You are on page 1of 53

MYASTHENI

A GRAVIS

GROUP 4
MYASTHENIA GRAVIS
Introduction
01 Learning 05 Medications
Outcomes/Overview

Anatomy and 06 Nursing / Medical


02 Physiology Management
Parts involved and
affected in the disease Surgical Management
07 Pre-op and
03 Pathophysiology
Disease Process of
post-op care
Myasthenia Gravis

04 Clinical
Manifestations
Learning Outcomes
At the end of the discussion, the learners will be able to:

1. Understand the overview of the disorder in terms of: definition,


causes, risk factors, and diagnosis.
2. Identify the parts involved and affected in Myasthenia Gravis
3. Elaborate the disease process of the disorder
4. Identify the clinical manifestations of the disorder.
5. Recognize the medications indicated in the disorder.
6. Identify nursing and medical management that are necessary in
the care of the patient.
7. Recognize the nursing responsibilities during pre-op and post op
care in the surgical management of the disorder.
01 Introduction

Overview of the
disorder
Overview of the Disease

Definition Causes Risk Factors Diagnosis


What is Myasthenia Gravis?

● Myasthenia Gravis is a chronic neuromuscular


autoimmune disease characterized by weakness and rapid
fatigue of any of the muscles under your voluntary control. It's
caused by a breakdown in the normal communication between
nerves and muscles.

● There's no cure for myasthenia gravis, but treatment can help


relieve signs and symptoms, such as weakness of arm or leg
muscles, double vision, drooping eyelids, and difficulties with
speech, chewing, swallowing and breathing.

● Myasthenia gravis affects the voluntary muscles of the body,


especially those that control the eyes, mouth, throat and limbs.
CAUSES
● Antibodies
In myasthenia gravis, your immune
system produces antibodies that block or
destroy many of your muscles' receptor
sites for a neurotransmitter called
acetylcholine. With fewer receptor sites
available, your muscles receive fewer nerve
signals, resulting in weakness.
CAUSES
● Thymus gland
Researchers believe that the thymus gland
triggers or maintains the production of the
antibodies that block acetylcholine. Large in infancy,
the thymus gland is small in healthy adults. In some
adults with myasthenia gravis, however, the thymus
gland is abnormally large. Some people with
myasthenia gravis also have tumors of the thymus
gland (thymomas). Usually, thymomas aren't
cancerous (malignant), but they can become
cancerous.
CAUSES
● Other causes
Rarely, mothers with myasthenia gravis have
children who are born with myasthenia gravis
(neonatal myasthenia gravis). If treated promptly,
children generally recover within two months after
birth. Some children are born with a rare, hereditary
form of myasthenia gravis, called congenital
myasthenic syndrome.
RISK FACTORS
● LIFESTYLE RISK FACTORS
A 2018 Swedish study found that those with late-
onset disease had a more disadvantageous pattern
of lifestyle-related risk factors, and a higher rate of:
● Smoking
● Obesity
● Physical activity
● Insufficient diet (including a lower rate of fish
consumption)
RISK FACTORS
● GENERAL RISK FACTORS
General risk factors found to increase the chance of a
person getting MG include:
● Being a woman between the ages of 20 to 30
● Being a man between the ages of 60 to 70
● Having specific genetic markers called HLA-B8 or DR3
● Neonates (newborn infants) with mothers who have
abnormal antibodies that have entered the baby’s
body via the placenta during pregnancy
DIAGNOSIS
The doctor will perform a complete physical exam,
as well as take a detailed history of your symptoms.
They’ll also do a neurological exam. This may consist
of:
● checking your reflexes
● looking for muscle weakness
● checking for muscle tone
● making certain your eyes move properly
● testing sensation in different areas of your body
● testing motor functions, like touching your
finger to your nose
DIAGNOSIS
Other tests that can help your doctor diagnose the
condition include:

● Repetitive nerve stimulation test


● Blood testing for antibodies associated with MG
● Edrophonium (Tensilon) test: a drug called
Tensilon (or a placebo) is administered
intravenously, and you’re asked to perform
muscle movements under doctor observation
● Imaging of the chest using CT scans or MRI to
rule out a tumor
Anatomy and
02 Physiology
03 Pathophysiology
(Disease Process)
What voluntary muscles are
involved?
• Eyes: most likely will be the first sign something
is wrong
• Throat: Another common hoarse voice and
problems swallowing
• Face
• Arms/Legs
• Respiratory muscles for breathing (in severe
cases)
Key players involved
• Neuromuscular Junction
• Neurotransmitter: Acetylcholine
• Immune System: produces the anti-
bodies against the nicotinic receptors.
• Thymus gland
• Acetylcholinesterase: an enzyme that
breaks down acetycholine
PATHOPHYSIOLOGY
In myasthenia gravis the issue arises at
the neuromuscular junction. This is the
place where the motor neuron and muscle
fiber meet and the neurotransmitter
acetylcholine is released.
• At this junction are receptors on the
muscle fiber called nicotinic
acetylcholine receptors that become
stimulated when acetylcholine is
released and cause contraction of the
muscle fiber.
• In myasthenia gravis the nicotinic
acetylcholine receptors are being attacked by
antibodies the immune system has created
and are not working properly.

• These receptors can NOT access the release


of acetylcholine and cause muscle
contraction, so there is muscle weakness.
04 Clinical
Manifestations
“W E A K N E S S”
● Weakness (neck, face, arms, legs, hands)

● Eyelid drooping (one eye/both, PTOSIS)

● Appearance mask-like: no expression/sleepy

● Keeps choking or gagging when eating


(difficulty swallowing)
● No energy (very fatigued)

● Extraocular muscle involvement leading to


double vision (STRABISMUS)
● Slurred speech (voice may be hoarse/soft)

● Shortness of breath (can extend


to respiratory muscles)
05 Medications
Pharmacologic intervention consists
of two groups of medications

• Short-acting anticholinesterase
compounds

• Corticosteroids
Anticholinesterase
• The most effective anticholinesterase drugs
are pyridostigmine (mestinon) and
neostigmine (Prostigmin).

• Dosages are highly individualized, based on


physiologic response to the medication.

• The goal is to achieve the maximum benefit


(muscle strength and endurance) with the
fewest side effects (excessive salivation,
sweating, nausea diarrhea, abdominal
cramps, or tachycardia).
Corticosteriods (Prednisone)
● are directed toward reducing the levels of serum
Ach receptor antibodies. Corticosteriods may
temporarily worsen manifestations; however, this
is followed by gradual improvement in muscles
strength.

● After a peak of improvement is reached and


maintained for several weeks, the dosage of both
prednisone and anticholinesterase medication
may be gradually decreased. A low maintenance
dose of alternate-day precautions may be
effective for many months or years.

● Precautions with any steroid therapy are


important, including potassium supplements if
indicated and liberated use of antacids.
Corticosteriods (Prednisone)
● Potential complications of steroid use are
cataracts, hypertension, diabetes, fluid
retention, delayed wound healing, insomnia,
and osteoporosis. Other treatments include
azathioprine (Imuran) and cyclosporine
(Sandimmune), which reduce the level of
circulation ACh receptor antibodies, and
plasmapheresis and IVIG.
06 Medical &
Nursing
Management
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.
Nursing Management
● The patient is educated about strategies to conserve
energy.
● The patient is encouraged to apply for a handicapped
license plate to minimize walking and from space to
schedule activities to coincide with weak energy and
strength levels.
● Clients with MG are usually managed in an outpatient
setting, when clients are hospitalized for diagnosis or
during a crisis, the nursing management may be
pertinent.
Nursing Management
● Instruct client to sit upright when eating,
to swallow only when the chin is tipped
downward the chest, and never speak
while food is in the mouth.
● Provide client and family with information
about MG and its treatment.
● Explain how to recognize myasthenic and
cholinergic crisis and how to have a plan
to seek medical interventions, if
necessary.
07 Surgical
Management
(Pre-op &
Post-op care)
Thymectomy
● Thymectomy (surgical removal of the thymus gland) can produce
antigen-specific immunosuppression and result in clinical
improvement. It can decrease or eliminate the need for
medication. In one study 92% of post-thymectomy patients had
symptomatic improvement, with 50% of them no longer requiring
pharmacologic therapy (Wilkins & Bulkley, 1999).
Anatomically complete thymus Small intracapsular and intrathymic
gland in a myasthenia patient. thymoma.
A Thymectomy can be performed as an open surgery
or as a minimally invasive surgery. In open surgery,
your surgeon splits the central breastbone (sternum)
to open your chest and remove your thymus gland.

Minimally invasive surgery to remove the thymus


gland uses smaller incisions. It might also involve:

Video-Assisted Thymectomy
Robot-Assisted Thymectomy
Video Assisted Thymectomy
● Video-assisted thymectomy. In one
form of this surgery, surgeons make a
small incision in your neck or a few
small incisions in the side of your
chest. They then use a long, thin
camera (video endoscope) and small
instruments to see and remove the
thymus gland.
Robot Assisted Thymectomy
● In this form of thymectomy,
surgeons make several small
incisions in the side of your chest
and remove the thymus gland
using a robotic system, which
includes a camera arm and
mechanical arms.
Considerations for Anesthesia Management

● Anesthesia Technique
● General Anesthesia
● Muscle Relaxants
● Neuromuscular Blockade Monitoring
● Sugammadex.
PREOPERATIVE CONSIDERATIONS
• In reviewing the care plan with the patient, the provider must
underline that physical stress such as that associated with
surgery can exacerbate the disease process, but that the
disease process generally returns to baseline.

• If the surgery is elective, a time when the patient is in a stable


phase should be chosen for optimal surgery and anesthesia
outcomes.

• As a rule, most immunosuppressants will not interact with


anesthesia.
PREOPERATIVE CONSIDERATIONS

• If the patient is experiencing an acute exacerbation or having


a myasthenic crisis but requires emergent surgery, every effort
should be made to optimize the patient’s condition first.

• Optimization includes plasmapheresis, immunosuppressive


therapy such as exogenous corticosteroids, and discussing
postoperative ventilation therapy with the patient.
PREOPERATIVE CONSIDERATIONS
• This practice should be used with caution as the results of one
study indicated that patients with MG who did not take their
morning dose of pyridostigmine on the day of surgery were at
higher risk of development of respiratory distress.

• Avoidance of respiratory depression should also be a priority


for the anesthesia provider, and premedication with sedatives
or opioids should not be routine practice, as it is extremely
difficult to predict which patients with MG will undergo
postoperative myasthenic crisis (PMC).
INTRAOPERATIVE CONSIDERATIONS
• Educate patient and family that physical stress associated with
surgery can temporarily exacerbate disease process.

• If surgery is elective, it should be performed during a stable


phase of the disease process.

• If surgery is emergent and patient’s condition is unstable, every


effort should be made to optimize patient’s condition first.

• Discontinuation of pyridostigmine treatment before surgery is


debatable; it allows for quicker onset and smaller dosing of
nondepolarizing NMBAs but also increases risk of respiratory
distress.
INTRAOPERATIVE CONSIDERATIONS
• In general, most immunosuppressants do not
interact with anesthesia.

• Azathioprine therapy should be discontinued, as it


prolongs effect of succinylcholine and inhibiting
effects of nondepolarizing NMBAs.

• Avoid premedication with sedatives or opioids as


routine practice because of CNS depression effects.
 
POSTOPERATIVE CONSIDERATIONS

• It is important to recognize that patients with MG are


undergoing an increasing number of surgeries that may be
nonrelated to MG (thymectomy).

• Procedures were matched by propensity scoring, and overall,


surgical patients with MG had higher risk of postoperative
complications (pneumonia, septicemia, and intensive care unit
[ICU] admission), longer hospitalizations, and higher medical
expenditures.
POSTOPERATIVE CONSIDERATIONS

• Postoperative considerations for patients with MG include


specific extubation criteria, avoidance of ICU admission, and
analgesia.

• Routine ICU admission with mechanical ventilation should be


avoided if at all possible and is not recommended because of
the increased risk of respiratory failure and airway-associated
complications.
POSTOPERATIVE CONSIDERATIONS
• Opioid analgesics produce respiratory depressant
effects that could be dangerous in the
postoperative period, especially if superimposed on
muscle weakness, but it is equally important to
avoid excessive surgical stress (pain), which could
precipitate a myasthenic crisis. Therefore, optimal
pain management is crucial.
THANK YOU FOR
LISTENING!
Catarungan, Kristine

Corsino, John Kenneth

De Andres, Carizza

De Asis, Santanah Fameh

Diega, Jullina

BS NURSING-III

You might also like