Hi Jennifer ,I answered jennifer after her first meaasge elsewhere , that was at the xolair comment .

I said
Re: Idiopathic Anaphylaxis
Reply #27 - Today at 09:54:52 Quote Modify Remove
Hi jennifer Wink

Welcome to our forum Smiley
You are not alone Wink I am in a very similar position but ahve moved a bit further forward than you Wink

Tryptase ;- This is very hard to catch high , only 30% of all anaphylaxis show high tryptase .  So a high is useful but a normal does not mean its not anaphylaxis Wink

There are several causes of angiodeama , not just histamine , and I wander if you have been tested for these . HAE - heridatory angiodeama , autoimmune agiodeama from lupus , thyroid , adrenals , and histamine .

HAE - compliments - C1 , C3 and C4
Lupus - ANA , C2
Thyroid - free T3 T4 and thyroid peroxidase enzymes
adrenals - adrenal antibodies and 9 am cortisol ( off steriods ) don't do this until you ahve other treatments .
histamine - tryptase , 24 hour methyl histamines and prostoglandins .

I ahve idiopathic angiodeama Wink

I have lip , tounge and inner airway swelling , dizziness and fast heart beat , in a serious reaction .
Your doctors are wanting evidence of a typical anaphylaxis , you may never give them it . You are however having angiodeama which is affecting your airway . This is classed as idiopathic anaphylaxis , as per the protocol designed by Dr green burger .

Classification of Idiopathic Anaphylaxis (IA)

(Adopted from Roy Patterson, M.D. textbook on Idiopathic Anaphylaxis)
Disease      Symptom
Generalized (G)      Urticaria or angioedema with bronchospasm (asthma)
Hypotension (reduce blood pressure), syncope
Angioedema (A)      Angioedema with upper airway compromise
(Laryngeal, pharyngeal, tongue)
Frequency of episodes      More than 6 episodes per year: Frequent (F)
Less than 6 episodes per year: Infrequent (I)
Summary of Diagnoses      IA-G-F, IA-A-F, IA-G-I, IA-A-I
Variations of IA Diagnoses
Corticosteroid dependent
IA- (CSD-IA)      Applied when patient is controlled by prednisone but has recurrent episodes of IA below a threshold of prednisone.
Malignant IA (M-IA);      Applied when patient requires high doses of prednisone to control IA. The dose is arbitrarily set at 30mg daily
Or 60mg on alternate days but much higher doses may be required.
IA-Questionable (Q)      Applied for a patient with possible IA where Documentation of objective findings is unsuccessful and diagnosis is uncertain.
IA-Variant (V)      Applied when symptoms of IA vary from classical IA.
IA-V may subsequently be classified as IA-I, IA-G, IA-Q or USIA
Undifferentiated Somatoform IA (USIA)      Symptoms mimic IA but no objective findings are documented and the symptoms are not responsive to the regimen of IA.

For several years steriods have been used as the controling mediaction . More recantly new meds have come out which can stabilise the condition Wink

I was where you are last year . I am now off steriods day to day and going to ed every 3 months ish , as compared to every 3 weeks .

Pimidine has alcohol as its preservative and I couldn't use it as alcohol is a major trigger for me .

I will list my meds so you can get a feel for where I am at now . I think You are under medicated sweets .

Am :-
ranitidine 300mg
ceterizine 10mg
piriton 4 mg ( same as clomitron ) drug :- chorpheneramine mealate
hydrocortisone 10mg ( adrenals not working )
hydroxizine 25mg
oxycodone 15mg
paracatamol 1g ( tylenol )
cyclizine 50mg

Inhaler - symbicort 200/6 - slow acting albutarol class drug  and steriods

Lunch :-
paracatamol 1g
piriton 4mg

Tea
paracetamol 1g
piriton 4mg

8pm :-
oxycodone 15mg
Ranitidine 300mg
singulair 10mg
piriton 4mg
hyroxizine 25mg
cyclizine 50mg

Bed
paracetamol 1g

I was having daily throat tightenings and abdominal symptoms , until singulair and ranitidine was added . I have been on hydroxizine for a month and it has helped my brain fog and cysytis so much .

Its spring , pollen is not good for me . I ahve been in ED , 6 times the first year in march and april and 5 times last yr in march and april all with epi pen level reactions .

So far , cross fingers and everything else , I have not been to ED , YET , and its mid April .

Pollen is a problem to me , but it needs other triggers to make me ED level Wink

In IA / mcas (ill expain next ) , we have a collection of normal mast cells which are behaving badly .

We may have a tryptase rise , but only in a short time frame in a serious reaction . 1-2 hrs post onset of symptoms . So catching it is hard . thats what the other tests are designed to do .

MCAS /MCAD , is a new diagnosis , which is having its diagnostic criteria honed down . It is where people have all the symptoms of mastocytosis but don't fit the criteria . The drugs are the same .

mastocytosis has extra , mishapen mast cells in clumps , in skin and bone marrow , which misbahave and burst with triggers . With so many extra cells , to release tryptase , it is higher in general terms and/or in a reaction . This is called clonal .

There is one exception , mmas , which is monoclonal mast cell activation . This is where there are extra mast cells but not enough in big enough clumps to be mastocytosis . these patients will ahve a normal tryptase

So as you can see there are 2 types of mast cell disease which do not show high tryptase . Most doctors do not know this. I have a paper about this if it will help .

Triggers are common to all of these conditions inc HAE . Pollens are a common trigger . people with mast cell disease can ahve true allergies as well , shown by high IGE in their blood . As a group we must NEVER come off our antihsitamines for testing , it is dangerous .

There is an article in the triggers section by Deb , our forum lead , which will help . Controlling triggers is central to managing this Wink

Mine are sulphites , alcohol , vinegar and anything fermented , stress emotional and physical , infection , hormones , heat .

For the time being , whilst you find a mast cell doctor . There are 3 things to do doll ;-
Suss your triggers and avoid avoid avoid
use the low histamine diet :-
www.urticaria.thunderworksinc.com/pages/lowhistamine.html
keep a diary , of foods , lotions and potions and meds , and your symptoms , i sussed so many of my triggers this way

xolair , will work , if you have a IGE allergy . There are several routes to mast cell activation known and many not and IGE is only one . So it may work , but may not .

Singulair works by blocking leucotrines found in the lungs . I have found this has helped my airway swelling considerably . I was blue all the time when walking , I am much better , only blue if I exert myself , and far less so . It has also helped my general swelling , legs and hands in particular . It works regardless of the cause of swelling Wink

The drug groups used for mast cell angiodeama , are :-
anti histamines :- type 1 drowsy - benadryl (us ) piriton UK ( clomitron ), ketiofen   and non drowsy - ceterizine and allegra .
Type 2 - ranitidine
lecotrine inhibitors :- singulair
mast cell stabilsers :- gastrocrom and ketiofen

I am not on a mast cell stabiliser yet , its my next step .

My doctors are also struggling to diagnose anaphylaxis . I have had 26 Epi pen level reactions. I think yours need to know that anaphylaxis is possible without a tryptase rise . Mine are open to having proof .

When you are swollen , I think an adrenaline nebuliser would be useful . NO alcohol Smiley I have had this when I have not had any systemic symptoms - dizziness , high/ low pulse or high / low blood pressure .

I completely understand where you are at sweetie Wink I have been there with a stridor xxxxxxxxxxxxxxxxxxxxxxxxxxxxxxxxxxxxxxxx

I also have rebounds in the days post a biggie . It takes me a week to recover from each dose of adrenaline .

Ive got so much to chat about Wink Ill stop there for now Wink

Many hugs
Josie

Jennifer answered in detail , which i will answer all here  

Re: Idiopathic Anaphylaxis
Reply #28 - Today at 10:47:31 Quote
I have had every angioedema test.  They are all negative.  

Thyroid test come back negative or within range.  
Lupus is negative.  Did test Positive for ANA but on the very low range. 1:160 smooth and speckled.  But the RNA and Florescent test were negative.
HAE  all C1,C2, and c4 were in range.

Adrenal system is fine ( and they usually check after larger doses of steroids).
And tyrptase was normal.

I have had some elevated levels on my Alkaline Phosphate back in 2010 and on my Sed rate from time to time.

My initial IgE was 55 when I came in, while on my medications, it is still between a 22-27.

My drug list is as follows:

10-40 mg Zyrtec
4 mg chlorpheniramine 1 tablet 4 times a day.
300 mg ranitidine 2xs a day
10 mg singulair at night
50 mg savella 2 times a day ( fibromyalgia)
Gabapentin 100 mg 2 at night and 1 in the morning (nerve damage due to ear infection that wasn't treatable by antibiotics; became allergic to all the ones usable).
Vitamin B in the morning (helps with energy and nerve damage repair)
Flonase 2 puffs once a day
Plumocort turbo inhaler (asthma)

I also have a epinephrine inhaler (Primatine Mist in US) I don't know if you have anything like that in the UK.
Active ingredient (in each inhalation)      Purpose
Epinephrine 0.22 mg.
I suggested this to my Physician and it seems to work but not this last episode. ( had used it three times and the swell was coming back). It has helped on the smaller reactions with my lips.

I have some hydroxyzine as well ( it is mainly for back up or as needed).

-I have also so always had the intestinal swelling, even as a child with the hives.  I always knew they were coming because of the diarrhea and nausea.  And without fail every ester I was drugged up in church and not really enjoying the Easter egg hunts....

-Infections or viruses can trigger episodes in me as well.  Any flu or even a cold ( with this last episode, I think it wouldn't go away even on the high amounts of steroids was b/c I was starting a cold.  I got it about 5 days after the ER visit.)

-I haven't tried the low histamine diet but it is going to hard...sometimes the only thing I can stomach is Iced tea and not having that is going to suck.


-The last episode did have me in the ER and the only strange thing was that my pulse oxygen level never went down to far.  I do have raynaud's as well and I am wondering if that might effect and pulse ox machine and their readings.  My hands were blue and I still had excellent oxygen levels.

So there is a second bit of history....And i moved my original post to the introduction section.....  Smiley

I will answer here on the second half ;-0  

I can see you are a long way down this road . I am glad HAE and autoimmune angiodeama have been excluded

As for your blue hands and pulse ox levels . Blue hands = low circulation , it could be the raunards , but I would be extreemly suspicious of those readings . In my past role as an acute nurse I would have changed you to an ear probe or asked the attending doctor to take an arterial blood gas , as this is the best most direct route of assesing the composition of gasses in the blood and it is a quick analysis in a blood gas machine in the ED .

So .......................................... anaphylaxis wise you have :-
swelling of your mouth , nose and throat
gastric involvement with pain , swelling , diarrhoea and vomiting
asthma

The primatine mist is what i was refering to - an epinepherine inhaler , it has alcohol as its preservative , so It made me worse ;-(

I wanted to just say some bits about some of your drugs

Savella , is a snri , this affects the level of serotonin . Its its not advised to be prescribed with epinepherine as it leads to a high risk of hypertensive crisis and arrythmia ( high or low irregular heart ryhyms ) ( prescribing information from savella .com ) . In general terms , serotonin should be measured in patients with a angiodeama as the cause may be mast cell ( very low / high ) , but can be due to a condition called carciniod syndrome ( very high ) . Have you been tested for this ?  I ( and others ) have found messing with our serotonin level , even if it is very low , can affect our mast cell disease . I was on duloxitine a SSRI and it made me shock more esp with bright flushes ( but this is just my experience ) .

The other class of drug favored for fibromyalgia is tricyclic antidepressants in varying doses . In all doses it interacts with epinepherine increasing risk of arrythmia . This risk is seen as higher in anti depression doses . ( as per BNF UK )

Vitamin B , has the high potential for anaphylaxis . The intravenous form most so .

Your reaction to bee's interests me , as bee stings are known to cause reactions in patients with mastocytosis without true (ige ) allergy . Have you been tested for allergy to bee's in a controlled way or with blood tests ?

I think you defiantely have anaphylaxis and i think you need more testing for mast cell disease despite your stubbornly low tryptase . What is your level ? Does it rise , within the normal limits in a reaction . Mine is 3 . You can have mast cell disease with a negative tryptase .

Where abouts do you live ? As I  really want you to see a specalaist asap as I think your current doctors , even though they are working within their knowledge , have no idea about mastocytosis , anaphylaxis or mast cell disease and are currently putting you at risk

When I am reactive . I notice this a my lower legs going blue with minimal exertion , itching trough my meds in area i will go on to swell in , or blue knees and upper arms on exertion - walking to the loo , I increase my piriton to the full 6 times a day including in the night .

ketiofen is widely used as its an antihistamine and mast cell stabliliser .
I am on hydroxizine twice a day now , 25mg , and it is making a major difference to my symptoms .

You have had a horrid run . I hope through this forum you will find the right doctors I have been where you are and know there is a lot of support here

Jose

Hi Jennifer ,

I am on hyroxizine and ceterizine ( the drug in zyrtac ) .
You need to see a mast cell specialist . Where do you live , as there are specalists and others here , who live in the US will be able to advise you further .

Are you ready to seek specalist opinion . It wasn't until I got to specalists that I got yo a level of meds that are helping me through this spring .

Josie

Hi Joan

Thanks I doubled up my singulair to 10mg am and pm as my legs were going blue with minimal exertion . I discussed it with my GP , the drs book in the UK says 10mg a day , so he will not prescribe any more . i am restricted to this by my number of tablets so , thats at an end for now .
I can increase my ceterizine as I can buy thee xtra doses over the counter .
Allegra , was ok , but all its corn flour had my bowel a mess and a liquid version is not available here . So a bit stumped there as well .

I would love to ahve gastrocrom ( nalcrom here ) but my GP won't prescribe anything unless the speclaist says ;-(

So having to go careful until my appointment , which is in July at the moment . I am on call for any cancelations .

I am hoping to move soon , 3 weeks , after I have done this I will have funds to see the specialist privately if I haven't had an appointment by then . I am also waiting for recordings of my 999 calls and the paperwork. This will prove my anaphylaxis . i want these for the appointment .

I have flushed and vomited tonight . I am just fed up .

hugs
Jose

Hi jennifer ,

Dusting , yep , I understand . In my days before active illness i always damp dusted . My hoover has a hepa filter and I have a care team , part of that involoves domestic duties and dusting for me . My worst combination is cat hair and pollen in house dust , a quick to ER reciepe for me . You are absoloutely not alone

Jose