Others Take On Pots!

[anna]

Ok after the post on POTS PR I thought I would see what came up when I searched "Grinch syndrome"

I was a little shocked and I am not the sort that is easily shocked!

Have a read of this link from the British Heart Foundation the UK's leading heart charity.

Exercise may be better than drugs for rare ‘Grinch Syndrome’, says study

Treating people with a rare heart condition known as Grinch Syndrome with exercise rather than medication could better improve their quality of life. Formally known as Postural Orthostatic Tachycardia Syndrome (POTS), the condition means people’s heart rates increase if they sit or stand upright because their heart is too small to pump blood effectively around the body

http://www.bhf.org.u...aspx?page=13311

Now I know I am a pedantic sort and can be a right pain when I get stuck on an issue but am I reading this Quote right. Does it read; Grinch syndrome (official name POTS)?!

[anna]

On an other well used UK site; http://www.webmd.boots.com/heart-disease/news/20110621/exercise-may-help-treat-rare-heart-condition

"Grinch Syndrome' or Postural Orthopaedic Tachycardia Syndrome (POTS), is a condition in which the heart rates increases if the patient sits or stands upright."

I must say at least this article does put in a good small piece (medical view from Dr. Grubb) at the end of the article!

[anna]

Conclusions

Patients with POTS had a smaller heart coupled with reduced blood volume compared with healthy controls. We therefore propose the name the Grinch syndrome to focus on this pathophysiologic state. The marked orthostatic tachycardia in these patients seemed to be a physiologic compensatory response to a smaller stroke volume that was attributable to cardiac atrophy and hypovolemia. The function of the autonomic nervous system was intact in POTS patients. Short-term exercise training increased cardiac size and mass and expanded blood and plasma volume, and thus improved or even cured POTS. 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 nondrug therapy for POTS patients.

http://content.onlinejacc.org/cgi/reprint/55/25/2858.pdf

What gets me a little fed up is that there is some logic and sound medical theory behind this study but statements like:

"Short-term exercise training increased cardiac size and mass and expanded blood and plasma volume, and thus improved or even cured POTS. 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 nondrug therapy for POTS patients."

Is just so short sighted, it deliberately groups all forms POTS together to have better impact.

Maybe the whole small or weak heart muscle has some credence given so many EDS folk have POTS symptoms and EDS folk tend to have poor muscle tone and a problem perfecting good muscle tone. Thus this could be a real point that needs looking into in much more depth.

[jangle]

Question: If POTS is simply the result of deconditioning, then why isn't like 30% of the American population suffering from POTS?

[ramakentesh]

I've responded to this many times with the following points:

1. The fact that exercise helps pots only proves that exercise improves pots and not that all pots is caused by deconditioning or a small heart. Exercise does so many things for all sorts of things. 2. The gronch study found no autonomic abnormalities because they did not for any of the abnormalities documented in other research.

3. Animal models of the vascular system after exposure to zero gravity demonstrates uprehulation of enos and increased vasodilation and reduced sympathetic activity.

4. Not all pots patients have reduced cardiac size or low blood volume.

[Clairefmartin]

Anna (and others), this is kinda long, but a prtion from what I wrote about regarding this on my STOP POTS blog (i cut and pasted it, pardon the formatting issues), there is more on my site, but this explains the issue and the implications:

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."

So he claims that a small heart size due to deconditioning is causing POTS. He bases this claim on a study comprised of 18 POTS patients, 13 of which had small hearts, who were compared to 16 healthy control patients. He chose those 18 patients based on a "mysterious" (insert sarcasm here) new method of studying POTS patients , which are a mixed bunch, being that POTS is a syndrome known to have numerous causes.

His study group specifically did not include patients with autonomic dysfunction, and in fact the study states that, “ All patients met the inclusion without exclusion criteria for POTS”. POTS Grrl actually emailed Dr. Levine, and he was kind enough to respond, to ask what he meant by that statement since we could not find it in the paper he cited.

He responded by noting the following:

This definition was contained in his NIH grant application and is as follows: "[p]atients with postural hypotension secondary to autonomic neuropathy (diabetic, amyloid, familial), spinal cord lesions (syrinx, traumatic), or degenerative central nervous system disease (multiple-system atrophy, pure autonomic failure, Parkinson’s disease) will be excluded. Similarly, individuals with hypotension stemming from identifiable hypovolemia, dehydration, hemorrhage, vomiting, medications (a- or b-adrenergic antogonists, diuretics, venodilators), or endocrine abnormalities (Addison’s disease, hypothyroidism) will be excluded."

So (in plain English), he excluded the vast majority of POTS patients, and based on his small study size, of which ONLY 13 had small hearts, he proposes to rename the entire POTS population. And not just to rename them us, but to give POTS a name that is negative, degrading, and actually a false representation of the majority of POTS patients. He excluded anyone with known hypovolemia (low blood volume). That is a TON of us, no matter the underlying cause. Endocrine abnormalities excludes the entire Hyperadrenic POTS (also know as Hyperadrenergic or Hyper POTS) population. About half of POTS patients are known to have some autonomic neuropathy involvement (see the Neurology Supplement from 1995, written by Dr. Low who first defined POTS, and the Autonomic Neuroscience Consensus Statement from 2011).

Thus, if you design a study to exclude significantly more than half of the known patient population by excluding those with autonomic neuropathy, hypovolemia, and Hyperadrenergic states - your study is not representative of the entire patient population.

Levine's actual study methods were fine and met general scientific standards for conducting a study to be published in a peer reviewed journal of esteem. We all agree that exercise is a good and viable treatment option for POTS patients. His study was fine for the purposes of identifying the underlying cause(s) of POTS in this specific subset of patients with small hearts, as eliminating patients with known causes of POTS from the study will help focus the research on those with causes that have not been identified yet. However, you cannot credibly draw conclusions about the cause of an illness for the entire patient population from a study population that does not include at least half of the known patient population.

How was a study with such obvious flaws in its conclusions published in such a highly regarded medical journal? Why have no leading researchers in the POTS and Dysautonomia world spoken out? Your guess is as good as mine. But our fundraising, insurance, and hard earned dollars go to these doctors, and it is time they do the right thing. Maybe they thought this whole Grinch thing would just go away - but it has not - and the events of this past week are a direct result of this controversial, ridiculous, and unscientific study's conclusion that POTS is caused by a small "Grinchy" heart. And on top of the ABC news situation, they are still touting the Grinch term at conferences, as is evidenced in this write up of a luncheon last month where Dr. Fu (of Levine's team) actually used the Grinch term not just in regards to POTS, but to all women! See Expert: Women at cardiac disadvantage, and you'll see in that Dr. Fu apparently thinks "the reference to a Grinch’s heart was comparing the heart size and blood volume of women to men." so women are Grinche's because our hearts and blood volume are small when compared to men. WHAT??? So now I'm offended on two fronts; as a POTSy - and as a woman. And for the record - my heart is completely normally sized and works perfectly, at least when I'm laying down, as evidenced by over a dozen ECC's over the last decade.

JUST TODAY (3/5/12) a new article was released by About.com (finally!) with Dr. Richard Fogoros speaking out against the Grinch terminology. Here's a link to his article.

POTS - Who's The Real Grinch?

By Richard N. Fogoros, M.D., About.com Guide March 5, 2012

THE ABC PROBLEM

Here is a portion of our posted call to action (by myself and POTS Grrl), which sums up what happened with ABC:

ACTION ALERT-Tell ABC NEWS Not to Disrespect POTS Patients

Last night (2/28/12) ABC World News with Diane Sawyer did a segment on Cardiogenic Syncope. They showed a preview clip of tonight's segment with fainting astronauts, and we know that is going to be about POTS(Postural Orthostatic Tachycardia Syndrome). Fellow POTSy(POTSGrrl) called ABC News tonight and spoke with the reporter who prepared the POTS/astronaut story. Claire from STOP POTS called as well, and spoke with the reporters assistant.

After she told her she had POTS, she said something like "you mean Grinch?" This means that ABC may be doing a story on POTS tomorrow night and calling it the dreaded Grinch Syndrome- a degrading and negative term invented by one doctor who did a deeply flawed study on a mere 18 POTS patients, and decided that all POTS (in the million+ person POTS population) was caused by deconditioning, and that all POTS patients have small hearts like the Grinch. It is disrespectful, disgusting, and more importantly, WRONG. Credible POTS researchers have known for years, and have published numerous studies, noting that POTS is caused by many different ailments; neuropathies, autoimmune diseases, EDS, viral infections, MCAD, pregnancy, head/neck traumas, Chiari malformations, post-viral onsets, pregnancy, etc. Moreover, as most POTS patients have had detailed cardiac imaging, we know that most POTS patients do not have small hearts.

We have been planning and mobilizing to campaign against the use of this negative and inaccurate term on a nationally broadcast network show. We are reaching out to all the major Dysautonomia related organizations, and I have sent a lengthy email directly to the producers of the show through various family media contacts. But we still need your help!....

........See the continuation and full text here.

So we posted this on our blogs, sent out emails to other bloggers, and I posted it on every Facebook site I could find and asked others to re-post. I started tweeting. You started tweeting, You started posting. And you (and I) complained to ABC! The response and mobilization of the POTSies, family, and friends was immediate - and amazing. The email mentioned above was sent out by a family member of mine, who has media contacts all over the world. It worked it's way to the producers of the show, and we were told they contacted Mayo, Cleveland Clinic, and Vanderbilt for information. This all took place on the day of the broadcast.

In my opinion, and only my opinion, based on what I know of how media works - the segment was ready to go and completely based on the small Grinch heart cause of POTS. I think our (our being the entire POTS community that mobilized) efforts were the reason they did not use the specific term, but they still conveyed the idea that POTS is caused by a small heart. They did end the segment with a short blurb saying that POTS has other causes. But within the originally aired program, they used the word CURE, the word EXERCISE, and talked about SMALL HEARTS. They featured dramatically fainting astronauts, and a lady on an exercise bike. They may have been trying to tie into the NASA theme, but unfortunately what was conveyed to many was that POTS is caused by deconditioning and small hearts (aka The Grinch Syndrome), and we can all just hop on our exercise bikes like the lady in the segment and we'll be cured.

The reality is that POTS and Dysautonomia patients already face an uphill battle. Our illness is hard to understand, hard to explain, and most doctors and the public don't even know it exists. We get dismissed as having anxiety, or being lazy, or being hypochondriacs - until we get to the right doctors and are validated with a diagnosis. Getting to those doctors sometimes takes years. For me, it has taken almost a decade. I have had a wonderful Internist that read up on POTS and has been trying to find me the right local doctors (with no luck) for years. I have seen countless local specialists who know nothing about POTS, and was hospitalized for 9 months last year during a pregnancy that caused my POTS to go bonkers. I believe the care I received made my condition worse - because the medical professionals didn't know anything (and I guess were too busy to find out) how to care for someone in my medical situation. I almost died because of that, and almost lost my baby as well. I am left home bound and very often bed ridden due to the 9 months of forced bed rest, when if they had just listened to me when I told them I couldn't breath, and it was not "just my POTS," it was actually over a dozen Pulmonary Embolisms, we may have avoided some of these issues. If they had listened to me about getting up and moving (like my wonderful Physical Therapist at John Hopkins did for the 3 weeks I was with her) and read up a bit about bed rest being the worst possible thing for POTS, I may be in much better shape and not passing out after being upright for 90 seconds. My point is that I, and many others, go through enough having dysautonomia - it is a daily physical, mental, and emotional battle - and the thought of my syndrome being renamed after the Grinch is terrifying and infuriating.

THE FALLOUT

So as a result of the "Grinchy" heart story playing out on ABC, even without using that term, damage was done. I am fortunate that I have wonderful and supportive people in my life that have read up on what I have and grasp at how hard even simple tasks are for me. Or they try. But not everyone else does. So the fallout from this report was immediate. I started hearing from people about responses from family and friends telling them they just needed to exercise and they would be cured! Or that they must be lazy to have had POTS for so long and not be cured yet. Or even well meaning people worried about their small hearts, and did they have a recumbent bike yet? Here are some exact quotes from Facebook boards, groups, the comments section on ABC, and via email:

"Get a load of this...just found it posted on my wall. whew boy....."Hi ----name deleted----- ABC news tonite reported that nasa has come out with an important study on POTs syndrome including things that can be done to strenghten or enlarge the heart muscle...love you"

"It is hard enough to get doctors that don’t know anything about this condition take you serious but to add media that misinterpret this condition makes it hard for any of us to be taken serious. One of my own family members called after this was aired and told me they can’t me serious anymore and can’t believe I would lie about my condition and that I need to get off my couch and get exercising so I can take care of my family properly. I wish it were that simple but it is not and now I look like a liar to my family and friends. Please make sure if you are going to mention a condition you get the information correct because you know who is going to be effected by your interview/articles."

"I know just about everyone is posting about this but I must say I'm somewhat pissed and discouraged about ABC's story tonight. Yes pots is getting media coverage but the wrong type of information is being given. This is why most doctors think the way that they do about pots. This is why my neuro takes pots as joke. After watching this my boyfriend looks at me and says "See, what have I been telling you about exercise" :-/ "

"I'm not going to play the show for my husband for just that reason - he'll say "See, I keep telling you that the one thing you don't make the kids do is exercise regularly". I don't want to hear it -- I can only make them do so much!"

"Just got a call from my grandmother saying she didn't know I had a small heart and wondering why she never heard of this before today... She also wants to know why I sent her all that information a few months ago if it wasn't true. That was so upsetting to think my grandmother thinks I am lying to her and I just made everything up. She told me she will still love me but will not take me serious anymore and I better getting exercising like the doctor said so I can take care of my children again and stop being my mother. I can't believe that really just happened."

And so on, and so on. The worst was a post by the sister of the woman featured on the segment:

"My name is Leigh Ann Poole. My sister, Kathy Chandler, and I were at the Cleveland clinic for a week of additional cardiac and neurological tests when she was asked to share her story about POTS. This has been a very debilitating condition for her and many other people; and so she agreed to share her story and even repeat tests that were difficult and painful for her – for the camera – so people could learn and understand more about this very life alternating diagnosis. In fact this taping took about 6 hours to film and required her to be stuck 3 additional times for an IV. I accompanied her, often pushing her in a wheelchair, and watched all of the videoing for this story. So what happened? ABC used a few seconds of her story, took it highly out of context, and made her out to be really without disability and even pronounced her “cured” – even when they knew that she hadn’t even received her test results back to determine the cause of her POTS (and there are MANY). Further – she has submitted several responses to this blog that have not been published (even though posts before and after her submission were posted). She has friends and family now thinking her “prescription is simple” and all she has to do is exercise – oh and of course since ABC has declared her “cured” that she can now go back to her everyday life. People are even posting on this site that she doesn’t even have moderate POTS because ABC has made her disabling condition of extremely high heart rate (160) and extremely low blood pressure, extreme fatigue, blurred vision, etc out to be that she is just a “chronic fainter” who is now cured. Very disappointing and an outright total mischaracterization of the facts. I decided to try to post to see if in fact ABC will post my comments since they apparently will not even let her defend herself from these lies on a blog.

- Eye Witness

I would also like to add that we could not be more happy with the very thorough care Kathy received from her GREAT doctor – Dr. Jaeger – and the Cleveland Clinic. They truly are wonderful and hopefully will be able to help her.” (http://abcnews.go.co...comment-1050902)"

THIS STORY HAS BEEN DAMAGING TO MANY, AND ABC NEEDS TO DO THE RIGHT THING AND CORRECT THEIR BAD REPORTING.

Edited March 23, 2012 by Rachel

[issie]

Claire,

Yeah, I loved your blog about it. It's so not right. I don't see how the media grabs this one concept and ignores all the other information that is out there. They need to talk to some of us in other subsets - the ones excluded from his study.

Issie

[anna]

Really then it is quite obvious, that when articles like the British Heart foundation one http://www.bhf.org.u...aspx?page=13311 quoting Dr. Levine's study get printed in places meant for the medical profession to read, many of them would take it as it reads period! as most medics do not have time to go through all the theories and data out their.

So this protocol ends up being used as the definitive way to treat POTS, no matter the cause and ultimately no matter the cost!

We have seen this happen in the UK with regards to treating all CFS/ME patients; our National Institute of Clinical Excellence, whom set statutory guidelines to all the NHS (Our national health service) decided to go with the research work that stated that CFS/ME is best treated with CBT and GET(Graded Exercise Therapy).

A large number of specialists in the field of CFS/ME have explained to 'NICE' that GET has been proved to be ineffective and probably actually dangerous to many CFS/ME patients, but NICE have continued to argue that CBT and GET is the way CFS/ME has to be treated.

[jenglynn]

Jangles-

EXCELLENT point! Even though I've had autonomic /Autoimune issues my whole life, I have always been fit. I'm not saying it was easy for me (also have probable EDS) but have always been in shape. My husband, on the other hand, is DE-conditioned and perfectly healthy. Poor Grinch, getting all the blame!