POTS: The Basics

I'm re-upping this, as we are almost done with the new nonprofit website, and most of my new "material" will be headed over there.  This a POTS 101 type summary, hopefully easy to understand and useful :)  ---Claire

POTS: An Overview

Postural Tachycardia Syndrome

POTS: The Basics
Find links to in depth information and resources for POTSies at on our Dysautonomia SOS website (under construction).

POTS is short for Postural Orthostatic Tachycardia Syndrome.   It is referred to by some physicians and researchers as Postural Tachycardia Syndrome, or just Postural Tachycardia.  It is a type of Orthostatic Intolerance, and also a type of Dysautonomia.   Let’s break down what that name means:

Postural means relating to posture, or a change in position.

Orthostatic means relating to, or caused by erect posture (standing up).

Tachycardia refers to a rapid heart rate.

Syndrome means a collection of symptoms, and not a disease. Technically, a syndrome is defined as a group of signs and symptoms that occur together and characterize a particular abnormality.

So, POTS is a collection of symptoms that result from a heart rate increase when standing up.  When a person with POTS stands up, the heart pumps faster to help keep the blood pressure up, and try to help the blood in their body reach their brain and other vital organs.  The body of a person with POTS does not respond to gravity the same way a non-POTS person does. 


The majority of POTS patients are female, of child bearing age, and under 35.  There are thought to be anywhere from 500,000 to millions of people in the US with POTS and other forms of Orthostatic Intolerance.   There are no solid published statistics or data at this time, these are estimates from the major research centers that treat POTS.

Bye Bye Bella Flora: Parenting From A Bed; Grief, Guilt, & Gratefullnes...

A post from my personal blog:

Bye Bye Bella Flora: Parenting From A Bed; Grief, Guilt, & Gratefullnes...: My daughter is 11 months old today.  She is a  gorgeous, bright, happy baby - granted who doesn't sleep very well - but she is such a joy ...

Pregnancy and POTS: Experiences & Resources PART 2

In PART 1 of this mini-series, we discussed my first (excellent) POTS pregnancy and the data published regarding having healthy children when you have POTS.  Now on to the tough part of this, as my second pregnancy was a nightmare.  So here's an oversimplified version of what happened, and I'm including some thoughts and links regarding medications at the end.

The Second (NIGHTMARE) POTS Pregnancy:

In December of 2010, I knew something was wrong.  I had been functioning most days for the last few years since my son was born, with fluctuating symptoms that I had been trying to hide from business related folks, and had given up fixing with doctors.  But they were tolerable - episodes of tachycardia, light-headedness, chronic pain and fatigue after working long hours, and severe hypoglycemia.

I was suddenly feeling like I was going drop, and spinning, and my heart rate was nuts.  I was also not retaining any fluids, no matter how much salt loading and drinking I did.  Then I found out I was pregnant right after Christmas, and everything changed.  Within a week, I was sick, bed bound, and knew something was severely wrong. The first week of January was my first trip to the ER after blacking out when I stood up too fast to grab my son (who decided climbing on a counter top was a good idea!), and I couldn't get my heart rate under control after, even laying flat.  This is also when a month+ of vertigo began.  Horrendous vertigo, like I had never before experienced.  

The month of January I was back and forth to the ER and was admitted multiple times.  Then in early Feb I was sent to a rehad/nursing center because I needed constant monitoring, continuous IV fluids, and had a PICC line placed.  Only once the IV fluids were upped did I start to see improvements with the vertigo.  Unfortunately, my PICC line was not cared for, and I developed massive blood clots along the line that were ignored by the staff at the nursing home.  We had to threaten legal action to get me medically released, as the doctor at that horrible place blew me off and thought I was having muscle pain - and was massaging Aspercreme into my clotted area (nice).

After coming home, my nurse was appalled at what I told her and saw the state of my PICC line and sent me to the ER.  We found out I had 5 DVT's and my jugular was completely clotted off. I hadn't been able to turn my neck for days, and they had been rubbing my clotted area at the nursing home, and the clots had broken off and started migrating to various dangerous locations.  I was admitted again to the hospital, but was still blown off when I complained of extreme chest pain and difficulty breathing, and told "it's just your POTS" and sent home after a week.  Well - it was not just my POTS - it was over a dozen bilateral pulmonary embolisms (PE's), plus a 2 cm clot in my right atrium.  Turns out they had sent me home on the wrong dose of Lovanox (a blood thinner) and ignored my OB doctors demands for my clotting factor to be tested.  If I had not kept going back to the ER and demanding they do something or I was going to pull out that stupid PICC line myself, I would have died, along with the baby.  Being pregnant, bed-bound, and having a PICC line is the trifecta of blot clotting risks.  Add to that the fact that they finally tested me for clotting factor conditions, and I came out positive for the Factor V Leiden gene, so basically I was a clot waiting to happen.  It took me almost dying to get the treatment I needed, and even after that - it was a 9 month struggle and fight with the hospital and doctors around here.  That's why I advise people to have a plan in place and a support network of good doctors familiar with your brand of POTS.

Pregnancy and POTS: Experiences & Resources PART 1

This is a big issue for women and families affected by POTS, and the topic I get the most questions about.  Being that POTS affects mostly women of childbearing age, and there are very few resources out there, and even fewer research articles, this is not surprising.   I have heard from patients that their doctors are telling them not to have kids, which is heartbreaking, and the science just doesn't back that up for MOST women with POTS.  POTS already robs us of so many things- don't let it rob you of kids (if you want them)!  If you  don't have any life threatening genetic diseases that can be passed on, are not on any medications that keep you alive that would damage a baby, and really want kids - there is no reason not to consider it in my opinion.   It is a very personal decision.  Your underlying cause of your POTS is a HUGE consideration, as well as your functionality and support system.  Of course consult with a doctor about anything medical, as I am not one.

The issue of what drugs to take, how to plan, and what to expect can make for a frightening experience if you don't get as educated as possible on the subject.

I tell everyone:  PLAN FOR THE WORST, HOPE/PRAY FOR THE BEST.

Things to consider when planning for the worst:

-Do you have a good medical team of doctors, including a POTS knowledgeable cardiologist, a high risk OB, and possibly a neurologist? 

-Do you have a support network in place in case you are bed bound, non-functional, or severely ill during pregnancy?  Especially if you have other children?

-Do you have a support network in place to help with the baby after you give birth, especially if recovery takes longer than expected or you have complications from birth?

-Are you on any medications that could possibly harm the baby, and are you willing to try and function without them if necessary?

-Do you have any underlying conditions that could further complicate pregnancy, and specialists and support lined up to help deal with those possible complications as well? 

Hopeful Dysautonomia Video from Lette!

This is Lette from Irish Dysautonomia....and I LOVE THIS VIDEO!  There is hope for everyone, and some very wise advise in this video, watch the whole thing if you are up for it!





I have had periods of remission as well; and know that food, physical activity, and not becoming depressed by living on the support group boards are all important factors in improving quality of life. Remissions and recovery DO HAPPEN! What has helped me currently in my yucky state of full on POTS/Dysautonomia is trying to help others empower and educate themselves (and myself), and finding BALANCE. Wishing you all a HOPEFUL day! --Claire

Day 2: Yoda Does POTS (favorite quote)

Challenge: Quotation Inspiration. Find a quote that inspires you (either positively or negatively) and free write about it for 15 minutes.

Well, this was a really really easy pick for me - I have an absolute favorite quote that I have both a love and hate relationship with in terms of my chronic illness, POTS.


 Drum roll......

My quote comes from a little green man who has inspired me my whole life. His name is Yoda.

"Try not. Do, or do not.  There is no try."

I even found a nice graphical illustration for the quote:

Why I love this quote:

It's completely true.  There really is no try.  You either do something, or you don't.  I'm not TRYING to oversimplify, I actually AM oversimplifying.   

The definition of try is (according to the The American Heritage® Dictionary of the English Language):

try  (tr): To make an effort to do or accomplish (something); attempt: tried to ski.


Why I hate this quote: 

It leaves no room for excuses, and I admit I still fancy a good excuse once in a while.

"Trying" to me implies failure.  

"Did you do your physical therapy exercises today?"

"I TRIED, but I wasn't able to." 

What that means in my life is that I actually DID three or four reps, felt like my heart was pounding out of my chest and my muscles were spasming painfully, and I stopped.   So I didn't just try, I did it.  I did not fail.  I did not complete the entire task or goal, but I DID something.

If I skipped doing my PT all together, than I did not try, or DO any PT.  And there is nothing wrong with that.  Some days I can't even get out of bed for much of the day, and can barely make it to the restroom.  I have to consider what to spend my limited energy on, as I have a 7 month old that I care for alone while my husband is working, and a four year old that I like to be functional enough to hang out with in the evening when he gets home.

I know that on bad days, "trying" much of anything will land me in even worse shape for a week afterwards.  I have learned to trust my body, and push my limits when appropriate, and DO when appropriate - but I have finally gotten to a place where I am ok with not doing as well.  

And there is no try.    Simple as that.

Skype Meet Up Tonight! 8PM EST

Hello VA/DC/MD (and beyond!) POTSies!

Tonight is our first try at a Skype meet up!  Until we get enough people participating that we can split up geographically - I opened this up to anyone anywhere affiliated with POTS - including parents, caregivers, etc...

In order for you to participate, I need you to send me a contact request so I can add you to the conference call group "Team STOP POTS".

Here's how to do this:

1.  Get a FREE Skype account at www.Skype.com

My Guest Post of Living With Bob: Staying Sane With A Chronic Illness

I was honored to have this article published by Rusty Hoe on her popular blog - that I adore - Living with Bob (Bob is Dysautonomia).

 If you haven't checked it out, you should!  Her writing and sense of humor dealing with this maddening disease are amazing.

Monday, 12 March 2012

No Need For Padded Walls: Staying Sane With Chronic Illness

Fourteen , count 'em - FOURTEEN - months either at home or in the hospital/doctors offices for me, with no social outings.  And I'm not the only one, or even close to the one with the longest record of time spent indoors.  I had been feeling a bit overwhelmed by the lack of outdoor time, and constant focus on my illness, my kids (with no break), and searching for ways to get better.  Life is about balance (insert "oooommmm" yoga zen breathing pause for effect here).  Even if you are not completely bed or home bound such as myself, you may be severely limited by your illness, and going a bit bonkers being indoors more than the average healthy person, not to mention being sick.

 

So how the heck do we all stay sane?  I REALLY want to avoid the padded walls of a psych ward, even though the padding wouldn't do much good - as I can't walk far enough to run into them.  Padding on the floor would be great though, in case of random face planting incidents. And as sexy as helmets can be - I'm trying to avoid those too.  But just in case, here are a few stylish options, for those of you that unintentionally share my love of hugging the floor and have a fear of the sharp hard edges found in bathrooms:

From Battling the Grinch, to Battling the Bulge; Cooking Healthy for a Month

This is going to seem really tame and maybe boring after the excitement of the last week or two with the "Battle of the Grinch" as I've come to call it. By the way,  Dr. Levine (who coined the term Grinch Syndrome) wrote a response to the excellent article on About.com about POTS and the Grinch controversy, featured here.   Levine danced around the issues, and you can see my thoughts in the first comment listed if you care to.

From "Battle of the Grinch" to "Battle of the Bulge," and I do like a dramatic transition - I have been struggling with blood sugar swings, and eating out of a cooler of pre-made/pre-packaged food daily that my husband kindly puts together for me (I can't sit up long enough to make food or last long enough in the kitchen to prepare anything).  Add to that being gluten intolerant, having random anaphylactic reactions to foods/smells (possible MCAD), and you have a recipe for linited and fearful eating habits. Not to mention fat thighs and a post baby belly that looks like my daughter could still live in there at 6 months old.  My son actually told me I have a big butt tonight.  He was being silly, but kids don't lie!  Talk about a reality check.

Identitiy Crisis: Causes of POTS and Dysautonomia Unknown?

Well, I am once again on the hunt.  But this time is different, and the stakes are higher.  I have had POTS now since the end of 2002, with many ups and downs, but always managed to bounce back, or push through.  I had a few fairly bad spells where I was told I "just have POTS", and was given meds (or not), and basically told to just deal with it.  I was constantly given the run around by local doctors, and got burnt out on the constant scoffing, anxiety blaming, or being passed on to yet another specialist because they didn't want to deal with my issues.  So this is partially my fault - I should have kept pushing, even though the process is hard to maintain while trying to live, and it gets exhausting.

My health had been declining in 2009 and 2010 with the increased stress in my life (business related), and leaving all of these health issues untreated and unchecked for so many years while working in my typical type-A fashion was catching up with me.  Then I got pregnant in December of 2010 with our second child, and I have been mostly bed bound, and completely hospital or home bound, ever since.  I like to call it, "POTS gone wild".  I go everywhere by medical transport.  I cannot tolerate more than one or two minor activities, even if seated with my legs elevated, per day.  I barely can cope with caring for my infant daughter, and am constantly pushing my health boundaries to play with my preschool aged son.  It has been 14 months since I left the house to do anything social, anything other than be hospitalized (for over 9 months total) or go to a doctors appointment.

Why do I mention this?  Because I truly believe that if someone had found the cause of my POTS, and if I had made it more of a priority, even though I was functioning at a moderate level, I would not be in this situation.

So I am now on the hunt for answers, and have been searching the medical literature and any credible resources I can find for ALL the causes of POTS, and I am demanding to be tested for ALL OF THEM.  

And so should you.

"Idiopathic POTS", in my not so humble opinion, is an idiotic notion.  It means that the cause is unknown.  I have had many doctors tell me that some people "just have POTS".  What a load of crap.  Something is causing my heart rate to go up at least 30 beats per minute when standing.  Something is causing me to pass out or have near-syncope events after a few minutes (if I'm lucky).  Something is causing all the terrible effects and symptoms of POTS that we all experience, and that certain SOMETHING may be a serious underlying disease or health condition.

The problem in diagnosing the cause of POTS is that there are only two main centers that contain a sufficient array of specialists across all of the medical fields that encompass the multitude of possible causes of POTS - and that even know anything about POTS.  The Mayo Clinic in Rochester, and Vanderbilt University in Nashville.  That's it.  In all of America.  The Cleveland Clinic is fairly well known too, but most people in the autonomic world say go to Mayo or Vandy.

Thankfully, I have an appointment scheduled in July for Vanderbilt.  It has been scheduled since November.  That is a 9 month wait.  Unacceptable.  The wait for Mayo is even longer, as is the wait to get in to see the famed Dr. Grubb (over a year), who has published loads of articles on POTS.   There are so few actual POTS specialists, and so many POTS patients, that we are all waiting ridiculous amounts of time to be seen.  Locally, all the specialists that could diagnose what is causing my POTS are completely spread out, and many times they don't and won't talk to each other.  And they only specialize in their specific field or study, and may miss something, or pass me off to yet another doctor.  I am trying to find some answers locally in the mean time, to avoid the 12 hour car ride to Vandy, and avoid continuing in this state for another 5 months, but it's not looking good.

Why only two centers?  This baffles me.  It baffles me more so because I have learned how common POTS and Dysautonomia are.  Part of the problem is that most of the public and the medical community does not know anything about POTS or Dysautonomia.  It's a ridiculous notion, when a low estimate is that 500,000-1,000,000 people are living with POTS, and I'm not even sure what the current stats are for Dysautonomia in general.  So POTS by definition is not rare.  It is more common than MS, Parkinson's, and Autism (stats cited in this post).  But you can go to any decent sized city and find multiple doctors that treat all three of those other diseases.

As mentioned previously, POTS can be caused by diseases affecting a number of of the bodies systems.  This makes being a POTS specialist tough.   This is why we usually get passed from doctor to doctor in a variety of specialties. They include (but are not limited to): Cardiologists, Neurologists, Endocrinologists, Rheumatologists, Allergists, Immunologists, Gastroenterologists, Hematologists, Dermatologists, and even Ophthalmologists.    

THIS IS A PROBLEM!

The many bodily systems possibly affected by Dysautonomia and POTS.

POTS has an identity crisis. Is it autoimmune?  Is is post viral?  Is it EDS?  Is it a problem with your aldosterone?  Maybe, maybe, maybe, and maybe.  All of those possibilities (and there are many, many more), would require the specialized knowledge of at least three different doctors.  And therein lies the problem. 

So my idea, for a long time, has been to compile all the possible causes of POTS, checklist style, with a diagnostic guide for patients (and maybe doctors) to use to find answers.  I am a firm believer that before saying the cause of your POTS is unknown - or "idiopathic" - you, and any responsible doctor, must exhaust all options.   However unlikely based on your presenting symptoms, the fact that you have POTS means you should be tested for all the known causes, until one comes back positive.  They should be ranked in order of prevalence, and broken down by symptom groupings.  If all of the known scientific causes come back negative, then fine - the science isn't there yet, but my feeling is that most doctors don't even know what this list consists of, except maybe at the two big medical centers I mentioned.

I firmly believe that the key to getting the best treatment is empowering yourself with the most knowledge possible.   This is a BIG project (for me alone at least).  But how many people have died of the underlying disease causing their POTS?  We don't know.  I always hear that POTS is not fatal.  But untreated autoimmune diseases can be, as well as certain forms of mastocystosis. Both cause POTS. We don't know how many people are misdiagnosed, and miss out on years of their lives, when things may have been different with the correct treatment.

I do know that I am not the only one stuck in my house, in a wheelchair, waiting for answers.

So I am starting this list, and this may be a long project, but someone has to start.

If you would like to be involved in this project, and/or a number of others - please contact me.  I am working with NDRF in a large expansion and website revamping of the charity, and we need your help.  We can not continue to live with poor quality of life, stuck indoors, waiting on the few doctors that know about POTS to see and treat us.  We need to educate ourselves, the public, and the medical community about this disabling illness that is not "idiopathic", and we need to find the cause for our own POTS.  We need to team up, as a community, and let the medical professionals know that we are not idiots, we are people, and we are educated, and we want answers and treatment.

---Claire

Bye Bye Bella Flora: The Dysautonomia Dementors and POTS Patronus

From my personal blog:

Bye Bye Bella Flora: The Dysautonomia Dementors and POTS Patronus:

Sunday, February 12, 2012

The Dysautonomia Dementors and POTS Patronus

I love Harry Potter.  I love the books, I love the movies, I love the brilliant world created by JK Rowling.

My life right now is pretty consumed by POTS and Dysautonomia, so I find myself making comparisons and analogies everywhere I look.  Which is actually not many places, considering I am home bound at this point - but does involve lots of movies, online stuff (no porn of course- I swear!), and books.

So when I last watched Harry Potter and the Prisoner of Azkaban (while in the hospital), which is when we first see the Dementors, I burst into tears.  I saw that dark black ghostly scary looking thing sucking the life out of Harry and them him passing out with the screaming noises in the background - and all I could think of was how that was how I felt day in and day out.  That was at the height of my "dark times", but I think many people struggling with dysautonomia and other life force/energy-sucking diseases can relate. POTS, Dysautonomias, CFS/ME, Fibro, Lupus, people going through Chemo, RA, any of the "spoonie" illnesses - we all struggle with days when the Dementors swoop in and gobble up every last bit of energy, happiness, and the ability to move and function.

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: