MYASTHENIC PATIENT IN CRISIS:
Respiratory Care Management
INTRODUCTION
Myasthenia gravis is a chronic
autoimmune neuromuscular disease and the
most common disorder of the neuromuscular
junction. The term myasthenia gravis, which
is Latin and Greek in origin means “ grave ,
or serious , muscle weakness The disorder
interferes with the chemical transmission of
acetylcholine ( ACH )between the axonal
terminal and the receptor sites of voluntary
muscle. Acetylcholine is a neurotransmitter
use to communicate information and it bind
to acetylcholine receptors to activates the
muscle and cause a muscle contraction but in
the disease condition this normal binding of
acetylcholine to the receptors is block by the
antibodies (immune proteins) that causes
weakness in the skeletal muscle, which are
responsible for breathing and moving parts of
the body including the arms and legs.
There are two clinical types of
myasthenia gravis: ocular and generalized. In
ocular myasthenia gravis, the muscle
weakness is limited to the eyelids and
extraocular muscle. In generalized type of
myasthenia gravis the muscle weakness
involves a variable combination of muscle of
the mouth and throat responsible for speech
and swallowing (bulbar muscle), limbs and
respiratory muscle.
The hallmark of myasthenia gravis is
a chronic muscle fatigue. The muscle become
progressively weaker after periods of activity
and improves after period of rest. The degree
of muscle weakness involved varies greatly
among individuals ranging from localized
form limited to eye muscle, to a severe or
generalized form in which many muscle,
sometimes including those that control
breathing. Sign and symptoms include facial
weakness: ptosis ( drooping of the eyelid;
diplopia (double vision ) are the first
noticeable symptoms: opthalmoplegia (
paralysis or weakness of one or more of the
muscle that control eye movement, difficulty
in breathing , speaking, chewing and
swallowing; unstable gait and weakness in
the arms, hands, fingers and legs. When the
diaphragm is involved, ventilatory failure can
develop producing what called “myasthenic
crisis “ which impair deep breathing, and
coughing resulting to mucous accumulation,
airway obstruction , alveolar consolidation
and atelectasis.
Myasthenia gravis affects both men
and women and occurs across all racial and
ethnic groups. It most commonly impacts
young adult women (under 40) and older men
(over 60) but can occur at any age including
child hood. Myasthenia gravis is considered
as an autoimmune disease because the
immune system which normally protects the
body from foreign organism mistakenly
attack itself. Developing a myasthenia gravis
is associated with abnormality with thymus
and genes. But the exact etiology of the
disease remains unknown and needs further
research and studies.
CASE REPORT
A 56 year old Filipino Male is a High
School teacher. He is an active badminton
coach and spends weekdays on his farm. He
lives in a bungalow house atleast 5km away
from the main road but with good ventilation
and with drinking water refilled through a
water refilling station. He is a smoker
consuming ½ packs per day and occasionally
drinks gin with an average of 2 bottles /week..
He is also allergic to crab, fish and shrimp.
Five days prior to admission, the
patient was teaching when suddenly he felt
weak and noticed slurring of speech with
slightly increased salivation. He visited a
physician to which it was said to be due to
acidity . He was prescribed medicine which
he could not recall but did not help relieve the
symptoms. The next day, patient noticed to
have progressive symptoms now associated
with drooping of his eyelids, drooling saliva
and sleepless night. He would eat half of what
he normally eats due to difficulty masticating
and later dysphagia. Concerned, the patient
consulted a different physician to which he
was diagnosed as CVD, Infarct and
subsequently admitted. While being managed
on a provincial hospital, physician suggested
he undergo CT scan that later revealed
normal result. Due to dysphagia and
difficulty in masticating, NGT was suggested
but the patient refused thus, having him feed
intravenously. The next consecutive days
prior to admission in the institution, patients
signs and symptoms progresses to having
diplopia, tingling sensations in the buccal
area and limited facial expression. Having a
normal CT scan report, he was referred to a
HEENT specialist which also shows normal
result. Over few hours, the patient’s physical
status declined progressively having the
family decide to proceed to this institution.
Upon receiving the patient, he was
interviewed for any conditions that may be
related to his condition that later revealed the
patient has been taking anticonvulsant
therapy at a young age for his seizures, was
hospitalized due to Malaria for 2 weeks year
2015 and had an accident falling from riding
a carabao hitting the right side of his head.
Patient was then asked to count continuously,
at the count of 37and above patient’s speed is
deteriorating and speech is slurring.
Following the test, patient was also given
Neostigmine that has improved his muscle
strength for ten minutes . A diagnosis of
Myasthenia Gravis was written on the
patient’s chart. A pyridostigmine 60mg TID
and a prednisone 50mg BID was ordered to
address muscle weakness, proceeded with
NGT insertion and vital signs recorded
accordingly.
As he was being accompanied to
NDCH for MRI, he suddenly went to Cardiac
arrest due to ARF: Aspiration Pneumonia and
was revived for 5 minutes. ABG was done
pre-hooking with results of: Normal Acid
Base Balance with overcorrected
oxygenatiom @ 80% FiO2 via BVM. Hooked
to Mechanical Ventilator with parameters of
BUR: 20, Vt: 320, I:E-1:3, FiO2: 60%. ABG
was taken after 30 minutes resulting to
Uncompensated Respiratory Alkalosis with
uncorrected oxygenation at 60% FiO2
wherethen FiO2 was increased to 80%.
Ceftriaxone 2mg via IV line, Clindamycin
300mg/tab q6 and salbutamol 2.5mg/2.5ml 1
neb q12 was ordered by his pulmonologist.
Later, his MRI (stroke Protocol) result
reveals no acute infarct, acute intracranial
haemorrhage, focal mass and edema ,no
white matter bright signals, both cerebral
hemispheres, which may reflect
microvascular ischemic changes, areas of
degenerative demyelination and/or gliosis
and no brain herniation and hydrocephalus.
He was transferred to the intensive
care unit for close monitoring. Gram’s
staining was done having a result that shows
occasional gram positive cocci seen in single
pairs with occasional gram negative bacilli
with moderate pus cells and few epithelial
cells noted. He was ordered for Chest X-ray
after intubation that revealed hazy opacities
on right lower lung suggestive of pneumonia
with the ET tube in place. Complete Blood
Count was simultaneously done showing
increase WBC and neutrophilic segment.
Upon assessment, with the present
manifestations of myasthenia gravis, patient
exhibits normal vesicular breath sounds on
the left lung while diminished breath sound is
heard over the right lung with thin, greenish
sputum being suctioned. Two days after
hooking, FiO2 is decreased to 60%, 20 gtts is
incorporated to Salbutamol neb q12, with
increasing BP furosemide was given 20 mg
via IV line. Next day, FiO2 was titrated to 40
%, and RR decreased to 12, CPT was done.
Patient was found unremarkable that had him
shift from AC to SIMV with BUR of 14, PSV
of 13 and FiO2 of 30%. On his fourth day on
MV, patient status has been improving thus,
he was weaned by decreasing BUR by 2
every two hours until 6, pressure support by
2 every 2 hours until 7. Later that afternoon,
he was shifted to spontaneous mode, psv of 7
, 30% FiO2. ABG was taken revealing
Partially compensated Respiratory Alkalosis
with uncorrected oxygenation @ 30% FiO2
via MV with SaO2 of 96.3%. Chest X-ray
was also done showing interval clearing of
the right lower lung pneumonic process.
The next day, another ABG was taken
now revealing a result of uncompensated
respiratory alkalosis with corrected
oxygenation @ 30% FiO2 via MV. RSBI was
taken with results of 35.85, an RR of 19bpm
and Vt of 0.53lpm.
Ceftriaxone and Clindamycin was to
complete for 7 days. Since patient was
unremarkable, he was hooked to t-piece @
4lpm then extubated after an hour of
observation and good response. After
extubation, he was given salbutamol +
ipratropium 2.5mg/2.5ml and budesonide
250mcg/ml. From salbutamol 2.5mg/ml plus
20 gtts MRS q12, and CPT ,it was the revised
to salbutamol 2.5mg/ml 1 neb plus 10 gtts
MRS q12 and CPT, he was suctioned as
needed via nasal route in precaution for
aspiration and distressed. He was managed to
ICU for another 5 days with improving status
and transferred to wards.
DISCUSSION
Myasthenia Gravis’ cause is not yet
completely understood but involves:
1. Autoimmine Antibodies (IgG)
 MG is related to ACh (Acetylcholine)
receptor antibodies (IgG antibodies)
that blocks the nerve impulse
transmissions at the neuromuscular
junction.
 This IgG antibodies disrupt the
chemical transmission of Ach at the
neuromuscular junction by blocking
the ACh from the receptor sites of the
 muscular cell, and by destroying the
receptor sites.
 Making it impossible for the ACh to
bind to the receptor site causing
absence of muscle contractility.
2. Thymus Gland Disorders
 It is believed to produce antibodies
damaging acetylcholine receptors.
3. Genetics
 A type of MG caused by genetic
defect or a congenital syndrome
known as congenital myasthenic
syndrome is said to be inherited by
children born to mothers with MG
during pregnancy with some of the
antibodies and exhibits some
manifestations of MG few months
after they are born.
PRODUCTION OF ANTI BODIES (IgG)
BLOCKAGE OF ACETYLCHOLINE RECEPTORS
DECREASED NUMBER OF ACHR
FAILURE OF NEUROTRANSMITTER TO ATTACH TO
THE ACHR
LOSS OF MUSCLE FUNCTION OR PRESENCE OF
MUSCLE WEAKNESS
In the patient’s medical history, there is no
abnormalities of the thymus and thyroid
gland that may have caused the occurrence of
Myasthenia Gravis, however the correlation
of the seizure and anticonvulsant drugs to
unmasking MG is being studied. In some
journals relating to this event, it was found
that in 1916, Fearnsides described a woman
with epilepsy developd MG 3 years after the
onset of epilepsy, according to Pages and
Passouant 1953 a male patient has both
epilepsy and MG, Hoefer et. al 1958,
reported 8 MG patients in association with
epilepsy and seizure disorders, in 1964,
Norris et. al reported 3 epileptic patients with
an anticonvulsant drug overdose developing
neuromuscular transmission disorder, proved
by an expirement of demonstrating the toxic
effect of an anticonvulsant therapy
specifically phenytoin in rats and in 2003, Su
et al reported 4 MG patients who have been
taking anticonvulsant therapy has developed
MG. Although, the prevalence of
anticonvulsant therapy precipitating MG has
few research and studies, some studies shows
that variety of anticonvulsant therapy have
been associated in MG. To support the
hypothesis of the coexistence of the two
disorders, further studies should be done.
As was mentioned, Myasthenia Crisis
is a life threatening complication of MG. It
may be precipitated by variety of factors but
most often of the concurrent causes involves
pulmonary. In the patient case, the
Respiratory failure is caused by aspiration of
saliva.
RESPIRATORY CARE MANAGEMENT
Oxygen Therapy
High flow or low flow device can be used
to give oxygen therapy needed by the patient.
It is used to treat Hypoxemia decrease work
of breathing and decrease myocardial work.
(Des Jardins.,2016)
 Flow rate : 60L/min
Bronchopulmonary hygiene therapy PEEP: 5 cmH2O
Because of excessive mucous production
and accumulation associated with
myasthenia gravis, bronchopulmonary Nutrition and other issues during
hygiene therapy enhance the mobilization of mechanical ventilation
bronchial secretions.
In patients with Respiratory Care for
Myasthenic Crisis, low carbohydrate feeds
Lung Expansion therapy are the preferred solution (Varelas et al.,
Use to maintain the patency of the lungs
2002). In addition to the aforementioned
and to prevent developing atelectasis caused
measures, abnormal laboratory values that
by myasthenia gravis.
could affect muscle strength should also be
corrected. Potassium, magnesium, and
Aerosol Therapy/ Bronchodilator therapy phosphate depletion can all exacerbate
Bronchodilators may be useful in
myasthenic crisis and should be replete.
maintaining airway patency and overcoming
Anemia can also increase weakness, and
bronchospasm. As well as the mucolytic
several experts recommend transfusions
agent that helps loosen the secretion to be
when hematocrit values are under 30%
able to remove out of the airways.
(Kirmani et al., 2004; Ahmed et al., 2005).
(Szathmáry et al., 1981; Liggett et al., 1988)
Prophylaxis for deep vein thromboses, such
as administration of subcutaneous heparin
Mechanical Ventilation sodium or use of pneumatic compression
MV may be needed to provide and support
boots, is recommended.
alveolar gas exchange and eventually return
patient to spontaneous breathing.
Large tidal volumes of 10 mL/kg, along with RECOMMENDATION
pressure support of 5 to 15 cm H2O and
GOALS
positive end-expiratory pressure (PEEP) of 5
to 15 cm H2O, are encouraged to limit
 Minimize risk of aspiration
atelectasis, provided peak airway pressures
(prophylaxis, RSI)
can be maintained below 40 cm H2O
(Kirmani et al., 2004; Varelas et al., 2002).  Minimize risk of perioperative
The degree of support required is patient respiratory failure (judicious NMBs
dependent and should be adjusted based on & opioids) & anticipate need for
ABG analysis. Inspired gas humidity should post-op ventilation
be around 80% at 37°C. Various modalities,  Minimize risk of myasthenic or
cholinergic crisis
IDEAL PARAMETERS:  Optimize neuromuscular function
VC-AC or VC-SIMV  Prolong quality of life
RR 10-12 breaths per minute
I:E : 1:2
FiO2: 60- 80%
SUBJECTIVE OBJECTIVE ASSESSMENT PLAN IMPLEMENTATION EVALUATION
“madi eti GS: Patient is Sedate Patient DAY 2
pinagsao’k” restless , on a
semi-fowler’s
position
IPPA
Inspection:
Ptosis Inability to Acetylcholinesterase Pyridostigmine Ability to
Opthalmoplegia control muscle Inhibitors 180-540 mg Q12 control muscle
Limited facial (presenting (+)
expression symptoms of Opthalmoplegia
(inability to MG)
smile)
Drooling Increased WOB
Aerosol Therapy Salbutamol
Supraclavicular Nebulization TID No retractions
retraction
Presence of
secretion
Auscultation: Bronchopulmonary CPT and Suction the
Fine crackles on hygiene patient every after Decrease
right middle to nebulization intensity of
lower lobe of the crackles at right
lungs middle to lower
lobe of the
lungs
LABORATORY
and
DIAGNOSTIC
TEST

Gram’s Stain:
Smear shows Presence of Aerosol therapy and Salbutamol + 20gtts. No current
occasional gram infection due to bronchopulmonary MRS TID and result
positive cocci inability hygiene and suctioning every after
seen in single manage the Antibiotics nebulization,
pairs with airway Ceftriaxone and
occasional gram Clindamycin
negative bacilli.
Moderate pus
cells and few
epithelial cells
noted.
Chest
Radiograph
Day 1 Suggestive of Antibiotics and Ceftriaxone Q12 and Interval
Pneumonia adding parameter PEEP of 5 cmH2o on clearing of
MV opacities seen at
Hazy opacities on the right lower
Right Lower lung
Lung
ET in Place
Clinical
Chemistry
Creatinine: 0.77
mg/dL
Potassium: 3.73 Due to diuretics
mmol/L given
Sodium:
164.4mmol/L
FBS: 150.06
mg/dl
Total Cholesterol:
258.48 mg/dl

CBC:
Day 1
WBC: 14.29 x Day 2
109/L Presence of Antibiotics Ceftriaxone WBC: 10.29 x
Neutrophilic bacterial 1 to 2 g via IV Q12 109/L
Segment of 0.86 infection Neutrophilic
segment 0.78
ABG:
Normal acid base
balance with Decrease the flow of Decrease to 60% Normal Acid
overcorrected Oxygen Base Balance
oxygenation with corrected
@80% via BVM Oxygenation @
60% FiO2 via
MV