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The ME/CFS/FM/POTS/Long COVID Septad – a Treatment Roadmap

From Unraveled A Patreon Broadcast from Dr. Kaufman and Dr. Ilene Ruhoy

The Unraveled Podcasts: two experts discussing complex chronic illnesses over dozens of podcasts.

Dr. David Kaufman MD – A published researcher as well as physician, Dr. Kaufman spent many years immersed in HIV/AIDS research and treatment in New York serving in various Directorships. From 1988 to 2005, he was the prinicipal investigator in 15 HIV/AIDS trials. He has over 35 years of experience in healthcare administration, management, and research, and his clinical experience spans Internal Medicine, HIV, Lyme Disease, and chronic illness.

In 2012, seeking something new, he moved to the San Francisco Bay area and began to focus on chronic fatigue syndrome and allied disorders at the Open Medicine Institute. In 2017, he and Dr. Chheda opened the Center for Complex Diseases focusing on ME/CFS, dysautonomia, autoimmune diseases, chronic infectious diseases, small intestine bacterial overgrowth, and mast cell activation syndrome (MCAS).

Dr. Ilene Ruhoy MD PhD – Originally from New York City, Ilene S. Ruhoy, MD, PhD trained in both pediatric and adult neurology at the University of Washington and Seattle Children’s Hospital, then received her PhD in Environmental Toxicology. She also has a certification in acupuncture, and graduated from the University of Arizona Integrative Medicine Fellowship. A board-certified neurologist, she is the founder and medical director of the Center for Healing Neurology, an integrative neurology practice in Seattle, and is the medical director for the EDS/Chiari Center at Mt. Sinai South Nassau Hospital.

Dr. Kaufman – an internist – and Dr. Ruhoy – a neurologist – began a more or less weekly Patreon channel called Unraveled: Understanding Complex Illness in December of last year, in which they discuss disorders like ME/CFS, hypermobility disorder, mast cell activation syndrome, small fiber neuropathy, postural orthostatic tachycardia syndrome (POTS), infections and environmental exposures. The podcasts include some live interactive sessions in which patients/caregivers can ask questions. It’s a rare chance to see two experts who are deeply immersed in these diseases publicly dig into their many different aspects. They state:

“We are two docs from New York now working in Seattle. As New Yorkers, we talk a lot, we talk fast, we interrupt each other, and we have a lot of fun. While we are passionate about our work, we also love to laugh and hope you will laugh with us as you learn”.

When I asked Dr. Kaufman if it was okay to cover some of the podcasts on Health Rising, he said it was, and added this:

“Our goal, our reason for doing this, is to get the word out there and not just to patients but to their families and especially to other physicians. I am quite passionate about the desperate need to recruit more physicians to this work both for ME/CFS and the exploding epidemic of Long Covid. In addition to pulling/luring physicians into this work–which is incredibly challenging and rewarding–I want to help educate primary care physicians since they are–or should be–the backbone and foundation of healthcare in general and particularly for this patient population.”

If it’s appropriate, please share the podcast with your doctor! Those who watch the podcast will surely be encouraged to see two such intelligent and likable doctors discussing them in such an easygoing and informative manner – I certainly was.

These doctors are on the cutting edge. Seeing patients day in and day out, they are, in many ways, ahead of the science. Their unique experience shows up in their framing of the key, interconnected factors present they believe permeate these illnesses.  (Note how many of these were hardly in the discussion even for ME/CFS/FM experts ten years ago). Of the seven factors of the Septad, only four (infection, dysautonomia/POTS, autoimmunity, small fiber neuropathy) are being pursued with any vigor at all in ME/CFS research.

These are, of course, their own views, and other doctors may view these diseases differently. Readers should also note that the following is attached to the beginning and end of every podcast.

If I remember correctly, subscribing to the podcast on Patreon costs $9 a month with a discount for a year. (If you’re cash-strapped, you could subscribe for a month once or twice a year and catch up on the podcasts in that way.) Check out the Unraveled podcast here.

The Septad – Podcast #3, January 1st 2023

The Septad (or septet :)) presents a “A classic set of concerns that these patients present with” Dr. Ruhoy

An Origin Story

Dr. Kaufman reported that about 4 years ago, 20 doctors got together to talk about MCAS and set up listserv. Within 2 or 3 months, Andy Maxwell, an East Bay cardiologist – “a totally brilliant guy” – puts an email up asking if every mast cell patient had these characteristics. The light bulb went on and Kaufman realized that his patient had these characteristics…So emerged “the Septad”.

Kaufman stated that the Septad “creates a map that allows the physician…to organize what I’ve heard in a much more usable and actionable way” for patients with chronic illness. The seven all interact – they are all connected and come together to create these diseases.

#1. Hypermobility Syndrome

“What we don’t appreciate enough about connective tissues is that they are everywhere” – Dr. Ruhoy

Kaufman and Ruhoy went way beyond the conventional hypermobility (the thumb bent back to the wrist type of thing). Most of the hypermobility they’re talking about is invisible. The connective tissue that are affected are everywhere – they line our gut, our nerves, our muscles, our brain…They keep our organs in place.

The iceberg model of Ehlers-Danlos Syndromes (EDS) and Hypermobility spectrum disorder (HDS). Joint hypermobility can cause many problems.

Their conception goes way beyond how flexible you are; you don’t have to be really flexible to have a connective tissue problem. The converse is true – being really flexible doesn’t mean that you have a connective tissue disorder either – you can be hypermobile and be completely healthy – check out the gymnasts in the Olympics. (I’ve heard the same is true for POTS.  Some people with high heart rates when upright are completely healthy as well).

Being hypermobile only becomes a disease state when the other things (infections, contaminants, etc.) trigger an inflammatory response that targets the connective tissue. It’s the other six things that turn hypermobility into a disease state – and it all starts with inflammation…

#2. Mast Cell Activation Syndrome/Mast Cell Activation Disorder

“Mast cell activation is a critical part of the septad.”

A mast cell that’s ready to pop? While mast cells can emit 1000s of substances, the blue blobs are filled with histamine.

Because connective tissues just happen to be filled with lots of mast cells, the connective tissue issue leads right into the second spoke of the wheel so to speak – mast cells. The skin, gut, nasal mucosa – that are exposed to the environment and stretch and move – are filled with mast cells. They are also part of the innate immune system = first responders. Any kind of traumatic event can call them forth.

Mast cells are an ancient part of our immune system and they don’t produce just histamine. They contain 1000s of chemicals and if they get annoyed, they can dump them all into the bloodstream.

Dr. Kaufman suggested picturing millions of mast cells releasing thousands of chemicals – many of which are pro-inflammatory and some of which damage the connective tissues – throughout your body and you get a picture of how impactful mast cells can be. Mast cell activation, then, “a critical part of the septad”.

For more on mast cells:

The Strange Syndrome: MCAS, ME/CFS, Fibromyalgia and Long COVID

 

#3 Postural Orthostatic Tachycardia Syndrome (POTS)

“I think POTS is one of the greatest drivers of their illness. It’s vastly underrated and misunderstood… and until I fix or manage, I should say, their POTS, I can’t make progress…and I can’t deal with other things. I can’t treat their infection until I get their POTS down.” Dr. Kaufman

Ruhoy remembered when POTS was thought to be a more psychological disorder. Not so anymore. There are several different types of POTS – if your heartbeat only goes up 22 beats, you can still have POTS.  If you stand up and your rate goes from 52 to 72 and stays there – that is not normal. Don’t let your doctor tell you that. Your brain is not getting enough oxygen.

Dr. Kaufman really emphasized how important he thinks POTS is:

“I think POTS is so beyond important. If you as a patient say something about POTS and the doctor doesn’t know about it or doesn’t believe in it – you try to finish quickly and get out….there’s no point in continuing.”

As an editorial aside, he said there’s no excuse for an internal medicine, primary care doctor, or neurologist not knowing about POTS in this day and age.

Could a POTS/Long COVID/ ME/CFS Connection Explain Much?

The POTS discussion naturally leads to one on…

#4 Small intestinal bowel overgrowth (SIBO) and Gastroparesis

“I believe that leaky gut and POTS are huge drivers of the disability in our patients.” Dr. Kaufman

The circle continues with Dr. Kaufman’s next “big one” SIBO (and acid reflux), as both are caused by autonomic nervous dysfunction which causes reduced gut motility (the ability to move the food along), which results in dysbiosis (unhealthy gut flora) and SIBO. Reduced gut motility allows the “little bugs”, as Dr. Kaufman put it, to swim up from the colon into the small intestine where they don’t belong.

Dr Kaufman laid it out nicely. The bacteria from the colon enter the rich realm of the small intestine where they metabolize the foods producing substances that help break down the gut wall, causing leaky gut. Once that happens, the “you know what hits the fan”. Lipopolysaccharides from gut bacteria enter the blood, triggering an inflammatory response including a leaky blood-brain barrier and probably mast cell activation. (Note that you can have SIBO without having any gut symptoms.)

Plus, as Dr. Ruhoy pointed out, the small intestine is filled with mast cells – and connective tissues line the gut wall. Kaufman said that he believes that leaky gut and POTS are huge drivers of the disability in his patients.

The origin of the SIBO problem – a gut motility problem caused by autonomic nervous system issues – makes treating SIBO a challenge. Getting rid of the bugs, Dr. Kaufman, asserted, is a “piece of cake”. While it can take months to do that, keeping the SIBO from coming back is the real challenge.

Add in the gut-brain and gut-immune interface that’s also present, and you can see how crucial a player the gut can be.

#5 Autoimmunity

Dr. Kaufman believes autoimmunity drives POTS, which probably figures in the dysautonomia, and the systemic inflammation and complaints that patients have that are often so vague, discomfiting, and challenging. For me, it was great to hear someone provide an explanation for the many weird, almost indescribable symptoms that people with these illnesses experience.

Dr. Ruhoy believes that mast cells recruit other immune cells to the fight – causing the immune system to be very “autoreactive” and producing things like Sjogren’s autoantibodies. Its not that everyone with Sjogren’s Syndrome antibodies has Sjogren’s Syndrome, but their presence constitutes “trouble” – and perhaps could eventually turn into Sjogren’s or other autoimmune illnesses.

Kaufman is a big believer in autoantibodies being related to POTS and uses a German laboratory.

Dysautonomia International Conference Pt III: The Autoimmunity Revolution in POTS

#6 Infection

“If I have a patient who is not getting better, I will start over and look for infection again.” Dr. Kaufman

Dr. Kaufman – It’s critically important to look for evidence of infection. They could be as simple as recurrent strep throat or herpes virus, or tick-born infections. Virtually everyone has EBV, HHV-6, HSV-1, and CMV and sometimes they reactivate. The big rabbit hole of rabbit holes – the tick-borne infections – appear to be like the herpesvirus infections in that they can become reactivated as well.

Once you start looking for virus-triggered chronic illnesses, you can find them. They are nothing new. Dr. Ruhoy noted that many post-infectious neurological disorders exist and mentioned acute disseminated encephalomyelitis (ADEM) – an MS-like condition that occurs 1-3 weeks after an infection, and febrile infection-related epilepsy syndrome (FIRES). These conditions are recognized throughout the medical community because the localized, objective damage they produce has been easy to spot. That’s something that ME/CFS and its allied diseases lack at this point.

Diagnosis continues to be a problem. There are antibodies, for instance, for tick-borne infections, but attaining the gold standard for pathogen detection – using PCR and culture – is very difficult. Herpesviruses are in a bit better shape, but relying on the gold standard – using PCR to find the bug – still means that you can miss people with reactivated herpesviruses using these techniques. The vagueness makes it more difficult for physicians.

Still, Dr. Kaufman said, “If I have a patient who is not getting better, I will start over and look for infection again”.

Small Fiber Neuropathy (SFN)

Small fiber neuropathy (SFN) – damage to the small unmyelinated nerve fibers in our skin, eyes, and many other places – can contribute to dysautonomia, but Dr. Ruhoy focused on pain production. The majority of her patients have SFN. (SFN first showed up in our class of diseases in fibromyalgia. It’s FM’s contribution to ME/CFS, POTS, and long COVID).

Unlike the vagueness that using antibody results entails, the diagnosis of SFN is more straightforward: it’s a histologic diagnosis (structural) made under a microscopic. The pain presentation from SFN is different in these diseases than in other disorders. For one, it’s more diffuse in and does not work its way up from the feet. It’s probably linked to autoimmunity and mast cell activation.

Immune Cells May Be Killing the Small Nerve Fibers in Fibromyalgia

We are dozens of episodes behind…

Take the Poll! Tell us how many of the “Septad” your health care provider is assessing…

THE SEPTAD POLL

YesNo
Hypermobility
Mast cell activation
POTS/Dysautonomia
Small intestinal bowel overgrowth
Autoimmunity
Infection
Small fiber neuropathy

Results – I don’t know why the results are not showing but here they are so far.  Thus far POTS is getting assessed quite a bit while hypermobility, SIBO, and small fiber neuropathy are not getting assessed much. They are below.

How many of the “Septad” is your health provider assessing?
Yes No
Hypermobility 95 (27%) 259
Mast cell activation 124 (35%) 230
POTS/Dysautonomia 215 (61%) 139
Small intestinal bowel overgrowth 89 (25%) 265
Autoimmunity 149 (42%) 205
Infection 157 (44%) 197
Small fiber neuropathy 78 (22%) 276

Health Rising is not affiliated with or receive any funding from the Unraveled Patreon series. 

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