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Au Di Minor Case Study Myasthenia Gravis
Au Di Minor Case Study Myasthenia Gravis
Au Di Minor Case Study Myasthenia Gravis
Zachary Siemieniak
Andrews University Dietetic Internship
April 26th, 2018
Siemieniak 2
Introduction
J.J., a 23-year-old female, presents to the emergency department on 4/8/18 with chief
complaints of oral swelling, difficulty swallowing, diplopia, and generalized weakness. Patient
with a height of 64 inches (5' 4"), weight of 59.9 kg (132 lbs), and ideal body weight of 54.5 kg
(120 lbs). Her BMI was 22.66 kg/m², which indicates a normal weight for height. J.J was
diagnosed with myasthenia gravis in 2014, and is currently following with Dr. Gallagher,
Neurologist at the University of Michigan. She complains of a "flare up" of her myasthenia
gravis. Symptoms includes double visions, muffled speech, and dysphagia.
Patient states symptoms started last Thursday (4/5/18), explaining, "First it was my eyes,
starting to get blurred, then double vision and has progressively gotten worse," and how she has
had this kind of swollen throat/choking feeling (onset of event). Patient states that the muscles in
her throat felt weak starting last Friday and noticed it as she was having trouble swallowing both
solids and liquids. No gagging or choking. On 4/7/18, she states she started having shortness of
breath that comes and goes. Sometime since Thursday patient and mother also noted slurred
speech. “Feels like there is something in her lips, tongue and throat, that when she says the letter
‘B’ it sounds like an ‘M’. States she is not talking normally. States her last flare up was end of
January, and she called her Neurologist; she was on Prednisone 10 mg at the time and was told to
take 3 tablets daily. Symptoms went away in a week and was on Prednisone 30 mg daily since
then according to patient. She feels it is worse at this time. She was hospitalized earlier in
November 2017 for myasthenia crisis. Symptoms are increasing over the last 3 days.
This particular patient was chosen as the topic of a minor case study because of her
incredibly unique disease state, one that is not commonly taught in nutrition related courses.
Myasthenia Gravis is a rare chronic autoimmune disease marked by muscular weakness without
atrophy and caused by a defect in the action of acetylcholine at neuromuscular junctions. Many
of its implications can affect the nutritional status of the individual dealing with said disease. The
study began on 4/8/18, and ended on 4/12/18. The focus of this study is to review the diagnosis,
treatment and physiology of Myasthenia Gravis, as well as provide current medical nutrition
therapy recommendations for further management of Myasthenia gravis in affected individuals.
Siemieniak 3
Social history
Obtaining social and dietary history from J.J. was difficult as she complained of muffled
speech and having extreme fatigue of her jaw muscles (needed for speaking). She is single, has
no children, with occupation and family responsibilities unknown. Standards of living, and
religious affiliation were not assessed. Patient states that she will be going to her mother’s
residence upon discharge, which is a ranch style home. She normally lives alone. J.J. does not
smoke/use smokeless tobacco, or consume alcohol. She is otherwise a normally healthy woman
of her age, with an acute exacerbation of myasthenia gravis.
The rationale for thymectomy relies on the central role of the thymus. Despite the lack of
controlled studies, thymectomy is recommended as an option to improve the clinical outcome or
promote complete remission.1 New video thoracoscopic techniques have been developed to offer
the maximal surgical approach with the minimal invasiveness and hence patient tolerability.1
Patient does not have extensive past medical history with many of her previous
admissions being with another hospital system, mainly the University of Michigan Hospital in
Ann Arbor, MI. Patient states her last flare up was at the end of January, where she called
her Neurologist; she was on Prednisone 10 mg at the time and was told to take 3 tablets daily.
Symptoms went away in a week and was on Prednisone 30 mg daily since then according to
patient. She feels it is worse this time. She was hospitalized earlier in November 2017 for
myasthenia crisis of a similar severity. She follows Dr. Gallagher in Ann Arbor, University of
Michigan, Neurologist. J.J states she may have last seen him in February 2018. J.J. mentions her
next follow up is sometime in May 2018.
Patient has been taking Prednisone 30 mg daily since February. Reports she did not
tolerate Azathioprine. Still on Mestinon 60 mg QID. On Cyclosporine 100 mg daily, prescribed
by doctors in Ann Arbor; started in November at 50 mg daily for 1 week and 100 mg daily after.
States she has been taking her pills, and she “just crushes them.” She denies fever, chills, cough,
CP, wheezing, abdominal pain, nausea/vomiting, diarrhea, constipation. Denies any problems
with controlling secretions. States she has double vision, frontal headache, on and off shortness
of breath. Legs feels weak bilaterally. Facial weakness as well as eyelids. Presenting vital signs
were stable. Initial laboratory work-up was largely unremarkable. Hospitalist service asked to
admit for further work up and management of patient's myasthenia gravis, possible flare up.
On 4/9/18, patient continued to complain of double vision, slurred speech, and left eyelid
drooping. She reports her breathing and leg weakness have been better compared with yesterday.
Patient states she has experienced decreasing appetite for the past few weeks. She only has small
bites of food because she is afraid of "tiring her chewing muscles." Denies any fever, chills,
shortness of breath, chest pain, abdominal pain, or any urinary symptoms.
Per Dr. Ward, Neurologist at U of M, “the wrong prescription could put this patient into
respiratory failure. Please look at the Myasthenia Gravis website for a list of potentially
dangerous medications for this individual." Acetylcholine receptor Ab (4/10/17) revealed
equivocal result. MRI completed on 4/11/17: results proving negative for thymoma.
Siemieniak 5
Per neurological examination, J.J. is fully alert and oriented to person, place, and time;
patient is fully aware of her environment; her speech has a nasal character to it and language
functions are all preserved. Pupils are equal in size, reactive to light & accommodation; no APD;
visual fields are full to confrontational testing in each eye. Perception to light touch and/or
temperature sense is preserved bilaterally. Muscles of facial expression - she has a moderate
degree of bilateral facial weakness involving the orbicularis oculi musculature bilaterally as well
as the lower muscles of facial expression. Hearing preserved bilaterally with no observable
palatal weakness. Recent imaging studies found no significant issues.
Per neurology the impression of the exam was as follows: Myasthenia gravis
exacerbation manifested by isolated ocular bulbar weakness resulting in clinical features of
double vision, dysphagia, and speech change (nasal). There is no evidence of expiratory muscle
involvement and thus she is not considered to be in myasthenic crises. However, with the
dysphagia and potential risk of aspiration, rapid immunomodulatory treatment is necessary.
4/10/18: Patient complains of double vision since Thursday (4/5/2018) with no change
since admission. Denies difficult breathing or shortness of breath. Denies any difficulty
swallowing. Encouraged to call for assist before OOB and when needed. Call light within reach.
No distress noted at this time.
4/11/18 Family asleep at BS. Hand grasps equal, speech slurred, follows all commands.
Denies any numbness or tingling in any extremity. Verbalized double vision, which she has had
since 4/5/2018, is improving. Denies shortness of breath. Denies any difficulty swallowing.
Speech therapy followed up with patient, findings are as follows: Patient received french
toast, eggs with cheese, Boost and apple juice. She was able to eat her food for 20 mins with no
complaints of feeling tired, but then was noted to slow rate of intake. She then stated that she
was beginning to get tired. Patient was noted to begin with her eggs with cheese (high protein,
high calorie) and french toast. She received 2 glasses of apple juice, but drank her Boost rather
than the juices while the SLP was in the room. No swallow difficulties were demonstrated
during this meal. Patient demonstrated awareness of SLP's recommendations. No further ST
follow up indicated at this time.
Family members (step-father and mother) states that they want the patient to be
transferred to U of M for further management because they believe "U of M is more familiar
with her condition" and "she got better right after being transferred to U of M last time." They
were frustrated about the slow improvement of her condition. They are questioning if we are
using the same medications and checking her respiratory status often enough. It was politely and
calmly explained to the patient and family that IVIG and medications may take time to see the
improvement. It will be not safe for her to be discharged and taken to U of M on their own. Dr.
Petrilli at U of M accepted patient and it could take up to two days for the bed availability.
Family and patient updated and they agree on staying at our facility until the transfer. MRI chest
with and without contrast (4/11/17): No findings to suggest a thymoma. Neurology is following
(Dr. Wasielewski), appreciated for recommendations. Continue Prednisone 50 mg daily,
cyclosporine 100 mg daily, Mestinon 60 mg TID, and IVIG for total of 5 days.
4/12/18 Transfer to University of Michigan for further evaluation. Pt placed on BiPAP
for increased work of breathing - respiratory rate improved from 40-50 to 20s. NIF remains -50,
ABG without significant hypercarbia. CT neck performed to evaluate for other causes of her
Siemieniak 7
dyspnea, no obvious airway compromise on my review (and no stridor), though final radiology
read is pending. Family updated several times today at bedside.
To restate and summarize her case, J.J. is a 23 yo F diagnosed with myasthenia gravis in
2014, followed in the past by Dr. Ward (per notes) and is currently following with Dr. Gallagher,
Neurologist at U of M. She complains of a "flare up" of her myasthenia gravis. Symptoms
includes double visions, muffled speech, and dysphagia. Neurology was consulted, IVIG (30 g)
was given for 4 days, along with Prednisone 50 mg daily (increased to 30 mg daily),
pyridostigmine 60 mg TID, and cyclosporine 100 mg daily. negative inspiratory force and forced
vital capacity have been checked every 4 o 8 hours. Her condition was slowly improving, and her
right soft palate appears weaker than left. CT scan of neck was ordered (result pending - had a
CD copy). Toward the end of hospitalization, patient started to have tachypnea and increasing
working of breathing although NIF have been ranging between 40s and 50s, mostly 50s. BiPAP
was placed and her last set of ABG was 7.43/42/151/100 (pH/pCO2/O2/O2 saturation). Patient
appears to be comfortable after being placed on BiPAP. Patient was transferred to U of M per
Dr. Williamson and Dr. Morgan for further management of Myasthenia gravis exacerbation.
There are two types of medications used to treat MG. One group—anticholinesterases—
temporarily relieves the symptoms of MG.2 Another group—immunosuppressants—attacks the
disease at its source.2 By suppressing the body’s immune system, these drugs stop the body from
damaging the neuromuscular junction in the first place.2
Immunosuppressants help prevent the body from producing the harmful antibodies that
cause MG weakness in the first place.2 At the same time, they also reduce the body’s production
of good antibodies—which makes you more susceptible to infection and other diseases.2 While
one is taking immunosuppressants, it is imperative to avoid people with contagious diseases. One
may need to avoid crowds and wash hands frequently.
There are a number of other medications used to treat MG. These include azathioprine
(Imuran), mycophenylate mofetil (CellCept), tacrolimus (Prograf), methotrexate, cyclosporine
(Sandimmune, Neoral), and cyclophosphamide (Cytoxan, Neosar).2 If one of these medications
helps a particular myasthenic, its use can eliminate the need for prednisone, or allow a lowering
of its dose.2 These medications are complicated to administer. They can take months to become
effective, the dosage varies from person to person, and each drug has its own side effects and
drug interactions.2
One of the newer therapies used to treat myasthenia gravis that is resistant to traditional
approaches is the monoclonal antibody rituximab (Rituxan).2
Siemieniak 9
A thymectomy is the surgical removal of the thymus gland. The thymus—located in the
upper chest behind the breast bone—plays an important role in the development of the immune
system and is abnormal in about half of individuals with MG. Some individuals develop
thymomas or tumors on the thymus gland. Generally, thymomas are benign, but in rare cases
they can become malignant.
Doctors recommend thymectomies for individuals with thymomas, and for most patients
under age 60 with moderate to severe generalized myasthenia. According to the National
Institute of Neurological Disorders and Stroke, surgery reduces MG symptoms and may cure
some people, possibly by re-balancing the immune system. This improvement, however, is
unpredictable and may take several months or several years after surgery to occur.
Immune globulin therapy can be used to treat rapidly worsening MG. Immune globulin is
a human blood product pooled from multiple donors who are carefully screened. By providing
the body with normal antibodies from donated blood, IVIg treatments appear to temporarily
modify the immune system. For most individuals, MG weakness typically improves within a
week of treatment and lasts for several weeks or months. IVIg treatments are very expensive and
offer short-term relief from MG symptoms until longer acting immune modifying treatments are
effective.
Side effects—for instance, headache or allergic symptoms–are usually related to how fast
the drug is administered. Slowing the infusion rate can help with this. IVIg infusions are
sometimes repeated at monthly intervals to sustain the treatment effect.
Siemieniak 10
J.J reports to last eating food on (4/7/18), and had fish for dinner. She had trouble
swallowing, felt like food got stuck in her throat. Last time she drank (4/9/18) water, at around 4
pm with just sips at a time, she states, "it feels that it come up her nose, feeling weird, but no
coughing or gagging." Per speech evaluation, the last time she experienced a crisis event, she
followed a Level 3 - Advanced Dysphagia;Thin liquids.
as tolerable. Patient was provided with Boost Plus Chocolate BID to help meet estimated needs,
while eating solid foods was tiring for her jaw muscles.
Unable to obtain 24-hour food recall during nutrition interview. Upon discharge patient
was prescribed a Level 3 - Advanced Dysphagia diet with thin liquids, to make chewing easier
and less tiresome. Patient was accepting of the diet order and showed no signs/symptoms of
intolerance to food or GI discomfort. Patients stomach was not distended, she did not complain
of issues consuming foods, and had regular bowel movements.
Good nutrition is important for everyone. This is especially true in people with chronic
disorders like myasthenia gravis (MG) with which some, but not all, patients will experience
difficulty with chewing and swallowing.3 However, weakness of the tongue, jaw, mouth and
throat muscles may make it difficult for some to chew or swallow food. At times of weakness,
some may get fatigued during meals and have difficulty eating the kind or amount of food that is
necessary to obtain adequate nutrients and calories.3 This could result in malnutrition and
unexpected weight loss.
For myasthenics, it is important to plan your meals when your strength is optimal. Eat
slowly and rest between bites if necessary.3 When fatigue is a problem later in the day, try eating
your main meal earlier. Instead of three larger meals, try eating five or six smaller meals. If one
is taking anticholinergic drugs like Mestinon®, eat said meal about an hour after taking
medicine.
Modifying the consistency of food can make it feasible for individuals to get proper
nutrition when having difficulty with eating and drinking.3 Preparing foods in a way that makes
them soft, tender and easy to chew and swallow will reduce complications of consumption.
Chop, mash or puree foods. Moisten dry foods with liquid. While eating, take sips of liquid to
soften foods in the mouth and help prevent solids from sticking in the throat. The thin
consistency of fluids makes them more likely to get aspirated into the lungs because they travel
quickly down the throat.3 Commercial thickeners can be added to thin liquids to give them a
more manageable consistency and reduce this risk.3
Body position and your mealtime environment are important. When eating, sit upright in
a chair and tilt your head forward. Avoid distractions while eating. Occasionally, anticholinergic
Siemieniak 12
medicines like Mestinon® may cause cramping and diarrhea. Aggravating foods include those
with a high fat content, greasy foods, spicy foods and dairy products. One exception is yogurt
with active cultures, which is actually beneficial.
When taken over an extended period of time, steroid medicines such as prednisone can
cause bone thinning.4 While taking steroids, it is important to have adequate amounts of calcium
and vitamin D in the diet from a variety of sources. Recommendations usually include 1500 mg
of calcium and 400 to 600 I.U. of vitamin D daily from food and supplements.4 Steroid use can
also cause fluid retention, so it is important to reduce sodium and increase potassium in your
diet.4
Medications taken by people with myasthenia gravis can have side effects that necessitate
dietary modification as ordered by the doctor.4 One side effect that might be encountered is
diarrhea caused by anticholinestrase drugs such as Mestinon or Neostigmine. If occasionally
diarrhea occurs, drink extra liquids to replace the fluid lost. This is in addition to the daily
requirements of eight (8) glasses of liquid per day.
Fluid retention is another side effect encountered by the MG patient. High doses of
steroids such as ACTH or prednisone may cause the body to retain water.4 Steroids cause
retention of salt or sodium which in turn retains water.4 Don’t add salt to foods when cooking or
at the table. Avoid certain foods such as commercially prepared soups, smoked of prepared
meats such as bacon, sausage, lunchmeat, ham and other pork products. Read labels on foods
bought in the grocery store for sodium content.
Other effects of steroid medication can be lowered levels of potassium, fluctuations of
appetite, and weight gain.4 Potassium is important for many of the body’s normal activities.
These include muscle contraction and nerve transmission of the heart, stomach and intestines and
muscles of the arms and legs.
Prognosis
The current treatments for MG are sufficiently effective that the outlook for most patients
is bright.5 Although the treatments may not cure MG, most patients will have improvement in
their muscle strength. In some cases, MG may go into remission during which treatment can be
modified or reduced.5 There is much that can be done, but still much to understand. New drugs to
improve treatments are needed. Research plays an important role in finding new answers and
treatments for MG.
With treatment, most individuals with myasthenia can significantly improve their muscle
weakness and lead normal or nearly normal lives. Sometimes the severe weakness of
myasthenia gravis may cause respiratory failure, which requires immediate emergency medical
care.
Conclusion
Throughout this case study, I became more familiar with Myasthenia Gravis. I
investigated treatment options, typical diagnostic tests, and prescribed medications for said
condition. Furthermore, I gained an understanding of the relationship between Myasthenia
Gravis and its effect on obtaining adequate nutrition. This is a relatively minor case of
Myasthenia Gravis, in a patient who is not troubled by financial burden, had access to quality
food and is at a good nutritional status prior to exacerbation.
Nonetheless, this case provided me with a greater understanding of the disease, and how to be
more prepared for future patients who deal with the same condition. I would have liked to
retrieve more nutrition history from J.J. to more accurately describe ways to improve patient care
and improve prognosis. Overall this was a rewarding experience, and I hope I can share my
knowledge of Myasthenia Gravis with interns of my own one day, or with my co-workers.
Siemieniak 15
References
Appendices
Pyridostigmine Muscle strengthener should not be used with other Diarrhea or stomach pain.
(MESTINON) cholinesterase inhibitors Drooling or
more saliva than usual.
Siemieniak 17
Nausea or vomiting.
Rash.
immune globulin 10% protein liquid Other drugs may interact headache, fatigue, nausea,
(PRIVIGEN) preparation of with immune globulin, chills, vomiting, back pain,
polyvalent including prescription and pain
human immunoglobuli over-the-counter medicines,
n G(IgG) for vitamins, and herbal
intravenous products
administration
cycloSPORINE Immunosuppressive concomitant use of allergic
(SANDIMMUNE drug nonsteroidal anti- reactions, anemia, anorexia
) inflammatory drugs , confusion, conjunctivitis,
(NSAIDs) with cyclosporine, edema, fever
particularly in the setting of
dehydration, may potentiate
renal dysfunction
ferrous sulfate Iron supplement Coffee, tea, eggs and milk m constipation;
ay prevent the full absorption upset stomach;
of iron black or dark-colored
stools; or.
temporary staining of the
teeth.
Asthma Mother