I posted this in August last year. Due to having a crazy week (Insomnia/painsomnia) and a dentist's appointment today I find myself too exhausted to write a new blog post.
My own diagnosis has moved away from "just" PoTs (although that is still part of it) and is now called Severe Autonomic Nervous System Dysfunction, which as far as I can tell is just a long winded way to say Dysautonomia. Which isn't a medical term that seems to have caught on in the UK. All it means is instead of just the basic PoTs stuff going on I have developed O.I (orthostatic intolerance - my blood pressure drops in to the toilet on standing), breathing difficulties which have been with me since the MG/ not MG diagnosis, bladder problems ( I get acute urinary retention on a regular basis), unable to maintain my body temperature just to name a few. This is a new and emerging area of medicine and many medics are yet to catch up on PoTS let alone Dysautonomia. Straight away please let me direct you to this website pots.uk.org so if you are currently battling to get a diagnosis you can print this guide for gp's and take this with you at your next appointment.
The first symptom I developed was sweating heavily when sleeping, be it a nap or a proper night's sleep. That was back in 2006 and which I now know is reactive hypoglycemia (blog post) from monitoring my blood glucose levels. Throughout my life I had been told I had low blood pressure (not a prerequisite for PoTS as people with all levels of blood pressure can have it), I had a lot of dizzy spells, odd cravings for salt, palpitations. I put my palpitations down to anxiety, as I had always been quite an anxious person. However once my anxiety issues had been dealt with and I learned how to combat them I continued to be left with a feeling of my heart wanting to beat out of my throat on random occasions. I would also on a regular basis get very lightheaded when changing from a sitting to standing position.
So many patients with PoTS are misdiagnosed as having anxiety, health anxiety or depression or other mental health labels before they eventually get diagnosed correctly that it is criminal. Unfortunately once you have had the mental health label attached by the medics it is a hard one to shake off. Even now when going into hospital as an emergency, I still get somatiform disorder brought up when it has been completely and utterly disproven. The problem is that to an uninformed doctor PoTS can sound very like anxiety, racing heart rate, tremors the feeling of adrenaline coursing through your body and an exaggerated flight or fight response. With the majority of PoTS patients also being women it can be even harder to be taken seriously. It seems the modern medical profession still believe in the condition hysteria.
By the time I discovered the little known condition of PoTS, like many I had been written off by the medical profession as overly anxious, internet searching for syndromes with probable somatiform disorder. It took me over a year of battling to finally get the test that would prove beyond all reasonable doubt I had PoTS. A medical condition at the time my old gp had told me didn't exist. For more info on my experience of the Tilt Table Test please click here as I don't want to bore you by repeating myself.
My main symptoms at the time of diagnosis were as follows
- feeling faint or fainting (aka pre-syncope and syncope. Please remember only about 30% of PoTS patients faint but many feel faint)
- feeling like an elephant was sat on my chest and not being able to breathe properly
- racing heart rate every time I changed position, even rolling over in bed.
- greying out, especially after eating. (My peripheral vision greys out)
- Insomnia (BIG TIME)
- Migraines and shockingly bad almost migraines
- Cognitive deficit (aka brain fog /aphasia/ short term/long term memory problems)
- Chest pain (too many times to mention I thought I was going to have a heart attack)
- Reduced sweating (some people start getting excessive sweating, I only get excessive sweating at night)
This list probably doesn't cover all my symptoms and unfortunately many PoTS symptoms also merge into EDS symptoms as in the gut problems and bladder problems that many of us with both conditions have. It can get very difficult to differentiate the two. For a full list of symptoms please click here.
So what can you do if you suspect you have PoTS?
I can only base this on how I went about getting my diagnosis but it is the same advice I give everyone who asks my advice.
Firstly get hold of a decent blood pressure monitor especially one that can also take your pulse at the same time. Here is a list of blood pressure monitors that have been validated by the British Heart Foundation as accurate enough to monitor your blood pressure at home. If you can afford it get one with a memory so it records your readings so that you can't be accused of making the numbers up. It is important to understand that blood pressure does not play an important part in getting a diagnosis. Some people with PoTS have normal blood pressure, some have low and others have high. Why you need the BP monitor is so that you can start tracking your pulse and your blood pressure. If you can't afford a blood pressure monitor for whatever reason, a pulse oximeter ( a little device than can read your pulse by clipping onto your finger) is the next best thing and you can pick them up online from as little as £10.
Please remember people with PoTS tend to have very cold fingers (and toes) so before using a pulse oximeter ensure your fingers are warm enough to provide an accurate reading. Before my second tilt table test (yes I was stupid enough to do it twice) I had to sit with my fingers in a bowl of warm water as the pulse ox couldn't get a reading.
Secondly understand what PoTS is!
PoTs is a rise in your pulse / heart rate of at least 30 beats per min (bpm) or hitting 120bpm or over, within the first ten minutes of standing. In children (up to the age of 19) the rise needs to be over 40bpm. If your heart rate doesn't increase by 30bpm or over 120bpm within the first ten minutes of standing it is unlikely to be PoTS.
When a normal person goes from sitting to standing their pulse / heart rate will increase on average by 15-20bpm. Within a minute or so their heart rate will go back to normal. A person with PoTS could start with a baseline heart rate of 80bpm and on standing increase to at least 110bpm. Then as they continue to stand their pulse could increase further. I know on my own tilt table test my own heart rate increased to 150bpm, unfortunately I don't know what my baseline measurement was. Usually my pulse is in the 80's and if that was the case I certainly managed the increase of 30bpm and smashed the 120bpm threshold.
Because many people (but not all) with PoTS also seem to have low blood pressure that is why I recommend getting a monitor. It helps to see what your blood pressure and pulse are doing after specific triggers such as climbing the stairs, raising your hand above your head, eating - particularly carb loaded meals. Many people with PoTS find their symptoms are much worse after eating heavy carb meals, so it is recommended to eat high protein low carbohydrate meals which don't cause postprandial symptoms.
Thirdly conduct your own poor man's tilt table test.
You maybe wondering what on earth is a poor man's tilt table test, that is ok I was left scratching my head after first hearing this term on a forum called DINET. Because none of us own our very own tilt table designed for testing people for PoTS amongst other things, we can replicate how we will respond to the actual test by completing the poor man's tilt table test and some gp's who are PoTS savvy will do this in their consulting room or at the very least conduct a standing test.
To conduct a poor man's tilt table test, you need somewhere comfortable and relaxing to lie down, without interruptions and preferably away from harsh or natural light. A wall to stand against which is adjacent to the relaxing lie down area however if you don't have this don't worry you can still conduct the test. You also need your bp monitor / pulse ox and a handy sidekick to record your test results. Also your sidekick can rescue you should you faint whilst completing this test so please be careful and do not attempt to complete it without having someone with you. Please also ensure that the area is safe should you decide to face plant, so free from things you could hurt yourself on. Cushions or pillows on the floor can help prevent injury!
To start the test you need to lie down and relax, no talking, no interruptions for 20 minutes. If you can darken the room in anyway before starting the test do. At the end of the 20 minutes with as little movement as possible take your blood pressure and pulse readings. These are your base line readings and what you will use to compare against the reading you get during the test.
Once you have your baseline readings stand as quickly as you can placing your back against the wall. The wall just helps prevent you from moving, people with PoTS tend to be natural fidgets because we either faint or feel like we are going to faint. Moving keeps the blood flowing, we don't want you to do that whilst you are standing up as it may mess up the results. If you don't have a wall to prop yourself up against try and keep your legs as still as possible.
Then at 1 minute, 2 minutes, 5 minutes, 7 minutes and 10 minutes take readings of your blood pressure and pulse or get your trusty assistant to do it. After the ten minute reading has been taken, sit down get your legs elevated and have a drink of water. You may notice that your feet / legs have gone a nice shade of red or purple. Don't be alarmed this is called blood pooling and is very common with PoTs.
Now take a look at your readings if it shows an increase of 30+ bpm / 120 bpm this is positive for PoTS. Do not despair if it doesn't show PoTS one negative poor man's tilt table test does not mean it's not PoTS, it may just mean you weren't particularly symptomatic that day. Try to complete the test on a day when your symptoms are more evident.
Other things that might show during a poor man's tilt table test are things like orthostatic intolerance ( your blood pressure goes below 90/60 on standing), you may have also fainted, if so I am sorry. The blood pressure readings could also reveal if you suffer from hyper-PoTS, which is where you have high blood pressure and PoTS. This needs to be treated differently to "normal" PoTS as most doctors will avoid giving you medication that will increase your blood pressure.
If you can't for whatever reason do the poor man's tilt table test you could perhaps try what is known as the standing test. Instead of lying down you sit down for 5 to 10 minutes to get your baseline pulse and blood pressure readings and then stand for as long as you can. Taking your readings at the same intervals as the poor mans test. This can also show PoTS. Remember PoTS stands for postural (position) orthostatic, tachycardia (fast heart rate / pulse), Syndrome (collection of symptoms and no two patients are alike).
What to do next?
Print off the gp's guide from http://www.potsuk.org/gp_guide and book an appointment with your gp or a doctor at the practice who has been the most supportive. Sometimes these aren't necessarily the same. Whilst waiting for your appointment take a look (if you are in the UK) at the Doctors list on the same website. This will give you an idea of who you can ask to be referred to, not all cardiologists are knowledgeable in the area of PoTS so it makes sense to see an expert, rather than someone who will give you the run around. For international readers DINET also has a Physician's list.
If you have a disappointing gp visit and they still will not listen to you even when provided with your own test results and the gp guide, do not give up. Try every doctor at the practice if you have to and if that doesn't work contact the practice manager. If you get no joy after all of that try reaching out to one of the many UK facebook PoTS groups. They have members all over the country and they maybe able to help you find a new gp practice with more sympathetic gp's who are aware of the condition.
If you have a disappointing consultants visit because despite your request of being sent to a specific doctor who is knowledgeable about the condition and you didn't get to see one, you have a right to ask for a second opinion and ask once again to see one of the doctors named on the potsuk.org website.
You will get there in the end!
Most doctors will not confirm a diagnosis of PoTS without conducting a Tilt Table Test, ECG and possibly 24 hour blood pressure / heart monitoring. I was diagnosed on my tilt table results alone possibly because from 2007-2011 I had so many other investigations conducted they decided nothing more was needed.
The good news is that many people recover from PoTS especially if they have primary PoTS. Primary PoTS is usually caused by pregnancy, a virus, trauma or prolonged bed rest. Astronauts suffer from PoTS when returning to the Earth's atmosphere and when their bodies re-adjust to the Earth's gravitational pull their symptoms disappear.
Teenagers who develop PoTS also have a high chance of growing out of it. Researchers believe that teenagers develop PoTS due to the hormonal changes and growth spurts they are subjected to. Patients like these tend to grow out of their symptoms by their mid- twenties.
Patients with secondary PoTS, so PoTS that has happened due to a primary condition such as cancer, autoimmune diseases (like M.S, Lupus, Sjogrens), Ehlers Danlos Syndrome can also look forward to on the whole having the condition managed through medication and lifestyle changes (exercise, increased fluids, salt, adequate rest).
However I would be remiss if I didn't state that there are patients like me that have refractory PoTS (it doesn't respond to medication) and go onto develop more issues relating to our autonomic nervous system. We are the minority though not the majority. I don't want to scare anyone who has just started on this journey I just want to be truthful.
My symptoms now are pretty much the same as when I first started on this journey with PoTS / Dysautonomia. I still have Ptosis on and off which no one has ever been able to explain. I still have issues with my breathing, fatigue, syncope and pre-syncope. A good day means that my heart isn't continually feeling like it is fighting out of my chest on every change in posture. A bad day means not being able to sit up in bed without feeling faint, my heart rate not dropping below 100bpm even at rest.
With my combined conditions each day is very much different with a new set of symptoms to overcome. It is exhausting, it makes me angry, it makes me sad and quite a lot of the time it makes me laugh because my body is just so screwed up!
One last thing.....if you are diagnosed with any type of tachycardia, including PoTS and you hold a driving licence you must inform the DVLA. You can do that via this Link. You will also need to contact your car insurance providers.
Another great source of information is Stars another charity which helps people suffering with a variety of conditions including PoTS.