Log: MCAS with Primary Immunodeficiency

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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?
I dont take any POTS meds. My heart rate goes super high b/c of my MCAS. I was only taking my MCAS meds during the Holter monitor, when the drugs didnt work as well, the Holter monitor did catch my heart rate going over 140.


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.
Yep it would be the MCAS meds I would need to be off of to get the most accurate reading for the TTT and other autonomic tests. If I did have POTS it would be secondary to MCAS.


I forgot, is the issue that your doctors will not prescribe it or that your insurance will not pay for it?
Just cant find anyone that is willing to prescribe and I dont really know why.

I agree and I would avoid Cyclo at all cost (if it were me) b/c it is too dangerous.
Yeah I want to follow with my theories of clearing the plasma cells. That plus Rituxan should do the trick. If doc wants me to I wouldnt mind trying some tyrosine kinase inhibitors.
 

Gingergrrl

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I dont take any POTS meds. My heart rate goes super high b/c of my MCAS. I was only taking my MCAS meds during the Holter monitor, when the drugs didnt work as well, the Holter monitor did catch my heart rate going over 140.
Do you think it is possible that you would also benefit from POTS meds? I can't remember if you have tried any in the past?

Yep it would be the MCAS meds I would need to be off of to get the most accurate reading for the TTT and other autonomic tests. If I did have POTS it would be secondary to MCAS.
If you stop the MCAS meds for the TTT, are you at risk of anaphylaxis? I did not stop any of my MCAS meds when I did my second TTT in early 2016 (which was about five months before starting IVIG). I am wondering, even if you have POTS purely secondary to MCAS, if POTS meds could still be helpful?

Just cant find anyone that is willing to prescribe and I dont really know why.
:bang-head::bang-head::bang-head:

Yeah I want to follow with my theories of clearing the plasma cells. That plus Rituxan should do the trick. If doc wants me to I wouldnt mind trying some tyrosine kinase inhibitors.
I already forgot, which meds are considered tyrosine kinase inhibitors?
 
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Do you think it is possible that you would also benefit from POTS meds? I can't remember if you have tried any in the past?
Ive tried Fludrocortisone and that helped with the tachycardia but not much with the MCAS then it stopped working for me and I became reactive to it.

If you stop the MCAS meds for the TTT, are you at risk of anaphylaxis? I did not stop any of my MCAS meds when I did my second TTT in early 2016 (which was about five months before starting IVIG). I am wondering, even if you have POTS purely secondary to MCAS, if POTS meds could still be helpful?
No, I dont get anaphylaxis. POTS meds would probably be useful but I'll just become allergic to them like what happened to the Fludrocortisone. Better to just tackle the MCAS.


I already forgot, which meds are considered tyrosine kinase inhibitors?
Its also a chemo drug but works more specifically. Its main target are mast cells. Ex: Imatinab
 
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Update: On the last steroid that's commercially available, Betamethasone. After this there are no more steroids I can try, as I am allergic to all of them (Hydrocortisone, Cortisone, Methylprednisolone, Prednisolone, Dexamethasone, Prednisone and Fludrocortisone).

New hem-onc thought my symptoms were psychosomatic, until I reminded him to check out my IgE and histamine levels. Then he took it back. Seems like he has a memory problem, as I told him that in the last appointment and I'm sure my Immunologist told him too when he chatted with him. Hoping he was just having a moment. Getting next Rituxan infusion in 2 weeks and Hem-onc wants me to wait till that day before he'll discuss doing anything more. Right now the Betamethasone, which I've been on for 4 days now, is working ok enough but I really doubt it will continue to for 2 more weeks. Sent him Afrin studies (https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4903110/) again. I guess all I can is badger him when my symptoms start to worsen again.
 

Gingergrrl

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Ive tried Fludrocortisone and that helped with the tachycardia but not much with the MCAS then it stopped working for me and I became reactive to it.
Not sure if this means anything but I also did not tolerate Fludrocortisone (Florinef) at all. It did nothing for my POTS, tachycardia, etc, and only made me feel worse. I tried it for several months at several doses in 2014 and it was useless. It also gave me headaches.

No, I dont get anaphylaxis. POTS meds would probably be useful but I'll just become allergic to them like what happened to the Fludrocortisone. Better to just tackle the MCAS.
I don't know if you have tried compounded meds but am wondering if you are reacting to one of the inactive ingredients, dyes, or fillers, like I was in 2015?

Its also a chemo drug but works more specifically. Its main target are mast cells. Ex: Imatinab
I just Googled this and realized that you are talking about Gleevec. In your situation, I would think that Gleevec would be very relevant and a good choice to try. Are you doctors saying no to Gleevec?

Update: On the last steroid that's commercially available, Betamethasone. After this there are no more steroids I can try, as I am allergic to all of them (Hydrocortisone, Cortisone, Methylprednisolone, Prednisolone, Dexamethasone, Prednisone and Fludrocortisone).
I am sending good wishes that the Betamethasone works for you and you do not become allergic to it.

New hem-onc thought my symptoms were psychosomatic, until I reminded him to check out my IgE and histamine levels. Then he took it back.
This stuff is maddening when doctors do not believe you until you remind them of your test results and then they suddenly have a change in memory :bang-head::bang-head::bang-head:

Getting next Rituxan infusion in 2 weeks and Hem-onc wants me to wait till that day before he'll discuss doing anything more.
Best wishes for your next infusion. I just updated my Ritux thread and now have confirmation that I do not need a Prior Auth from my new insurance and my maintenance infusions will be covered. We are aiming for my next infusion to be the first week of May with the goal to keep stretching out the interval between infusions.
 
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Not sure if this means anything but I also did not tolerate Fludrocortisone (Florinef) at all. It did nothing for my POTS, tachycardia, etc, and only made me feel worse. I tried it for several months at several doses in 2014 and it was useless. It also gave me headaches.
When Fludro worked for me it was great, no noticeable side effects.

I don't know if you have tried compounded meds but am wondering if you are reacting to one of the inactive ingredients, dyes, or fillers, like I was in 2015?
I've tried compounded drugs (most recently the Betamethasone). I react just as much to fillers as I do to the drugs itself.

I just Googled this and realized that you are talking about Gleevec. In your situation, I would think that Gleevec would be very relevant and a good choice to try. Are you doctors saying no to Gleevec?
So far his only objection is side effects and cost. My current symptoms are far worse than most of th epossible side effects. And according to Afrin, MCAS pts dont need as high as dosages as the Masto pts (200mg vs 400mg for masto). As for cost, it is really expensive here in the US but I could get 90 tabs for 100mg for a little less than $700 from Canadian pharmacies. Still expensive but much more affordable.

I am sending good wishes that the Betamethasone works for you and you do not become allergic to it.
Allergy to it is already setting it. Its effectiveness will continue to decrease until I just react to it. This is interesting. With Rituxan I should have no B cells (CD19 and CD27+ shows that) and should therefore not be able to develop anymore allergies. My allergies may be both IgE-dependent and IgE-independent. So it therefore may not be enough to just bring my IgE down.

This stuff is maddening when doctors do not believe you until you remind them of your test results and then they suddenly have a change in memory :bang-head::bang-head::bang-head:
Stress from that revved up my mast cells all night last night and kept waking at night. But I sent him more studies that he asked for. Im gonna keep in contact probably every other day via patient portal.

Best wishes for your next infusion. I just updated my Ritux thread and now have confirmation that I do not need a Prior Auth from my new insurance and my maintenance infusions will be covered. We are aiming for my next infusion to be the first week of May with the goal to keep stretching out the interval between infusions.
Thanks! Thats awesome about the new insurance and stretching out times of infusion. I'll check out your post.
 

Gingergrrl

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When Fludro worked for me it was great, no noticeable side effects.
When the Fludro worked for you, did you feel that it was helping symptoms of POTS or something else?

I've tried compounded drugs (most recently the Betamethasone). I react just as much to fillers as I do to the drugs itself.
That is a bummer and I am sorry to hear that. My first compounded med (in 2015) was Ketotefin since there was no other option in the US and you cannot purchase it at a regular pharmacy.

As for cost, it is really expensive here in the US but I could get 90 tabs for 100mg for a little less than $700 from Canadian pharmacies. Still expensive but much more affordable.
Is Gleevec a pill vs. infusion? For some reason, I had assumed it was an infusion. If it is a pill, can you just purchase a few (vs. 90 tabs) to save money and see how you react to it before buying a bigger quantity?

My allergies may be both IgE-dependent and IgE-independent. So it therefore may not be enough to just bring my IgE down.
Have your allergies been life-long or more recent?

But I sent him more studies that he asked for. Im gonna keep in contact probably every other day via patient portal.
Does he seem open to the studies that you are sending him so far? I have had some doctors who are very open to this and others... not so much :mad:

Thats awesome about the new insurance and stretching out times of infusion. I'll check out your post.
Thank you and it is a huge relief! I am expecting my B-cells to still be at zero when I do the Lymphocyte Subset Panel (probably in early April) but I am almost hoping that they are not b/c then it would mean that this remission is not dependent upon B-cells being at zero and additional Rituximab to keep them there. For now, either way, we plan to keep stretching out the interval and monitoring B-cells and symptoms, with next infusion to be the first week of May.
 
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When the Fludro worked for you, did you feel that it was helping symptoms of POTS or something else?
While I dont think I have POTS, the Fludro did help with the constant tachycardia and sweating.

Is Gleevec a pill vs. infusion? For some reason, I had assumed it was an infusion. If it is a pill, can you just purchase a few (vs. 90 tabs) to save money and see how you react to it before buying a bigger quantity?
There probably is an infusion option but I didnt look into it. The Canadian pharmacies Ive seen seem to have a set number of tablets you can order so it doesnt seem like you can order only a few tabs to try.

Have your allergies been life-long or more recent?
Until about 3 years ago I only had an allergy to dust. Though Ive had chronic fatigue for a long as I could remember (which is why I got into stimulants and nootropics) and Im pretty positive my mast cells were the cause because it all feels the same but worse.

Does he seem open to the studies that you are sending him so far? I have had some doctors who are very open to this and others... not so much :mad:
I asked the new hem-onc to keep in touch with me with what he thinks after I messaged him the studies yesterday and today he actually called me so thats a good sign. He impressed me in the 1st appt by taking such initiative to take on my case when so many turned me down then kinda really stressed out me out the next appt with that psychosomatic stuff. But it seems like hes keeping to his word, that he will do the best he can for me as hes having me do more testing.

Thank you and it is a huge relief! I am expecting my B-cells to still be at zero when I do the Lymphocyte Subset Panel (probably in early April) but I am almost hoping that they are not b/c then it would mean that this remission is not dependent upon B-cells being at zero and additional Rituximab to keep them there. For now, either way, we plan to keep stretching out the interval and monitoring B-cells and symptoms, with next infusion to be the first week of May.
Awesome, cheers to your continued improvement!
 

Gingergrrl

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While I dont think I have POTS, the Fludro did help with the constant tachycardia and sweating.
Since the Fludro helped with the constant tachy and sweating, I am still wondering if you do have POTS (as a co-morbid disorder with MCAS which is extremely common and is what I had... plus a few other things). In my case, the Fludro did not work and made me worse but there are many other POTS meds out there (like beta blockers, Midodrine, Mestinon, etc).

There probably is an infusion option but I didnt look into it. The Canadian pharmacies Ive seen seem to have a set number of tablets you can order so it doesnt seem like you can order only a few tabs to try.
Thx for explaining.

Until about 3 years ago I only had an allergy to dust.
That's interesting and I had been curious if you were allergic to many things growing up or had sub-clinical MCAS symptoms (like I did) prior to the first major episode.

I asked the new hem-onc to keep in touch with me with what he thinks after I messaged him the studies yesterday and today he actually called me so thats a good sign
That is a very good sign if he is reading the articles you are sending him and called you!

Awesome, cheers to your continued improvement!
Thank you!
 
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Since the Fludro helped with the constant tachy and sweating, I am still wondering if you do have POTS (as a co-morbid disorder with MCAS which is extremely common and is what I had... plus a few other things). In my case, the Fludro did not work and made me worse but there are many other POTS meds out there (like beta blockers, Midodrine, Mestinon, etc).
Maybe I'll end up trying some of those depending on what my Neuro says during those tests I'll be having soon.

That's interesting and I had been curious if you were allergic to many things growing up or had sub-clinical MCAS symptoms (like I did) prior to the first major episode.
I believe my MCAS was subclinical since I was at least 5. I believe this was my source of CFS. Up until 3 years ago my mast cells would only flare up with any amount emotional stress/exertion and with moderate physical exertion. My main symptoms were low blood pressure, palor, and terrible fatigue to the point that I slept way too much and spent most of my time on the couch exhausted.

That is a very good sign if he is reading the articles you are sending him and called you!
I'm not entirely sure what to expect. But heres hoping for the best!
 
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Maybe I'll end up trying some of those depending on what my Neuro says during those tests I'll be having soon.
It will definitely be interesting to see what the Neuro says.

I believe my MCAS was subclinical since I was at least 5.
I also feel that I had sub-clinical MCAS for most of my life and it only became acute/severe in early 2015 following toxic mold exposure that my immune system could no longer fight.

I'm not entirely sure what to expect. But heres hoping for the best!
Am sending good thoughts and hoping for the best, too :thumbsup:
 

Learner1

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I'd lost track of this thread for awhile, glad I've found it again and have caught up. I have some random thoughts and questions...

My ME/CFS specialist, after cinsulting with Afrin, has put a few patients on Gleevec and says they've done very well.

For MCAS testing, I've never had high tryptase, mostly high prostaglandin D2 and chromogramin A. Tryptase is typically high only in the middle of a reaction. Theoharides doesn't even recommend it anymore as its so hard to catch.

High dose IVIG is really useful. I'm ptetty sure you've seen the attached - Weinstock used 2g/kg which is a whopping amount. My doctor stsrted me at .75g/kg. I get it done at home by a nursing service with support meds to reduce my reaction and a slower drop speed and extra saline. I get Ativan, Toradol, Benadryl and Solumedrol with Benadryl, dexamethasone, Zofran, boswellia and curcumin afterwards. Can you share the case study and get it prescribed?

I'm allergic to corn, milk and gluten which makes most meds tricky. I've found that compounding them (and watching what they put in for fillers) and using the children's version or an injectible version works best.

You may find a POTS med helps. Pyridostigmine (Mestinon) has been a big help and I went through 4 beta blockers and carvedilol before finding Troprol XL finally slowed down my racing heart.

Now, I have some questions for you two and @ClaireKnowles

I am to begin Rituximab in 2 months, the soonest they have space for me to do it. There was a long list of support meds most of which I'm allergic to, but I have solutions for all except Zyrtec, which I'm to take the day of and a few days after. I'm allergic to the fillers in all OTC antihistamines. The nurse said to skip it, but it doesn't seem like a good idea. I had a bottle of fexefenadine compounded for $400 which is ridiculous and I ran out. I currently take ketotifen and benadryl and Zantac.

Any thoughts on a H1 antihistamine that is milk and corn-free?

I know the infusion is fairly easy, but what changes may I experience after? How worried will I have to be about catching stuff from other people?

Is there a point one gets off of Rituximab and is well? My insurance denied it so I'm concerned about long term...

Any other thoughts or advice?

Thanks!
 

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Any thoughts on a H1 antihistamine that is milk and corn-free?
I can't remember, have you tried Atarax? It was originally my rescue med (b/c I don't tolerate Benadryl well) long before I ever did IVIG or Rituximab. It is an old-school H1 but for me it did not have the toxicity of Benadryl. I got it compounded w/some kind of non-allergic filler in a basic veggie capsule at the time. My compounding pharm could definitely make their meds without milk or corn (I am not allergic to either, but many are, and I know my compounding pharm could do it so I assume others could, too). I also got Ketotefin from the same compounding pharmacy. I could give you their info privately if helpful and I know they can ship their products.

I know the infusion is fairly easy, but what changes may I experience after?
I did not have any side effects from the Ritux and it is SO much easier to tolerate than IVIG! I did a very slow infusion speed for every single Ritux infusion (this is necessary for me with all fluid) vs. the infusion speed guidelines in the packaging insert. I read the entire package insert, and all materials that I could get my hands on, plus the nurses at my infusion center gave me a copy of the instructions that they receive (not sure if your nurse will give you that if you are doing at home vs. infusion center?) but it gave me a full picture of every possible side effect.

How worried will I have to be about catching stuff from other people?
I was initially worried but in my case it never happened. I still have not had a traditional cold, flu, etc, since Jan 2013 and our doctor said this is b/c I am still so autoimmune that my body immediately attacks and destroys any pathogens it encounters. But he said this is a double-edged sword b/c it is still attacking my own body as well.

I was at the hospital every day for 2.5 months when my mom was dying (one year ago) while getting Rituximab and I did not wear a mask. It was the height of flu season at the hospital and every single member of my family (six other people) ended up getting sick (with various things) and I was the only person who did not get sick and I was the one who was technically "immuno-compromised". It is bizarre but I have never caught anything to this day.

The only thing our doc said for sure was not to get any vaccines, or even be exposed to anyone who has had a live vaccine, (like flu shot, shingles vaccine, chicken pox vaccine, etc). I'm sure I have inadvertently been around someone who has had a vaccine (just standing in line at CVS) but if a close friend or family member has had one, I would not be right next to them or have them over to my apt during that time period as an extra precaution.

Is there a point one gets off of Rituximab and is well? My insurance denied it so I'm concerned about long term...
That is the goal (to get off it and maintain your remission). In my case for the maintenance infusions (following the first year), we are now stretching out the interval from 3 mos, to 4 mos, to now 5 mos plus testing B cell levels.

I am not off of it yet and my next infusion is tentatively 5/3. No one knows what happens to me when I stop it (our doctor says this is the "10 million dollar question"). I was very lucky to get insurance approval (w/a huge battle with my old insurance) but it was MUCH easier to get approval than IVIG. My new insurance approved Ritux with no problem (after some initial confusion that we cleared up). If yours denied it, and all appeals are exhausted, can you apply to get it free from Genentech?