OK, after hearing a few "horror stories" such as recently by bluesky, and other MG patients, I have decided to try and do something.

but guess I was too naive about it.

wish you all the best,

alice

Not every respiratory symptom related to MG, curtails an emergency situation or is immediately life threatening. In fact, most of the times it is not. Yet, involvement of the respiratory muscles in this illness can have a significant impact on the quality of life, and significantly affect functional ability. Therefore it is important to recognize those symptoms as early as possible in order to institute proper support and possibly prevent life threatening complications.


What are the symptoms and signs that suggest respiratory muscle involvement in myasthenia?

Shortness of breath on physical exertion-eg-climbing steps, walking
Shortness of breath when talking.
Heavy breathing following a meal.
Chest pain or chest tightness..
Feeling dizzy after exertion
Feeling "tired"
Multiple awakening during the night, and non-refreshing sleep.
Waking up with a feeling of suffocation. Sometimes as if you have had a nightmare.
Morning headaches.
Unexplained fatigue.

Most of those symptoms are not specific, and may be unrelated to MG, but their occurrence or worsening; warrant a proper respiratory evaluation by a respiratory specialist with a good understanding of neuromuscular disease.

Some of the symptoms are easier to recognize as related to breathing problems, others are less obvious and are the result of CO2 retention. It is important to be aware of the fact that many of them may mimic depression or anxiety, and can be easily over looked and attributed to that.

A very easy test to perform on your own, if you experience any of those symptoms, or have concerns of respiratory muscle weakness, is to take a deep breath (as much as you possibly can) and then try counting, with taking in air again.

A normal and healthy person should be able to count to around 50, being unable to count more then 10 is usually an indication for respiratory support. Anything in between will suggest some degree of respiratory muscle weakness.

What can you do to feel better, until you have proper evaluation?

Rest as much as possible, and try to avoid significant physical or emotional exertion.
Stress can significantly increase the heart rate, and the oxygen demand and make things worse.

Find a sleeping position that is most comfortable and you don't experience as much shortness of breath. Sleeping in a semi-supine position may be helpful.

All the tests should be done and interpreted properly by a respiratory specialist with experience and understanding of neuromuscular disorders.


A sleep lab, is a good starting point. Most neuromuscular disorders will have some manifestations during sleep, even before there is evidence for significant impairment during the waking hours. The reason for that is that during sleep there is always some degree of relaxation of the respiratory muscles, and gas exchange is less effective.

It is very important to assess both oxygen saturation and CO2 level,during the test, as there are patients with significant respiratory muscle weakness who do not have sleep apnea and manage to keep levels of O2 within the normal to low range, and do have significant CO2 retention.


All the respiratory tests are based on the inhalation and exhalation of air into a special device that measures the volume of air, the flow rate, and the pressures.

The basic spirometry tests measure the amount of air that can be inhaled and exhaled during a normal deep breath, without resistance.

It is important to know that as those volumes are not only dependent on the force of respiratory muscles but are affected by other factors, such as the resistance of the chest wall etc. they can be misleadingly normal, during one single respiratory effort, such as is done in those tests, even in the face of significant clinically important respiratory muscle weakness.

Therefore it is important to directly assess the respiratory muscle force. This is done by inhaling and exhaling against a fixed resistance.

The MIP –Maximal Inspiratory Pressure, gives a good idea of the general respiratory muscle strength.
The MEP-Maximal Expiratory Pressure, gives more indication as to the intercostal muscles.
Snif-is a better measurement of the diaphragmatic strength, and as it is the only test done by nose breathing it is more accurate then the others in patients with significant facial muscle weakenss.

As the hallmark of myasthenia is muscle fatigue and not muscle weakness, it is very important to assess the endurance as well. this is done by the MVV test, which checks the amount of air that can be breathed during repeat effort, as opposed to the single effort done in the standard spirometry tests.

The results of all those tests taken together should give a good general idea of the degree of respiratory muscle involvement (if any)

Oxygen saturation can be in the normal range or slightly lower, even in patients with significant respiratory muscle weakness, and is not a good parameter to follow alone in MG..

Patients with MG do not usually have a significant oxygenation problem, unless nearly paralyzed or have another respiratory complication, and most do not require supplemental oxygen.

In conclusion:

We suggest that every patient with generalized MG will have a full respiratory evaluation at least once.
In case that there is no significant respiratory muscle involvement it would give a good baseline for further follow up in case respiratory symptoms do occur at a later stage. This is important as the normal values of respiratory tests can differ among people, and therefore early recongnition of respiratory muscle involvement is only possible if the normal for that person is known, by showing a trend of worsening even if still within the low normal range.
If there is a significant involvement of the respiratory muscles this will allow proper assessment of its severity, and the need for respiratory support.

Patients that have symptoms suggestive of respiratory muscle weakness, should have a repeat evaluation if the first one is non-conclusive, as it may show a trend, and be more informative the second time.

Although all those tests are effort dependent, most patients can perform them very well, after given proper explanation . therefore abnormal results in a patient with respiratory symptoms should not be interpreted as lack of effort during the test, unless there is clear evidence that the patient is not able or is unwilling to co-operate.

Patients with depression, chronic fatigue etc. have normal or near normal respiratory tests, as it does not require significant motivation or effort.

Thanks, Alice. Yes, the task is daunting but the idea brilliant. And necessary. I have some things to add at this time.

MG Breathing "warning" Symptoms:

Sudden shortness of breath episodes
Chest wall muscles cramping or spasming
Generalized weakness that progresses to muscle cramps or spasms anywhere
Waking up choking


Breathing tests:

MIP and MEP: The highest number is always given, even when a series of four or more are done. That does not give a clear enough picture of what is going on with the patient. Numbers that trend downward are as important as a single absolute number. This is true of the other tests but none as important as MIP and MEP since MG is all about weakness that gets worse with repetitive activity. Duh.

Arterial Blood Gases:

Neurologists AND pulmonologists often do not understand the significance of this test (or non-significance too), particularly when it shows hyperventilation. And then there are the doctors who do it while someone is on oxygen, which skews the results.

Don't ever mock your patient and say they must be anxious because their ABG tests showed they were hyperventilating!

Myasthenic Crisis:

DO NOT put an MG patient in a regular unit of a hospital. Due to the unpredictable nature of the illness and weakness while in an imminent or current myasthenic crisis, they need to be monitored in an ICU setting.

Standard treatment while in a crisis is not adhered to. Plasmapheresis is usually given though not always. Some patients can't have it but it should at the very least be the first "offer" of treatment. Treatment is a complicated issue since some people with MG do better with IVIG than plasma.


I'm sure I'll have more ideas, Alice. If you want this to "look pretty," I do have a Masters in design. When I used to work, I did a lot of marketing and design in the health care field. If you need my somewhat time and muscle restricted help, please let me know.

Please thank your colleagues for doing this.

Annie

Alice,

Very well written and you are correct when you say Pulmonologists don't understand MG or I should say, don't want to understand it. You wrote...

What are the symptoms and signs that suggest respiratory muscle involvement in myasthenia?

Shortness of breath on physical exertion-eg-climbing steps, walking
Shortness of breath when talking.
Heavy breathing following a meal.
Chest pain or chest tightness..
Feeling dizzy after exertion
Feeling "tired"
Multiple awakening during the night, and non-refreshing sleep.
Waking up with a feeling of suffocation. Sometimes as if you have had a nightmare.
Morning headaches.
Unexplained fatigue.

The above is my daily life..LOL Yet my pulmo pushed me off to my neuro and so on and so on so I just "live" with it. I have had a sleep study done and doesn't it figure that on that night, I slept well. It is so hard to tell what is MG and what may be something else {chest pain/heart} {dizzy/blood pressure} {suffocation/panic attack} <-- that is my personal favorite. LOL.

I can't hold my breath and count past 20, never mind inhaling again while counting, {I tried it} Keep up the good work Alice and I look forward to seeing how it goes for you. {I know I am working on speaking to EMS workers about MG}

Annie....

BRAVO on this one..

DO NOT put an MG patient in a regular unit of a hospital. Due to the unpredictable nature of the illness and weakness while in an imminent or current myasthenic crisis, they need to be monitored in an ICU setting.

I was in the hosp years ago and went into crisis and stumbled to the nurses station and passed out, later I learned they called a code blue on me. MG is very hard for Dr's and nurses to understand if they are not "involved" with the illness.

You and Alice are the best!

Great job!! It is wonderful that you are doing this!!
Kristy

One more thought, regarding funding of such an effort.

It may be possible for this to be funded by the MGFA. Or by some kind souls out there who want to make a difference.

I would also think that a CD or DVD of it may be more useful than a paper brochure type of delivery system. Perhaps a combo of the two.

Annie

Joanmarie, Everyone here is amazing AND has something to contribute, especially to this discussion!

I'm writing this here as to not lengthen the thread. Xanadu, this was completely the idea of Alice. And I've only added a very small amount compared to what she has already done!!!

Alice, Annie - thankyou for this wonderful idea and work. Somehow we have to rewrite the training of Neurologists so that they can understand the pulmonary issues. I have just had an appt where despite me talking of worsening breathing issues, despite me having two Lung Function Tests 3 years apart showing the decline ....despite the Sleep Study showing neuro patterns of apnoea... despite all the facts of the decline in lung function...nothing was done to help. The paperwork was ignored. No CO2 tests have ever been done. I can only assume that breathing issues were considered to be imaginary despite the b& w results of three independent tests. An appt booked for 6 months when you are complaining of respiratory distress is not appropriate!

Somewhere , somehow we have to make the Neurologists and Pulmonary Drs aware of how MG works so that they can work together and help each other and help the patient.

You posted and asked for input.

*please note that I am parsing paragraphs irregularly to facilitate ease of reading to those with visual issues*

First I am a healthcare professional. I can no longer practice my craft as the MG has left me with many and varied disabilities. But the knowledge remains. *edit*.
*edit*

*edit*

On to the actual content.
Any time you have an issue such as this, you should ALWAYS begin with your neurologist. He/she is the captain of the MG ship. He/she should be the one to forward you to a pulmonologist if necessary. *edit*

What is CO2 retention? Will the average person understand *edit* Will they understand that the poor quality of sleep and awaking with a headache are the symptoms that are related to CO2 retention? Will they understand that it is from the muscles tiring and failure to inhale deeply enough to blow of the CO2?
*edit* You are to inhale as deeply as possible then count while exhaling.

*edit*any respiratory distress should be evaluated immediately. *edit*MG patient can experience respiratory failure within minutes of presentation of first respiratory symptoms.

*edit*

*edit*

Monitoring of CO2 levels during a sleep study is not the standard of care either. It has to be ordered specifically and the indicators clearly delineated. There are several reasons for this, but the two main issues are invasive procedures on the patient when not necessary and insurance will not pay for this unless/until there are quantifying factors.

Again, point of standard of care--a respiratory therapist does not use one single effort when performing these tests. The standard of care is the average of a minimum of three efforts.

*edit* the most common of tests which is the PFT. That is the gold standard for initial encounter. Most literature is more compartmentalized. The patient needs to understand the tests As they are ordered not a laundry list of tests that may or may not be ordered.

I think you can see what I mean from the citations. *edit* I am sorry as I am certain you didn't post it to have it dissected, but you did ask and I answered.

*edit*
*edit*

Well guess that about covers it. You asked, I answered.