flop

In the past I would have easily met the criteria for POTS as we have plenty of 24hr ECGs showing my heart rate at 120-140 during the day when I was sending relatively still (eg waiting at the train station). Back then I didn't feel particularly ill - maybe a little breathless and fatigued but nothing that made me see a doctor about it.

In 2004 a viral illness turned what was probably compensated POTS into a full blown disabling illness with me feeling dreadful every moment I was upright and fainting many times per day.

The underlying cause of my POTS is Ehlers-Danlos Syndrome. My younger sister has never been properly evaluated herself but my rheumatologist diagnosed her and my Dad with EDS based on what I told him. My sister has recently stated to complain of vague tiredness and heat intolerance. I did a poor mans tilt test on her an it showed tachycardia 100 lying down and 124 standing so not quite enough for an official POTS diagnosis but close enough to suggest that she does have POTS. Now at the moment she functions perfectly well and can dash about like most people in their late 20s. I believe she has POTS but is not disabled by it at the moment (and I hope she doesn't ever have to go through the experiences I have over the past 7 years).

You should make a list of the aspects of your condition that make work difficult and a list of suggested accommodations that would help you overcome your disability (eg less hours or flexible hours, a footstool to elevate your legs, access to cold fresh water, able to take frequent short breaks to walk about and get your circulation moving). Employers have a legal responsibility to make reasonable accommodations to enable you to work.

Good luck,

Flop

My understanding is that when we get chest pain that seems to be cardiac (ie feels like a heart attack would feel) it is caused by too little blood flow getting down our coronary arteries. Unlike ischaemic heart disease it isn't caused by narrowings / blockages in the coronary arteries but by problems with blood flow in the area.

It is interesting to note that the coronary arteries receive their blood directly from the aorta but that blood flows down the coronary arteries during diastole (the gap between heart beats). When you have a fast tachycardia diastole gets shorter (ie there is less time between heart beats). This means that there is less time for the blood to flow down the coronary arteries. This would fit alongside and compound the thoracic hypovolaemia research. If there is less blood and less time for blood flow then the poor coronary arteries will struggle to get all the blood they desire.

Importantly POTS related chest pain should improve with rest and lying flat. Anyone getting new chest pain or different pain should always get checked out by a doctor but once you have been told it is POTS related and not ischaemic heart disease then you can treat the pain with rest and lying flat rather than having to visit the ER every time.

Flop

My tachycardia is Sinus Tachycardia too (by definition POTS is a sinus tachycardia as it is the autonomic control of the heart rate tat has gone haywire not the actual electrical system of the heart. Though of course many people have both POTS and SVT just to make things more interesting!).

Well controlled (ie a good day and on medications)

Lying - 60s

Sitting - 70s

Standing - 80-110

Climbing stairs - 100-130

Bad times

Lying - 60s

Sitting - 80-100

Standing - 120-180

Climbing stairs > 220

The fastest my sinus tachycardia was ever recorded was 238/min during a cardiac exercise tolerance test in 2003 (before I became ill with POTS)

Flop

Tests I've had:

GP (general practitioner / family medicine)

- ECG (= UK name for EKG), showed tachycardia.

Cardiologist

- ECG (tachycardia)

- Chest X-ray (normal)

- Echo (mild MR and TR, nothing significant)

- 24 hr Holter monitor (episodes of tachycardia)

- Cardiac memo (no recordings as I fainted and couldn't hold the device to my chest)

- External loop recorder (no syncope during the 6 weeks I wore the recorder)

- Tilt Table Test (TTT, continuous ECG, BP every 3 minutes) (heart rate 169, BP 96/90, diagnosed with POTS)

Electrophysiology Cardiologist

(I was referred to EP because of a family history of HOCM and they were worried I might be having episodes of VT)

- ECG (tachycardia)

- Electrophysiology Study (no arrhythmias induced)

- Implanted Loop Recorder (reveal device) (sinus tachycardia, sinus pauses)

Neurologist at specialist Autonomic Unit

- 24 hr BP monitor (episodes of tachycardia and hypotension)

- full 1hr TTT (with beat-to-beat BP recording) (showed POTS)

- autonomic function tests (valsalva, cold pressor response, hand grip) (no neuropathy)

- TTT after fasting then repeated 1 hour after liquid meal (confirmed POTS but no change with meal)

- Poor man's TTT (standing HR & BP for 15 mins) before and after exercise (cycle ergometer pedalled whilst lying flat) (confirmed POTS, POTS worse after exercise)

- Thermal threshold nerve tests (normal)

- Basic nerve conduction study (normal)

Rheumatologist

- diagnosed Ehlers-Danlos Syndrome by clinical examination, no testing done.

Several years later a neurologist found clinical signs of peripheral neuropathy and balance problems.

Neuro advised multiple blood tests, some were done but any not available in Wales.

-B12 levels (confirmed B12 deficiency), treating with B12 injections has improved the neuropathy symptoms

- I am still waiting for the MRI Brain & Spinal Cord, and Nerve Conduction Study that should have been requested last December.

Other tests done by my Gastroenterologist (all but flexi sig done last week) (I have chronic diarrhoea and over last few months have developed nausea and vomiting)

- flexible sigmoidoscopy (normal)

- OGD (upper endoscopy) (showed stomach full of bile)

- Oesophageal manometry (borderline normal but I was taking Alverine Citrate)

- 24hr Oesophageal pH (108 episodes f acid reflux in 18 hours but less than 5% of total time so test normal I was taking Ranitidine)

- Lactose-Hydrogen Breath Test (probably shows Lactose intolerance)

- Glucose-Hydrogen Breath Test (negative but a specialist has told me test should have used lactulose not glucose and been tested for longer that 2 hours as I may have gastroparesis complicating matters)

- B12 (within normal limits on monthly B12 injections)

- Red cell folate (high, on folic acid supplements)

- Full blood count (normal)

- Faecal elastase (awaiting result)

- Faecal pH (awaiting result)

- Stool microscopy, culture and sensitivity (awaiting result)

- Duodenal biopsies (awaiting result)

- Duodenal aspirate for culture (awaiting result)

- Transit time study (swallow capsules containing metal markers then have daily abdominal x-ray to track their progress) (I'm having this test next week)

My gastro problems are probably FODMAP intolerance causing diarrhoea, Small Intestinal Bacterial Overgrowth and possibly delayed gastric emptying. On a FODMAP free diet I go from diarrhoea 8-16 times per day to total constipation which makes me think that my underlying GI transit time is actually slow and the diarrhoea is due to the osmotic effect of undigested FODMAP sugars in my lower I system.

Sorry there is a lot of GI stuff there that may or may not be related to my autonomic dysfunction and Ehlers-Danlos Syndrome.

Flop

Fading out of sounds / hearing is a classic pre-syncope warning symptom. For me my vision and hearing often fade at the same time but they can happen separately. It is all related to the brain not getting enough blood flow / oxygen and so some brain functions start to switch off. Probably a good thing your daughter was lying down when this happened.

Flop

Hi Jen,

It sounds like you are having a really tough time at the moment. When I first developed severe problems with my POTS back in 2004/5 I was also fainting a lot, up to 10 times per day.

Whilst I can understand that your doctors are frightened that you will hurt yourself and have advised bed-rest to prevent injury I believe that bed rest will lead to deconditioning and ultimately make your symptoms worse. When I was first diagnosed my cardiologist told me that I was never to lie in bed all day and that no matter how ill I felt (even with flu) I was to sit in a chair with my legs down. He said this would force my body to try to counteract the force of gravity. He also said when I needed to concentrate to do something I cold curl my legs up in the chair or elevate them but after doing the task I should put them back down again.

Some of the initial measures to raise blood pressure include lots of water, lots of salt, compression stockings and abdominal compression. Many doctors also advocate raising the head of the bed on 2 bricks to encourage expansion of blood volume.

I hope you get to see a specialist who can help you soon,

Flop

Lieze, I'm assuming you live in a home without stairs at the moment? Have you considered trying to get your body accustomed to climbing stairs?

I used to have dreadful problems with heart rates over 200 climbing the stairs at work. I gradually made my body cope by climbing very slowly, one step at a time. My tendency was to get to the top quickly and then sit down. I started just going up 3-4 steps then back down and gradually increased the number of stairs until I could manage 13 at once (most UK houses have 13 stairs per floor).

I used to rent an apartment but now own a small house. I was really worried as the only bathroom is upstairs but mostly I can now walk up with just a bit of tachycardia. If I am having a really bad day I use my hands and feet for extra balance / safety but I've been in the house nearly 2 years and have not yet fallen or fainted on the stairs. Maybe you could work with a physical therapist on stair climbing - they often have sets of 3-4 stairs to teach people who are recovering from strokes etc.

Just my opinion. I hope you get to live in exactly the sort if house you want and if that means a stair-lift or some physio then good luck!!

Flop

I usually add a tiny pinch of Himalayan salt to each glass of water (not enough to taste salty, just enough to make the water feel soft in your mouth).

When I know I'm at risk of dehydration (hot weather without a/c, upset stomach etc) I drink dioralyte sachets in plain water. I avoid dioralyte relief though because it has cooked rice powder in it and messes up my stomach.

Flop

Drinking water in large volumes isn't that helpful for us. It is better to be constantly sipping fluids rather than having a lot at once. When you drink large volumes at once your body sends signals to the kidneys saying "oh dear, here comes a load of fluid, just get rid if it would you?"

You do need to take extra sodium with the fluids or your kidneys will just flush the fluids back out again. The sodium gets filtered out of the blood by the kidneys but the kidneys also have a mechanism to reabsorb the sodium and keep it in the blood. Due to osmosis water will follow the sodium back into the blood.

Don't overdo either the water or the salt. I find putting a tiny sprinkle of salt into each glass of water is about the right amount. You don't want the water to taste salty, just enough to make the water feel soft in your mouth (if you try it you'll know what I'm talking about!!). Personally I like to use Himalayan salt as it has loads of other minerals, tastes good and us a pretty pink colour).

Flop

I had skin prick allergy testing done a couple of years ago. I have stupidly severe hayfever and reactions to house dust mite.

I had to stop taking antihistamines and other allergy meds for 4 days before the testing. My test wasn't at all painful, they put a drop of liquid on the skin then used a plastic tool that the nurse put into the drop and rotated so it broke through the top layer of the skin. After about an hour I went back in to have the results "read" I had several large angry welts on my arm that were very itchy. I was then given antihistamines, a one-off dose of prednisolone and some hydrocortisone cream to rub on the welts. Within a few hours I was back to normal other than a few blotches on my arm.

I started having injection desensitisation treatment for dustmite and will hopefully get pollen too this winter.

My theory is that allergies cause the release of histamine into the body. Histamine is a potent vasodilator so lowers blood pressure. I find that having my allergies under control really helps my POTS symptoms too. Although I have to take mega doses of antihistamines Fexofenadine (allegra) 360mg twice per day (the usual dose is 120mg once a day!!).

Hi,

I have previously had the horrid tachycardia symptoms after regular dental anaesthetics and I didn't go numb.. I have even had molars filled without any anaesthetic at all (not something I'd recommend!!).

Last year my family found a new dentist who has been fantastic with me. He uses local anaesthetic on a cotton bud to numb the gum before doing the injections. He uses bupivacaine with epinephrine - I was worried about the epi but he explained that it is there to make the blood vessels contract so that the local anaesthetic stays in the area it is supposed to and isn't cleared away by the blood.

I don't know why I got so tachy from lidocaine but it doesn't happen with the bupivacaine + epi.

Another option is to talk to your dentist or PCP about taking small doses of a benzodiazepine before the visit to reduce the tachycardia response. My Dad has EDS and finds that 5mg diazepam the night before and 5mg an hour before allows him to tolerate treatment with no problems.

Best wishes for your appointment,

Flop

Sara is correct. The bundle-branches are special electrical conducting pathways in the ventricles. They take the electrical charge from the AV node and very quickly it travels down to the bottom of the ventricles via the bundle branches, the charge then spreads out through the heart muscle causing the venticles to contract from the bottom upwards.

When one of the bundle branches is damaged by a heart attack, it causes a change in the ECG (EKG). The QRS complex is the tall spiky bit on the ECG and it is normally very narrow. A bundle branch block makes the QRS complex wider as the electrical charge moves more slowly without the help of the fast conducting bundle branch fibres.

In some cases the bundle branch block can resolve in the early days after a heart attack but more usually it stays on the ECG as a marker of the old damage. In some people a bundle branch block is the only change on an ECG at the time of a heart attack. It will be helpful to doctors in the future if your Dad keeps a copy of his current ECG in his wallet. Then if he has chest pain in the future the ER docs can compare the ECGs they take with the copy of the ECG that is now "normal" for him.

I hope your Dad makes a speedy recovery from his heart attack,

Flop

I take ranitidine (generic Zantac) specifically to help with allergic reactions. My GP thought I was crazy when I said it helped with allergies as it is supposed to be used for reflux and heartburn. I showed the doctor some of the MCAD literature and pointed out that H2 blocker stands for histamine-2 blocker. I certainly get more allergy symptoms when not taking Zantac.

Flop

Duodenal biopsy to test for coeliac disease will only show positive if you have been eating gluten regularly for several months before the biopsy. This is because in coeliac disease (not gluten intolerance) the presence of gluten actually damages the wall of the duodenum and it is that damage that is seen under the microscope. If someone with coeliac disease follows a strict gluten free diet and then gas the biopsy they will get a false-negative result.

Flop

My POTS is secondary to Ehlers-Danlos Syndrome. When I was diagnose with EDS Prof Grahame asked about my family and thought that my paternal grandmother, father and sister also have EDS.

The only person to have POTS type symptoms other than myself is my sister. She doesn't get the consistent 30 beat rise in heart rate but does get an increase in hr and is gradually experiencing symptoms like fatigue, exercise intolerance and heat intolerance. I hope for her sake that she doesn't develop full blown POTS.

Flop

I was started on Paroxetine 20mg (Paxil) in 2006 for POTS. Last year one of my doctors decided that I should go onto Duloxetine (Cymbalta) instead.

Having once accidentally missed my paroxetine for a whole week (pharmacy error) I was terrified of feeling as ill as that again. My GP tried to put me on 10mg/day for a week then 10mg alternate days for a week but this was way too fast. In the end we mixed low doses of both meds (only with doctors advice!!).

One of my friends has weaned off paroxetine twice (before each of her pregnancies) and advised me to make tiny reductions in the dose each week. She found the easiest way was to get a liquid preparation of the SSRI so she could measure the volume in an oral syringe. I think she allowed about 3 months for the whole weaning process and didn't have any problems.

Good luck,

Flop

I've put 6-10 years. My POTS became suddenly disabling in 2004 following a viral infection. But knowing now that I have Ehlers-Danlos Syndrome, and looking retrospectively, I had had milder symptoms for many years. I hadn't previously realised that my fast heart rates of 130ish and feeling generally unwell a lot of the time were related to me being stood up all day. I hadn't bothered to check my pulse when I felt well (when lying down or sitting) only when I felt ill. Perhaps if I had realised it was a postural issue earlier I might have got treatment sooner, who knows??

Flop

PS - a few posts back someone asked if POTS was a form of Autonomic Neuropathy. The wording varies between doctors and hospitals but it is generally accepted that POTS is a form of autonomic dysfunction (=dysautonomia). This means that the ANS (autonomic nervous system) is not working correctly.

Neuropathy means that there is something structurally wrong with a nerve. In POTS it is often that the ANS is structurally okay but it is functioning abnormally.

Some people on the forum do have autonomic neuropathy. Some people have been diagnosed with small fibre neuropathy and/or large fibre neuropathy. The research into POTS and related autonomic problems is relatively new in medicine (since the 1990s mostly) and there is still an awful lot to be learnt!!

Hi, I think this is a question for your doctor. Sudden weight gain like that is often due to fluid building up, and given your recent CT scan and swollen belly, fluid in the abdomen might be the cause.

Can you get in to see your regular doctor in the next day or so? If not then going to urgent care or the ER would be sensible so that they can start to figure out what the problem is.

I hope you get some medical answers and can start to feel a bit better soon.

Flop

Sounds fab, if anyone has a way of recording it so I can watch it in the UK please let me know.

Flop

Hi Jonathan,

I'm in the UK and am on Octreotide. I had tried all the usual medications (water, salt, compression, fludrocortisone, beta-blockers, SSRI, midodrine, then Ivabradine (not a "usual POTS med")). I got referred to a specialist centre in the UK but they were very slow to actually give me anything to treat my POTS and syncope. Eventually I was put on a tiny dose of Octreotide in May this year (25 micrograms sub/cut twice daily).

I found the injections to be immediately helpful. I take them on top of a whold host of other medications. When I started the injections I was under close monitoring as a day-patient in hospital (probably over cautious). Before my first injection I could stand for 2 minutes and my heart rate rose from 70 to 120/min during that time. By 30 mins after the first injection I could stand for 9 minutes before needing to sit down and my heart rate stayed in the 80s.

At the 25 mcg dose I get relief of my symptoms for about 2 hours before it wears off. I did ask the UK specialists to increase my dose but I am still waiting for a decision from them.

I recently traveled to the USA and saw several doctors. One of them is using Octreotide for "refractory POTS" (where patients haven't responded to the usual medications). The starting dose being used in that hospital is 50 micrograms two or three times daily (they laughed at my "tiny" dose and suggested doubling it immediately). They titrate the dose up to 100 micrograms three times daily depending on the patient's response. I have just seen my own GP and managed to get a prescription for the increased dose and also larger syringes.

My initial thoughts on octreotide was that it was a "wonder drug" - it made a big difference. I'm now not so sure but I suspect that I am doing a lot more physical activity than previously and I am therefore now struggling more with the pain and fatigue side of my conditions rather than the BP and heart rate which previously were my predominant symptoms.

The initial side-effects for me were abdominal pain, bloating and cramping, constipation and diarrhoea. It makes your stools go a funny colour and you can't digest fats properly anymore (octreotide stops the gall bladder from contracting). I have chronic problems with my bowels and usually have a lot of diarrhoea, once settled on the injections they actually seem to reduce my diarrhoea slightly. For the first month or two I was very bloated and looked about 8 months pregnant but that has also settled down.

I've met Prof Rose Anne Kenney a couple of times at conferences in the UK (STARS have an annual patient's day at the heart rhythm congress). She seems very nice and very knowledgable. I know her interest is in syncope (particularly in the elderly) and if anyone in Ireland is going to be able to help you then she is probably that person. I don't know if she is using octreotide in her patients but if not she is the sort of doctor who would research it for you.

Flop

Hugs Caterpilly,

it is tough to have one major illness but add cancer and chemo and there is no wonder that your body is begging for a rest.

Can you list the medications you have tried (and what effect or side-effects they had) as there may well be treatments you haven't yet tried. Finding the right meds is tricky and involves trial and error to end up with a combination that works for you.

Flop

Hi Stace,

I haven't had either implanon or mirena myself but I did do extensive research about them 18 months ago when I was considering having a mirena fitted.

Firstly your doctor's advice that only women who have had children can have a copper IUD is (in the UK anyway) considered to be out of date. It is easier to fit an IUD in someone who has given birth naturally but it is perfectly possible to fit them in other women too. In fact in the UK the copper IUD is offered as a form of emergency contraception up to 5 days after unprotected intercourse even to girls in their early teens.

Implanon vs Mirena. The obvious difference is the location - implant in the arm (local anaesthetic, tiny incision, pressure dressing on upper arm) or fitted inside the uterus. Implanon lasts 3 years vs Mirena 5 years, but both can be removed at any time if you get side-effects or decide that you want a child. Hormone dose - Implanon hormones travel from your arm throughout the whole body via the blood, Mirena hormones work directly on the lining of the womb so a much smaller dose of hormone is needed (but some does still travel through your body). Because of the lower dose of hormones the side-effects are much less common with Mirena than with Implanon. I know that family planning clinics in the UK use hundreds of both systems and most people are satisfied and come back to have a new implant or IUS when theirs is running out, very few people have side-effects so severely that they want them removed early.

Hope you find something that works well for you,

Flop

Issie,

I now understand your question. Unfortunately the doctors who do treat POTS etc all have their own ideas and don't use the same medications or treatment strategies.

For example my local cardiologist in the UK read a paper that showed that one SSRI (Paxil = Paroxetine) was helpful for POTS and started me on Paroxetine. I was then seen at a UK specialist centre where the doctors say that all SSRIs are really bad for POTS and it may be causing my symptoms. Then I travelled to the USA and saw a specialist who said that SSRIs are helpful but that combination meds like Cymbalta may be even better.

3 doctors = 3 different answers.

The same applies to sub-groups of POTS or causes of POTS. Some doctors firmly believe that Ehlers-Danlos syndrome is a cause of POTS, another specialist thinks that EDS is overdiagnosed and that there is no proof of a connection (just co-incidence that many people have both POTS and EDS?). One doctor uses high-flow, low-flow and normal-flow as his descriptions of different sub-groups. Other doctors use hyperadrenergic POTS, post-viral POTs etc.

POTS is almost certainly not one disease but many different diseases sharing some common symptoms (this is why POTS is a syndrome = collection of symptoms not an actual disease).

Because every patient's POTS is different, every patient's treatment needs to be different too. Some people respond really well to say beta-blockers but other patients feel ten times worse on the same beta-blocker. Even the top specialists are using a trial and error approach on an individual patient basis. To create a fixed treatment regime for POTS just would not work. The best we can achieve at the moment is a list of medications that have helped some people (such as the list in the "Changes" DVD). There is no way of predicting who will respond well to which medications.

Until a lot more research has been done we are a long way from being able to product the sort of guidance you describe. Unfortunately because POTS is so rare, medications wouldn't bring in mega-bucks for drug companies so the drug companies don't pay for research. (A new medication for heart disease, diabetes or high cholesterol can earn billions of dollars for a drug company so that is where the research money is spent).

Flop

Flop

Issie,

have you seen the DINET documentary DVD "Changes"? This film includes most of the things that you listed in your post

- 2 doctors who treat autonomic dysfunction

- 2 patients explaining how their lives have changed

- demonstration of the varied symptoms of POTS

- discussion and lists of medication options.

The documentary took a lot of effort from the team who made it and is perfect for showing to both family/friends and sending to your doctors.

The main difference between running non-profit organisations for mainstream illnesses (such as diabetes, cancer, MS) and running one for rare invisible illnesses such as POTS is purely the number of people affected. Almost everyone knows someone with diabetes or cancer (1 in 3 of us will get cancer) so the whole population is aware of the condition. Where as hardly anyone has heard of POTS unless they have it themselves (and usually after a battle to find out what is wrong).

When lots of people are affected by an illness you only need a small proportion of them to donate money to collect a large sum of money to use for education and research.

When an illness is rare and disabling most of the people affected have little energy to give and are often in difficult financial situations.

DINET was founded by Michelle Sawicki because there was nowhere to find out medical information about dysautonomia. Michelle created the huge collection of medical literature that is on the main DINET site that allows us to research our own condition and guide our doctors. Along with the DINET website she formed this forum to let us share information with other people suffering the same symptoms.

Running DINET and it's various projects (maintaining the website, running and moderating the forum, the t-shirt project, making and distributing the documentary etc) takes a huge amount of time and commitment and it is all done by volunteers who themselves have POTS or other autonomic disorders.

I would love to have a huge non-profit organisation fighting for us but without lots more people and lots more money I don't see how it can be achieved. There are thousands of charities for different rare-diseases but how many of them have you heard of??

An approach that I favour is begining to grow in the UK. "Rare Disease UK" is an alliance for people with rare diseases and all who support them.

Key statistics:

there are over 6000 recognised rare conditions

1 in 17 people will develop a rare condition at some point in their lives

3.5 million people in the UK will be affected by a rare disease at some point

30 million people in Europe are affected by a rare disease

By joining forces with other rare diseases our voices can be more clearly heard. The much larger numbers of people in this sort of alliance mean that the group has much larger lobbying powers.

Stratergy:

- research into rare diseases

- prevention and diagnosis of rare diseases

- treatment of rare diseases

- information on rare diseases for patients and the public

- commissioning and planning for rare diseases

- care and support for rare disease patients

by working together this alliance managed to get an early day motion in parliament calling on the government to adopt the stratergy above - it was signed by 71 MPs.

My personal belief is that by joining forces with other rare diseases we have a much better chance of getting the attention, funding, research and treatments that we need.

Flop

Hi,

clonidine can lower your BP as well as lower your heart rate so cutting back might actually increase your BP. Do you have an understanding PCP that you can call to discuss the recent changes and advise you on altering your meds?

Flop