brought by deep inhalation
• Barotrauma
Contraindications
• Recent belly or chest surgery
• Chest pain, recent heart attack, or an unstable
heart condition
• Aneurysm
• Active PTB or respiratory infections
• Mechanically ventilated patients
• Unconscious
• Barotrauma or lung injuries
Sample Results
The PFT Machines
Spirometry Plethysmography
• Is a device with a • You sit or stand inside an
mouthpiece hooked up to a air-tight box that looks like a
small electronic machine. short, square telephone
• Can only measure: FVC, booth to do the tests.
Peak expiratory flow rate • Can measure up to TLC
PFT Result Expectancy of a Neuromuscular
Weakness
PFT of Patients With Neuromuscular Disease

• Normal lungs revealing restrictive result as


muscles became weak and won’t facilitate
thoracic expansion. (Decreased VC, FEV1, TLC)
• Residual volume stays the same as normal.
• Position changes directly affects thoracic
expansion. (seated to supine >20% decline in
FEV1 and vital capacity)
Pathophysiology
Impaired CNS Function

Loss of Respiratory Muscle Strength

Decreased Vital Capacity

Slow Shallow Breathing


Severe
Hypercapnia,
Hypoxemia
V/Q Mismatch

Acute Respiratory Failure Type 2 Decrease LOC,


Comatose, Death

Impaired Cellular Metabolism


Typical Clinical Signs and Symptoms of
Neuromuscular Disease
• Early Stage
– Dyspnea
– Easy fatigability
• Intermediate Stage
– Orthopnea
– Pulmonary hypertension
– Anorexia
– Chest pain/tightness
– Syncope
• Late Stage
– Cor pulmonale (right sided heart failure)
– CHF (left and right sided heart failure)
– Pulmonary congestion and systemic congestion
– Multiple organ dysfunction syndrome
Monitoring and Assessment
• Respiratory muscle weakness leads to fatigue
and respiratory failure. (must initiate positive
pressure ventilation or mechanical ventilation
to avoid rapid deterioration)
• Frequent monitoring of oxygenation status,
vital signs and level of consciousness. (ABG,
pulse oximetry, Nero vital signs and bp, temp,
pulse/cardiac monitoring)
Management
• Bronchial hygiene therapy (PD, PV, coughing
techniques)
• Breathing Exercises
• Non-invasive ventilation (CPAP, BIPAP)
• Mechanical Ventilation (worse scenario)
• Soft diets/NGT feedings (to reduce the risk of
aspiration)

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