A nurse is planning care for a client who has a deficit with cranial nerve II

MG, thought to be an autoimmune disorder, is caused by a loss of acetylcholine (ACh) receptors in the postsynaptic neurons at the neuromuscular junction. About 80% of all MG patients have elevated titers for ACh receptor antibodies, which can prevent the ACh molecule from binding to these receptor sites or can cause damage to them. MG is often associated with thymic tumors.

Assessment

1. Weakness and fatigue
2. Difficulty chewing
3. Dysphagia
4. Ptosis
5. Diplopia
6. Weak, hoarse voice
7. Difficulty breathing
8. Diminished breath sounds
9. Respiratory paralysis and failure

Complications

• This sudden onset of muscle weakness is usually the result of undermedication or no cholinergic medication at all. Myasthenic crisis may result from progression of the disease, emotional upset, systemic infections, medications, surgery, or trauma. The crisis is manifested by sudden onset of acute respiratory distress and inability to swallow or speak.

• Caused by overmedication with cholinergic or anticholinesterase drugs, cholinergic crisis produces muscle weakness and the respiratory depression of myasthenic crisisas well as gastrointestinal symptoms (nausea, vomiting, diarrhea), sweating, increased salivation, and bradycardia.

Primary Nursing Diagnosis

• Ineffective airway clearance related to difficulty in swallowing and aspiration

Diagnostic Evaluation

• Injection of edrophonium (Tensilon) is used to confirm the diagnosis (have atropine available for side effects). Improvement in muscle strength represents a positive test and usually confirms the diagnosis.
• MRI may demonstrate an enlarged thymus gland.
• Test includes serum analysis for acetylcholine receptor and electromyography (EMG) to measure electrical potential of muscle cells.

Holding your arms above your shoulders until they drop is one exercise that may be performed during the Tensilon test. In this test, the drug Tensilon is administered, and the response in the muscles are evaluated to help diagnose myasthenia gravis or to help differentiate between myasthenia gravis and other conditions.

Medical Management

Management is directed at improving function through the administration of anticholinesterase medications and by reducing and removing circulating antibodies. Patients with MG are usually managed on an outpatient basis unless hospitalization is required for managing symptoms or complications.

Pharmacologic Highlights

• Anticholinesterase drugs such as Pyridostigmine bromide (Mestinon), neostigmine bromide (Prostigmine); neostigmine methylsulfate can be given as a continuous infusion if the patient cannot take oral medication. Anticholinesterase drugs blocks the action of the enzyme anticholinesterase, thereby producing symptomatic improvement; atropine must be readily available to treat cholinergic side effects and medications must be administered on time, or the patient may be too weak or unable to swallow the drug.
• Prednisone is used to suppresses the autoimmune activity of MG
• Nonsteroidal immunosuppressants such as Azathioprine (Imuran), cyclophosphamide (Cytoxin) is used to suppress autoimmune activity when patients do not respond to prednisone; can produce extreme immunosuppression and toxic side effects

Nursing Interventions

1. Monitor respiratory status and ability to cough and deep breathe adequately.
2. Monitor for respiratory failure.
3. Maintain suctioning and emergency equipment at the bedside.
4. Monitor vital signs.
5. Monitor speech and swallowing abilities to prevent aspiration.
6. Encourage the client to sit up when eating.
7. Assess muscle status.
8. Instruct the client to conserve strength.
9. Plan short activities that coincide with times of maximal muscle strength.
10. Monitor for myasthenic and cholinergic crises.
11. Administer anticholinesterase medications as prescribed.
12. Instruct the client to avoid stress, infection, fatigue, and over-the counter medications.
13. Instruct the client to wear a Medic-Alert bracelet.
14. Inform the client about services from the Myasthenia Gravis Foundation.

Documentation Guidelines

• Respiratory status: Rate, quality, depth, ease, breath sounds, arterial hemoglobin saturation with oxygen
• Ability to chew, swallow, and speak (swallowing can be subjectively rated by the patient in anticipating ability to swallow food [0 =unable to swallow liquids to 5 = able to swallow regular diet]), food intake, daily weights
• Muscle weakness and strength, speed and degree of fatigue, ability to perform activities of daily living, response to rest, and plans for modification of activity
• Ptosis (can be rated by the nurse [0 = unable to open lid to 5 =uppermost edge of iris visible])

Discharge and Home Healthcare Guidelines

• Instruct the patient and family on the importance of rest and avoiding fatigue. Be alert to factors that can cause exacerbations, such as infection (an annual flu shot is suggested), surgery, pregnancy, exposure to extreme temperatures, and tonic and alcoholic drinks. Instruct the patient and family about drug actions and side effects, the indications for dosage alteration, and the selective use of atropine for any overdose.
• Stress the importance of taking the medication in a timely manner. It is advisable to time the dose 1 hour before meals for best chewing and swallowing. Explain the potential drug interactions (especially aminoglycosides and neuromuscular blocking agents, which include many pesticides). Encourage the patient to inform the dentist, ophthalmologist, and pharmacist of the myasthenic condition.
• Instruct patients about the symptoms that require emergency treatment, and encourage them to locate a neurologist familiar with MG management for any follow-up needs. Suggest that they collect a packet of literature to take to the emergency department in case the available physician is unfamiliar with this disease. (The Physician’s Handbook is available on request from the MG Foundation.)
• Instruct patients to wear MG identification jewelry. Suggest having an “emergency code” to alert family if they are too weak to speak (such as ringing the phone twice and hanging up).
• Instruct the family about cardiopulmonary resuscitation techniques, how to perform the Heimlich maneuver, how to contact the rescue squad, and how to explain the route to the hospital.
• Make a referral to a vocational rehabilitation center if guidance for modifying the home or work environment, such as a raised seat and handrail for the toilet, would be beneficial.

Exam

MSN Exam for Myasthenia Gravis (PM)

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Question 1

A female client has experienced an episode of myasthenic crisis. The nurse would assess whether the client has precipitating factors such as: 

A

Getting too little exercise

B

Omitting doses of medication

C

Increasing intake of fatty foods

D

Taking excess medication

Question 1 Explanation: 

Myasthenic crisis often is caused by undermedication and responds to the administration of cholinergic medications, such as neostigmine (Prostigmin) and pyridostigmine (Mestinon). Cholinergic crisis (the opposite problem) is caused by excess medication and responds to withholding of medications. Too little exercise and fatty food intake are incorrect. Overexertion and overeating possibly could trigger myasthenic crisis.

Question 2

The nursing assistant reports to you, the RN, that the patient with myasthenia gravis (MG) has an elevated temperature (102.20 F), heart rate of 120/minute, rise in blood pressure (158/94), and was incontinent off urine and stool. What is your best first action at this time?

A

Administer an acetaminophen suppository.

B

Reschedule patient’s physical therapy.

C

Notify the physician immediately.

D

Recheck vital signs in 1 hour.

Question 2 Explanation: 

The changes that the nursing assistant is reporting are characteristics of myasthenia crisis, which often follows some type of infection. The patient is at risk for inadequate respiratory function. In addition to notifying the physician, the nurse should carefully monitor the patient’s respiratory status. The patient may need incubation and mechanical ventilation. The nurse would notify the physician before giving the suppository because there may be orders for cultures before giving acetaminophen. This patient’s vital signs need to be re-checked sooner than 1 hour. Rescheduling the physical therapy can be delegated to the unit clerk and is not urgent. Focus: Prioritization

Question 3

Helen is diagnosed with myasthenia gravis and pyridostigmine bromide (Mestinon) therapy is started. The Mestinon dosage is frequently changed during the first week. While the dosage is being adjusted, the nurse’s priority intervention is to:

A

Administer the medication exactly on time

B

Evaluate the client’s emotional side effects between doses

C

Evaluate the client’s muscle strength hourly after medication

D

Administer the medication with food or mild

Question 3 Explanation: 

Peak response occurs 1 hour after administration and lasts up to 8 hours; the response will influence dosage levels.

Question 4

Karina a client with myasthenia gravis is to receive immunosuppressive therapy. The nurse understands that this therapy is effective because it:

A

Promotes the removal of antibodies that impair the transmission of impulses

B

Decreases the production of autoantibodies that attack the acetylcholine receptors.

C

Stimulates the production of acetylcholine at the neuromuscular junction.

D

Inhibits the breakdown of acetylcholine at the neuromuscular junction.

Question 4 Explanation: 

Steroids decrease the body’s immune response thus decreasing the production of antibodies that attack the acetylcholine receptors at the neuromuscular junction

Question 5

The diagnostic work-up of a client hospitalized with complaints of progressive weakness and fatigue confirms a diagnosis of myasthenia gravis. The medication used to treat myasthenia gravis is:

A

Atropine (atropine sulfate)

B

Didronel (etidronate)

C

Prostigmine (neostigmine)

D

Tensilon (edrophonium)

Question 5 Explanation: 

Protigmine is used to treat clients with myasthenia gravis. Atropine (atropine sulfate) is incorrect because it is used to reverse the effects of neostigmine. Didronel (etidronate)is incorrect because the drug is unrelated to the treatment of myasthenia gravis. Tensilon (edrophonium) is incorrect because it is the test for myasthenia gravis.

Question 6

A physician diagnoses a client with myasthenia gravis, prescribing pyridostigmine (Mestinon), 60 mg P.O. every 3 hours. Before administering this anticholinesterase agent, the nurse reviews the client’s history. Which preexisting condition would contraindicate the use of pyridostigmine?

A

Ulcerative colitis

B

Intestinal obstruction

C

Blood dyscrasia

D

Spinal cord injury

Question 6 Explanation: 

Anticholinesterase agents such as pyridostigmine are contraindicated in a client with a mechanical obstruction of the intestines or urinary tract, peritonitis, or hypersensitivity to anticholinesterase agents. Ulcerative colitis, blood dyscrasia, and spinal cord injury don’t contraindicate use of the drug.

Question 7

Which of the following is not an autoimmune disease?

A

Myasthenia gravis

B

Alzheimer's disease

C

Graves disease

D

Insulin-dependent diabetes mellitus

Question 8

The nurse is caring for a client admitted with suspected myasthenia gravis. Which finding is usually associated with a diagnosis of myasthenia gravis?

A

Cogwheel rigidity and loss of coordination

B

Progressive weakness that is worse at the day’s end

C

Ascending paralysis and loss of motor function

D

Visual disturbances, including diplopia

Question 8 Explanation: 

The client with myasthenia develops progressive weakness that worsens during the day. Visual disturbances, including diplopia is incorrect because it refers to symptoms of multiple sclerosis. Ascending paralysis and loss of motor function is incorrect because it refers to symptoms of Guillain Barre syndrome. Cogwheel rigidity and loss of coordination is incorrect because it refers to Parkinson’s disease.

Question 9

A client with myasthenia gravis ask the nurse why the disease has occurred. The nurse bases the reply on the knowledge that there is:

A

A reduced amount of neurotransmitter acetylcholine

B

A decreased number of functioning acetylcholine receptor sites

C

An inhibition of the enzyme ACHE leaving the end plates folded

D

A genetic in the production acetylcholine

Question 10

The initial nursing goal for a client with myasthenia gravis during the diagnostic phase of her hospitalization would be to:

A

Maintain the present muscle strength

B

Develop a teaching plan

C

Facilitate psychologic adjustment

D

Prepare for the appearance of myasthenic crisis

Question 10 Explanation: 

Until diagnosis is confirmed, primary goal should be to maintain adequate activity and prevent muscle atrophy

Question 11

Helen, a client with myasthenia gravis, begins to experience increased difficulty in swallowing. To prevent aspiration of food, the nursing action that would be most effective would be to:

A

Change her diet order from soft foods to clear liquids

B

Place an emergency tracheostomy set in her room

C

Coordinate her meal schedule with the peak effect of her medication, Mestinon

D

Assess her respiratory status before and after meals

Question 11 Explanation: 

Dysphagia should be minimized during peak effect of Mestinon, thereby decreasing the probability of aspiration. Mestinon can increase her muscle strength including her ability to swallow.

Question 12

While reviewing a client’s chart, the nurse notices that the female client has myasthenia gravis. Which of the following statements about neuromuscular blocking agents is true for a client with this condition?

A

Succinylcholine shouldn’t be used; pancuronium may be used in a lower dosage.

B

Pancuronium and succinylcholine both require cautious administration.

C

Pancuronium shouldn’t be used; succinylcholine may be used in a lower dosage.

D

The client may be less sensitive to the effects of a neuromuscular blocking agent.

Question 12 Explanation: 

The nurse must cautiously administer pancuronium, succinylcholine, and any other neuromuscular blocking agent to a client with myasthenia gravis. Such a client isn’t less sensitive to the effects of a neuromuscular blocking agent. Either succinylcholine or pancuronium can be administered in the usual adult dosage to a client with myasthenia gravis.

Question 13

Jane, a 20- year old college student is admiited to the hospital with a tentative diagnosis of myasthenia gravis. She is scheduled to have a series of diagnostic studies for myasthenia gravis, including a Tensilon test. In preparing her for this procedure, the nurse explains that her response to the medication will confirm the diagnosis if Tensilon produces:

A

Brief exaggeration of symptoms

B

Symptomatic improvement of just the ptosis

C

Rapid but brief symptomatic improvement

D

Prolonged symptomatic improvement

Question 13 Explanation: 

Tensilon acts systemically to increase muscle strength; with a peak effect in 30 seconds, It lasts several minutes.

Question 14

A client with myasthenia gravis has been receiving Neostigmine (Prostigmin). This drug acts by:

A

Accelerating transmission along neural swaths

B

Replacing deficient neurotransmitters

C

Blocking the action of cholinesterase

D

Stimulating the cerebral cortex

Question 15

Myasthenia gravis reflects a deficiency in communication by _______________ because receptors for this neurotransmitter have been destroyed.

A

GABA

B

norepinephrine

C

dopamine

D

acetylcholine

Question 16

Myasthenia gravis is due to ____ receptors being blocked and destroyed by antibodies.

A

Acetylcholine

B

Epinephrine

C

Transient

D

Nicotinic

Question 17

In making a diagnosis of myasthenia gravis Edrophonium HCI (Tensilon) is used. The nurse knows that this drug will cause a temporary increase in:

A

Blood pressure

B

Muscle strength

C

Symptoms

D

Consciousness

Question 17 Explanation: 

Tensilon, an anticholinesterase drug, causes temporary relief of symptoms of myasthenia gravis in client who have the disease and is therefore an effective diagnostic aid.

Question 18

The most significant initial nursing observations that need to be made about a client with myasthenia include:

A

Ability to smile an to close her eyelids

B

Ability to chew and speak distinctly

C

Respiratory exchange and ability to swallow

D

Degree of anxiety about her diagnosis

Question 18 Explanation: 

Muscle weakness can lead to respiratory failure that will require emergency intervention and inability to swallow may lead to aspiration

Question 19

The nurse is teaching the female client with myasthenia gravis about the prevention of myasthenic and cholinergic crises. The nurse tells the client that this is most effectively done by:

A

Taking medications on time to maintain therapeutic blood levels

B

Doing muscle-strengthening exercises

C

Eating large, well-balanced meals

D

Doing all chores early in the day while less fatigued

Question 19 Explanation: 

Clients with myasthenia gravis are taught to space out activities over the day to conserve energy and restore muscle strength. Taking medications correctly to maintain blood levels that are not too low or too high is important. Muscle-strengthening exercises are not helpful and can fatigue the client. Overeating is a cause of exacerbation of symptoms, as is exposure to heat, crowds, erratic sleep habits, and emotional stress.

Question 20

Toy with a tentative diagnosis of myasthenia gravis is admitted for diagnostic make up. Myasthenia gravis can confirmed by:

A

A positive edrophonium (Tensilon) test

B

Brudzinski’s sign

C

A positive sweat chloride test

D

Kernigs sign

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Nursing Care Plan

Nursing Diagnosis

Ineffective Breathing Pattern related to neuromuscular weakness of the respiratory muscles and throat.

Desired outcomes

The patient will maintain an oxygen saturation of >92% and a respiratory rate of 12-20 with ADL’s.

Nursing Interventions

• Assess for signs of activity intolerance. Ask client to rate perceived exertion.
  • Rationale: Dyspnea on exertion, palpitations, headaches, or dizziness or patient states increased exertion level, are all signs of activity intolerance and decreased tissue oxygenation.
• Monitor pulse oximetry and report O2 saturation <92%.
  • Rationale: O2 sat of <92% indicates the need to supplement oxygen.
• Monitor the patients pulse oximetry every 4-6 hours.
  • Rationale: An O2 saturation of less than 92% may detect hypoxia and signals the need for supplemental oxygen.
• Encourage deep breathing exercises and administer oxygen if indicated
  • Rationale: Increases oxygen delivery to the body.

Nursing Diagnosis

Risk for Aspiration related to difficulty swallowing

Desired outcomes

Client is able to swallow independently without choking.  Able to maintain a patent airway.

Nursing Interventions

• Assess for signs of activity intolerance. Ask client to rate perceived exertion.
  • Rationale: Dyspnea on exertion, palpitations, headaches, or dizziness or patient states increased exertion level, are all signs of activity intolerance and decreased tissue oxygenation.
• Monitor pulse oximetry and report O2 saturation <92%.
  • Rationale: O2 sat of <92% indicates the need to supplement oxygen.

Nursing Diagnosis

Self-Care Deficit related to muscle weakness, general fatigue.

Desired outcomes

Patient is able to perform self-care activities independently and able to demonstrates ability to use adaptive devices for completion on ADL’s.