A blog about being too bendy. To raise awareness of Ehlers Danlos Syndrome.
Being diagnosed with POTS.
(1994-98).
The first time I remember passing out I was thirteen. My father has always passed out easily and it started happening to me occasionally too. When I was fifteen, I started getting chest pains and feeling breathless while playing football. My GP diagnosed exercise induced asthma and gave me an inhaler. It didn’t help. I was a regular visitor to the doctor and casualty as a teenager, usually with sport related injuries. Despite the injuries and chest pains I played sport three or four times a week. I was a happy, seemingly healthy guy.
When I left home and became a student at 19, my lifestyle changed. I stopped exercising, and was out at parties or nightclubs several nights a week. It was a lot of fun but I was treating my body terribly and I soon started to have problems, with headaches, tiredness, and joint pain. My GP said I needed to change my lifestyle. It made sense, but I was enjoying myself too much to follow his advice.
My symptoms became more frequent over the next year. I managed to keep functioning, but at Christmas in 1994 I got a chest infection. I thought I was getting better, but a week later while standing in a queue for a nightclub I felt so ill I thought I was going to collapse. A blood test showed ‘viral activity’. My GP thought I had glandular fever and advised two weeks bed rest. The first few days I felt fine, but then I started getting new problems. My heart frequently started racing, which was followed by a feeling of exhaustion. My GP thought panic attacks might be causing my very fast heart rate. But it only occurred after exercise or food so that didn’t seem to make sense.
I was referred to the local hospital, but by the day of the appointment my health had deteriorated even more. A blood test gave a normal result which was good, but the doctor found my blood pressure was very low. We had a surprisingly brief chat, then he diagnosed me with M.E. (Chronic Fatigue Syndrome). He commented ‘We could do lots of expensive tests, but there’s no point because it is M.E.’. The treatment he recommended was three more months of bed rest.
I didn’t know what to do then. I didn’t agree with my diagnosis or treatment and had lots of weird symptoms which worried me: my feet often turned purple, I had a constant headache, chest pains, and my heart pounded and raced a lot. Endless other theories were suggested: myocarditis, hepatitis, hypothyroidism, hypochondria, ‘You have a hole in your aura’! I took a list of questions to every hospital appointment, but they never had any answers and just kept prescribing more bed rest.
Months passed with no improvement. I felt dreadful every day, as well as confused and angry. But then I read a newspaper article that changed everything. It was about a girl in America who’d been diagnosed with M.E. but was later found to have Postural Orthostatic Tachycardia Syndrome (POTS), meaning a rapid heart beat while standing. People with POTS also experience fainting, fatigue, nausea, headaches, all the problems I’d been having.
As I had a history of passing out easily POTS was the first explanation I’d heard that made sense and I was immediately convinced I had it. The girl I had read about was being treated with medications to raise her blood pressure and lower her heart rate and seemed to be living normally again. The article said it was important to keep as active as possible and spend time upright, but on the advice of doctors I’d spent nine months lying in bed.
Bed rest has a significant effect on the human body. In one study cardiac output had reduced 23% after 10 days, muscle strength reduced 26% after 5 weeks, and spine bone mass had reduced 3% after 12 weeks. (ref).
POTS can be tested for using a ‘tilt-table’, which monitors a person’s cardiovascular response to moving from a horizontal to vertical position. I went to see my GP, but he’d never heard of POTS or the test. I found there were only three places in the UK at the time that had the equipment for tilt testing, and they all had a long waiting list.
I had to wait eight months for a tilt-table test. The whole time I was determinedly, stubbornly, pig headedly convinced I had POTS. When the day of the test finally arrived it felt like my future and my sanity were on the line, …..but I was right. 🙂
My blood pressure was very low at the start of the test. It dropped sharply as the tilt-table lifted and my heart rate doubled. I felt ill throughout the test. My heart rate reached 130 bpm and I passed out after 12 minutes. It felt like good news on the day because my diagnosis changed to a condition that was treatable instead of one that wasn’t. I thought my life was about to start again. However the test had shown a bad malfunction in my body’s cardiovascular system, which was far from good news.
I had no trust left in the consultant I was under, so my GP referred me to one of the large London hospitals. Unfortunately they knew little about POTS and wouldn’t consider any treatment that raised my blood pressure due to the risk of a stroke. They prescribed Fludrocortisone which I couldn’t tolerate. Then they tried fitting a heart pacemaker in the hope it would reduce my heart rate, but that didn’t help at all. Having wasted 18 months they referred me to Professor Christopher Mathias at the National Hospital for Neurology and Neurosurgery, a specialist in neurovascular medicine.
After numerous tests Professor Mathias prescribed Ephedrine and salt tablets. Ephedrine stimulates the sympathetic nervous system to constrict blood vessels. Together they raised my blood pressure 5-10% with no side effects. He also recommended a progressive exercise programme.
By the time I started Ephedrine I’d been ill for 3 years and lost nearly 3 stone (20 kgs). I was very weak so I started walking every day, a short distance at first. I’d tried exercise programmes previously which had failed, but with higher blood pressure it was working. Over 5 months I built up to walking more than 10 miles a day. The programme ended when I tore a ligament and couldn’t walk 10 yards anymore. That was disappointing, but a combination of Ephedrine and exercise had improved the way my body functioned and I was capable of far more.
Keeping active is vital for managing POTS (ref) (ref). Avoiding alcohol and not smoking also help (particularly cannabis (ref) (ref)). I first remember having POTS symptoms when I was nine. I exercised frequently as I grew up and tolerated the symptoms very well. But two years of the student lifestyle made me gradually worse until I was struggling to function and needed appropriate medical help. Unfortunately instead of exercise and spending time upright, I was prescribed nine months bed rest which made me quickly worse.
It’s easy to get frustrated about the inappropriate advice doctors gave me, but objectively such mistakes weren’t surprising. POTS has been described in medical journals dating back to 1940, but it wasn’t named as a condition until 1993 (ref). I became ill in 1994 when there was very little awareness of the condition and doctors were still experimenting to find effective treatments. There’s still more progress to be made, but diagnosis times have now improved and there are a number of well tested treatment options.
A tilt-test established I had POTS. However what was causing my POTS symptoms was still unknown…
