Mast Cell Activation

I have had serious abdominal pain since childhood, urticarias, cough problems, and other symptoms that match the new international criteria for MCAS. I had to do my own online research to basically self-diagnosis. Over a life-time I have received about a half dozen or more misdiagnoses, including childhood abdominal pain so severe I fainted and had my appendix taken out only to learn it was normal. I had dozens of x-rays of abdomen, and nothing could be found, all negative so lived for another twenty plus years with diagnosis and treatment for a duodenal ulcer, though that never appeared in any x-ray. Then I got diagnosis of IBS, which seems to be correct but researchers now know that IBS and MCAS often are comorbid syndromes. I've been prescribed various medications for IBS none of which did much. Finally a gastroenterologist tested me a new diagnosis Small Intestine Bacterial Overgrowth (SIBO) which came back positive. That explained the IBS symptoms. The gastroenterologist recommended a Xifaxin trial. It is a very expensive drug with no evidence of effectiveness as far as I could find any studies. So I use probiotics and Ultimate Meal green food for every breakfast, and this helps the IBS symptoms. But not the MCAS symptoms. At least I got four boxes of free samples of Xifaxin, which I can use for international travel if I get traveler's 'revenge'. For the newly emerging chronic cough problem I went to an ENT and allergist and all tests came back negative. Four different doctors and specialists could not figure out a diagnosis. A couple years ago I managed to convince my Medicare family practice doc to get me one of the genetic tests for mastocytosis. (My grandfather died of what my father reported was an 'odd' form of leukemia; my guess is it was mastocytosis leukemia.) That came back negative. But that test is not one for MCAS. There seems to be no good test yet available for MCAS. Finally last summer when I was in Canada my allergy-like symptoms got much worse, the cough kept me from going public. I went to the local drop-in family health clinic, and the doctor suggested, as I described my symptoms and research findings that I do two trials - a powdered inhaler and if that failed, a generic of Singulair. The inhaler only caused my chronic cough to get extremely worse. The Singulair reduced the cough symptom about 60% in a few days. I read a 2014 medical research article on MCAS symptoms and positive response to an antileukotriene is now one of the diagnostic criteria for MCAS. I take a low dose daily and it has seemed to continue to help. Osteopathic neck manipulation has also helped with chronic cough reduction, as there seems to also be a structural problem in part may be due to my mild scoliosis.

Here is my own short summary of diagnostic criteria based on my reading of medical research articles, Akin, Valent and Metcalf (2010) revised with Petra et al (2014):
Table II. Criteria for the diagnosis of mast cell activation syndrome (MCAS).
Idiopathic MCAS.
After primary and secondary rule out: no detectable clonal MC, no reactive disease, and no allergen-specific IgE Episodic symptoms consistent with mast cell mediator release affecting two or more organ systems evidenced as follows:
• Skin: flushing, urticaria pigmentosa, pruritus (itching), angioedema, dermatographism; rashes, hives
• Respiratory: wheezing, sore throat, stridor; [cough]
[Idiopathic anaphylaxis, with urticaria (during anaphylaxis, unlike SM) and high IgE.]
• Cardiovascular: chest pain, hypotensive syncope or near syncope, tachycardia
• Gastrointestinal: abdominal pain (cramping, bloating), nausea, vomiting, diarrhea, malabsorption, esophagitis; gastroesophageal reflux
• Naso-ocular: pruritus, nasal stuffiness; conjunctival injection
• Neurologic: headache, memory and concentration difficulties/brain fog, paresthesia, peripheral neuropathy
• Musculoskeletal bone/muscle pain, degenerative disc disease, osteoporosis/osteopenia
• Systemic: anaphylaxis, fatigue, faintness
Documentation of an increase of a validated urinary or serum marker for MC activation: esp. increased tryptase
Response to anti-mediator therapy (decr in frequency or severity or resolution of symptoms: e.g., H1, H2, antileukotriene meds

I have or have had 15 of these symptoms. Response to the antileukotriene seems fairly definitive to me.
Wishing everyone in this thread best of luck in getting good diagnosis and finding optimal medication and diet.

Finding what helps people with mast cell disorders either with mastocytosis or mcas or the newly discovered familial Tryptasemia or Hereditary Alpha Tryptasemia Syndrome, can take a lot of time and trial and error because mast cells release or control chemicals that can cause hundreds of symptoms. And because, as One mastocytosis patient told me, we are each like our own portable chemistry set.

For me the things that helped improve my quality of life most were eliminating triggers, staying hydrated, and finding a daily antihistamines (h1/h2 inhibitor combo) that I could tolerate and help, a leukotirine inhibitor, Which is like Singulair or Zyflo or One other I’ve never tried, and a mast cell calling medication which for me means Ketotifin tho some use Gastrochrom or some version of it.

Gradually raising the dose of Ketotifin till I was at a good for me level (most people don’t see improvement on it till they reach the right dosage for them which for me is 10mg three times a day) it has a 12 house half life so most people take it twice a day, but some of us find more improvement with three times a day. and most I’ve spoken with seem to find improvement with 2 to 6 mg doses.

For me staying hydrated has been elusive. I have reacted to the oral rehydration drinks I have tried. Next on my list is somethint called Nuun if I can find some without citrus. Currently I recieve iv hydration as needed.

I found and eliminated triggers to the best of my ability by keeping a symptom and possible trigger log (I use the app called “mysymptoms” available on both Apple and Android last I checked, and there’s are others like it.

Ummm that is not true. Ketitiphen, crommolyn and a h1 and h2 treat mcas

I am under the impression that all forms of POTS including MCAS have no treatment available for the actual dysfunction. I am treating the symptoms of POTS

Hi Everyone,
I am looking for any help, ideas or support. I feel so defeated. Still NO Diagnosis. I relate to everyone else on the blog that says they feel they are going crazy!! A doctor has even told me that my symptoms are obviously in my head and need to check out psychiatric since my tests are negative….Ok, so here is my story. I am 30 and going on 5 years with dealing with this madness and I am exhausted being my own advocate. I have seen so far up to 12 specialist each in their own specialty to figure out my various symptoms. I have always suffered from severe environmental allergies and asthma, but 5 years ago everything changed. I started getting worst without any obvious reasons. Loosing consciousness after eating, becoming allergic to foods I never been before; drugs as well; brain frog; massive weight gain or lost; body pains (I feel like im 86); face flushing; tongue/face/hands/legs swelling; Gastro issues; persistent fatigue…ect.. you name it I have experienced it all except the typical mastocytosis rash.

I have since had over 12 related ER visits including 4 Idiopathic anaphylaxis reactions; 3 Food related anaphylaxis; 1 insect sting anaphylaxis (then test negative for insect bites both skin and blood). I test negative for all test, allergy, tryptase serum, 24hr urine test, but still struggle everyday with my allergies. I take daily 40mg citrizine; singulair; symbicort nose pray; zantac on/off. Still can’t control these attacks and it is becoming destructive on my life since I no longer go out, lost work, postponed school. I now only eat a low-histamine Paleo white meat only diet and if I dare become lax on my strict diet I pay the consequence dearly.

To complicate things even more, I was diagnosed with a large brain tumor and 3 weeks later had emergency brain surgery 6 months ago. So my doctors at the moment all said that my symptoms have been compounded and now cannot distinguish some of the symptoms thus will not make a clear diagnosis of MCAS without positive tests results. But my symptoms and attacks keep getting worst since surgery.

Last year I was lucky and found a family doctor that believes something is wrong since I presented my medical binder (2inchs thick!!); Records and test after test and research articles for everything I was tested on! I am on my 3rd allergist and he seems to take my case seriously when I see him every 6 months. He mentioned that the Mayo clinic may help us find a diagnosis that deal with difficult cases of MCAS, but I live in Canada and cannot afford airfares and medical test in the US. Is there anyone that knows about funding, or test kits for my doc? Or even the cost of being diagnosed? Any resource? I’ll take any ideas or suggestions and even words of encouragement. ☺

I understand that a diagnosis bring little relief since there is no cure. It would stop the ER docs from thinking I’m the crazy lady, or worst! Delay treatment like I had a few times (cause they never seen or heard case like mine before) when I present with atypical allergic reactions.

In my experiences very few doctors know about MCAS and fewer know how to treat it and less diagnose it. ☹

I’m so sorry for the long story but I feel everyone can relate because cases of MCAS are beyond complicated.

i'm exhausted and feel defeated

I'm from Duluth MN as well. Saw your news tribune article as well. But like you have been struggling with this for years now. I was dealing with an undiagnosed chronic sore throat-cough and moderate digestive issues for a few years prior that I never got checked out....at the time it was a very annoying nuisance, I was somewhat miserable, but at least I could function decently enough. It wasn't until after I took something. Just like your cortisone shot, there was a trigger for me. two supplements together, niacin and chromium....took them in the morning, and immediately felt a odd cooling sensation of what felt like water, up my neck, and then down my left arm. Didn't think anything of it at the time. But then like 12 or so hours later, I had the worst psychological feeling I've ever had in my life, just like a sinking death, impending doom, oh my god something is wrong type feeling. Then A few hours after that, wow did things go to absolute hell. Shortness of breath, alternating massive burning sensations and whole body going completely numb, i Couldn't even sleep for the first 2 days or so. Every time I tried and started dosing off, I would get jarred back awake by my body. I thought it was the end. And well, 4 years later kind of wish it was actually considering the life it's given me or lack thereof. Been to emergency room twice since initial onset. Just React to absolutely everything now. There's a mine field of triggers that are just impossible to avoid. And the kicker is that medications-supplements-herbs are one of the worst triggers. How can I get better if I can't tolerate something that's meant to heal me.? I realized less is more when it comes to quality of life.....but it just hasn't been nearly enough. It seems the only way would be to live in a completely sterile bubble in which I wouldn't have to eat or drink anything. And that's obviously not very plausible. Not a lot of hope left. Anyway, I don't know if you'll see this a year or so later. But have you found anybody else in the Duluth area who's dealing with this? And have you been able to improve your health?