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CRNA principles

Anesthesia for patients with neuromuscular diseases

What is the etiology of osteoarthritis (OA)? Caused by repetitive joint trauma
Osteoarthritis is commonly associated with what age groups and conditions? Advancing age and morbid obesity
What does osteoarthritis most commonly effect? Degenerative disease effecting Articular surface of joints, most commonly hips and knees
What is the pathology of of Rheumatoid arthritis (RA)? RA is a immune mediated joint destruction characterized by chronic, progressive inflammation of synovial membranes.
True or false: RA is a systemic disease effecting women 30-50 years old? True
What part of the spine is most effected by RA? Cervical
What cardiovascular abnormalities are seen with RA? Pericardial thickening/effusion, dilation of aortic root may result in cardiac valve fibrosis.
What pulmonary abnormalities are seen with RA? Pleural effusions are common, restrictive lung changes may result from costochondral involvement
What hematopoietic manifestations are seen in RA? Anemia, plt dysfunction, thrombocytopenia
What dermatological conditions are seen in RA? Thin atrophic skin from disease/immunosuppressive drugs.
Treatment for RA/OA for patients who don't respond well to NSAIDS/steroids? Gold salts/cytotoxic drugs
Anesthetic considerations for RA/OA? Evaluate for cardiac/pulmonary diseases
Cervical spine considerations for pt's with RA/OA? Careful neck positioning due to high frequency of cervical spine involvement
Atlantoaxial subluxation risk for pt's with RA include? Protrusion of odontoid process into the foramen magnum during intubation.
During intubation, what does protrusion of the odontoid process into the foramen magnum compress? blood flow, spinal cord, brain stem
What diagnostic test is important for pt's with RA and why? Lateral XR's because atlantoaxial subluxation can be asymptomatic
How many mm of atlantoaxial instability necessitates and awake Fiber optic intubation with neck stabilization? 5 mm
Limits of jaw mobility in pt's with RA/OA include? TMJ/cricoarytenoid arthritis can complicate intubation
What are some complications from cricoarytenoid arthritis and what kind of ETT can you use in these patients? narrowing of glottic opening, as evidence by hoarseness/inspiratory stridor. Use of a smaller ETT tube.
Risks associated with cricoarytenoid arthritis? Post extubation airway obstruction
Pathology associated with Myasthenia Gravis (MG)? Autoimmune destruction/inactivation of postsynaptic acetylcholine receptors at neuromuscular junction. IgG antibodies against nicotinic act receptors in NMJ are found in pt's with MG.
Two Classifications of Myasthenia Gravis? Two classifications include: (1) only ocular (2)ocular and monocular muscle weakness
Other autoimmune disorders associated with MG include? RA, Hyperthyroid, hypothyroid
Ages and genes that MG most commonly effects? Men in their 60's-70's along with women in their 3rd decade of life
Effects of ocular muscles from MG include? Fluctuating ptosis, diplopia
Bulbar involvment causes what complications with MG? Laryngeal/pharyngeal muscle weakness resulting in dysarthria, difficulty chewing/swallowing, pulmonary aspirations, unable to clear secretions.
Severe MG is associated with what two types of muscle weakness? Respiratory muscles and proximal muscle weakness (neck and shoulders)
What conditions have unpredictable effects on MG and often lead to exacerbations? Infection, stress, surgery, pregnancy
Commonly used drugs for MG include? Anticholinesterase drugs increase the amount of act at the NMJ through inhibition of end plate achE
Most common drug used to treat MG? Pyridostigmine given PO every 2-4 hours.
Excessive administration of anticholinesterases may precipitate a cholinergic crisis, these symptoms include: increased weakness, excessive muscarinic effects, salivation, diarrhea, miosis, decreased HR.
How do you differentiate a cholinergic vs myasthenic crisis? Edrophonium test: Increased weakness after a max of 10mg of IV edrophonium indicates cholinergic crisis. Increased strength post edrophonium implies myasthenic crisis.
Treatments for MG include? Thymectomy if less than 55 years old, plasmapheresis for pt's with dysphagia/respiratory failure, anticholinesterase drugs, corticosteriods, cyclosporine, immunogloblin therapy
Potential problems with continuing MG therapy prior to anesthesia include? Increased vagal reflexes, can prolong duration of ester LA/sch
MG pt's with bulbar involvement are at risk for? Pulmonary aspiration
MG pt's may be sensitive to what medications pre-op? Respiratory depressants such as Opiods & Benzodiazepines. This can be seen with smaller than normal doses
Effects of NMBA on MG ? Increased sensitivity, if necessary give cisatracurium or mivacurium, make sure you carefully evaluate pulmonary status prior to extubation.
Effects of Succinylcholine (Sch) on MG? Unpredictable, prolonged effect, can cause phase II block
Most preferred drug for MG? Propofol due to its short DOA
What volatile agents are most desired for MG? Desflurane or Sevoflurane
Whats the greatest anesthesia risk to be concerned with MG? Post op respiratory failure
MG and pregnancy, increased weakness is common in what trimester? What type of anesthesia is preferred? 3rd trimester epidural anesthesia
Lambert eaton myasthenic syndrome manifestations? Paraneoplastic syndrome characterized by proximal muscle weakness that typically begins in the lower extremities. May spread to the upper limbs, bulbar, and respiratory muscles.
CA associated with Lambert eaton syndrome? Small cell CA of lung
Common manifestations of Lambert eaton syndrome include Autonomic dysfuction, dry mouth, male impotence.
Lambert eaton pathology Presynaptic defects of neuromuscular transmission. Antibodies to voltage gaited calcium channels on the nerve terminal reduce release of act at the motor end plate.
Lambert eaton syndrome muscle weakness is improved by? Muscle weakness improves with repeated effort and is improved less with anticholinesterase drugs.
Treatment for Lambert eaton syndrome? Guanidine HCL & 3,4 diaminopyridine: Increase release of ach producing improvement.
Guanidine HCL causes? Hepatotoxicity
What anesthetics are pt's with Lambert eaton syndrome sensitive to? Depolarizing and Non-depolarizing NMBA. If absolutely necessary give small doses of NMBA with careful neuromuscular monitoring.
True or false? Volatile agents are usually sufficient to provide muscle relaxation in pt's with lambert eaton syndrome. True
Duchanne's muscular dystrophy (DMD) pathology/symptoms? Abnormal dystrophin is produced, a protein found on the sarcolemma of muscle fibers. This develops muscle weakness and gait disturbance.
Age/Gender of DMD include? Children 3-5 years old, most commonly male from X-linked recessive disorder. Most are WC bound by age 12
Complications associated with DMD? Fatty infiltration causes psedohypertrophy of muscles, mostly calves. Progressive weakness/contractures eventually result in kyphoscoliosis, this causes retention of secretions and frequent pulmonary infections. Pulmonary HTN
How to delay disease progression of DMD? Glucocorticoid therapy
Cardiovascular complications of DMD? Atrial arrithmias, degeneration of cardiac muscle. Death occurs from respiratory or cardiac complications.
Differences between DMD and Beckers Muscular dystrophy (BMD)? Less common, presents later in life and progresses more slowly. Mental retardation is less common, usually reach 40-50 years old. Death=Resp complications
Anesthetic considerations for DMD and BMD? Association with MH suggested but not proven. Sch used safely, but best to avoid. Avoid pre-meds due to aspiration precautions.
Risk of using Sch in pt's with DMD or BMD include? Hyperkalemia triggering MH
True or False? Pt's with DMD or BMD can be sensitive to non-depolarizing NMBA? True
Myotonic dystrophy physiology? Slowing of relaxation after muscle contraction in response to electrical/percussive stimuli.
Principle manifestation of myotonic dystrophy ? Myotonia, muscle weakness. atrophy. Usually effects cranial muscles, distal muscles involved more than proximal.
Myotonic dystrophy multiple organ systems involved? Cataracts, premature frontal blindness. Hypersomnolence with sleep apnea. Endocrine dysfunction leading to pancreatic, adrenal, thyroid, and gonadal insufficiency.
Respiratory involvement of myotonic dystrophy leads to what pulmonary complications? Alveolar hypoventilatoin and decreased Vital capacity
GI involvement of myotonic dystrophy lead to what GI complications? GI hypo motility which can predispose to pulmonary aspiration
Cardiac manifestations of myotonic dystrophy? Atrial arrithmias, heart block, depressed ventricular function
How do you best describe myotonia? "stiffness" that may ease with continued activity. Patients report stiffness with cold temps.
Should Sch be given to pt's with myotonic dystrophy? No, ssh should not be given, it may precipitate intense myotonic contractions.
Should non-depolarizers be given to pt's with myotonic dystrophy? the response to Non-depolarizers is reported to be normal. May not prevent/relieve myotonic contractions. Reversal drugs aggregate myotonia.
Effects of opiods, sedatives, inhalational agents on pt's with myotonic dystrophy? Can cause sudden/prolonged apnea
Postop pulmonary complications of myotonic dystrophy? Prolonged hypoventilation, atelectasis, pneumonia. Use aspiration prophylaxis, agressive pulmonary hygiene with pt, incentive spirometry, careful postop monitoring.
Periodic paralysis disorders are characterized by? sudden attacks of transient muscle weakness/paralysis. Attacks can last for a few hours for up to 2 days.
What causes these attacks with periodic paralysis? Attacks/weakness are due to loss of muscle fiber excitability due to partial depolarization of the resting potential. Depolarization prevents the generation of action potentials, precipitating weakness
Sporadic episodes of weakness can worsen with what condition in patients with periodic paralysis? Hypothermia
Hypokalemic periodic paralysis is associated with what disorder? Hyperthyroidism
Hypokalemic periodic paralysis is associated with what channel? Calcium channelopathy
What precipitates hypokalemic periodic paralysis? Strenuous exertion, high carb meals
What part of they day is common for a patient with hypokalemic periodic paralysis to have symptoms? Early in the AM
What happens during a hypokalmeic periodic paralysis attack? Potasium/phosphorus levels are normal or slightly decreased, kidneys retain sodium, potassium, Chloride, and H20.
What happens during a hypokalmeic periodic paralysis attack to the ICF and ECF? Increased ICF volume and decreased ECF volume.
How do you treat an attack of hypokalemic periodic paralysis? 2-10g of K+, also encourage exercise
What should be avoided in patients with Hypokalemic periodic paralysis? Glucose solution, due to the uptake of glucose into cells, with changes in serum potassium can worsen the hypokalemia/weakness.
Hyperkalemic Periodic paralysis effects what channels Sodium channelopathy
Hypokalemic periodic paralysis can last how long? 3-4 hours to days!
Hyperkalemic periodic paralysis can last how long? Shorter more frequent attacks lasting 1-2 hours
What occurs at a cellular level with hyperkalemic periodic paralysis? Paralysis triggered by abnormal inactivation of sodium channels by a mild increase in potassium.
What happens with depolarization in pt's with hyperkalemic periodic paralysis? Sodium/water flows into cells with prolonged depolarization resulting in hyponatremia/hemoconcentration.
Conditions aggravated by hyperkalemic periodic paralysis? Hypothermia, pregnancy, glucocorticoids, potassium.
What happens during an attack of hyperkalemic periodic paralysis? Potassium levels can increase to >6 MEq/L but remain normal between attacks
Anesthesia considerations for hyperkalmeic periodic paralysis? Frequent K+ levels, avoid Sch!, monitor neuromuscular function, maintain core temperature and prevent shivering considering hypothermia can trigger these attacks. Careful ECG monitoring.
Created by: evanrn1983