Log: MCAS with Primary Immunodeficiency

[Billt]

So much knowledge you guys have !!!! Thanks for sharing .

 

[Blake2e]

Update:

Official diagnosis: POTS with limited autonomic neuropathy.

Of course, secondary to MCAS.

 

[Gingergrrl]

@Blake2e I just realized that I am super behind on this thread but plan to catch up with it over the weekend. Very interesting that your testing showed POTS and autonomic neuropathy like mine did in 2016. We’ll have to compare notes later

 

[Blake2e]

Testing sun allergy:

Last Rituxan infusion (6/26/2019) felt like it started helping immediately. Today was feeling a bit daring and tested my sun allergy on this fine sunny day with a temp of 93F. Sun rays were super intense, maybe shouldve chosen a cloudy day and not done this at noon. Total time under sun was probably 10 minutes while I was running errands (which I normally try to do after sunset). No hat, short sleeves but wearing pants. I used to always wear cap and hoodie when going outside for any amount of time and only recently Ive been wearing the cap/hoodie combo with less frequency.

Symptoms: Severe migraine; intense fatigue; nerve tingling in face, scalp and hands; some peripheral neuropathy acting up in hands; mild flushing but barely any POTS symptoms (slight increase in heart rate, no sweating or breathlessness).

Takeaway: Not bad considering I've taken no meds before testing this and just before my Rituxan a few days ago I was incessantly popping pills and only getting very slight relief. Indicates to me my MCAS is dipping into the more mild territory. When my MCAS is mild (or at least what I consider to be "mild" for myself) I get more severe migraines and fatigue. So things are looking pretty good with this last Rituxan. Took some Zyrtec (Kirkland brand) to remedy some of the migraine and fatigue. Looking forward to seeing how this changes over the next week.

 

[Gingergrrl]

@Blake2e I think I am following three of your threads now! I relate to so much of the medical stuff that you share (re: MCAS, POTS, IVIG & Rituximab) and finally just caught up on this thread. I am going to reply in 2 posts so my replies do not get too long for you or others to read in the future.

1) Had my 3rd Rituxan maintenance today. Went well. I was on a 2 month schedule very briefly but due to the conditions set with receiving this drug without cost I was forced onto a 3 month schedule.

So you are now doing Ritux maintenance infusions every 3-months? Is this your plan moving forward or will you be trying to reduce the interval between infusions? My last infusion was at a 5-month interval (for clinical reasons, not insurance driven) and if all continues to go well, we will do the next one at a 6-month interval (which would be the first week of Nov).

We last checked my B-cells (prior to my last infusion) when the interval between doses was approx 4.5 months and they were still at zero. We actually suspect they will still be at zero even with a six month interval between infusions. The true test will be what happens to me once the B cells start to grow back, and if they grow back healthy or diseased?

Sun allergy, as well as all allergies felt amazingly better.

This is great that your sun allergy is better! I finally tested this a few weeks ago when I attended my niece's graduation which was outdoors in the sun and it was a very hot day. I did wear a huge sun hat and sunglasses but I did not get an allergic reaction to the sun. Back in 2015, I could not be in the sun for more than a few minutes without getting a horrible skin rash and feeling very ill. My recent sun exposure (or maybe the heat itself?) was still challenging, and I felt out of breath walking back to the car, but overall, it was not too bad.

Metabolic age on my scale went from saying I was 2 years older than my real age to 3 years younger than actual age.

What kind of scale measures your "metabolic age"? I am now very curious about this! Someone last night (a total stranger) guessed that my age was 27 which was so odd since I am 48. I know that I look much younger than my age but I don't look that young which is absurd!

No longer retaining large amounts of water (angioedema) in face and gut.

I asked you this in one of your other threads but am now wondering if what you are calling "angiodema of the gut" is the same as what I refer to as "third spacing" (of the abdomen)? What symptoms do you experience with this?

On the tilt table my heart rate was 80-100 while in horizontal position then jumped to 150+BPM in the vertical position.

Mine was similar except that my HR before they raised the tilt table was lower than yours. But once they raised it, my HR immediately jumped up 48 bpm and then it continued to rise (this was back in early 2016 prior to me starting IVIG or Rituximab).

Chromogranin A looks like a good marker for mast cell activation

My main doctor considers Chromogranin A to be a good mast cell marker.

These numbers strongly refute my anaphlatoxin theory.

I just Googled "anaphylatoxins" and was not familiar with this term (which surprised me after all of the MCAS research that I did for several years ). According to Wiki (I know not the best source but...), it says:

Anaphylatoxins indirectly mediate:

Anaphylatoxins are able to trigger degranulation (release of substances) of endothelial cells, mast cells or phagocytes, which produce a local inflammatory response. If the degranulation is widespread, it can cause a shock-like syndrome similar to that of an allergic reaction.

• smooth muscle cells contraction, for example bronchospasms
• increase in the permeability of blood capillaries
• C5a indirectly mediates chemotaxis — receptor-mediated movement of leukocytes in the direction of the increasing concentration of anaphylatoxins

So... are these toxins that trigger mast cell degranulation, bronchospasm, blood vessel permeability of histamine, and I assume actual anaphylaxis? It says a "shock like syndrome similar to that of an allergic reaction". What does that mean if not anaphylaxis?

And what tests did you have to measure these?

 

[Gingergrrl]

Part 2...

Life is a game and an exciting, new strategy game has just started; good news as thats my favorite type of game. Lets see how high I can rack the scoreboard/accounts and overall life satisfaction for myself and close ones.

It was interesting to read your personal views on your life and future but I don't want to comment on this part since this is not a Member's Only thread and am going to stick with the medical stuff.

Good to hear you got your infusion, hopefully you experience another level of symptoms relief with it.

Thank you and I reached a point of significant improvement which the Rituximab is now maintaining (without IVIG since my last IVIG was one full year ago). But I am not sure if I am still having additional improvements or staying at this same level. It is so hard for me to judge and also the level of neck pain that I experience when I push it further really holds me back and I wish I could find something to help this.

The Rituxan allowed me to tolerate the adderall enough for it to actually work and get the brain energized and focused.

That makes sense (that it allowed you to tolerate the Adderall which allowed you to do all of your exams and work). Or at least I think that is what you meant?

What symptoms was it specifically thats still preventing you from doing so?

I answered this in your other thread but let me know if I missed anything and I will reply further.

Official diagnosis: POTS with limited autonomic neuropathy.

Do you know what your doctor meant re: "limited autonomic neuropathy"?

 

[Blake2e]

Update:

Last Rituxan was about 5 weeks ago. Tons of ups and down but mostly downs. The Xolair 2 months ago (before last Rituxan infusion) didnt work at all, but last month's Xolair worked but only for 2 weeks instead of the 4 wks its suppose to be. Was off steroids cold turkey for those 2 weeks, then after Xolair wore off symptoms rushed back. No sleep unless a lot of any of one these benadrly, doxepin, phenibut and melatonin. Barely getting 2 hours of sleep on days not on phenibut. POTs/adrenalization on high, all day long. Constant putrid sweating, no appetite, nausea, and diarrhea if I try to force myself to eat.

Fortunately last Xolair partially reset my allergies and drug resistances, so steroids (which I still have to cycle every 5 or so days) are working just enough to take the edge off and allow other drugs to work enough for me to eat at least half what I normally eat and get 4-6 hours of sleep.

Was transitioning back to the zero-carb/full-keto/carnivore diet, and it was optimizing my hormones and I was gaining muscle mass. For 2 weeks I was working out 2 days a week for 20-30 minutes each session, results from that amplified keto gains. People started asking me if I started working out again, bc of how much I physically was changing. Somewhat frequent flashes of my normal cheeky and flirty personality was coming out. Girls in the office talking about me with a gleam in their eyes and asking me if I'm a player (hilarious considering I havent had sex in over 2 years, since my allergies became unmanageable by normal means). Once again lots of weird flashes of my life returning to what it once was, something I havent experienced for a few months now.

After those 2 weeks of Xolair, no bueno times returned and daily life became a struggle and my natural happy state receded as my body started getting jacked up by my allergies again.


--Assessment: I believe being forced back to a 3 month Rituxan maintenance schedule significantly set back the rate of improvement I was getting. With so much time b/w infusions my state dropped to a lower level then what I was in the infusion before my most recent one. And this most recent Rituxan now has to bring me back up from that lower level to where I was previously and that takes time. Rituxan seems to steadily improve my non-IgE MCAS component over the 1st 2 months then stops or drastically slows down after that. Good indication of that, aside from my symptom severity, is that Xolair works for the 1st 2 months after Rituxan infusion but then stops after that.

--Plan: Now that I'm back on the 2 month Rituxan schedule I believe improvements should to start come back. Next one is in 3 weeks. Gonna judge if I'm gonna try to switch or add a new immuno-suppressant based on how that goes.


*** I know I said I was gonna reply to all the messages that have been building up but I'm having a hard time putting words on the screen and have been exerting my very limited energy on making sure I do my job, which is currently still 3 days a week, very well and without causing any alarms in any one that I'm sick or struggling. Thanks to steroids, Phenibut and some other nootropics I took earlier today for work, my head feels baerly okay enough to write.

Fortunately job is easy, the main impact MCAS has on my work currently is I sometimes take a little bit longer than normal to get a task done. Phenibut is still my miracle drug for work but I've been taking it for about 2 months, and tolerance plus my mast cell-induced drug resistance have made my dosages become monstrous compared to what it used to be. (used to be just 50mg, prior to MCAS, was enough for most of the day, and today I probably took about 3 grams. No physical dependency, no mental dependency (though I've tried plenty of supposedly addictive stuff and I've never experienced this with any drug. However, I have experienced physical dependency on drugs.), could easily take a few days off of it, and I have, and I get no withdrawal. If used right, like I'm doing, negative aspects of Phenibut arent an issue.

New drug I'm phasing in to replace Phenibut (GABA molecule with phenyl ring attached) while I give it a month or 2 for tolerance reset, is Picamilon (GABA molecule attached to Niacin) as it has much less of a chance of building up a tolerance where you need more for it to work like with Phenibut. Picamilon has no issues with withdrawal and dependency and could be taken daily without any of the issues associated with Phenibut. GABA is a mast cell stabilizer and if you get autonomic dysfunction it is also amazing for that. Crosses blood brain barrier easily and calms central nervous system. Only issue with Picamilon is your body needs to adjust to all the niacin and I guess if methylation has some sort of impact on your healthy then the niacin could interfere with that. I'm homozygous C667T for MTHFR and I never noticed a difference experimenting with that.

 

[Blake2e]

@Blake2e I think I am following three of your threads now! I relate to so much of the medical stuff that you share (re: MCAS, POTS, IVIG & Rituximab) and finally just caught up on this thread. I am going to reply in 2 posts so my replies do not get too long for you or others to read in the future.

So you are now doing Ritux maintenance infusions every 3-months? Is this your plan moving forward or will you be trying to reduce the interval between infusions? My last infusion was at a 5-month interval (for clinical reasons, not insurance driven) and if all continues to go well, we will do the next one at a 6-month interval (which would be the first week of Nov).

I was on a 3 month, then briefly on a 2 month schedule and experirence dramatic improvement, then was put back on a 3 month and lost some of the improvement from last time. Now I'm back on a 2 month schedule. I beleive my MCAS operates under at least 2 mechanism, IgE-mediated allergies and non-IgE mediated allergic reactions. Rituxan helps keep my IgE down but it also modulates my immune system in another way that helps with the non-IgE allergies. And I'm not sure what this second mechanism works, I thought anaphylatoxins but thats looking like it isnt.

We last checked my B-cells (prior to my last infusion) when the interval between doses was approx 4.5 months and they were still at zero. We actually suspect they will still be at zero even with a six month interval between infusions. The true test will be what happens to me once the B cells start to grow back, and if they grow back healthy or diseased?

Yep that will be the true test. Mine has remained at essentially zero every time I tested it.

This is great that your sun allergy is better! I finally tested this a few weeks ago when I attended my niece's graduation which was outdoors in the sun and it was a very hot day. I did wear a huge sun hat and sunglasses but I did not get an allergic reaction to the sun. Back in 2015, I could not be in the sun for more than a few minutes without getting a horrible skin rash and feeling very ill. My recent sun exposure (or maybe the heat itself?) was still challenging, and I felt out of breath walking back to the car, but overall, it was not too bad.

Nice! Sounds like you still avoid sunlight despite your MCAS being in remission, are you still avoiding your old allergies? I know youre avoiding that food dye (I believe it was) that gave you the worst anaphylaxis, My sun allergy has been a real nuisance once again more recently, though on days I take Phenibut that all my allergies, including UV, is suppressed a good deal. Attended an outdoor dinner party 2 weeks ago that was in the late afternoon while the sun was still out. Was in the light for 2-3 hours before sunset and while I still felt a bit sick and fatigued I was able to be somewhat normal and carry conversations and make jokes thanks to that.

What kind of scale measures your "metabolic age"? I am now very curious about this! Someone last night (a total stranger) guessed that my age was 27 which was so odd since I am 48. I know that I look much younger than my age but I don't look that young which is absurd!

I use this scale , no idea how accurate it is nor how its calculated but my metabolic age is 1-3 years more than my real age when MCAS is more active and I'm retaining a lot of water. An when I'm feeling good ans strong its 1-3 years below my actual age.

Nice, thats good. That guy was probably hitting on you.

I asked you this in one of your other threads but am now wondering if what you are calling "angiodema of the gut" is the same as what I refer to as "third spacing" (of the abdomen)? What symptoms do you experience with this?

Angiodema in the gut = bloating. What I have is likely more second spacing rather than third spacing, but it may very well be that. I dont quite know how to differentiate them.

My main doctor considers Chromogranin A to be a good mast cell marker.

Nice, I'm thinking it will be good for me too. Will see how it continues to match my plasma histamine lvls after further testing.

I just Googled "anaphylatoxins" and was not familiar with this term (which surprised me after all of the MCAS research that I did for several years ). According to Wiki (I know not the best source but...), it says:

So... are these toxins that trigger mast cell degranulation, bronchospasm, blood vessel permeability of histamine, and I assume actual anaphylaxis? It says a "shock like syndrome similar to that of an allergic reaction". What does that mean if not anaphylaxis?

And what tests did you have to measure these?

Clinically, IgE induced allergic reactions (including anaphylaxis) and complement or anaphlatoxin induced allergic reactions are indistinguishable from one another. I posted a few links about this in the previous page of this thread.

Biggest tests are testing complement levels. Mainly C3a, C4a and C5a. Elevated hsCRP is also a test that can indicate elevated complement activity. My total complement levels (CH50) and C4 levels were normal and my hsCRP was very low. This indicated anaphlatoxins dont play a significant role in my MCAS. hsCRP is not elevated in IgE allergic reactions (and is actually very low in these cases) and I found plenty of IgE-specific antibodies for the foods I tested. So role of IgE is further cemented as part of my MCAS pathophysio but now no idea what the other part of the pathophsyio of my allergies/MCAS operates under.

I'm going to directly test C3a and C5a, as they are most important part in MC activation, in the near future so I can have absolute certainty on whether or not this theory of mine truly applies in my case.


***

Part 2...



It was interesting to read your personal views on your life and future but I don't want to comment on this part since this is not a Member's Only thread and am going to stick with the medical stuff.

It's all good. Is it because of privacy?

Thank you and I reached a point of significant improvement which the Rituximab is now maintaining (without IVIG since my last IVIG was one full year ago). But I am not sure if I am still having additional improvements or staying at this same level. It is so hard for me to judge and also the level of neck pain that I experience when I push it further really holds me back and I wish I could find something to help this.

Do you know what the exact cause of the neck pain is? I would listen to your body and not continue to push if your body is feeling worse when you extent time b/w doses.

That makes sense (that it allowed you to tolerate the Adderall which allowed you to do all of your exams and work). Or at least I think that is what you meant?

Yep that is what I mean. Rituxan and Xolair together made that happen.

I answered this in your other thread but let me know if I missed anything and I will reply further.

Nope youre good, I understand.

Do you know what your doctor meant re: "limited autonomic neuropathy"?

Probably just damage/disease to autonomic nerves as I also have peripheral neuropthy as a consequence of my MCAS where I can easily damage my nerves depending on how reactive my MCAS is,



(Edit: fixed some grammar and tried to explain some things a little better)

 

[Gingergrrl]

--Plan: Now that I'm back on the 2 month Rituxan schedule I believe improvements should to start come back.

Yep that will be the true test. Mine has remained at essentially zero every time I tested it.

That is interesting that the benefits of Rituximab only seem to last for two months for you but not for three months (even though your B-cells remain at zero). Do you know why? My last Ritux infusion was at a 5-month interval but if I continue to do well, my next one will be at a 6-month interval. Although my doctor suspects, even at the 6-month interval, my B-cells will remain at zero which is the true test.

Sounds like you still avoid sunlight despite your MCAS being in remission, are you still avoiding your old allergies? I know youre avoiding that food dye (I believe it was) that gave you the worst anaphylaxis

I don't do well with heat and sun (I assume both from POTS and MCAS) but I no longer get the immediate skin rash from being in the sun. If I am going to be in the sun for a prolonged event, I wear a big sun hat and sunglasses. I'm also at risk of melanoma (b/c my dad recently had it) so I try to avoid being in the sun in general if I have a choice!

Angiodema in the gut = bloating. What I have is likely more second spacing rather than third spacing, but it may very well be that. I dont quite know how to differentiate them.

My doctor referred to my situation as "third spacing" but I really don't know how you would differentiate. I had not heard the term "second spacing" before.

It's all good. Is it because of privacy?

Yes and you were talking about some really personal info and this is not a member's only thread so I want to respect your privacy.

Do you know what the exact cause of the neck pain is? I would listen to your body and not continue to push if your body is feeling worse when you extent time b/w doses.

I do know the original cause which was a combination of a very bad car accident (in 2006) in which my car was struck with high force and flipped upside down and then an injury to my triceps tendon (in 2010) from Levaquin in which my shoulder and neck had to completely compensate for my arm. I get excruciating pain on the right side of my neck, right shoulder, and right arm. My former PT said that I have "cervical radiculopathy" which I am sure adds to the problem which I now believe is permanent b/c I have tried everything I can think of with no improvement.

 

[Blake2e]

That is interesting that the benefits of Rituximab only seem to last for two months for you but not for three months (even though your B-cells remain at zero). Do you know why? My last Ritux infusion was at a 5-month interval but if I continue to do well, my next one will be at a 6-month interval. Although my doctor suspects, even at the 6-month interval, my B-cells will remain at zero which is the true test.

Even at my worst days these days, theyre still nowhere near to how it was pre-Rituxan on the daily. But I have noticed a significant drop off in effect of Rituxan after 2 months. Rituxan is immuno-modulating something else in addition to targeting my B cells. I need to sit down and research all the possibilities. I already found a few studies that list and review all the possible immunomodulating effects they found Rituxan can, I need to sit down and really dive into it and start testing each theory with blood tests.

I don't do well with heat and sun (I assume both from POTS and MCAS) but I no longer get the immediate skin rash from being in the sun. If I am going to be in the sun for a prolonged event, I wear a big sun hat and sunglasses. I'm also at risk of melanoma (b/c my dad recently had it) so I try to avoid being in the sun in general if I have a choice!

Oh right, the "ginger" part in your name. Wise to be wary of the sun, ultra-white people (those with red-head genes) need to be vigilant against excessive sun exposure.

My doctor referred to my situation as "third spacing" but I really don't know how you would differentiate. I had not heard the term "second spacing" before.

https://www.pcc.edu/staff/pdf/892/9_07fande_revision.pdf this explains it somewhat well at least conceptually, but this is more just trivia for me and I dont have enough reason to research this more.

Yes and you were talking about some really personal info and this is not a member's only thread so I want to respect your privacy.

Oh, I dont consider that stuff personal. I'm not getting into the specifics that can be traced back to me or others around me.

I do know the original cause which was a combination of a very bad car accident (in 2006) in which my car was struck with high force and flipped upside down and then an injury to my triceps tendon (in 2010) from Levaquin in which my shoulder and neck had to completely compensate for my arm. I get excruciating pain on the right side of my neck, right shoulder, and right arm. My former PT said that I have "cervical radiculopathy" which I am sure adds to the problem which I now believe is permanent b/c I have tried everything I can think of with no improvement.

But you said this gets worse as you stretch out time b/w Rituxan infusions. Any ideas how this could be related to Rituxan?

 

[Blake2e]

Update:

Got Xolair this morning. Noticeable difference as the day progressed. Nootropics all of a sudden working a little better and mind was more easily entering flow state.

Good stuff. I expect Rituxan on a consistent 2 month schedule will be more amazing than anything else I've tried for MCAS.

What I need to do: Figure out what Rituxan is doing to immunomodulate my system in such a way that its effects seem to wane after 2 months.

Once my IgE levels drop off below 50 kU/L (or even closer to 0) and I continue to have issues when I extend time b/w Ritux infusions then I have some thinking to do. My IgE last tested was a little over 300 and comparing my food sensitivity at that level to when my IgE was at or over 900 is a vast world of difference. My bad days these past 6 months are nowhere near as bad as what the daily was like pre-Ritux.

 

[Gingergrrl]

Rituxan is immuno-modulating something else in addition to targeting my B cells.

That is interesting and when you figure it out, I would love to hear more! In my case, I suspect that Ritux is working in the traditional sense of keeping B-cells at zero so autoantibodies are lower.

Oh right, the "ginger" part in your name. Wise to be wary of the sun, ultra-white people (those with red-head genes) need to be vigilant against excessive sun exposure.

I don't have red hair and just react badly to the sun & heat for other reasons.

But you said this gets worse as you stretch out time b/w Rituxan infusions. Any ideas how this could be related to Rituxan?

Sorry, what I wrote was probably confusing and the neck pain (on the right side of my neck) that shoots down my right arm is untouched by Rituximab (and is a totally separate issue IMO). All of my other issues have improved (to different levels) except for this issue which is either purely structural or due to another cause that is not autoimmune.

 

[Blake2e]

General Update


In the 2-3 weeks following my previous Rituxan infusion, I was cold turkey off all corticosteroids. But after those few weeks I had to get back on them. What I did notice is that Xolair, when it works, has been resetting my allergies and the accompanying drug resistance to said allergens much more effectively in the past 4-6 months. So, the steroids are actually working for me pretty decently right now though I still need to cycle them every 7 or so days.


Weight has increased from 130 lbs at 9% body fat to 144 with 12% body fat. Gained 14 lbs in about 4-6 weeks, about 5.5 lbs of fat ☹ and 8.5 lbs of muscle which is pretty sweet. I think Im already pretty close to the exercising-weight I used to have, though strength-wise still pretty weak but getting stronger at a decent rate. At my lowest point with MCAS I was at 109 with 4% body fat, I shudder at the memory. So great improvement especially in such a short timeframe. Muscle memory is a great thing. Abs are slowly but surely coming in. Even with clothes on (even with a sweater) and not doing anything to stick out, I’m noticing I’m being eyed by girls and even guys more frequently; being blatantly checked out. Fun stuff. I’m working out 2-3 days a week, usually after work when I’ve taken Phenibut, for 20-30 minutes each session. Need to make sure I keep body fat b/w 8-12% range. (Edit: just about at my past exercising/bodybuilding-weight, but b.f. is too high. At my best physique, b.f. was at 7-8%, and muscles were more developed than currently. So not quite at my aesthetic as I used be. But no worries I'll get there and improve on it further.)


Next Rituxan infusion is this week (on the 2-month schedule right now). Really hoping this helps further extends the time I can go without steroids. When I was previously on the 2 month schedule, I was cold turkey off steroids for about 1.5 months without any apparent issues, so I’m hoping this will be the case again.

 

[Blake2e]

Update:

Really rough past week, after a steady decline in the 2 weeks prior to this week. Barely got any sleep, probably about 3 hours total this past week. Eating less than half than of what I was normally eating, noticeably skinnier after just a week of that, still not too bad though, probably down just 5 lbs. Good amount of muscle I just gained back came off.

Just got Rituxan the other day. Due to insurance issues, had to pay $8300 out of pocket and now I'm flat broke and my savings I was keeping for investing is gone. Might be getting reimbursed for it soon after Genentech sends my docs office a replacement Rituxan. Feeling very different already, very slight better still very significant. All my other meds are working a little better already.

Rough stuff. Symptoms made professional/work life really tough. Had a hard time hiding the stress one of my jobs was causing and ended up parting ways with them. So only working 1 day a week now, instead of the 3 I was previously doing. Had job a job interview today and the sudden shift in how my symptoms improved after the Rituxan was too sudden and I wasnt mentally prepared for that change and came off too nervous and fidgety and didnt get job offer from today's interview. A bit disappointing since what they were offering was so good. More interviews coming up so its all good.

Going to follow up with hem-onc in 2 weeks and see what he says about adding another med for my MCAS and secondary POTS. He seemed very concerned when I saw him before getting the Rituxan since I wasnt in too good of a condition and that I had to go back on the steroids. So we'll see what he says about that.

Overall allergy sensitivity I would say is down to about 20% of what it used to be, but its still a huge nuisance.

 

[Gingergrrl]

In the 2-3 weeks following my previous Rituxan infusion, I was cold turkey off all corticosteroids. But after those few weeks I had to get back on them.

Is there a reason that you are going off the steroids cold turkey and then having to start and stop them? I am asking b/c I am still doing my Cortef taper (under doctors supervision) and by the time I am completely done, it will literally have taken me about two years. I realize this is extreme and I doubt it would take most people as long as me, but can you do a slower taper instead of stopping and starting cold turkey?

Really rough past week, after a steady decline in the 2 weeks prior to this week.

I'm sorry to hear this

Just got Rituxan the other day. Due to insurance issues, had to pay $8300 out of pocket and now I'm flat broke and my savings I was keeping for investing is gone. Might be getting reimbursed for it soon after Genentech sends my docs office a replacement Rituxan.

Wow, that is horrible and I am sorry your insurance denied you. So far, I have had every infusion covered and my co-pay with my current insurance for each infusion is only around $200. I am only doing Rituximab 2x/year now (with next one ideally the first week of Nov) and my current insurance has had no issues. But if they do, I will apply to Genentech for financial aid. Is that what you did?

Going to follow up with hem-onc in 2 weeks and see what he says about adding another med for my MCAS and secondary POTS. He seemed very concerned when I saw him before getting the Rituxan since I wasnt in too good of a condition and that I had to go back on the steroids. So we'll see what he says about that.

Can you talk to him about doing a slower taper with the steroids?

Overall allergy sensitivity I would say is down to about 20% of what it used to be, but its still a huge nuisance.

Can you remind me if you have tried Atarax?

 

[Blake2e]

Is there a reason that you are going off the steroids cold turkey and then having to start and stop them? I am asking b/c I am still doing my Cortef taper (under doctors supervision) and by the time I am completely done, it will literally have taken me about two years. I realize this is extreme and I doubt it would take most people as long as me, but can you do a slower taper instead of stopping and starting cold turkey?

Tapering doesnt seem to work for me since I have quite a bit of drug resistance. So tapering is no different then just dropping it completely. Doctors dont advise I do that but I do it anyway since it doesnt make a difference. I dont really notice much of difference dropping them cold turkey. I usually drop it when my allergy symptoms are mild so I can give them a break so Xolair can better reset my allergy/resistance to them so theyll work better when I really need them again. But when my allergies are more mild I do feel extra fatigue and Im not entirely sure if the lack of steroids compounds that feeling or not. But when my symptoms are mild I take other drugs (OTC) and that fixes the energy problem pretty easily for me.

I'm sorry to hear this

Hoping Rituxan helps even more after this infusion I just got, and I'm getting Xolair in 2-3 weeks after my new insurance kicks in and get prior authorization done, so that should also help me.

Wow, that is horrible and I am sorry your insurance denied you. So far, I have had every infusion covered and my co-pay with my current insurance for each infusion is only around $200. I am only doing Rituximab 2x/year now (with next one ideally the first week of Nov) and my current insurance has had no issues. But if they do, I will apply to Genentech for financial aid. Is that what you did?

B/c I switched insurance providers and applied too late, I am for this current month without any coverage. And my hem-oncs office makes you pay upfront for the cost if you dont have active isnurance until genentech covers the cost of the drug then I'll get reimbursed if everything continues going smoothly.

Can you talk to him about doing a slower taper with the steroids?

They want me to do a slower taper, but I drop it off entirely on my own since tapering it vs dropping it completely doesnt make much of a difference to how I feel due to my drug resistance.

Can you remind me if you have tried Atarax?

I havent, but I dont see why I would respond to that any differently than I do any other gen 1 H1 antagonist or even any other drug Ive tried before. I'll just gain a resistance to it after a few uses. The only things thatll help with that resistance are steroids, Xolair and Rituxan.

 

[Blake2e]

Pretty confident, at least 90%, that the other main mechanism, aside from IgE, thats active in my MCAS is T-cell mediated mast cell activation.

Attached a few studies that helped me understand a little better. I had previously ruled this out but looking over my records and calendar I noticed my CD4 was only down when I was taking Hydroxychloroquine. Going through every other possible mechanism of MCAS, and while many do apply to me, Ive ruled out many as possible causes for me to be reacting to every single thing that enters my body. this conceptually fits the nature of my symptoms quite well and will do some further testing for the sake of confirmation Likely more cytokine testing. But by process of elimination this is all that remains. Hyper activity of Th2 cells.

Very tricky on how to handle this, as suppression of this is the equicalent to HIV/AIDS. But obvious solution is obvious, target mast cells. Asthey are the key to this powerful loop of T cells and mast cells activating each other, as noted on the attached studies.

I may break down these studies and others to explicate how I arrived to this conclusion in another post, if energy levels and mood lets me. Though if youre fairly familiar with MCAS pathophysio then this all mightve been super obvious to you from the onset and youre probably wondering why it took me so long to see this.

 

[Blake2e]

Update:

Rituxan seems to be working less and less for me just like every other drug Ive ever been on,mainly it T cell modulating aspect of it. If this persists I wouldnt be surprised if my B cells start to pop back up within next 6 months or so.

Things have been quite a struggle for me recently. Just got Xolair #21 today, 2 weeks after my last Rituxan. Before my last Ritux infusion, my MCAS reactivity and MCAS induced drug resistance was getting crazy and none of my drugs were working. But after, things started working again but large doses still needed. Lets see if this infusion helps Xolair work and decrease my reactivity and resistance levels as its often done in the past. I seriously doubt it will. Will be seeing my hem-onc next week, we'll see what happens.

Tofacitinib and it ability to thoroughly suppress Th2 seems very appealing, but getting it prescribed will be a challenge. And even if prescribed, off-label cost approaching 4k will prohibit its use. Maybe might just go for tyrosine kinase inhibitors as theyre much cheaper generally.

Also recently started eating the standard, natural human diet again aka PaleoKeto/Carnivore/ZeroCarb. Shouldn't have just jumped into however, die off + MCAS is too intense. Will be doing that with 1 carb meal a day for 1-2 weeks to more gently ease back into this way of eating.


Side note: the day after that recent Ritux, I went out on a 3 hr mountain hike of moderate difficulty. Just by it downregulating my IgE so much my overall sensitivity is probably teetering from 15 to 30% of what it once was. Somehow I survived. Immediately after that I slept for 17 hours straight, getting up only twice for diarrhea. Immediately after escaping the sunlight, my whole body felt wrecked and burnt out. Unsurprisingly Phenibut sped up my recovery, which took about 3 days to regain some degree of functionality and the urge to constantly sleep becoming more resistible. On the whole, that was a monstrously weaker response to UV compared to how absolutely dreadful it once was. And I realized that if my sensitivity isnt severe enough to trigger tachycardia and my HR is <80 while indoors, then this sort of weak reaction is what I can expect from staying outside on a sunny day.

 

[Blake2e]

Yesterday’s journal entry regarding symptoms:

>>Diarrhea 1 hr after breakfast. Crashed into bed at 6pm. Then vomiting at 7pm, 12am and 4am. And a nosebleed mixed in with the 4am vomit.


Above entry doesn’t mention my million other symptoms, but those were yesterday’s worst. All in all actually not that bad of a night, the secondary POTS when acting up enough makes sleep impossible and it wasn’t so severe last night. But still this is no good. Dropped about 10lbs in past 2 weeks. Hem-onc appt next week.


***


Edit:
I recently left one of my jobs, and had 3 interviews for 2 new jobs in the past 3 days. And just finished last one (1st part of this post was written right before it). Was made on-the-spot offers for both places since they liked me more than all the other candidates they interviewed. Wasn't nervous at all, despite without another job next month would be a financial hell, but the secondary POTS elevating my HR and making me sweat (was also wearing a suit on a hot day) made up for that. My ability to stay composed amidst a hurricane is uncanny.

Though mostly amazed that my normal charisma still came through despite POTS-induced insomnia and barely being able to hold down food this past week as well as all the other MCAS craziness resurging. I experience a lot of love-hate due to MCAS, when I'm normal I can win anyone over with personality (which can be learned by tweaking your energy to the right social frequency and dedicated study and implementation of what you learned) and when I'm feeling too sick I get very serious/intense and I'm like a people-repellent (rarely, if ever actually rude, but just bad/stressful energy).

But aside from health tanking, great week as both job offers pay significantly more than my previous one which was already pretty good.

Edit2:
Correction 3 job offers. Just got a 3rd one over the phone after expressing new interest in a place I said no to a few months ago.

 

[Gingergrrl]

Things have been quite a struggle for me recently. Just got Xolair #21 today, 2 weeks after my last Rituxan. Before my last Ritux infusion, my MCAS reactivity and MCAS induced drug resistance was getting crazy and none of my drugs were working.

Will you be stopping Rituximab or continuing some level of maintenance infusions?

Tofacitinib and it ability to thoroughly suppress Th2 seems very appealing, but getting it prescribed will be a challenge.

I just Googled Tofacitinab (Xeljanz) and it sounds dangerous! I couldn't quite figure out, is it an infusion or oral pill or something else?

>>Diarrhea 1 hr after breakfast. Crashed into bed at 6pm. Then vomiting at 7pm, 12am and 4am. And a nosebleed mixed in with the 4am vomit.

Do you have problems with absorption of meds, supplements/ vitamins, etc? Meaning do you have to take supplements or higher doses of certain things just to maintain a normal levels on blood tests? With most meds, I take tiny micro doses compared to the normal person but now with Armour Thyroid and certain supplements like Vit D, I have to take a much bigger dose but still not maintaining normal level on blood tests. Also with Potassium, B-12, Folate, Iron, etc, I have to supplement to barely maintain a normal level on tests and we think I have a malabsorption issue from MCAS. Do you have this, too?