MYASTHENIA GRAVIS

>Is a neuromuscular disorder characterized by failure of transmission of nerve impulses at the

myoneural junction.
>MG may be caused by decreased functioning of acetylcholine receptor sites.
>Failure of transmission to skeletal muscles and voluntary muscles in the body
>Associated with autoimmunity and thymus gland anomalies (thymoma – tumor in the thymus gland)

Risk Factors:

> 20-30 years old for women

> 60-70 years old for men
> Heredity

Assessment:

 Vision disturbances: Diplopia and Ptosis from ocular weakness

 Masklike facial expression form involvement of facial muscles
 Dysarthia and dysphagia from weakness of laryngeal muscles and pharyngeal muscles
 Extreme muscular weakness and easy fatigability with repetitive activity and speech
 Possible respiratory difficulty.

Diagnostic Evaluation:

 Serum test for Ach receptor antibodies, which is positive in 90% of patients
 Tensilon test (Endrophonium) : short acting cholinergic;
Positive Results: Definitive conclusion for MG. Muscle strength is improve, but muscle
weakness returns within 3 -5 minutes.
 Electrophysiologic Test: shows decremental response to repetitive nerve stimulation
 CT scan: reveals hyperplasia of the thymus, which is thought to initiate the autoimmune
response.

Medical Management:

Pharmacological:

> Acetylcholinesterase inhibitors/Cholinesterase Inhibitor

-Prostigmin (Neostigmine)
-Mestinon (Pyridostigmine)
-Mytelase (Ambenomium)

- These medications transmit neuromuscular impulses by preventing the destruction of Ach.

Therefore, there is increased muscle strength.

Nursing Interventions for Achterase Inhibitor

1. Monitor improvement of muscle strength and respirations.
2. Observe the client for signs and symptoms of cholinergic crisis caused by overdosing of the drug –
muscle weakness, increased salivation, sweating, tearing and miosis
3. Have readily available on antidote for cholinergic crisis (Atropine SO4)
4. Encourage the client to wear Medic Alert bracelet that indicates the health problem and the drug
taken
5. Instruct the client to take the medication before meals and in the morning for best drug absorption
6. Observe and report possible side effects and adverse reactions:
- N & V, diarrhea, abdominal cramps
-Increased salivation
-Tearing
-Miosis
-Possible hypertension
7. Administer drugs on time. To prevent myasthenic crisis
> Glucocorticoids. For anti-inflammatory and immunosuppressive effects
>Antacids. To prevent GI upset due to Glucocorticoids

Surgical Approach:

-Thymectomy (surgical removal of the thymus gland). 25% of people with MG have been found to
have thymoma. This surgery achieves remission for 5 to 10 years.

Other Approach:

Plasmapherisis: separation of Ach receptor antibodies from plasma.

Nursing Responsibilities:

>Monitor the patient’s RR, use of accessory muscles and O2 sat. to watch for possible respiratory
failure related to myasthenic or cholinergic crisis.
>Be alert for signs of an impending crisis:
-Sudden respiratory distress
-Signs of dysphagia, dysarthia, Ptosis and Diplopia
-Tachycardia. Anxiety
-Rapidly increasing weakness of extremities and trunk
-Monitor the patient’s responses to drug therapy
-Assess gag reflex and ability to swallow before giving anything PO
-Give cold water before starting feeding. To promote contractions
-Administer drugs on time
-Prevent infections
-Promote rest and comfort

Supportive Care
> Administer medications so their peak effect coincides with meals or essential activities
>Help the patient develop throughout the day to minimize fatigue
>Allow for rest periods throughout the day to minimize fatigue
> Provide assistive devices to help patient perform ADLs despite weakness
>For Diplopia, provide an eye patch to use n alternate eye to minimize risk of tripping and falling.
>To avoid aspiration
- Teach the patient to position the head in a slight ly flexed position to protect the airway during
eating
- Have a suction available that the patient can operate
- If the patient in crisis or has impaired swallowing, administer IV fluids and foods through NGT
-Elevate head after feeding
-If patient is on a mechanical ventilator, provide frequent suction, assess breath sounds and check
chest radiograph reports
> Show the patient how to cup chin in hands to support lower jaw to assist with speech
>If speech is severely affected, encourage the patient to use an alternate communication method,
such as flash cards or a letter board.

Education and Health Maintenance

>Instruct the patient and family about the symptoms of Myasthenic Crisis
>Teach the patient ways to prevent crisis and aggravation of symptoms
-Avoid exposure to colds and other infections
-Avoid excessive heat and cold
-Tell patient the patient to inform the dentist of condition because use of procaine (Novocaine) is not
well tolerated and may provoke crisis.
-Avoid emotional upset
>Teach the patient and family about the use of home suction
>Review the peak times of medications and how to schedule activity for best results
>Stress the importance of scheduled rest periods to avoid fatigue
>Encourage the patient to wear a medical alert bracelet
>Refer the patient and family to a support group such as www.myasthenia.org
Myasthenic Crisis

- is caused by undermedication or delayed medication

-clinical manifestations of Myasthenic Crisis is caused by undermedication or delayed medication
>Sudden rise in BP due to hypoxia
>Increase HR
>Severe respiratory distress and cyanosis
>Absent of cough and swallowing reflex
>Increased secretions, increased diaphoresis and increase lacrimation
>Restlessness and dysarthria
>Bowel and bladder incontinence

Interventions for MC are as follows:

-Increased doses of Cholinergics as long as the client responds positively to Tensilon
-Possible mechanical ventilation of respiratory muscle paralysis is acute.

Cholinergic Crisis

> Weakness with difficulty swallowing , chewing, speaking and breathing

>Apprehension, N & V
>Abdominal cramps and diarrhea
>Increased salivation
>Diaphoresis, lacrimation, fasciculations and blurred vision

Interventions for CC are as follows

-Discontinue cholinergic drugs until cholinergic effects decrease

-Provide adequate ventilation support
-Give Atropine SO4 as ordered

The followingshould be avoided:

-Muscle relaxants
-Barbiturates
-Morphine SO4
-Tranquilizers
- Neomycin

These drugs potentiate muscle weakness due to effect on neuromuscular junction.