bombsh3ll

Thanks for the update Jim, it is always interesting to learn about others' experiences & compare notes. Raised ES R & joint pain, especially large joints like shoulders suggests PMR, but that wouldn't explain an abnormal EMG. Do you have follow up? It may be worth discussing an empirical trial of steroid treatment to see if you responded. B xxx

I have tried a brace & it didn't help, but it would not really be expected to with vertical instability only, as the braces provide no upwards traction. I have the option of trying a halo brace, but my surgeon doesn't feel they give the best idea of postsurgical results (although some do). I would do it in a heartbeat too if I could know it would restore normal or reasonable cerebral blood flow & orthostatic ability! It has been 5 years for me now, on the severe end & medication refractory, & I am inclined to agree with the consensus of the leading experts that severe adult onset dysautonomia seldom spontaneously resolves without identification & treatment of an underlying cause (where this is possible). I think if a CSF leak can be definitively ruled out I will pursue this further. It is great to hear others' take on it, as I really value hearing different perspectives & there may be pluses & minuses that I hadn't thought of. B xxx

I am not at the point of making this decision yet, & am not asking for advice but am very interested to hear what other people would choose; A small but rapidly growing cohort of people with severe dysautonomia, most of whom have diagnosed or suspected EDS, have experienced dramatic improvement & in some cases complete cure, by undergoing craniocervical fusion surgery. The skull is lifted up, then fused to the neck in a rigid position, & the neck is also fused. There are variations on this theme but the procedure in my particular case would remove all movement in my neck, permanently. There is no guarantee the operation would help. Obviously this procedure is only performed where craniocervical instability is diagnosed, it is not done in the absence of this. (I have been diagnosed with cranial settling, which is a form of craniocervical instability). The mortality rate from the operation is 0-0.6% per the most recent research. Once I have definitively ruled out a spinal CSF leak as the cause in my case, this surgery is the next (& last as far as I can see for now) option to explore. So.. what would you do - permanently sacrifice ALL movement in your neck to have a chance (but no guarantee it would work) at being able to live again, or stay miserable syncopal & supine & not risk your neck? B xxx

I no longer salt load as such, as I personally found it unpleasant & ineffective plus it suppresses the renin-aldosterone axis long term, however I do use Trioral rehydration sachets which contain a decent amount of salt compared to other hydration drinks and are reasonably priced. One sachet makes up a litre, & it contains balanced electrolytes including potassium. If you don't like the taste it can be flavoured with a bit of fruit juice, diluting juice or other flavouring. I definitely could not stomach salt tablets (& I can usually eat anything as I am on omeprazole), but have no problems drinking Trioral. B xxx

I don't know about your area but certainly in the UK there are lots of mobile hairdressers who cut/style both men and women. They often charge less than a salon too. Just type your area and mobile hairdresser/barber into google & I bet you find loads. Also if you have any local advertising sites (we have Gumtree) where people buy and sell stuff, there are often mobile hairdressers advertising on there. B xxx

I am sure that will be helpful for others to know about. It is really disappointing that somewhere like Stanford would label your daughter's seizures psychogenic. That is only slightly less offensive than "hysteria", & belongs in the same century as offering to perform an exorcism. It sounds like you have come across a useful agent & I hope it continues to do the job. Out of interest though has their cardiologist ever discussed ivabradine, which lowers heart rate with no BP lowering effect at all? B xxx

PS have you ever seen or do you have the opportunity to see an autonomic specialist? It may be worth trying to do that if you can to get their report as a regular neurologist/cardiologist may not have sufficient condition-specific expertise to be able to comment on your long term prognosis. A number of the doctors I had seen that I got reports from could not give a definitive answer as to whether I had any scope for improvement as it was beyond their expertise, & I thought this was going to be a real problem for me. They were all agreeing that I was unable to work right now but only a couple were willing to commit to saying it was unlikely I would ever be able to. B xxx

I know I am in the UK & things are possibly different, but I got approved first time (I expected a fight because that seems to be what most people experience) & I based my case purely on physical symptoms & limitations. Yes, dysautonomia can both mimic anxiety in terms of some physical symptoms like a pounding heart, shaking etc, & be a cause of secondary anxiety due to having a horrible physical illness with no effective treatment, but that doesn't make your main problem anxiety. Did you have a diagnosed anxiety disorder prior to becoming ill at all? I would try and get the focus back on your physical symptoms because that is what will likely justify declaring you permanently disabled, whereas anxiety/depression can improve or go away, & has many proven effective therapies. B xxx

Licorice root can be incredibly helpful, it was life transforming for me until the effects waned after about a year. I did not tolerate fludrocortisone, yet licorice did not give me the unpleasant side effects. During that precious year I was able to make many happy memories with my children, who were very young at the time. I took Swanson's brand. I no longer take it now as it lost effectiveness, & my BP was going too high. As long as you monitor BP & electrolytes, & check with your own doctor that you have no individual contraindications/potential drug interactions, it can be amazing. It is also inexpensive to buy & not as dangerous as some other drugs used to manage POTS/OI. B xxx

I'm convinced I am low flow. Wish I could get my blood volume & Ang II measured! B xxx

I am not familiar with the system in the US, but have just been through the process in the UK. I have heard there are some kind of no-win-no-fee representation deals in the US where someone takes on your case for no cost to you upfront, & that these are quite helpful for people applying for disability. In terms of making your case, I really think you would be best placing more emphasis on physical symptoms rather than "anxiety", as not only are they generally regarded as more serious, if you are looking at a permanent claim it can be hard to make a prediction that an adult of your age will remain too "anxious" to work for the next several decades. It may be that having your main report come from a psychiatrist has skewed their focus towards the mental health side - have you got good documentation from your cardio/neuro? I think the main letter that swung it for me was from one cardiologist who stated something like "all the currently known treatments for POTS are only minimally effective". The assessing occupational health doctor also included in her report all the things I had tried, which showed that I had genuinely tried my best to improve my situation. Here, a lot of it also rests not on your specific diagnosis but how it affects you, so if you faint or have severe presyncope where you lose your vision & hearing, have to lie down etc, you need to get that across. If you can't stand/sit up for very long, they need to know that, & all the things it makes you unable to do. If it takes you over an hour to get washed & dressed because of blacking out, they need to know that, because they won't see it. If you have to physically attend any appointments/assessments, go there in a wheelchair, have someone accompany you, & do not drive yourself there. Ask if you can lie down on the examination couch or put your feet up on a chair whilst you answer the questions, as it helps you think more clearly by getting blood up to your head. It isn't milking it, it's just telling/showing it like it is. Best of luck, B xxx

I guess you have to look at disability benefits in the same way I regard a wheelchair - having it doesn't make you any more disabled than you are anyway, it just makes living with it a bit easier. It would have been much harder if I'd been refused, as living on my husband's wage alone would be tight. I am very thankful to have the financial stress taken care of & not have to go through repeated assessments after a certain time. I am sure I will get to that stage too. Even if I could get stable to just volunteer somewhere in a small capacity would be nice. B xxx

Same here. On the scrap heap at 39! Just have to remain hopeful of more treatments becoming available in time! B xxx

Thanks, here's hoping! B xxx

I haven't had the chemical stress test (& nor would I because I don't feel I could tolerate it), but I had a horrid reaction to IV iodine contrast CT dye the other week. I would describe it as haemodynamic rather than allergic as I had no rash, itching or wheezing etc, but it made me very flush & HR went nuts, similar to what you describe. I managed to get some IV fluid afterwards (wasn't going to let that cannula go to waste!) which helped things settle down a lot. Is there some way you could get a bag or two of saline to help you out if you are still feeling unwell? I hope things improve soon & that some useful data was gained from your test. B xxx

I received the final decision on my disability pension today - I have been awarded the upper tier of NHS medical retirement benefits, the category for people not expected to be able to return to the workforce in any capacity. Still shaky after reading the report, my heart was pounding when I opened the envelope! Whilst this is the best possible outcome & means a little extra financial security for my family, I am also sad seeing the extent of my limitations & poor quality of life set out in black & white - it makes Dickens look like Disney! I know I need to focus on the relief of this decision, I was dreading a rejection, having to appeal etc. I have already thanked God for the outcome, which is the one I had been praying for. I may also have the option of taking a lump sum, which would help me fund craniocervical fusion surgery if I decide to go ahead with it. It feels strange & ironic to have to fight so hard for years to convince people that you are ill, then be shaken up at reading back just how ill you are. Has anyone else had mixed feelings after getting their disability/pension award? B xxx

I find it hard to see how B12 deficiency would cause (or its treatment would improve) a structural problem like spinal stenosis. Both have been postulated to cause/worsen dysautonomia symptoms so are definitely worth excluding if relevant. B xxx

I use a wheelchair too, & wish I had bought one a lot sooner so I could have gone out more with my family rather than missing out. I try to walk a little when I can, & walk pushing my chair. It may look strange but I don't care. Sometimes people will joke that I'm missing a passenger, & I explain that I have a condition that means I can't stand for very long without fainting. I don't mind that at all as long as they aren't rude. Some things to consider when choosing a wheelchair are - how portable is it, can it fold easily & be transported in a car boot? Can you self propel or have someone to push you, or do you need an electric powered one? What about head support/leg elevation/tilt function? Tyres can be solid or air-filled - the latter is more comfortable but can get a puncture/deflate. I hired one first from a wheelchair hire company before getting my own - this is a good option if you want to try out a particular type before you buy it, or if you just need one for one-off occasions such as a special trip. Often rental places will sell second hand models as well which may be better than a private sale as you know it has been maintained & can go back if there's a problem. B xxx

Thanks for sharing, that is really interesting! Although only 12 people were studied & no control group was used, it does support a volume enhancing mechanism which is what most of us with OI could use. It never worked for me but I continue to keep my bed on a small slant (approx 10 degrees) now (previously is was raised to about 30 degrees). B xxx

Does anyone know of any published scientific research supporting that? I would be really interested if there is, as these are both measures I have tried. Dr Levine's study makes references to these practices in the discussion section, stating something to the effect that whilst the patients in the study were encouraged to do these things, so possibly this could have helped instead/as well as the exercise, these measures do not seem to prove effective in clinical practice. Not to say nobody benefits or should stop doing these if they find it helpful, just that it doesn't appear to have an evidence base that I am aware of. B xxx

I haven't tried it but would be interested to hear your experiences if you do. Will you be able to rent one to try or do you have to buy it? B xxx

I didn't like anything about clonidine although on paper it sounded great & I was excited to try it, but it made me even more syncopal unfortunately. This is one of the papers that directed me towards clonidine https://www.ncbi.nlm.nih.gov/pubmed/6295714 I have no doubt it can be greatly beneficial in some, but I think for me (being mineralocorticoid deficient) I am highly dependent upon raised sympathetic tone, as uncomfortable as it is, to maintain my BP. Also my resting HR can be on the low side, this may not be the case for you. What I would say is start with a small dose - whatever you think a tablet can be cut into, do half of that. You can always take more, but you can't go back and take less. Also don't take your first dose if you are alone, or if you plan to drive/work/look after children/go somewhere for the next 12 hours, until you know how it affects you. B xxx

I agree with your doctor's thinking, I believe that is the case with me as well. I just didn't tolerate the florinef but in theory it makes a lot of sense. B xxx

Whilst helpful for picking apart what haemodynamic abnormalities a patient may have & potentially guiding treatment, as well as sometimes for disability purposes, a TTT can sometimes be unhelpful & frustrating if it presents values/patterns within the normal range on that particular day. Tilt-reproducable POTS can vary depending on a lot of things such as hydration, time of day, menstrual cycle, medication etc. So a person can have POTS & not show up on the TTT, alternatively they can have orthostatic intolerance with no specific HR/BP deviations from the norm but nevertheless be profoundly unwell with severe symptoms, sometimes more so than someone with a higher HR increase, as what matters is blood flow to the brain as opposed to a HR number. Unless cardiac output is measured/estimated during the test, a critically low cardiac output, if compensated for with intense sympathetic activity creating a normal (or even increased) BP, will be missed. Equally unless transcranial doppler or other method of assessing cerebral blood flow is employed, cerebral hypoperfusion is also invisible unless the person loses consciousness. There is far more to orthostatic cerebral hypoperfusion & circulatory failure than superficial measurement of HR and BP, to which many providers often limit testing. Another potential scenario depending on where you are tested, is the person reading the data has not heard of POTS and sees sinus rhythm with no drop in BP and declares the test "normal" despite marked tachycardia being present, because they only know to look for postural hypotension or arrhythmias. This happened to me on my first TTT (with a HR in the 130s and BP 170/100). As with any medical test is really important to ask for a copy of your actual data than just the conclusion of the person interpreting it. B xxx

Licorice root worked amazingly for a year then the positive effects wore off & it left me with very high blood pressure. Its action is similar to that of florinef but without some of the side effects. Unfortunately I have not yet found anything else that helps me to stand, although I too take a small dose of diazepam now and again when the chest pain & non-psychogenic sense of anxiety/doom is really bad, & that really helps those symptoms. As far as I am concerned, taking a small dose of a benzodiazepine for a medical condition as opposed to recreational use, and sticking to your prescribed amount with no dose escalation carries no more risk of getting hooked than an asthmatic using their inhaler when they need to. Starting low with the verapamil if your BP is low at times is sensible, as its primary function is as an antihypertensive. Good luck, I hope you find the new med helpful. B xxx