MCAS and IGG Treatment-The Journey Begins Again.

[Misfit Toy]

I trialled both in the last 8 weeks. Mestinon (pyridostigmine) helped my POTS a little better but spiked my blood pressure to a steady 157/95, while the propranolol helped a little less and lowered my BP to 89/55. So, he's trying a small dose of pyridostigmine with a higher dose of propranolol, to improve my POTS, but keep my BP and heart rate stable.

Learner, was your BP low to being with? Mine is 80/54. I looked up the ingredients to the Mestinon and it has lactose in the pill and glycerin in the liquid. I am nervous. Is it expensive to have it compounded? I get so many meds compounded.

He explained with my BP and pulse so fast, untreated, my heart isn't filling up with blood, so I get hypoperfusuon in my brain - I fell asleep during a 10 minute tilt table test.

I don't know what hypoperfusuon is but it sounds nasty. I was out with the tilt table. I was so at rest. But again, did I really have a TTT? My back was just elevated. I have no idea what I had, but I had things all over and my head and what felt like gobs of vaseline. I went to the grocery store afterwards and people looked at me like I was homeless.

As for the MCAS flare, my ME/CFS specialist has me on Solumedrol the day of, dexamethasone for 4 days after, plus ketotifen, fexofenadine, both compounded, plus Zantac and quercetin. My ND has bumped up my vitamin C, folate, B12, and B5 to help the mast cell chemicals go away faster - all hypoallergenic. And boswellia, curcumin, and help oil to manage the headaches and nausea.

Wow, man...that's a lot. I will run this by my doctor. I will have this twice a week so bloody hell. That is all I can say but maybe with only the 2 grams per week it won't be so bad.

Maitland is away on vacation so she still has not talked to my doctor. Snails pace here. IVIG or IGG always takes a long time at least here.

I am glad things are working out so well for you though. Such good news.

Well it sounds like my POTS is autoimmunity too if she says its coming from Sjogrens, my AI disease. I guess that is what it means.

 

[Misfit Toy]

@Gingergrrl -no worries and take care of yourself. I remember that with Gammaguard. I would be okay and the next day was pain and exhaustion and lots of sleep.

@AndyPandy -thank you my dear Aussie friend. Much love to you and I hope your health is okay or tolerable with all that happened!

 

[Learner1]

Learner, was your BP low to being with? Mine is 80/54. I looked up the ingredients to the Mestinon and it has lactose in the pill and glycerin in the liquid. I am nervous. Is it expensive to have it compounded? I get so many meds compounded.

Yes, I needed it compounded due to the lactose and glycerin. It was about $200 for a 2 month supply. However, I won an appeal with my PBM a couple of years ago, where duecyo my corn, milk, gluten and egg allergies, they understand that medically, I need compounded meds and I'm reimbursed at the generic price of the equivalent.

I have hyper POTS with higher heart rate and BP to begin with, so increasing my BP isn't good, but may help you.

I don't know what hypoperfusuon is but it sounds nasty. I was out with the tilt table. I was so at rest. But again, did I really have a TTT? My back was just elevated. I have no idea what I had, but I had things all over and my head and what felt like gobs of vaseline. I went to the grocery store afterwards and people looked at me like I was homeless.

I was strapped upright to a table at a 70 degree tilt.. The doctor had me describe what I felt, minute by minute, through the test. I was uncomfortable the entire time, and in thevlsdt 2 minutes, went to sleep from the effort of maintaining that position. He said that was due to the hypoperfusion.

I also did a weird test with sweat collectors on my arms and legs with electrical stimulation.

Wow, man...that's a lot. I will run this by my doctor. I will have this twice a week so bloody hell. That is all I can say but maybe with only the 2 grams per week it won't be so bad.

I don't like any of these drugs, but they get me through it, and its only 5 days out if every 3 week cycle. I couldn't imagine doing it to me twice a week - I'd have to be on the support meds all the time, plus sticking myself in the abdomen. But, you need to do what works best for you.

Maitland is away on vacation so she still has not talked to my doctor. Snails pace here. IVIG or IGG always takes a long time at least here.

I am glad things are working out so well for you though. Such good news.

Yes, its been unpleasant at times and a bit of a roller coaster, but my 2 good weeks have been getting better and better - I don't collapse in the grocery line, in parking lots or while standing andcbrirfly talking to people anymore, so I am more functional while the IVIG works on the autoimmunity. The propranolol and pyridostigmine bring the symptoms to.more normal, though I still have trouble with intensity of movement and I need to constantly monitor and manage my BP.

Well it sounds like my POTS is autoimmunity too if she says its coming from Sjogrens, my AI disease. I guess that is what it means.

It probably is. The CellTrend test could show it. I encourage you to read the case study again - its very close to what I've been doing, and the lady was very sick and got better. I think the key is getting the right support drugs in place, which for me means compounding everything...and even so, I'm still MCAS reactive - yesterday, I had fexofenadine, benadryl, Solumedrol, and zantac in my system and still broke out in hives from the adhesive in the dressing the nurse used. But things are definitely improving!

 

[Misfit Toy]

@Learner1 -I am on medicare and medicare part D which means no medications are covered that are compounded.

I am not as hopeful anymore.

I couldn't imagine doing it to me twice a week - I'd have to be on the support meds all the time, plus sticking myself in the abdomen. But, you need to do what works best for you.

This is exactly how I feel. Right above. But since IVIG was so life altering and for weeks...I don't know. This is just a way to see I guess.

I also am allergic to glycerin and lactose. The glycerin is a real issue.

I will read it. TY!

 

[Gingergrrl]

Hi MT, I am home from my 2nd Day of IVIG and back-tracking to reply to your posts from last night. I hope you are feeling better today.

I had to have all kinds of testing including the TTT. Honestly, that was easy. My head was just tilted up. I am not sure if it even was the tilt table test. People describe it like it's awful.

From your description, it does not sound like you had a TTT test. In a TTT, you are lying flat on what is like a narrow bed and they completely strap you to it so you cannot move your arms or legs. Then they tilt you up (usually about 60 degrees) and you are attached to the table in a standing position but you are suspended in the air so your feet are not touching the ground.

It is a horrible feeling and when they tilted me (my test was early 2016 at Stanford), my HR immediately shot up 48 bpm and then it went up a grand total of about 60 bpm. I was gasping for air and had difficulty speaking. It was one of the only times that I thought I might pass out (but I did not). It confirmed "significant POTS" which we already knew. I was supposed to be off the beta blocker for 2 wks prior to the TTT but all doctors felt this was not safe for me so I was only off of it for one dose (that morning dose) and still showed significant POTS. This was all pre IVIG & Ritux and I think I would do much better on a TTT now (but do not know for sure).

She wants me to go on Mestinon for weakness and low BP. it's for another condition; myasthenia gravis. I do not have that. I am nervous and have not started it.

Mestinon is for myasthenia gravis but it is used off-label in many conditions like LEMS, POTS, etc. I tried it in 2014 and had a horrible reaction to only 1/8th of a pill (but that does not mean that you will).

He said to give mestinon a try but that Northera may be a better option for POTS.

I was very close to trying Northera (Droxidopa) but it is still on patent in the US (no generic) and had four dyes in it that I am allergic to and it cannot be compounded. There were some other reasons why we did not feel it was a safe med for me so I never tried it and stuck with Midodrine (which works well for me).

He also wants me to try Medrol in a small amount.

I have never tried Medrol or Prednisone but was wondering how they are different from each other?

Honestly, I just feel hopeless. I feel again like having my thyroid out was the worst thing I could have done to myself and it gives me such a feeling of hopelessness because it's done. There is nothing I can do about it.

I relate to this feeling, especially re: things that I wish I could have done differently with my mom's medical care, but there is nothing we can do without going back in time. I am hoping you will find peace with this issue.

Learner, was your BP low to being with? Mine is 80/54.

I know you were asking Learner but my BP was 80/50 for 2-3 years and NOTHING increased it until I started IVIG and now it is usually 100/70 and I can feel the difference.

I don't know what hypoperfusuon is but it sounds nasty.

Hypoperfusion is when there is not enough blood (perfusion) going to organs (like the heart, lungs, brain, etc).

was out with the tilt table. I was so at rest. But again, did I really have a TTT? My back was just elevated. I have no idea what I had, but I had things all over and my head and what felt like gobs of vaseline.

I am almost wondering if you had an EEG vs. a TTT?

Well it sounds like my POTS is autoimmunity too if she says its coming from Sjogrens, my AI disease. I guess that is what it means.

If your POTS is from Sjogrens, then it is Autoimmune POTS.

@Gingergrrl -no worries and take care of yourself. I remember that with Gammaguard. I would be okay and the next day was pain and exhaustion and lots of sleep.

I have never tried anything but Gamunex but so far this 2-Day cycle which I just finished went well. I usually only take my pre-med (Atarax) before IVIG but yesterday and today I took a second one at the end and I am wondering if this is what made the difference (or if this batch was just more tolerable for me)? I've also had a few times that I thought I was in the clear and then got the horrible headache and immune reaction 24-48 hrs later so you never know.

@Learner1 -I am on medicare and medicare part D which means no medications are covered that are compounded.

I have Medicare and a private secondary insurance but no compounded meds are covered for me whatsoever. Luckily my only two compounded meds are Ketotefin and Atarax and I only take the Atarax as a pre-med for IVIG and only fill the prescription 2-3x/year. The most expensive med I have (from CVS and not even compounded) is Cortef which I plan to eventually taper off of if it takes a year, I will do it.

 

[Learner1]

I've read up on Medicare Part D. Apparently you can appeal the compounded drugs. I'm years away from Medicare, so this will be important for me as I need almost everything compounded.

Q2. The plan has denied my initial request for the prescription and the subsequent appeal to the plan. How do I appeal to MAXIMUS Federal?

Answer:

If you are a properly appointed representative, you may request a standard or expedited reconsideration by filing a signed written request with MAXIMUS Federal Services. You must file the request for reconsideration within 60 calendar days from the date of the notice of the plan’s redetermination, unless the time frame is extended by MAXIMUS Federal for good cause.

A written request may be made using the model Request for Reconsideration form provided by the plan with the redetermination letter. Any other written document may also be submitted as long as it is signed and includes the following:

1. Enrollee’s name;

2. Enrollee’s Medicare Claim Number;

3. Identification of the item for which Reconsideration is requested, e.g., the prescription drug, including dose and quantity;

4. Name of the authorized representative, if applicable, and documentation of valid appointment; and

5. Name of the Part D plan that made the determination.

To request a reconsideration of a determination by a Part D plan to deny coverage of a Part D drug that is non-formulary (i.e. is not on the plan's formulary), the prescriber must determine that all covered Part D drugs on any tier of the formulary for treatment of the same condition would not be as effective for the enrollee as the non-formulary drug, would have adverse effects for the enrollee, or both, and provide a written statement to this effect with the appeal request

Sourced here:

http://www.medicarepartdappeals.com/content/frequently-asked-question

 

[Gingergrrl]

I've read up on Medicare Part D. Apparently you can appeal the compounded drugs. I'm years away from Medicare, so this will be important for me as I need almost everything compounded.

In my case, I actually do not have Medicare Part D and my only prescription coverage is through my secondary insurance (who I don't name publicly) but I have bigger fish to fry with them ...

 

[Learner1]

Oh, I had to fight my PBM to get reimbursed for compounds. They put up a HUGE smokescreen, but then grudgingly admitted that with my allergies and MCAS, I'm truly a patient who needs them. It's a winnable battle. But I understand picking your battles! You go, girl!!

 

[Misfit Toy]

Hey guys- @Learner1 and @Gingergrrl -I am having IGG this Wed. Sub-q. 1 gram. I have a feeling I won't be around much on here. I am so busy with craziness. Lots of stuff going on and that time of month. Not doing too well. Exhausted. I have to go out today and do a lot and then I am going to crash. Tomorrow neurologist appt. So tired.

But, I will be back and try and check on here. Wanted to let you guys know. I am nervous.

I also apologize that I have not been around. I had a horrible MCAS attack last night and was up until 5 am. I hate MCAS more than any other malady.

Okay, bye! Wish me luck. I am sure 1 gram won't be too tough!

 

[Gingergrrl]

Hey guys- @Learner1 and @Gingergrrl -I am having IGG this Wed. Sub-q. 1 gram. I have a feeling I won't be around much on here. I am so busy with craziness.

Don't worry about not being around here and you'll keep us updated when you can. Good luck with the IGG!

 

[Misfit Toy]

Good luck with the IGG!

Thanks, @Gingergrrl. Will let you know how it goes. To 1 gram!

 

[Gingergrrl]

Thanks, @Gingergrrl. Will let you know how it goes. To 1 gram!

A toast to one gram...

 

[Learner1]

Good luck to you!

I hope it goes well on Wednesday for you! I'll be getting IVIG all the way across the country, and will be thinking of you!

 

[Misfit Toy]

Good luck to you!

I hope it goes well on Wednesday for you! I'll be getting IVIG all the way across the country, and will be thinking of you!

Thank you, Learner! I know you will do fine. To us both getting IGG.

I am worried about the sub-q because I am realizing it will take up two days of my week. I have no idea how I will do so this is a concern. Will I be down two days per week? A lot to see.

I have to take Benadryl an hour before, Zantac or Pepcid.

Also, an electrolyte drink. What do you both take? I used to have gatorade with the IVIG, but I think it's so sweet. What is your ideal electrolyte drink?

To Gamunex-C!

 

[Learner1]

Thank you, Learner! I know you will do fine. To us both getting IGG.

Cheers!

I am worried about the sub-q because I am realizing it will take up two days of my week. I have no idea how I will do so this is a concern. Will I be down two days per week? A lot to see.

Probably best to take it one step at a time and see how it goes. I'm fervently hoping you'll have a livable experience...

I have to take Benadryl an hour before, Zantac or Pepcid.

I'm to make sure I'm well hydrated the 2 days before. Then, I take fexofenadine, ketotifen, zantac, benadryl, and dexamethasone as support. They give me Solumedrol instead of dexamethasone the day of and a saline IV chaser...

Also, an electrolyte drink. What do you both take? I used to have gatorade with the IVIG, but I think it's so sweet. What is your ideal electrolyte drink?

Well, the saline helps. Ice cold coconut water would be my second choice. It's plenty sweet - I've gotten out of the habit of sweet things so most things are too sweet.

To Gamunex-C!

Keep us posted!

 

[Misfit Toy]

I have to write this really quick. I go in for IGG today. I am so nervous as I am so sick. I got my period on Saturday and it has been like my whole life has turned upside down from it. Utter exhaustion, pain, etc.

Last night I went to bed and woke up at 5:30 after only a few hours of sleep. Heart pounding, thirsty. Something is up with my adrenals.

So, this is not the day to do IGG but I can't cancel. I feel so betrayed by my body. I am so miserable.

I went to the neurologist yesterday who said I do not have POTS or dysautonomia. I had Ansar testing...?? I did not have the tilt table test and all came back fine. I was so upset because what is causing me to not be able to stand without feeling like my knees will buckle?

She is sending me for the tilt table next. I left, got into my car and started crying.

The last week has been hell, I have barely been able to leave my apartment, etc.

Alright, must go. Chug fluids and start this today. Wish me luck. I am so worried and sick. My feet (peripheral neuropathy) is acting up big time.

 

[Learner1]

Good luck with the IgG! Hope you do ok.

And the tilt table test sounds like a good idea.

 

[Gingergrrl]

I have to write this really quick. I go in for IGG today. I am so nervous as I am so sick.

I am sorry you are feeling so sick and I am hoping that the IGG is helpful.

I went to the neurologist yesterday who said I do not have POTS or dysautonomia. I had Ansar testing...??

I have not had Ansar testing and was diagnosed with POTS based on two TTT's plus informal testing by several doctors. That is great if it turns out that you do not have POTS! Although you may still have other forms of dysautonomia even if you don't have POTS, or your symptoms may be due to something else.

She is sending me for the tilt table next.

I think that is good just to rule it in or out and be certain.

Wish me luck.

Good luck and you will do great.

 

[Misfit Toy]

I am not sure I can do this again. I did it and within minutes of having it I felt dizzy. I don't even know how I drove home. I have been sleeping and my mouth is so dry. I just had diarrhea. I put a call into Maitland. I feel awful and like death.

 

[Gingergrrl]

I am not sure I can do this again. I did it and within minutes of having it I felt dizzy. I don't even know how I drove home. I have been sleeping and my mouth is so dry. I just had diarrhea. I put a call into Maitland. I feel awful and like death.

We cross-posted at the exact same time. So you are done with IGG and back home now? My infusion is 7-8 hours each day (and I would never be able to drive home afterward). You will have a delayed reaction to it (at least I do with high dose IVIG) and this is to be expected.