The A-Z of PoTS and dysautonomia - all the terms (almost!)

Like many people, I hadn't ever heard of PoTS (postural orthostatic tachycardia syndrome) or dysautonomia before I was diagnosed with it. I had all these symptoms that didn't make sense, was told it was all anxiety, which I was sure it wasn't, and then eventually found my way to the right doctor who immediately sent me for tests.

Back then I wish I had known about helpful resources so that I could have better armed myself with knowledge to ask all the right questions. I am lucky as my doctor is very comprehensive, but there are still aspects that would have been useful to know prior to my appointments with him. So I hope this A-Z of PoTS / dysautonomia is helpful, and provides resources for you to further research.

I want to emphasise that this list does not cover all aspect of PoTS / dysautonomia and certainly does not cover the complex bodily systems or medical terms relevant to PoTS. Instead, it covers aspects of PoTS that relate to living with the condition. I am not a medical professional and this should not be taken as medical advice in any way. It is for informational purposes only. Please consult a medical professional for your healthcare.

What is PoTS / dysautonomia?

PoTS stands for postural orthostatic tachycardia syndrome and is a form of dysautonomia. As Dysautonomia International describes: the 'Autonomic Nervous System controls the "automatic" functions of the body that we do not consciously think about, such as heart rate, blood pressure, digestion, dilation and constriction of the pupils of the eye, kidney function, and temperature control. People living with various forms of dysautonomia have trouble regulating these systems, which can result in lightheadedness, fainting, unstable blood pressure, abnormal heart rates, malnutrition, and in severe cases, death'.

A

A is for anxiety. No don't worry, I'm not going to tell you that anxiety causes PoTS, and Dysautonomia International make this clear too. There is often overlap in the symptoms of both conditions, and many people who go on to be diagnosed with PoTS seem to have been told by a doctor that they have anxiety (as I was). Perhaps this is one of the reasons it takes so long for people to get a PoTS diagnosis.

A is also for advice. PoTS and dysautonomia are often described as being under-recognised, but not necessarily that rare. However, I think many of us will have had difficulties with getting a diagnosis as many doctors don't appear to be as informed as they could be. I had one look at me blankly and say 'POTS, you mean like pots and pans' when I described my conditions. Why she thought I was talking about cooking is another question . . . So it is definitely best to see a specialist doctor and seek their advice. If you are in the UK then PoTS UK has a list of doctors who treat people with the condition.

B

B is for blood pressure. The relationship between PoTS / dysautonomia and blood pressure appears complicated, and should be discussed with your specialist doctor.

C

C is for cardiologist. It is likely a cardiologist who is best placed to treat you if you are diagnosed with PoTS or dysautonomia. They may well refer you to have tests or to see doctors of other specialties such as a respiratory doctor or rheumatologist.

C is also for compression clothing. Some doctors recommend using compression clothing such as tights or leggings. To quote POTS UK: 'Compression of the lower limbs causes an increased blood return to the heart from the superficial veins in the legs. There has not yet been any research to prove that compression tights improve symptoms in PoTS, but some patients have reported them to be helpful'.

It is possible to get compression tights on prescription in the UK and you can order them online too. Have a look at this post for recommended strength of compression tights. Speak with a doctor before trying as they may not be recommended if you have other medical conditions. If you can't get on with tights (I couldn't) then compression leggings are another option. They don't have the same level of compression though. I like the SKINS leggings, which is the brand PoTS UK mentions.

D

D is for diagnosis. This can be complicated and other conditions should be ruled out. As described by PoTS UK, to be given a diagnosis of PoTS, a person needs to have:

• PoTS symptoms mostly when upright over a period of at least 3 months.
• A sustained increase in heart rate of greater than 30 beats per minute 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.
• There is usually no drop in blood pressure on standing.

D is also for diet. While there isn't a 'PoTS diet' there are some recommendations given by PoTS UK on fluids, carbohydrates and meals (more on salt below!). Always speak to your doctor before making any changes. As PoTS UK describe '[i]ncreased fluid intake is advised to increase circulatory blood volume, particularly in the morning . . . Overall, aim for a total of at least 2-3 litres of fluid per day'. Further advice is that eating 'small amounts at regular periods throughout the day can be helpful. Avoid large meals high in refined carbohydrate e.g. sugars, white flour. After eating, blood is diverted to the digestive tract and away from maintaining blood pressure and heart rate which may increase symptoms.  Consider having a rest after eating and avoid strenuous activities' (PoTS UK).

E

E is for ECG. During the process of investigating symptoms a person may be asked to have an ECG (electrocardiography) in order to rule out any heart problems that may cause symptoms similar to PoTS / dysautonomia.

E is for exercise. Exercise and PoTS / dysautonomia is challenging. Being dizzy, light-headed and having episodes of syncope for some, means simply sitting up or walking is difficult, never mind exercise. However, as Dysautonomia International describe: deconditioning 'may exacerbate symptoms, so it is important to take steps to prevent deconditioning from occurring, or to reverse it if the patient has already become deconditioned'.

Seeing a specialist physiotherapist / physical therapist may be beneficial (and was for me). They can advise on appropriate levels of exercise which may be done lying down, or sitting, and how to progress at a pace that is suitable for you.

F

F is for fluids. Many patients are advised to increase their fluid intake to 2-3 litres per day, and to make sure they are well hydrated in the morning in particular. As a note, 'Be aware that drinks containing caffeine can worsen tachycardia and symptoms in some people. However, caffeine can elevate blood pressure and improve wakefulness, and some patients find this useful' (PoTS UK).

G

G is for gender. While 'POTS can strike any age, gender or race, ... it is most often seen in women of child bearing age (between the ages of 15 and 50). Men and boys can develop it as well, but approximately 80% of patients are female' (Dysautonomia International).

G is for Gatorade. Many of my Instagram friends with PoTS in the US swear by this drink. It provides fluids and electrolytes that may help increase blood pressure.

H

H is for hEDS. Hypermobile Ehlers-Danlos Syndrome and Hypermobile Spectrum Disorder are conditions associated with PoTS. It is reported by PoTS UK that '50% of patients with HSD and hEDS met the diagnostic criteria for PoTS in a recent study by Celletti and colleagues.  Equally a survey of UK patients with PoTS identify 50% had a diagnosis of hypermobility'.

Personally, my cardiologist referred me for an assessment with a specialist rheumatologist for suspected hEDS. If your doctor does not suggest a referral it may be worth requesting one.

I

I is for Ivabradine. This is a medication that can be prescribed for PoTS, and one that slows the heart-rate (PoTS UK).

J

J is for journal articles (yep, I couldn't think of much else!). If you want to read some articles written by medical specialists, then PoTS UK has a list of some helpful ones on their website.

K

K is for, hmm, yes, can't think of anything. Onto L . . .

L

L is for the Levine protocol. This an exercise programme devised by Professor Levine after a study undertaken with a small group of PoTS patients. It is slightly controversial, and isn't a treatment offered by the NHS in the UK.

Personally, exercise has been highly beneficial for me, but (and it's a big but) I saw a specialist physiotherapist who devised an exercise programme specific to me and taking account of my other chronic illness conditions. Everyone is different, and specialist input is likely very important before embarking on an exercise programme.

M

M is for menstruation. It is recognised that menstruation may make symptoms of PoTS worse (PoTS UK).

M is also for Midodrine. A medication used for PoTS, midodrine is an alpha agonist. As PoTS UK describe, the 'aim of an alpha agonist is to narrow blood vessels to help return the blood back to the heart'. Your doctor can advise on appropriate medication.

N

N is for neuropathic PoTS, one of the sub-types of PoTS alongside Hypovolaemic PoTS and Hyperadrenergic PoTS. You can read more about these on this page of the PoTS UK website.

O

O is for orthostatic hypotension. This is a 'common condition characterized as a drop in blood pressure that occurs when a person stands up. OH can cause lightheadedness, dizziness or even causing a person to faint. Symptoms can also be subtle or absent' (National Organisation for Rare Disorders).

P

P is for PoTS UK, which is a great organisation providing a lot of resources and raising awareness and funds for more research. Definitely have a look at the website for more info!

P is also for pedal exerciser. If a physiotherapist recommends recumbent exercise, then this is a fairly low cost way to 'cycle' but without having to buy an exercise bike. I am a big fan and have used mine for years. You simply sit (or some people lie down) on your sofa or chair and pedal. Bonus is that you can watch TV at the same time!

This post on using a pedal exerciser may be helpful!

Q

Q is for questions. I often leave an appointment and then remember what I wanted to ask on the way home . . . Going in with a list is really helpful and my post on the next steps after a PoTS / dysautonomia diagnosis may be helpful.

R

R is for recumbent exercise, such as on a pedal exerciser or through activities such as Pilates. It is often thought best to begin with recumbent exercise if you live with PoTS.

R is also for remission, and for some this is possible as some patient stories presented by PoTS UK indicates.

S

S is for salt. An increased salt intake of 6g daily is recommended for some people with PoTS to help with increasing blood pressure (STARS). Personally I was recommended to use pink Himalayan salt. However, you should always consult a doctor first as some people with other medical conditions or certain forms of PoTS should not increase salt.

S is also for syncope (fainting). Some people with PoTS / dysautonomia experience fainting, particularly when moving from a lying or sitting position to standing.

T

T is for tachycardia. A symptom of dysautonomia, tachycardia refers to a heart-rate that is too fast. As the NHS describes in 'PoTS, the autonomic nervous system does not work properly. There's a drop in blood supply to the heart and brain when you become upright and the heart races to compensate for this'.

T is also for tilt test. Having a tilt test is a typical diagnostic test if a doctor suspects a person has PoTS. It is when 'your heart rate and blood pressure are measured while you lie on a specially designed bed that can be titled upright. The measurements are taken several more times while the bed is slowly tilted into a more upright position' (NHS).

T is also for temperature regulation, a symptom that some people with dysautonomia find to be an issue.

U

U is for under-recognised. As with many conditions, there isn't much awareness of dysautonomia in the broader society as there are for other medical conditions such as diabetes or migraine. Personally I have found that many doctors aren't as aware of dysautonomia as I think they should be either . . .

W

W is for warm environments. Hot weather can be a trigger for PoTS symptoms, and lead to increased tachycardia and other symptoms. Keeping cool in the summer is challenging but something like a headache hat for migraine attacks or ice towel may be helpful.

X

X is for x-ray. Hmm, not sure if x-ray is used for anything related to dysautonomia, so moving onto Y. Actually to Z, because I can't think of a Y . . .

Y

Y is for nothing (comment below if you know a Y term), so moving on to Z!

Z

Z is for zebra. There is an association between PoTS and Hypermobile Ehlers-Danlos Syndrome. The zebra is the symbol of EDS.

As described by the Ehlers-Danlos Society, medical students have been taught for decades that, '“When you hear hoofbeats behind you, don’t expect to see a zebra.” In other words, look for the more common and usual, not the surprising, diagnosis ... The zebra became our symbol because those with Ehlers-Danlos syndrome and hypermobility spectrum disorder are the unexpected. “Sometimes when you hear hoofbeats, it really is a zebra”'.

Useful websites

PoTS UK

Dysautonomia International

STARS (Syncope Trust and Reflex Anoxic Seizures)

NHS - PoTS page

Helpful reading

POTS - Together we Stand: Riding the Waves of Dysautonomia

The Dysautonomia Project: Understanding Autonomic Nervous System Disorders for Physicians and Patients

Don't forget to pin the post!

Please follow the advice of your doctor as to all medical treatments, supplements and dietary choices, as set out in my disclaimer. I am not a medical professional, and this post, as well as all other posts on this blog, are for informational purposes only.