Author: pinksalt

POTS (Postural Orthostatic Tachycardia Syndrome) can cause a variety of symptoms. One of the most common ones (and personally, most frustrating ones) is brain fog.

What is Brain Fog?

Brain fog is described as “cognitive dysfunction”, in areas such as thinking, remembering, concentrating, and reasoning to a level that interferes with daily activities. (1) According to Vanderbilt’s Autonomic Dysfunction Clinic, 80-90% of POTS patients report brain fog as a symptom. Vanderbilt describes brain fog as “difficulty thinking, concentrating, or paying attention; trouble remembering things; cloudy or fuzzy feeling in head; and having problems finding the right words.” According to Dysautonomia International ,this can occur even while patients are lying down or seated, which limits their ability to attend school or to work.

How Does POTS Cause Brain Fog?

When a person stands up, gravity naturally pulls our blood down into the lower half of our bodies.

In a healthy individual, the body is able to compensate for this and push blood back up to the heart and the brain. In POTS and other Dysautonomias, there is a malfunction in the body’s ability to do this (for a variety of reasons). As such, blood instead pools in the lower extremities. Simply put, there isn’t enough blood getting up to the brain.

In POTS, there can be two reasons why this doesn’t happen:

1. Low blood volume (there’s not enough blood, so it can’t reach all the way up)

2. Loose blood vessels (lack of constriction doesn’t push blood back up)

Sources:

1. Dysautonomia International https://dysautonomiainternational.org/blog/wordpress/cognitive-dysfunction-and-brain-fog-in-pots/#:~:text=One%20of%20the%20most%20common,that%20interferes%20with%20daily%20activities.

Dysautonomia is an umbrella term used to describe several conditions with one common cause- a dysfunction of the autonomic nervous system, or ANS. POTS (Postural Orthostatic Tachycardia Syndrome) and NCS (Neurocardiogenic Syncope) are the two most common forms of Dysautonomia.

What is the Autonomic Nervous System?

The autonomic nervous system controls all of your body’s “automatic” functions, such as heart rate, breathing, blood pressure, digestion, and temperature control. It is divided into the Sympathetic and Parasympathetic nervous systems. The Sympathetic functions are “fight or flight” and Parasympathetic are “rest and digest”.

How Common is Dysautonomia?

Dysautonomia is NOT rare- it impacts an estimated 70+ million people worldwide.

What are the different types of Dysautonomia?

There are more than 15 different dysautonomias (3). Two of the most common ones are POTS (Postural Orthostatic Tachycardia Syndrome) and NCS (Neurocardiogenic Syncope).

POTS (Postural Orthostatic Tachycardia Syndrome) is estimated to impact 1 out of 100 teenagers and, including adult patients, a total of 1,000,000 to 3,000,000 Americans. POTS can cause lightheadness, fainting, tachycardia, chest pains, shortness of breath, GI upset, shaking, exercise intolerance, temperature sensitivity and more (1).

NCS (Neurocardiogenic Syncope) is the most common form of dysautonomia. It can cause fainting spells that happen once or twice in your lifetime or multiple times every day. NCS is also called situational syncope or vasovagal syncope (2).

What are the other types of Dysautonomia?

Some of the 15+ subtypes of Dysautonomia include:

POTS (Postural Orthostatic Tachycardia Syndrome), characterized by an increased heart rate upon standing (30 BPM+) and other orthostatic intolerance symptoms.

NCS (Neurocardiogenic Syncope), is single or multiple fainting spells.

OH (Orthostatic Hypotension), characterized by decreased blood pressure upon changes in position (sitting or standing).

IST (Inappropriate Sinus Tachycardia), characterized by sustained increased heart rate, regardless of position. Usually 100BPM+ at rest. (5)

AAG (Autoimmune Autonomic Ganglionopathy), is a rare condition in which the body’s immune system mistakenly attacks and damages certain parts of the autonomic nervous system. (6)

FD (Familial Dysautonomia), also known as Riley-Day Syndrome, is a rare inherited disorder that impacts nerves throughout the body. (8)

PAF (Pure Autonomic Failure), is a neurodegenerative disorder of the autonomic nervous system clinically characterized by orthostatic hypotension. (9)

MSA (Multiple System Atrophy), a rare condition of the nervous system that causes gradual damage to nerve cells in the brain. This affects balance, movement and the autonomic nervous system, which controls several basic functions, such as breathing, digestion and bladder control. (10)

BF (Baroreflex Failure), is characterized the loss of buffering of blood pressure and is characterized by volatility of the blood pressure and heart rate (7).

AD (Autonomic Dysreflexia), is a form of autonomic dysfunction associated with spinal cord injuries. Usually, when an AD attack occurs, there is an irritant impacting the person below the level of their spinal cord injury, and because their autonomic nervous system cannot process messages properly, this results in a severe spike in blood pressure, flushing above the spinal injury, nasal stuffiness and other symptoms. (4)

DAN (Diabetic Autonomic Neuropathy), is a secondary (to diabetes) form of Dysautonomia where nerves become damaged.

Questions? Was this helpful? Want to see more details or posts on any of the above? Let me know in the comments!

Sources:

1. Dysautonomia International http://www.dysautonomiainternational.org/page.php?ID=34
2. Cleveland Clinic https://my.clevelandclinic.org/health/diseases/6004-dysautonomia
3. The Dysautonomia Project https://thedysautonomiaproject.org/identifying-dysautonomia/#:~:text=There%20are%20at%20least%2015,adult%2C%20and%2For%20geriatric.
4. Dysautonomia International http://www.dysautonomiainternational.org/page.php?ID=35
5. Dysautonomia Support Network https://www.dysautonomiasupport.org/forms-of-dysautonomia/#:~:text=forms%20of%20dysautonomia%20include%3A,Failure%2C%20and%20Multiple%20System%20Atrophy.
6. RareDiseases.org https://rarediseases.org/gard-rare-disease/autoimmune-autonomic-ganglionopathy/#:~:text=Autoimmune%20autonomic%20ganglionopathy%20(AAG)%20is,normally%20fight%20infection%20(antibodies).
7. New England Journal of Medicine https://www.nejm.org/doi/full/10.1056/nejm199311113292003#:~:text=True%20baroreflex%20failure%20entails%20the,unambiguous%20dysfunction%20of%20arterial%20baroreflexes.
8. Mount Sinai https://www.mountsinai.org/health-library/diseases-conditions/riley-day-syndrome#:~:text=Familial%20dysautonomia%20(FD)%20is%20an,contain%20all%20the%20body’s%20genes.
9. Mayo Clinic https://www.mayoclinicproceedings.org/article/S0025-6196(19)30265-4/fulltext#:~:text=Pure%20autonomic%20failure%20(PAF)%20is,of%20the%201925%20seminal%20description.
10. NHS https://www.nhs.uk/conditions/multiple-system-atrophy/#:~:text=Multiple%20system%20atrophy%20(MSA)%20is,breathing%2C%20digestion%20and%20bladder%20control.

MCAS stands for Mast Cell Activation Syndrome. “It’s like being allergic to everything and nothing all at once”, via @PinkSaltCollective instagram. It is an inappropriate response, or activation, of the body’s mast cells- often without a true ‘allergy’ (though people with MCAS can also have allergies and anaphylaxis).

Plenty of MCAS patients get told after allergy testing that they aren’t allergic to anything, despite having severe allergic reactions such as hives, swelling, itching, GI issues, migraines, sinusitis, etc.

Interestingly, MCAS can sometimes cause POTS or dysautonomia-type symptoms, with symptoms such as tachycardia and low blood pressure.

According to Dr. Raj, over 30% of POTS patients test positive for MCAS.

Whether this is a chicken or the egg situation, we don’t necessarily know, but it is worth noting the strong overlap in patients. For some, treating MCAS can help with POTS symptoms.

What are Mast Cells & Mediators?

Mast cells are responsible for the body’s allergic reactions. They secrete mediators such as histamine and tryptase (among others) – this process is called degranulation. During a true invasion, this helps to protect our bodies. During an MCAS episode, this degranulation is inappropriate.

Levels of these mediators can be measured as a part of testing for MCAS (blood and urine samples).

How is MCAS Tested for & Treated?

MCAS testing is sensitive and can be hard to get a ‘positive’ test during an episode. As such, many practitioners suggest that diagnosis be based on symptoms and response to therapy.

Treatment often includes: H1 blockers (such as Allegra, Zyrtec, Claritin), H2 blockers (such as pepcid), mast cell stabilizers (such as Cromolyn or Ketotifen).

Tests often include: Serum tryptase, serum histamine, 24 hour urine (N-methylhistamine, 11B -Prostaglandin F2α (11B-PGF2α) and/or Leukotriene E4 (LTE4))

EDS stands for Ehlers-Danlos Syndromes.

There are 13 subtypes of EDS.

What is EDS?

EDS or the Ehlers-Danlos Syndromes are a group of connective tissue disorders that can be inherited and are varied both in how they affect the body and in their genetic causes.

What are symptoms of EDS?

They are generally characterized by joint hypermobility (joints that stretch further than normal),skin hyperextensibility (skin that can be stretched further than normal), and tissue fragility.

What are the Types of EDS?

There are 13 types of EDS according to the Ehlers-Danlos society:

Classical EDS (cEDS)

Classical-like EDS (clEDS)

Cardiac-valvular EDS (cvEDS)

Vascular EDS (vEDS)

Hypermobile EDS (hEDS) – most common

Arthrochalasia EDS (aEDS)

Dermatosparaxis EDS (dEDS)

Kyphoscoliotic EDS (kEDS)

Brittle Cornea Syndrome (BCS)

Spondylodysplastic EDS (spEDS)

Musculocontractural EDS (mcEDS)

Myopathic EDS (mEDS)

Periodontal EDS (pEDS)

What are Symptoms of EDS?

Symptoms can include but are not limited to:

• Frequent joint dislocations, sprains, or strains
• Velvety smooth skin
• Extra stretchy skin
• Easy bruising, bleeding, or scarring
• Extra bendy joints (eg. can touch thumb to forearm)
• Unusual scarring
• Unusual stretch marks

There is a fair bit of overlap between hEDS and POTS, as POTS, EDS and MCAS tend to occur in a cluster of 3.

If you have POTS, you’ve probably heard these two terms- vasoconstriction & vasodilation.

What is Vasoconstriction & Vasodilation?

Vasoconstriction is the constriction, or tightening, of blood vessels- things get tighter and blood is able to be pushed back up against gravity. In other words, vasoconstriction is the narrowing (constriction) of blood vessels by small muscles in their walls. (1) Vasoconstriction is often associated with increased blood pressure.

Vasodilation is the relaxation of blood vessels. Things get looser, and blood is able to flow more freely (if there is enough of it), or it can pool if there isn’t enough vasoconstriction to counteract gravity. Vasodilation is caused by the muscles in the walls of the blood vessels relaxing. Vasodilation is often associated with reduced blood pressure.

Why Should I Care?

As you can see above, vasoconstriction and vasodilation directly impact blood flow and blood pressure. A subset of POTS is caused by chronic vasodilation, which can be helped by vasoconstriction. Another subset of POTS is hypothesized to be caused by low blood volume, which vasoconstriction can help compensate for.

For some folks with low blood pressure, adding a medication that is a vasoconstrictor can help alleviate symptoms. Likewise, if high blood pressure is an issue, sometimes a vasodilator can help. It’s also hypothesized that some brain fog, if caused by low blood volume or vasodilation, can be helped by vasoconstriction. For me personally, understanding these concepts just helps me understand a bit more what’s going on with my body.

Fun fact: caffeine is a natural vasoconstrictor. That’s why it’s often used for energy & migraines.

Sources:

1. Mount Sinai https://www.mountsinai.org/health-library/special-topic/vasoconstriction#:~:text=Vasoconstriction%20is%20the%20narrowing%20(constriction,%2C%20drugs%2C%20or%20psychological%20conditions.

In May of 2022, Halsey announced that they have diagnoses of POTS, EDS & MCAS. For many, these may just sound like a bunch of random letters. But to many with POTS, EDS & MCAS, these are an all-too-familiar triad of syndromes that can significantly alter life.

WHAT IS POTS?

POTS stands for Postural Orthostatic Tachycardia Syndrome. For more information, see my post on POTS. In summary, POTS consists of several symptoms including increased heart rate upon standing, dizziness, migraines, fatigue, etc.

WHAT IS EDS?

EDS stands for Ehlers-Danlos Syndrome. It is a collagen/ connective tissue disorder often characterized by hypermobility. There are several types of EDS, but the most common is hEDS.

WHAT IS MCAS?

MCAS stands for Mast Cell Activation Syndrome, where the body’s mast cells (allergy cells) respond inappropriately.

HOW DO THESE 3 SYNDROMES RELATE?

As is often the case, there isn’t sufficient research and doctors don’t entirely know for sure. However, there are some pretty well tracked theories, that I’ll explain.

1. Faulty Collagen – EDS & POTS

EDS means faulty collagen, and often weak blood vessels. If your blood vessels don’t constrict, blood can’t get pushed up to your head & heart, leading to POTS symptoms.

2. Angry Mast Cells- MCAS & POTS

When mast cells get activated, they release mediators such as histamine, which can be vasodilators. Many mast cells are right near nerves, so these mediators can act on these neighboring nerves. This can cause dysautonomia or POTS-like symptoms such as tachycardia or blood pressure changes.

3. Altered Mast Cells- EDS & MCAS

Cells of the body are surrounded and supported by a network called the Extracellular Matrix (ECM), which can be made from structural materials like collagen. Mast Cells can stick to the ECM, which in turn alters their behavior. EDS, as a connective tissue disorder, could change the ECM enough to cause a change in Mast Cell behavior. (Source: EDS Society)

4. Symptom Overlap

In addition to the interplay of these 3 syndromes, I want to highlight that each of them can cause overlapping symptoms. For example, take a common symptom like headache:

POTS: headaches from lack of blood to brain

EDS: headahces from instability

MCAS: headaches from too much histamine

Other common symptoms include general pain, sinus issues, GI issues, fatigue, dizziness… all of which could be caused by any 3 or a combination of the above.

But there is hope.

1. Treatment Can Help!

Treating any of these syndromes can help improve symptoms in others. For example, treating MCAS with antihistamines and mast cell stabilizers can significantly help with GI issues, headaches, and even POTS symptoms (remember the angry mast cells next to nerves? Calm them down and you may calm down your POTS reaction. In theory. Didn’t work for me, hehe. However treating my MCAS did significantly improve the severity and frequency of my headaches/ migraines)

2. Raising awareness for future research

The more we can raise awareness about each of these syndromes individually, as well as the connection between them, the more research funding they can get.

3. You are not alone.

Browse through this page (and my instagram community) and find hundreds of others who are dealing with these same syndromes, or symptoms of these syndromes. Who are diagnosed and trying treatments, or who are still searching for answers.

Remember- your symptoms and experiences are valid. You deserve health and wellness.

As always, sending light, love, salt & spoons.

POTS stands for Postural Orthostatic Tachycardia Syndrome. For more information on what POTS is or how it’s treated, feel free to read our post that explains it all.

TREATMENTS FOR POTS

The first line of treatment for POTS is typically lifestyle modifications. This can include increase hydration, electrolyte intake (via electrolyte drinks and/ or salt tabs), and exercise therapy. These should be individualized to each patient.

Pharmaceutically there are several medications that can treat POTS. What’s important is that you try to find any underlying causes and treat those. These can include other autoimmune diseases or vitamin deficiencies. If left with POTS, there are several classes of medications you can try.

POTS often occurs alongside MCAS (Mast Cell Activation Syndrome) and EDS (Ehlers Danlos Syndrome). In some cases, addressing these other syndromes can improve POTS symptoms.

Compression stockings (socks, tights, or even abdominal binders) can help with symptoms as well. Check out my post on compression for more details.

POTS stands for Postural Orthostatic Tachycardia Syndrome.

Basically, this means that when upright, the body does not properly counteract gravity. Not enough blood returns up to the heart and brain, so the heart beats faster in response.

POTS falls under the umbrella term dysautonomia, or a dysfunction of the autonomic nervous system.

POTS is a syndrome, or group of symptoms. No two POTSies have exactly the same experience. Symptoms include dizziness, lightheadedness, fatigue, headaches, palpitations, brain fog & more. Sleep disturbances are common in POTS.

POTS is not rare. It is estimated to impact between 1-3 million Americans, or about 1 in 100.

HOW IS POTS TREATED?

Typically, an increase in water and salt intake is recommended. (Note: this is may contraindicated in certain types of POTS so check with your doctor). Pharmaceuticals such as beta blockers (to lower heart rate), florinef (to increase blood volume), midodrine (to increase vagal tone), mestinon (to increase nerve/ muscle communication), and others are often used. Treating the underlying cause, if known, can help. POTS can be a secondary condition due to other conditions that can cause autonomic neuropathy, such as diabetes, Lyme disease, or autoimmune disorders such as lupus or Sjögren’s syndrome. (Source: JHM)

For more POTS resources, check out our other posts.

According to Johns Hopkins, there are 3 different kinds of POTS: Neuropathic, Hyperadrenergic, and Hypovolemic. These types aren’t distinct, as many patient exhibit symptoms of multiple types at the same time. The category of your POTS can also change (eg. it can become hyperadrenergic later on)

Neuropathic POTS is a term used to describe POTS associated with damage to the small fiber nerves (small-fiber neuropathy). These nerves regulate the constriction of the blood vessels in the limbs and abdomen.

Hyperadrenergic POTS is a term used to describe POTS associated with elevated levels of the stress hormone norepinephrine. In hyperadrenergic POTS, blood pressure is often elevated upon standing. A common test for hyperadrenergic POTS (aka “hyperPOTS”) is to take a plasma norepinephrine laying & standing. If it rises more than 600 between laying & standing, that’s suggestive of hyperPOTS.

Hypovolemic POTS is a term used to describe POTS associated with abnormally low levels of blood (hypovolemia).