RecipeForDisaster

I love these ideas. I’m going to try to meet each of my parents for a short walk as they are within a short drive. Then I plan to hold a Zoom meeting with my sister's family and hopefully my parents, during dinner. I have a fancy dinner planned just for the two of us, but I’d love to swap food.

Oh, and this is greatly improved with IV fluids...

I find that low pulse pressure correlates with how badly I’m doing, but I don’t see a lot of people interested in it.

All I can say is I feel your pain.... haven’t found anything much on testing but I’m on lots of meds anyway. Genetic testing does show a novel abnorality. I’m doing autonomic tests again tomorrow. Hopefully the midodrine will help - I find it does to some extent, I just can’t handle a big dose.

I use 2L, usually LR or 1L of NS and one of LR, at about 125mL per hour. I run them as slowly as possible to finish the infusion while I’m up, usually 5am-9pm, dragging a pole the whole time.

I had several tests done... one for Familial Dysautonomia is 

ELP1/IKBKAP

This is one of the things that is the most bothersome to me - I sleep so lightly, it takes nothing to wake me again and again! It could be a little sound, an itch, temperature not perfect... it’s awful. I sleep "well" as in I can fall asleep and have good hygiene, but there is not much that helps this problem.

Interesting! I found a company (Prevention Genetics) that will do it for about $600. I’m not sure if any is covered my insurance. I have a geneticist who is brilliant and knows these genes, but hasn’t been answering me.

This was going to be just this specific area, for familial dysautonomia, not the whole exome. I’d be willing to do that if someone was interested.

Wow, that is impressive! I have not gotten anywhere with finding a company to "fully sequence" it. I don’t have the usual mutation associated with familial dysautonomia, but I do have the variant on it.

I think it’s Eastern European Jews, which I do have on one side... but I’m not positive.

I saw a geneticist who found a previously undiscovered variant on my IKBKSP/ELP1 gene. He recommended I have complete sequencing done but didn’t say where I could get it done, or even answer me when I asked. Has anyone had this done or had a variant on this gene? It’s associated with familial dysautonomia which seems more and more likely the more my mom complains about suspicious symptoms (she doesn’t know I have any of them myself - I do not mention my health). I found a couple of places on the Internet, but am not sure about randomly picking a place...

I saw this - wow, is that interesting. I have never found any kind of oral rehydration to work very well, but IV fluids are magical.

It’s amazing how no one seems alarmed about my weight loss, either. I am quite light for my height and was never overweight. I supplement calories like crazy and still don’t gain... I have no GI issues but also lost my appetite permanently about 18 months ago. I force a lot of meals anyway!

Persistent 20 percent weight loss after having been an extremely stable weight -no matter what- for 30 years or so. I was the same size since I was 12 and now I’m smaller!

11 hours ago, Pistol said:

Dear @merkat30 - I question the accuracy of the BP equipment that took the reading you posted. I do not think that you would be capable of actually having a diastolic BP that high. No doubt you were hypertensive but these digital BP readers are notorious for taking inaccurate readings when your BP is either too high or too low.  I found that to be very much the case for me, I only use a manual cuff since several digital ones were consistently incorrect when I rechecked readings manually. If possible I would get a different, more accurate machine, or get a manual one. Most Retail pharmacies can teach you how to use one correctly. 

I too have both high and low BPs and could not at all tolerate Clonidine, but we are all different. Be careful!!!

Me, too, exactly. I bought a quality BP cuff and it never accurately measured my BP which is low. I would do one arm manually or have my RN husband do it, and use the automated cuff on the other arm. My BP doesn’t vary that much between arms, and I'd get something like 74/56 manually, and 118/70 with the automated cuff. I have the same experience in medical settings. "Oh look, your BP is great!" when I was talking when they took it, and just came in from traffic and aggravation, AND they use a machine. I think they are good - I used them as an EMT - but not meant for those low (or high?) readings. 
 

The problem is that they always want to set me up with a 24 hour monitor - that would make me look like a liar since the readings would be inaccurately high. I have charted hourly BPs for more than 24 hours.

I don’t have hyper POTS and have very low BP - "better" for me is higher, towards 80/60 if I can! I don’t find much effect on my HR from adderall. Maybe it’s a little lower if I’m doing better, but I’m on a beta blocker anyway. 
 

So, when I take a dose of Adderall, I’m less orthostatic, better able to exercise, and my BP is higher/healthier.

No, but I only took mine 6 days per week, and I now take it as needed. I have quite a bit on hand from when I realized I wouldn’t pay to take it daily.

I can’t get a prior authorization which is why I don’t take it regularly - I have a script but it’s too expensive without it. 

I feel somewhat better when I take as little as 2.5mg adderall. 5mg is better. I have better BP readings and exercise tolerance and definitely am less orthostatic.

There are legitimate versions available... but these were a lot cheaper! I stitched the edges of the pillows together, but I tried just using Velcro straps at first. Thanks, though! I love to "invent"!

Thanks. Pillows strapped to my feet help, and oddly, so have supplements. 

For me, it does not seem to relate to the sun. Anything that has pressure on it at night becomes red, hot, and painful, but my ears seem to do this for no reason as well. 

I seem to have erythromelalgia, too. It affects my ears, as well as my heels and elbows at night... 

Can you take a bath instead of showering? It helps me to be laying down and have the pressure of the water around me. You’d probably like weighted blankets like I do - to me they are the sleeping equivalent of compression stockings. I also use sequential compression devices for a hour or so each evening. That machine doesn’t want to go into the shower, though. I have a really hard time getting my 30-40mmHg stockings on, so I use a mixture of these interventions.
 

The stockings are definitely hot in the summer. I try to keep splashing myself with water...

Just slippage while adjusting myself - if one isn’t in good contact, the other feels like a shock - the same happens with regular TENS use. Ouch!