Hydration, Salt, and Peeing: The Renin-Angiotensin-Aldosterone System

I guess I should use the technical name for peeing in this title - urination - but it's a catchier to say pee.  Because that's what many of us POTS folks do (and various other forms of Dysautonomia), we pee A LOT.

Why is this?  And why the heck do we have to eat so much salt?  

Well, there is this thing called the

Renin-Angiotensin-Aldosterone System.

 

It's a big deal. It takes place mainly in your kidneys and adrenal cortex, and affects your blood pressure directly.  Lets break down the three components:

 

1. Renin is a protein (enzyme) released by special kidney cells when you have decreased salt (sodium levels) or low blood volume, and stimulates the formation of Angiotensin in blood and tissues. 

2. Angiotensin then undergoes a series of reactions that convert it to Angiotensin II (AII in the diagram below), which in turn stimulates the release of Aldosterone from the adrenal cortex. 

3. Aldosterone is the main mineralocorticoid (steroid hormones that balance electrolytes) hormone secreted by the adrenal cortex, the principal biological activity of which is the regulation of electrolyte and water balance by promoting the retention of sodium (and, therefore, of water) and the excretion of potassium.  In plain English - Aldosterone helps your kidneys retain salt, and therefor retain fluids, helping to keep blood volume and blood pressure up.

Here is a diagram of the entire system.  

Find Your POTS Cause: The Wide World of Mast Cells (Sourced)

The proposed relationship between POTS and MCAD.

MCAD and Mastocytosis are a confusing set of diseases and conditions.  What aren't  these days?   I'd like to post about a nice, simple, straight forward and easy to understand condition for once, but alas, MCAD is exactly the opposite.


 What are Mast Cells?

Mast cells are cells of the immune system that are found around blood vessels in the skin, gastrointestinal tract, respiratory tract, and genitourinary tract, and are highly associated with nerves. They release chemicals including histamine that are very irritating and cause itching, swelling, and fluid leakage from cells.

"Mast cells are known to be the primary responders in allergic reactions, orchestrating strong responses to minute amounts of allergens. Several recent observations indicate that they may also have a key role in coordinating the early phases of autoimmune diseases, particularly those involving auto-antibodies."

What is MCAD?
As desribed technically, "Mast cell activation disease comprises disorders characterized by accumulation of genetically altered mast cells and/or abnormal release of these cells' mediators, affecting functions in potentially every organ system, often without causing abnormalities in routine laboratory or radiologic testing. In most cases of mast cell activation disease, diagnosis is possible by relatively non-invasive investigation. Effective therapy often consists simply of antihistamines and mast cell membrane-stabilising compounds supplemented with medications targeted at specific symptoms and complications. Mast cell activation disease is now appreciated to likely be considerably prevalent and thus should be considered routinely in the differential diagnosis of patients with chronic multisystem polymorbidity or patients in whom a definitively diagnosed major illness does not well account for the entirety of the patient's presentation."

What does that mean?  In simpler terms - MCAD is a collection of hard to diagnose (because they often don't show up in standard blood work) conditions related to "mast cells behaving badly."  Mast cells are almost everywhere in the body.  When they break down, it is called degranulation, and in MCAD the mast cells do this in a seemingly random way; releasing histamine that the body reacts to, causing all kinds of skin reactions, anaphylaxis (throat closing/itching/blood pressure drops, etc...), and even causing heart rate fluctuations and shortness of breath in some.

MCAD is also called MCA, or MCAS (the "S" stands for syndrome), and the current definitions by the WHO (World Health Organization) are being debated.  Currently because of the WHO definitions of MCAD, it is very hard to get a solid diagnosis, and it is an under-diagnosed and misunderstood condition by many physicians. 

#HAWMC Day 8: Best POTS Convo Ever

WEGO HEALTH ACTIVISTS WRITERS CHALLENGE: Best conversation I had this week. Try writing script-style (or with dialogue) today to recap an awesome conversation you had this week.

"We have a great new drug for you to try, it cures POTS and dysautonomia." --Doctor

"Really?  You can't be serious." --Me

"And (dramatic pause), we figured out what was causing all your random food reactions, and you can now eat whatever you want." --Doctor

"This can't be real." --Me

"You will be walking, then running, and able to scuba dive and snowboard again. You'll be able to care for your kids, have wild nights out on the town (and indoors) with your husband, and you can eat out wherever you want." --Doctor

I'm Angry and I Know It (wiggle wiggle wiggle): Day 7 WEGO Challenge

WEGO Health Writers Challenge Day 7: Health Activist Choice! Write about what you want today.

I'm Angry and I Know It (Wiggle Wiggle Wiggle)

Well then, this will not win me any "she has such a great attitude" points, but today I want to write about how much I hate my life and am angry right now.  Because it sucks.  I am pissed, and there will be swearing- oh yes, there will - and you have been warned. This is not a pity seeking post, its an explanation of what life is like, and how you can better understand and be there for any person you know with a chronic - or LONGTIME - illness.  Not to be confused with the flu or allergies you have been bitching about on twitter.

Updated Article Library!

LIBRARY: Articles For A Doctor/ER Visit

I have found that many of my local doctors have very little (if any) knowledge about POTS and Dysautonomia.  I usually try and bring at least one journal article with me to give them, and I stress that these are peer reviewed journal articles, not just random stuff from Google or Wikipedia.  They are research studies published in medical journals, and should give you credibility in the doctor's eyes, and if you are lucky, won't bruise their ego :) 

I have categorized them by issues common to POTS sufferers when trying to obtain treatment from both general practitioner doctors, specialists unfamiliar with POTS, and ER doctors.  Most are full text - but some are only abstracts, your doctor should be able to use it to look up the full article if they feel it's relevant.  It is a starting point - I DID NOT CITE THESE IN PROPER SCIENTIFIC CITATION FORM INTENTIONALLY.  Hopefully they are more accessible this way and you can pick and choose which one's you need. You may want to add the most relevant articles to your POTS Binder. If you have any to add or topics you'd like covered, please leave a comment or contact me. 


General POTS Info/Treatment:

1.  The Postural Tachycardia Syndrome (POTS): Pathophysiology, Diagnosis & Management (this in my opinion is by far the best synopsis of POTS for any unfamiliar doctor, and the complete article is free) by Satish R Raj, MD MSCI, Autonomic Dysfunction Center, Division of Clinical Pharmacology, Departments of Medicine & Pharmacology, Vanderbilt University, Nashville, Tennessee, USA

2.  Orthostatic Intolerance (an AMAZING guide to all the forms of OI, including POTS, NCS, etc...) Dr. Stewart, Associate Chairman of Pediatrics, Director, Center for Hypotension, Westchester Medical Center; Professor of Pediatrics and Physiology, New York Medical College.

3.  The neuropathic postural tachycardia syndrome . Robertson D., Jacob Recanati Autonomic Dysfunction Center and the Department of Internal Medicine C, Rambam Medical Center, Haifa, Israel.

New Autonomic Clinic Opening in Norfolk, VA (NEAR ME!)

So for any Virginians (and other East Coaster's) that haven't been able to get a full autonomic work up - I have great news!  (Insert happy dance while seated!)

I met with Dr. Chemali, who just came to Norfolk, VA from the Cleveland Clinic, and specializes in Dysautonomia, and has worked with some of the best minds in the field.  As we all know, Mayo, Vanderbilt, and Cleveland Clinic are where you go to get quality autonomic testing done.

Well, he is opening a full lab right here in Norfolk!  It will be located in the new Heart Hospital of Sentara Norfolk General.  My understanding is that it will be fully up and running in September.

This is a God send for me, and I think many others.  There is a SERIOUS shortage of proper equipment to diagnose Dysautonomia in Virginia, and even more so there is a shortage of specialists. Some facilities may have a few tests they can run, but their doctors don't really diagnose and treat the big picture that is dysautonomia.   I was shocked when Hopkins had no one to help me while I was there.

My hope is that Chemali will train up a few doctors and get the ball rolling, or maybe bring in another specialist or two, and start seeing patients and doing clinical trials/research like crazy.

Here is a link to Dr. Chemali and the Neurology Group's Info.   No one else in his group treats dysautonomia of any sort (I've seen a few - don't bother), so make sure you ask for him.  His nurse and the staff are very nice.  This is very exciting!

Now; a quick illustration/exploration of various happy dance options (modified to work with your POTS of course):

the nun happy dance

the Calvin and Hobbs happy dance

the robot with no arms happy dance

the cool turtle happy dance

the all time classic Snoopy happy dance

Hope everyone is having a great and unPOTSy day!
--Claire

The ABC News Aftermath; Junk Reporting and THE GRINCH Making Life Harder for POTS Patients

As many of you in the Dysautonomia community know, ABC recently aired a segment on World News Tonight with Diane Sawyer about POTS (on 2/29/12).  Leading up to that broadcast, a series of events took place that amazed me and reinvigorated my faith in the power of patients as a community.  We mobilized.  We complained. We used every connection we could.  And it worked - sort of.

Here is a breakdown of the history of, and problem with "The Grinch Syndrome", along with a timeline of what took place over the last week.  I also include a call to action (once again) from our amazing POTS warriors to force change that is long overdue.  

THE GRINCH PROBLEM

There is a researcher out of Texas named Dr. Levine who proposed that POTS be renamed The Grinch Syndrome.  He did this based on his study that was published in the Journal of American College of Cardiology in 2010 called Cardiac Origins Of The Postural Orthostatic Tachycardia Syndrome. The presumptuous name of the study is the first clue as to how preposterous this "study" and its "conclusions" are.  Levine and his researchers claim that, "These results suggest that POTS per se is indeed a consequence of deconditioning and that carefully prescribed exercise training can be used as an effective non-drug therapy for POTS patients."

Decoding Hyperadrenergic POTS & How to Maintain a Functioning Lifestyle

***I am trying to find out what website this came from so I can link to it, it's circulating on Facebook right now, if you know please contact me.  I love this breakdown of what hyperPOTS patients go through (I'm in the process of nailing down this diagnosis).  ENJOY! 

My interpretation of the hyper POTS state.

Decoding Hyperadrenergic POTS & How to Maintain a Functioning Lifestyle.

by Kimberly ResilientSwan Swanson on Saturday, February 4, 2012 at 1:05pm ·


Decoding for Chronic Conditions in General

What do I mean by decoding? Decoding is a term that I use to describe the process of learning about your condition/body, developing and then adhering to a long term treatment plan. Let me more fully explain this process in general and then apply it to the Hyperadrenergic POTS patient. The first step to decoding a particular chronic health condition is to begin listening to your body. People who are healthy never listen to their bodies, because they never had to before. Everything functioned as it should, so what’s there to pay attention to? You may be thinking, “What does a person need to listen for?” When a person begins to notice a particular symptom or groups of symptoms they need to become an investigator by making some observations about their condition. When do their symptoms begin? What triggers them? What makes them better? What makes them worse?

POTS is NOT a rare illness!

I keep hearing the phrase "rare" disease, "rare" illness, and "rare" disorder.  I am guilty of using this phrase myself.

But what is "rare"?  Not regarding steak, but in terms of an illness or disease?

The National Institutes of Health (NIH)  defines a rare disease or illness as:

"The prevalence of a rare disease is usually an estimate and may change over time. A rare (or orphan) disease is generally considered to have a prevalence of fewer than 200,000 affected individuals in the United States. Certain diseases with 200,000 or more affected individuals may be included in this list if certain sub-populations of people who have the disease are equal to the prevalence standard for rare diseases."

Now lets look at POTS. 
Postural Orthostatic Tachycardia Syndrome is defined by Low et.al: