Cheatography
https://cheatography.com
A cheat sheet describing the management of Neuromuscular patients from a ventilation standpoint.
This is a draft cheat sheet. It is a work in progress and is not finished yet.
Types of Neuromuscular Diseases
Myasthenia Gravis |
Chronic Disorder, Acute events of muscle weakness and fatigue. Descending paralysis. |
Guillain Barre |
Ground up, Ascending paralysis. Usually triggered by an acute infectious process. |
ALS |
Amyotropic Lateral Sclerosis. Progressive neuro-degenerative disease |
Muscular Dystrophy |
Rare group of genetic diseases. Duchenne MD is the most common form among children and Myotonic is the most common in adults |
Multiple Sclerosis |
A slow progressive CNS disease, characterized by disseminated patches of demyelination in the brain and spinal cord. Patients usually experience exacerbations and remissions. |
Spinal Muscular Atrophy Disorders |
A group of hereditary disorders characterized by skeletal muscle wasting due to progressive degeneration of anterior horn cells in the spinal cord and of motor nuclei in the brain stem |
Post-Polio Syndrome |
A group of symptoms that develops years or decades after paralytic poliomyelitis and usually affects the same muscle groups as the initial infection |
Diaphragmatic Paralysis |
May be unilateral where only one side of the diaphragm is affected. |
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Clinical Presentation
Muscle weakness and fatigue |
Exercise intolerance |
Myopathic face (drooping face) |
Pathophysiology of NM Disease
Inspiratory Muslces |
Weakness of these muscles lead to decreased Vt, VC and FRC. May cause atelectasis and alveolar collapse |
Expiratory Muscles |
Weakness of these muscles lead to decreased ability to cough and move secretions. Increased risk of pneumonia. |
Airway Muscles |
Weakness of these muscles lead to decreased ability to protect the airway from aspiration and to speak/swallow. Risk of airway collapse and aspiration pneumonitis |
Lab Findings
ABG |
On arrival, these patient may present with acute ventilatory failure with hypoxemia. |
PFT |
These patients present with decreased lung volumes, decreased MIP and MEP. VC is the most commonly measured parameter for these patients. |
Creatinine Kinase |
Increased in many myopathies. Sign of muscle fiber necrosis. |
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Ventilation Strategy
Indications |
20/30/40 rule: VC < 20 ml/kg, MIP <-30 cmH2O and MEP <40 cmH2O or pH <7.35/PaCO2 > 45 mmHg |
Main concerns |
Typically these patient's lungs are healthy. Our main concern here is protecting the airway and preventing pneumonia. |
Target Gas |
Since the lungs are not the issue we would expect these patients to have a normal gas. |
Type of Ventilation |
These patient benefit from both positive pressure and negative pressure ventilation. Although PPV is seen most often in the hospital setting. PPV can be invasive or noninvasive |
Mode |
Typically VC-CMV will be used in these patients. |
Parameters |
Normal parameters would be used such as: VT 6-8ml/kg, RR 8-12, flow greater or equal to 60 LPM, Ti 1.0, PEEP 5 and FiO2 0.21. Pplat should be <30cmH2O. |
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Adjuncts and Other Treatments
Inline Suction |
Because these patient's muscle are weak they often cannot generate a cough and bronchial hygiene may become difficult. To prevent disconnection with with ventilator, inline suction should be used. |
Cough Assist |
Helps the patient generate a strong cough and improve bronchial hygiene. Cough assist can be used in the long term tracheostomy patient. |
Incentive Spirometry/Hyperinflation Techniques |
Helps mobilize secretions and prevents infection. Breath stacking is often used in these patients. |
Pharmacology |
Steroids for MG, plasmapheresis, pain management, sedative and anxiolytic may be required. |
Challenges With Patient Management
Most NM diseases have no treatment or have a long process leading to long term care. These patients would be ventilated for a long duration of time likely resulting in the use of a tracheostomy.
Be sure to check cuff pressures regularly and change trach as needed. Normal cuff pressure is 20-30cmH2O. |
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