Anaphylaxis

 

So now we know what the immune system does when it is working properly.

In anaphylaxis, a process has occurred in many, BUT NOT ALL, cases.

 

This is known as allergy sensitisation. In allergy, the body thinks something is "bad" when it isn't.  It then makes IgE to defend it. This attaches to mast cells. When the substance is exposed to again (allergen), it joins with the antibody, which stimulates the mast cell to split open and release contents. The degree of symptoms depends on how much IgE the person has to that allergen. There are 6 classes of IgE, with a range of up to 30. Over 100 is anaphylaxis level.

Anapahylaxis can happen by many other mechanisms:

Immunologic: Other immunoglobulins (IgG-insect stings, blood).

Complement, which can be activated by any perceived "bad" substance, which leads to anaphylactic reactions. The same symptoms apply but can present variably.

Physical triggers:  heat, cold, exercise

Certain drugs:  Morphine, all opiates, "mycin" derivative antibiotics.

For more on triggers, see "Triggers" page.

 

The result, if either IgE or another trigger is involved, is that mast cells open very quickly, in very large numbers, overwhelming the body with their contents:

Histamine

Prostoglandins

Leukotrienes

Heparin

Serotonin

Mast cell tryptase

and other chemicals on the "About Mast Cells" page

 

For more details see "Mast Cells."

 

 

 

 

 

 

 

 

 

 

gnosis .

http://www.epipen.co.uk/

More personal note ;-)

Many of us put our heads in the sand or just want our illness to do one ;-) so we either ignore things or go the other way, convince ourselves we can control our mast cell dsiease . Medicines help and avoiding triggers( other pages) but anaphylaxis is the one part of our condition which will not do the same everytime or do as you want it to do. As someone who tried this for several months-thought i could decide when I needed the EPIPEN. I nearly left it too late. i went unconcious before it worked. But it did work and bought me round and I am here today 32 anaphylaxis later and over 50 doses of adrenlaine later to be able to say these things to you xxxxx i cant say its pleasant or an expereince i would want anyone to go through. But you will be ok ;-) After abiggie as I call it expect some aftershocks- near epipen reactions in the days following because it takes 3-4 days for the mast cells to re seal and stopp spilling contents.

 

I find I am awake for 24hrs( per epipen ) and then sleep for 2-3 days . Then pass alot of urine , all that fluid that left my blood and went into my tissues, causing all the swelling and low blood pressure trouble - has made its way back to my kidneys and isnt needed because it was replaced. Then around then, the emotion of what has happened hits me and I let the tears roll . then I get back to normal xxx

 

Note;- if you are still being investigted read notes on other possible diagnosis (mast cell conditions )

 

i now have a new normal ;) it took time for me to be ready to accept it but I have life again ,differnt but it mine ;-)

This is dialy symptoms which I manage but I dont worry unless I feel properly ill -Then I use my epipen .it works.Im still here and so will you .Without it your body wont cope xxx so go for it

 

The link button takes you to the UK EPIPEN website were you can learn even more about anaphylaxis and register your EPIPENS so you get reminders to keep them in date. They last about a year.

 

You can get practice pens which you can use once month to keep your technique perfect ;-) You can't do it wrong or harm yourself ;-)

 

If you find your EPIPEN out of date and in an emergency. You can use it, but it may not be as effective, but is better than No EPIPEN . You must not use them if there are crystals in them.

 

I know just the thought of anaphylaxis is scary but being prepared will save your life ;-)  Also,

if you think you're in anaphylaxis, use an Epipen. Any doctor or parameidc would rather have you alive with an Epipen in than dead without.

 

After using an EPIPEN, you must go to A&E or call an ambulance. The EPIPEN can make you feel much better, but you need other medicines to stop the reaction from recurring . The Epipen gives you time to get the other important treatments you need.

 

An Epipen dose raises your blood pressure and pulse, which is vital. The staff will give you antihistamines and fluids you needs ;-)

Paramedics will come and give further adrenaline -whats in the epipen -  as necessary, along with:

IV drugs (into a vein through a plastic device)

IV PIRITON

IV HYDROCORTISONE

IV fluids- NOT dextran or plasma expanders:

ideally, *IV RANITIDINE*

OXYGEN - Please inform paramedics if you are a CO2 retainer; you will know if you are.

NEBULISERS- drugs breathed in through a mask. They get mixed with air into tiny droplets, which take the medicine deep into the lungs to help reduce swelling and open them up.

 

They may need to support your breathing with a machine, if you are doing poorly. You won't know anything about it if you are unconscious, but just so you know, they will look after you 100%

 

Up to and including ICU (Intensive Care):  Admission . Again you will be asleep and they will wake you up when you're ready.

...........There are very good and safe treatments

Adrenaline epinepherine  in self-injectable pens:

EPIPENS

We should all carry 2, within expiration date; they last about a year.

 

If you find you are having any of these symptoms, call 999, (or 911 in the US)  and say, "Anaphylaxis"

 

LIE DOWN, or, if your chest is bad, as low as you can tolerate. DON'T STAND, unless you have to:

NOW USE YOUR EPIPEN.

Facts:

Can be given through clothes.

They should be given, as per instructions, to

the upper, outer part of the thigh.

Take out of box and yellow canister.

Remove Grey /Blue safety;  

point black/orange end at thigh and

push against the thigh strongly, like throwing a dart.

Stop hand when contact made;

A clicking noise will be heard.

Hold in place for 10 seconds!

Remove with care, as the needle will come out;

Rub the area vigorously for 10seconds.

These cause Anaphylaxis,

which is a life threatening condition where serious physical changes from mast cell contents lead to shock. Each individual will have different symptoms predominating, but this is the list of symptoms; not every person will have every symptom.

 

Fast heart - then slow

Low blood pressure - from fluid loss from blood into tissues

Lungs filling with fluid

Airways spasming

Throat swelling- closed

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

 

Please dont take any chances!

The Pen doesn't hurt, and right now you need it.

As you gain expereince of your reactions you will notice no 2 reactions are exactly the same in how the run . But In time you will find you notice different groups of symptoms.

 

I have 4 types . remember you may have one or 2 or 5 ;-)

 

Fast - to a full reaction in 3 minutes from my first symptoms

 

medium - 1/2 an hour of symptoms then into afull reaction fast

 

Slow symptoms, controlled with oral antihistamines - which then 4 hrs later go to afull reaction

 

Slowburn - were I have symptoms constantly but they don't become serious .

 

You will know when you need an epipen ;-)

 

Over time you will work out your early warning symptoms . These come baout 2-5 minutes before other symptoms

- For me ( and others) these are

 

- Feeling a suddenly grumpy / angry for no reason

 

- cant find a word  

 

- Stomach churning

 

- feeling bothered

 

In response to this I and others have some antihistamines - commonly Piriton (UK). If a reaction is going to stop this will do it. You MUST USE an EPIPEN if you feel unwell .

I call my Shock / reaction –

“The Full Monty “

I am going to try and explain how it feels when I react . The first thing that happens is that I get

1. Intensely hot , burning.  Often my face but also anywhere else on my body . Grumpy / excessively frustrated

2. Flushed on face and upper body , very rarely legs

3. Then I start itching , mouth for inhalants and foods ,

4. the nose , my nose blocks almost immediately and I have to mouth breathe , ( indication of full reaction ) / coupe moment

5. any other area of my  body may be intensely itchy , face , hair , body , lips , inside front of mouth , back of mouth , back palette .

6. Swelling starts on face / lips , genitals / legs . Bowel cramps / loose stools - all mucous membrane effects . Tongue up at front.

7. My ears pop and I yawn a lot

8. Then my heart rate goes up , to the point that I feel it is “ banging out of my chest “ then

9. my chest feels tight . I cant take a deep breath in ,

10. my throat feels itchy / tight , right inside where I cant get to it . Voice down / croaky ( sally sex worker voice )

11. I cant swallow my saliva

12. I then get wheezy in my chest , breathing very fast , blue lips / chin

13. c  then you can hear the wheeze from afar ,

14. A  Excessively thirsty , B:- shaky ,  C :- jittery ,  D :-  dizzy  even sitting

15.  Sometimes :- get impending sense of doom

16.  

A =  Airway :- Then I lose my voice and my throat closes completely .

                    :- I cant breathe through my  nose . 2 hands on my throat

B   = Blue      :-Legs / hand blue , lips /chin / face remain blue

C  = confused  :-Confused shouting

D  = Dizzy :-   Light headed , need to lie down ,

E = remain all of 14

17 . Unconscious –

So Now you know how to look after yourself .

These are big meds but for anaphylaxis we need to use big medications .

 

The emotional side for me at least is horrible. In the moment the mast cell contents make me anxious and moody . I can cry when the adrenlaine kicks in . After that my body is in self preservation mode until day 5 after when the emotion of it all hits me. I find  it helps me to let myself be- cry or whatever I need to do and the emotions do pass ;-)

 

Families react in various ways , some go over protective some dont speak about it at all . This is hard if you feel you need to talk xx More discussion on this in the - BIG chnage section ;-)

 

If you have come here after your first ever anaphylaxis , you need to be refered to an Immunologist . This is accordace with the current NICE guidelines - feel free to point this out to your GP . You may need to travel to see them but they will help work out what is happening with you . Some anaphylaxis is true allergy and as long as you avoid your allergen you will be fine .

 

Some allergist don't beleive in anaphylaxis without true -IGE positive tests .if this happens you can seek a second opinion. They may call your reactions pseudoallergies or intolerences

 

For testing- You DO NOT , need to stop your medications.All test can be done with them . It may say so on the appointmrnt letter .This is general advice . No doctor will expcat you to put yourself at risk for an appointment .

If you ring and the pa/secetery says you have to be med free, ask her to ask the doctor for you because you have had an an anaphylaxis .

 

They will not expose you to your allergen in clinic . If they want to test they can do so in your blood

 

If this is happening and you have daily symptoms or are continuing to have full monties , then have look at the other sections including investigations ;-) Please feel free to contact me on the details given .

 

Its important you keep a guide to your triggers- can be called intolernces, pseudoallergies- anything which causes you daily symptoms or anaphylaxis .

 

This is a guide for you to carry and use .It has sections on working out triggers and emergency care as detailed here . Depending on your condition, as each mast cell condition is different I will be putting up extra parts for the guide on the background .

 

 

Mastocytosis -

 

Mast cell activation syndrome

 

Idiopathic anaphylaxis

 

 

If you are a doctor, nurse or other health professional. Please find the info on this site and please feel free to contact me .

 

http://www.youtube.com/watch?feature=player_detailpage&v=G9D-Vfmbt4s

This link explains anaphylaxis by true allergy and is clear.

Remember: Mast cells can be activated in several ways.

emergency guide WAO anaphylaxis guidelines_JACI 2011 oxygen mask nebuliser Rapid infusion bags-To get the fluids in quick IV fluids Epipen

Defining Anaphylaxis

Anaphylaxis has been described since          . Despite this, there continues to be a debate over an exact definition.  The current World Health organisation defintion is ;-

 

By the NHS as:

Anaphylaxis is a severe, potentially life-threatening, allergic reaction that can affect many of the systems of the body.

( ref)

 

Several assessment and defintion tools have been proposed. The most useful in current use is "Ring' et al's Theory.  Click the "Link-Its" in the "Idiopathic Anaphylaxis" section.

The World allergy association ( 2012 ) have suggested the following criteria .

 

-If the individaul had blood pressure below 90/50 -felt as dizzyiness ,lighthheadedness -

and /or

breathing difficulaties

 

Then an epipen should be used and help sought preferable at the same time but if the person is alone EPIPEN ..

 

This si reiterated in a recnat article on Ananaphylaxis in the BMJ

 

Things to know -

 

- You dont have to pass out to have anaphylaxis

 

- You must lie down and not stand unless absolutely essensial -like fire /flood etc

 

- Anxiety is amast cell symptom

 

_ anything can be a trigger

 

_ It wont behave the same each time

 

- It learns new trciks

 

- It has no consistant rules

 

- Murphys law applies-if your prepared you won't need it -But if your not -You well may do

 

-  if you feel very unwell -you ARE unwell- get some help

 

Other bits if info -

 

-histamine cna give you wdown thoughts

-those with dialy symptoms can expereince histamine based type of seasonal affective disorder .

 

Histamine takes perfectly working brains and turns them to mush - making remebering what happened hard-This is expected .

 

Videoing yourself or sending yourself texts of early symptoms cna help as the texts will be timesd and dated .

 

Most of all listen to your body and redpond - it will do all it can to keep you going -the EPIPEN will help it do this until help arrives .

I imagine you are now going oh my god, I have anaphylaxis . It can be mild to very severe .

It is possible to care for yourself and live your life, even if you dont know all your triggers -see triggers for more details

 

Please dont be scared of the EPIPEN, when you need it you wont feel the injection  .

 

Diagnosis is hard- So trust your instinct .

Mast cell tryptase is not a reliable measure of anaphylaxis .Only 30 % of patients in full anaphylaxis show with high mast cell tryptase. this is worst for food reactions .

 

The optimum time for the blood to be taken is between 30- 120mintes after symptoms begin. After this is drops off rapidly.Having serial measurements in actue (2hrs ) 6 hrs and 12 hours and a good day baseline make it amore useful tool . Diagnosis of anaphylaxis should be based on clinical signs .

Due to the number of tools availble and relative rareity of anaphylaxis -about 30% are diagnosed accurately in A&E /ER .However serious symptoms are universily treated properly .

In IGE anaphylaxis, the symptoms are fast and come every time an allergen is encountered; the seriousness will vary time to time.

 

In non IgE anaphylaxis, the progress of the reaction can vary. This is different from what many doctors expect. I am working on changing this perception .

 

So in yourself, symptoms may be different, with different triggers.

 

Don't try and second guess your reactions if you feel unwell:

Lie down, use your EPIPEN and

CALL help:

999/911

emergency guide

Normal - a much overused and arbitory word which bears little resembelence to the life lived or the person living it

EPIPEN and basic life support training

After an anaphylaxis you will be seeing a resusitation officer- They are responsible for all staff training, keeping crash trollys ( with all the kit staff needs ) and training individuals with life threatening conditions .

 

This appointment is a one off .You can take freinds and family .

 

Anapahylaxis and resusitation guideline change -improved ever 5 years or so .I have the most up to date guidelines here.

 

For practical skills and keeping skills up to date several organisations offer first aid training .

 

In the UK we have had recant adverts which are accurate and work .

 

This is the advert

 

 

Here are more details

Including a practice AP

 

 

 

This link is to training

 

 

St Johns ambulance offer

courses

For a first aid guide

 

 

For detailed first aid training

and refresher courses

 

 

 

http://www.bhf.org.uk/heart-health/life-saving-skills/hands-only-cpr.aspx http://www.youtube.com/watch?v=JR0aZX1_TD8&feature=youtu.be http://www.bhf.org.uk/heart-health/how-we-help/training/heartstart-uk.aspx https://www.sja.org.uk/sja/support-us/the-difference/get-a-free-first-aid-guide.aspx https://www.sja.org.uk/sja/training-courses.aspx

The Epipen works on two receptors on cells,

the alpha receptor, which makes blood vessels tighten up (called vasoconstriction), which increases blood pressure.

This prevents and relieves low blood pressure, hypotension and shock.

 It also reduces oedema (swelling in the airway, larynx, voice box).

The beta receptor, which opens airways (broncodilation), thus relieving wheeziness, and reducing mast cell mediator release- also reduces hives (skin red welts).

images (14)

Before Anaphylaxis /mast cell

After Anaphylaxis

images (16) Prevent-Anaphylaxis epinephrine-process-s630x510

 Epipen treatments take up to 5 minutes to work. If it doesn't improve symptoms after 5 minutes,

or, if symptoms return before the ambulance arrives, administer the second Epipen in the other thigh.

Remove the grey/blue  safety cap,

Black/orange end towards the thigh.

Major mediators of anaphylaxis (9 13 18 44 50e52 57)

Mediators / cells Action

Histamine (via H1‒H4 histaminergic

receptors)

Pruritus (itching), tachycardia (fast heart), rhinorrhoea (runny nose), bronchospasm (tightening of airway tubes) (H1)

Endothelial release of nitric oxide (NO), leading to

vasodilatation and hypotension (H1)(see subpage)

Hypotension, flushing and headache (H1, 2)

Inhibitory presynaptic (H3) release- endogenous

epinephrine (see "Subpage")

Chemotaxis and mast cell cytokine release (H4)

 

Tryptase Activates: complement, coagulation and kalikrein‒kinin

system leading to angioedema, hypotension, and

disseminated intrvascular coagulation

 

Respiratory and gastrointestinal tract mast cells

contain less tryptase than connective tissue mast

cells (tryptase may not increase in food anaphylaxis)

 

Platelet activating

factor (PAF)

 

Responsible for systemic mast cell activation;

high concentration of PAF and low concentrations of

PAF-acetylhydrolase may predispose to severe reaction

 

Low serum ACE concentrations may also contribute

to severe anaphylaxis

 

Eosinophils Pro-inflammatory (release mediators from their granules)

 

 

Adapted from-Nassar et al 2012

What happens now?

Mast cell conditions

My Reaction -

 

Generally held, so-called "Truths" about anaphylaxis:

 

- that reactions behave the same every time

* Not true, even for ige true allergy

 

- that the level of a previous reaction dictates the strength of the next one

* Not true

 

My Reactions behave differently, due to many factors,

including:

 

Trigger:

Infection

Hormones

Foods

Inhaled substances from environment

Medications on board

Tiredness

Rate of absorbtion of trigger (Inhaled, alcohol, sugar:  fast!)

Combination of triggers

Recent reactions (snowball effect)

Stress (how full my bucket is)

 

If a reaction can be stopped, I can stop it with oral meds.

 

You should never withhold an Epipen .delay is epipen use is the highest correlated factor in death from anaphylaxis .

(Nasser et al 2012)

also asthma , infection and hormones in females

Current Guidelines

Anapahylaxis is real issue in Mast Cell disease

Anaphylaxis means :-  against protection.  This is due to the fact that the process of anaphylaxis is the immune system gone wrong.   Confused.co.uk ;-)

Our immune systems are made up of 2 parts, our inborn, (innate) immunity, and Acquired immunity. Our innate immunity predates our acquired immunity by many thousands of years.

Innate immunity is comprised of:

Complement, a cascade of chemicals, which at the end, kills non-self cells but also activates mast cells.

Phagocytes, which eat dead cells or non-self cells, identified by antibodies or external markers on the cell.

Stomach acid, which kills the majority of incoming bacteria.

Mucous membranes:  nose, mouth, lungs, bowel, bladder, which have little hairs-called cilia, which move the mucous to the outside world, which catch bugs.

Tears in eyes, protecting them from infection and damage.

 

Acquired immunity is a system in which the body identifies a substance as disease-causing (pathogenic) cancer or non-self and then takes 2 weeks to make antibodies. (It takes 2 weeks to make these antibodies!)  There are several classes-IgA, IgG, IgE, IgD.  IgE attaches to mast cells (more later). When the problem substance comes back into the body, the antibody recognises it and elicits a complement cascade, which splits open the cell and kills it:  job done.

 

There are cells involved in making the antibodies, t cells; they becomes t killer, t memory, which assist immunity on other occasions. ( see about mast cells )

EpiPenNewPensFeatures2

Old pen style below - new to the right

http://www.epiclub.com.au/index.php?page=education-injector\newsplash