21 Mar 2016

Disabling effects of PoTS shown in largest ever survey and how educating health professionals can help


Postural orthostatic Tachycardia syndrome or PoTS, is a complete bastard of a condition. It doesn't kill, thankfully, but it can quite often make you feel like you have no life in you.
It can be a condition on its own or a comorbidity of another condition, such as mine, Ehlers Danlos syndrome. 
In its extreme, life-altering state, it's pretty rare and extremely misunderstood. The charity PoTS UK, has teamed up with researchers and conducted the biggest ever survey of people - mainly women - living with the condition. The research, A profile of patients with postural tachycardia syndrome and their experience of healthcare in the UK is published in the British Journal of Cardiology.

It covered causes, symptoms and treatment options. You can find the whole survey here. 
Below are a few excerpts: 

Postural tachycardia syndrome (PoTS) is a recently recognised condition that usually affects younger women, who develop symptoms of orthostatic intolerance and a persistent tachycardia on standing upright. Healthcare professionals, patients and the national patient support group (PoTS UK) together created a survey, and the responses of 779 UK PoTS patients were analysed. The most common symptoms of PoTS at presentation were the triad of fatigue, lightheadedness and palpitations. Mobility, ability to work or attend education, and quality of life were significantly restricted. Cardiologists, followed by patients, were most likely to be the first to suggest the diagnosis of PoTS. Patients waited a mean of almost four years from presentation to obtain their diagnosis and, meantime, psychiatric mislabeling was common. Advice given to patients regarding lifestyle changes was variable, and those referred to specialist practitioners for help, found practitioners had limited knowledge about management of PoTS. Increased education of healthcare professionals and improved services for patients are recommended. 

And this is key. I find a little knowledge is a dangerous thing. If a health professional has heard of PoTS, their knowledge is usually incomplete and often based on faulty assumptions, such as, "Well your blood pressure didn't change quickly enough when you stood up so it can't be PoTs," or, "We did a 24 hour ECG and you were fine," despite numerous spikes in heartbeat of more than 30bpm when just standing or walking slowly up stairs (or half a staircase).

Treatments vary depending on if your symptoms are blood pressure or cardiac arrhythmia driven. For me, I have little blood pressure response, but my EDS means my blood vessels in my lower extremities are too stretchy to constrict quickly enough when I stand. This means blood begins to pool in my extremities, my brain tells my heart it isn't getting enough oxygenated blood and so the heart races to restore the flow. I feel dizzy, light-headed, pressure in my chest from a racing heart and nausea. It can continue for a while after I sit or sometimes have to lie down after the after-effects can linger for hours. On occasion, that's not enough and I have to put my legs in the air as well - tricky if you're out! If no chair is around, you'll see me bending completely over to try to even things out. So much for 'invisible illness'. 

The report says of PoTS:  

Diagnosis is usually made by active stand test or tilt-table testing. Few treatments for PoTS have been tested in randomised-controlled trials. Patients are initially managed with increased fluid and salt intake to increase blood volume, avoidance of symptom triggers (heat, prolonged standing, alcohol, drugs that induce tachycardia or hypotension), small frequent meals (low in refined carbohydrates), graded exercise (initially in a horizontal position) and compression garments to reduce venous pooling. Drug treatment can be aimed at reducing tachycardia (low-dose beta blockers, ivabradine, pyridostigmine), vasoconstriction (midodrine), boosting blood volume (fludrocortisone, desmopressin) or for their sympatholytic effects in hyperadrenergic PoTS (clonidine, methyl dopa).

showing heart rate of 151
My heart rate from 69 sitting to 151bpm 15 seconds later walking slowly half way up the stairs

Interestingly, 92% of the survey respondents were female and half had an additional diagnosis, like myself, of EDS. Many reported fatigue as one of the most debilitating symptoms.

Physicians call conditions like this 'benign' - i.e, you don't die from it. But believe me it feels anything but benign; it can be utterly life-altering and not in a good way.

As for diagnosis, many were wrongly told their condition was psychological or psychiatric- "It's all in you head, dear girl." Just writing that makes me extremely cross. And the least aware and the most important to educate? Your first port of call, the family GP. The report says: 

"In only 7% of cases did the GP suggest PoTS as a diagnosis. This may be due to lack of awareness in primary care about PoTS or the non-specific and multiple symptoms that are associated with PoTS. However, it is possible to undertake a 10-minute stand test in a consulting room, and perhaps this should be considered in patients with relevant medically unexplained symptoms or chronic fatigue, especially if there is a postural link to symptoms."

The impact of PoTS is considerable, with 23% becoming wheelchair users (including me, part-time for long distances) with 37% are unable to work. I am no longer able to be 'employed' but my time is used as a freelance writer, voluntarily with Special Needs Jungle and as a Trustee of Genetic Alliance UK. I am a 'real' Patient Advocate - I live it, and I work to help others. In other words, I don't do it for a living and I can't walk away into a better job when I fancy it. 

Tania in wheelchair with dog
One of my first times out in a wheelchair in 2013

The most important recommendations in the survey report are education of health professionals and the need to improve accessible and relevant rehabilitation services. It's no good saying go away and do some gentle exercise when often even getting out of bed is impossible. 

I pay for a one-to-one Pilates instructor, who completely understands the conditions I have because she is similarly affected. Her career choice has kept her mobile (most of the time!). I'm aiming for the same thing, though it is often two steps forward and one step back; sometimes two back. I have been awarded a Patient Fellowship to attend the three-day European Conference on Rare Diseases in Edinburgh in May - that's a big goal to increase my fitness for!

But even now, three years on from the onset of chronic illness and pain, depression can flip on easily, from someone's unkind or careless word from someone - another battle to guard against. As a mum of older teens with both Asperger syndrome and milder EDS, I can't just think about my own needs. If it wasn't for my husband, I wouldn't be able to manage. 

Congratulations to Leslie Kavi of PoTS UK, named as a co-author of the report. Great work and hopefully this will be printed out by as many medical practitioners as patients. If you're a patient or carer, print the report out and pass it to your GP, physiotherapist, workplace if you have one or child's school or college. They're most probably too busy to find it, so help them out with their understanding. 

The survey has many more detailed figures and much more information. Once again, find the report here: http://bjcardio.co.uk/2016/03/a-profile-of-patients-with-postural-tachycardia-syndrome-and-their-experience-of-healthcare-in-the-uk/