Log: MCAS with Primary Immunodeficiency

[Blake2e]

Very interesting thread. I am very curious to know if either of you have dermatographia ? @Gingergrrl you said IVIg put the MCAS into remission, how was the MCAS diagnosed and what were your symptoms ? I have zero safe foods and can no longer tolerate the meds I took for 18 years for Hashimotos, I have dermatographia all day and night, I can't touch anything, my own hand on my arm
or hip, causes hives and wakes me up repeatedly . How severe was the MCAS ?

I do have dertmagraphia, how does yours bother you? For me I just get red very easily and some swelling but compared to everything else it isnt much. You cant even have lean chicken breast? Have you tried non-fortified white rice? I react to every rice I've tried but this one: https://www.walmart.com/ip/Super-Lucky-Elephant-Jasmine-Thai-Hom-Mali-Rice-5-lb/24689309

Do you have elevated IgE levels?

Do either of you had or have severe chemical sensitivity ? The mint on floss makes me sneeze , if someone is chewing mint gum I sneeze , the smell of shampoo, soap , any chemical , makes me cough , mucus and feel like a just got the flu and I used to smell markers and perfume and candles , I had zero chemical sensitivity so I don't know what this even is happening.

I do a quick sniff or touch would set me off terribly. But with steroids and some other drugs I'm a little less sensitive to it right now but I still stay as far away as possible. I can't even use soap when I shower.

I had my first round of IVIg . I got extremely sick . My throat was so painful I ate a lot of cough drops which made me sick. Mucus was pouring out of me. I felt very hot but no fever . I was shaking I was so cold . I got 18 very large swollen, itchy mounds and I have no idea it bugs attacked me over 3 days or if it was the IVIg . They are much less itchy and the swelling went down but they left purple marks behind . I'm
Not sure how this works . If we can't tolerate smells , food or basic medicine , how is a medication with so many donors , even remotely a good idea? I became violently ill, I ended up in the ER and they wanted to do a spinal tap
And I refused . My sedimentation rate was 89, I don't even know what that means , no one explains anything, they just said "an immune response ". Ok, how illuminating .

What dose of the IVIG did you have? Steroids and the other drugs Im on help me tolerate the low-dose (30g) IVIG I get. Did you take benadryl or any steroids as pre-medication before the infusion? Thats what many do to prevent reactions. If you didnt I would consider doing that before your next infusion. It sounds more like your reaction may have been a large part a reaction to the bug bites. Sedimentation rate is how much inflammation you have and yours is quite high.

The mental confusion is unbearable . The stiff neck and head pain . The nerve pain is torture . Loose stool, every single day. Do we have too much stomach acid or too little ? Today I was told I have "severe asthma" and sleep apnea. I don't understand any of this. Why do I have constant tinnitus and when I turn my head I hear whoosh whoosh , every time ? I'm
So tired I can't believe what a loser I've become . I can't even focus to order shampoo online . It's like never ending tasks that are beyond me but I think I've done them . I freeze up
A lot . I don't have the energy to argue with liars which are mainly the staff in dr's offices , the latest trying to make me pay fully for iron infusions . Does iron become a problem for others ? I have no idea where it's going .

We have too much stomach acid. Histamine promotes production of stomach acid. The ear stuff is also an allergy symptom.

Same here I feel like I've become loser, life used to be so easy before this, whatever I put my focus on I would excel in quickly. Now I feel like I have Alzheimers all the time. I'm sure the staff arent liars, I get paranoid like that too, the excess inflammation promotes a lot of unfounded negative thoughts. I get the same thoughts too but I try to analyze them and not get caught up in them.

Are you anemic/ iron-deficient? If you are that could be one of the reasons for your fatigue. Iron is not a problem for me

As for tilt table , a cardiologist I think is the best person to do the test . I was positive for antiphospholipid , no one said that was a problem .

Do you have POTS? If I were you I would consider looking for another doc and, if you havent, maybe a hem-onc.

Has a anyone tried xolair ? Is it ok to do
This with IVIg ? I was told to do it by 2 dr's and one said don't . Can someone explain why dr's keep trying to give me steroids when I'm already immune suppressed ? I thought IVIg was to boost the immune system , how can this be good to mix with steroids ? They did push solu medrol day 2 of IVIg since things were not going well but since then , the internist and pulmonologist are trying to get me to take steroids . Anyone get crushing head pain from IVIg ? I mean really bad , like you want to die head pain ?

I've been on Xoliar for almost 15 months. Worked great in the beginning until I started reacting to it. When I react to drugs my body kills the drug and it has no effect on me. Steroids and other immunosuppressant sometimes help in making xolair work for me sometimes. Xolair with IVIG is fine, I do both. If I were you I would try really hard to get on Xolair as I think it will help you a lot.

Your immune system is hyperactive in one regard and under-active in another. Steroids would help with over-activity. Do you have primary immunodefeciency? IVIG is immune boosting especially if youre immunodefcient but its suppose to help the MCAS because it is immunomodulary. If I were you I would listen to your doctors and take the steroids as they say, especially before the IVIG. I do get some minor headaches from the IVIG but my pre-meds help me quite a bit, my last infusion went pretty well.

I'm mot sure how this remotely is living . There doesn't seem to be a point anymore to what I'm doing , it's like torture for the hope I get to live yet I just worsen and suffer . Makes no sense .

I get an slight anti-drug vibe from you. To get better you have to start embracing drugs. If youre not able to understand why a certain drug is recommended because your symptoms are wreaking havoc on your brain then I say you should learn to trust your doctors more and do what they say. And if you cant trust your current one then find a doc you can trust, I agree this is torture. But I wont, and you shouldnt, quit till every avenue is explored. I suggest finding distractions if you havent. Books, tv shows, youtube, movies anything.

IVIg is not all that great , maybe high dose is not such a good idea . The very nice nurse choked me with her gross perfume and let me know she has never seen anyone get better from IVIg so that was delightful, I really wonder who had certain MCAS and how was that determined and by who.
I'm also curious if either you have biopsy confirmed small fiber neuropathy or if either of you had intense black mold exposure
Thanks

Tell the nurse the perfume bothers your symtoms and she wont wear it next time. So you did high dose IVIG? What was the dosage and over how many days was it done and did you do the entire dosage or did you stop in the middle? My nurse was telling me the opposite that she has many patients that got better of whatever condition they had with high dose IVIG and I've read tons of studies where high dose IVIG helped with autoimmunes and MCAS.

Have not had biopsy for small fiber neuropathy, have you?, I did get a bone marrow biopsy and was negative for mastocystosis. As far as I'm aware Ive not had black mold or any kind of mold exposure.

 

[Blake2e]

Update: Rituxan has def made a difference in my symptoms. My tolerance for steroids won't improve significantly till my next Xolair, so the steroids are only helping a tiny bit. My immuno sent referrals to 3 hem-oncs I found that deal with mast cell diseases. Gotta wait to see if they accept my case, will call soon to check up on them. Seeing my immuno this week and going to see if he can consult with my current hem-onc and see what he thinks of proteasome inhibitors.

On day 5 of 28 for the Holter monitor. Still need to reach out to Cardiologist to see if doing 2 weeks will be enough since I've been symptomatic enough to give them plenty of data points.

 

[Blake2e]

***I have no idea how to reply to this in sections , hope this works . I get wheals , big red and itchy . I also get deeply imprinted with everything, clothes , socks , paper, sheets , walking on carpet . It brands into me and itches like crazy .it is out of control. No, not even chicken . I tried eating plain white rice . I've tried everything. That rice looks like it has a lot going on but I'll try it, thanks .

My dermatographia doesnt itch and I dont get hives. I get a hyperadrenergic response to histamine, so my body pumps out massive amounts norepinephrine and I believe that is why I dont get anaphylaxis, hives and itching. Though I do get a lot of the other typical allergy symptoms. The rice I react to are the ones that a fortified (including iron), let me know how that rice brand works for you.

***Same here, I recently introduced soap . I just burn and itch . I used any bath product I wanted all my life. I used creams and scrubs and whatever smelled good , I have never been sensitive to anything at all. Now every single thing is a problem . Right now I burn and itch because I used cream because I can't stand it anymore!

I dislike it too, especially the intolerance to deodorant so when I sweat I just need to deal with the stink.

***Yes, they gave me the pre meds, didn't make any difference . I did 25g , three days in a row , 75 total . I didn't have bug bites . They were lumps from the IVIg , the nurse called them big bites but the neuro didn't and gave me steroids . Unless bugs lived on me for 4 days , pretty sure it was the IVIg . It's a possible side effect . Because of the dermatographia, they were pretty swollen and very itchy .

Sorry I misunderstood what you were saying about the "bug bites." Interesting though the high dose didnt help you yet. Maybe it will help the next time or with higher dose? hmm

***i am confused about the stomach acid . One told me too little and another told me too much . I took digestive enzymes for months and added more acid and then they said use pepsid , opposite things . I also have ibs and small fiber so who know what my stomach acid is doing , I have more to consider than MCAS . No, lol, the staff was lying , there are a lot of issues with money and bills when it comes to dr's . They're businesses and want to make money . They do this with a lot of patients . It's complicated and no need to bore you with the details .

Its too much acid I was hearing both things too before I was diagnosed. Typically those with MCAS have too much stomach acid, so if you have other conditions they may play a role in it too.
If theyre hassling you needlessly then I would consider switching docs, though it sounds like you found a good doctor.

***maybe they're being metabolized very quickly, sorry it stopped working but it's a common complaint . What work needs to be done to get on xolair ? Do you prep yourself somehow? The allergist/immune just ordered xolair and they set up the infusion . I didn't take it because I didn't know dermatographia was a symptom of a larger problem , I was burned and ill and thought hives would resolve . When she sent me to the MCAS focused allergist , that office wanted me on IVIg . Each dr had a different idea , one advocated IVIg and the other xolair . Due to immune deficiency and small fiber and many problems , I went with the IVIg but am discussing with them about combining , however , no need to jump into xolair until
I can find a way to reduce the ivig backlash IVIg brought with it. It wasn't a simple headache , they were going to do a Spinal tap, I had a lot of side effects which wasn't surprising considering how reactive I am to simple things . Yes they gave me all the pre meds , made no difference , they ended up pushing solu medrol to reduce the crushing head pain and large red bumps but it did absolutely nothing and now they're discussing if IVIg is ok for me so I don't know .

No, its not that it metabolizing fast, I never had that issue before. Its just that the drugs loses all effect on me, I believe the IgE deactivates things before theyre absorbed in the gut.

Before I do Xolair I premedicate, typically with oral albuterol and time one of my 2 daily doses of Hydroxychloroquine for 1 hour before injection. If insurance covers them I would go for both Xolair and IVIG. But since you reacted to the IVIG, I would do the xolair first then after 2-4 weeks after the Xolair is working then get the IVIG so you react less. Doing the Xolair 1st will help with the IVIG backlash. Just in case you erent doing it drinking a lot of water helps and slowing down the infusion rate should help. Though from what Ive read and hear the headache is sometimes just something you have to deal with.

No one said take steroids everytime . They did not want me on steroids , they're trying to accomplish the opposite , they pushed solu medrol as things went downhill . I went to an internist to help me coordinate my care and find a pulmonologist, neuro wanted me to do that . He gave me a 6 day course of oral steroids and neuro did not agree until we get to the bottom of why I've lost my voice and cough and can't breathe . They say asthma . But no , ongoing steroids the specialists don't want me on. Inhaler or oral .

Ah now I understand better.

***ive tried plenty of drugs . The problem is I am now intolerant , even drugs that I need for Hashimotos that I took for many years with no problem . They had to reduce my dose by 2/3 so Hashimotos is not controlled . My body rejects all food , medication , chemicals , frankly , I think I am poisoned by black mold . I've never had allergies in my life . I could eat cement .

Black mold could be a cause of MCAS. What are you doing about it?

***lol! The nurses don't care . I asked the first day and so did other patients . Where do You live? Must be a special place .
I have not had a bone marrow biopsy but it was suggested . There is too much going on . I'm very ill . I don't heal . The biopsy for the small fiber is usually 2 punches but they did just one because it's so advanced it's even in mg tongue , face , scalp . They biopsy site is still an open wound . I don't heal . They are trying to avoid any puncture . They blew 6 veins during IVIg . Everyone is different .
Unless you have a lot of nerve symptoms it might not be necessary to have a biopsy for small fiber

Thats right, I remember how some nurses could be. I think I got a little lucky being in the region that I am in. I'm in a pretty religious area. Thats rough, the not healing. I dont know much about that. Do you think the MCAS is the cause of the healing problem? What kind of immunodeficiency do you have? I had my 1st ever vein blown with my most recent Rituxan, that sucked. 6 most suck even more.

My nerve symptoms only come on when my MCAS symptoms get bad enough (which is pretty frequent). I doubt its a small nerve issue. Though I'll keep that in mind and talk to my neuro about it just in case.

@Blake2e did you have the tongue depressor test for dermatographism? They scratch one inner arm and you get hives in the exact lines the stick was dragged on your arm? Then hives spread allover ? People are not allergic to sticks and if they were , it would be a random shaped hive and remain local , only where scratched . Dermatographia is from mast cells so you ignite it in one area and you've activated the mast cells which are circulating and you end up with hives covering your body . The allergist did that at the first visit but I already had daily hives for 6 weeks before I got in to see her .
The hives are in the shape of skin writing , you can write your name , your clothes imprint every detail . I ask because I want them know what the potential is with my sympyoms and treatment .

I didnt have the tongue depressor test. I did get scatched on my arm and I did get swelling and reddening where I was scratched. I dont get hives and I dont think my dermatagraphism spreads, honestly my other symptoms are so much more of a hassle that I end up not paying much attention to what my skin is doing. I do know that my dermatographism does come on very easily with the slightest of pressure and in response to heat/cold and I can write my name on my skin.

One dr told me those with true MCAS almost all have dermatographia but I see a lot of people say they have MCAS without dermatographia so I don't know how to gauge how treatment went
General hives and redness is different than dermatographia. The latter means skin writing , put a sheet on your legs and you will have any ripple or line in the sheet , transfered to your legs. There was a good write up I think in a Boston paper and the guy was sick for 5 years and they put him through a lot but then his clothes were imprinting and that's when they saw the dermatographia and he was found to have MCAS. Mine started with dermatographia, it came on like an avalanche . I don't think I had MCAS before that although the dr's said I push myself and concealed it which is possible .

A lot of symptoms of MCAS overlap with CFS , mold exposure , etc . There is not much on verified MCAS and xolair or IVIg . I wish there was a good database on people with MCAS that meet specific criteria , blood and urine work , dermatographia, all these "allergies" and then see how we do on treatments . It's a very grey area at this point.

What works for one person with MCAS may not work for someone else with MCAS. Trial and error. I'm approaching my MCAS in similar manner that I've seen studies approach allergic IgE-mediated disease and in manner similar to treating autoimmunes, BTW do you have elevated IgE?


Learner1 explains here a little how to do the quotes:
https://forums.phoenixrising.me/ind...ry-immunodeficiency.62093/page-2#post-1013114

 

[Blake2e]

*** your dermatographia is interesting, it doesn't itch ? I'm sick of the dermatographia, I've been itching almost around the clock for 15 months straight . I had to stop wearing under garments . It's like everything is being chipped away at . The dermatographia is really bad , I can't sleep because of it . I thought it was the least of my problems but I can't stand it anymore , it never leaves me alone , ever. Thanks for the rice recommendation . I wish I knew the ok foods . I guess my gut is all messed up .

No, fortunately I dont get itchy. Keep looking for foods. Some supermarkets wash their chicken breast with salt water, try to find a place that doesnt.

*** I've only had 1 round of low dose IVIg . I think 75g over 3 days is low . I don't know but I think
So. Once the swelling from over histamine reaction went away , the areas looked like a bunch of tiny bumps. They were so itchy , steroids , rx antihistamines , Allegra , topical steroid , nothing took the edge off , just an itch so bad . It could have been bug bites but they started after the second infusion and multiplied over about 4 days , 18 sites total . Dr thought of was IVIg related. They all left purple blotches . I've started scarring after skin reactions , I really don't know . Like I have band aids etched into my arms for 5 months now , like branded me and all this is new , no sensitivity whatsoever before, none . I hope the IVIg will help but I guess everyone is different . My system is so destroyed I guess I don't have any faith it will help . I had 3 different stink bomb nurses and 1 said well , some people get a little out of it but it wears off after a few weeks and after long term use they end up with more problems. The other one said it doesn't work and the 3rd one said the patients are all old and it doesn't work so maybe hope for me . The patients are not all "old" and she wasn't the brightest anyway . I got sores on the roof of my mouth during IVIg and she said "it's from
The candy you're eating ". I had cough drops because my throat had the worst sore throat of my life , that was definitely from the IVIg , it was so bad . I dread all these symptoms again because I live with every moment being like I have a very bad flu. I have no idea why .

That is rough. If I were you I would do Xolair then a month or so later try the IVIG again. Try to start the Xolair asap.

***nyc is a hassle . Unless you're wealthy , very wealthy , you're getting abused by medical offices , I've witnessed numerous meltdowns from patients losing it from the endless abuse . Forget compassion , I would be grateful if they just flipped me off and gave me medical advice .

I'm actually originally from NYC and I was thinking of going back but now I dont think I want to.

***thats interesting . The MCAS focused allergist said it was about metabolizing too fast. But what you said makes sense . Do you think in some cases the efficacy just goes down ?

Efficacy def goes down. I seriously doubt any metabolizing is happening in my body, just my immune system killing whatever drug before the drug can do anything.

*** yeah xolair was approved at the first visit with the allergist , after she scratched me , I was covered in dermatographia allover my face , neck , everywhere , top to bottom . She called her nurse in and told her to start xolair approval . A week later I was to go have the infusion . It's a long story what set this off. But I was not told this was part of a mast cell disorder . She talked about allergies and I said to what ? I don't have allergies and she used a stick ! I told her suddenly I can't use anything and I had a product routine . I went for 9 months , deteriorating at rapid speed before ever hearing of MCAS . It's such a convuluted story , I have a lot of trauma from it . So had I known what she obviously knew , I would have started the xolair. After I was so ill any sound sent me into a spiral and my pulse was 130s and I was slumping over , losing hearing from nerve damage , I mean I had no ear or sound issues and I now have hearing loss , fear of noise , vertigo , constant tinnitus , I can feel the cilia moving and it itches and hearing my heart when I turn my head . I don't know . It's definitely not all MCAS , that's for sure. I can reproduce hearing my heart. Y just tilting my head , I hear it in the ear I tilt my head towards . Maybe a vascular issue . Saw an ent and all she said was "you have vertigo , your neck and jaw are seizing up and you must have worked with heavy machinery as you have permanent hearing loss in both ears ". I never worked with heavy machinery . I'm frustrated because I'm losing my faculties . The more time that goes by , the more permanent damage . Last thing left is to die from heart failure . There was a progression for example the tinnitus came and went before it became permanent and then it was at a bad pitch befoye it got really loud and then the heartbeat stuff started and the ear itching came 10 months back but is so bad . Nerves are involved . I don't know , the neuro is focused on the IVIg . She felt it urgent as did the second allergist . First allergist felt xolair was urgent . They are not in agreement . I'm ready to throw in the towel . I can't have all these defects and symptoms and ever be even sort of ok . I can't hide it in public anymore , I look sick and I don't make sense and I'm passing out , I'm a useless , diseased thing . My willingness to do all the IVIg and xolair and whatever else is because it's the end of the road . I know it and the dr's know it .

Do both Xolair and IVIG, there are no issues with doing both. Both are urgent.

***i wish I lived where you did . Religious people can be quite kind . I'm not sure what's causing the not healing , I used to heal incredibly well . Very well. If I barely get scratched , so lightly I don't even know I was scratched , I get scabs in lines that sit there for 3 months and leave scars . Bruises come and go . Weird hard lumps under the skin on my legs . My hair and nails stopped growing and my baseline is very quick growing hair and nails . It's all relative . My nails ridged and then turned wavy , my tongue is wavy . First allergist kept hinting I had cancer and said I might have more than MCAS . Always cryptic . It's been hell medically . I ask what do you mean , what are you considering and I get "all I know is you have very severe symptoms and it could be more extensive than MCAS ". I look into
It and ask if she means mastocytosis and she says go see an oncologist . I ask why and it's bsck to well you're very ill. The not healing is weird . It's like my body is dead . Right now I have hives on my eyelids and chest , tired of this .

I agree you should see a hematologist/oncologist and get evaluated for Mastocystosis, it sounding a little like you may actually have it.

***thats good you're spared the itching . Is it improving with IVIg and xolair ?

Im on really low dose IVIG (30g) and that hasnt done anything for my MCAS. Xolair is very helpful when it works and improves everything.

***i don't think so . But the dr that tested it had labs that other dr's said had to be wrong . I asked him and he said the labs are correct . For example my iron will be 200 and then 3 weeks later it's a 19. But according to his lab , the IgE looks normal to me . I noticed some of the Ig tests were flagged red . I've been trying to get those records for months . Sounds absurd but this is how bad my healthcare has been and I would be long dead if I didn't endure constant stress , pushing and pleading . But for now I think it's ok so I don't get any of this . Nothing has helped the MCAS and everyday I think I don't have it but I can't deny the dermatographia nor all the intolerances and 3 dr's said I have it . But maybe it's from environment . There is something missing here .

Then you probably have IgE-independent MCAS (or maybe Mastocytosis). A tyrosine kinase inhibitor may work for you. I really recommend finding a hem-onc knowledgeable in mast cell disorders.
Heres a little help with finding one: http://www.mastcelldisease.com/physicians/wpbdp_category/new-york/

I'll read this , thank you . I tried all the antihistamines, cromolyn , quercitin blah blah . I have zero safe foods so maybe I have a leaky gut?

I hope I didn't ruin your thread , I can delete . I'm really unwell , not linear tonsay the least and feel a lot of stress to find my cats a good home because if I die they will starve to death or be put in a shelter ,

Its all good. You should start a log like mine as well. It could be really helpful.

EDIT: Just wanted to add something. Its natural to feel down, we have plenty of reasons to feel that way, but try to re-direct that energy into action as much as you can. Keep going and going and going until you cant go no more. Youve already shown your resilience but try not to dwell in the bad too much, accept whats going on now but never stop challenging it.

 

[Blake2e]

Im not one that actually gets inspired by motivational vids but this is one that I've come back to over and over since 2013 (from even before I knew I had mcas).

"Let me tell you something you already know. The world ain’t all sunshine and rainbows. It’s a very mean and nasty place and I don’t care how tough you are it will beat you to your knees and keep you there permanently if you let it. You, me, or nobody is gonna hit as hard as life. But it ain’t about how hard ya hit. It’s about how hard you can get hit and keep moving forward. How much you can take and keep moving forward. That’s how winning is done! Now if you know what you’re worth then go out and get what you’re worth. But ya gotta be willing to take the hits, and not pointing fingers saying you ain’t where you wanna be because of him, or her, or anybody! Cowards do that and that ain’t you! You’re better than that!"

 

[Gingergrrl]

@Blake2e I got behind on your thread and plan to catch up on Sunday (so I can read it all in detail and not be rushed). I think someone tagged me in this thread asking about IVIG and MCAS but I can't seem to find the original post? I hope you are doing well (or as well as possible). More later...

 

[Blake2e]

@Blake2e I got behind on your thread and plan to catch up on Sunday (so I can read it all in detail and not be rushed). I think someone tagged me in this thread asking about IVIG and MCAS but I can't seem to find the original post? I hope you are doing well (or as well as possible). More later...

The person that tagged you deleted their posts on this thread for some reason. I'm doing more or less okay. Rituxan is helping but my tolerance to steroids is so far only slightly improved but it wont get better till my Xolair next week. So Im just waiting and distracting myself with school and youtube.

 

[Gingergrrl]

Havent noticed anything with posture or when standing/walking.

Sorry for my delayed reply and I was going to reply over the weekend when the board went off-line. I had been curious about postural symptoms (like changes in BP or HR when you are standing and/or walking). If you don't notice any changes, it is less likely that you have POTS but it will be interesting what the TTT shows (I think you will be having a TTT but now I am not sure)!

Also some sauerkraut, I know its high histamine but when Im not too reactive it

Wow, that is amazing that you can eat sauerkraut which is very high histamine like you said! I have not tested sauerkraut (just b/c it is not something that I eat). I suspect that I would be fine with it now that I am in remission but it would have killed me back in 2015!

I should try lamb to see if it could be a safe food for me. Did you eat lean lamb?

I think it was lean lamb but am not certain. It was organic, grass-fed lamb from Whole Foods (so it did not have hormones or antibiotics in it) but I don't really know if it was lean.

Nice! I'll likely have to deal with the same thing after I get this mast cell business taken care of so its good to see another long-time user recovering.

It is definitely possible b/c I had been taking 15 mg of Cortef per day since May 2015 until I started this taper about six months ago. I am now in the middle of reducing to 7.5 mg per day and should be done with this segment of the taper in 3-4 more weeks. Each phase of the taper takes me about 6-7 weeks. The goal is to get off Cortef but at this rate it will take at least another six months.

My immuno sent referrals to 3 hem-oncs I found that deal with mast cell diseases. Gotta wait to see if they accept my case, will call soon to check up on them.

That is amazing that there are three possible doctors in your area who are familiar with MCAS & mast cell diseases! I didn't realize the hem-oncs treated MCAS (vs. mastocytosis/cancer) and my doctor is an allergist /immunologist.

On day 5 of 28 for the Holter monitor. Still need to reach out to Cardiologist to see if doing 2 weeks will be enough since I've been symptomatic enough to give them plenty of data points.

How is it going so far with the holter monitor?

The person that tagged you deleted their posts on this thread for some reason.

That was so weird b/c I had planned to reply to her when the board came back on-line and I had more time and wonder why she deleted her post? Oh well!

Rituxan is helping but my tolerance to steroids is so far only slightly improved but it wont get better till my Xolair next week. So Im just waiting and distracting myself with school and youtube.

I am glad the Ritux is helping and am hoping you will get even more improvement with your next Xolair. YouTube is addictive and a great distraction. I like to watch the late-night comedians, the news channels, music videos, and a bunch of random nonsense.

 

[Blake2e]

Sorry for my delayed reply and I was going to reply over the weekend when the board went off-line. I had been curious about postural symptoms (like changes in BP or HR when you are standing and/or walking). If you don't notice any changes, it is less likely that you have POTS but it will be interesting what the TTT shows (I think you will be having a TTT but now I am not sure)!

I will be having the TTT, will be doing the full autonomic function testing next friday. I havent noticed anything when standing, unless I do it to abruptly. I slow down all my movements because I physical activity does bother me. Walking beyond a very slow pace has at least a slight effect on me, hard to gauge since I'm pretty much house-bound.

Wow, that is amazing that you can eat sauerkraut which is very high histamine like you said! I have not tested sauerkraut (just b/c it is not something that I eat). I suspect that I would be fine with it now that I am in remission but it would have killed me back in 2015!

To me the histamine content in sauerkraut feels so little compared to the tons my mast cells release, that it barely registers that Im consuming more histamine.

I think it was lean lamb but am not certain. It was organic, grass-fed lamb from Whole Foods (so it did not have hormones or antibiotics in it) but I don't really know if it was lean.

Did you not have an intolerance to fats?

That is amazing that there are three possible doctors in your area who are familiar with MCAS & mast cell diseases! I didn't realize the hem-oncs treated MCAS (vs. mastocytosis/cancer) and my doctor is an allergist /immunologist.

One of them said no to me since he didnt deal with my type of mast cell disorder. Im seeing another one in 2 weeks (though I'll be checking in to see if they have cancellations) and I'm waiting on the 3rd one to review my records when hes back in his office. I'm seeing another Immunologist next week to see their opinion and what they can do. But at this point strong immunosuppressants is what I need and from what I'm seeing hem-oncs are the ones that can prescribe them.

How is it going so far with the holter monitor?

Not bad so far. On day 12 of 28.

I am glad the Ritux is helping and am hoping you will get even more improvement with your next Xolair. YouTube is addictive and a great distraction. I like to watch the late-night comedians, the news channels, music videos, and a bunch of random nonsense.

Thanks. Tons of late-night comedians here as well, especially Craig Ferguson fully addicted to his show haha

 

[merylg]

Interesting thread! I’d just like to say regarding IVIG that I got to ‘give it a burl’ & I had similar reaction each time of stiff neck & seeming like raised pressure in my head. It only lasted a short time & I managed it just with antihistamine.

I did not see any great benefit so I ceased having infusions. I don’t regret trying it. On to the next thing... which has been low dose Doxycycline but I cannot tolerate that for very long either!

Meantime been exploring Whole Genome Sequencing which I think has been worthwhile. I will explore that in some other thread.

 

[Gingergrrl]

I will be having the TTT, will be doing the full autonomic function testing next friday.

Best wishes for your TTT on Fri.

To me the histamine content in sauerkraut feels so little compared to the tons my mast cells release, that it barely registers that Im consuming more histamine.

That is interesting and back in 2015, food dyes and then food itself became the triggers of anaphylaxis for me until I was allergic/reactive to all food but water (and ended up in the hospital). Once I was stabilized on IV Benadryl, I was put onto the SIGHI diet (very low histamine).

With my MCAS doctor (who I started seeing two months after the hospitalization), we kept adding new foods and the histamine content was critical for me. All food and additives on the SIGHI diet were ranked from 0-3 re: histamine content, if they were histamine liberators, etc. I only ate foods that were zeros or ones for about 1.5 years until the IVIG put it into remission and now I can eat anything (except I still avoid FD&C dyes and contrast dyes). Sauerkraut (or pickled and fermented foods) were all a "3" (and truly probably even higher) and would have killed me at that time.

Did you not have an intolerance to fats?

I did not and fats were not high histamine (vs. it just depended on the individual item). Once I was stabilized from the hospital and we were adding foods, both olive oil and organic butter were very safe foods to me. I used to eat ground lamb cooked with olive oil (or butter) and salt and white rice or sweet potato for almost every meal until I could slowly add more foods. I remember adding chicken breast, ground beef or ground turkey as alternatives to the ground lamb (also cooked with olive oil and salt) plus either the white rice or the sweet potato. Then I slowly kept testing other foods like apples, blueberries, carrots, and low histamine fruits and veggies.

Now I can eat anything included leftovers, fermented foods, even alcohol. I just continue to avoid the foods that I was truly allergic to life-long (prior to MCAS) and avoid dyes, especially yellow #5/Tartrazine and contrast dyes. I am also no longer reactive to smells (like paint, smoke, perfume, cleaning supplies, etc). I avoid them if possible but if I can't avoid them (like if someone next to me in line or at a movie has perfume, I am not reactive).

One of them said no to me since he didnt deal with my type of mast cell disorder. Im seeing another one in 2 weeks (though I'll be checking in to see if they have cancellations) and I'm waiting on the 3rd one to review my records when hes back in his office. I'm seeing another Immunologist next week to see their opinion and what they can do. But at this point strong immunosuppressants is what I need and from what I'm seeing hem-oncs are the ones that can prescribe them.

I am so glad that you are being persistent and proactive and I know you will find the right doctor. I never found a Neuro (back in 2016) and ultimately had to give up but luckily in my case, my main doctor (an internist) and MCAS doctor (allergist/immunologist) were able to create a treatment plan with IVIG and Rituximab that put me into remission over the course of two years without any Neuro being involved. It would have been great to find a Neuromuscular specialist (b/c I had such extreme muscle and breathing weakness that my main doc felt was from the LEMS autoantibody) but the Neuros were beyond useless. I would love to go back to the Neuros who doubted me but it is not worth my time.

Not bad so far. On day 12 of 28.

Tons of late-night comedians here as well, especially Craig Ferguson fully addicted to his show haha

I have never watched Craig Ferguson but in case you are still trying to distract yourself with YouTube (or you just want a good laugh), I watch Stephen Colbert, Seth Myers, Bill Maher, and Trevor Noah (and maybe others that I am forgetting)?

 

[Gingergrrl]

Interesting thread!

Hi Meryl and it is great to see you!

I’d just like to say regarding IVIG that I got to ‘give it a burl’ & I had similar reaction each time of stiff neck & seeming like raised pressure in my head.

I honestly do not think it is possible to do IVIG without it triggering a headache/ head pressure and stiff neck. This happened for me with every infusion for two years (although it definitely varied in the severity). For me the pay-off was worth it.

It only lasted a short time & I managed it just with antihistamine.

I did not see any great benefit so I ceased having infusions. I don’t regret trying it.

Did you try low dose for immune deficiency or high dose for autoimmunity? I think the last time I chatted with you, you had discovered some autoimmunity (but I might be totally confused)! How long did you try it for?

On to the next thing... which has been low dose Doxycycline but I cannot tolerate that for very long either!

Is the Doxy for Lyme or something else?

Meantime been exploring Whole Genome Sequencing which I think has been worthwhile. I will explore that in some other thread.

Definitely keep us posted and I had been wondering how you were doing. Best wishes with whatever you decide.

 

[Blake2e]

Still with the ups and downs. Found a new Hematologist (doesnt know a lot about MCAS) but said hes willing to take on my case and do his best for me. All the other hem-oncs I tried to see turned me down. My original hem-onc (who prescribed the Rituxan) is still in the picture but this new one will be my main one.

I was purposefully letting myself get worse so I can test my tryptase levels then the day after that I started taking the steroids again and it didnt work and my symptoms flared up that night and continue to today. 110mg of Zyrtec let me get some sleep last night though. Every time I covered myself with my blanket my heat intolerance kicked in (something that wasnt much of an issue past couple of months) and gave me tachycardia, sweating etc.

During the "up" days had brutal debilitating fatigue but my tolerance to drugs and foods was improved. Chocolate felt like a genuine stimulant. Took DHEA, agmatine, L-Glutamine. Felt a little like a man/normal again. Even felt well enough to set up a date for tomorrow, though might need to cancel.

Might be trying Cytoxan soon. Will try to get IV rather p.o., since I think the former will work better. It should wrk well with Rituxan. We'll see how thing goes. Gotta give the new hem-onc Afrin's studies as well.

Edit: Saw autonomic Cardiologist, after seeing results of my month-long Holter monitor, he doesnt think I have autonomic dysfunction or POTS. Diagnosed with sinus tachycardia and he said it is a stress response to my mast cells.

 

[Gingergrrl]

Found a new Hematologist (doesnt know a lot about MCAS) but said hes willing to take on my case and do his best for me. All the other hem-oncs I tried to see turned me down. My original hem-onc (who prescribed the Rituxan) is still in the picture but this new one will be my main one.

I just read your update, Blake, and I am glad that you found a new doctor and hoping that it works out well.

I was purposefully letting myself get worse so I can test my tryptase levels then the day after that I started taking the steroids again and it didnt work and my symptoms flared up that night and continue to today.

This may not relate to your situation (but I hate to see you purposely making yourself worse!) so just in case it is relevant, I never had a positive Tryptase test. Even when my histamine was 4x the normal limit and my prostaglandin levels were off the charts, and even when I was having anaphylaxis and in the hospital, my Tryptase was still negative.

110mg of Zyrtec let me get some sleep last night though.

Was this a typo or did you literally take 110 mg of Zyrtec?! When my MCAS was the most reactive, I took 20 mg/ day Zyrtec (and my MCAS doctor said he had some patients who were as high as 50 or 60 mg/day but that was very high and very uncommon). Have you tried Atarax (sorry if I've already asked you this a million times).

Might be trying Cytoxan soon.

Is the Cytoxan for MCAS or something else? I have never heard of this before (outside of cancer).

Edit: Saw autonomic Cardiologist, after seeing results of my month-long Holter monitor, he doesnt think I have autonomic dysfunction or POTS. Diagnosed with sinus tachycardia and he said it is a stress response to my mast cells.

My initial diagnosis (following the first Zio Patch in 2013 which is like a Holter Monitor) was "Inappropriate Sinus Tachycardia" or "IST" but the diagnosis was later changed to POTS (and then even later changed to "Autoimmune POTS").

 

[Blake2e]

I just read your update, Blake, and I am glad that you found a new doctor and hoping that it works out well.

Thanks. The new doc's energy seems promising.

This may not relate to your situation (but I hate to see you purposely making yourself worse!) so just in case it is relevant, I never had a positive Tryptase test. Even when my histamine was 4x the normal limit and my prostaglandin levels were off the charts, and even when I was having anaphylaxis and in the hospital, my Tryptase was still negative.

My 2nd tryptase levels werent elevated in comparison to the base. But I equally reactive both times, just the 2nd time around felt worse because I had quite a good "up" in between and got sick of feeling so sick. But thats good to know. I dont think Im ever gonna let myself get worse on purpose again. Every time I do it for a test, I end up even worse afterwards. Like the day after I did the tryptase test steroid I was using didnt work anymore and symptoms increased. Im getting another tryptase done but I'll only do it if my symptoms happen to get worse unintentionally,

Was this a typo or did you literally take 110 mg of Zyrtec?! When my MCAS was the most reactive, I took 20 mg/ day Zyrtec (and my MCAS doctor said he had some patients who were as high as 50 or 60 mg/day but that was very high and very uncommon). Have you tried Atarax (sorry if I've already asked you this a million times).

No typo. Rituxan allowed me to make use of some drugs that stopped working for me but intolerance quickly increases. So Ive increased to that dose over the span of a few months where I would take once a week. I dont recommend anyone doing this. You havent asked me that before. I have not tried Atarax. But it would likely become the same as with everything else.

Is the Cytoxan for MCAS or something else? I have never heard of this before (outside of cancer).

Cytoxan is Cyclophosphamide. It's used for cancers and as an immunosuppressant. https://en.wikipedia.org/wiki/Cyclophosphamide

My initial diagnosis (following the first Zio Patch in 2013 which is like a Holter Monitor) was "Inappropriate Sinus Tachycardia" or "IST" but the diagnosis was later changed to POTS (and then even later changed to "Autoimmune POTS").

Howd the process of them changing the diagnosis go? Did they just run more tests? I have that neuro appt that I rescheduled before coming up, they may find a different diagnosis though I doubt it since I dont have any orthostatic intolerance. Id have to let myself get pretty reactive for that appt ugh not sure what to do.

Edit: added some stuff and changed some stuff for accuracy

 

[Gingergrrl]

Thanks. The new doc's energy seems promising.

I dont think Im ever gonna let myself get worse on purpose again. Every time I do it for a test, I end up even worse afterwards.

I agree and don't think it is worth it to do something to purposely make yourself worse.

No typo.

Holy cow, that is a high dose of Zyrtec! You weren't joking about building up a tolerance

You havent asked me that before. I have not tried Atarax. But it would likely become the same as with everything else.

I am curious if Atarax would work better for you than some of the other H1 antihistamines. It was my "rescue med" for about 1.5 years w/allergic reactions prior to IVIG.

Cytoxan is Cyclophosphamide.

Is that the same med as the "Cyclo" trials that many people have mentioned on the board in the past few years? If so, I didn't realize that it was called Cytoxan. Is that trial done or still ongoing?

Howd the process of them changing the diagnosis go? Did they just run more tests?

Yes, lots more tests including two Tilt Table Tests in 2014 and 2016. At the end of 2014 I switched to a Cardio who was an autonomic specialist and he put me in hospital for a week and did lots of tests and diagnosed me with POTS. We tried lots of meds, which did give some symptom reduction, but ultimately he was not able to help me and I stopped seeing him (although was a nice person).

In 2016, I had a second TTT at Stanford which showed "Significant POTS" and I had other abnormal autonomic testing, too, like the QSART test. But the most important thing to emerge from that trip was the positive autoantibodies on the Mayo testing (they sent the blood to Mayo, I did not go there). That changed everything for me moving forward.

 

[Blake2e]

I agree and don't think it is worth it to do something to purposely make yourself worse.

Problem is I'll have to do it again for the upcoming Neuro appointment so they can properly evaluate me for autonomic dysfunction. Ugh gonna have to push that appointment for later since it is near the day for my licensure exam and I cant be feeling that bad that day.

I am curious if Atarax would work better for you than some of the other H1 antihistamines. It was my "rescue med" for about 1.5 years w/allergic reactions prior to IVIG.

I see no harm in trying. I'll ask my immunologist for it.

Is that the same med as the "Cyclo" trials that many people have mentioned on the board in the past few years? If so, I didn't realize that it was called Cytoxan. Is that trial done or still ongoing?

I think it is, I recall skimming some threads about it a while ago. I'm not sure about the trials as I wasnt really following it. I'm going to see if I can get the hem-onc to consider tyrosine kinase inhibitors before we try the cytoreductive drugs like cyclophosohamide which can cause whole body hair loss and infertility (if I ever get healthy again it would be nice to have a family).

Yes, lots more tests including two Tilt Table Tests in 2014 and 2016. At the end of 2014 I switched to a Cardio who was an autonomic specialist and he put me in hospital for a week and did lots of tests and diagnosed me with POTS. We tried lots of meds, which did give some symptom reduction, but ultimately he was not able to help me and I stopped seeing him (although was a nice person).

In 2016, I had a second TTT at Stanford which showed "Significant POTS" and I had other abnormal autonomic testing, too, like the QSART test. But the most important thing to emerge from that trip was the positive autoantibodies on the Mayo testing (they sent the blood to Mayo, I did not go there). That changed everything for me moving forward.

The autonomic cardio didnt carry out as many tests as I thought he would (just Holter monitor). But the Neuro Im seeing soon will do all that minus the Mayo testing.[/QUOTE]

 

[Gingergrrl]

Problem is I'll have to do it again for the upcoming Neuro appointment so they can properly evaluate me for autonomic dysfunction.

Do you mean that you will stop taking meds for a TTT or for something else? Prior to my TTT (and other autonomic testing) at Stanford in early 2016, they wanted me off all POTS meds and MCAS meds for two weeks prior to the testing! At that time, I told them that this was not possible and without the MCAS meds, I would not be able to eat my 4-5 foods without anaphylaxis and without the Atenolol my HR would be in the 160's or 170's every day I would not even be able to make the trip.

So they ultimately agreed that I could remain on the MCAS meds and just for the day of the test I did not take my Atenolol or Midodrine. All of my testing was abnormal and my HR went up 48 bpm as soon as they tilted the table and then it continued to rise. Once it was all over, I took the Atenolol.

At present, I have many days that I don't take my MCAS meds b/c I no longer need them (and would stop them to see what happens but am advised to wait until I am completely done with my Cortef taper in about six months). But I never skip the Atenolol b/c I feel the remission from POTS is the most precarious (and the shortest) of all my symptoms and I am not willing to risk it. I've been in remission from MCAS for 2+ years but only about six months from POTS.

I see no harm in trying. I'll ask my immunologist for it.

If you do try Atarax, let me know how it goes.

I think it is, I recall skimming some threads about it a while ago. I'm not sure about the trials as I wasnt really following it. I'm going to see if I can get the hem-onc to consider tyrosine kinase inhibitors before we try the cytoreductive drugs like cyclophosohamide which can cause whole body hair loss and infertility (if I ever get healthy again it would be nice to have a family).

If we are talking about the same chemo med, Cyclo, can also cause bladder cancer. It is totally different than Rituximab and much more dangerous (which I'm sure you know and I don't mean to sound alarmist or bossy)!

The autonomic cardio didnt carry out as many tests as I thought he would (just Holter monitor). But the Neuro Im seeing soon will do all that minus the Mayo testing.

I wonder if it would be worth it for you to do the Mayo DYS1 Panel (autoimmune dysautonomia). This was the core of my illness-- that autoimmunity was behind everything including the POTS and dysautonomia. But I know this might not be the case in your situation.

 

[Blake2e]

Do you mean that you will stop taking meds for a TTT or for something else? Prior to my TTT (and other autonomic testing) at Stanford in early 2016, they wanted me off all POTS meds and MCAS meds for two weeks prior to the testing! At that time, I told them that this was not possible and without the MCAS meds, I would not be able to eat my 4-5 foods without anaphylaxis and without the Atenolol my HR would be in the 160's or 170's every day I would not even be able to make the trip.

So they ultimately agreed that I could remain on the MCAS meds and just for the day of the test I did not take my Atenolol or Midodrine. All of my testing was abnormal and my HR went up 48 bpm as soon as they tilted the table and then it continued to rise. Once it was all over, I took the Atenolol.

At present, I have many days that I don't take my MCAS meds b/c I no longer need them (and would stop them to see what happens but am advised to wait until I am completely done with my Cortef taper in about six months). But I never skip the Atenolol b/c I feel the remission from POTS is the most precarious (and the shortest) of all my symptoms and I am not willing to risk it. I've been in remission from MCAS for 2+ years but only about six months from POTS.

Yes I'll have to be off everything for at least 2 weeks so Im super reactive and my heart rate stays above 140 all day. Aka the point where all I'll be thinking about is offing myself. I'm so tempted to just skip this, I see no upside of doing the autonomic testing. When my MCAS is controlled I get no autonomic dysfunction so I just need to treat the MCAS. If I am diagnosed with POTS (which I doubt I will be-- no or very very mild orthostatic intolerance), how is that gonna help me? I doubt even with that I will get high dose IVIG.

If you do try Atarax, let me know how it goes.

Yep I'll post it here and tag you.

If we are talking about the same chemo med, Cyclo, can also cause bladder cancer. It is totally different than Rituximab and much more dangerous (which I'm sure you know and I don't mean to sound alarmist or bossy)!

Youre right cyclo is too much for now. According to this study (https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4903110/) I still have 4th and 5th line of therapy to try before the last line which is cyclo is a part of. Going to try to get tyrosine kinase inhibitor or a drug that depletes me of plasma cells so I can bring my IgE levels down to zero or very close.

I wonder if it would be worth it for you to do the Mayo DYS1 Panel (autoimmune dysautonomia). This was the core of my illness-- that autoimmunity was behind everything including the POTS and dysautonomia. But I know this might not be the case in your situation.

I should probably do that. I messaged one of my neuros about it a while back but he never responded. Gonna see if I can get my Naturopath to prescribe it.

 

[Gingergrrl]

Yes I'll have to be off everything for at least 2 weeks so Im super reactive and my heart rate stays above 140 all day. Aka the point where all I'll be thinking about is offing myself. I'm so tempted to just skip this, I see no upside of doing the autonomic testing.

I know you said that you don't have POTS or OI but if you skip your POTS meds for a TTT, your HR is in the 140's all day? I just want to make sure that I understand. Were you taking POTS meds when you wore the holter monitor?

When my MCAS is controlled I get no autonomic dysfunction so I just need to treat the MCAS. If I am diagnosed with POTS (which I doubt I will be-- no or very very mild orthostatic intolerance), how is that gonna help me?

Or... do you mean that you do not take any meds for POTS and it would be the MCAS meds that you would be skipping for the TTT? I think you are saying that in your case, if the MCAS is under control, then you have no POTS or tachycardia? My situation was different and even when my MCAS was in complete remission, I still had POTS. And I actually had POTS for two years (2013 & 2014) prior to even developing MCAS in 2015. Now it is all in remission but the POTS was the final thing to go into remission in 2018.

I doubt even with that I will get high dose IVIG.

I forgot, is the issue that your doctors will not prescribe it or that your insurance will not pay for it?

Youre right cyclo is too much for now. According to this study (https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4903110/) I still have 4th and 5th line of therapy to try before the last line which is cyclo is a part of. Going to try to get tyrosine kinase inhibitor or a drug that depletes me of plasma cells so I can bring my IgE levels down to zero or very close.

I agree and I would avoid Cyclo at all cost (if it were me) b/c it is too dangerous.

I should probably do that. I messaged one of my neuros about it a while back but he never responded. Gonna see if I can get my Naturopath to prescribe it.

I would think that any doctor (even a naturopath) could order these tests if they wanted to. The Neuro who first ordered them for me was a one-off consult and was not my regular doctor.