Medical staff

Hello-If you are here you patient will be carrying this link with them with their EPIPENs

 

This page is on the spot advice for medical staff- Nurses , Doctors, EMT's -who are treating pts with a Mast cell disease in an emergency - Anaphylaxis

Your patient will have diagnosis of or being investigated for

- mastocytosis

- Urticaria pigemntosa

- TEMP

- mast cell activation syndrome

- Monoclonal mast cell activation syndrome

- idiopathic Anaphylaxis - NO known allergen

-Idiopathic angiodeama and Urticaria

Treat as Anapahylaxis

as per hospital protocol

 

-NO Plasma expanders (Dextran)

-Add Ranitidine IV if availble

 

Ask-

Are they taking beta blockers- Drug name ending in OL

(beware of trade names)

In which case use GLUCAGON as first line treatment .

 

ASK -

Are they on Tricycylic antidepressants ?

- Risk of hypertension and arrthmias with EPINEPHERINE

 

 

 

 

 

 

 

The symptoms of anaphylaxis are -

 

Fast heart - then slow

Low blood pressure - from fluid loss from blood into tissues

Lungs filling with fluid

Airways spasming

Throat swelling closed

Stridor

swelling all over the body external and internal

Hives/welts

Flushing -red -nettle type rash described as instant sunburn

Unbearable itching

Diarrhoea

Vomiting

Ultimately collapse death if not treated

 

These patients can present typically or atypically

Common atypical presntations

 

- collpase with no Airway symptoms

- Compensated shock-with hypertension -quickly giving way to hypotension -below 90 systolic

- NO hives

- Tachycardia in excess of 150

- hours or days of symptoms before tipping over into anaphylaxis

- Gastric anaphylaxis

-presenting as acute coronary syndrome

 

HISTAMINE MESSES WITH MEMORY -

This fact will help you in history taking

 

- Factors known to make anaphylaxis more likley inc co-morbid states  

 

- Pre existing asthma

- cardiac diseases

- Infection

- stress esp emotional

- known psychaitric illness

- premenstural week /day

- Disruption of normal routine

- Atopic rhinitis -severe atopic disease

 

Drugs

- beta blockers

- NSAIDS

- ACE inhibitors

- Ethanol ( alochol )

- Recreational drugs

 

High risk groups

- infants-due to being unable to comminacte symptoms

- Adolesants- due to increase in risk taking behaviours

- Preganacy - due to meds for strep b

- Elderly -higher risk from stings

- patients with mast cell conditions - esp with stings from wasps and bees

World allergy oraganisation 2011

Other mechnaisms by which mast cells are triggered include -

SEE TRIGGERS SECTION FOR FULL LIST

 

Triggers differ from IGE allergens in several ways

1) They don't have to be protiens ( many use GPRC recpetors)

 

2) they can be dose dependant or depndant upon how any mechanisms are involved encouraging an anaphylaxtoid reaction in the moment of symptoms becoming serious .

 

3) Can be OK one day at not the next

 

But you patient is your expert . Sometimes on triggers-depending on how long they have been sick - But ALWAYS on thier symptoms .

 

Common first symptoms

- Anger or anxiety for no reason often denied by patient

- Word finding problems / vauge

- Stomach churning

- Voice dropping

 

Indications of worstening condition along side these early symptoms

 

- Wanting to be sat up when already sat up - even if spo2 is within limits  

ige non ige

Differntial Diagnois -

If Carciniod tumors are suspected ( neuroendocrine tumors - Then Glucagon should be used as first line

 

If the patient has Heriditory Angiodeama -traditional treatments for anaphylaxis will not work - see medicines

No -Under any circumstances  

OPITES ,

CT Contrast Iodine based

Protamine / heparin

Aspirin  and nonsteroidal anti-inflammatory drugs (NSAIDs)

Mycin antibiotics

Beta Blockers

Blood products, autoimmune conditions, coexisting infection . other immunoglobulins-IGG,IGA,IDD   -  

Certain intravenous anaesthetic

agents ,  muscle relaxant s (see Medications -anaesthetic guidelines )

Alcohol as preservative

(and in clensing wipes/ gels )

 

 

 

 

 

 

See Symptoms section

 

Number 3- is the immunologic mechanism for anaphylactoid reactions where the term

‘anaphylactoid’ is used to designate reactions that are indistinguishable   -

 

In risk of serious symptoms and death

 

from anaphylaxis  but lack a

demonstrable IgE allergic mechanism

 

30% of all anaphylaxis is Idiopathic -No IGE cause or other is demonstarated

 

Patients can have many Idiopathic reactions.

Processes by wihich NO drugs and some triggers cause anaphlactoid reactions

 

1) Drugs which cause disturbance of arachidonic acid metabolism by cyclo - oxygenase  inhibitors such as [3](See medications )

 

2) a drug that possesses the pharmacologic

property of causing direct, nonimmunologic release of

mast cell mediators.

 

3) By complement activation by immune complexes,-  caused resulting in production of complement - Anapahlatoxins - C3a and C5a ( See triggers and Anapahylaxis )

In the case of seemingly idiopathic reactions other conditions ( see differential diagnosis ( under Mast cell conditions ) will be investigated for .

 

If this patient does not have a diagnosis -

or

has mastocytosis

or

Is Idiopathic

Please take blood for mast cell tryptase within 2 hrs of symptoms beginning ideally but anytime will be useful up to 6 hrs , then a second level at 12 hrs( this being achieved should not impede discharge )  

A group of patients remain Idiopathic and continue to have anaphylaxis

Other known processes by which Mast cells are triggered

 

By Direct action on mast cells

-Excercise - for many patients this can be alow level , even afew steps

-Heat

- Cold

 

Neruro actions on mast cells

- Stress through cortictrophin releasing hormone and substance p

- Also serotonin

 

Alochol - 50% of patients with mast cell conditions tolerate NO alochol .

 

Cytokines

 

Immediate and long term actions of leucotrines and prostoglandins ( See medication and Mast cells )

 

Sulphites -Food preservatives

Through an acid based pathway in the lungs causing bronconstriction and mast cell activation

 

Diesel fumes

 

Infections /viral infection -activate complement independantly and also when antibodies are made

 

Molds activating compliemt through phagocyte action

 

High hitamine / High hisitdine/ aspirin foods

 

See triggers list for full list ( though anything can be a trigger )  

 

Some processes are not yet ellucidated and these are termed secretoguoges

-Cleaning materials , personal care products

 

Triggers can be called pseudo allergies-which isnt used by patients as it doesnt suggest the seriousness of anaphylactoid reaction-You cant be pseudo dead . Also termed intolerences and sensitivities .

 

Idiopathic anaphylaxis or anaphylaxis within mast cell conditions is not the same as intolerences . This patient is not slightly precious and belonging to the main contain nuts brigade - They have alife threatening anaphylaxis .

 

Patients expereincing anaphylaxis will be aware of this and may react negitively to their triggers being described as such .

Other points

 

Patients with mast cell conditions can also have IGE allergies , but not to anaphylaxis . These are at the same rate as the general population .

 

 common triggers

included foods (eg, peanuts, fish, nuts, eggs), drugs

(NSAIDs, aspirin, penicillin, cephalosporins, insulin,

sulfonamides, blood products, vaccines, and enzymes

such as trypsin, chymopapain, and streptokinase),

exercise, and latex.

 

Author as an expmale . I have true allergies to cats, house dust and grass . Normal IGE below0.35 . There are 6 classes up to IGE 35. All 3 allergies total 35 IGE. My total IGE is 188.

 

Despite RAST to 100 allergens, extensive patch testing and selctive skin prick testing No other alleregn has been found .

 

I have had 32 anaphylactoid reactions with in total over 50 doses of epinpeherine . My triggers are numerous and listed in the trigger section .

 

I have investigated for all possible causes inc- mastocytosis, vocal cord dysfunction , carcions syndrome, pheoctyocythoma, myloma (the high IGE ) , lymphoma , been scanned head to toe

 My diagnosis is Idiopathic anaphylaxis with angiodeama .

 

Pts are prone to infections .

 

 

 

Pictures for Reference

Anesthetic pre medication and guidelines

Pre-medication for major and minor procedures and for radiology procedures

with and without dyes:

• Prednisone 50 mg orally (20 mg for children under 12) 24 hours and 1—2 hours prior to

surgery

• Benadry1 (Generic: diphenhydramine) 25-50 mg orally (12.5 mg for children under 12) or

Atarax (Generic: hydroxizine) 25 mg orally, 1 hour prior to surgery

• Zantac (Generic: ranitidine) 150 mg orally (20 mg for children under 12) 1 hour prior to

surgery

• Singulair (Generic: montelukast

10 mg orally (5 mg for children under 12) 1 hour prior to

Surgery

Drugs to be avoided:

• Aspirin and non-steroidal anti-inflammatory medications

• Morphine, codeine derivatives

• Vancomycin

 Some of the Drugs to Avoid may be given if absolutely necessary, if given

with a prep to stabilize mast cells. Please refer to one of our mast cell experts for

instructions.

AVOID THESE DRUGS

General Drugs 􀀁 Alcohol

􀀁 Amphoteracin B

􀀁 anticholinergic drugs

􀀁 Dextran

􀀁 Dextromethoraphan

􀀁 Ethanol

􀀁 Polymyxin B

􀀁 Quinine

􀀁 Vancomycin IV

􀀁 􀀁-adrenergic blockers

􀀁 􀀂-adrenergic blockers

Pain Medications 􀀁 Opioid narcotics (may be

tolerated by

􀀁 some individuals)

􀀁 Toradol

􀀁 Non-steroidal antiinflammatory

drugs (unless the

patient is

􀀁 already taking a drug from this

class)

May be tolerated

􀀁 Fentanyl (may require adjunct

treatment with Zofran)

􀀁 Tramadol

Muscle Relaxants

High risk of anaphylaxis

Not tolerated

less anaphylaxis associated

􀀁 Pancuronium

􀀁 vercuronium

 

High anaphylaxis risk

􀀁 Atracurium

􀀁 Doxacurium

􀀁 D-tubocurarine

􀀁 Metocurine

􀀁 Mivacurium

􀀁 Succinylcholine

 

In between

 

rocuronium

 

Local Anesthetics

Not tolerated

􀀁 Benzocaine

􀀁 Chloroprocaine

􀀁 Procaine

􀀁 Tetracine

 

Tolerated

􀀁 Bupivacaine

􀀁 Lidocaine

􀀁 Mepicacaine

􀀁 Prilocaine

􀀁 Levobupivacaine

􀀁 Ropivacaine

Intraoperative Induction

Meds

tolerated

􀀁 Ketamine

􀀁 Midazolam

􀀁 Propofol

Inhaled Anesthetics tolerated

􀀁 Sevoflurane

Please note: the majority of the information in this quick reference guide was taken from the article by Gould and

Park, and placed into this quick reference guide for ease of use by ER practitioners.

References:

1. DVDs from the TMS Annual Conferences, 2006, 2007, 2008, 2009.

2. What You Should Know About Anesthesia-It Could Save Your Life. Nancy Gould and Regis Park.

www.tmsforacure.org

3. Mastocytosis: Perioperative Considerations. V.A. Goins.

AORN Journal. December 1991: 54(6):1229-1238.

4. Mastocytosis: Current Concepts in Diagnosis and Treatment.

L. Escribano, C. Akin, M. Castells, A. Orfao, DD.Metcalfe, Annals of Hematology(2002); 81 677-690

 

 

 

 

 

 

tolerated

􀀁 􀀁-adrenergic blockers

􀀁 􀀂-adrenergic blockers

􀀃

􀀃

Pain Medications 􀀁

AVOID THESE DRUGS

 

Opioid narcotics (may be

tolerated by

􀀁 some individuals)

􀀁 Toradol

􀀁 Non-steroidal anti-􀀃

inflammatory drugs (unless the

patient is

􀀁 already taking a drug from this

class)

􀀃

 

􀀁 Fentanyl (may require adjunct

treatment with Zofran)

􀀁 Tramadol

􀀃

Muscle Relaxants

􀀃

􀀁 Atracurium

􀀁 Doxacurium

􀀁 D-tubocurarine

􀀁 Metocurine

􀀁 Mivacurium

􀀁 Succinylcholine

􀀃

􀀁 Pancuronium

􀀁 vercuronium

Page 2 of 2

QUICK REFERENCE GUIDE

Copyright 2010. The Mastocytosis Society, Inc. All Rights Reserved.

Valerie M. Slee, Chair, Board of Directors

23 Camelot Dr. Shrewsbury, MA 01545 • Phone: 508-842-3080 • Fax: 508-842-2051 •

E-mail:chairman@tmsforacure.org • Web: www.tmsforacure.org

Local Anesthetics 􀀁 Benzocaine

􀀁 Chloroprocaine

􀀁 Procaine

􀀁 Tetracine

􀀁 Bupivacaine

􀀁 Lidocaine

􀀁 Mepicacaine

􀀁 Prilocaine

􀀁 Levobupivacaine

􀀁 Ropivacaine

Intraoperative Induction

Meds

􀀁 Ketamine

􀀁 Midazolam

􀀁 Propofol

Inhaled Anesthetics 􀀁 Sevoflurane

Please note: the majority of the information in this quick reference guide was taken from the article by Gould and

Park, and placed into this quick reference guide for ease of use by ER practitioners.

References:

1. DVDs from the TMS Annual Conferences, 2006, 2007, 2008, 2009.

2. What You Should Know About Anesthesia-It Could Save Your Life. Nancy Gould and Regis Park.

www.tmsforacure.org

3. Mastocytosis: Perioperative Considerations. V.A. Goins.

AORN Journal. December 1991: 54(6):1229-1238.

4. Mastocytosis: Current Concepts in Diagnosis and Treatment.

L. Escribano, C. Akin, M. Castells, A. Orfao, DD.Metcalfe, Annals of Hematology(2002); 81 677-690.

symptoms new site

Here are short versions of the information on this site

 

Symptoms

 

Triggers

 

Medictions

 

Testing

 

Mastocytosis compact guide

 

 

Anestetics guide

Downloads

Treatment focus Triggers for forum Mastocytosis compact Anesthesiamast

Anesthetic Drug Guidelines

Anesthesiamast

 

Most Up to date Articles

WAO anaphylaxis guidelines_JACI 2011

2011 -Most recient review of Differentail diagnosis, treatments and care of mast cell disorders

J Hematol Oncol. 2011; 4: 10.

Published online 2011 March 22. doi:  10.1186/1756-8722-4-10

PMCID: PMC3069946

Mast cell activation disease: a concise practical guide for diagnostic workup and therapeutic options

 

Gerhard J Molderings,1 Stefan Brettner,2 Jürgen Homann,3 and Lawrence B Afrin4

Author information ► Article notes ► Copyright and License information ►

Go to:

Abstract

Mast cell activation disease comprises disorders characterized by accumulation of genetically altered mast cells and/or abnormal release of these cells' mediators, affecting functions in potentially every organ system, often without causing abnormalities in routine laboratory or radiologic testing. In most cases of mast cell activation disease, diagnosis is possible by relatively non-invasive investigation. Effective therapy often consists simply of antihistamines and mast cell membrane-stabilising compounds supplemented with medications targeted at specific symptoms and complications. Mast cell activation disease is now appreciated to likely be considerably prevalent and thus should be considered routinely in the differential diagnosis of patients with chronic multisystem polymorbidity or patients in whom a definitively diagnosed major illness does not well account for the entirety of the patient's presentation.

 

In English - If you have sysmptoms all over and no one diagnosi expains the all -Then mast cells should be considered ;-) Although systemic masto is rare -This isn't ;-)

IGE independant anaphylaxis and Mast cell mediators

il33-4ige

The link is an original article on early online view - Not yet published

 

Interleukin-33 amplifies IgE synthesis and triggers mast cell degranulation via interleukin-4 in naïve mice†

 

M. Komai-Koma1, F. Brombacher2, P. N. Pushparaj3, B. Arendse2, C. McSharry1, J. Alexander4, R. Chaudhuri1, N. C. Thomson1, A. N. J. McKenzie5, I. McInnes1, F. Y. Liew1,3, D. Xu1,*

Article first published online: 15 JUN 2012

 

DOI: 10.1111/j.1398-9995.2012.02859.x

 

© 2012 John Wiley & Sons A/S

 

Abstract

 

Keywords:

allergen independent;IgE;IL-33;IL-4 dependent;mast cell degranulation

Abstract

Background

The regulation and function of IgE in healthy individuals and in antigen-naïve animals is not well understood. IL-33 administration.increases serum IgE in mice with unknown mechanism. We tested the hypothesis that IL-33 provides an antigen-independent stimulus for IgE production and mast cell degranulation.

 

Methods

IL-33 was administered to naïve wild-type (WT), nude and ST2−/−, IL-4−/−, IL4Rα−/− and T-or B-cell-specific IL-4Rα−/− mice. IgEand cytokines were quantified by ELISA. T- and B-lymphocyte numbers and CD40L expression were determined by flow cytometry. Anaphylaxis was measured by temperature, mast cell degranulation and histamine release.

 

Results

IL-33 enhanced IgE production in naïve WT, T-IL-4Rα−/− but not in ST2−/−, IL-4−/−, IL-4Rα−/− or B-cell-specific IL-4Rα−/− mice, demonstrating IL-33 specificity and IL-4 dependency. Moreover, IL-4 was required for IL-33-induced B-cell proliferation and T-cell CD40L expression, which promotes IgE production. IL-33-induced IL-4 production was mainly from innate cells including mast cells and eosinophils. IL-33 increased mast cell surface IgE and triggered degranulation and systemic anaphylaxis in allergen-naïve WT but not in IL-4Rα−/− mice.

 

Conclusion

IL-33 amplifies IgE synthesis and triggers anaphylaxis in naïve mice via IL-4, independent of allergen. IL-33 may play an important role in nonatopic allergy and idiopathic anaphylaxis.

 

In English - Il33 and il4 together -reach a threshold -and cause anaphylaxis -through IGE -the "allergy" route -with NO ALLERGY .The mice had NEVER been exposed ;-) - so This may play an important role in Idiopathic anaphylaxis - Anaphylaxis with NO known cause .

NO DRUGS

Broad overview of ways mast cells are triggered

Immunologic - IGE , compliment

 

Non immunologic -stress -neurogenic

 

Direct activation -drugs, excercise ,alochol

 

Physical -cold/heat  

These can come from any source

Mast cells inflammation

2013 - Most recent article reviewing the current knowledge of full mast cell opening -degranulation and full discussion of susbstances known to activate mast cells causing selective chemical release .

 

This backs up the discussions I have here on known mast cell triggers and chemicals relased .Also the involveent of mast cells in many conditions .

mast cell inflammation

Mast cell activation syndrome proprosed guidelines and further review published 2012

mast cell definitions MCAD - Diagnostic Proposal-1

Mast cellactivtion syndrome as asperate entitiy to systemic mastocytosis and single system condtions with mast cell eiteology was first described in 2001 .The first paper from Dec 2010 was the first to begin to define these new condtions .

1) monoclonal mast cellactivation syndrome - were the individual has the ckit mutation - on codon 816 . Resreach into patients with iddiopathic anaphylaxis found a number had the ckit mutataion and some mast cells in bone marrow but not enough to fullfil the criteria for systemic mastocytosis as per WHO defintions .

 

2) mast cell actibation syndrome .

 

Issues remain with quaiity of tests avilble for mast cell mediators . the second paper discusses this and sets cirteria for mast cell tryptase levels and mast cell activation (MCA's ) . Further work is needed as a number of patients remain tryptae negitive by this criteria . 24 hour collections of urine for histamine and prostoglandins are acknowledged as suseful test but criteria -numbers wise as to what would be seen as indicitive - remains to be established .

 

In practice most specilists see double normal histamine levels as indicitive .

 

Issues remain with catching these chemicals at high points . Esp as this rmeains not well known to Er's and EMT staff