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30 Cards in this Set
- Front
- Back
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What are the some of the pulmonary consequences that neuromuscular disease (NMD) may include?
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- hyperventilation or hypoventilation
- sleep apnea - aspiration - atelectasis causing hypoxemia - pulmonary hypertension (HTN) - cor pulmonale |
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What are the clinical signs & symptoms of NMD?
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- initially: dyspnea, fatigue, orthopnea, or symptoms of cor pulmonale (occur secondary to muscles inability to maintain adequate ventilation)
- sometimes power & volume of voice decline - deterioration may lead to rapid shallow breathing & hypoventilation (increased PaCO2) |
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What does normal lung parenchyma reveal w/ NMD?
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- restrictive defect (decreased VC, FEV1, TLC)
- normal RV & diffusing capacity corrected for alveolar minute ventilation |
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What do positional changes associated w/ NMD suggest?
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- diaphragmatic weakness
- seated to supine: >20% decline in FEV1 & VC |
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What does an ABG for a patient w/ NMD look like?
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- decreased PaO2, decreased PaCO2
- deterioration leads to increased PaCO2 |
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What does progressive NMD lead to?
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- fatigue & respiratory failure (may necessitate MV, so monitor carefully to determine when to initiate)
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What does monitoring of NMD involve?
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- serial measurements of Plmax, VC, & ABG values
- may monitor maximal nasal sniff inspiratory force & nocturnal oximetry - close monitoring of all respiratory function is important |
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What does respiratory muscle weakness in patients w/ NMD lead to?
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- respiratory insufficiency & retained secretions
- consider NIV or MV via tracheostomy - augmentation of secretion clearance & cough |
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What is increasingly used to treat acute deterioration associated w/ respiratory muscle weakness in the patient w/ neuromuscular disease?
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- NIV (pneumonia or surgical event)
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What is an FDA approved technique for treating NMD?
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- diaphragmatic pacing for spinal injuries
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What are some neuromuscular disease disorders of the muscle?
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- Duchennes muscular dystrophy
- myotonic dystrophy - polymyositis |
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What are the characteristics of Duchennes muscular dystrophy?
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- X-linked recessive muscle-wasting disorder
- manifests early by waddling gait, lordosis, & frequent falls - most are wheelchair bound by age 12 - death occurs by age 20, usually due to secondary respiratory infection |
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What begins to happen to a patient w/ Duchennes muscular dystrophy at around age 12?
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- point of significant respiratory function decline
- adapt a rapid, shallow breathing pattern - progression leads to PPV (initially only nocturnal) |
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What is the most common type of muscular dystrophy (MD) in adults?
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- myotonic dystrophy
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What are some characteristics of myotonic dystrophy?
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- respiratory dysfunction is common
- respiratory muscle weakness - OSA & CSA very common even at early age - aspiration |
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What do patients w/ myotonic dystrophy tend to be sensitive to?
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- anesthesia & respiratory depressants so prolonged postoperative monitoring is required
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What are some treatments associated w/ myotonic dystrophy?
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- nocturnal NIV for O2 and ventilation issues, while central hypoventilation requires tracheostomy & MV
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What is polymyositis?
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- inflammatory myopathy of unknown cause
- ventilatory insufficiency & failure are usual (if occur, parallel the progression of limb weakness) |
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What are the mainstay of management for patients w/ polymyositis?
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- corticosteroids
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What do 10%-30% of patients w/ polymyositis develop?
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- interstitial lung disease w/ diffuse infiltrates predominantly in the bases
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What are some disorders of the neuromuscular junction?
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- myasthenia gravis (MG)
- Lambert-Eaton syndrome (LES) |
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What is myasthenia gravis (MD) characterized by?
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- intermittent muscle weakness
- worsens w/ repetition - improves w/ anticholinesterase medications - progressive loss of muscle function often starting w/ eye muscles - better in morning, worsens through day |
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What is MG caused by?
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- antibodies that inactivate ACh-R, blocking electrical impulse transmission
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What abnormalities are common w/ MG?
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- abnormalities of thymus gland are common
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In what type of patient does MG typically occur in?
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- younger females
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How is MG diagnosed?
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- by the presence of anti-ACh-R antibodies & improvement w/ use of edrophonium
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What do treatments for MG include?
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- thymectomy & anticholinesterase drughs
- plasmapheresis to remove anti-ACh-R antibodies |
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What do pulmonary complications due to MG depend on?
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- affected muscles
- upper airway obstruction - exertional dyspnea - ventilatory failure |
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What do patients w/ MG typically display?
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- decreased TLC, VC, Plmax, PEmax
- sensitive to impending respiratory weakness |
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What is a myasthenic crisis?
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- acute respiratory insufficiency caused by infection, surgery, excessive anticholinesterases
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