Sleep Study Results: "A Unique Case"

Dear Andrea, 
My sleep study results are in, and my doctor says I'm a unique case.  He says I don't have any signs of Muscular Dystrophy related sleep issues, that I just have sleep apnea.  For that reason, he's prescribing a CPAP.  Didn't I read CPAP is not recommended?  If so, now what?!

​Sincerely,
CPAP Ordered

Dear "CPAP Ordered,"
Your sleep study experience is SO common. But how could so many be a "unique case" and "just have sleep apnea?!"  The truth is, when we see a specialist who does not specialize in neuromuscular breathing weakness or the clinician does not have experience interpreting sleep studies of individuals living with neuromuscular conditions, inappropriate equipment can be prescribed.  In some cases, nothing is prescribed, because we are not "bad enough," according to their interpretation of our results. So many of us have been through this, so you are not alone!

Another common problem is the sleep study did not include monitoring for both oxygen saturation and exhaled Carbon Dioxide (end tidal CO2). Lacking the "full picture" of our sleep can lead to supplemental oxygen being ordered, a more dangerous example of mismanagement. (You can learn more about the danger of unventilated supplemental oxygen for those with neuromuscular breathing weakness at https://breathewithmd.org/oxygen-caution.html.)
 
What your doctor is ordering, CPAP, is not advised for those with weakened breathing muscles, because it offers one continuous positive airway pressure that blows while you’re inhaling and exhaling.  It can be difficult or exhausting to exhale against that pressure and make us feel worse. For more about this, consider watching my video “BiPAP or Bi-level Versus CPAP: Understanding Why CPAP is NOT for Neuromuscular Disease,” at https://youtu.be/A1gH6hfZigo. 
 
The point of assisted ventilation at night is to rest the breathing muscles by assisting with the process of moving air into and out of the lungs, either reducing the effort or completely eliminating that effort. Only bi-level devices (often referred to as “bipap,” and portable ventilators) move air into and out of the lungs by offering two different positive airway pressures, one for inhaling (IPAP), and a second much more reduced pressure for exhaling against, (EPAP).  In some cases, with ventilators using what's called "active circuit," EPAP is set to zero, eliminating all effort to exhale.  

It can be difficult to confront a clinician who is not familiar with neuromuscular breathing weakness and is incorrectly prescribing CPAP.  Trust me, I've been there; 2013 was my "lucky year" for that!  This clinician may even be known as an expert in your area and has prescribed CPAP hundreds if not thousands of times for individuals with central and obstructive sleep apneas. You may feel intimidated challenging his or her opinion.  

While it is not impossible for an individual with a neuromuscular condition to have central or obstructive sleep apnea, it’s more likely that the sleep study equipment is interpreting the weak, shallow breathing and hypoventilation (under ventilation) as lack of breathing or “apnea.” It takes someone with a trained eye to realize what they are seeing is instead lack of muscle effort caused by the neuromuscular condition.  
 
If you are looking for documentation to share with your clinician in the effort of self-advocating to get the appropriate care, you might want to share an article published in the MDA Quest magazine called, “Not Enough ZZZzzzs.” It’s available at https://www.mda.org/quest/article/not-enough-zzzzzzs. Another easy-to-follow document is Muscular Dystrophy Canada’s, “Guide to Respiratory Care for Neuromuscular Disorders” available at https://muscle.ca/wp-content/uploads/2019/09/RC13guide-EN.pdf.  You can find Chapter 7: Noninvasive Ventilation on page 25. 

​​If after sharing materials and discussing this with your clinician, he or she is still insisting you need CPAP (as mine was), you have the right to request a second opinion referral to another clinician that YOU select. A neuromuscular pulmonary specialist, usually located at a university-based hospital, is your best bet for getting an accurate assessment and plan of care for your breathing weakness.

You may feel like this will offend or disappoint your current clinician and cause conflict.  Understand that clinicians are used to this and understand that patients  sometimes need another opinion.  And when it comes to issues with our breathing, I am not exaggerating when I say that your life could depend on the decision to see a different clinician.  Appropriate care can make a tremendous impact on your health and quality of life!  I have seen this so many times in the individuals I've met through Breathe with MD, Inc.  

​Having said all of that, I hope my response to your question has been helpful and that the resources I shared provide the additional details you need to assist you in getting the care that’s right for you. 
 
Best Wishes,
Andrea