Ten Most Frequently Asked Questions

Grab your favorite beverage and get comfortable!  In this lengthy blog post, I'm covering the 10 most frequently asked questions I'm asked by individuals living with a neuromuscular disease (NMD) and/or their loved ones.  This blog is jam-packed with videos and links to resources for additional information.  As always, don't use this blog post as a substitute for medical care.  Seek medical advice about breathing muscle weakness in NMD from a licensed clinician who specializes in the breathing issues of those with NMD.  

Question 1:  Could I be having problems with my breathing?  I've gotten several variations of this question, often with a variety of symptoms being shared.  A common theme I see is that many adults struggle to accept that their breathing might be affected by their NMD and hope it will just get better on its own or that they are imagining the issue.  I get it; it's scary to think about something so critical as our breathing being affected.  However, my advice is always the same:  If you even THINK you might have a problem with your breathing, or if you haven't had an evaluation of your breathing by a clinician who specializes in the breathing issues of those with NMD, DON'T DELAY seeing a specialist.  It should be one of the first things you do after you are diagnosed with a NMD, and it should be followed as frequently as your clinician recommends (usually at least annually).

Don't know where to find a clinician?  University-based hospitals that include a neuromuscular clinic typically have a pulmonary specialist, neurologist, and/or physiatrist (doctor of physical medicine and rehabilitation, PM&R) who understand breathing muscle weakness in NMD.  You can also ask you peers, particularly those with NMD in your area.  

If you are new to the concepts of breathing muscle weakness, Muscular Dystrophy Canada has published an easy-to-understand, comprehensive, "Guide to Respiratory Care for Neuromuscular Disorders." You can find it at muscle.ca/wp-content/uploads/2019/09/RC13guide-EN.pdf.  You can also find a lot of information on the Breathe with MD, Inc. website.

Question 2: How can supplemental oxygen be dangerous?  
Unventilated supplemental oxygen led to my sister Cheryl's death and is why the nonprofit organization Breathe with MD, Inc. exists.  Simply put, if you have weakened breathing muscles and low Oxygen saturation, using supplemental oxygen via a nasal cannula while not on mechanical ventilation can make things worse, particularly if you are unable to effectively exhale the waste product of the air we breathe:  Carbon Dioxide (CO2).  

The problem for those with weakened breathing muscles is difficulty moving air into and out of our lungs, a process called ventilation.  When we don't ventilate our lungs effectively, it can lead to an imbalance in the level of Oxygen and Carbon Dioxide in our lungs and blood.  Use of supplemental oxygen does not improve ventilation and can, in some cases, turn off the respiratory center in the brainstem that tells us to breathe on our own.  What we need instead is bi-level mechanical ventilation.  I encourage you to watch the short video below. You can get even more detailed information, including links to medical articles to share with your clinician, at breathewithmd.org/oxygen-caution.html.

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Question 3: Why is CPAP bad for me?
CPAP is not recommended for those with breathing muscle weakness, as it offers one continuous positive pressure that is inhaled and exhaled against.  When you have weak breathing muscles, exhaling against pressure can be tiring, difficult, and make you feel worse.  Instead, you need bi-level ventilation (sometimes referred to as "BiPAP" or a portable ventilator), as these devices offer two different positive pressures.  A higher pressure is used for inhalation (IPAP), and a much lower or no pressure is used to exhale against (EPAP).  Watch this short video that explains the difference in more detail.   

Question 4: What is CoughAssist, and why is it recommended?
The CoughAssist is a respiratory device sometimes referred to as an "insufflator/exsufflator."  Those are big words that mean it helps to make a weak and/or ineffective cough stronger and more productive at removing secretions or mucus from the airways.  Learn more in this short animation below. Be sure to ask your NMD care team to determine your peak cough flow (PCF), the velocity of air being expelled from the lungs after a cough maneuver. This measurement can determine whether or not you need assistance with coughing.  

The CoughAssist can also be used to stretch the breathing muscles much like you would stretch other tightened muscles.  I use CoughAssist multiple times a day when well and as often as I need to cough when I'm sick.  It's kept me from developing pneumonia or being hospitalized.  You can read more about my experience with the CoughAssist in a past blog post at ​breathewithmd.org/ask-andrea/the-device-you-love-to-hate.  

To learn the specifics on how to use CoughAssist, check out the manufacturer's documentation at https://www.usa.philips.com/healthcare/product/HC0066000/coughassist-t70-mechanical-insufflator-exsufflator#documents.  And lastly, review the benefits of using CoughAssist by watching this short animation.  

Question 5: How do I know if my ventilation settings need to be increased?
Reach out to your respiratory care team to open a dialogue about why you might need to increase or change your device settings.  Speaking from my personal experience, I knew my settings needed to be changed when I didn't feel like I was getting a large enough breath from my ventilation while lying down. Another time, I was experiencing some of the symptoms I had before I began using assisted ventilation such as excessive daytime sleepiness, fatigue, and needing 10-12  hours of sleep to feel rested.  

Sometimes an overnight, at-home "oximetry study" is useful to measure and record your Oxygen and/or Carbon Dioxide levels during sleep.  Think of it as a home sleep study with less equipment and easy enough for you or a caregiver to set up. Recording pulse oximetry and capnography can guide the decision making process for ventilation settings changes.  Ask your NMD care team more about this option.  

Question 6: How can I adjust to using noninvasive ventilation (NIV) at night?
This is a common question of "NIV newbies." I was an unusual case; I took to NIV right away.  Many find that starting to use NIV an hour or two during the evening while relaxing, reading, watching TV, or playing a video game is a good way to get comfortable with it before trying to sleep at night with it on.  Others find they simply cannot adjust to their current mask system and need to try something else.  Mask preference is an individual choice; I love nasal pillows and have no issues using them at high pressures.  Some hate them and prefer nasal, oronasal or full face masks.

Do not hesitate to call your respiratory equipment provider to ask to try different masks and to share any problems, concerns, or questions you may have about the new equipment.  Asking your peers is a great way to find out what masks might work best for you.  A good way to reach peers is in the Breathe with MD Support Group.  Learn a little about what makes it special in this short animation, and if you're not already a member, consider joining today.  

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While I'm sharing about the support group, let's move onto another question about it.  

Question 7: Why can't my physician, respiratory therapist, or other care provider join the support group?
The questioner usually explains they want to share knowledge found in the support group with a clinician, usually one that is located outside of the US.  While I don't doubt that a clinician could learn from us, the last thing most group members want is for clinicians with different view points to debate a "best practice" for management and/or intervention. Further, while you may be comfortable with your own physician or respiratory therapist (RT) being a member of the group, understand that others might not want their personal health challenges being read, commented on, or even chronicled for informal research by those clinicians. We make no exceptions to this rule; medical professionals are not allowed to join the Breathe with MD Support Group, unless they personally have a NMD or a loved one does (which is the case for a small number of members).

As an alternative, I suggest that you invite your clinician(s) to visit our website https://breathewithmd.org/index.html​, our public Facebook page, or find us on Instagram, Twitter, and/or YouTube.  I personally put a lot of effort into sharing educational materials on our social media platforms, and our website includes a lot of medical articles that can be viewed and/or printed.

Question 8: Why can't I share posts from the support group? 
Like all private groups on Facebook, it is not possible to share a post from within the Breathe with MD Support Group. That is part of the privacy enabled with the group.  You can copy any website links or details about resources, and then paste them into a new post on your own Facebook wall or within another group.

Question 9: Does Breathe with MD, Inc. purchase respiratory equipment for individuals with muscle disease? 
At this time, our small US registered 501(c)(3) nonprofit does not provide any financial support or durable medical equipment for individuals with NMD.  You can learn more about our mission and programs at breathewithmd.org/missionandprograms.html. 

Question 10: Is there an exercise I can do to make my breathing muscles stronger? 
Unfortunately, with diseased muscle tissue, we don't have the benefit of making it stronger like with healthy muscle tissue.  Instead, we can stretch our chest wall muscles so that they may be less restrictive to our rib cage and lung expansion, over time allowing us to fill our lungs with more air.  Talk to your NMD care team about lung volume recruitment or breath-stacking.  Additionally, you may recall that earlier in this blog post I mentioned use of CoughAssist can stretch the breathing muscles.  

Well, that's it; I've covered the 10 questions I am most frequently asked by individuals in the NMD community. As always, seek the advice of a licensed clinician for any questions or concerns you have about your respiratory health. The advice I share here is solely based on patient perspective.  

Wishing you all the best,
​Andrea