If you want, created a memes feature, then push that tube and add it to the fel-
Hi everyone, and welcome back to Airway First, the podcast from the Children's Airway First
Foundation. I'm your host, Rebecca St. James. My guest today is board-certified pediatric
cardiologist and pediatrician, Dr. Andrew Maxwell. Dr. Maxwell received his medical degree
from Johns Hopkins Medical School and a residency in pediatrics at the University of California
at San Francisco, followed by a clinical and research fellowship in pediatric cardiology
at Lucille-Sotler Packard and Stanford Hospitals and Children's Hospital of Philadelphia. His
research interests include study of endothelial control, evasomotor tone, nitric oxide, sports
cardiology, dysautonomia, hypermobility syndromes, and mass cell activation syndromes. For his
research, he received an American Heart Association Burger Award for Research in Molecular Biology
and was an American College of Cardiology Young Investigator Award finalist. Additionally,
he has published many articles and book chapters on these subjects. For his clinical work,
he has been voted by his peers as a top doctor in Northern California annually since 2017.
You can find out more about Dr. Maxwell at heartofthevalley.us. Now, let's jump into
my interview with Dr. Andrew Maxwell.
All right. Great. Thank you for joining us today, Dr. Maxwell. I appreciate you taking
some time to be here.
Thank you for inviting me.
Absolutely. All right. I'm just going to jump right into it because there's a lot to talk
about. This is a topic that I think a lot of our parents don't think about, but we've
had enough that I've had questions specifically around this, so I really want to give it the
full hour so we can really dig deep into this. I want to start off for those that don't know
them. Let's define, and I have to look at it because I will slaughter it, otherwise autonomic
dysfunction or dysautonomia, and how this relates to things like autoimmune disease
or L-er's, Dan Lo, those type of conditions.
Sure. Okay. Yeah. You didn't slaughter it at all. Autonomic dysfunction or dysautonomia.
It's very much an umbrella term that is a collection of disorders of the autonomic nervous
system. We have the autonomic nervous system and everything that runs in the background.
Typically, we think of it as the parasympathetic and the sympathetic nervous system that make
up that. There is the enteric nervous system that also is there as well. For the most part,
the parasympathetic and the sympathetic nervous system are what we're talking about. Furthermore,
we're usually talking about a dysfunction of the parasympathetic nervous system. A big
part of that is the vagus nerve. We'll probably talk quite a bit about the vagus nerve there.
That doesn't mean that the sympathetic is not also part of the problem because what
we have is sympathetic overdrive trying to correct what the parasympathetics aren't doing
correctly. The sympathetic gets into the picture as well.
That's pretty high level. When we're talking about these kind of conditions, is this something
that we see, and I'll just say just kind of as an umbrella of boys and girls. I know
this could be young, this could be teenagers, but just in general, boys and girls, do you
see this in both? Do you see this more in one? Is this something that you're born with
or that you develop? How does this work?
Well, it's in both boys and girls and it's of course all ages from birth till elderly,
but there's definitely peaks and there is gender bias depending on underlying triggers
and where you are in your age group. It's very, very common when you bring in all the
different levels, what I call grades of dysautonomia. I've traditionally graded this from
zero to four and the meaning of each is zero would be no dysautonomy at all, no symptoms
of dysfunction of the autonomic nervous system at all.
Grade one being that of an otherwise normal healthy teenager. If we think about them getting
head rushes upon standing up and losing their vision, sometimes even fainting, what we call
vasovagal syncope, which is very common in teens as well as maybe even their fatigue
and their tendency to want to sleep long hours, maybe a form of mild autonomic dysfunction.
Athletes we even see this more. There's grade two dysautonomia, which is really just more
than grade one, more than the typical teen, but not yet grade three, which I'll define
in a moment. Athletes are often in the grade two category where they're even more dysautonomic,
they're more standing up and losing their vision and more maybe tendency to faint and
needing extra help to manage their autonomic dysfunction. Teen girls are often found to
be grade two and a lot of that has to do with development of estrogen cycling. Their bodies
are struggling with the development of their cycle. Also, it happens on top of rapid growth
in height and both those play into developing more than the standard teen dysautonomia.
Then there's grade three and the definition of that is it's now interfering with school
sports and social life. When you're so symptomatic that you're quitting your sport because you
feel lousy at an upright sport particularly or you're missing a lot of days of school
or getting behind, maybe it's because of foggy thinking, maybe it's because just feeling lousy
when waking up with nausea and inability breakfast and things like that. Or you're telling your
friends, look, I cannot hang out with you guys because I pay the price over the next couple
of days if I stay out late. That's starting to interfere with school sports and social life.
That's grade three and that is where we see a lot of the illnesses that are requiring a lot of
medical attention to get them to be able to stay in school in sports. Then finally, grade four is
where it's so significant that someone is requiring instrumentation or other items like, for instance,
G-tubes and NG-tubes and walking assistants or maybe even they're just bedbound.
So grade four is pretty significant interference with life and often requires a lot of
therapies of various types. When you look at the whole wide spectrum of dysautonomia, you can see,
yeah, dysautonomia is almost ubiquitous around us if you include the grade ones and grade twos.
Now, a lot would say, well, okay, yeah, those are just the normal development and expected and
it doesn't require much more than some lifestyle modifications to improve those. So if we eliminate
grades one and two and just focus on three and four, then of course it's much less common,
but still pretty common. And of course, I have a clinic full of these patients and they were
and they were coming to me long before I became what someone may view as someone to seek out for
this. So just in my area as a cardiologist, having all these patients coming for shortness of breath
and exercise intolerance and chest pain and palpitations, all the things you would see a
cardiologist for and the clinic is just full of these patients from my normal cardiology catch
area. So it's pretty common. And you said something there that I want to touch on.
When you're talking about kids coming to you because they're having exercise exhaustion,
could these kids be coming to you because they're told they have what is exercise induced asthma
or something like that. Otherwise, they're normal, they function normally, you know, 14,
15, 16 year old. Yeah, of course. I mean, that's the that would be the very first thing to assess
when someone comes in with exertional shortness of breath. Okay, well, do you have them the
single most common reason for that, which is undiagnosed or oftentimes remote history of asthma
as a kid. And now they had no idea that this is coming back in a different form, that they're not
wheezing, then they're not acting like they had asthma when they were little. But now they're
exercised, induced shortness of breath. And we might do very commonly do PFTs before and after
albuterol or Zopin X, and do exercise testing to see if we can bring that out. So that would be
a very, very common outcome of a patient that comes in with shortness of breath. But usually,
I can tell those patients because it's only one single symptom, or maybe they have chest tightness
as well, that kind of picks them apart, as opposed to those who are coming in with chronic fatigue
and dizzy upon standing and palpitations with minor activity or no activity at all. So those
patients with broader symptoms are usually, now there's I mean, they can't have both. And certainly,
you know, very often, I'm like, well, yeah, you have all this autonomia, you have all these problems.
But yes, you also have some, we need some attention to your pulmonary function too. And so you're
going to benefit to some extent from an inhaler as well. And just to make sure that I'm saying this
correctly as we continue our discussion, it's correct to call these disorders, correct?
Not, is it, do I call it disorders or is it dysfunction? What would be the right term?
Oh, I think, I think it's fair to call it either one of those. Okay.
Disfunction disorders, I think are synonymous enough that you can use it either one.
Okay, excellent. So these disorders, is this something that is typically,
a child is born with? You know, can you catch this early? Or does something in life just trigger it?
I mean, I know within girls, you know, you mentioned puberty can often
impact it, but is there something that triggers these and maybe makes it
worse in some children? That's part of the evaluation that I do and I think should be done is,
okay, what are the genetics of the person? What is the environmental exposure that may even occurred
from birth on? And so a lot of these patients will get some genetic testing done and find that they
have either a connective tissue issue that makes them more susceptible to what's going to happen
in the future with dysautonomia, or they have sort of an obligate, what's called mass cell
activation syndrome, which eventually we're going to probably get into with some of these questions,
that activates mass cells and mass cells then lead downstream to connective tissue disorders
and directly to dysautonomia. So a lot of it can be somewhat pre-ordained at birth based on genetics,
but I really do think that it's a genetics meets environment issue. We're perfectly healthy,
what I call the canary in the coal mine. So if you think about a canary is a perfectly healthy
bird, nothing wrong with a canary. It has a nice life unless it's put into a bad environment like
a coal mine where it's the first person to become sick. And that is what a lot of these patients are,
they're canaries in coal mine where they're born with genetic variants that if they're in the right
environment, they're going to thrive and not have a problem. But they may not thrive so well in a,
for some, a very mildly toxic environment, maybe mold in their environment or as I am trying to
investigate whether even just the presence of blue-green algae blooms with cyanotoxins in the water
could affect these canaries with their genetics that make them particularly susceptible.
And so for some of them, it starts at a very, very early age. A lot of these patients will have
either the history of, yeah, they had GI issues from practically birth where they weren't
tolerating formulas and it was a struggle to get the right foods and calories into them to grow.
And they've always had abdominal bloating and sensitivity to foods and all that.
Or they end up with a pan's-pandis-like picture where they're showing early on OCD type characteristics,
ticks, clinginess to parents and all the things that you would use to give a clinical diagnosis
of pans or pandas. And that is maybe the six or seven or eight-year-old that's showing that
and they may have had GI issues before that. But then by the time they're hitting nine and above,
they're starting to convert over to more of a what we call POTS picture. We haven't used that term yet,
Postero-Orthostatic Tachycardia Syndrome, which is somewhat under the umbrella of dysautonomia.
And I say that with a lot of caution because I give entire lectures on how POTS and dysautonomia
are distinct and not real, one's not really a subset under the dysautonomia. But the point is,
is these patients will transition from a pans to a POTS type of physiology. And that would be a very
common way that young people enter into this problem. So there's that now. Now we have with COVID,
we have everyone coming in from post COVID long haul and some coming in with, you know, vaccine
induced vaccine triggered. I haven't seen it for a while now, but for the first year of the vaccine,
there was a lot of patients coming in who were perfectly fine, but developed some dysautonomia,
mass cell activation syndrome, immediately after having a vaccine. Those were usually short lived,
what I call short COVID syndrome. But now we have so many that are triggered after having
the COVID virus itself. And now a couple of years into dysautonomia. So.
Wow. So the virus could actually trigger it.
Yeah. Well, I mean, there's a lot of theories about how that occurs. Is it the virus itself still
chronically living in the person and just low levels and causing dysfunction? Is it the spike
protein that's been left behind and it just needs, the body needs to clean up the spike protein? And
it's a spike protein is a very mass cell inflammatory agent. It activates mass cells.
And if any of it's left around and the mass cells are easily triggered,
their mass cells just continue to be activated and causing downstream effects, including
dysautonomia. Then there's a question of whether COVID distracts the immune system and allows for
other latent organisms to come out. So the most commonly thought of one would be Epstein-Barr virus.
You know, most of us have converted from negative to positive Epstein-Barr virus sometime in our
teens and have fought off the mono infection. And now the virus is just living within our
nerve cell bodies in our immune system keeps that in check, except maybe during something
like COVID, it distracts that and allows those to come out. And of course, we see the
similar situation with people who have had varicella zoster. Also a virus that just lives
in dormant in our nerve cells until our immune system is distracted and it comes out again as
shingles, right? So people might have that virus come out and you have shingles. And of course,
we see that with an aged population where their immune system is not so much distracted, it is
just waning and the varicella zoster virus comes out. So it's very similar concept when we talk
about COVID allowing for Epstein-Barr virus to come out again and basically cause a chronic
mono and maybe the basis of a lot of chronic fatigue syndrome. Okay. Well, then I guess this
seems like a good time to segue into how does dysautonomia affect the organs within the body?
I'll dive right into that. Okay. Well, okay. Well, I mean, one of the concepts that I like to
teach or get this concept across is that it's very hard to have dysautonomia by itself, that there's
usually this at least combination of the mass cell activation and dysautonomia that occur together.
And then there's eventually some connective tissue issues come into play as well. And
I have a probably some slides that might be worth
Oh, yes, please showing here that highlights that. If I could just find one. Okay. This would
be a good one. So I'm going to share my screen here. And it's going to be this one. And while
you're pulling that up, I'm going to let our listeners know that I'll put a link to a book
that you did a chapter on that we're discussing excerpts from. I'll disjoint it.
But they might be interesting. Okay. So you see my slides. Okay. There I do. Yes. Okay. So
So when you say how are organ systems affected, I mean, when we're talking about the autonomic
nervous system, you're pretty much talking about every single organ system in the body is affected
when the autonomic nervous system isn't working well. And so here's all the different organ
systems broken down and all the symptoms that may fall under those organ systems. But I've color
coded them according to how much is actually due to dysautonomia, which is in the light blue,
versus how much of those symptoms might be occurring from the mass cell activation that
the copathology of mass cell activation versus those who develop some hypermobility or even
full blown Ehlers-Danlos. Some of those symptoms are coming from that. And the final category,
this green is sort of the consequences of there's upper airway resistance syndrome. There's the airway
issue and cranial cervical instability. So some of the consequences of having long standing
dysautonomia mass cells and hypermobility leads to cranial cervical instability and airway loss and
even CSF spikes and leaks and pressure. So all these different symptoms can be due to all the
different pathologies that kind of circle around dysautonomia. So that's I think a really important
concept. And you know, this leads to this concept I have what's called the pentad. And this picture
shows the five entities of the pentad where you've got the dysautonomia, the mass cell activation,
the hypermobility or full Ehlers-Danlos. And those three things together often lead to some pretty
significant GI dysmotility. And also autoimmunity is there. And some of that autoimmunity may
start the process, but certainly later on autoimmunity comes into the picture as a consequence
of these other things. And all these different arrows are basically showing their specific pathways.
In fact, I've got them all named here. Specific pathways on how one plays off the other. One
causes the other. And so you can see these three pathways here, dysautonomia leading to GI dysmotility,
which activates mass cells and mass cells then cause a dysautonomia. So this cycle by itself is a
very big problem, but it certainly goes in all different directions, one causing the other.
Did I answer that question or? You did. And while we're on the slide, I mean, you've got the vagal
nerve identified on here. And that's something, I mean, anybody that's been on Instagram recently,
it's the vagal nerve is everywhere. You know, activate your vagal nerve, tap into your vagal nerve.
First of all, what is the vagus nerve? And, you know, how is it working with all of these different,
I guess, connections here? Okay. Well, this is the, a little diagram here of the autonomic nervous
system that splits up into that parasympathetic and sympathetic. And if we're talking about mainly
the parasympathetics being dysfunctional, you can see this particular nerve here, this very big one
that goes to just about every organ in the body. They're showing it going to the lungs and heart and
GI tract here, but it really innervates even, you know, the sweat glands and, and certainly the
arteries and veins in the body are controlled by the vagus nerve. So vagus nerve, cranial nerve
number 10, very big nerve in the body, when it becomes dysfunctional, the entire parasympathetic
nervous system becomes dysfunctional. And so that's what that's about. It runs on something called
acetylcholine. So there's that end nerve terminus of the vagal nerve, and it's showing acetylcholine
being produced that then acts on the target tissue, whatever that might be, maybe the sinus node of the
heart. So it puts a break on the heart of the sinus node of the heart to slow down a racing heart.
So we generally think of a paucity, a lack of acetylcholine when the parasympathetic, when the
vagus nerve is not working well. So anything that can enhance acetylcholine production can be very
helpful there. So that's where a lot of people are, you know, focusing on how do we enhance the
activity of the vagus nerve? You enhance that activity, then this idea of the sympathetic overdrive,
adding to the problems, and here's all the problems that sympathetic overdrive has to,
palpitations, racing heart, anxiety, insomnia, suddenly hyperventilating. So breathing faster
than you should. There's the connection with breathing again, putting someone on the edge of
a panic attack, creating migraine headaches and worsening brain fog because low carbon dioxide
in the blood from hyperventilation clamps down blood vessels to the head and changes your sensory
nerve function so that you feel, you know, your numbness in your fingers and toes and maybe even
in your face as well, where it feels like you're on the edge of a panic attack all the time.
And with the migraine headaches and worsening brain fog, one of the strongest determinants of
cerebral blood flow is your carbon dioxide level. When you hyperventilate, those blood vessels clamp
down and you can't get blood to your brain. So you're in a fog and it causes migraines.
This can even be determined when I exercise patients and see their CO2. It should be
40 and even getting behind in the middle of exercise. They retain CO2 a little bit because
they can't keep up. And these patients hyperventilate all through all stages of their exercise test,
where they're, you know, even low CO2s and when everybody else is high with their CO2.
Wow. That is one of the connections with the airway that is found in this autonomia.
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As a reminder, this podcast and the opinions expressed here are not a medical diagnosis.
If you suspect your child might have an airway issue, contact your pediatric airway dentist
or pediatrician. And now let's jump back into today's podcast.
So just to kind of put you on the spot then, I'm not saying things we see on Instagram,
please nobody take this and run off to your doctor, are accurate, but are some of the
concepts accurate? Can we actually stimulate our vagus nerve by doing things like where we put our
tongue in our mouth or breathing exercises, things like that? Yeah, sure. And there's multiple
different programs that have been developed and everybody has their own go to program that they
have tried with or without success. And that's all to modulate the vagus nerve. So DNRS, the Stanley
Rosenberg method, the Gupta program, LENS, brain tap, cranial sacral therapy to some extent,
there's books on it that I have listed here. And I will encourage the patient who has the
capability of engaging with these programs and being patient with themselves and with the programs
to do this because any help would get this way, it means less medication. So that's a good thing.
True. So we've touched a little bit on how breathing can impact this, but sleep can also
impact these disorders. Oh, yes. Yeah, I mean, of course, sleep becomes a major issue for a lot
of patients. Partly is that they're in this sympathetic overdrive. So they have this insomnia
where their body just will not settle. So they have basically fight or flight turned on all the
time. And so their body is under a threat. And so they can't settle, or if they do sleep, they'll
wake up easily. Now, part of that is because of this concept of airway loss. And we can talk about
all the reasons why these patients have an airway loss. But again, the body's basically saying,
I cannot let this person sleep because they're going to lose their airway. And that's going to
be a very, very big problem for this person. So they again can't sleep because of that.
And then the other issue would be if they do go to sleep and they don't fully obstruct,
then they have what's called upper airway resistance syndrome. And that is basically
a mild form of obstructive sleep apnea, where you don't actually have full apnick episodes,
where you arrest your breathing and then gasp for air, like full obstructive sleep apnea.
Instead, you just underventilate. And in this case, you retain carbon dioxide and your CO2
in your blood is too high. And blood vessels then open up to your brain and allow excessive blood
flow into your brain case, which is a fixed space. And there's a concept called the Monroe Kelly
Doctrine, which basically states that you can only fit so much inside a brain case. And that's
brain, CSF, and blood. And so if I don't have it on this set of slides. So if you open up the
blood vessels to the head and allow lots and lots of blood flow to occur while you're sleeping
and retaining CO2, then it has to displace cerebral spinal fluid. In the meantime, as it's trying
to displace this, your intracranial pressure goes up. So these patients have signs and symptoms of
increased intracranial pressure. And if the CSF can't get out of the way fast enough,
then it has to find a way to leak out. And so these patients tend to leak out the ends of
the cuffs of their cranial nerves and particularly olfactory nerve across the cribsiform plate into
their nasal sinuses. So some of these patients wake up in the morning with a gusher fluid coming
out their nose that's unexplained. And it's CSF that's basically leaked out of their
where it should be in their brain case. You're saying a literal gush. We're not talking a running
nose. A literal gush. Everybody's different. Certainly I have patients who sit up, lean
forward, and they'll have a gusher fluid. And the problem is, it's too random and too
surprising that they can't grab the sterile tube that I have waiting for them
to catch the gush. Some have and three or four times we've now measured it for the,
you know, what's called beta-transferrin, which should prove or disprove that it's CSF.
Unfortunately, every time it comes back, beta-transferrin negative, and you know,
one person may say, well, that proves it's not CSF. But the trouble is, is there's a
enzyme that very easily scrubs beta-transferrin, breaks it down very easily. And the second
highest concentration of that in the body is actually in our nasal mucosa in our lymphatics.
And so any CSF percolating through the nasal mucosa is going to have the beta-transferrin
scrubbed from it. So it turns out negative. So we're still trying to prove it, but that's
kind of where that stands. Wow. Okay. All right. So let's talk a little bit about some of the
consequences of this autonomic. Yeah. Well, we can go back to the slide that
shows all the symptoms and basically say, okay, all those symptoms are consequences.
And you say, yeah, but some of those are mast cell and some of those are hypermobility.
Yes, but you saw the other slide of how the interplay of between the five entities. So
even if you only started with this autonomia over time, you activate mast cells over time,
a lot of hypermobility issues start to arise. It may not be generalized. You may not show,
hey, I can now touch the floor with my hands when I couldn't before. I mean, not be able to show,
hey, all my joints are now extra flexible. One of the common is development of
temporal mandibular joint TMJ issues. So let's start to click, pop their jar,
have pain on eating. That's a very common one. And the other is what's happening to your airway.
So you start to develop a hypermobile components of the structures of your airway.
So you lose your airway more than you used to. And part of that is this development of what's
called cranial cervical instability. And let me see if I got a nice slide of that.
Okay. So here we go, cranial cervical instability. You've got the skull here. This is the base of
the skull showing and then C1 and C2. And as the ligaments start to tenderize, and that's often
from mast cells being activated and tenderizing the ligaments that hold the vertebrae, the cervical
vertebrae in place, they start to tenderize and C1 slips forward. And it has a lot of consequences.
One is that it compresses the internal jugular vein and that's the main
route for the CSF to escape the brain case. So it's actually compressed and preventing
CSF from escaping. The other is it flattens out the vertebral basilar artery. This is
blood flow to the brainstem. So you get sort of poor blood flow to the brainstem causing a lot of
symptoms from that. And that's a lot of that is due to consciousness and brainstem activities.
And then the final is what is being compressed next to that vein. And that's three important
cranial nerves, 9, 10, 11. So this little transverse process starts to injure cranial nerves 9, 10,
11. Well, we already said cranial nerve 10 is the vagus nerve. So here's all going full circle,
the dysautonomia activated mast cells, mast cells, tenderized connective tissue creating a hypermobile
state. And now this hypermobile state is turning around and injuring the vagus nerve creating
dysautonomia. So there's a full circle there for you. Only one example of several. And the
interesting thing is these patients have 9 and 11 also injured. So when you see a dysautonomia
patient that also has signs of cranial nerve 9 and 11 injured, then you know, that should
flag that, hey, now the dysautonomia is arising from this injury right here at the, as the, as
these nerves are exiting the skull. So cranial nerve nine injury is oftentimes those that an
ENT might think of. So this, these would be one of, one of the branches, the cranial nerve 9
goes back up to the middle ear. And it causes vertigo, hyperacusis, phonophobia, middle ear pain,
tinnitus. So when a person has all those symptoms, sometimes something called patchless
eustachian tube, which is where their eustachian tube is stuck open. And so in doing so, they can
hear themselves chewing, they can hear themselves talking inside their own head, and they can hear
themselves even breathing. And okay, now you got your eustachian tube stuck open, another sign of
a cranial nerve 9 injury. And then cranial nerve 11 is the nerve that's a motor nerve that goes to
your trapezius muscle. And so if it's injured, it creates this naughtiness of the trapezius muscle,
this really tight soreness in the trapezius. And we've all probably heard of this thing called
coat hanger pain that dysautonomia patients get, particularly POTS physiology patients get coat
hanger pain. And that's kind of a fatigue angina type, you know, soreness, the same, same type of
pain you would have if you're experiencing heart pain from angina. It's kind of like the same kind
of pain occurring in the shoulders, but it's not does that doesn't include naughtiness. So coat,
this is distinct from coat hanger pain that these patients have. And it's very naughty, tight muscles
that, you know, when they're trying to get a massage, or they're working with physical therapists,
they say, I can't get these knots out, they're just really, really tight. So if you have a patient
that has that, and they have the ear findings, and the other things that go with cranial nerve 9
outside the ear would be globus, a mass in the back of the throat, pain at the base of the tongue.
So these are all other cranial nerve 9 injury symptoms. Then you really know, okay, this
dysautonomia is arising right at where this CCI is occurring. All right, so that all
kinds of that on airway, because let's see if I have any, I don't think I have any pictures in
this slide, but when the when the C1 slides forward, it actually takes away airway space as well.
And some of these patients, when you do what's called a cone beam CT scan to assess airway,
you'll see the narrowest portion of the airway being with this C1 pressing into the back.
And so sometimes the loss is up front from a floppy tongue from a TMJ that slides backwards,
and or just from airway tissue being extra mobile, but sometimes it's the C1 from the rear.
Yeah, and I'll put a link to that for our parents to take a look at. We've seen some of those images
with Dr. Liao, for example, that they're CBTs, they're fascinating to look at.
Okay. So with these symptoms, what kind of treatments are available?
Okay, well, you know, when you get down to the idea of treatment, management, again, kind of has
to be bigger than just straight dysautonomia, you have to manage those other four entities that are
usually associated with it. But you also have to take into account the grade, right? You're not
wanting to do a whole lot for someone who has grade one dysautonomia, other than, you know,
making sure they're eating right, hydrating well, extra salt in their diet, exercising,
particularly leg strengthening or thigh strengthening exercises like squats and leg presses and
leg extensions, those are a big part of that, sleeping well. So those are all the lifestyle
strategies of grade one dysautonomia and grade two, like I said, oftentimes the athlete who has that,
and so they may have to add compression garments, which athletes often wear because of that's
occurring because of their grade two dysautonomia that they need those. Then getting into grade three,
where it's harder to manage and you need more strategies or even grade four, where you're even
needing picks and ports and G tubes and GJ tubes and wheelchairs and, you know, walking devices and
things like that. So, you know, you have to take that into consideration where they are. And this
little graph here kind of gives you that idea of just how much therapy you need. But the next concept
is, you know, what are you targeting? Are you targeting way back at the patient's genetics?
Are you targeting the triggers, which might be, you know, something in the environment, you know?
And if a patient seems to be environmentally exposed, then you remove that from the environment or,
as I've had more success, removing the patient from the environment, which I call location sabbatical,
and that's often successful. Then having attention to some of their various metabolic needs,
like finding out their iron is lower, their vitamin D is low, or they have trouble methylating and
things like that. So, things about their genetics or their current nutritional state that need
attention. Then attention to the consequences of those environment being triggered and activating
things like mast cells. So, now you got leaky gut, you've got mast cell activation. So, attacking
those. And then finally, directing therapy to the symptoms themselves. And a lot of my strategy
is directed to the symptoms. And that's simply because we can get some instantaneous improvement
with directing toward the symptoms. But also, just a lot of our therapies, at least in Western
Medicine, that's what it's geared toward. You know, you have dizziness upon standing,
let's give a medicine that improves dizziness upon standing. You have nausea, let's give a medicine
that suppresses nausea on and on and on for the symptoms. Now, to some extent, it's more than just
attacking the symptoms because if you make a person feel better, then they can do more physical
activity. And maybe the physical activity is going to help these things upstream. Or if you,
you know, make their GI tract feel better and suppress their nausea, they can actually eat and
get some good nutrition in. So, there are reasons to attack the symptoms themselves that have long
term consequences. But you definitely want to be working on this earlier stuff here. So, that's a
concept that you really want to impress upon, you know, other providers. We didn't really get into
this idea that some of this symptoms that lead to this phenomenon called POTS,
some of it is dysfunctional autonomic nervous system, and some of it is just the connective
tissue issue, too stretchy of connective tissues. But every patient, when you assess them, you have
to decide, you got to come to some sort of assessment of how much of their symptoms are
due directly to autonomic dysfunction and how much is actually because they now have or always have
had stretchy, hyper-compliant connective tissue. Because if all your therapy is directed toward
restoring autonomic function, yet most of their symptoms are rising because they have extra stretchy
tissue, you're not going to get anywhere. You're only going to put on therapies that are potentially
causing side effects, but not helpful. And so, some of this time you have to say, okay, a lot of this
is connective tissue, autonomic dysfunction therapies are not going to help here. Let's concentrate
on what we do for extra stretchy vessels. And before we get too far away from the connective
tissue, just for a parent that might, and I say this knowing that I could be one of those parents,
what's the difference between you have a child that can put some incredibly bending, the very,
very limber versus, okay, now we've passed into connective tissue. For example, again,
I'm going back to Instagram that says, if you can do this with your thumb, you have some sort of
connective tissue. Well, I don't, I can just do that with my thumb. So how can you tell the difference?
Yeah, well, certainly doing what's called a bite and score is helpful. And I don't dismiss that.
I do bite and scores on every single patient that comes in. And that includes, you know, how far
can you touch your thumb to your forearm? Can you bend your pinky back beyond 90 degrees?
Can you flex your arms when they're outstretched beyond 180 degrees, right? You know, beyond the
plane that they should be stopping at. Can you bend your knees back? Can you touch the floor
with your palms with your legs straight? Those are all the components of the bite and score.
And certainly, it's helpful to get a bite and score and determine how hypermobile someone is.
But a lot of my patients only show their hypermobility in their nasal pharyngeal region.
So they don't, they're not generalized hypermobile, but they have TMJ dislocation,
they have signs of cranial cervical instability, and they have airway loss,
all everything to do with their, their nasal pharyngeal region. And so, you know, is that because
the offending agent is actually being breathed in, you know, and, and mass cells are being
activated locally to tenderize tissue in the nasal pharyngeal region rather than general.
Now, for some people, even if that's the entry point of the offending agent,
they still become generalized hypermobile over time. Everybody's different. And then there's a
whole other set of patients who are actually hypomobile. They're stiff. They say, I can't
have, there's Danilos because I, I'm stiff. It's like, yes, because your body has compensated
for the hypermobility by developing too tight of muscles. The muscles are trying to take the place
of what the ligaments are failing at. So almost a musculoskeletal version of sympathetic
overdrive, right? So when the parasympathetic is not working, the sympathetic goes into overdrive
and causes all kinds of problems. So this is, you know, hypermobility leading to, it's not like a
conscious decision that the person made is that some people will respond to hypermobility by developing
their muscles becoming taunt and trying to stabilize very important joints. And so they're
stiff. So that has to be looked at and considered. Wow. Okay. So typically at the end of a podcast,
I always hand the floor back over to our guests because y'all are the experts. So is there,
is there a last thought or anything that you would like to leave with parents with regards to
disorder? Sure. Well, I mean, since this is an airway focused talk, I'd like to summarize,
you know, exactly how all of this impacts the airway. I've said a lot of it, how summarize it.
I mean, first you've got this issue of parasympathetic overdrive that's causing someone to hyperventilate.
So in the daytime, wall conscious, usually in an upright position, someone is hyperventilating and
breathing too quickly, driving down their CO2 and causing a lot of symptoms from a low CO2
and a patent airway. Okay. So everything's patent in that position and wall conscious.
Then you go to sleep at night and the various forces that take over, which might be a floppy
airway, it might be a tongue that falls back or in a lot of our patients, they've had some sort of
retractive, you know, orthodontics done. So they've lost mouth space from retractive orthodontics.
So their tongue never really fits in their mouth. Well, even when they're conscious and awake,
but certainly when they're sleeping and it falls back, a bigger problem, or they may have tonsils
that are too big. So, you know, extra tonsillar tissue and that of course is more common in the
mast cell activation patient. And they have floppy, they have a TMJ that falls back for a lot of
these patients. It's their ligaments to their jaw joint or lax enough that the TMJ actually
falls back significantly enough to cause airway obstruction or contribute to it.
The rest of their musculature in their neck is extra floppy. And so they're losing,
they're airway that way. And then as I mentioned, a lot of them have cranial cervical instability
where C1 slips forward and compresses from behind. So all of that leads to an airway loss at night
and under ventilation, rise in their CO2, hyperemia, which means, you know, extra blood flow into the
brain case and displacement of CSF. A lot of symptoms, particularly the grade four dysautonomia
patients, that's where their symptoms are mainly arising from is this flip-flop of low CO2 in the
day to high CO2 at night and back to low when they wake up and back to high when they go to sleep.
So back and forth spikes and leaks in CSF was what I call spiky leaky syndrome. And so
all of that is airway. And so tackling the airway has a big impact on preventing this
spiky leaky. Yes, doing whatever it takes to slow down the excessive breathing during the day will
help, but also preventing the nighttime retention of CO2 by whatever means. The first strategy,
of course, is trying CPAP and BiPAP. Of course, that's not a long-term strategy,
but finding ways to open the airway when they're sleeping at night so they don't under ventilate
can prevent this spike in leak phenomenon. And I know I said I was going to give you a little
last word, I totally lied. If a parent suspects that their child might have any of these conditions,
where do they go first? Well, I mean, there's certainly a lot of complex care doctors around
the country who know these concepts. We, a lot of us are on the same groups and talk to each other
by email groups and such. And so we're all aware of each other's thought processes along these lines.
So a lot of those doctors are very helpful for this, and that includes everything from
western-trained doctors that may be in institutions to private, but western-trained,
to naturopaths, to the whole gamut. If they understand these concepts, they can be very helpful.
I think the helpfulness comes from the healthcare provider's willingness to cross
traditional silos of healthcare. For instance, I'm a cardiologist, and I cannot stop at being a
cardiologist to help these patients. In fact, whenever I see a patient, I say, the last place
you want to look for the problem is the heart. The heart's working perfectly well. And so there's
nothing wrong with the heart. It's everything else, but you have chest pain, you have shortness of
breath, you have exercise intolerance, the kind of thing that you'd come to a cardiologist for.
And if I'm not willing to work outside the cardiology silo, then you're not going to get much
help from me, and you're not going to get much help from any other doctor who stays inside their silo.
So that's really what you're looking for is the doctor who understands how broad this is,
and that they're very comfortable working across different organ systems, no matter what their
original training was. Awesome. Well, I thank you so much for coming on and sharing all this
information. And for any of our providers that are listening, I know Dr. Maxwell will be at
a few conferences in 2024. So I'm also going to include those links and the show notes so that
y'all can attend, see him in person. So thank you so much for being here.
Well, I love talking about this stuff. So thank you.
Thanks again to today's guest, Dr. Andrew Maxwell for sharing his medical insight into each of you
for listening to today's episode. If you're new to our podcast, please don't forget to subscribe.
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Take care, stay safe, and happy breathing everyone.