“At first it was hard for her to get her mind around the fact that a simple virus could do so much to her.” New York Times article
The New York Times has a clever feature every month called “Diagnosis” where a doctor presents a mystery illness and asks readers to guess what it is.
The latest involved an active and high-performing 21 year old female college student who got a cold, shook it off but felt tired and out of it. A couple of weeks later she got out of bed – and hit the floor.
Whatever happened – it was quick and it was devastating
She made it to the bathroom where she saw a pale, ashen grey-lipped face staring back at her in the mirror. Two hours later she was in the emergency room. She didn’t realize it but her life as she knew it, was over.
It had been a rough year and a half for her: two back surgeries followed by whiplash and headaches from an auto accident. She was taking a pain-killer, a sleep medication and an antidepressant to help with her headaches and her sleep. Now, after a seeming mild cold, she was unable to stand without feeling like she was going to black out.
The next day, still at the hospital, she’s felt better except when she stood up – and her pulse skyrocketed to 140 beats/minute. She goes on to see a neurologist, gets a CT scan done and more.
All her medical records are posted on the article and the author, a doctor, takes the readers step by step through the process. It’s fascinating process. Then she turns it over to the readers. What does the patient have?
An amazing array of possible diagnoses pour in that if anything gave me some compassion for complex choices facing doctors. There was Marfan Syndrome, carotid-cavernous sinus fistula, stroke, migraine, chiari formation, conversion reaction, Lyme, anxiety, central nervous system infection, posterior encephalopathy syndrome, cerebral hypotension, Susac’s syndrome, reversible cerebrovasoconstriction syndrome, serotonin syndrome, sinus venous thrombosis, cerebral autosomal dominant arteriopathy, NMDAR encephalitis. pituitary tumor, temporal lobe epilepsy.
One doctor says she’ll eat crow if the patient doesn’t have a cerebral spinal fluid leak. Another self-proclaimed world expert on dizziness says she must have serotonin syndrome.
It’s a sophisticated crowd yet it takes 300 replies before someone gets what many of us already know: she has postural orthostatic tachycardia syndrome (POTS).
- Mystery Solved – See Think Like a Doctor: Swept Off Her Feet Solved
Postural Orthostatic Tachycardia Syndrome (POTS)
This young lady was lucky. The fact that she was taking an anti-depressant and anti-anxiety drug and had been seen at a pain clinic after an auto accident would have lead many doctors to conclude that her problems were psychological. After a careful examination of her records, however, the young doctor attending her noticed that her heart rate had skyrocketed when she was asked to stand in the emergency room. Her blood pressure, however, didn’t drop. That meant she wasn’t dehydrated – and it meant the doctor had no idea what was going on.
The doctor knew enough, though, to give her a tilt table test and curious about what was going on, accompanied her to the test. The results of the test introduced her to a new diagnosis: postural orthostatic tachycardia syndrome.
Could you have POTS without knowing it? Let’s take a short look at POTS.
Symptoms
According to Dysautonomia International the symptoms of POTS include: fatigue, headaches, lightheadedness, heart palpitations, exercise intolerance, nausea, diminished concentration, tremulousness (shaking), syncope (fainting), coldness or pain in the extremities, chest pain, shortness of breath, gastric motility problems and many others.
POTS ranges in severity from relative mild to being severely disabling. Dysautonomia International states POTS affects between 1-3 million people in the U.S., most of them women.
POTS Occurs When
- A sustained increase in heart rate of greater than 30 beats per minute occurs within 10 minutes of standing
- Those aged 12-19 years require an increase of at least 40 beats per minute
- These criteria may not apply to those with a low heart rate when resting
While a tilt test is preferred a home test can be diagnostic.
The Home POTS Test
- Lie down and rest.
- Measure your heart rate and blood pressure.
- Stand up and measure both after 2, 5 and 10 minutes. Be careful – you may get dizzy or faint.
- If your BP drops more than 20/10mmHg within 3 minutes, it’s not POTS. It’s OH and that requires a more detailed neuro work up, as it’s usually caused by something worse than POTS
Mayo Clinic Introduction to POTS
Finding Help
If your home tests suggests you have POTS (or if just your blood pressure drops precipitously which means you have another form of orthostatic intolerance) contact your doctor. If he/she doesn’t know about the disease -as they very well may not – look for a specialist.
- Check out Dysautonomia International’s Doctor’s Map here.
- POTSUK has a list of UK doctors here.
Prognosis
The NY Times article indicates the young woman knows that POTS is not going to go away. Some POTS patients do very well with treatment. Others, however, do not and there is no known cure for POTS.
A young woman’s post in the comments section of the NY Times article demonstrated how life-altering even well-managed POTS can be:
“While I am very glad to hear that this young woman was diagnosed so quickly, I also want to make it clear that her story did not end with that diagnosis. Though the basics of POTS are known – a malfunctioning autonomic nervous system which causes orthostatic intolerance and manifests in dizziness, migraines, joint pain, pounding heart, inability to sleep, depression, anxiety, chest pain, etc. – there exists no cure.
Lifestyle changes such as increasing salt and fluid intake, wearing compression stockings, gradually increasing exercise in addition to medication trials can mitigate some symptoms, but many POTS patients are unable to ever reach a stable and symptom free life.
Personally, I suffer daily from pain and discomfort due to POTS and I have lived this way for eight years. Now, a year after my diagnosis and in the care of one of the nation’s few POTS specialists at the Mayo Clinic in Arizona, my health is very bad.
Every day I battle myriad symptoms and the desperation and depression that come with living your teens and twenties under the cloud of a chronic illness. I am an ambitious and relentlessly driven person, and yet I had to repeatedly withdraw from college due to my illness. Even though I eventually graduated, which itself was constantly in doubt, I am not yet able to work and am back with my parents.”
Low Diagnostic Rates and Funding
The NIH does not include either POTS or orthostatic intolerance in the 250 or so diseases and conditions for which it tracks funding levels. There’s a good reason why. It’s only funding six POTS-associated studies to the tune about $1.5 million dollars a year.
POTS has got a couple of things going against it. It was first described in 1993 and it mostly affects women. It fits in a class of disorders that often affect women, are pretty common and cause a lot of distress but don’t usually kill. The NIH essentially ignores all these disorders. They include POTS, fibromyalgia, ME/CFS, interstitial cystitis and others.
Check out this POTS Cheat Sheet from Dysautonomia International
Ten Facts Doctor Should Know About POTS
1. POTS is a disorder of the autonomic nervous system. Approximately 50% of POTS patients have sudomotor neuropathy, and 20% have cardiac dropout on MIBG scans, which may be due to cardiac autonomic neuropathy.(1-4)
2. POTS is not rare. POTS impacts an estimated 1,000,000 to 3,000,000 Americans;(5-10, 30) 80-85% female, most commonly women of childbearing age.(7,8,11,12) POTS occurs around the world, but estimates are not available for other countries.
3. Research indicates that POTS may be an autoimmune condition. Antibodies targeting alpha1, beta1, and beta2 adrenergic receptors, muscarinic3 receptors,(12) g-AchR receptors,(3,13) and numerous cardiac lipid raft proteins(14) have been identified in POTS patients. Preliminary studies are underway exploring the use of IVIG in POTS.(15)
4. POTS or POTS like symptoms can appear secondary to other autoimmune conditions, such as Sjogren’s syndrome, lupus, antiphospholipid syndrome, celiac disease, ankylosing spondylitis, sarcoidosis, Lambert Eaton myasthenic syndrome, myasthenia gravis, and mulitple sclerosis.(9,12,16-21)
5. POTS can cause symptoms throughout the body including tachycardia, palpitations, chest pains, lightheadedness, fainting, nausea, fatigue, gastroparesis or rapid gastric emptying, migraines, blood pooling in the extremities, Raynaud’s, tremulousness, sleep abnormalities, cognitive impairments and more.(9,12,22)
6. The majority of POTS patients are hypovolemic despite adequate hydration. Standard blood and urine tests may not detect this hypovolemia, as the patient is typically deficient in plasma and RBCs. Blood volume analysis with a radio-tracer can be used to evaluate a POTS patient for hypovolemia.(23)
7. POTS is often misdiagnosed. The average time to diagnosis is 5 years and 11 months.(24) 85% of POTS patients are told it’s “all in their head” or given similar psychiatric labels prior to receiving a POTS diagnosis,(24) but research shows that POTS patients are no more likely to have psychiatric disorders than healthy controls.(25,26)
8. Most teens do not “outgrow” POTS. A 10 year retrospective study of Mayo Clinic’s pediatric POTS patients found that 60% had improved symptoms but still had POTS, 20% had recovered, and 20% had worsened or stayed the same since the time of diagnosis.(27)
9. The severity of POTS symptoms varies. Approximately 25% of POTS patients are so disabled that they cannot work or attend school.(9) The disability seen in POTS has been compared to COPD and congestive heart failure.(9)
10. Patients with suspected POTS should be referred for autonomic function testing including tilt with heart rate variability, Valsalva and QSART. In some cases, supine/upright catecholamine testing, blood volume analysis with a radio tracer, and skin biopsies to screen for sudomotor and sensory small fiber neuropathy may be used. An investigation should be made to search for underlying or contributing conditions such as autoimmunity, Ehlers-Danlos syndrome(28) or mast cell disorders.(29)
Resources
- Dysautonomia International
- PoTSUK
- National Institute of Neurological Disorders (NINDS) on POTS
More POTS Blogs From Health Rising
- From Chronic Fatigue Syndrome to Fibromyalgia To POTS To Success: One Woman’s Journey Through the Medical Profession
- Standing Clear: Drug Stops Orthostatic Intolerance in ME/CFS In Lab – Next Steps
- Problems Standing? Studies Suggest Autoimmunity Causes Orthostatic Intolerance: Implications For Chronic Fatigue Syndrome
- The Blood (Volume) Will Out: Is Ischemia A Key in Both POTS and Chronic Fatigue Syndrome?
- Insights From the 24th Autonomic Nervous System Symposium #1: Autoimmunity / CO2 / Bad Reflexes and Your Next Diagnosis
- Dysautonomia 101 From Lauren Stiles For Chronic Fatigue Syndrome and Fibromyalgia
P.O.T.S. just another label…labels…
My daughter was injured by one shot of Gardasil (HPV vaccine) in September 2010. She was diagnosed with POTS/Dysautonomia by May 2011. We are one of many families world wide that have children who have been seriously injured by the HPV vaccine (either called Gardasil or Cervarix). A very high percentage have been diagnosed with POTS and other autoimmune diseases. Most are disabled and suffer years of pain and other debilitating symptoms. Please go to Sanevax.org if you want more information. This organization has been a tremendous help to our family.
The different triggers are just amazing. I wonder what the common thread is…
Cort, we’ve all had vaccines. That’s something common to most likely all ME/CFS sufferers.
Recognizing post-Gardasil symptoms has made me think that perhaps all ME/CFS has been in the final analysis caused by vaccines.
i’ve had POTS many years ago and managed to get SALT in me then swished it down with some water and a minute or two i was feeling better! i barely made it to the kitchen for the salt but the way i felt, so very, very ill was just awful!
has anyone tried SALT?
now though, i’m on high BP pills! what a mess M.E. is! my dear mom had heart failure and COPD but was stronger and did more then i could!
My daughter developed POTS after Gardasil, as well. Excellent article!
She was also diagnosed with severe thiamine deficiency post gardasil and I have seen a few studies that indicate thiamine deficiency can be connected to POTS.
Is OH Orthostatic Hypotenstion?
I would strongly suggest that any medical practitioner view POTS as a SYMPTOM, not as a standalone dx. POTS has been found to be associated with such things as systemic inflammation, histamine intolerance (associated with a hyperactivated immune system), etc, POTS has also been associated with elevated nitric oxide, and both histamine and nitric oxide dilate blood vessels, paving the pathway for POTS-like symptoms.
Large amounts of nitric oxide may be produced by activated macrophages, and nitric oxide synthase can be produced in neutrophils in response to inflammation.
hint: Look for what’s causing a sustained (systemic) inflammation, including a recent Recombinant vaccination, which requires a very potent adjuvant, such as the Gardasil HPV vaccine, which is notorious for awakening dormant pathogens in formerly healthy children and adults. Be creative and do imaging on the Cisterna Chyli to asses the impact on the lymphatic system. An enlarged Cisterna Chyli may be used a gauge to determine systemic involvement, and may also reveal the efficacy of a particular recover protocol.
Hi l phillips…do you have an email address?
PPL@MAILBOX7.NET
My daughter was also diagnosed with POTS/autonomic dysfunction after HPV vaccination.
You can have POTS with other conditions too, like EDS/Hypermobility. And guess what, orthostatic tachycardia and poor circulating blood/pallor and cold extremities, was part of the Ramsay ME definition. So, if you started with acute viral onset and meet the criteria for ME, or feel you have something other than autonomic related symptoms, then it could be ME, not chronic fatigue.
I was recently diagnosed with POTS in UK, having had a ME/CFS diagnosis for many years before that. One label is about as helpful as the other. I ‘treat’ with obscene amounts of water and salt, which takes me from ‘please-kill-me-now’ to merely ‘pretty damn rough’. I also have reactive hypoglycemia (adrenaline dominance?) which again I ‘treat’ with a very low carb diet and occasional bio-identical progesterone cream. Any improvement I make is incredibly fragile, easily broken by a normal day out or a cream cake.
My expectation is that POTS has the same cause as CFS/ME, just with slightly different effects.
Pamela have you looked at ivabradine . I take half a tablet for cfs related POTS in the mornings. It has improved my symptoms enormously with negligible side effects, I can now walk which means that I am seeing improvements in my heart rate with improved muscle tone. It is one effective drug that is available in the UK. A cardiologist suggested it.
Very interesting article, thanks.
I don’t see a list of references, can you please direct me to where they are?
There are just some links in the blog but if you go to the DI site I think there are some for that paper.
Cort, you ask the $million dollar question re what could be the common thread. Risking this being too long a comment, I’m curious whether this idea resonates with anyone:
I find it intriguing that this 21 year old had not only 2 back surgeries (and an infection) but also a car accident with headaches and whiplash in the 18 months before the onset of POTS after a slight cold.
I’m a former family physician with ME/CFS x nearly 20 years and have been researching a theory that traumatic stress can be cumulative and plays an underestimated role in initiating chronic illnesses of all kinds because it can profoundly affect nervous system and immune system functioning (along with other physiological processes). I’m not talking about psychological issues causing symptoms here, but measurable changes in our physiologies that we are learning about in trauma research and studies in brain plasticity.
I’ve been curious about how different triggers of illness, such as infections and immunizations described by others above, as well as stressful life events (which so often precede the onset of a chronic illness), are actually precipitators of illness rather than the cause. This could help explain why so many of us have different triggers and onset stories (my onset was slow, following a series of deeply stressful as well as traumatic events with no evidence of infection). What we are learning about trauma and triggers may also help better understand exacerbations as well as unique variations we each have with our illnesses. We are also learning, for example, that adverse childhood experiences (ACEs) increase risk for chronic illnesses exponentially. It’s more complex than I can describe here, but the evidence is remarkable.
Does this concept resonate with anyone here? Have any of you experienced traumatic events that might have felt relevant to your illness but unexplainable – whether in childhood, going back to prenatal life or with a difficult birth, or occurring before the onset of your illness?
Here are some links on some of the research I refer to:
Here are a few abstracts I just found when looking up POTS and traumatic events (including traumatic brain injury):
http://www.ncbi.nlm.nih.gov/pubmed/20865679
http://www.ncbi.nlm.nih.gov/pubmed/25708501
http://www.ncbi.nlm.nih.gov/pubmed/22143364
ACE research described on NPR:
http://www.npr.org/blogs/health/2015/03/02/387007941/take-the-ace-quiz-and-learn-what-it-does-and-doesnt-mean
A woman with fibromyalgia and POTS-like symptoms who recovered when she was treated for the trauma of a childhood surgery with ether:
http://www.psychotherapy.net/interview/interview-peter-levine
Yes, indeed a fascinating topic. It must apply to a subset of patients because the pattern shows up again and again. Not to everyone but certainly to some. A series of events occur and then something, sometimes quite innocuous, tips them over the edge.
They are discovering in CRPS that an infection combined with a wound is necessary to trigger it. No infection no CRPS. An infection without a wound – no CRPS. An infection and a wound (and probably something else) and their system goes bananas. I have a blog on it somewhere. As usual I’ve forgotten most of the details 🙂 but I was struck that there is a pattern in time and they’re figuring out what sets up CRPS.
I’m not sure if occurs in everyone with CRPS or not.
Complex regional pain syndrome (I had to look it up!) and reflex sympathetic dystrophy (RSD) tend to have a link to trauma as well – I hadn’t heard about the need for both an infection and a wound to be present before. It is a fascinating (if completely disturbing, especially if you have any of these diagnoses) and I think “patterns” is what this is all about.
Retired neurologist Robert Scaer (website http://www.traumasoma.com/articles.html,) talks about whiplash (as occurred for the 21 year old college student in this blog post), RSD, and chronic illnesses in his two books on trauma:
The Body Bears the Burden
http://www.amazon.com/The-Body-Bears-Burden-Dissociation/dp/0789033356/ref=pd_bxgy_b_img_y
The Trauma Spectrum
http://www.amazon.com/Trauma-Spectrum-Hidden-Wounds-Resiliency/dp/0393704661/ref=sr_1_1?s=books&ie=UTF8&qid=1354235259&sr=1-1&keywords=trauma+spectrum
Here are a couple of blogs we did on it:
http://www.cortjohnson.org/blog/2014/08/01/autoimmune-research-new-options-chronic-pain/
http://www.cortjohnson.org/blog/2013/11/09/chronic-regional-pain-syndrome-fibromyalgia-mecfs-spreading-neuroinflammation-model/
Thank you veronique for all this information. I already ordered The Body Burden.
You might want to read a similar one; Das HWS-Trauma by Dr Bodo Kuklinski
(in german, available on Amazon.com) He makes the relation with acquired mitochondrial desease and gives some therapeutic solutions.
Hi Chris,
thanks for the info
It doesn’t look like Dr Bodo has any versions in English (on amazon or with a google) at this point?
My 15 year old daughter has been very unwell for a few years and I have been searching To figure out why? I feel in my gut that “something” Other than psychiatric issues are causing the vast number of different “symptoms” she has been experiencing…. ( not that there is anything to be ashamed of or insignificant with Phyciatric diagnosis!) The pediatrician and MANY docs I’ve taken her to Have without adequate testing determined she has anxiety, panic attacks, depression, migraines, ADD and oppositional diffiance…. I have with trepidation gone along with these diagnosis in order to help her in any way but continue to look for underlying causes… We have been treating her with migraine meds, anti anxiety and anti depressants, ADD meds and lots of therapy, tutors and help from ADD experts…. She has had negative reactions to all the meds we keep trying and doesn’t seem to be responding to therapy…. My daughter is one of those kids who catches everything and doesn’t get rid of it quickly…. She went through a period of serious strep infections that wouldn’t clear and just hasnt been “right” since… She complains daily of body pain as well as a number of other symptoms…she also used to have a tremendous amount of energy and required less sleep than most kids her age, but now can barely keep her eyes open during school, naps for hours after school and is always tired…. She can sleep most of the weekend and yet remain tired….. I keep asking drs to do more involved blood work and have taken her to different pediatricians and Adolsescent generalists, neurologists, ENT’s, gynecologists, phyciatrists, natural med docs, orthopeds and dentists…. They have been unwilling to do the in depth bloodwork I have asked for and my daughter is very uncooperative with needles, drs and just tired of drs telling her she has anxiety, depression and needs to try harder….she says she wants to but physically can’t and no one understands, including me ( she has always been a straight A motivated student till recently when she has “fallen apart” and her grades have gone from a 3.9 to barely getting by…. She can’t stay awake, focus or pay attention…. And is EXTREMELY frustrated…. If she pushes herself hard to do activities, play sports and study groups she gets sick with a bad cold…. The background info is that I am ill and have been getting progressively more so…. After many years of tests, drs and every medicine imaginable I was recently diagnosed with hashimotos thyroid, chronic fatigue syndrome, fibromyalgia and various interwoven auto immune disease, as well as an impaired immune system…. It has been mentioned that bc of all I have been through its possible I’m projecting it on my daughter…. I was also told initially my issues were Phyciatric which of course makes me question my daughters diagnosis….. But as I said my gut just tells me we don’t have the correct answers and barely anything but basic bloodwork, an EEG, MRI and many physical exams have been done….
Sorry so long but I am wondering if anyone can recommend specific tests and bloodwork and what kind of dr to see…. It has been a problem to find any adolescent drs as most I have found are peds who say they do adolescents but don’t do them well or drs who are not good with adolescents and my scared and uncomfortable daughter only gives yes and no answers and won’t tell them what’s going on and they want to hear it from her not me…. I’m open to ANY suggestions or advice!!!! Thank you for taking the time to read this and respond!!!!!
Caryn,
I am sorry to hear that both you and your daughter are having such medical issues. I can relate; my teenage daughter, although a few years older, also has the usual symptoms associated with chronic fatigue syndrome, fibromyalgia, IBS, POTS, and cognitive dysfunction, to name a few.
I don’t know where you live, but my best advice would be to find a doctor trained in functional medicine. You can see if one is in your area at https://www.functionalmedicine.org/ That said, several naturopaths, and chiropractors may have training in this area too. We actually took our daughter to a naturopath, and although she did many of the good tests that I’ve read about, I believe her interpretation of one of the tests led us down a wrong path. We just started seeing another naturopath, who is trained in functional medicine, and who has come up with a different diagnosis and treatment plan. So, time will tell what happens.
My point above is that any number of things can be causing your daughter’s or your own symptoms. These include, but are not limited to 1) SIBO, 2) intestinal permeability (leaky gut), 3) mold exposure, 4) food intolerances, 5) other toxic exposure, just to name a few GI related ones. My other point is that in my daughter’s case, any one particular root cause could be linked to ALL the symptoms she is having, and it is taking time, money and effort (hers especially) and all our patience to continue the testing to mostly rule out which problems NOT to address.
I know there are some good chronic fatigue specialists out there who are helping many people. My personal experience has been that, as good as they are, the ones we have seen (and we’ve seen a few) were more interested in addressing my daughter’s symptoms to provide some relief rather than digging deeper to get to root cause. Of course, providing symptomatic relief, even if it doesn’t get to the root cause, is extremely important too. It’s just a mindset difference I have noticed.
Since there is no end to how many diagnostic tests one can take, I would recommend finding a practitioner who can provide some perspective on which tests are the most productive. You may find you have to do the more mundane (CBC) blood tests to rule out a whole host of things, before you can get to some more meaningful ones.
I’ll recommend 2 books as references. The first is one written by Courtney Craig, a former CFS patient and now a practitioner in NYC. Her website is http://www.drcourtneycraig.com/
She wrote a book called “All My Test Results are Normal: A Smart Guide to Testing for Chronic Fatigue Syndrome”. I might have added a couple more tests to that book, but if you’re starting from scratch, I consider it a good source, considering my personal experience and journey in advocating for my daughter’s health.
The second book i’ll recommend is really a great introduction to what functional medicine is all about and why, in my opinion, you have to get to root cause. It is called “Just Be Well: A Book for Seekers of Vibrant Health”. Don’t let the title fool you. It is basically a series of case study reviews, written in plain English, which covers various body symptoms. I really like this book; the only thing I wished is that it was a bit more prescriptive on perhaps the order of what one should test for.
Sorry for a long reply, but I hope it helps. Don’t give up; tell your daughter to hang in there. Easy to say, hard to do. My daughter has had to take medical leave from school to focus on proper treatment. So, I understand probably more than you realize the impact on yourself and your family.
Good luck!
Regards,
Scotty81