1 00:00:00,000 --> 00:00:08,160 If you want, created a memes feature, then push that tube and add it to the fel- 2 00:00:08,160 --> 00:00:33,480 Hi everyone, and welcome back to Airway First, the podcast from the Children's Airway First 3 00:00:33,480 --> 00:00:41,040 Foundation. I'm your host, Rebecca St. James. My guest today is board-certified pediatric 4 00:00:41,040 --> 00:00:49,080 cardiologist and pediatrician, Dr. Andrew Maxwell. Dr. Maxwell received his medical degree 5 00:00:49,080 --> 00:00:56,240 from Johns Hopkins Medical School and a residency in pediatrics at the University of California 6 00:00:56,240 --> 00:01:02,480 at San Francisco, followed by a clinical and research fellowship in pediatric cardiology 7 00:01:02,480 --> 00:01:09,200 at Lucille-Sotler Packard and Stanford Hospitals and Children's Hospital of Philadelphia. His 8 00:01:09,200 --> 00:01:17,160 research interests include study of endothelial control, evasomotor tone, nitric oxide, sports 9 00:01:17,160 --> 00:01:25,840 cardiology, dysautonomia, hypermobility syndromes, and mass cell activation syndromes. For his 10 00:01:25,840 --> 00:01:33,920 research, he received an American Heart Association Burger Award for Research in Molecular Biology 11 00:01:33,920 --> 00:01:41,120 and was an American College of Cardiology Young Investigator Award finalist. Additionally, 12 00:01:41,120 --> 00:01:47,200 he has published many articles and book chapters on these subjects. For his clinical work, 13 00:01:47,200 --> 00:01:54,920 he has been voted by his peers as a top doctor in Northern California annually since 2017. 14 00:01:54,920 --> 00:02:02,240 You can find out more about Dr. Maxwell at heartofthevalley.us. Now, let's jump into 15 00:02:02,240 --> 00:02:05,440 my interview with Dr. Andrew Maxwell. 16 00:02:05,440 --> 00:02:11,040 All right. Great. Thank you for joining us today, Dr. Maxwell. I appreciate you taking 17 00:02:11,040 --> 00:02:12,280 some time to be here. 18 00:02:12,280 --> 00:02:14,840 Thank you for inviting me. 19 00:02:14,840 --> 00:02:19,720 Absolutely. All right. I'm just going to jump right into it because there's a lot to talk 20 00:02:19,720 --> 00:02:26,720 about. This is a topic that I think a lot of our parents don't think about, but we've 21 00:02:26,720 --> 00:02:31,920 had enough that I've had questions specifically around this, so I really want to give it the 22 00:02:31,920 --> 00:02:36,600 full hour so we can really dig deep into this. I want to start off for those that don't know 23 00:02:36,600 --> 00:02:42,840 them. Let's define, and I have to look at it because I will slaughter it, otherwise autonomic 24 00:02:42,840 --> 00:02:48,440 dysfunction or dysautonomia, and how this relates to things like autoimmune disease 25 00:02:48,440 --> 00:02:52,000 or L-er's, Dan Lo, those type of conditions. 26 00:02:52,000 --> 00:02:59,360 Sure. Okay. Yeah. You didn't slaughter it at all. Autonomic dysfunction or dysautonomia. 27 00:02:59,360 --> 00:03:09,760 It's very much an umbrella term that is a collection of disorders of the autonomic nervous 28 00:03:09,760 --> 00:03:15,280 system. We have the autonomic nervous system and everything that runs in the background. 29 00:03:15,280 --> 00:03:20,160 Typically, we think of it as the parasympathetic and the sympathetic nervous system that make 30 00:03:20,160 --> 00:03:28,360 up that. There is the enteric nervous system that also is there as well. For the most part, 31 00:03:28,360 --> 00:03:34,560 the parasympathetic and the sympathetic nervous system are what we're talking about. Furthermore, 32 00:03:34,560 --> 00:03:40,280 we're usually talking about a dysfunction of the parasympathetic nervous system. A big 33 00:03:40,280 --> 00:03:48,400 part of that is the vagus nerve. We'll probably talk quite a bit about the vagus nerve there. 34 00:03:48,400 --> 00:03:53,800 That doesn't mean that the sympathetic is not also part of the problem because what 35 00:03:53,800 --> 00:04:01,080 we have is sympathetic overdrive trying to correct what the parasympathetics aren't doing 36 00:04:01,080 --> 00:04:05,400 correctly. The sympathetic gets into the picture as well. 37 00:04:05,400 --> 00:04:14,040 That's pretty high level. When we're talking about these kind of conditions, is this something 38 00:04:14,040 --> 00:04:19,200 that we see, and I'll just say just kind of as an umbrella of boys and girls. I know 39 00:04:19,200 --> 00:04:23,800 this could be young, this could be teenagers, but just in general, boys and girls, do you 40 00:04:23,800 --> 00:04:29,560 see this in both? Do you see this more in one? Is this something that you're born with 41 00:04:29,560 --> 00:04:32,240 or that you develop? How does this work? 42 00:04:32,240 --> 00:04:40,120 Well, it's in both boys and girls and it's of course all ages from birth till elderly, 43 00:04:40,120 --> 00:04:48,680 but there's definitely peaks and there is gender bias depending on underlying triggers 44 00:04:48,680 --> 00:04:56,400 and where you are in your age group. It's very, very common when you bring in all the 45 00:04:56,400 --> 00:05:02,200 different levels, what I call grades of dysautonomia. I've traditionally graded this from 46 00:05:02,200 --> 00:05:08,480 zero to four and the meaning of each is zero would be no dysautonomy at all, no symptoms 47 00:05:08,480 --> 00:05:13,080 of dysfunction of the autonomic nervous system at all. 48 00:05:13,080 --> 00:05:20,240 Grade one being that of an otherwise normal healthy teenager. If we think about them getting 49 00:05:20,240 --> 00:05:25,280 head rushes upon standing up and losing their vision, sometimes even fainting, what we call 50 00:05:25,280 --> 00:05:32,560 vasovagal syncope, which is very common in teens as well as maybe even their fatigue 51 00:05:32,560 --> 00:05:44,280 and their tendency to want to sleep long hours, maybe a form of mild autonomic dysfunction. 52 00:05:44,280 --> 00:05:53,280 Athletes we even see this more. There's grade two dysautonomia, which is really just more 53 00:05:53,280 --> 00:05:58,000 than grade one, more than the typical teen, but not yet grade three, which I'll define 54 00:05:58,000 --> 00:06:05,000 in a moment. Athletes are often in the grade two category where they're even more dysautonomic, 55 00:06:05,000 --> 00:06:11,360 they're more standing up and losing their vision and more maybe tendency to faint and 56 00:06:11,360 --> 00:06:22,000 needing extra help to manage their autonomic dysfunction. Teen girls are often found to 57 00:06:22,000 --> 00:06:31,200 be grade two and a lot of that has to do with development of estrogen cycling. Their bodies 58 00:06:31,200 --> 00:06:39,440 are struggling with the development of their cycle. Also, it happens on top of rapid growth 59 00:06:39,440 --> 00:06:49,200 in height and both those play into developing more than the standard teen dysautonomia. 60 00:06:49,200 --> 00:06:56,000 Then there's grade three and the definition of that is it's now interfering with school 61 00:06:56,000 --> 00:07:01,280 sports and social life. When you're so symptomatic that you're quitting your sport because you 62 00:07:01,280 --> 00:07:08,640 feel lousy at an upright sport particularly or you're missing a lot of days of school 63 00:07:08,640 --> 00:07:14,480 or getting behind, maybe it's because of foggy thinking, maybe it's because just feeling lousy 64 00:07:14,480 --> 00:07:22,080 when waking up with nausea and inability breakfast and things like that. Or you're telling your 65 00:07:22,080 --> 00:07:27,680 friends, look, I cannot hang out with you guys because I pay the price over the next couple 66 00:07:27,680 --> 00:07:33,440 of days if I stay out late. That's starting to interfere with school sports and social life. 67 00:07:33,440 --> 00:07:43,360 That's grade three and that is where we see a lot of the illnesses that are requiring a lot of 68 00:07:43,360 --> 00:07:50,800 medical attention to get them to be able to stay in school in sports. Then finally, grade four is 69 00:07:50,800 --> 00:07:59,840 where it's so significant that someone is requiring instrumentation or other items like, for instance, 70 00:07:59,840 --> 00:08:07,040 G-tubes and NG-tubes and walking assistants or maybe even they're just bedbound. 71 00:08:07,040 --> 00:08:16,560 So grade four is pretty significant interference with life and often requires a lot of 72 00:08:19,680 --> 00:08:27,440 therapies of various types. When you look at the whole wide spectrum of dysautonomia, you can see, 73 00:08:27,440 --> 00:08:36,000 yeah, dysautonomia is almost ubiquitous around us if you include the grade ones and grade twos. 74 00:08:36,000 --> 00:08:42,160 Now, a lot would say, well, okay, yeah, those are just the normal development and expected and 75 00:08:42,160 --> 00:08:49,200 it doesn't require much more than some lifestyle modifications to improve those. So if we eliminate 76 00:08:49,200 --> 00:08:56,240 grades one and two and just focus on three and four, then of course it's much less common, 77 00:08:56,240 --> 00:09:01,280 but still pretty common. And of course, I have a clinic full of these patients and they were 78 00:09:01,280 --> 00:09:08,400 and they were coming to me long before I became what someone may view as someone to seek out for 79 00:09:08,400 --> 00:09:16,000 this. So just in my area as a cardiologist, having all these patients coming for shortness of breath 80 00:09:16,000 --> 00:09:23,360 and exercise intolerance and chest pain and palpitations, all the things you would see a 81 00:09:23,360 --> 00:09:29,280 cardiologist for and the clinic is just full of these patients from my normal cardiology catch 82 00:09:29,280 --> 00:09:35,760 area. So it's pretty common. And you said something there that I want to touch on. 83 00:09:36,720 --> 00:09:41,840 When you're talking about kids coming to you because they're having exercise exhaustion, 84 00:09:42,800 --> 00:09:49,760 could these kids be coming to you because they're told they have what is exercise induced asthma 85 00:09:49,760 --> 00:09:54,000 or something like that. Otherwise, they're normal, they function normally, you know, 14, 86 00:09:54,000 --> 00:10:00,080 15, 16 year old. Yeah, of course. I mean, that's the that would be the very first thing to assess 87 00:10:00,080 --> 00:10:05,760 when someone comes in with exertional shortness of breath. Okay, well, do you have them the 88 00:10:05,760 --> 00:10:13,040 single most common reason for that, which is undiagnosed or oftentimes remote history of asthma 89 00:10:13,040 --> 00:10:18,960 as a kid. And now they had no idea that this is coming back in a different form, that they're not 90 00:10:18,960 --> 00:10:24,720 wheezing, then they're not acting like they had asthma when they were little. But now they're 91 00:10:24,720 --> 00:10:32,080 exercised, induced shortness of breath. And we might do very commonly do PFTs before and after 92 00:10:32,960 --> 00:10:39,840 albuterol or Zopin X, and do exercise testing to see if we can bring that out. So that would be 93 00:10:41,040 --> 00:10:46,320 a very, very common outcome of a patient that comes in with shortness of breath. But usually, 94 00:10:46,320 --> 00:10:52,400 I can tell those patients because it's only one single symptom, or maybe they have chest tightness 95 00:10:52,400 --> 00:10:58,160 as well, that kind of picks them apart, as opposed to those who are coming in with chronic fatigue 96 00:10:58,160 --> 00:11:03,760 and dizzy upon standing and palpitations with minor activity or no activity at all. So those 97 00:11:04,320 --> 00:11:10,800 patients with broader symptoms are usually, now there's I mean, they can't have both. And certainly, 98 00:11:10,800 --> 00:11:16,320 you know, very often, I'm like, well, yeah, you have all this autonomia, you have all these problems. 99 00:11:16,320 --> 00:11:22,240 But yes, you also have some, we need some attention to your pulmonary function too. And so you're 100 00:11:22,240 --> 00:11:28,080 going to benefit to some extent from an inhaler as well. And just to make sure that I'm saying this 101 00:11:28,080 --> 00:11:32,480 correctly as we continue our discussion, it's correct to call these disorders, correct? 102 00:11:33,600 --> 00:11:38,800 Not, is it, do I call it disorders or is it dysfunction? What would be the right term? 103 00:11:38,800 --> 00:11:43,440 Oh, I think, I think it's fair to call it either one of those. Okay. 104 00:11:43,440 --> 00:11:47,280 Disfunction disorders, I think are synonymous enough that you can use it either one. 105 00:11:47,840 --> 00:11:53,680 Okay, excellent. So these disorders, is this something that is typically, 106 00:11:54,880 --> 00:12:00,640 a child is born with? You know, can you catch this early? Or does something in life just trigger it? 107 00:12:00,640 --> 00:12:04,400 I mean, I know within girls, you know, you mentioned puberty can often 108 00:12:04,400 --> 00:12:08,720 impact it, but is there something that triggers these and maybe makes it 109 00:12:08,720 --> 00:12:15,920 worse in some children? That's part of the evaluation that I do and I think should be done is, 110 00:12:15,920 --> 00:12:21,200 okay, what are the genetics of the person? What is the environmental exposure that may even occurred 111 00:12:21,840 --> 00:12:29,520 from birth on? And so a lot of these patients will get some genetic testing done and find that they 112 00:12:29,520 --> 00:12:36,320 have either a connective tissue issue that makes them more susceptible to what's going to happen 113 00:12:36,320 --> 00:12:41,360 in the future with dysautonomia, or they have sort of an obligate, what's called mass cell 114 00:12:41,360 --> 00:12:45,840 activation syndrome, which eventually we're going to probably get into with some of these questions, 115 00:12:46,800 --> 00:12:53,920 that activates mass cells and mass cells then lead downstream to connective tissue disorders 116 00:12:53,920 --> 00:13:06,640 and directly to dysautonomia. So a lot of it can be somewhat pre-ordained at birth based on genetics, 117 00:13:06,640 --> 00:13:14,080 but I really do think that it's a genetics meets environment issue. We're perfectly healthy, 118 00:13:14,080 --> 00:13:18,720 what I call the canary in the coal mine. So if you think about a canary is a perfectly healthy 119 00:13:18,720 --> 00:13:24,560 bird, nothing wrong with a canary. It has a nice life unless it's put into a bad environment like 120 00:13:24,560 --> 00:13:30,800 a coal mine where it's the first person to become sick. And that is what a lot of these patients are, 121 00:13:30,800 --> 00:13:36,240 they're canaries in coal mine where they're born with genetic variants that if they're in the right 122 00:13:36,240 --> 00:13:42,000 environment, they're going to thrive and not have a problem. But they may not thrive so well in a, 123 00:13:42,000 --> 00:13:53,920 for some, a very mildly toxic environment, maybe mold in their environment or as I am trying to 124 00:13:53,920 --> 00:13:58,720 investigate whether even just the presence of blue-green algae blooms with cyanotoxins in the water 125 00:14:00,960 --> 00:14:06,000 could affect these canaries with their genetics that make them particularly susceptible. 126 00:14:06,000 --> 00:14:12,400 And so for some of them, it starts at a very, very early age. A lot of these patients will have 127 00:14:12,960 --> 00:14:20,240 either the history of, yeah, they had GI issues from practically birth where they weren't 128 00:14:20,240 --> 00:14:26,880 tolerating formulas and it was a struggle to get the right foods and calories into them to grow. 129 00:14:26,880 --> 00:14:33,600 And they've always had abdominal bloating and sensitivity to foods and all that. 130 00:14:33,600 --> 00:14:44,240 Or they end up with a pan's-pandis-like picture where they're showing early on OCD type characteristics, 131 00:14:44,240 --> 00:14:50,480 ticks, clinginess to parents and all the things that you would use to give a clinical diagnosis 132 00:14:50,480 --> 00:14:58,800 of pans or pandas. And that is maybe the six or seven or eight-year-old that's showing that 133 00:14:58,800 --> 00:15:05,440 and they may have had GI issues before that. But then by the time they're hitting nine and above, 134 00:15:05,440 --> 00:15:12,720 they're starting to convert over to more of a what we call POTS picture. We haven't used that term yet, 135 00:15:12,720 --> 00:15:21,520 Postero-Orthostatic Tachycardia Syndrome, which is somewhat under the umbrella of dysautonomia. 136 00:15:21,520 --> 00:15:27,200 And I say that with a lot of caution because I give entire lectures on how POTS and dysautonomia 137 00:15:27,200 --> 00:15:37,920 are distinct and not real, one's not really a subset under the dysautonomia. But the point is, 138 00:15:37,920 --> 00:15:49,360 is these patients will transition from a pans to a POTS type of physiology. And that would be a very 139 00:15:49,360 --> 00:16:00,800 common way that young people enter into this problem. So there's that now. Now we have with COVID, 140 00:16:02,320 --> 00:16:08,560 we have everyone coming in from post COVID long haul and some coming in with, you know, vaccine 141 00:16:08,560 --> 00:16:15,680 induced vaccine triggered. I haven't seen it for a while now, but for the first year of the vaccine, 142 00:16:15,680 --> 00:16:21,920 there was a lot of patients coming in who were perfectly fine, but developed some dysautonomia, 143 00:16:21,920 --> 00:16:28,800 mass cell activation syndrome, immediately after having a vaccine. Those were usually short lived, 144 00:16:28,800 --> 00:16:36,240 what I call short COVID syndrome. But now we have so many that are triggered after having 145 00:16:36,240 --> 00:16:44,720 the COVID virus itself. And now a couple of years into dysautonomia. So. 146 00:16:44,720 --> 00:16:47,520 Wow. So the virus could actually trigger it. 147 00:16:47,520 --> 00:16:53,200 Yeah. Well, I mean, there's a lot of theories about how that occurs. Is it the virus itself still 148 00:16:53,200 --> 00:16:59,920 chronically living in the person and just low levels and causing dysfunction? Is it the spike 149 00:16:59,920 --> 00:17:04,320 protein that's been left behind and it just needs, the body needs to clean up the spike protein? And 150 00:17:04,320 --> 00:17:11,040 it's a spike protein is a very mass cell inflammatory agent. It activates mass cells. 151 00:17:11,680 --> 00:17:15,120 And if any of it's left around and the mass cells are easily triggered, 152 00:17:15,840 --> 00:17:21,600 their mass cells just continue to be activated and causing downstream effects, including 153 00:17:21,600 --> 00:17:29,280 dysautonomia. Then there's a question of whether COVID distracts the immune system and allows for 154 00:17:29,280 --> 00:17:36,480 other latent organisms to come out. So the most commonly thought of one would be Epstein-Barr virus. 155 00:17:37,040 --> 00:17:43,200 You know, most of us have converted from negative to positive Epstein-Barr virus sometime in our 156 00:17:43,200 --> 00:17:51,280 teens and have fought off the mono infection. And now the virus is just living within our 157 00:17:51,920 --> 00:17:58,080 nerve cell bodies in our immune system keeps that in check, except maybe during something 158 00:17:58,080 --> 00:18:03,680 like COVID, it distracts that and allows those to come out. And of course, we see the 159 00:18:05,360 --> 00:18:10,800 similar situation with people who have had varicella zoster. Also a virus that just lives 160 00:18:10,800 --> 00:18:17,200 in dormant in our nerve cells until our immune system is distracted and it comes out again as 161 00:18:17,200 --> 00:18:23,920 shingles, right? So people might have that virus come out and you have shingles. And of course, 162 00:18:23,920 --> 00:18:30,480 we see that with an aged population where their immune system is not so much distracted, it is 163 00:18:30,480 --> 00:18:39,760 just waning and the varicella zoster virus comes out. So it's very similar concept when we talk 164 00:18:39,760 --> 00:18:46,480 about COVID allowing for Epstein-Barr virus to come out again and basically cause a chronic 165 00:18:46,480 --> 00:18:55,680 mono and maybe the basis of a lot of chronic fatigue syndrome. Okay. Well, then I guess this 166 00:18:55,680 --> 00:19:04,880 seems like a good time to segue into how does dysautonomia affect the organs within the body? 167 00:19:04,880 --> 00:19:16,240 I'll dive right into that. Okay. Well, okay. Well, I mean, one of the concepts that I like to 168 00:19:17,760 --> 00:19:26,480 teach or get this concept across is that it's very hard to have dysautonomia by itself, that there's 169 00:19:26,480 --> 00:19:34,240 usually this at least combination of the mass cell activation and dysautonomia that occur together. 170 00:19:34,240 --> 00:19:41,520 And then there's eventually some connective tissue issues come into play as well. And 171 00:19:42,800 --> 00:19:45,840 I have a probably some slides that might be worth 172 00:19:46,880 --> 00:19:55,200 Oh, yes, please showing here that highlights that. If I could just find one. Okay. This would 173 00:19:55,200 --> 00:20:04,480 be a good one. So I'm going to share my screen here. And it's going to be this one. And while 174 00:20:04,480 --> 00:20:08,400 you're pulling that up, I'm going to let our listeners know that I'll put a link to a book 175 00:20:08,400 --> 00:20:13,520 that you did a chapter on that we're discussing excerpts from. I'll disjoint it. 176 00:20:14,400 --> 00:20:21,200 But they might be interesting. Okay. So you see my slides. Okay. There I do. Yes. Okay. So 177 00:20:21,200 --> 00:20:30,480 So when you say how are organ systems affected, I mean, when we're talking about the autonomic 178 00:20:30,480 --> 00:20:35,920 nervous system, you're pretty much talking about every single organ system in the body is affected 179 00:20:35,920 --> 00:20:40,720 when the autonomic nervous system isn't working well. And so here's all the different organ 180 00:20:40,720 --> 00:20:47,760 systems broken down and all the symptoms that may fall under those organ systems. But I've color 181 00:20:47,760 --> 00:20:52,560 coded them according to how much is actually due to dysautonomia, which is in the light blue, 182 00:20:53,120 --> 00:20:57,440 versus how much of those symptoms might be occurring from the mass cell activation that 183 00:21:00,800 --> 00:21:08,000 the copathology of mass cell activation versus those who develop some hypermobility or even 184 00:21:08,000 --> 00:21:13,440 full blown Ehlers-Danlos. Some of those symptoms are coming from that. And the final category, 185 00:21:13,440 --> 00:21:20,240 this green is sort of the consequences of there's upper airway resistance syndrome. There's the airway 186 00:21:20,240 --> 00:21:27,600 issue and cranial cervical instability. So some of the consequences of having long standing 187 00:21:27,600 --> 00:21:35,280 dysautonomia mass cells and hypermobility leads to cranial cervical instability and airway loss and 188 00:21:35,280 --> 00:21:45,360 even CSF spikes and leaks and pressure. So all these different symptoms can be due to all the 189 00:21:45,360 --> 00:21:51,280 different pathologies that kind of circle around dysautonomia. So that's I think a really important 190 00:21:51,920 --> 00:21:59,600 concept. And you know, this leads to this concept I have what's called the pentad. And this picture 191 00:21:59,600 --> 00:22:05,600 shows the five entities of the pentad where you've got the dysautonomia, the mass cell activation, 192 00:22:05,600 --> 00:22:11,920 the hypermobility or full Ehlers-Danlos. And those three things together often lead to some pretty 193 00:22:11,920 --> 00:22:20,080 significant GI dysmotility. And also autoimmunity is there. And some of that autoimmunity may 194 00:22:20,080 --> 00:22:27,040 start the process, but certainly later on autoimmunity comes into the picture as a consequence 195 00:22:27,040 --> 00:22:32,880 of these other things. And all these different arrows are basically showing their specific pathways. 196 00:22:32,880 --> 00:22:40,800 In fact, I've got them all named here. Specific pathways on how one plays off the other. One 197 00:22:40,800 --> 00:22:47,680 causes the other. And so you can see these three pathways here, dysautonomia leading to GI dysmotility, 198 00:22:47,680 --> 00:22:54,240 which activates mass cells and mass cells then cause a dysautonomia. So this cycle by itself is a 199 00:22:54,240 --> 00:23:00,800 very big problem, but it certainly goes in all different directions, one causing the other. 200 00:23:03,120 --> 00:23:08,400 Did I answer that question or? You did. And while we're on the slide, I mean, you've got the vagal 201 00:23:08,400 --> 00:23:16,000 nerve identified on here. And that's something, I mean, anybody that's been on Instagram recently, 202 00:23:16,000 --> 00:23:20,800 it's the vagal nerve is everywhere. You know, activate your vagal nerve, tap into your vagal nerve. 203 00:23:20,800 --> 00:23:28,800 First of all, what is the vagus nerve? And, you know, how is it working with all of these different, 204 00:23:31,120 --> 00:23:42,720 I guess, connections here? Okay. Well, this is the, a little diagram here of the autonomic nervous 205 00:23:42,720 --> 00:23:47,760 system that splits up into that parasympathetic and sympathetic. And if we're talking about mainly 206 00:23:47,760 --> 00:23:54,400 the parasympathetics being dysfunctional, you can see this particular nerve here, this very big one 207 00:23:54,400 --> 00:23:59,680 that goes to just about every organ in the body. They're showing it going to the lungs and heart and 208 00:23:59,680 --> 00:24:08,080 GI tract here, but it really innervates even, you know, the sweat glands and, and certainly the 209 00:24:08,080 --> 00:24:15,520 arteries and veins in the body are controlled by the vagus nerve. So vagus nerve, cranial nerve 210 00:24:15,520 --> 00:24:22,240 number 10, very big nerve in the body, when it becomes dysfunctional, the entire parasympathetic 211 00:24:22,240 --> 00:24:29,200 nervous system becomes dysfunctional. And so that's what that's about. It runs on something called 212 00:24:29,200 --> 00:24:38,800 acetylcholine. So there's that end nerve terminus of the vagal nerve, and it's showing acetylcholine 213 00:24:38,800 --> 00:24:46,800 being produced that then acts on the target tissue, whatever that might be, maybe the sinus node of the 214 00:24:47,360 --> 00:24:53,680 heart. So it puts a break on the heart of the sinus node of the heart to slow down a racing heart. 215 00:24:54,480 --> 00:25:02,000 So we generally think of a paucity, a lack of acetylcholine when the parasympathetic, when the 216 00:25:02,000 --> 00:25:09,440 vagus nerve is not working well. So anything that can enhance acetylcholine production can be very 217 00:25:09,440 --> 00:25:14,320 helpful there. So that's where a lot of people are, you know, focusing on how do we enhance the 218 00:25:14,320 --> 00:25:22,480 activity of the vagus nerve? You enhance that activity, then this idea of the sympathetic overdrive, 219 00:25:24,240 --> 00:25:27,840 adding to the problems, and here's all the problems that sympathetic overdrive has to, 220 00:25:27,840 --> 00:25:34,400 palpitations, racing heart, anxiety, insomnia, suddenly hyperventilating. So breathing faster 221 00:25:34,400 --> 00:25:38,480 than you should. There's the connection with breathing again, putting someone on the edge of 222 00:25:38,480 --> 00:25:43,840 a panic attack, creating migraine headaches and worsening brain fog because low carbon dioxide 223 00:25:43,840 --> 00:25:50,720 in the blood from hyperventilation clamps down blood vessels to the head and changes your sensory 224 00:25:50,720 --> 00:25:57,520 nerve function so that you feel, you know, your numbness in your fingers and toes and maybe even 225 00:25:57,520 --> 00:26:04,400 in your face as well, where it feels like you're on the edge of a panic attack all the time. 226 00:26:04,400 --> 00:26:09,920 And with the migraine headaches and worsening brain fog, one of the strongest determinants of 227 00:26:09,920 --> 00:26:15,360 cerebral blood flow is your carbon dioxide level. When you hyperventilate, those blood vessels clamp 228 00:26:15,360 --> 00:26:20,720 down and you can't get blood to your brain. So you're in a fog and it causes migraines. 229 00:26:21,920 --> 00:26:27,360 This can even be determined when I exercise patients and see their CO2. It should be 230 00:26:27,360 --> 00:26:31,840 40 and even getting behind in the middle of exercise. They retain CO2 a little bit because 231 00:26:31,840 --> 00:26:40,000 they can't keep up. And these patients hyperventilate all through all stages of their exercise test, 232 00:26:40,000 --> 00:26:45,280 where they're, you know, even low CO2s and when everybody else is high with their CO2. 233 00:26:45,280 --> 00:27:01,200 Wow. That is one of the connections with the airway that is found in this autonomia. 234 00:27:15,440 --> 00:27:31,360 You're listening to Airway First, the podcast from the Children's Airway First Foundation. 235 00:27:32,000 --> 00:27:37,360 You can find out more about CAF and our mission to fix before six on our website at 236 00:27:37,360 --> 00:27:44,560 childrensairwayfirst.org. The CAF website offers tons of great resources for both parents and 237 00:27:44,560 --> 00:27:50,480 medical professionals. In our parents portal and clinicians corner, you can find educational 238 00:27:50,480 --> 00:27:56,080 and informational content, including videos, blogs, our recommended reading lists, comprehensive 239 00:27:56,080 --> 00:28:01,840 medical research, podcasts, events, parent support, and educational opportunities. 240 00:28:03,120 --> 00:28:07,680 Parents are also encouraged to join the Airway Huddle, our Facebook support group, 241 00:28:07,680 --> 00:28:11,520 which was created for parents of children with airway and sleep-related issues. 242 00:28:11,520 --> 00:28:18,960 You can access the Airway Huddle support group at facebook.com backslashgroups backslash airway 243 00:28:18,960 --> 00:28:25,200 huddle. Are you interested in being a guest on the show? Then shoot us a note via the contacts 244 00:28:25,200 --> 00:28:32,000 page on our website or send us an email directly at infoatchildrensairwayfirst.org. 245 00:28:33,120 --> 00:28:38,480 As a reminder, this podcast and the opinions expressed here are not a medical diagnosis. 246 00:28:38,480 --> 00:28:43,440 If you suspect your child might have an airway issue, contact your pediatric airway dentist 247 00:28:43,440 --> 00:29:13,280 or pediatrician. And now let's jump back into today's podcast. 248 00:29:13,280 --> 00:29:18,400 So just to kind of put you on the spot then, I'm not saying things we see on Instagram, 249 00:29:18,400 --> 00:29:24,480 please nobody take this and run off to your doctor, are accurate, but are some of the 250 00:29:24,480 --> 00:29:31,440 concepts accurate? Can we actually stimulate our vagus nerve by doing things like where we put our 251 00:29:31,440 --> 00:29:39,120 tongue in our mouth or breathing exercises, things like that? Yeah, sure. And there's multiple 252 00:29:39,120 --> 00:29:48,560 different programs that have been developed and everybody has their own go to program that they 253 00:29:49,120 --> 00:30:01,120 have tried with or without success. And that's all to modulate the vagus nerve. So DNRS, the Stanley 254 00:30:01,120 --> 00:30:11,280 Rosenberg method, the Gupta program, LENS, brain tap, cranial sacral therapy to some extent, 255 00:30:11,280 --> 00:30:19,200 there's books on it that I have listed here. And I will encourage the patient who has the 256 00:30:19,200 --> 00:30:25,760 capability of engaging with these programs and being patient with themselves and with the programs 257 00:30:25,760 --> 00:30:32,000 to do this because any help would get this way, it means less medication. So that's a good thing. 258 00:30:33,920 --> 00:30:40,240 True. So we've touched a little bit on how breathing can impact this, but sleep can also 259 00:30:40,240 --> 00:30:48,960 impact these disorders. Oh, yes. Yeah, I mean, of course, sleep becomes a major issue for a lot 260 00:30:48,960 --> 00:30:56,720 of patients. Partly is that they're in this sympathetic overdrive. So they have this insomnia 261 00:30:56,720 --> 00:31:03,520 where their body just will not settle. So they have basically fight or flight turned on all the 262 00:31:03,520 --> 00:31:14,000 time. And so their body is under a threat. And so they can't settle, or if they do sleep, they'll 263 00:31:14,000 --> 00:31:20,320 wake up easily. Now, part of that is because of this concept of airway loss. And we can talk about 264 00:31:20,320 --> 00:31:28,800 all the reasons why these patients have an airway loss. But again, the body's basically saying, 265 00:31:28,800 --> 00:31:32,720 I cannot let this person sleep because they're going to lose their airway. And that's going to 266 00:31:32,720 --> 00:31:37,920 be a very, very big problem for this person. So they again can't sleep because of that. 267 00:31:37,920 --> 00:31:43,840 And then the other issue would be if they do go to sleep and they don't fully obstruct, 268 00:31:43,840 --> 00:31:48,800 then they have what's called upper airway resistance syndrome. And that is basically 269 00:31:48,800 --> 00:31:56,160 a mild form of obstructive sleep apnea, where you don't actually have full apnick episodes, 270 00:31:56,160 --> 00:32:01,600 where you arrest your breathing and then gasp for air, like full obstructive sleep apnea. 271 00:32:01,600 --> 00:32:10,160 Instead, you just underventilate. And in this case, you retain carbon dioxide and your CO2 272 00:32:10,160 --> 00:32:18,880 in your blood is too high. And blood vessels then open up to your brain and allow excessive blood 273 00:32:18,880 --> 00:32:25,120 flow into your brain case, which is a fixed space. And there's a concept called the Monroe Kelly 274 00:32:25,120 --> 00:32:33,440 Doctrine, which basically states that you can only fit so much inside a brain case. And that's 275 00:32:33,440 --> 00:32:42,400 brain, CSF, and blood. And so if I don't have it on this set of slides. So if you open up the 276 00:32:42,400 --> 00:32:47,920 blood vessels to the head and allow lots and lots of blood flow to occur while you're sleeping 277 00:32:47,920 --> 00:32:56,720 and retaining CO2, then it has to displace cerebral spinal fluid. In the meantime, as it's trying 278 00:32:56,720 --> 00:33:03,920 to displace this, your intracranial pressure goes up. So these patients have signs and symptoms of 279 00:33:04,560 --> 00:33:09,840 increased intracranial pressure. And if the CSF can't get out of the way fast enough, 280 00:33:09,840 --> 00:33:15,920 then it has to find a way to leak out. And so these patients tend to leak out the ends of 281 00:33:15,920 --> 00:33:22,640 the cuffs of their cranial nerves and particularly olfactory nerve across the cribsiform plate into 282 00:33:22,640 --> 00:33:29,360 their nasal sinuses. So some of these patients wake up in the morning with a gusher fluid coming 283 00:33:29,360 --> 00:33:35,680 out their nose that's unexplained. And it's CSF that's basically leaked out of their 284 00:33:37,360 --> 00:33:41,920 where it should be in their brain case. You're saying a literal gush. We're not talking a running 285 00:33:41,920 --> 00:33:48,480 nose. A literal gush. Everybody's different. Certainly I have patients who sit up, lean 286 00:33:48,480 --> 00:33:55,600 forward, and they'll have a gusher fluid. And the problem is, it's too random and too 287 00:33:57,600 --> 00:34:05,280 surprising that they can't grab the sterile tube that I have waiting for them 288 00:34:05,280 --> 00:34:11,920 to catch the gush. Some have and three or four times we've now measured it for the, 289 00:34:12,960 --> 00:34:17,840 you know, what's called beta-transferrin, which should prove or disprove that it's CSF. 290 00:34:18,400 --> 00:34:24,320 Unfortunately, every time it comes back, beta-transferrin negative, and you know, 291 00:34:25,040 --> 00:34:30,240 one person may say, well, that proves it's not CSF. But the trouble is, is there's a 292 00:34:30,240 --> 00:34:38,560 enzyme that very easily scrubs beta-transferrin, breaks it down very easily. And the second 293 00:34:38,560 --> 00:34:44,960 highest concentration of that in the body is actually in our nasal mucosa in our lymphatics. 294 00:34:44,960 --> 00:34:51,840 And so any CSF percolating through the nasal mucosa is going to have the beta-transferrin 295 00:34:51,840 --> 00:34:57,040 scrubbed from it. So it turns out negative. So we're still trying to prove it, but that's 296 00:34:57,040 --> 00:35:04,400 kind of where that stands. Wow. Okay. All right. So let's talk a little bit about some of the 297 00:35:04,400 --> 00:35:12,720 consequences of this autonomic. Yeah. Well, we can go back to the slide that 298 00:35:13,440 --> 00:35:18,320 shows all the symptoms and basically say, okay, all those symptoms are consequences. 299 00:35:18,320 --> 00:35:23,280 And you say, yeah, but some of those are mast cell and some of those are hypermobility. 300 00:35:23,280 --> 00:35:30,720 Yes, but you saw the other slide of how the interplay of between the five entities. So 301 00:35:30,720 --> 00:35:35,920 even if you only started with this autonomia over time, you activate mast cells over time, 302 00:35:36,720 --> 00:35:42,400 a lot of hypermobility issues start to arise. It may not be generalized. You may not show, 303 00:35:42,400 --> 00:35:48,400 hey, I can now touch the floor with my hands when I couldn't before. I mean, not be able to show, 304 00:35:48,400 --> 00:35:56,240 hey, all my joints are now extra flexible. One of the common is development of 305 00:35:58,160 --> 00:36:05,520 temporal mandibular joint TMJ issues. So let's start to click, pop their jar, 306 00:36:05,520 --> 00:36:10,800 have pain on eating. That's a very common one. And the other is what's happening to your airway. 307 00:36:10,800 --> 00:36:19,760 So you start to develop a hypermobile components of the structures of your airway. 308 00:36:19,760 --> 00:36:25,360 So you lose your airway more than you used to. And part of that is this development of what's 309 00:36:25,360 --> 00:36:30,080 called cranial cervical instability. And let me see if I got a nice slide of that. 310 00:36:30,080 --> 00:36:42,640 Okay. So here we go, cranial cervical instability. You've got the skull here. This is the base of 311 00:36:42,640 --> 00:36:49,840 the skull showing and then C1 and C2. And as the ligaments start to tenderize, and that's often 312 00:36:49,840 --> 00:36:58,640 from mast cells being activated and tenderizing the ligaments that hold the vertebrae, the cervical 313 00:36:58,640 --> 00:37:04,800 vertebrae in place, they start to tenderize and C1 slips forward. And it has a lot of consequences. 314 00:37:05,360 --> 00:37:09,120 One is that it compresses the internal jugular vein and that's the main 315 00:37:09,120 --> 00:37:15,920 route for the CSF to escape the brain case. So it's actually compressed and preventing 316 00:37:15,920 --> 00:37:21,520 CSF from escaping. The other is it flattens out the vertebral basilar artery. This is 317 00:37:21,520 --> 00:37:27,840 blood flow to the brainstem. So you get sort of poor blood flow to the brainstem causing a lot of 318 00:37:27,840 --> 00:37:34,000 symptoms from that. And that's a lot of that is due to consciousness and brainstem activities. 319 00:37:35,120 --> 00:37:44,240 And then the final is what is being compressed next to that vein. And that's three important 320 00:37:44,240 --> 00:37:52,160 cranial nerves, 9, 10, 11. So this little transverse process starts to injure cranial nerves 9, 10, 321 00:37:52,160 --> 00:37:58,320 11. Well, we already said cranial nerve 10 is the vagus nerve. So here's all going full circle, 322 00:37:58,320 --> 00:38:03,920 the dysautonomia activated mast cells, mast cells, tenderized connective tissue creating a hypermobile 323 00:38:03,920 --> 00:38:08,560 state. And now this hypermobile state is turning around and injuring the vagus nerve creating 324 00:38:08,560 --> 00:38:16,320 dysautonomia. So there's a full circle there for you. Only one example of several. And the 325 00:38:16,320 --> 00:38:24,720 interesting thing is these patients have 9 and 11 also injured. So when you see a dysautonomia 326 00:38:24,720 --> 00:38:30,000 patient that also has signs of cranial nerve 9 and 11 injured, then you know, that should 327 00:38:30,800 --> 00:38:36,960 flag that, hey, now the dysautonomia is arising from this injury right here at the, as the, as 328 00:38:36,960 --> 00:38:44,960 these nerves are exiting the skull. So cranial nerve nine injury is oftentimes those that an 329 00:38:44,960 --> 00:38:51,200 ENT might think of. So this, these would be one of, one of the branches, the cranial nerve 9 330 00:38:51,200 --> 00:38:59,920 goes back up to the middle ear. And it causes vertigo, hyperacusis, phonophobia, middle ear pain, 331 00:39:02,080 --> 00:39:07,760 tinnitus. So when a person has all those symptoms, sometimes something called patchless 332 00:39:07,760 --> 00:39:14,640 eustachian tube, which is where their eustachian tube is stuck open. And so in doing so, they can 333 00:39:14,640 --> 00:39:19,680 hear themselves chewing, they can hear themselves talking inside their own head, and they can hear 334 00:39:19,680 --> 00:39:28,320 themselves even breathing. And okay, now you got your eustachian tube stuck open, another sign of 335 00:39:28,320 --> 00:39:35,040 a cranial nerve 9 injury. And then cranial nerve 11 is the nerve that's a motor nerve that goes to 336 00:39:35,040 --> 00:39:42,320 your trapezius muscle. And so if it's injured, it creates this naughtiness of the trapezius muscle, 337 00:39:42,320 --> 00:39:49,440 this really tight soreness in the trapezius. And we've all probably heard of this thing called 338 00:39:49,440 --> 00:39:56,080 coat hanger pain that dysautonomia patients get, particularly POTS physiology patients get coat 339 00:39:56,080 --> 00:40:04,320 hanger pain. And that's kind of a fatigue angina type, you know, soreness, the same, same type of 340 00:40:04,320 --> 00:40:08,720 pain you would have if you're experiencing heart pain from angina. It's kind of like the same kind 341 00:40:08,720 --> 00:40:14,320 of pain occurring in the shoulders, but it's not does that doesn't include naughtiness. So coat, 342 00:40:14,320 --> 00:40:21,280 this is distinct from coat hanger pain that these patients have. And it's very naughty, tight muscles 343 00:40:21,280 --> 00:40:25,200 that, you know, when they're trying to get a massage, or they're working with physical therapists, 344 00:40:25,200 --> 00:40:31,040 they say, I can't get these knots out, they're just really, really tight. So if you have a patient 345 00:40:31,040 --> 00:40:38,160 that has that, and they have the ear findings, and the other things that go with cranial nerve 9 346 00:40:38,160 --> 00:40:44,320 outside the ear would be globus, a mass in the back of the throat, pain at the base of the tongue. 347 00:40:45,440 --> 00:40:51,600 So these are all other cranial nerve 9 injury symptoms. Then you really know, okay, this 348 00:40:52,400 --> 00:41:01,040 dysautonomia is arising right at where this CCI is occurring. All right, so that all 349 00:41:01,040 --> 00:41:07,680 kinds of that on airway, because let's see if I have any, I don't think I have any pictures in 350 00:41:07,680 --> 00:41:14,560 this slide, but when the when the C1 slides forward, it actually takes away airway space as well. 351 00:41:14,560 --> 00:41:20,400 And some of these patients, when you do what's called a cone beam CT scan to assess airway, 352 00:41:21,520 --> 00:41:28,480 you'll see the narrowest portion of the airway being with this C1 pressing into the back. 353 00:41:28,480 --> 00:41:36,720 And so sometimes the loss is up front from a floppy tongue from a TMJ that slides backwards, 354 00:41:36,720 --> 00:41:45,520 and or just from airway tissue being extra mobile, but sometimes it's the C1 from the rear. 355 00:41:46,960 --> 00:41:51,680 Yeah, and I'll put a link to that for our parents to take a look at. We've seen some of those images 356 00:41:51,680 --> 00:41:57,360 with Dr. Liao, for example, that they're CBTs, they're fascinating to look at. 357 00:41:57,360 --> 00:42:04,960 Okay. So with these symptoms, what kind of treatments are available? 358 00:42:05,520 --> 00:42:13,440 Okay, well, you know, when you get down to the idea of treatment, management, again, kind of has 359 00:42:13,440 --> 00:42:19,120 to be bigger than just straight dysautonomia, you have to manage those other four entities that are 360 00:42:19,120 --> 00:42:25,040 usually associated with it. But you also have to take into account the grade, right? You're not 361 00:42:25,040 --> 00:42:31,120 wanting to do a whole lot for someone who has grade one dysautonomia, other than, you know, 362 00:42:31,120 --> 00:42:36,240 making sure they're eating right, hydrating well, extra salt in their diet, exercising, 363 00:42:36,240 --> 00:42:41,440 particularly leg strengthening or thigh strengthening exercises like squats and leg presses and 364 00:42:42,240 --> 00:42:48,960 leg extensions, those are a big part of that, sleeping well. So those are all the lifestyle 365 00:42:48,960 --> 00:42:55,680 strategies of grade one dysautonomia and grade two, like I said, oftentimes the athlete who has that, 366 00:42:55,680 --> 00:43:02,320 and so they may have to add compression garments, which athletes often wear because of that's 367 00:43:02,320 --> 00:43:09,680 occurring because of their grade two dysautonomia that they need those. Then getting into grade three, 368 00:43:09,680 --> 00:43:15,440 where it's harder to manage and you need more strategies or even grade four, where you're even 369 00:43:15,440 --> 00:43:23,520 needing picks and ports and G tubes and GJ tubes and wheelchairs and, you know, walking devices and 370 00:43:23,520 --> 00:43:29,840 things like that. So, you know, you have to take that into consideration where they are. And this 371 00:43:29,840 --> 00:43:37,360 little graph here kind of gives you that idea of just how much therapy you need. But the next concept 372 00:43:37,360 --> 00:43:43,360 is, you know, what are you targeting? Are you targeting way back at the patient's genetics? 373 00:43:43,360 --> 00:43:48,000 Are you targeting the triggers, which might be, you know, something in the environment, you know? 374 00:43:48,720 --> 00:43:54,640 And if a patient seems to be environmentally exposed, then you remove that from the environment or, 375 00:43:55,280 --> 00:44:02,720 as I've had more success, removing the patient from the environment, which I call location sabbatical, 376 00:44:03,200 --> 00:44:10,560 and that's often successful. Then having attention to some of their various metabolic needs, 377 00:44:10,560 --> 00:44:18,960 like finding out their iron is lower, their vitamin D is low, or they have trouble methylating and 378 00:44:18,960 --> 00:44:27,120 things like that. So, things about their genetics or their current nutritional state that need 379 00:44:27,120 --> 00:44:34,640 attention. Then attention to the consequences of those environment being triggered and activating 380 00:44:35,200 --> 00:44:40,080 things like mast cells. So, now you got leaky gut, you've got mast cell activation. So, attacking 381 00:44:40,080 --> 00:44:48,800 those. And then finally, directing therapy to the symptoms themselves. And a lot of my strategy 382 00:44:49,440 --> 00:44:56,320 is directed to the symptoms. And that's simply because we can get some instantaneous improvement 383 00:44:56,320 --> 00:45:02,000 with directing toward the symptoms. But also, just a lot of our therapies, at least in Western 384 00:45:02,000 --> 00:45:07,360 Medicine, that's what it's geared toward. You know, you have dizziness upon standing, 385 00:45:07,360 --> 00:45:12,080 let's give a medicine that improves dizziness upon standing. You have nausea, let's give a medicine 386 00:45:12,080 --> 00:45:21,200 that suppresses nausea on and on and on for the symptoms. Now, to some extent, it's more than just 387 00:45:21,200 --> 00:45:27,600 attacking the symptoms because if you make a person feel better, then they can do more physical 388 00:45:27,600 --> 00:45:35,280 activity. And maybe the physical activity is going to help these things upstream. Or if you, 389 00:45:35,280 --> 00:45:39,360 you know, make their GI tract feel better and suppress their nausea, they can actually eat and 390 00:45:39,360 --> 00:45:45,840 get some good nutrition in. So, there are reasons to attack the symptoms themselves that have long 391 00:45:45,840 --> 00:45:54,160 term consequences. But you definitely want to be working on this earlier stuff here. So, that's a 392 00:45:54,160 --> 00:46:03,040 concept that you really want to impress upon, you know, other providers. We didn't really get into 393 00:46:05,280 --> 00:46:12,480 this idea that some of this symptoms that lead to this phenomenon called POTS, 394 00:46:13,040 --> 00:46:17,840 some of it is dysfunctional autonomic nervous system, and some of it is just the connective 395 00:46:17,840 --> 00:46:23,920 tissue issue, too stretchy of connective tissues. But every patient, when you assess them, you have 396 00:46:23,920 --> 00:46:32,160 to decide, you got to come to some sort of assessment of how much of their symptoms are 397 00:46:32,160 --> 00:46:37,920 due directly to autonomic dysfunction and how much is actually because they now have or always have 398 00:46:37,920 --> 00:46:45,920 had stretchy, hyper-compliant connective tissue. Because if all your therapy is directed toward 399 00:46:45,920 --> 00:46:51,600 restoring autonomic function, yet most of their symptoms are rising because they have extra stretchy 400 00:46:51,600 --> 00:46:56,480 tissue, you're not going to get anywhere. You're only going to put on therapies that are potentially 401 00:46:56,480 --> 00:47:02,400 causing side effects, but not helpful. And so, some of this time you have to say, okay, a lot of this 402 00:47:02,400 --> 00:47:08,080 is connective tissue, autonomic dysfunction therapies are not going to help here. Let's concentrate 403 00:47:08,080 --> 00:47:13,600 on what we do for extra stretchy vessels. And before we get too far away from the connective 404 00:47:13,600 --> 00:47:20,000 tissue, just for a parent that might, and I say this knowing that I could be one of those parents, 405 00:47:20,000 --> 00:47:25,440 what's the difference between you have a child that can put some incredibly bending, the very, 406 00:47:25,440 --> 00:47:30,480 very limber versus, okay, now we've passed into connective tissue. For example, again, 407 00:47:30,480 --> 00:47:35,840 I'm going back to Instagram that says, if you can do this with your thumb, you have some sort of 408 00:47:35,840 --> 00:47:41,440 connective tissue. Well, I don't, I can just do that with my thumb. So how can you tell the difference? 409 00:47:41,440 --> 00:47:49,360 Yeah, well, certainly doing what's called a bite and score is helpful. And I don't dismiss that. 410 00:47:49,360 --> 00:47:54,000 I do bite and scores on every single patient that comes in. And that includes, you know, how far 411 00:47:54,000 --> 00:48:00,160 can you touch your thumb to your forearm? Can you bend your pinky back beyond 90 degrees? 412 00:48:00,160 --> 00:48:06,560 Can you flex your arms when they're outstretched beyond 180 degrees, right? You know, beyond the 413 00:48:06,560 --> 00:48:13,440 plane that they should be stopping at. Can you bend your knees back? Can you touch the floor 414 00:48:13,440 --> 00:48:17,520 with your palms with your legs straight? Those are all the components of the bite and score. 415 00:48:17,520 --> 00:48:23,920 And certainly, it's helpful to get a bite and score and determine how hypermobile someone is. 416 00:48:24,480 --> 00:48:31,040 But a lot of my patients only show their hypermobility in their nasal pharyngeal region. 417 00:48:31,600 --> 00:48:36,320 So they don't, they're not generalized hypermobile, but they have TMJ dislocation, 418 00:48:36,320 --> 00:48:40,480 they have signs of cranial cervical instability, and they have airway loss, 419 00:48:40,480 --> 00:48:47,760 all everything to do with their, their nasal pharyngeal region. And so, you know, is that because 420 00:48:47,760 --> 00:48:54,240 the offending agent is actually being breathed in, you know, and, and mass cells are being 421 00:48:54,240 --> 00:49:01,760 activated locally to tenderize tissue in the nasal pharyngeal region rather than general. 422 00:49:02,480 --> 00:49:06,480 Now, for some people, even if that's the entry point of the offending agent, 423 00:49:06,480 --> 00:49:12,560 they still become generalized hypermobile over time. Everybody's different. And then there's a 424 00:49:12,560 --> 00:49:17,520 whole other set of patients who are actually hypomobile. They're stiff. They say, I can't 425 00:49:17,520 --> 00:49:24,080 have, there's Danilos because I, I'm stiff. It's like, yes, because your body has compensated 426 00:49:24,080 --> 00:49:29,680 for the hypermobility by developing too tight of muscles. The muscles are trying to take the place 427 00:49:30,240 --> 00:49:35,680 of what the ligaments are failing at. So almost a musculoskeletal version of sympathetic 428 00:49:35,680 --> 00:49:41,760 overdrive, right? So when the parasympathetic is not working, the sympathetic goes into overdrive 429 00:49:41,760 --> 00:49:47,360 and causes all kinds of problems. So this is, you know, hypermobility leading to, it's not like a 430 00:49:47,360 --> 00:49:55,600 conscious decision that the person made is that some people will respond to hypermobility by developing 431 00:49:56,640 --> 00:50:02,800 their muscles becoming taunt and trying to stabilize very important joints. And so they're 432 00:50:02,800 --> 00:50:15,440 stiff. So that has to be looked at and considered. Wow. Okay. So typically at the end of a podcast, 433 00:50:15,440 --> 00:50:21,520 I always hand the floor back over to our guests because y'all are the experts. So is there, 434 00:50:21,520 --> 00:50:27,120 is there a last thought or anything that you would like to leave with parents with regards to 435 00:50:27,120 --> 00:50:34,720 disorder? Sure. Well, I mean, since this is an airway focused talk, I'd like to summarize, 436 00:50:34,720 --> 00:50:40,400 you know, exactly how all of this impacts the airway. I've said a lot of it, how summarize it. 437 00:50:40,400 --> 00:50:49,280 I mean, first you've got this issue of parasympathetic overdrive that's causing someone to hyperventilate. 438 00:50:49,280 --> 00:50:56,400 So in the daytime, wall conscious, usually in an upright position, someone is hyperventilating and 439 00:50:56,400 --> 00:51:02,320 breathing too quickly, driving down their CO2 and causing a lot of symptoms from a low CO2 440 00:51:03,120 --> 00:51:08,720 and a patent airway. Okay. So everything's patent in that position and wall conscious. 441 00:51:09,520 --> 00:51:19,360 Then you go to sleep at night and the various forces that take over, which might be a floppy 442 00:51:19,360 --> 00:51:29,840 airway, it might be a tongue that falls back or in a lot of our patients, they've had some sort of 443 00:51:29,840 --> 00:51:38,240 retractive, you know, orthodontics done. So they've lost mouth space from retractive orthodontics. 444 00:51:38,240 --> 00:51:42,880 So their tongue never really fits in their mouth. Well, even when they're conscious and awake, 445 00:51:42,880 --> 00:51:48,720 but certainly when they're sleeping and it falls back, a bigger problem, or they may have tonsils 446 00:51:48,720 --> 00:51:53,360 that are too big. So, you know, extra tonsillar tissue and that of course is more common in the 447 00:51:53,360 --> 00:52:01,680 mast cell activation patient. And they have floppy, they have a TMJ that falls back for a lot of 448 00:52:01,680 --> 00:52:07,280 these patients. It's their ligaments to their jaw joint or lax enough that the TMJ actually 449 00:52:07,280 --> 00:52:12,160 falls back significantly enough to cause airway obstruction or contribute to it. 450 00:52:13,280 --> 00:52:18,320 The rest of their musculature in their neck is extra floppy. And so they're losing, 451 00:52:18,320 --> 00:52:23,120 they're airway that way. And then as I mentioned, a lot of them have cranial cervical instability 452 00:52:23,120 --> 00:52:30,640 where C1 slips forward and compresses from behind. So all of that leads to an airway loss at night 453 00:52:30,640 --> 00:52:39,520 and under ventilation, rise in their CO2, hyperemia, which means, you know, extra blood flow into the 454 00:52:39,520 --> 00:52:49,360 brain case and displacement of CSF. A lot of symptoms, particularly the grade four dysautonomia 455 00:52:49,360 --> 00:52:57,520 patients, that's where their symptoms are mainly arising from is this flip-flop of low CO2 in the 456 00:52:57,520 --> 00:53:03,360 day to high CO2 at night and back to low when they wake up and back to high when they go to sleep. 457 00:53:03,360 --> 00:53:11,280 So back and forth spikes and leaks in CSF was what I call spiky leaky syndrome. And so 458 00:53:12,000 --> 00:53:20,640 all of that is airway. And so tackling the airway has a big impact on preventing this 459 00:53:20,640 --> 00:53:27,200 spiky leaky. Yes, doing whatever it takes to slow down the excessive breathing during the day will 460 00:53:27,200 --> 00:53:36,320 help, but also preventing the nighttime retention of CO2 by whatever means. The first strategy, 461 00:53:36,320 --> 00:53:40,480 of course, is trying CPAP and BiPAP. Of course, that's not a long-term strategy, 462 00:53:41,680 --> 00:53:47,120 but finding ways to open the airway when they're sleeping at night so they don't under ventilate 463 00:53:47,760 --> 00:53:53,760 can prevent this spike in leak phenomenon. And I know I said I was going to give you a little 464 00:53:53,760 --> 00:54:00,960 last word, I totally lied. If a parent suspects that their child might have any of these conditions, 465 00:54:02,320 --> 00:54:10,560 where do they go first? Well, I mean, there's certainly a lot of complex care doctors around 466 00:54:10,560 --> 00:54:18,320 the country who know these concepts. We, a lot of us are on the same groups and talk to each other 467 00:54:18,320 --> 00:54:28,480 by email groups and such. And so we're all aware of each other's thought processes along these lines. 468 00:54:28,960 --> 00:54:39,200 So a lot of those doctors are very helpful for this, and that includes everything from 469 00:54:39,200 --> 00:54:47,920 western-trained doctors that may be in institutions to private, but western-trained, 470 00:54:47,920 --> 00:54:59,200 to naturopaths, to the whole gamut. If they understand these concepts, they can be very helpful. 471 00:54:59,200 --> 00:55:08,720 I think the helpfulness comes from the healthcare provider's willingness to cross 472 00:55:09,680 --> 00:55:16,000 traditional silos of healthcare. For instance, I'm a cardiologist, and I cannot stop at being a 473 00:55:16,000 --> 00:55:22,720 cardiologist to help these patients. In fact, whenever I see a patient, I say, the last place 474 00:55:22,720 --> 00:55:29,200 you want to look for the problem is the heart. The heart's working perfectly well. And so there's 475 00:55:29,200 --> 00:55:34,240 nothing wrong with the heart. It's everything else, but you have chest pain, you have shortness of 476 00:55:34,240 --> 00:55:39,120 breath, you have exercise intolerance, the kind of thing that you'd come to a cardiologist for. 477 00:55:39,120 --> 00:55:45,840 And if I'm not willing to work outside the cardiology silo, then you're not going to get much 478 00:55:45,840 --> 00:55:50,800 help from me, and you're not going to get much help from any other doctor who stays inside their silo. 479 00:55:50,800 --> 00:55:57,200 So that's really what you're looking for is the doctor who understands how broad this is, 480 00:55:57,200 --> 00:56:03,360 and that they're very comfortable working across different organ systems, no matter what their 481 00:56:03,360 --> 00:56:12,080 original training was. Awesome. Well, I thank you so much for coming on and sharing all this 482 00:56:12,080 --> 00:56:16,720 information. And for any of our providers that are listening, I know Dr. Maxwell will be at 483 00:56:16,720 --> 00:56:21,920 a few conferences in 2024. So I'm also going to include those links and the show notes so that 484 00:56:21,920 --> 00:56:25,920 y'all can attend, see him in person. So thank you so much for being here. 485 00:56:25,920 --> 00:56:28,880 Well, I love talking about this stuff. So thank you. 486 00:56:31,520 --> 00:56:38,160 Thanks again to today's guest, Dr. Andrew Maxwell for sharing his medical insight into each of you 487 00:56:38,160 --> 00:56:44,480 for listening to today's episode. If you're new to our podcast, please don't forget to subscribe. 488 00:56:44,480 --> 00:56:51,120 And if you enjoyed today's episode, leave us a review or comment telling us about what you enjoyed the most. 489 00:56:52,240 --> 00:56:57,440 You can stay connected with the Children's Airway First Foundation by following us on Instagram, 490 00:56:57,440 --> 00:57:05,200 Facebook, X, LinkedIn, and YouTube. Parents can also join us via our Facebook support group, 491 00:57:05,200 --> 00:57:10,640 the Airway Huddle, at facebook.com backslashgroups backslashairwayhuddle. 492 00:57:10,640 --> 00:57:15,600 You can find tons of great content for parents and medical professionals 493 00:57:16,160 --> 00:57:21,680 on our Parents Portal and Clinicians Corner on our website at childrensairwayfirst.org. 494 00:57:23,280 --> 00:57:26,720 If you'd like to be a guest or have an idea for an upcoming episode, 495 00:57:26,720 --> 00:57:32,720 shoot us a note via the contacts page on our website or send us an email directly at infoat 496 00:57:32,720 --> 00:57:40,080 childrensairwayfirst.org. And finally, thanks to all the parents and medical professionals out there 497 00:57:40,080 --> 00:57:44,240 that are working to help make the lives of kids around the globe just a little bit better. 498 00:57:44,240 --> 00:58:09,440 Take care, stay safe, and happy breathing everyone.