Most Shocking (Good Or Bad) Change To Accompany Your Pots Dx?

[jenglynn]

Hi!!!

LindaJoy started a great thread about how P,O.T.S has changed her relationship for the "not-so-good"... and it got me thinking how many of us go through this every day. It's not like we have a sign we wear that explains it. When I was first was DX'ed I thinking felt slightly relieved. I'm NOT crazy, not for that anyway. Not too long ago I requested all my records- WOW! Never realized how much these doctors hated me!!! The office notes were the best- and so many lies! Anyway, I digress. I won't list all all my DX but I have a very aggressive autoimmune 'disease" I guess because they've never seen it attached to autonomic system before (that's why I'm not sure it is,,, anyway), autoimmune mediated autonomic dysfunction neuropathy-I have 30-45 seconds standing before I'm totally unconscious. No one has found anything to help. Tried IVIG- my body violently rejected it. It got into all my organs and blood. We are still waiting for that to get out of all my cells so we can try again, (sonething else,of course) I have EDS, Post Concussion Disorder (think I'm at 12 now for sure) and some other stuff too but those are the most dangerous at this point. I like to end with the happy because I try to be an optimist!

Cons: how truly SICK I feel- most of the time. The debilitating fatigue. Its fuuny- I hated my job , but now that I'm not working- everyone has their lives. The loneliness! Most importantly the major strain and scary problems it has caused with my husband I love so dearly. The realization at who my "real" friends were some who were not- and it shocked me, because I've always been there for them. This will sound odd given the statements before, how much I WANT to be alone and need it. Next, this is shallow, but what these diseases has done to my body as a whole- physically. I've lost so much hair and weight not in a good way.. I don't look the same, horrible pallor, etc. There are many more, but that's enough to dwell on. I HATE how these illnesses have taken away my ability to be a normal mother and wife. I've missed out on so much. I've tried a few times to go to sporting events- I always ended up passed out, once with a very nasty head injury. The kids would worry, they have been through enough.

PROS: I HATED my job! I do not have to go back!!!!!! The TRUE humanity and good I've found in people since this has happened. People who dont even know me, bringing our family meals!!! Grocery shopping. Sponsoring us for Christmas, had a benefit fundraiser that I'm embarrassed to say got us $22,000. There is so much more, money wise, but I don't want to sound greedy.. But it was almost excessive. People coming to visit me. My little Type A Self had to learn to accept help- not only that- but ASK! So hard. I could do everything, right????? Lol. Yeah, right. I think that even though I can't physically take care of my kids as in dinner, housework, driving, events, etc... I MiGHT be a better Mom, I actually listen to them now. I was always so busy that I was doing 5 things at once.., we have 6 kids- so I'd have at least 3@ a time asking me ths or that. I'd get frustrated. Or I'd listen halfway and do the "Oh REALLY? Wow!" thing. Still can't believe that it almost always applies, now I really hear them. I Enjoy them so much. Yes, I miss out on alot- but I am the most blessed of all. My best friend is my husband!s ex-wife. You read that right. My step kids mom is my BFF, She has 3 year old twin boys who i could not love more had i given birth to them, and that's how she treats MY bio kids,. We decided we were going to raise them all together and equal..... So all 8 kids are brother and sisters. The month I spent in hospitals- they kept all 8 kids so Rob could be with me. I NEVER take this for granted, nor them, I think, It's a miracle. There are so many more but last thing- it brought my extended family so close again and my dad, brother mom, etc. I think we all realized we could lose one of us at ANY time and never take each other for granted again! I live in Wisconsin- they are 2000 miles away in CA. I hate geography but you know- we really appreciate each other. In fact, my dad and his wife (who I consider my mom) wil be here tomorrow. I have learned a lot and my priorities were a mess- well, maybe not a 'mess' but misguided, Yes, a relatively clean house is important but not spending all day, every day- being the martyr- mumbling and slamming things around while I did it all. As awful as this ordeal has been for us, I'm not sure we could have learned these lessons without something this serious. Don't get me wrong- Pollyanna I'm not, but I know that God has a path and plan for us.... We just have to keep looking for it. I have a feeling when we realize it- it will be clear as day!

I always say this was a pretty drastic way for Him to get my attention, but I never listened before. The more I could get done and and a horrible adrenaline junkie besides- plus all of these illnesses I had that no one know about yet. I know I said I was done.... LAST pro for tonight. My daughter is 13. She was born with a hole in her heart. Like me, she was diagnosed with a connective disuse disease at 13 months old, she couldn't sit upon her own and was close to two when she walked. Like me, this child was so flexible but now that I know more she knows not to show off that flexibility. The genetic doctor at the very large famous facility I was at was pretty sure I have EDS just based on the physical exam. Scary part to me at age 11 she started getting syncope. In 2 years she's had 14 syncope spells. ALL before any of this of this had begun had re-started with me. So the doctors told us just I heard years ago- she stood up too fast, some people are probe to fainting, yada yada, We both have chronically large and dilated pupils and our left is 25% bigger (I think- because my main artery on that side is very small compared to the right). Not sure about the artery yet for her. We are going to be doing some very advanced genetic testing, The genetic doctor said it was not uncommon for newborns to be born with large holes in their heart because of the stretchiness of the tissue. Hers was large and she was quite symptomatic, they tried to fix it but could not because everything was just like Gumby. So she was on oxygen, was always monitored, apnea monitor and monthly echocardiograms. When she was 6 it was there one month and TOTALLY gone the next. Not shrinking, not half the size, GONE. Another pro would be finding a cure so she doesn't have to suffer like we do- that goes for ALL of our kids, ANY kids, or ANY person. This facility however would not test her because there is no link to EDS and autonomic.

Thanks for listening! Like I said I could add 100+ more to each list. God Bless

Jen

[bellgirl]

You are such a positive person, and inspiration for us all, Jen!! I hear you, and this disorder definitely changes us, gets our priorities straight, and helps us appreciate all the ones who love us and we love unconditionally, the small moments; some of us learn easier than others...lol. I was always kind of a stubborn sort, so this has humbled me a bit. You're definitely loved here!

Edited May 24, 2012 by corina

[jenglynn]

Oh Kimbelllgirl- my cup runneth over., I don't know what to say except you are the same... Positive well loved, Abd always a word if encouragement. What a lovely compliment. I really mean that.

Thank you and many any blessing to you,

Jen

[HopeSprings]

Pro- see, I told you I'm not crazy. Validation.

Con - what do you mean there's no cure?

[puppylove]

Pros- I have learned a lot. Like getting straight A's is not the most important thing in life, and not to judge people, be thankful for the little things to name a few.

Cons- Feeling guilty about causing turmoil within my family, strained realtionships- especially with my Mom, not getting to be a normal teenager. Also, I used to hate school but I wish I could go back now.

[ophelialit]

Pro--I might FEEL crazy, but I'm not ACTUALLY crazy....awesome!

Con--No, I can't (fill in the blank___ go shopping, to the beach, for a bike ride, out to dinner, to a party, etc.) because I'm (also fill in the blank___dizzy, tired, cranky, woozy, out of breath, in pain, dealing with a crazy heart rate.) Booooo....

[Mytwogirlsrox]

Pro-- my husbands work is transferring us to California to be closer with my family. Incredible since we haven't lived within 2500 miles of my family in 8 years

Con--- I'm still very upset, confused, depressed, etc about the diagnosis

[McBlonde]

Pro .... Now I know I'm not crazy. I have objective medical measurements that prove otherwise.

Con.... My life, as I knew it, it over and won't be coming back.

[DoozlyGirl]

Hi Jen,

You have a really great outlook, especially considering your journey. I hope my story will shed some light on a possible path to better health for you.

There IS a link that connects EDS and dysautonomia, and its dysfunctional mast cells. Research has shown that misbehavin' mast cells can wreak havoc throughout the entire body or localized in one area. There are a bunch of us who are being diagnosed with all three of these disorders. Dr Afrin, a hematolgist/oncologist at Medical University of South Carolina in Charleston firmly believes that EDS and POTS/Autonomic Neuropathy is caused by these overreactive mast cells, and since these mast cells are also linked to just about any inflammatory disorder, it could cover the autoimmune side to your AN. He is curently treating over 300 patients with mast cell issues, seeing his first patient in 2008.

Mayo is not yet on board with this connection, but Dr Afrin and Dr Mairianna Castells, an allergist/immunologist at Brigham and Womens/Harvard in Boston are the two leading physicians in the US who ARE conecting those dots.

I met with Dr Afrin last week Monday and he is absolutely convinced that chronic mast cell activation is to blame for my entire 37 year history of ongoing chronic health issues. We are now testing to find out which mediators are causing my issues. I have a strong family and personal history of autoimmune and inflammatory diseases, as well as aneurysms, atrial septal defect (hole in heart), and pulmonary hypertension in my mom, and aneurysms in her brother, 2 sisters and father, as well as dozens of her distant relatives. I've had a cavernous hemagioma of my skull, which all point to EDS, vascular subtype. I have autonomic neuropathy and severe orthostatic hypotension, and have gone from bedridden for 6 months to somewhat 20 percent functioning with dysutonomic treatment and after being on excalating doses of MCAS medications, histamine-reducing diet and other adaptations, I am at 60 percent functioning 70 percent of the time.

Misbehavin' mast cells will release histamine and begin a cascade of releasing potentially hundreds of chemicals into the body. Several of these chemicals are known to increase vascular permeability, which leads to hypovolemia and in order to save the heart and brain, counter regulatory mechanisms kick in (i e sympathetic overdrive/adrenaline rush). For me, I get flush, which is a sign of histamine release, I get puffy, and sometimes even swell, a sign of vascular leaking, and my brain fog, pooling and erratic HR and BP, (especially narrow pulse pressure) are signs of low volume in my circulation. I have experienced syncope, but now recognize that I have to lie down and raise my legs to get blood immediately back to my brain and heart. I will chug fluids, pop a benedryl or other HI and a H2, and in time my cascade will stop and I will be able to function again.

There are tons of mediators that are released so the treatment depends upon those chemicals that are released, but in general antihistamines (both H1 and H2) are the place to start. Many of us are unable to tolerate meds, so we start out slow and begin with the OTC meds that have less potential of causing issues. For me, I can't tolerate the FD&C dyes. Others can't tolerate the fillers. Check out the mast cell discussions on this forum as well as the Mast Cell Disorders Forum and see if you can find any other similarities.

I'd love to hear Dr Afrin's thoughts on your experience with IVIG. I wonder if IVIG contains mast cell degranulators, which are things that are known to cause these cascading reactions called degranulation. You may want to consider contacting Dr Afrin by email with a brief synopsis of your journey and ask for his perspective to see if mast cell issues could be causing your issues. You could ask if he'd be interested in working with one of your docs. Testing could be tricky, since it has to be performed to his specificiations and samples kept chilled from the time collected all the way though testing. That's the reason I traveled all the way to South Carolina to have testing performed the correct way to ensure best possible chance at confirming my diagnosis.

Let me know if you have any questions. Best wishes,

Lyn