DoozlyGirl

Congratulations, jangle. Go get em!

Dana,

You have such an angellic voice and at the same time you nail the raw and haunting notes, which is really really special. I thoroughly enjoyed listening to your videos. You have an unbelievable talent, which I hope you can continue to pursue. I agree with Issie, your tone and range is far better than most of the contestants on any of the talent shows on TV these days.

Issie, thanks for sharing.

Lyn

Rich,

My urine and blood samples were recently sent to Mayo for PGD2 testing by Dr Afrin. Dr Castells at Harvard/Brigham and Women's is also testing for PGF2, another prostaglandin metabolite. FYI: N-Methylhistamine (urine and blood) as well as Anti-Xa Unfractionated Heparin (blood) and tryptase (blood) are the other available mast cell mediator tests.

Lyn

Issie,

Nasalcrom, gastrocrom and ketotifen are mast cell stabilizers and the ketotifen also has H1 properties. I couldn't find a dye free Zantac either, and I stay away from the 75 mg due to the blue dye (Blue #1 if i recall properly). Zantac 150 mg has red iron oxide, and I have yet to find any reference to this dye causing mast cell issues. There is plenty of convincing documentation linking the FD&C numbered dyes to mast cell degranulation.

I too wonder about the long term effects of lowered stomach acid and lowered pH. These H2 and PPI meds are only approved by the FDA for 2 to 6 weeks of treatment time. I will take digestive enzymes when needed and regularly take a good multi-strain probiotic. I also add psyllium to my daily smoothie. I miss each of them if I skip em.

Lyn

I did a quick search and several sites listed only 4 FDA approved H2 blockers(said they are available OTC and prescription) in the US:

• Cimetidine (Tagamet)
  
• Ranitidine (Zantac)
  
• Famotidine (Pepcid)
  
• Nizatidine (Axid)
  

There are likely other available H2s in other countries.

Tagamet not only inhibits DAO production, which is necessary for histamine breakdown in the body, it also inhibits the P450 enzyme pathway in the liver and causes issues with detoxifying a host of other meds, most notably propranolol.

Zantac is said to have been devolped from cimetidine and is much stronger without the negative safety profile. Famoditine is said to have even fewer safety issues or adverse drug reactions in general, but is less potent than ranitidine. I have yet to hear of other mast cell patients taking nizatidine, so not sure if that H2 blocker is an good option, but it would be worth a review for someone who doesn't tolerate the other H2 blockers.

I have read of patients who needed to be put on a mast cell stabilizer, as Ana mentioned, before they could tolerate a H1 or H2.

Good luck finding antihistamines you can tolerate.

Lyn

diamond,

I'm sorry to hear you are reacting to the antihistamines. It may be the active ingredients, but it could be the inactive ingredients, such as the FD&C dyes (colors with a number, such as Yellow #5, Red #40, Blue # 1) which are known mast cell degranulators. These are called azo dyes in the mast cell literature. I go into anaphylaxis from Yellow #5 and #6 dye and to a lesser degree to the other numbered dyes, but seem to tolerate the oxide dyes. These are often written as red oxide or red oxine and also come in yellow and black. Zantac has the red oxide dyes. I have written many times on this topic, so feel free to search my posts and you'll find more details if you think this may be the case for you.

Some are reacting to inactive ingredients, such as lactose or citric acid. And yet others react to a source of the inactive ingredient, such as corn or gluten or soy.

There are dozens of antihistamines as well as dozens of formulations to try. You can also try to start out slow, take a 1/4 or 1/2 of the pill for a week and if goes well slowly increase the dose. Once you found a H1 that works, add in a partial dose of H2. Many of us have to take antihistamines 2-4 times a day. I always take a H1 at the same time as a H2, as I've read it works best in combination. Some even have to have their meds compounded without any fillers.

For example, I tolerate the following H1's:

• Zyrtec tablets and liquid gels as well as CVS cetirazine (generic Zyrtec),
  
• Dye free brand Benadryl liquid gels and Walgreens dye free diphenhydramine (generic Benedryl) liquid gels
  
• Brand Allegra and Target fexofenadine (generic Allegra) - oxine dyes only
  
• Prescription Hydroxyzine tablets (white ones)
  
• Walmart Equate loratidine (generic claritin)
  

And the following H2's:

• Walmart Equate famotidine (generic Pepcid AC)
  
• Brand Zantac - oxine dyes only
  

Good luck with this trial and error method of finding a antihistamines.

Lyn

I too can relate. Birth control pills made my migraines worse. Advil and Anaprox (prescription level of Aleve) helps, but irritates my stomach. I have found bioidentical progesterone cream (prescription and compounded by a special compounding pharmacy) to do wonders to regulate my periods. I take one click (20 mg) on day 14-28. When I am off it, my periods suck, but on it they are much more manageable.

Good luck,

Lyn

If you have a connecting flight, you can arrange for a wheelchair escort to get you around the airport. I flew Delta last month and I am so glad I arranged for the wheelchair. That was a first for me and I thought I'd feel funny about it, but it was fine. This way I didn't have to rush to my next gate, and i could save my energy for other things. The wheelchair escorts even offered to stop to a restroom or store if needed. I was also surprised that the gate agents moved my seat on each of my travel legs, moving me closer to the front of the plane, into preferred seating rows and I didn't have to pay extra. I didn't have to ask for this.

I also wore compression garments (spanks), drank lots of water, and had salty snacks on hand, which helps keep my BP up. When traveling, I always wear slip on shoes so I can clear security faster and slip them off during the flight if needed, as well as wear layers in case my temperature varies throughout the flight. I kept my meds and other aids in a carry on I kept with me at all times. I choose the window seat, so I can lean into the window to sleep.

Safe travels,

Lyn

j,

Those symptoms could very well be mast cell degranulation related. How does she do with claritin/loratidine?

Lyn

Yes. Most people with MCAS have a "normal" tryptase. Which tests have been run? Where were they performed? Was the urine and blood chilled immediately and kept on ice even during transport?

Which H1 antihistamine did she take? And which formulation? How/what did it make her feel? I haven't read about the body needing time to adjust to the antihistamines with mast cell patients, unlike other meds. But it could be other factors causing her issues. It is quite possible she is reacting to something other than active medication.

Many of us are extremely sensitive to medications and fillers or dyes are a big issues for many of us. The azo dyes (generic chemical name for FD&C dyes) asre known mast cell degranulators. I can't tolerate any of the FD&C numbered dyes, especially the red and yellow ones. Beware that even white pills (ie generic zyrtec have FD&C Blue #1) may have these dyes. Others have issues with artificial flavorings or artificial sweetners in the readitabs or liquid versions. Others react to the source of the ingredient, such as corn, gluten, lactose, etc. Once you find a formulation that works, don't automatically assume any formulation by any manufacturer works, because these fillers/dyes change in every generic and are different among manufacturers.

It has been trial and error for me to find the brands/generics that work. Many of us also ned to taper up the dose from say a 1/4 to 1/2 of the dose. When I first began taking my H1 (generic zyrtec that contained Blue #1), I also was taking verapamil (Yellow # 5) and followed the docs recommendation (non mast cell specialist) and took 3 in my first day. Big mistake! I was a mess, and landed in bed for 3 days with major OH symptoms and flushing, signs of serious mast cell reaction. I stopped taking the meds, and returned to my baseline. Neurologist figured out verapamil was an issue and d/c it. On my second attempt, I started with brand zyrtec (no dyes) and began with 1/2 pill, then took one pill a day the first week and did ok, added the second pill the next week and added the third pill the third week to get to 3 X day. I then added generic pepcid (no dyes) the next week. I have since found that CVS generic zyrtec is dye free and can take that. And I have replaced several doses of my generic pepcid with brand zantac (150 mg - red oxide, these oxide dyes seem ok for me), especially before bed and first thing in the morning due to heartburn.

Also which H2 is she planning on taking?

I have learned to be cautious and read every label before taking anything. I hope my experiences can help your daughter. Best wishes in figuring it all out.

Lyn

Julie,

Congratulastions on finding a piece of your health puzzle. It is wonderful when one of us can move our chess pieces forward!

There is tons of great information on this site about mast cell disorders. I have recently been worked up for mast cell activation syndrome (MCAS) and two sites in particular were very helpful for me in the beginning. There is a mast cell disorders forum and The Mastocytosis Society, which includes information on mast cell disorders, including activation disorders.

To add to Christy's post, a tryptase level below 20 will typically rule out mastocytosis for most people. But a tryptase above 1 can point towards chronic mast cell activation. Mast cell activation is the term used to describe the process when mast cells degranulate and dump or leak their granules which contain histamine, heparin, tryptase, prostaglandins, and over 200 other mediators. The diagnosis is made most commonly by a unique constellation of symptoms and laboratory tests showing elevated mediators. Unfortunately there are only a few mediator tests available, but the most common ones are N Methylhistamine (urine and blood), PGD2 or PGF2 (2 different prostaglandin mediators found in blood or urine), heparin (blood). A biopsy sample stained with special stains can also identify misbehavin' mast cells. These tests are very sensitive to temperature or staining technique and are best to be performed by a mast cell specialist and at their laboratory. Most people with MCAS do not have true allergies (mediated by an IgE event) but still trigger similar reactions. But true allergies can coexist with MCAS. These reactions do include symptoms that POTS and other autonomic dysfunctions can cause. Many of the medications used to treat us are also known mast cell degranulators, such as beta and alpha blockers, pain medications such as codeine and morphine based meds, anesthestics, contrast media, etc.

My typical reactions start with flushing, a headache and orthostatic hypotension, many times with an really high sitting BP, which bottoms out when standing and I sometimes get an elevated HR. They get more severe when I start to vomit and I will get chest congestion/tightness, major lower extremity swelling, and angioedema. Histamine causes increased vascular permeability and the liquid portion of blood can escape into the tissues leading to hypovolemia. When I have a really scary reaction, my HR does not elevate at all upon standing, a sign of relative bradycardia. By this time I know to remain laying down and elevate my feet. I am in a full blown anaphylactic reaction and require mast cell medications to stop the degranulaiton before cardiovascular collapse. Since there are so many mediators, there are literally thousands of presentations. My mast cell specialist has seen over 300 MCAS patients and the first 70 patients all had a unique presentation.

During a mast cell flare, any number of symptoms can occur. If the mediators are released quickly, an acute episode occurs. These patients call themselves "shockers". I am a "leaker" where my episodes can take hours to progress, but both can lead to anaphylaxis. There is an autoimmune component to MCAS. If your symptoms subside when you take an H1/H2 combo, then this is telling that your may have a mast cell disorder. There are also many other medications to help stabilize these mast cells from misbehavin' and triggering. And then there are medications to block the mediators from their receptors and hopefully stop the chain reaction.

If any of this sounds familair, then I suggest you check into those sites I mentioned above. I didn't see the connection at first, but several mast cell opatients on this site convinced me to check it out. And I am eternally grateful for their input and prodding. This is why I continue to post about my experiences with mast cell on this site, in case others will see a connection.

Best wishes in connecting the dots.

Lyn

question? does it have to be doctors or can patients submit for "who" or "icd" or whoever to get pots as a diagnostic code. could we have our state representatives help us with this orwhat. cause really if there was a code, it would make it better for our doctors, pharmacists, insurance, and for the court with disability and for ourselves. i definitely think that if this is something we could help happen, there are a lot of us that would be willing to do something to help get us a diagnostic code.

Great question. I am not familiar with setting up disease codes, but am familiar with establishing procedure codes. Procedure codes for a new medical procedure are designated by the AMA and approved/established by CMS (Centers for Medicare and Medicaid Services). I wonder how disease codes are established?

I use 337.9 for everything.

There is also no specific code for Small Fiber Neuopathy, Mast Cell Disorder, Chiari 1 Malformation, or a lot of specific Connective Tissue Disorders.

hhhmmmm everything I have doesn't seem to exist in medical land.... LOL

Can I join the Island???

You should see the list of dx codes my docs put on my work-ups they cover everything since specifically nothing has a code, there is usually around a dozen codes listed.

Charmed,

Last week, my GP and I found a code for "systemic mast cell disorder" which is the closest we could find for MCAS.

Lyn

Nina,

There is a mast cell disorders forum online that has great dietary information. Most mast cell patients follow the histamine diet on the chronic urticaria website, which is also listed on the mast cell disorder forum. You can find both sites with online search.

Diamond,

PPIs will decrease stomach acid production, but they do not block histamine. Recommended OTC H2s for MCAD treatment include zantac (randitidine) and pepcid (famitodine), as well as several prescription H2s. Mast cell experts recommend taking a H1 and H2 simultaneuosly as they work better blocking a mast cell reaction in combo. Most people take their MCAD meds anywhere from two to four times a day for scheduled meds and breakthrough meds when needed. Some people with excessive stomach acid wil take a PPI in addition to their histamine blockers.

Have you checked out a low histamine diet? I know that I am much better able to limit my reactions when I limit my triggers, especially food triggers and chemicals/food dyes.

Lyn

Maiysa was just diagnosed with MCAS by an allergist in the SW. PM her in case she doesn't see this.

Naomi,

I am currently take a H1/H2 combo 3 to 6 times a day as well as singulair 10 mg up to 2 times a day, and hydroxyzine 20 mg/zantac each night before going to bed. My H1/H2 combo is usually Zyrtec 10 mg and Zantac 150 mg, but I have been known to take fexofenadine (generic Allegra) 180 mg and famotidine (generic pepcid) 20 mg during the mid day just to mix it up and use up these pills. I'll take a 325 mg apirin if the H1/H2/singulair doesn't break my disabling headache and dizziness and 5 times now the headache goes away within 10 minutes. I also take dye free diphenhydaramine for significant reactions.

While it has taken a months to sort out the formulations I can tolerate (can't tolerate Yellow #5 or #6, Red #40 or Blue #1) so many of the store generics are out, unless they have iron oxide dyes, which I seem to tolerate. I am still awaiting results from the labwork from Dr Afrin's appointment and should be hearing soon which meds he suggests. Meanwhile, he told me to hit the antihistamines HARD. And yes, I am MUCH better on these meds than without them. Saw significant improvement with hydroxyzine, the parent of zyrtec, likely because it crosses the blood brain barrier, as well as singulair and asprin, because I likely have increased prostaglandins (based off of clinical picture).

Lyn

Just saw Mary last week and she'll continue to see me. Dr C is only seeing patients as a consultant, making recommendations and others will be regularly seeing autonomic patients. Froedtert still needs to hire another autonomic specialist, since Dr C has such limited clinical hours. Rush is my backup. Right now, I am seeing solid improvement on my new MCAS regimen and have 2 MCAS docs, so that's buying me some time.

Hi Steph,

Crazy we have to meet with way. I've been at this 2.5 years post diagnosis and NOTHING surpises me anymore. I have autonomic neuropathy and crazy orthostatic hypotension, but my HR does not hit the highs, so I didn't meet the criteria for POTS. So in my case, typical POTS meds to lower HR wouldn't work for me. I do rely on fluids and salty snacks when I have orthostatic episodes.

You'd think you could get a decent clear cut diagnosis out of Mayo, but you'll soon learn that often there are differences in approach and knowledge depending upon the specialty. Was your study performed and read out by a cardiologist, general neurologist or autonomic neurologist? If you met with a cardiologist or general neurologist, you could request a second opinion by a autonomic neurologist, and have your results reviewed in consultation.

Best wishes in nailing down your diagnosis.

Lyn

Emma,

I agree with everyone's suggestions. Were you sleeping at the time or just about to fall asleep? During the time I wore an event monitor for 4 days, my HR dropped to 27 while I was sleeping. Pretty scary, as I woke up choking and had pushed the alert button on my monitor. That test got me a referral for a sleep study and referral to the sleep neurologist. I was diagnosed with central and obstructive sleep apnea and a APAP (automatic CPAP machine) was ordered. APAP really helped me for nearly 2 years.

You may want to ask for a event monitor which can capture any drops over several nights.

Best wishes,

Lyn

Ohhh I forgot something....

Since antihistamines (both H1 and H2) work so well at eliminating some symptoms, then you may want to keep pursuing mast cell activation as a cause of your POTS.

Thanks for posting such a detailed description of your experience at Mayo in Arizona. Sounds like you had a thorough comprehensive evaluation.

I wanted to point out that a tryptase of 2.2 will rule out systemic mastocytosis, but mast cell activation syndrome (MCAS) is still on the table for you. I have been chasing down that diagnosis for months and just met with Dr Afrin, a top MCAS specialist at Medical Univeristy of SC two weeks ago.

Dr Afrin and Dr Castells, from Brigham and Women's in Boston, the other top MCAS specialist feels it is to risky to have patients stop their mast cell meds for testing. I am so glad you did not have worse symptoms, as the travel, change in weather, eating restaurant foods, stress of testing and anxiety of meeting new docs are often huge triggers for mast cell patients.

Patients with EDS and POTS (especially H POTS) and increasingly finding that treating chronic mast cell activation is a huge part in managing their EDS and POTS symptoms. This triad of disorders is fast becoming linked in various ways thorugh the latest research. Several members from DINET have already been diagnosed with all three disorders. Many with the triad of illnesses anecdotally state they can't make sustainable strides in treatment until adequately treating the mast cell disorder.

If you react negatively to your betablocker, then you may want to consider if it is causing symptoms, as most patients with MCAS can't tolerate beta blockers as well as dozens of other meds, because they are known to degranulate mast cells, meaning they cause histamine release causing varied symptoms depending upon which mediators are released.

Check out the Mast Cell Disorders Forum, if you haven't done so yet.

Best wishes in sorting this all out.

Lyn

Great news! Best wishes and keep us posted!

For those of you who have had odd reactions to IV contrast media, you may want to consider that contrast media is a known mast cell degranulator, meaning it is known to release histamine and other mast cell mediators. People with normal body chemistries may only feel the slight funny taste in their mouths and the sensation they just peed themselves because their bodies can hold their counter regulatory mechansims in check. But those of us with messed up autonomic nervous systems and potentially trigger happy mast cells will undoubtedly have a host of reactions that the average CT tech has not witnessed before.

I don't by the "anxiety due to the test or results theory". Contrast is obviously causing some kind of cascade in us, and the breathing issues may be attributed to decreased cardiovascular output or mast cell/histamine related chest constriction or something else entirely. It would make sense that our varied symptoms after contrast injection is related to our varied alltered body chemistry.

Good luck on the results lemons and Sue.