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Posted by on May 10, 2013 in The Symptoms |

Typical Symptoms, Anyone?

Typical Symptoms, Anyone?

If you wish to discuss your symptoms, please head for The Symptoms Exchange in the forum

Here, There And Everywhere

Mastocytosis affects all systems, organs and tissues of the body.

In particular, skin, liver, spleen, lymph nodes, bones and bone marrow, lungs, gastrointestinal system, eyes and blood are the most documented as adversely impacted by these diseases.

Research exists but much more needs to be done to further identify and understand the less studied effects of mast cell disorders on the heart, brain and female reproductive system.

Will You Pass Me The Menu, Please?

Signs and symptoms of Mastocytosis are many and variable per patient and between patients.

Some patients experience only a few symptoms, some experience many, some have all of them at various times or persistently. There is no way to fully predict what each patient will experience.

So, it is as if your mast cells wake-up, grab the symptoms menu and decide which symptoms they will serve you next.

Why do the symptoms vary so much?

In a word? Permutations.

And in many words?

  • There are different types of mast cells (depending on the tissue they reside in).
  • Each of these types has a different set of mediators they can release.
  • The tissues these mast cells reside in are different. Some mast cells are close to major organs. Others, close to nerves. Still others, close to blood vessels.
  • The intensity of the release varies depending on the trigger type and amount of trigger. So your mast cells can just leak or explode.
  • When the mast cells leak, they can release everything or selectively release some mediators only.
  • The mast cells can be severely deformed or be borderline deformed, therefore presenting a varying level of dysfunction.
  • Some patients have just one mutation, others may have more than one. Patients having more than one mutation may not all have the same mutations.
  • Each mutation leads the mast cells to behave in a different way, producing different aberrant mediators, leading to different symptoms.
  • And then there’s another twist that makes the situation worse yet: most of the mastocytosis patients also have mutations in other genes and proteins that are integral to mast cell function.

The Biological Permutation Math

So, you do the biology math.

These cells produce hundreds of different mediators.

One mutation leads to aberrant release of maybe not all mediators in the mast cell but probably a sizable subset.

But there are multiple mutations in most patients with mast cell disease. How any one mutation might interact with any other one or multiple mutations remains to be determined.

And there are multiple genes beyond just the one affecting mast cells that are mutated in most mast cell disease patients.

Each mediator doesn't cause just one problem. Each mediator has an entire array, a unique array, of effects – direct effects, indirect effects, local effects, and remote effects.

So, when you do all the multiplying implicit in all of this biology math, you begin to understand why this disease will present with such extreme variety of symptoms.

Initially, only a few signs and symptoms may be present. Especially if diagnosis takes several years, the signs and symptoms escalate in type, severity, frequency and persistence, eventually leading to permanent disability or death.

Below is one list of 58 signs and symptoms reported to the Mastocytosis Association Canada by patients consistently over the past 10 years.

    • skin lesions or sores
    • skin rash, spots, redness
    • hives
    • persistent fatigue
    • itching
    • flushing & severe sweating
    • joint, bone pain
    • headaches
    • tachycardia (racing heartrate)
    • eyes tearing/dry, eye pain
    • persistent body/tissue pain
    • difficulty exercising
    • vertigo
    • episodes of low body temperature
    • unexplained Vitamin B12 deficiency
    • scents/odors/chemical reactions
    • difficult menses (females)
    • numbness & tingling in face and extremities
    • skin feels on fire
    • unexplained anxiety
    • sudden drops in blood pressure
    • fainting
    • persistent diarrhea
    • vomiting
    • unexplained weight loss
    • cognitive impairment
    • sinus problems
    • chest pain
    • vision problems
    • hair loss
    • mouth sores
    • nausea
    • swelling & inflammation
    • odd reactions to insect stings
    • anesthesia difficulties
    • anemia
    • thyroid problems
    • decreased bone density
    • unexplained weakness
    • shortness of breath
    • sunlight sensitivity
    • temperature (hot/cold) sensitivity
    • difficulty with foods, drinks
    • anaphylactoid reactions
    • anaphylaxis
    • gastrointestinal pain, bloating
    • unexplained medication reactions
    • enlarged liver/spleen
    • liver/spleen/bladder/kidney pain
    • enlarged lymph nodes
    • frequent urination
    • recurring infections
    • neuropathic pain
    • constipation (MCAS)
    • iron deficiency
    • unexplained bruising, bleeding
    • malabsorption
    • intermittent tinnitus or hearing problems

And no post on this site would be complete without a quote from the now familiar Dr. Afrin :

 The symptoms are often subtle, chronic-persistent or chronic-intermittent, or chronic waxing-and-waning. There are different symptoms at different times. Often there are no apparent triggers, but there also are plenty of patients who very clearly know what their triggers are.

The constitutional issues are all over the map, including fevers and/or chills or, most commonly, neither frank fever nor frank chills but rather just a sense of feeling cold all the time. You have to ask about that specifically because if you just ask about fever and chills, they’ll often deny both.

The fatigue and malaise can be just terrible, to the point of truly utterly disabling the patient.

Unprovoked sweats (often, but not always, isolated to the evening and night hours) are another frequent symptom.

Some of these patients have anorexia, weight loss, and sometimes early satiety, usually as a result of an enlarging spleen. Much more commonly, though, we see issues not with weight loss but rather with weight gain, sometimes hugely so. I've had a number of these patients come to me after a gastric bypass which did help them lose weight but didn't address any of the myriad of symptoms they had before the gastric bypass which the surgeon never realized or chose not to address prior to surgery in spite of the much greater likelihood of there being a common problem underlying both the obesity and the plethora of other symptoms.

The pruritis can be anywhere on the scale from non-existent to atrocious.

Also, these patients not uncommonly have odd allergies such as things you wouldn't think it’s possible to be allergic to.

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Posted by on May 11, 2013 in The Symptoms |

Symptoms Severity Ranking

Symptoms Severity Ranking

 

Do you have any questions about mastocytosis symptoms? Then please head for The Symptoms Exchange forum and you'll find plenty of company.

The Most Common Symptoms Ranked

In 2008, a study was published by a leading French mastocytosis research organisation, which ranked 38 most common mastocytosis symptoms by their impact on quality of life and disability.

Study Design and Objectives

A case-control study (Case-Control Cohort Study of Patients’ Perceptions of Disability in Mastocytosis) was initiated in September 2004 by AFIRMM to examine patients’ disability due to mastocytosis. The objectives of the cohort study were to:

  • evaluate the patients’ perception of disability,
  • establish and validate a composite score for disability,
  • determine the most important symptoms causing the patients’ perception of disability, and
  • correlate disability with mastocytosis classification and presence of the D816V KIT mutation and an elevated level of serum tryptase.

How The Study Was Structured

During visits by mastocytosis patients to French hospitals between 1999 and 2004, it was noticed that they tend to suffer from a very wide variety of common symptoms and that they often feel disabled by these symptoms.

To gain a clearer picture of overall disability in mastocytosis and the type and severity of symptoms causing disability, researchers developed two questionnaires. The first was a questionnaire asking the patients about their overall perception of disability, and the second was a questionnaire asking the patients about their perception of the severity of 38 symptoms in 12 categories (skin, allergy/flush/shock, gastrointestinal tract, rheumatology, asthenia, neurology/psychiatry, respiratory, urology, infection, hemorrhoidal inflammation, libido, and sweat).

In September 2004, the questionnaires were sent to 703 adult mastocytosis patients. Responses to both questionnaires were obtained from 363 patients. The 363 patients included individuals with CM and both indolent and aggressive forms of SM, but none of the patients had mast cell leukemia

In addition, the questionnaires were administered to 90 control healthy subjects with no family members suffering from mastocytosis.

Study Conclusions

The mean scores and the standard measures of disability indicated that most mastocytosis patients suffer from disabilities due to the disease. Surprisingly, the patient’s measurable and perceived disabilities did not differ according to WHO disease classification or presence or absence of
the D816V KIT mutation or an elevated serum tryptase level. Also, 32 of the 38 symptoms were more common in patients than controls.

The conclusion reached was that mastocytosis patients suffer from more symptoms and greater disability than previously thought, that mastocytosis may therefore be under-diagnosed, and that the symptoms of the indolent forms of mastocytosis might be due more to systemic release of mediators than mast cell burden.

Patients feel disabled by their symptoms regardless of whether they have CM or SM, The overall extent of disability does not differ significantly between these two main types of mastocytosis, and the overall disability is unrelated to the presence of the presence of the D816V mutation or an elevated serum tryptase level.

Symptoms Severity Ranking
Symptom Rank Controls Patients
Number Experience
Any
Disability
Severe or
Intolerable
Disability
Number Experience
Any
Disability
Severe or
Intolerable
Disability
Psychological Impact 1 90 9 (10%) 1 (1%) 363 261 (72%) 120 (33%)
Asthenia 2 90 34 (38%) 3 (3%) 362 296 (82%) 102 (28%)
Pruritus 3 90 25 (28%) 3 (3%) 363 299 (82%) 82 (23%)
Food allergy-intolerance 4 90 9 (10%) 0(0%) 363 222 (61%) 97 (27%)
Erythemateous crisis 5 90 17 (19%) 1 (1%) 363 293 (81%) 69 (19%)
Muscle and joint pain, cramps 6 90 36 (40%) 3 (3%) 363 276 (76%) 71 (20%)
Pollakiuria 7 90 58 (64%) 6 (7%) 362 263 (73%) 64 (18%)
Drug allergy 8 90 16 (18%) 0 (0%) 363 205 (56%) 70 (19%)
Aerophagia/eructation 9 90 43 (48%) 1 (1%) 363 229 (63%) 62 (17%)
Dyspnea-bronchoreactivity 10 90 15 (17%) 3 (3%) 362 154 (43%) 94 (26%)
Headache 11 90 34 (38%) 4 (4%) 362 250 (69%) 48 (13%)
Bone pain 12 90 16 (18%) 0 (0%) 363 196 (54%) 65 (18%)
Reduced sexual relations 13 90 11 (12%) 4 (4%) 362 132 (36%) 65 (18%)
Epigastric pain 14 90 35 (39%) 2 (2%) 362 249 (69%) 40 (11%)
Ocular discomfort 15 90 43 (48%) 1 (1%) 363 219 (60%) 55 (15%)
Memory loss 16 90 32 (36%) 0 (0%) 362 240 (66%) 34 (9%)
Tinnitus 17 90 29(32%) 1 (1%) 363 166 (46%) 47 (13%)
Pseudo-occlusive syndrome 18 90 20 (22%) 0 (0%) 363 199 (55%) 36 (10%)
Infections (bronchitis, rhinitis) 19 90 25 (28%) 2 (2%) 363 182 (50%) 38 (10%)
Olfactive intolerance 20 90 33 (37%) 1 (1%) 363 188 (52%) 39 (11%)
Social Interaction 21 90 9 (10%) 0 (0%) 362 200 (55%) 26 (7%)
Depression 22 90 19 (21%) 0 (0%) 362 205 (57%) 22 (6%)
Mobility 23 90 6 (7%) 0 (0%) 363 153 (42%) 35 (10%)
Anaphylactic shock 24 90 12 (13%) 0 (0%) 363 160 (44%) 30 (8%)
Sweating 25 90 19 (21%) 2 (2%) 363 169 (47%) 30 (8%)
Stomatitis 26 90 28 (31%) 1 (1%) 363 145 (40%) 34 (9%)
Flush 27 90 9 (10%) 0 (0%) 363 190 (52%) 23 (6%)
Performance Status 28 90 11 (12%) 1 (1%) 362 187 (52%) 25 (7%)
Hemorrhoids 29 90 19 (21%) 1 (1%) 363 156 (43%) 23 (6%)
Cough 30 90 22 (24%) 0 (0%) 362 171 (47%) 9 (2%)
Ear/nose/throat inflammation 31 90 13 (14%) 0 (0%) 362 120 (33%) 20 (6%)
Erectile function/ability to make love 32 90 10 (11%) 2 (2%) 362 71 (20%) 34 (9%)
Nausea, vomiting 33 90 20 (22%) 0 (0%) 363 179 (49%) 12 (3%)
Diarrhea 34 90 6 (7%) 0 (0%) 363 127 (35%) 10 (3%)
Warts 35 90 14 (16%) 1 (1%) 363 82 (23%) 10 (3%)
Pain 36 90 4 (4%) 0 (0%) 362 71 (20%) 7 (2%)
Folliculitis 37 90 7 (8%) 1 (1%) 362 56 (15%) 6 (2%)
Dysuria 38 90 6 (7%) 0 (0%) 362 51 (14%) 3 (1%)

 
You can download the AFIRMM study from the link below:

DownloadDownload PDF 212K Case-Control Cohort Study of Patients’ Perceptions of Disability in Mastocytosis
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Posted by on May 12, 2013 in The Symptoms |

Osteopenia/Osteoporosis

Osteopenia/Osteoporosis

Have you been diagnosed with either of these conditions? Do you want to share your treatment and compare it with others? What kind of lifestyle changes did you implement as a consequence of OP? For all of these, and more, head to the Osteopenia and Osteoporosis forum.

Why Is Osteopenia/Osteoporosis In ‘The Symptoms’ Section?

Because, like with urticaria pigmentosa, it is a visible sign that something connected to mast cells may be going wrong. If you experience unexplained bone fractures, without any particular force being exerted on your bones, this should prompt your physician to test for mastocytosis.

However, not many physicians know that.

What The Heck Is Osteopenia?

Let's get this one out of the way quickly. Here's the blurb about Osteopenia, straight from WebMD:

Osteopenia refers to bone mineral density (BMD) that is lower than normal peak BMD but not low enough to be classified as osteoporosis. Bone mineral density is a measurement of the level of minerals in the bones, which shows how dense and strong they are. If your BMD is low compared to normal peak BMD, you are said to have osteopenia. Having osteopenia means there is a greater risk that, as time passes, you may develop BMD that is very low compared to normal, known as osteoporosis.

Now, what is peak BMD?

 The amount of bone tissue in the skeleton, known as bone mass, can keep growing until around age 30. At that point, bones have reached their maximum strength and density, known as peak bone mass.

So, let's be clear. If you are over 30, your bone mineral density  WILL be lower that peak BMD. That is because

All people begin losing bone mass after they reach peak BMD at about 30 years of age.

And, even if you are 30 or below …

Some people who have osteopenia may not have bone loss. They may just naturally have a lower bone density.

Uh? So, you have no bone loss and you are still told there's something wrong with you?

One thing everyone agrees on, osteopenia does not require treatment. It's NOT a disease. True, osteopenia is a risk that you may be on the way to osteoporosis. And osteoporosis is, in turn, a heightened risk of bone fractures. So, osteopenia  is a risk of a heightened risk of bone fractures …

My opinion : if you want to take precautions due to osteopenia, check vitamins D, K2, A and calcium. Steer well away from medical drugs.

Did you read the first to words of the previous paragraph? Remember, I am NOT a doctor.

Do your homework. There's a decent amount of medical research that questions what osteopenia  really means.

OK, Got That. What is Osteoporosis, Then?

Watch this short (1:27) clip. It tells you enough to get you going.

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So, in osteoporosis, your calcium balance is off kilter. You remove more calcium from your bones than you put back in.

You see, you need calcium for very important stuff in your body. Bones are the calcium bank, all the calcium ‘money' is stored there. When your body needs calcium, it is shaved off the bones and supplied to the other parts of the body through the blood stream. That “shaving off” thing is called bone resorption. That resorption is performed by bone cells called osteoclasts. But, simultaneously, there are some other bone cells that build new bone, which are called osteoblasts. They both work in tandem, to keep the bone healthy.

However, with mastocytosis, even if your calcium balance was OK, your bones are still being depleted of calcium . If you read the post What Are Mediators, you know that mast cells release lots of mediators (heparin, serotonin, chymase, renin, prostaglandin …)  that interfere with the work of osteoblasts, an interference which causes new bone buildup to slow down while old  bone is still resorbed at the same rate.

You can have osteoporosis even if you do not have mastocytosis. That is called primary osteoporosis, that is, it is not dependent on the existence of another condition. If your osteoporosis is deemed to be caused by mastocytosis, it is called secondary mastocytosis. This distinction will be important later, when we talk about the probability of fracture risk.

Osteo-whats?

Yes, I know, they don't make it easy, do they? And the first clip above did not make it very clear.  Watch this clip, maybe it will become clearer.

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So, there's one guy removing old bone and one guy adding new bone. If both guys work at the same pace, all is well. That is, provided there is enough building material for new bone, in this case, calcium.

If one starts to work faster than the other, or if one starts to slow-down, an imbalance occurs. That imbalance also exists if there are fewer new bone builders than there  are guys breaking down old bone. That distinction will be important when we talk about  prescription drugs for osteoporosis.

Fragility Fractures

If you suffer from osteoporosis, you run an increased risk of experiencing fragility fractures, which are …

… defined as fractures resulting from a fall from a standing height or less, or presenting in the absence of obvious trauma. Fragility fractures affect up to one-half of women and one-third of men over age fifty, and are often associated with low bone density. Such fractures occur most commonly in the hip, spine, and wrist.

Bone Mineral Density …

… Or BMD, as it is affectionately known, is a measure of … ? Bone Density!

Is bone density a measure of bone strength? The jury is still out. You will find medical research insisting that it is and medical research arguing that it isn't.

There's a story that comes to mind, though, about a twig and a tree …

DEXA Scan

The way BMD is measured is through a non-invasive, low radiation scan called a DEXA scan. The aim of the scan is to estimate bone strength by measuring a proxy called bone density.

You can learn a bit about the DEXA scan here.

What’s The Score?

When you get your DEXA scan result, simple minds like mine become perplexed.

The result talks about Z-scores, T-scores, standard deviations …  Oh, my!

Let's look at the basic definitions:

The scores are expressed as standard deviations, which is a measure of variability based on an average or expected value. Don't worry if you did not understand that,  just look at what constitutes a normal range T-score.

Z-score: A comparison between your BMD and the bone density of someone who is of the same age and sex as you.
T-score: It is the bone mineral density at the site (hip, wrist …) when compared to the young normal reference mean. It is a comparison of a patient's BMD to that of a healthy thirty-year-old of the same sex and ethnicity.

A positive T-score number means your Bone Mineral Density is  above average, a negative number means it is below average.

Now, about the ranges: a T-score of

  • above -1 is normal
  • between -1 and -2.5 is classed as osteopenia (where bone density is lower than average but not low enough to be classed as osteoporosis)
  • below -2.5 is classed as osteoporosis

A bone density scan can help diagnose osteoporosis, but your BMD result is not the only factor that determines your risk of fracturing a bone.

I’ve Got A Below-Normal T-score, What Does It Mean?

It means that you may have a higher probability than normal to have a bone fracture. But the T-score will not tell you what that increased probability is. There are several additional criteria that need to be considered, in addition to the T-score to find out that probability increase.

So how do you find out how what your increased risk is? Well, if your physician has not told you already and you know your T-score, you can find out yourself by using the WHO FRAX tool.

Here's a video that tells you how to do just that.

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A couple of more things:

  • When the FRAX tool gives you your increased probability of fracture, in the UK version of the FRAX tool, you will have a link to The National Osteoporosis Guideline Group (NOGG). This is a second tool which, based n the results of the FRAX tool, will give you (and your physician) an indication as to whether you should be treated or not treated and be given lifestyle guidance or not treated and just keep an eye on the condition. If the NOGG tool is not available in your country, you can still access it and enter your data manually here. But be aware I don't know whether the results will be correct for your particular situation, as they are possibly related to geographical location and ethnicity.
  • The FRAX tool does not take into account your Vitamin D / Calcium status. Quite surprising, as these 2 supplements go a long way to providing additional building blocks for the osteoblasts to improve bone health.

The Treatment

The treatment of choice is alendronic acid. And here, I will leave you to your own devices. Do your home work and learn about the efficacy and the side effects of this drug. Spend some time thinking about the estimated additional level of fracture risk you are incurring at your level of affliction. And then decide whether alendronic acid is for you.

Please, do yourself a favour. Do not blindly accept treatment by alendronic acid WITHOUT doing your own due diligence. If you do your research, you may want to know that alendronic acid is also called a bisphosphonate.

An alternative choice of treatment may be Vitamin C and calcium supplements. However, you then need to do your own research into balancing Vitamin D3 with Vitamin K2 and complementing Vitamin K2 with Vitamin A.

Quite convoluted.

Maybe you should come around to our Nutrition section of this site, once it is completed. Oh, and if you want to know stuff about bisphosphonates, head for the forum. We can have a good ‘ole chat about that.

So Many Quotes In This Post ….

… And not a single reference.

I know … Dreadful!

On purpose, though. All of the above are my opinions only. I did do my own research on the topic, but I encourage you to do the same.  Hey, go and get your own references!

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Posted by on May 13, 2013 in The Symptoms |

Urticaria Pigmentosa

Urticaria Pigmentosa

Let's watch this brief 3:23 clip, to get the gist of Urticaria Pigmentosa (UP). The video transcript is below, as the commentator is not easy to understand.

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Video Transcript

Urticaria pigmentosa is an uncommon rash that usually affects the neck, arms, legs and trunk of children and young adults.

The rash consists of reddish brown spots that turn into hives when they are rubbed or scratched. Sometimes, the spots will blister.

It is a type of mastocytosis, in which there are brown patches on the skin due to an abnormal collection of mast cells.It is caused by excessive numbers of inflammatory mast cells within the skin.

UP most often affects infants, with the first patches appearing at a few months of age. They are often confused with insect bites at first, but persist and gradually increase in numbers for several months or years. They can appear in any part of the body, inclusing the scalp, the face, trunk and limbs.In young children it is common for the patches to blister when rubbed. If many patches are activated at the same time, the infant may become irritable, but it is uncommon for severe symptoms to arise. Over the next few years, the UP becomes less irritable and eventually the patches fade away. By the teen-age years, most patches will have gone. In adults, UC can develop for the first time. Few or many patches appearing may be unsightly as well as itchy. Unfortunately, in adults, UP tends to be long-term. It is also more likely to be associated with internal symptoms. To check for symptoms of UP, check for the presence of mast cells. One can test for these symptoms by rubbing one of the brown patches. within a few minutes, the rubbed area becomes red, swollen and itchy. This is known as the Darier's sign and confirms the presence of Mastocytosis.

The main symptom is brownish patches on the skin. Rubbing the skin causes the hives … Younger childres may develop a fluid-filled blister through the scratch. The face may also become flushed. UP is not serious and does not require any treatment in most cases, however, the following can be helpful:

  • Anti-histamines
  • Mast Cell Stabilisers
  • Low-dose aspirin:  This can help some patients, but must be taken cautiously, as aspirin can also worsen symptoms.
  • Topical steroids:  steroid creams applied for several months can reduce itching, but the patches tend to re-occur after a few months. Topical steroids are only valuable for limited areas.
  • And lastly, photo-chemo-therapy:  This form of UV radiation is the most effective treatment for adults with UP. Two or Three treatments each week are required for several months. Photo-chemo-therapy lessens the itch and improves the appearance.The UP is likely to re-occur in 6 to 12 months, but the treatment can be repeated.

Now, lets get into a bit more detail and specifics:

Cause

The majority of urticaria pigmentosa cases are caused by a point mutation at amino acid 816 of the proto-oncogene c-kit.[2] c-kit is a transmembrane protein which, when bound to Mast Cell Growth Factor (MCGF), signals the cell to divide. Mutations in position 816 of c-kit can result in a constant division signal being sent to the mast cells, resulting in abnormal proliferation. Different mutations have been linked to different onset times of the disease. For example, the Asp816Phe and Asp816Val mutations (the aspartate normally at position 816 in the c-kit protein has been replaced with phenylalanine or valine respectively) have been associated with early manifestation of the disease (mean age of onset: 1.3 and 5.9 months respectively).[3][4]

Irritants

Several factors can worsen the symptoms of urticaria pigmentosa:

  • Emotional stress
  • Physical stimuli such as heat, friction, and excessive exercise
  • Bacterial toxins
  • Venom
  • Eye drops containing dextran
  • NSAIDs
  • Alcohol
  • Morphine

The classification of NSAIDs can be disputed. Aspirin, for example, causes the mast cells to degranulate, releasing histamines and causing symptoms to flare. However, daily intake of 81 mg aspirin may keep the mast cells degranulated. Thus, while symptoms may be worsened at first, they can get better as the mast cells are unable to recharge with histamine.

Diagnosis

The disease is most often diagnosed as an infant, when parents take their baby in for what appears to be bug bites. The bug bites are actually the clumps of mast cells. Doctors can confirm the presence of mast cells by rubbing the baby's skin. If hives appear, it most likely signifies the presence of urticaria pigmentosa.

Treatments

There are no permanent cures for urticaria pigmentosa. However, treatments are possible. Most treatments for mastocytosis can be used to treat urticaria pigmentosa. Many common anti-allergy medications are useful because they reduce the mast cell's ability to react to histamine.[1]
At least one clinical study suggested that nifedipine, a calcium channel blocker used to treat high blood pressure, may reduce mast cell degranulation in patients with urticaria pigmentosa. A 1984 study by Fairly et al. included a patient with symptomatic urticaria pigmentosa who responded to nifedipine at dose of 10 mg po tid.[5] However, nifedipine has never been approved by the FDA for treatment of urticaria pigmentosa.
Another mast cell stabilizer Gastrocrom, a form of cromoglicic acid has also been used to reduce mast cell degranulation.

Epidemiology

Urticaria pigmentosa is a rare disease, affecting fewer than 200,000 people in the United States.

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Posted by on May 14, 2013 in The Symptoms |

Anaphylaxis

Anaphylaxis

If you have suffered anaphylaxis and want to talk about your experience, or if you have questions about anaphylaxis, head for the Anaphylaxis Event forum

Webmaster note:

I had intended to summarise many articles I'd read about anaphylaxis for my site. I was well in the process of completing my summary, when I stumbled on the following article, which I found to be all encompassing and much, much better that anything else I had read on the topic. Importantly, It opens a debate which I did not know existed (I'm won't reveal what the debate is yet, just read read on, it's well worth it!)

So, reluctantly, I threw my own summary I'd slaved on for a couple of hours in the wastebasket and decided to include the whole article, with some minor edits.


This post is an edited version  from Asthma and Allergy Information Research. Click on the link if you wish to see the unedited version. The opinions expressed below reflect the opinions of the original author and not necessarily the opinions of the webmaster. Always take the guidance of your doctor, in spite of what you read on the internet.

What is anaphylaxis?

Anaphylaxis is the word used for serious and rapid allergic reactions usually involving more than one part of the body which, if severe enough, can kill.

The word anaphylaxis was coined when scientists tried to protect dogs against a poison by immunising them with small doses. Far from being protected, the dogs died suddenly when they got the poison again. The word used for protection by immunisation is ‘prophylaxis', so the scientists coined the word ‘anaphylaxis' to mean the opposite of protection. What the scientists saw in the dogs helped them to understand that the same can happen in humans.

Scientists now use the word ‘anaphylaxis' to mean any immune reaction of this type, even if it is not serious. But most doctors use it to mean a life-threatening rapid allergic reaction.

Unfortunately this kind of ‘harmful immunisation' happens to a few of us not just from injections but from ordinary foods such as nuts. Quite literally, “one man's meat is another man's poison”. Our immune system, which is there to protect us from infection, goes wrong and harms or even kills us.

Injections of many kinds occasionally cause anaphylaxis. Penicillin, injected clot-busting drugs used after heart attacks, and a host of other kinds of injection can occasionally do to human beings what the experiments did to dogs.

What happens in the body during anaphylaxis

Anaphylaxis happens when the body makes the ‘wrong' kinds of antibody to protein in our food or to something like a drug.  The ‘wrong' kind of antibody is called immunoglobulin E or IgE for short. IgE sticks to mast cells and basophils in our bodies. When the same protein or drug reaches the IgE on the cells, these substances are released, causing blood vessels to relax, which makes them leaky and can cause swellings and a fall in blood pressure. At the same time they can make the breathing passages become narrow.

Histamine is one of these substances released from the cells. Antihistamines are medicines which stop histamine from working. So you might think that they would be good for nut allergy, and so they can be when the reaction is mild. But as tablets, they take about an hour to get into the bloodstream properly, and this is far too slow to save lives in serious reactions.

Antihistamines also don't prevent all the effects of histamine, for example effects of histamine on the heart, in serious reactions.

More importantly, histamine is not the only dangerous substance released during anaphylaxis.  Antihistamines do not protect you against these other substances. This is another reason why antihistamines used alone will not save life in some really serious attacks of anaphylaxis.

Fortunately, adrenaline (epinephrine), the standard treatment for life-threatening anaphylaxis, works against all the most dangerous aspects of anaphylaxis, not just those caused by histamine.

What are the common causes of anaphylaxis?

  • Foods: especially nuts, some kinds of fruit, fish and less commonly spices
  • Drugs: Especially penicillins, anaesthetic drugs, some intravenous infusion liquids, and things injected during x-rays. Aspirin and other painkillers (called NSAIDs) can produce very similar reactions.
  • Latex: mainly in rubber latex gloves, catheters, other medical products, but also in many things encountered in daily life. Sufferers are nearly always health care workers, mainly nurses, or have other occupational contact with latex. They may get anaphylaxis from bananas, avocados, kiwi fruit, figs, or other fruits and vegetables including even potatoes and tomatoes.
  • Bee or wasp (yellow jacket) stings when these cause faintness, difficulty in breathing, or rash or swelling of a part of the body which has not been stung. If you just get a very large swelling of the part of your body which was stung, you are probably not going to have anaphylaxis if stung again.
  • Unknown: A substantial proportion of sufferers have no cause found despite all efforts, even in the most expert clinics. Doctors call such unexplained attacks ‘idiopathic anaphylaxis' The word ‘idiopathic' in practice means we don't know the cause. Worrying as it is, death from this is very rare indeed. However, there must be a cause or causes. Some cases are bound to be simple failure to find a cause.  The explanation is NOT psychological in the vast majority. So in most cases this is a disease for which medical science has not yet discovered the cause.
  • Exercise may precipitate such reactions in some (‘exercise-induced anaphylaxis'), and so may exercise after food, sometimes apparently irrespective of what the food is, but in other people after specific foods. This is called ‘exercise-induced food-dependent anaphylaxis'.
  • Medicines called beta blockers used for heart disease or high blood pressure can change mild reactions from another cause into severe anaphylaxis because they block the body's main defence against anaphylaxis.
  • Wrong diagnosis of anaphylaxis: a proportion (about 10%) of people sent to specialists with a diagnosis of anaphylaxis have a mistaken diagnosis and have not had anaphylaxis. If this might be true in your case, it is well worth finding this out as you may be spared unnecessary fear and wrong treatment.

How can you tell if someone is having anaphylaxis?

Anaphylaxis usually happens quickly. Anaphylaxis can produce:

  • An itchy nettlerash (urticaria, hives)
  • Faintness and unconsciousness due to very low blood pressure. Unlike an ordinary fainting attack, this does not improve so dramatically on lying down.
  • Swelling (angioedema)
  • Swelling in the throat, causing difficulty in swallowing or breathing
  • Asthma symptoms
  • Vomiting
  • Cramping tummy pains
  • Diarrhoea
  • A tingling feeling in the lips or mouth if the cause was a food such as nuts
  • Death due to obstruction to breathing or extreme low blood pressure (anaphylactic shock)

Faintness with a nettle rash or swelling coming on quickly is probably anaphylaxis. Ff there is also difficulty in breathing, the danger is greater. Faintness with difficulty in breathing alone will sometimes be due to a panic attack, but can also be due to anaphylaxis.
In the early stages it may be difficult, even for a doctor, to be sure whether the cause of symptoms is anaphylaxis or fainting or a panic attack. If there is doubt, it is sometimes best to use the treatment for anaphylaxis, but the treatment should then be reviewed with a doctor because unnecessary treatment for anaphylaxis is a bad idea. Learn the rules for knowing when to treat, to minimise the chance that you will use the treatment when you should not.

Fainting and Anaphylaxis: clues which may help you tell the difference

(this guide is not perfect; you need a doctor if in doubt.)
Anaphylaxis
Fainting
Colour
Pink, typically
Pale, typically
Pulse
Fast, usually
Slow, usually
Blood pressure
Can remain low lying down
Normal when lying down
Other features which may be present
Nettlerash
Swelling
Difficulty breathing
Tummy pain or diarrhoea
The person has probably fainted before.(Some people do faint, others don't.)

 

People who are allergic to foods often notice the effect in seconds, and their life may be in jeopardy within a few minutes. Sometimes, a reaction takes much longer to start, an hour or so, but can still be extremely serious. Improvement can also happen quickly, especially with the right treatment. A few people then have a second wave of anaphylaxis, so people who have had a serious anaphylactic reaction should be observed medically for about six hours or overnight.

Fortunately there is a highly effective treatment: adrenaline (epinephrine). But adrenaline (epinephrine) needs to be given as an injection, and is dangerous if used incorrectly. If you need to have adrenaline (epinephrine) available for yourself or someone in your family, it is important that you and anyone else who may have to give the adrenaline (epinephrine) should be properly trained.

It is possible for anaphylaxis to be mild and to need little or no treatment. You would not think so from most of the information you read. Of course a life-threatening attack of anaphylaxis may look mild in its early stages, so you need to go for medical help just in case, and need to have the emergency treatment immediately available in case things start to go more badly wrong. In most cases it is better to err on the side of treating anaphylaxis early rather than to leave treatment rather late. But the fact is that many people get over anaphylaxis even without treatment.

The fact that previous anaphylaxis has been mild does not guarantee that it will not be dangerous in future. Most sufferers with mild attacks do seem to have relatively mild ones if they occur again. But deaths have occurred in people who had only mild attacks before. The answer is to be prepared.

What is the best treatment for anaphylaxis?

There is one drug which will work against all the effects of all the dangerous substances released in anaphylaxis. It is adrenaline (epinephrine). For serious attacks, it is a vital treatment. You need to inject it; inhalers may no longer be an option.

All these devices with adrenaline (epinephrine) are only reliable if you follow the instructions. They can be dangerous if used the wrong way. If you need them it is important that someone teaches you how to use them properly. If you cannot get such teaching (and you should be able to), then make sure you read the instructions with great care.

Adrenaline (epinephrine) cannot help you if you do not have it with you.

Keep the adrenaline (epinephrine) in a clearly marked robust pouch or case so that it is easy to keep with you. Protect it from light and from high temperatures. In hot climates, keep the container out of direct sunlight and overheated locations such as parked cars. For example in a bag of beach things you can keep it wrapped in several layers of clothing, while it is still rapidly retrievable by tying one end of a cord to the container and the other end to the handle of the beach bag.

How long will the epinephrine (adrenaline) keep? 

Not forever. Make sure that you check the expiry date on the syringe and make sure that you have a replacement by the time this date comes. It really can lose a lot of its effectiveness if you let it go out of date.

Some manufacturers will let you know when the time comes to replace the kit. But you have to fill in a coupon and send it to them when you get your kit. Otherwise they obviously can't give you this service.

Be sure to follow the storage instructions. The Epipen must not be kept in a refrigerator, and no epinephrine injection device must be allowed to freeze (replace if this happens accidentally, e.g. in a car left in freezing conditions long enough).

Often the adrenaline (epinephrine) solution goes yellow or brown when it is becoming useless, but you can't rely on this. It can also become useless without changing colour. So do take care to follow the manufacturer's storage instructions.

Carrying the injection kit around: special containers

Special pouches for injection kits and inhalers are available from the  Allergypack website.

What is adrenaline (epinephrine)?

Adrenaline (epinephrine) is a quick-acting hormone. Our body produces it from two glands sitting just above each kidney. Adrenaline  (epinephrine) production happens naturally in the body when we meet an emergency; the adrenaline (epinephrine) makes our heart pump faster, widens the air passages in the lungs, and tightens up our blood vessels. We get the well-known feeling of alertness and the feeling of a rapid heartbeat and tremor which comes from suddenly being in an emergency. Not only does adrenaline (epinephrine) ready the body and the mind for ‘fight or flight', but adrenaline (epinephrine) also works against all the effects of anaphylaxis.
So adrenaline (epinephrine) is the body's own natural quick-acting hormone for emergencies. When we inject it from a syringe, we can give more than the body can produce quickly.

This means you get more benefit, but also more side-effects and some dangers. In most people adrenaline (epinephrine) is very safe if you use the right dose in the right way, but it is important to understand what you are being asked to use.

When should I inject adrenaline (epinephrine)?

Some specialists say you should inject adrenaline (epinephrine) for any reaction. We don't agree.

Our recommendation is the 3D Rule

  1. Definite reaction: evidence of a reaction should be obvious.
  2. Deterioration: the aim is to inject BEFORE life is in immediate danger. If the reaction is improving by the time you get the adrenaline (epinephrine), just keep the adrenaline (epinephrine) handy for six hours in case it gets worse again.
  3. Death seems any sort of possibility if the deterioration continues another 5 to 10 minutes.

Only two things cause death:  the '2D' RULE

  1. Difficult breathing whether due to swelling in the throat or to asthma. If it really seems to be just asthma, an asthma inhaler may work. But adrenaline (epinephrine) will help in both asthma and throat swelling.
  2. Deteriorating consciousness: once the child or adult patient is unconscious, life is in danger, if only from inhaling vomit, quite likely in a food allergy reaction. Make sure you know the ‘recovery position' known to every competent first-aider. But give adrenaline (epinephrine) to prevent unconsciousness if that seems increasingly possible.

Finally, the '1D' RULE

  1. Do give adrenaline (epinephrine) if in doubt! If you think there may be any risk to life because of difficulty in breathing or because it seems possible the patient is beginning to feel faint or ‘pass out', then the earlier you give the adrenaline (epinephrine), the better it will work.

Do I need to use more than one injection of adrenaline (epinephrine)?

Sometimes, in really bad anaphylaxis, someone may need more than one adrenaline (epinephrine) injection, but nearly always one injection is enough to save life. Too many injections of adrenaline (epinephrine) can definitely be dangerous. For example a young man who gave himself five injections probably died from the injections and not from the anaphylaxis. So your doctor's advice is vital; you may be able to stand more or less adrenaline (epinephrine) than the next patient, and your doctor can give you advice which is right for you.
Some doctors recommend carrying more than one injection dose, and others do not. Some of the injection kits can give more than one dose, but the spring-loaded automatic injectors can only give one dose.

The dose of adrenaline (epinephrine) in the injection kits is usually 0.3 mg for an adult, which is rather low for a really dangerous reaction in a full-sized adult. The reason for this is safety. We know that this dose works in the vast majority of people, but that a few people would get bad side effects from a higher dose. So we recommend this smallish dose because it is the safest overall. But your doctor may be able to give advice more appropriate for you. Hospital doctors would often give 0.5 mg, nearly twice as much. Some would give 1.0 mg, but our opinion is that this is an uncomfortably large dose even for most young adults to give all at once unless the situation is much more desperate than it usually is.

We recommend that the decision on whether you need one or two injection kits is one for which you need your doctor's advice.

How long will an adrenaline (epinephrine) injection carry on working after I have injected it?

Many doctors say that the adrenaline (epinephrine) will wear off in 15 to 20 minutes. This would often mean you might not get to a hospital in time, e.g. if you reacted whilst on holiday. The published evidence suggests that adrenaline (epinephrine) injected under the skin (subcutaneously) works for much longer than this, perhaps longer than the 150 minutes for which one set of research workers carried on measuring adrenaline (epinephrine) in the blood of their volunteers. Our feeling is that the same would be true for injections in to the muscle at the side of the thigh (intramuscular).
The bottom line is that single doses from the widely used injectors have an excellent record for patient survival. So in practice one injection does seem to do the job and enable people to get any extra help they need under nearly all circumstances.

But if you are in a remote area, you should carry more than one dose.

What are the side effects of adrenaline (epinephrine)?

In the doses we recommend, trembling, palpitations (feeling your heart beating fast), and a feeling of tension or anxiousness. These are normal effects of the adrenaline (epinephrine) and soon wear off. Higher doses cause an extremely unpleasant feeling and may be dangerous to the heart.
If you have high blood pressure, or an abnormal heart rhythm, or narrowing of the coronary arteries, or if you are treated for depression with an unusual medicine called a monoamine oxidase inhibitor, special caution is needed with adrenaline (epinephrine), and a specialist should advise you.

Injecting adrenaline (epinephrine) in the wrong place can be dangerous. People have accidentally injected adrenaline (epinephrine) into their thumb when trying to figure out how the syringe worked or when trying to check why it did not work (probably because they did not apply it to the skin at right angles, jamming the mechanism). This is dangerous. Adrenaline (epinephrine) can shut off the whole blood supply by constricting the blood vessels at the base of the finger or thumb. The result is likely to be gangrene.

Injecting other places can be dangerous too. Inject adrenaline (epinephrine) into the muscle of the side of the thigh, nowhere else, unless you are a doctor and know exactly what you are doing.

For debate:

Is adrenaline (epinephrine) being prescribed far more often than necessary?

Deaths of young children from anaphylaxis are very rare. Yet huge numbers of children now go around with adrenaline (epinephrine) injection kits. All medical treatments have side effects and dangers. Although adrenaline (epinephrine) injections given correctly are remarkably safe, they may be more dangerous than the disease if the risk to life from the disease is small enough.
A group of paediatricians in the UK suspects that the dangers of adrenaline do indeed outweigh the benefits. They are planning to find out how many children with food allergies under the age of 16 years actually die or nearly die from anaphylaxis per year. Their aim is to find better rules for deciding whether a child's life is in danger, so that far fewer children with allergies will need adrenaline kits.

If such research succeeds, vast numbers of parents would be able to heave a sigh of relief. Their families and their children's teachers and carers would be spared unnecessary fear and the burden of keeping and using adrenaline.

It is amazing but true that we don't have even remotely reliable figures for the number of deaths and near-deaths from food allergy. This is because of the way death certificates are filled in and turned into statistics, and to some extent because of the low importance given to allergy in the training of doctors. Without this knowledge we can't tell parents how the risk without treatment balances up against the risk of treatment. Widespread prescription of adrenaline is recent and we don't know the risks as well as we would like. What we are doing now is the best we can manage with inadequate information. Better information offers a definite hope of better treatment, which, according to the research group, could mean much less treatment.

Why do we prescribe adrenaline (epinephrine) for so many children ?

A few years ago it was a real rarity for a child in the UK to have adrenaline (epinephrine) to use for anaphylaxis. Now hundreds of children in Leicestershire alone have adrenaline (epinephrine), and the same is true in other parts of the UK and world-wide. Almost every school in Leicestershire now has at least one child with adrenaline (epinephrine) for food allergy, and most schools have more than one.
In Leicestershire there seem to have been three children who have died from nut allergy in the last five years, and we found out about each these through newspaper reports or by chance. So there may well have been other deaths we do not know about.

Parents with nut-allergic children tell me that they would happily see tens or hundreds of thousands of children given adrenaline (epinephrine) to save one child's life. So even just taking our local experience, it looks as if we are doing what parents want.

Since we know that national statistics on deaths from food allergy are very unreliable, they may be a big underestimate, so the risk may be greater than we know.

People who have died from nut allergy have often not had particularly life-threatening reactions before. This means that we have to regard almost all nut allergy as life-threatening. We knowingly prescribe adrenaline (epinephrine) for children in whom we are perfectly aware that the risk is very low. We say so to their parents. We prescribe adrenaline because this is what parents in general want and on balance it is what we feel we would want for our own children if we faced the same problem.

Not infrequently we find ourselves under pressure from a parent to prescribe adrenaline when we feel that the risk really is far too low to justify that; these discussions are difficult because of the great uncertainties.

Personally, I regard the prescribing of an adrenaline kit as an evil, but the lesser of two evils in an inadequately understood situation. If there were a safe way of prescribing less, I would welcome that with great relief.

What are the disadvantages of adrenaline (epinephrine)?

Having to keep adrenaline kits at home, at school and when out and about is a serious nuisance. Having to remember to take it wherever you go is another burden on your life.
The fact that you have been told you need the adrenaline is a constant reminder of the risk of death. If that risk is in fact vanishingly small (for example much smaller than the risks from accidents, infections, or drug abuse), then the very fact that you have the adrenaline may harm you and your family by imposing another stress on your life on top of the others which you may face.

The cost of repeated and duplicated prescriptions and the time of your specialist and family doctor and nurses at clinics and at school is not trivial. These funds could otherwise be spent on other health care.

For all these reasons we should not take the view that we may as well prescribe adrenaline just because ‘at least it can't do any harm'. It can and it does.

But if the risk of not having adrenaline is bigger than the risk from having it then we should offer adrenaline. We should then not be swayed by prejudice against the treatment, perhaps based on the fact that ‘we never did it before' (when there was not so much nut allergy) or on under-reporting of the dangers of nut allergy because the medical statistics are unreliable.

What are the flaws in the argument that adrenaline is prescribed far more often than necessary?

There seem to be flaws in the argument and research plan as we have seen it in print. This may be because the authors were asked to be brief.
The job of doctors and experts is to establish the facts as well as possible, to explain these clearly, to make recommendations where we feel that known facts justify these, but to make final decisions after letting parents tell us what their priorities are. The reason for this is that such decisions are never a question of fact alone, but necessarily involve value judgements. Experts may be no better than lay people when it comes to these. Parents and patients have a right to have their views taken into account.

The authors seem overconfident that we can count the number of deaths from anaphylaxis accurately. Our information is that Death Certificate information is not accurate for this, and that not all deaths will become known to paediatricians. Over-busy paediatricians may not be as reliable at reporting incidents as the researchers hope. Standards of expertise and practice vary so much that treatment is an inadequate indicator of severity.

Most important of all is this question. How many children is it worth issuing with adrenaline kits to save the life of one child? I have debated this with groups of parents of nut-allergic children. Always they have come up with figures which astonish me, typically in the region of 100,000 children issued with kits to save one life.

Of course these parents would change their minds if it became clear that the risk of death from adrenaline was greater than the risk of death from the allergy. But this argument is likely to be difficult, because both figures should be very low.

People will reject the argument that deaths are unimportant because they are few if safe and acceptable measures can prevent them. Society provides other examples of public insistence on safety measures which seem hard to justify on quantitative grounds.

When it comes to judging the psychological disadvantages of having adrenaline around, doctors really cannot make the decision without serious discussion with parents, and even with the children.

Our verdict

The arguments against adrenaline kits are serious ones, which need to be explored and debated. My feeling is that we will become more restrictive about adrenaline prescription as we learn more.
The most important reason why there is an argument is that we do not know as much as we need to know. Research to establish the facts is necessary.

Doctors must recognise the value judgements involved, and the right of parents in making those value judgements. As in the case of other difficult medical decisions, this will not be simple because both doctors and parents vary in their personal qualities and judgements. There will always remain instances of disagreement. But exercising humanity and good sense does not mean that a doctor must always give way to a parent.

Debate fuelled by careful research is necessary and healthy.

Other treatments for anaphylaxis

Remember that this or any web page cannot give reliable individual advice. Your own expert medical adviser will be able to give you better individual advice. You may however find the page useful for discussion.

Before adrenaline (epinephrine). (But do not delay adrenaline / epinephrine).

  • Lie the patient down if there is faintness or low blood pressure, or unconsciousness But if there is swelling in the throat with difficulty in breathing and there is not a serious problem with faintness, it is better to sit the patient up to avoid making the throat swelling worse. What if you have faintess and throat swelling? Decide which is most life-threatening. Get help quickly.
  • Ensure the patient does not choke or inhale vomit. Vomiting is especially likely if food allergy was the cause of anaphylaxis. Put the patient in the recovery position'. If you don't know what that is, ask a doctor or nurse, or a well-trained first-aider.

After giving adrenaline (epinephrine), OR some of the following may be appropriate if adrenaline (epinephrine) is clearly unnecessary but you are nevertheless concerned.

  • Get prompt medical help. In Britain, the ideal is often a paramedic ambulance to rush the patient to the nearest hospital which has an Accident and Emergency Department.
  • Antihistamine tablets or syrups are helpful for really mild anaphylaxis but unlikely to save someone's life in serious anaphylaxis. Firstly, they get into the bloodstream too slowly. Secondly they don't protect against all the things which happen in anaphylaxis.
  • Antihistamine injection: probably helps, but there is no need to carry one about; carry adrenaline (epinephrine) instead.
  • Steroid injection: Usually given by doctors for severe anaphylaxis treated in the surgery or in hospital. Probably makes no difference to saving life, but may prevent other symptoms once the emergency is over. They take four to eight hours to start doing anything noticeable.
  • Intravenous fluids: regularly used in hospital for treating the low blood pressure and bad circulation in anaphylaxis, and very important as part of the treatment.
  • Oxygen may be given by ambulance crew or doctors.
  • There are other treatments which doctors know about. There is no substitute for prompt medical care, preferably in an Accident and Emergency Department (Casualty, Emergency Room) or Children's emergency department. Often, of course, the nearest family doctor will be the only doctor you can get quickly enough.
  • Six to twelve hours observation in hospital. A small proportion of people who have anaphylaxis will have a second attack after the first one has passed. These second or ‘late' reactions can on occasion be dangerous. Just how often such reactions happen is controversial, but experts on anaphylaxis recommend that the patient should be kept under observation overnight or for at least six hours. It is surely sufficient to spend this time in a waiting area where the patient will never be left unobserved; provided the patient is reasonably well there is no need to occupy an expensive hospital bed for this.

When the immediate emergency is over

Sometimes it is not certain that the patient had anaphylaxis. There may be other explanations if the features were not typical. There are at least two useful tests which can be done during the hours after the emergency. One is to take a clotted blood sample (the doctor will know what this means) and test for sormething called ‘mast cell tryptase'. The other is to collect urine for a few hours (the exact timing is not always regarded as all that important, but emptying the bladder immediately after the reaction and then collecting for 2 or 4 hours is fine) and test this to measure something called ‘methylhistamine' (this is what histamine turns into when your body inactivates it).
Referral to a specialist is necessary if the cause of your anaphylaxis is not known and you have not seen a specialist. Whether or not the cause is known, a specialist will be able to help you to guard against future attacks.

Some medicines can make anaphylaxis worse: Beta blockers

Beta blockers are medicines used to treat high blood pressure, some heart rhythm problems, and some other conditions. Unfortunately they can make asthma worse, even when they are only used in the form of eye drops for an eye problem called glaucoma (increased pressure in the eye).
Anaphylaxis can also be made worse by beta blockers, and beta blockers will make treatment with drugs like adrenaline (epinephrine) less effective. In fact some patients who have only urticaria or angioedema (angiedema, angioneurotic edema) when off beta blockers will collapse with a drop in blood pressure when they are on beta blockers. So beta blockers can turn bearable skin reactions into dangerous reactions with shock.

 

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Posted by on May 15, 2013 in The Symptoms |

Anaphylaxys – The Movie

Anaphylaxys – The Movie

If you have suffered anaphylaxis and want to talk about your experience, or if you have questions about anaphylaxis, head for the Anaphylaxis Event forum
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Edited Video Transcript

Anaphylactic Shock, By Darbi Lara

During the course of this presentation we will

  • Describe the pathophysiology of anaphylactic shock
  • List the different causes of anaphylactic shock
  • Discuss the life-threatening complications of anaphylactic shock
  • Understand the importance of gathering critical data through physical assessment and obtaining an accurate medical history
  • and describe the treatment of anaphylactic shock

By the end of this presentation we will have gained a better understanding of the nurse's role in the early recognition and treatment at the anaphylactic shock.

Anaphylactic shock is a type of distributive shock which is the result of an allergic response within the body. It's a life threatening event.

Almost any substance can cause this severe allergic reaction within the body. These substances are known as antigens and can be introduced to the body by ingestion or injection or through the skin or respiratory tract.

When an individual is hypersensitive to a substance that has been introduced to their body, their own antibodies react to trigger an immune response. During the immune response, biochemical mediators are activated. These mediators cause a whole host of problems.

Massive peripheral vasodilation results in an increase of capillary membrane permeability wich causes vascular fluid to be lost into the surrounding tissues, worsening the hypovolemic state. Coronary vessels constricts, causing severe cardiac depression. Smooth muscle constricts, causing bronchoconstriction, intestinal, urinary bladder and uterine constriction.

Along with these more critical reactions, biochemical mediators also cause excessive mucus accretion, inflammation and cutaneous reactions, further complicating matters.

In the end, what we have is major perfusion and ventilation issues. Hypovolemia and decreased venous return lead to a decrease in cardiac output. Airway obstruction leads to poor ventilation and all of this leads to ineffective tissue perfusion, decreased tissue and cellular oxygenation, which finally results in impaired cellular metabolism.

You can see how death can result from airway obstruction or cardiovascular collapse or both. Due to the pathophysiology of anaphylactic shock, it is not surprising to see any or all of these signs and symptoms.

According to Palance critical care nursing textbook, anaphylactic shock is a severe systemic reaction that can affect any number of organ systems. The actual clinical manifestations depends on the extent of multi system involvement. Symptoms most often appear within just minutes of being exposed to the antigen, but it is possible that they may not occur for up to one hour. It's important to note that following the resolution of symptoms they can reappear during a one to twelve hour window. The patient should be vigilantly monitored during this time.

In anaphylaxis, oftentimes the cutaneous effects are seen first. These effects are most commonly seen on the face and in the mouth and throat. When a patient complains about itching and redness or hives or swelling are seen, nurses should be suspecting an allergic reaction. The patient may appear restless, apprehensive or anxious and may complain about feeling warm. They may complain about a sensation of fullness or a lump in their throats. They may be having trouble breathing or may complain of a tightness in their chests. They may complain about nausea, vomiting, diarrhea, cramping and/or abdominal pain. Careful, thorough assessment is critical in the early stages of anaphylaxis. Early recognition and treatment are key the to the patient's survival.

Along with a full assessment, it is just as important to quickly begin asking questions of the patient or family to get a clear picture of what is going on. It is critical to find out as early as possible just what is happening with the patient. Along with gathering signs and symptoms, the nurse should obtain a complete medical history. Early on in the interview with patient and family, the nurse needs to gather information about what the patient was doing just prior to the onset of symptoms. Any allergies the patient has, a complete list of any medications that the patient is currently on and any other medical conditions the patient has. All of this is imperative to getting the best medical care as quickly as possible without causing further harm.

Here is a list of some of them are common causes of anaphylactic shock, but keep in mind that there are literally hundreds of potential culprits :

  • Foods and food additives
  • eggs, milk, shellfish, nuts, food coloring, preservatives
  • Diagnostic agents
  • iodinated contrast dye
  • Biologic agents
  • blood and blood components
  • insulin
  • gamma globuline
  • vaccines
  • Environmental agents
  • pollens
  • mold
  • spores
  • sunlight
  • animal hair
  • latex
  • Drugs
  • antibiotics
  • aspirin
  • narcotics
  • local anesthetics
  • barbiturates
  • Venoms
  • bees, wasps
  • snakes
  • jellyfish
  • spiders
  • fire ants

Step one in the collaborative treatment for anaphylactic shock is to remove the offending antigen if at all possible. If it is medication or blood that it's causing their reaction, obviously the infusion should be discontinued immediately. Sometimes, however, it will be impossible to remove the cause, because it is either unknown or because they have already entered the patient's system, as would be the case with a bee sting.

The second step is to reverse the effects of the biochemical mediators that are causing all of the signs and symptoms. Here we go straight to the ABC : Airway, Breathing and Circulation, all in an effort to promote adequate tissue perfusion. Depending on the severity of symptoms, the patient may need to be intubated and mechanically ventilated. Either way, oxygen therapy will most likely need to be started. The nurse will need to start a large bore IV so that fluids can be quickly infused to increase vascular volume, thus increasing blood pressure and cardiac output. Epinephine will be given to dilate the airways, constricts the blood vessels, stabilize blood pressure and to stop the further release of those biochemical mediators. Benadryl is given to block the histamine response which will help to relieve some of the swelling, itching and redness.

Cortical steroids may also be given to prevent any additional delayed allergic reactions and to stabilize those leaky capillary membranes. Other drugs may be used to reverse the effects of cardiac depression and vasodilation.

One of the most essential responsibilities of a critical care nurse is to prevent anaphylactic shock. It is so important for the critical nurse to use preventative measures such as identifying patients who are at risk and monitoring and assessing the patient's response to the administration of medications and other infusions like blood and blood products.

The nurse should have a complete list of the patient's allergies and if possible, a detailed list of the types of responses the patient has for each allergy. The more information the nurse has, the better she will be able to care for her patients.

For the patient who is in the midst of anaphylactic crisis, the nurse will need to facilitate ventilation by positioning the patient to help with breathing and by teaching the patient to breathe slowly and deeply. The nurse will need to protect the airway through quick administration of ordered medications in order to replace vascular volume.

The nurse will need to insert a large-bore peripheral IV and quickly begin administering the ordered fluids. The patient can be positioned with the legs elevated in a sort of modified trend alan berg position to aid in venous return during the volume replacement process.

The nurse can promote comfort and emotional support by administering medications to relieve itching, applying warm or cool compresses to the skin and may need to cover the patient's hands to discourage scratching.

It is important to remember that one of the most essential responsibilities of a critical care nurse is to prevent anaphylactic shock. When anaphylactic shock occurs, it is critical that the condition is recognized early through gathering vital information through careful assessment and history taking. The nurse should always remember her ABC's to ensure the best and earliest treatment for anaphylactic shock.

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