bombsh3ll

The top dysautonomia specialist in the UK to my knowledge is Dr Matthias in London. I haven't seen him personally but I may do, if craniocervical instability/settling is excluded for me/ I need more supporting info from an autonomic specialist in order to release my pension / cannot get to see Dr Grubb (US, would be my number 1 choice if money no object and physically able to travel). Unfortunately he only practices privately. 

I have also seen Prof Newton in Newcastle where I went to get diagnosed, and UCLH in London where I went for further autonomic tests. Neither were able to offer me any treatment but to be fair I had already tried everything except methylphenidate and IV saline myself (I worked as a GP). 

I hope you manage to find somebody willing to try to help. Sometimes, a doctor who doesn't know much about the condition but cares & is willing to learn and try different things with you is worth far more than someone supposedly specializing in dysautonomia who isn't willing to listen or think outside the box if the first one or two meds don't help. 

B xxx

2 hours ago, Shepard1 said:

It would be safe to say that a review by a vascular surgeon or a Doppler assessment to obtain your ankle brachial index (ABI) which will identify if you have any arterial component to your neuropathy.  

That would be a sensible precaution but depends a lot on other factors, for example if you are female under 30, non smoker and no family history of early cardiovascular disease, you are unlikely to have arteriopathy. 

If you are male, diabetic, overweight and over say 40 years old, smoke etc it becomes much more of a concern. 

Were you given any specific recommendations regarding compression at the time of dysautonomia diagnosis? They can be expensive to buy so if you can get a prescription it helps. 

I wear class 2 activa knee high stockings underneath, and class 2 waist high sigvaris compression tights over the top. The former help the latter slide on easily and provide a bit extra compression. 

I can understand however that if you live in a warm climate this may not be comfortable or appropriate. 

I personally do not seem to gain much benefit from them, perhaps slightly and they are comfortable so I continue to wear them, however I do not have any swelling or discoloration suggesting pooling, whereas if you do, you may have more to gain. 

B xxx

14 minutes ago, RecipeForDisaster said:

I usually wake up blazing hot, nauseous, and tachycardic at about 2-3am. I despise it.... it makes it so hard to fall back asleep.

Was that happening before you raised your bed? Also how high is your bed raised?

It could be that sleeping significantly head up is causing cerebral hypoperfusion, leading to those symptoms as compensation (like during the day). 

My bed used to be up higher than it is now, but I lowered it as a) it made no difference (although I was on licorice root then) b) it was uncomfortable c) I like there being at least a few hours in the day when my brain is adequately perfused and d) I was concerned that if I fainted whilst asleep I would have no way of falling flat to restore perfusion. 

B xxx

3 minutes ago, Pistol said:

You mentioned that you found a doctor willing to prescribe the infusions. Is it possible to get them initially via peripheral stick ( which is safer ) and see how you respond before having surgery for a permanent - or semi-permanent access? 

Yes that is what I am doing initially, when my supplies arrive I will be doing a bag weekly over 4 hours as a trial via peripheral IV. The drawbacks with that are the expense in having home nurses come to my house (£100 a time) and also that my veins are very poor due to hypovolaemia. 

Also the cardiologist retires in July so if I wanted him to refer me for some form of central access I would need to do it before then as it is very difficult to find specialists here willing to support fluids. It would then be easier to get the treatment and access maintained in the future if it was already established. 

I will have to see how it goes with peripheral access. The Ruzieh study in 2017 had most patients on peripheral access only and found most were able to wean from fluids after a few months. That would be my ideal - I do not retain fluid because I don't produce renin or aldosterone, hence I can't stand or do any upright exercise, however the things that stimulate renin and aldosterone are upright posture and exercise, so if I were able to break out of that cycle, I may not need it long term, or alternatively it could be something I reserve for special occasions. 

I am very concerned about the clot risk (more than the infection risk as this is somewhat modifiable by how the device is cared for), in fact in some respects I am thinking how on earth could I face the risks of a central line when I am too afraid to go back on combined hormonal birth control, which I have also considered in the hope of a volume boost. 

I don't think I am massively at risk of a clot any more than anyone else, it is just that having daily symptoms of hypovolaemic shock with the chest pain, tachycardia and presyncope/syncope it would be difficult for me to recognize if I did have a life threatening clot as these are all potentially presenting symptoms of one, so I would either be at A&E all the time, or not go there the one time it was a clot rather than just the usual.

Was this something you wrestled with and how do you deal with it? I read one story of a patient whose usual POTS symptoms of chest pain, tachy, fainting etc were just a bit worse one night and her husband made her go to hospital when she really didn't think it was worth it as they couldn't do anything for her, but she went that time and turned out to have multiple PEs. She did not know if they had been there all along (she had only been unwell 6 months) and it was actually that misdiagnosed as POTS, or if the immobility caused by the POTS had led to them. I don't know if the POTS went away after they were treated, but she had to stop her combined birth control straight away. 

B xxx

21 hours ago, Kerri said:

Have you learned anything since your original post? I do hope you’re feeling well. I have a 10mm pineal cyst, as of 2017, which was originally discovered in 2015. I too have POTS and would be curious of any connection to Dysautonomia. 

Me too! I had not been made aware of it by the doctors ordering my scans, but applied to obtain my results to send for a neurosurgical opinion on possible cranial settling. I discovered I have a 1cm pineal cyst which was first seen April 2017 and unchanged on my scan March 2019.  

It is reported to be benign in appearance and may be an incidental finding but I do wonder. Also have splitting headaches but only when upright. 

I am going to discuss my recent scan result with the neurologist next week.

B xxx

I think the fears about benzodiazepines are greatly exaggerated and sadly prevent a large number of people from using a helpful medication, due to abuse of this class of drug by a minority. 

I take a low dose of diazepam since developing POTS, have never needed to increase my dose nor become addicted. 

It is certainly less of a risk than other drugs prescribed for dysautonomia and/or mental health.

If your doctor is happy with it, I wouldn't worry. It is when people start escalating their doses or taking it recreationally (often alongside other drugs) that harm is done.

B xxx

Hi Machair,

Generally the advice is to have your whole bed on a tilt ie with bricks or books or something under the top feet, rather than just raising your head on extra pillows.

I have personally not found it helpful but still have mine on a slight incline. 

B xxx

43 minutes ago, RecipeForDisaster said:

PICCS can last but not usually as long. They can migrate, get pulled out, clot off..

I've heard enough bad stuff about PICCs that I've already ruled that out. For me if I have the chance to get a port and can reliably source the needles, plus access it myself, I would go for that. I think the Groshong if not, because I like the smooth ending and valve that reduces risk of air entry & clots. From what I have read, a pump is required to infuse via Groshong because of the valve, but the person I know in the UK just hangs her bag on a coat hanger over a door and lets it run by gravity. 

Basically if you have something like this put in privately in the UK, you are left on your own to manage it or have extortionate home nursing costs.

B xxx

17 minutes ago, RecipeForDisaster said:

I suppose I'm not strictly resting for 5 minutes because I pump with the other hand a minute before deflating. The actual deflation doesn't require anything on my cuff, it's just a loosened screw and I let it deflate at the same rate, I don't speed it up or slow it down. So, I don't move at all while I'm taking the reading, just listening. I'm guessing and - hoping - that my BP isn't artificially high from this activity, when I am getting readings I need the low 70s!

I think if you have your routine down & know what is normal for you, you would be able to detect any deviations from your normal, which is what matters. 

For me, when I am near passing out, I wouldn't have the ability to even try with a manual sphig.

B xxx

On 5/7/2019 at 5:18 AM, Derek1987 said:

What was the diagnosis that gave you the win?

I am in the UK and still fighting for release of my occupational pension, but I think having EDS diagnosed helped me get government disability. 

You have mentioned your father having similar problems, & EDS is hereditary. Any current or previous flexibility, skin, joint, digestive or surgical problems?

If you think that could be you, look up the Beighton score & maybe see a geneticist. 

B xxx

16 hours ago, Trying said:

She is not lighthead, dizzy, or faint when upright

I would celebrate that rather than focusing on the numbers. 

IMO the BP readings are not consistent with OH, they go up initially then come back down to near baseline, but there is narrowing of the pulse pressure to 16 after 8 mins which can reflect low stroke volume, and the HR rise of >30 in under 10 min is consistent with POTS. 

It may be that the medications she is on are improving things from what they would be unmedicated.

Where did she go for the blood volume test?

Has her specialist diagnosed a type of dysautonomia?

B xxx

16 hours ago, RecipeForDisaster said:

Yes, this is true. Patients with ports can hook themselves up when the Huber needle is left in. It can stay in for a week before it needs to be changed. Otherwise, if no needle is in, a nurse needs to come and access the patient.

 

Ports need to be flushed monthly if not in use, and other types of access like Groshongs, PICCs (probably the most common, hangs out of the arm), and Hickmans need to be flushed daily. You can swim with a port if not accessed but not with the others. But, you can connect your own infusion with everything but a port.

That's really helpful info thanks! I am not sure yet what my choices would be. I would like the option that has the best safety profile and longest lifespan. I think I would choose a port if that is available to me including being able to obtain the necessary supplies for it. I do not know how I would manage to wash with a line hanging out of my chest or arm, as I can only have baths (although that might change with hydration!). Also it would be so easy for it to get caught on clothing or turning over in bed and pulled out. 

Do you know if everyone with some or all types of central access device has to take an anticoagulant? I was a GP and never dealt with any in primary care. In the hospital everyone was injected with heparin daily anyway.

If I decided to go ahead with central access, I would have a thrombophilia screen first to make sure I had no underlying clotting disorder, however 5 years immobile with POTS, on combined birth control until recently, I probably do not. 

B xxx

But if you are pumping/deflating with the other hand instead of sitting at rest, surely the concentration & hand activity would interfere with your reading?

I don't think I'd be able to take mine that way, but I guess if you are consistent in the way you are taking it, you would still notice a deviation from your norm.

B xxx

Having worked as a GP I would strongly advise against the wrist BP monitors, as they are notoriously inaccurate.

It is also not possible to take one's own BP with a traditional sphygmomanometer and stethoscope, and even those when used in clinical practice are to a degree subject to the user's skill (and hearing)!

I would recommend an Omron automated arm cuff BP monitor. Make sure it is correctly placed (the cuff shows you which part lies over the brachial artery), and that you are using the correct size cuff for your arm. 

A skinny adult sometimes requires a small or paediatric cuff, a heavy adult will need a bariatric cuff. Most people can get away with medium. 

B xxx

I can understand reluctance to see a mental health professional when most of us have at some point had to deal with clinicians who believed our dysautonomia symptoms were pyschosomatic, however a psych consult can be extremely validating and helpful. 

Not only are they extremely good at recognizing when patients have a physical issue that is not consistent with any known psychological diagnosis, (for example when symptoms are orthostatic in nature & resolved by volume expansion), & providing useful supporting documentation to validate your illness, they also have a range of medications in their prescribing arsenal such as benzodiazepines, stimulants, sleep meds, atypical antidepressants etc that can be helpful in dysautonomia but that other specialists are unfamiliar with or not comfortable prescribing. They are also often more aware of side effects that can be caused by drugs prescribed by cardiologists or neurologists eg beta blockers, as these are also frequently used to manage anxiety disorders. 

Anxiety and depression are common in all chronic illnesses, & I think it is a shame that teams caring for patients with cancer or MS or HIV etc automatically have psychological services attached, whereas people living with dysautonomia which can be much more limiting & destructive to quality of life are usually only advised to seek psychological help as a fob-off by dismissive medical doctors.

B xxx

Hi Megan,

I get similar night time episodes, minus the digestive symptoms. 

Couple of thoughts - sudden diarrhoea could either be due to the elusive carcinoid you've been scanned for, or alternatively mast cell related.

The other possibility that comes to mind is if either of those remedies you mention have magnesium? I take that deliberately to help me go.

B xxx

19 hours ago, KiminOrlando said:

Hey, @Derek1987. We have the same job! Tedious, isn't it? And I have yet to get paid. Where is HR? I would like to complain.

Me too, wish the pay was better & we ever got a holiday from it!

I was a GP when I became ill. I continued working for a while, 2 days a week, my job was mostly sitting down anyway, I had a chair that tilted back and a crate under my desk to put my feet on, but I reached the point it was no longer safe or sustainable and had to give up working. 

I was never into the academic side of medicine, I much preferred the front line, but if I became able to work again, I would go into research in the field of autonomic disorders. 

Not everyone has to give up work completely though, many people do stabilize on medication, I am just difficult because I produce no aldosterone and do not tolerate the only synthetic replacement.

Even if you have to retrain in another occupation, if you can sit upright consistently without syncope or disabling symptoms, there are still a lot of rewarding paths out there. Flexible hours, scope to work from home are things to look for.

I hope you manage to find something that fits. 

B xxx

1 hour ago, Pistol said:

Hi @bombsh3ll - it is per 20 Fl Oz, or 591 ml. 

That's not as bad, but definitely doesn't measure up to ORS solution.

B xxx

13 hours ago, JimL said:

Went on the GI docs portal today and it said I have Chronic atrophic gastritis without bleeding. That doesn't sound good. 

Hi, I would be inclined to suspect one main unifying underlying pathology behind all of these things, all occurring in a short space of time. Possibly autoimmune.

Hope you manage to get some answers. I am very interested to hear how you get on with Dr Levine. 

B xxx

24 minutes ago, Pistol said:

In the US a port has to be accessed by an RN since it is imperative to maintain completely sterile procedures during access

That's confusing, most of the people I have come across with ports are in the US who talk about running their fluids themselves - maybe they just leave the needle in between nurse visits?

I am not sure what type of central access I would be offered or have a choice between. I would need to make sure the supplies for it were accessible, and I think the special Huber needles for a port might be hard to get. 

There is one patient in the UK I know of with a Groschong central line which hangs out of her chest. She and her family manage it themselves. I think I would worry about that getting caught and pulled though, and being more susceptible to infection. 

I have had two prior central lines in hospital in the past, both hung out from my neck which wasn't ideal, but I expect if it was intended for longer term a better position would be chosen. 

B xxx

After failing at pretty much every oral medication, I have been prescribed a trial of weekly IV saline by my private cardiologist😀. Unfortunately, although the saline itself is not too expensive, the home nursing agency that will come and administer it to me is cost-prohibitive if I were to do it regularly (I live in the UK). 

What is even better is that he has offered to refer me for a port or other similar long term IV access if it is helpful, so that I would be able to administer it myself. (I am however understandably worried about the risks this entails).

Unfortunately though I do not have much time as he retires in July, so I need to go through the process of trial and error with the fluids in a short timescale before deciding whether to take up the offer of central access. 

I would really like to hear people's experiences with regular IV fluids, what type of access you have, any problems with it, when, how much and how often you get fluids, how fast you run them and what type of fluid?

Initially I will be doing 1 litre of normal saline over 4 hours, once weekly, but I know some people get daily, some get less often, some feel better run fast, others slow, so would like to hear what is working for you!

B xxx

25 minutes ago, Pistol said:

Gatorade contains Sodium 270 mg, Potassium 75 mg, Sugars 12 gm. 

Is that per litre? Glad I asked, I will stick with my trioral!

B xxx

Hi Lavender90,

I have hyper-pots type symptoms and BP that goes up on standing but normal neurotransmitters on the tilt. 

Mestinon didn't help my orthostatic intolerance & lightheadedness either in terms of symptoms (to be honest I didn't really monitor HR/BP on it but I am more concerned with how I feel than numbers as long as nothing is dangerously off).

It did significantly help with my sluggish bowel motility which is lifelong not changed by POTS, however I have other laxatives for that so didn't continue with it. 

I'm sorry it didn't help for you, but at least you have tried it and can cross it off. That is better than not trying something and potentially missing out on a good treatment. 

B xxx

28 minutes ago, Lily said:

Gatorade does not have a lot of electrolytes in it.  You might try adding salts (regular and low-sodium together).

Thanks, I had assumed it was an electrolyte type drink from it being recommended so often in POTS/hypovolaemia, but if that's not the case & it is just another brand of sugary drink I don't think I'll bother with the high shipping costs!

B xxx

4 hours ago, JimL said:

Have you ever been tested for sleep apnea?

No, I can see that being a logical step but I have no breathing symptoms and I don't really fit the clinical picture or the demographic. It doesn't always necessarily wake me from sleep either, I can wake spontaneously as I'm a light sleeper, feel OK for a couple of seconds then feel my heart start pounding & it kicks off. 

Also I don't think I could ever sleep with a CPAP machine!

B xxx