bombsh3ll

it is really hard but I would limit the time and energy spent on/with people in your life who don't at least try to understand. 

I find directing people to online resources explaining the condition helpful. It is easier than trying to explain it myself and can seem more credible to people if on a medical/scientific website. Whether they bother to read it is a good indicator of their attitude and if they genuinely care for you. 

I have always been close to my dad, and he provides a lot of day to day practical help in my life. He can however be very insensitive, making comments like "why don't you go and have a game of badminton", "what about trying a hypnotist" & "you seem to just sit around a lot" and repeatedly asking my to explain my condition. Just when I think he has got it, he'll do something like shout at me for knocking his feet whilst desperately trying to pull out a kitchen stool to sit on to avoid a collapse. 

I think I probably should be more patient with my dad though, as I know I can be short tempered when I can barely function and he is preoccupied with very trivial concerns over his own health like a one day belly ache or his dentures not fitting properly. He is 77 and walks several miles a day, never having known barely a day's real illness in his life. He has bad varicose veins and a small asymptomatic aneurism, and I can see that I get EDS from his side, but it has never caused him any limitations. If I am honest it is probably partly because I am jealous.  If he makes an inappropriate or hurtful remark now, I try to ignore it rather than take the bait. 

I have never been that close to my mum, but she has dealt with my illness a lot better. She has never made an issue about me needing to use a wheelchair. 

Having a chronic illness has helped me learn what and who really matter in life, and I have become a lot better at asserting what I need and what I can and cannot do. 

I have wished many times that there was some way I could have people sample POTS for an hour or so, like a mega dose of furosemide or temporary bilateral carotid artery clamp 

Thank you so much for replying with so much detail. I am sorry your daughter is still struggling. I appreciate your honesty regarding the POTS - it is my only reason for considering this surgery, IF the neurosurgeon feels I have something anatomically correctable by surgery. Other than permanent lightheadedness, presyncope and inability to be upright, I have no neurological symptoms and believe my head and neck pain to be related to hypoperfusion. 

Hearing this, I think I would want to try a halo vest brace first if possible, to see if my condition improved with the pressure off the brainstem, and only go for the surgery if it helped. 

I ordered some of this a while back, sadly it had no positive effect for me (although nor does midodrine - I am already intensely vasoconstricted) and I also then discovered it has diuretic effects so stopped taking it. 

On 7/19/2017 at 8:00 PM, DizzyGirls said:

She will have C3-T3 fused with the possibility of O-C1 and C2 also if they don't get the stability that they need.  It's a two neurosurgeon deal and roughly 8 hours long!  Going to be a grueling day and following days for her.

Hi do you mind me asking how this procedure went and what improvements your daughter has gained from it? I was recently diagnosed with hypermobile EDS and paid for upright scans (I am in the UK). They were reported as normal but apparently craniocervical instability can be missed unless a neurosurgeon trained in this field looks at the films. I am currently in the process of getting an opinion from Dr Gilete in Barcelona. I would love to be able to find a correctable cause of my severe POTS & don't believe it is down to lax veins as my onset was very sudden after a forceful valsalva, whereas veins would surely tend to stretch out over time. I am sure something shifted or became squeezed in my brain. I get a lot of headaches and never had any prior to POTS onset. I hope your daughter is doing better after her surgery, it must be especially difficult dealing with these problems at such a young age.

Beta blockers reduce cardiac output and cerebral blood flow. They may help some symptoms in some people, but in the main POTS involves a reduced stroke volume and cerebral blood flow so it makes no sense to compound that. They are popular simply because they make the numbers look better, but the fast heart rate in POTS isn't the cause of the problem, it is the body's attempt to compensate for whatever your underlying problem is eg hypovolaemia, pooling, impaired venous constriction etc. If we felt well we would neither know nor care what our heart rate was.

That looks like a catheter stand! I think it is easier for me to just pull the tights on rather than faff around with glue, but it is great to hear about other options. I also like to idea of some abdominal compression, it makes me feel like I am doing something even though there is no noticeable benefit. I tried an abdominal binder once but that did make me feel as if I was trapping blood in my lower body and preventing it getting back up to the heart. 

17 minutes ago, p8d said:

I wear thigh highs because I can’t tolerate the waist-high ones.

How do people get thigh highs to stay up? I tried a pair once and they kept rolling down. I'm not very mobile and was only sitting too, so I don't know how these are meant to stay up!

You are right about getting them on though - my heart rate rockets whilst I am yanking mine up!

I love the Juzo designs but they are very pricey.  I wear cheap patterned leggings over the top of my tights.

On 2/2/2018 at 8:41 PM, WinterSown said:

Three years ago I weighed 60 more pounds than I do now. Losing my appetite is part of my symptoms. I was greatly out of shape. Are you working with a PTD? If you are very complicated, and who of us is not, you might consider to begin a reconditioning program with a physical therapist, your doctors can give you a script.

I'm in the UK so no medical help unfortunately. I read online about exercise being beneficial, and bought a recumbent bike. During several months of licorice root being effective I optimistically bought a treadmill too. I also lift arm weights whilst watching tv, plus my arms get a workout from using my self propelled wheelchair.

I'm sorry you've struggled with appetite loss, that is an issue for me too. When I first became unwell and started reading about POTS, I convinced myself I had gastroparesis, but I now realize the nausea is just a symptom of cerebral hypoperfusion for me (plus the volume of salt and water I chug), and that my motility is in fact OK. I don't doubt that I've lost lower body muscle mass too though from sitting all the time. 

It is a real inspiration for me to read about other people exercising with this., keep it up!!!

I wear sigvaris waist high class 2, with a pair of activa knee high class 2 underneath. This gives a little extra compression and also helps the tights to slide on. 

I never thought I had the discolouration issue myself until I recently noticed it after a bath. I usually put my compression stockings and tights on first thing though and have probably just never noticed it before as I never really see my feet or lower legs unless in bed. 

I don't actually think compression helps me much though and think I am hypovolaemic rather than pooling blood, and from what I've red the acrocyanosis seems to be related to poor blood flow to the skin rather than pooling.

On 2/1/2018 at 9:06 AM, WinterSown said:

get on the bike and ride for the joy of it, stop before it's not fun anymore.

If I only exercised when it was fun, that would be never  I exercised out of vanity to look good when I was younger, so I was not unfit, but I never enjoyed it. I wish I had treasured the ability to walk more when I had it though. I dread exercise now having to do it whilst so presyncopal & really have to force myself.

I listen to music and/or read whilst I ride my recumbent bike. I can only manage a few minutes on the treadmill, and I do wear a polar heart rate tracker to make sure my heart rate doesn't go beyond 85% of my maximum HR for age. You can easily look up the tables for this. Everyone is different but for me, I know I am very deconditioned as a result of POTS (NOT the other way round) and like the peace of mind of knowing I am not working my heart dangerously hard for someone so out of shape. 

I also find it best to exercise in the evening when my blood volume is highest after fluid & salt loading all day.

Good luck!

If you have no access to medication currently, something I found tremendously helpful for a while was licorice root capsules by swanson. They are a very cheap herbal supplement that works similarly to florinef which you can buy from amazon or health food stores. 

Sorry you're dealing with this at such a young age.

B x

Hi DonSimon,

I went to A&E straight away when I became unwell, and many times after that. You mention a GP appointment, are you in the UK?

Unfortunately here there is little to no NHS help for people with POTS or dysautonomia, you are lucky if you even get a diagnosis, but at least the early visits to hospital helped rule out life threatening and/or treatable causes like Addison's disease or a structural heart problem for example. 

You might also get IV fluids in the hospital which can tremendously improve your quality of life for a few hours. 

Best wishes,

B x

I really hope it helps you, do let us know! How much are you getting and over how many hours?

I go for a blood volume test on Monday. I have fluids and supplies in my house and am really hoping I can get a bag or two in soon after coming home!

I wish I had your husband and your doctor!

I am a GP myself & have occasionally been able to get a friend to give me fluids at home but it is rare & for logistical reasons has to be run quicker than I'd like. 

I would really love a port but in the UK this is really unlikely to happen. 

The only tips I have are trying to make sure you are as warm and hydrated as poss before starting (neither is easy), warming the veins with a hot water bottle or something, and if it is just plain saline in the bag with no additives, a litre bag can be microwaved for 2 mins to take the chill off. 

My veins are only really visible at all in the evening after I have been chugging salt & water all day then ridden on my recumbent bike - if you are like me then having the cannula put in and secured at night, even if you don't plan to run the fluids until morning when you will likely get most benefit from them, might be best rather than your husband stabbing around in the morning when you have no veins. 

Best wishes I hope it brings you some relief. 

I am ashamed to say this now as a doctor myself, but I probably wouldn't have (initially) believed someone either who presented with my symptoms, all normal tests etc had it not happened to me. I would like to think I would have been open minded enough to listen and look into it rather than dismissing them though. There are still a lot of hypochondriacs, chancers and malingerers in the world but a big red flag is when a previously completely fit and healthy person who never visited doctors suddenly becomes incapacitated. 

Being in London is a great start! I would ask for a referral to the automic unit at UCLH, or alternatively Dr Nick Gall, a cardiologist at King's College Hospital. Both of these are NHS. Keep pushing and see a different GP if you are getting nowhere. 

On 12/28/2013 at 2:31 AM, ramakentesh said:

Its good stuff. But tolerance becomes an issue after about two months. Im no longer on it but its worth a shot for anyone struggling (I mean more than usual) Tlak to a doc and all that.

Bumping old thread here, I recently posted about having developed tolerance to licorice root caps myself (after about a year) and potentially changing to Florinef. Is the effect of this similarly short lived, or is tolerance less of an issue given you are replacing a hormone (which in my case is almost completely absent) rather than blocking an enzyme which presumably upregulates? 

9 minutes ago, vxmike said:

If my blood is in my legs and yours doesn't exist it might as well be the same when we're standing!

I really like that way of putting it!

Do you find the midodrine helpful at all or would it be worth considering stopping it? - you mention that alone it didn't help.

I tried midodrine briefly - my BP went high, pulse went low and I was still presyncopal & couldn't stand  I don't feel it is for me as I am already very vasoconstricted as a natural response to hypovolaemia. 

Alternatively you could maybe ask Dr Grubb about a short acting antihypertensive for overnight. 

Where do you live? If you are in the UK, anywhere in England there is an NHS specialist autonomic centre in London that your GP can refer you to. It should be possible to get a TTT done at your local cardiology dept though. Perhaps take your GP a set of home readings lying to standing, along with the info on the POTS UK website - there is a section called POTS for medics. Unfortunately dysautonomia is not well known and you have to be very assertive and proactive. If you can afford, to speed things up you could pay to see a private cardiologist. I recently did, as I was diagnosed with POTS on the NHS but still left treating myself with salt and licorice root, and he is going to work with me trying medications after I complete blood volume and diabetes insipidus tests. 

That's really interesting Ann, I'm glad you've found your cause.  My POTS began acutely after a very forceful valsalva - trying to blow up a waterbomb which I thought was a balloon - so i have often wondered if the raised ICP from this caused some kind of cerebral vascular event. I have had 2 normal brain MRIs though - but yours didn't show up with this? 

I have thought of getting a cerebral MR angiogram and venogram - I am in the UK though and have to pay privately for such tests. 

What is the treatment now you know what you have?

B x 

Thanks vxmike that is really encouraging that you've had a good response, and also good to know Dr Grubb's opinion on it. I haven't ruled it out entirely but do want to address volume issues first - I have a blood volume test scheduled in 2 weeks. I also do not feel I have pooling as such, just general hypovolaemia secondary to not producing renin or aldosterone. My resting HR is also not high which is a concern for me re this drug - I found some studies in which a resting HR of below 60 or even 70 precluded use of the drug (granted these were for other conditions not POTS) but I would be a bit worried about my resting HR dropping too low, particularly whilst asleep.

Has this been an issue for anyone or has your doctor said anything about this?

Did you stop your beta blocker in preparation for the test? If not this could have skewed the results and masked the POTS response if you do have it.

I have POTS with severe orthostatic intolerance and presyncope/lightheadedness all the time. I can only stand for a couple of minutes, this improves in the late evening after I have been drinking water and salt all day. I can also be sitting with perfectly normal BP and pulse but feel awful. 

The amount of urine I pass in 24 hours, and the fact that licorice root helped me so much initially, plus I feel best at the end of the day when my blood volume must be highest, all point me towards thinking my problem is chronic hypovolaemia. My symptoms are basically those of stage 1 or 2 hypovolaemic shock. I also lost 6kg in the first few weeks I became ill, which had to be fluid weight. 

I don't have any other autonomic symptoms other than those causing poor cerebral perfusion. I've always taken medication for acid reflux and constipation all my adult life, which hasn't changed. 

It may in theory be possible to take both fludrocortisone and desmopressin with careful monitoring. Do you have regular blood tests for electrolytes?

Did you increase gradually up to you current dose or just start it all at once? It may be that your best dose is a bit lower. Where is the weight gain, is it around your ankles or all over? Do you think it is fluid retention or has your appetite increased? If I gain weight when I restart it I will take that as a good sign that I am retaining fluids rather than just getting the unwanted increased adrenaline & vasoconstrictive effects that I previously experienced at low doses without any sign it had helped me retain any fluid.  Has fludrocortisone helped you be more upright/functionall overall?

Hi I have also tried desmopressin (alongside the licorice), which stopped me having to pee during the night but sadly didn't help me stand any. When I started the licorice root, the effects were quick and dramatic, but waned over time. 

I definitely believe reduced blood volume and inability to hold fluids is my issue. I did a urine collection to exclude a pheochromocytoma, and one 24 hour container which holds a max of 5 litres wasn't enough. This led to the discovery that I pee around 6 litres per day - upper limit of normal being 3 litres. 

I had a hypertonic saline test for diabetes insipidus which I have to repeat as it was inconclusive - not fun! I actually hope I am not diagnosed with DI because I already know the treatment for that which is desmopressin doesn't help my POTS, and want to pursue the fludrocortisone avenue as my renin and aldosterone are barely detectable.

I don't really get muscle weakness (other than being generally deconditioned secondary to POTS) or cramping so can't answer that. Are you diagnosed with DI or did you just try the desmopressin to see if it helped - and does it help OI for you?