Tryptase Test

Tryptase

How is it used?

The tryptase test is a useful indicator of mast cell activation. Mast cells are large tissue cells present in highest amounts in the skin, the lining of the intestine and air passages, and the bone marrow. They release tryptase and other substances as part of the body's normal response to injury but also may release them as part of an allergic response. The tryptase test may be used:

• To confirm a diagnosis of anaphylaxis. Anaphylaxis is primarily diagnosed clinically, but a total tryptase may be ordered, along with a histamine test, to help confirm anaphylaxis as the cause of someone's acute symptoms. This is especially true if the person has recurrent episodes and/or if the diagnosis is uncertain.
• To help diagnose mastocytosis (too many mast cells) or a mast cell activation disorder. Mastocytosis is a rare group of disorders associated with an abnormal increase in the number of mast cells, which may accumulate in the skin (cutaneous mastocytosis) or in organs throughout the body (systemic mastocytosis).

Other tests may be used to evaluate a person's health status and to help rule out other conditions that can cause similar symptoms. These may include:

• Allergen-specific IgE blood tests to help determine the cause of an allergic reaction
• Complete Blood Count (CBC) to evaluate red and white blood cells
• Comprehensive Metabolic Panel (CMP) to evaluate organ function
• 5-hydroxyindoleacetic acid (5-HIAA) urine test to rule out a carcinoid tumor that may cause similar symptoms, such as flushing, diarrhea, and/or wheezing
• Gastrin test to look for increased secretion of this hormone, which may cause stomach or intestinal ulcers

Occasionally, a tryptase test may be performed postmortem to help determine if anaphylaxis was the cause of a person's death.

When is it ordered?

Tryptase is not a frequently ordered test. Anaphylaxis is usually diagnosed without testing for tryptase, and mastocytosis is rare. A tryptase test is sometimes ordered when a person has symptoms that suggest anaphylaxis, especially when the diagnosis is not clear and/or the symptoms are recurrent. Symptoms of anaphylaxis may include:

• Flushing
• Swelling of the throat, face, tongue, and/or eyes
• Low blood pressure
• Nausea, vomiting, diarrhea, abdominal pain
• Cardiac arrhythmias
• Light-headedness or dizziness
• Difficulty breathing, wheezing
• Itching, often with visible hives
• Confusion and/or loss of consciousness

Many of these symptoms are also seen with other conditions.

This test may also be ordered when a health practitioner suspects that a person may have cutaneous or systemic mastocytosis or a mast cell activation disorder. People with such conditions may have many of the same symptoms and signsas people with severe allergies but often without any specific trigger, such as exposure to a specific food (such as peanuts) or a bee sting. People with systemic mastocytosis may have symptoms that indicate organ involvement, such as peptic ulcers, chronic diarrhea, and joint pain. There may be enlargement of organs such as the liver, spleen, or lymph nodes. There may be skin involvement with rashes or characteristic red, blistering lesions.

A tryptase test may be ordered after a person's death to help determine if anaphylaxis was the cause of death.

What does the test result mean?

Normal tryptase results may indicate that a person's symptoms are not due to mast cell activation, or there could be a problem with sample timing. With anaphylaxis, tryptase levels typically peak about 1 to 2 hours after symptoms begin. If a sample is drawn too early or late, results may be normal. If a histamine test is also performed, it can be compared to the tryptase levels. Histamine concentrations peak within several minutes of the onset of anaphylaxis and fall within about an hour. If the timing of sample collection was appropriate and neither the histamine or tryptase concentrations are elevated, it is unlikely that a person had anaphylaxis, but it cannot be ruled out.

Acutely elevated tryptase levels in a person with symptoms of anaphylaxis indicate it as the likely diagnosis.

Persistently elevated tryptase levels in a person with symptoms of mast cell activation suggest that the person has mastocytosis. Additional testing is required to confirm this diagnosis. Tryptase levels are thought to correlate with mast cell "burden" (quantity) in those with systemic mastocytosis.

Is there anything else I should know?

Mast cells contain different forms of the enzyme tryptase, called alpha (α) and beta (β) tryptase, in both inactive and active (mature) forms. Laboratory testing can be performed to measure total tryptase, which is all of the forms together, or mature tryptase. In most cases, a total tryptase is ordered, but sometimes both a total and mature tryptase may be ordered and the ratio of the two compared. As the predominant mature tryptase in the blood is usually beta tryptase, this is essentially a comparison between total tryptase and beta tryptase. Total-to-mature tryptase ratios that are less than 10 are suggestive ofanaphylaxis, while ratios greater than 20 are suggestive of systemic mastocytosis.

If systemic mastocytosis is suspected, an elevated tryptase test may be followed by a bone marrow aspiration and biopsy to determine if systemic mastocytosis is present. Typically, there are increased numbers of mast cells in the bone marrow in this disease.

Tryptase can also be elevated with asthma, myelodysplastic syndrome (a type of bone marrow disorder), acute myelocytic leukemia, and with any condition that activates mast cells.

The release of tryptase from mast cells may be triggered by a wide variety of substances, but reaction to a food is thought to be the most common cause of anaphylaxis.

Anyone can have mastocytosis, but children are more frequently affected with cutaneous mastocytosis. In children, mastocytosis is more likely to be self-limited and may be transient.

Studies have linked genetic mutations with some cases of systemic mastocytosis. One of the common mutations identified is a codon-816 C-KIT mutation. Testing for this mutation is not routine but may occasionally be performed.

Common Questions

1. Can tryptase testing be done in my doctor's office?
2. If I think I have an allergy, should I have a tryptase test done?
3. How is anaphylaxis treated?
4. If I have an elevated tryptase, does this mean I have mastocytosis?

1.  Can tryptase testing be done in my doctor's office?

No, the tryptase test is a specialized test that is not offered in every laboratory and the sample must usually be sent to areference laboratory for testing.

2.  If I think I have an allergy, should I have a tryptase test done?

Your health practitioner may order specific allergy tests to help determine the substances you are allergic to, but a tryptase test would usually be done only if severe allergic symptoms are present. Most people with allergies will never need to have a tryptase test performed.

3.  How is anaphylaxis treated?

Anaphylaxis can be rapidly fatal and requires immediate medical treatment with injections of epinephrine and other medications. This is followed by careful monitoring as it is not uncommon for anaphylaxis to recur within a couple of days of the initial episode. Those who are known to have severe allergic reactions are encouraged to carry a kit that contains an emergency injection of epinephrine with them at all times.

4.  If I have an elevated tryptase, does this mean I have mastocytosis?

Not necessarily. Tryptase is just one of the criteria that your healthcare provider will consider before making a diagnosis of mastocytosis, which is a rare disorder.

Histamine Test

Histamine Test

Why Get Tested?

To help confirm a diagnosis of anaphylaxis, mastocytosis, or mast cell activation

When to Get Tested?

When you have symptoms such as flushing, nausea, throat swelling or low blood pressure that may be due to a life-threatening allergic reaction; sometimes when your doctor suspects that you have mastocytosis or mast cell activation

Sample Required?

A blood sample drawn from a vein in your arm or a 24-hour urine collection

Test Preparation Needed?

None for anaphylaxis, but timing of the sample very soon after the beginning of symptoms is important. If testing is done for other conditions, you may be instructed to refrain from taking antihistamine and other medications. This should be discussed with your doctor.

The Test Sample

What is being tested?

Histamine is a substance that is released from specialized cells called mast cells when they are activated, often as part of an allergic immune response. This test measures the amount of histamine in the blood or urine.

Mast cells are large tissue cells found throughout the body. They are present mainly in the skin, the lining of the intestine and air passages, and the bone marrow. Mast cells are part of the body's normal response to injury as well as allergic (hypersensitivity) responses. They contain granules that store a number of chemicals, including histamine and tryptase, which are released when mast cells become activated. Histamine is responsible for many of the symptoms in persons with allergies.

Concentrations of histamine in the blood and urine are normally very low. Significant increases can be seen in people with a severe allergic reaction and in those with a disorder in which the number of mast cells increase (proliferate) and/or activate without apparent allergies.

The activation of many mast cells is associated with a severe form of acute allergic reaction termed anaphylaxis, which can cause hives (blisters on the skin), reddening of the skin (flushing), low blood pressure, severe narrowing of the air passages, and even death. With anaphylaxis, histamine concentrations in the blood increase rapidly, rising within 10 minutes of the start of symptoms and returning to normal within about 30 to 60 minutes. This increased production is also reflected a short time later in the urine as histamine and its primary metabolite, N-methylhistamine, are excreted.

Histamine and tryptase levels may be persistently increased in people with mastocytosis. This rare condition is associated with abnormal proliferation of mast cells and their infiltration and accumulation in the skin (cutaneous mastocytosis) and/or in organs throughout the body (systemic mastocytosis).

How is the sample collected for testing?

A blood sample is obtained by inserting a needle into a vein in the arm. For a 24-hour urine collection, all of the urine should be saved for a 24-hour period. It is best to keep the sample in a cool, dark place such as a refrigerator.

NOTE: If undergoing medical tests makes you or someone you care for anxious, embarrassed, or even difficult to manage, you might consider reading one or more of the following articles: Coping with Test Pain, Discomfort, and Anxiety, Tips on Blood Testing, Tips to Help Children through Their Medical Tests, and Tips to Help the Elderly through Their Medical Tests.

Another article, Follow That Sample, provides a glimpse at the collection and processing of a blood sample and throat culture.

Is any test preparation needed to ensure the quality of the sample?

If anaphylaxis is suspected, it is important to collect the sample very soon after the beginning of symptoms. If testing is done for other conditions, you may be instructed to refrain from taking antihistamine and other medications. This should be discussed with your doctor.

The Test

1. How is it used?
2. When is it ordered?
3. What does the test result mean?
4. Is there anything else I should know?

How is it used?

The histamine test is a useful indicator of mast cell activation. The test may be used to help confirm that a person has had ananaphylactic reaction, or it may be used to help diagnose mastocytosis, a rare group of disorders characterized by abnormal proliferation of mast cells.

Anaphylaxis is usually diagnosed clinically, but a histamine test may be used along with a tryptase test to help confirm anaphylaxis as the cause of someone's acute symptoms. This is especially true if the person has recurrent episodes and/or if the diagnosis is uncertain. A blood histamine test must be collected rapidly, as soon as symptoms develop.

Instead of a blood test, a histamine test performed on urine collected over a 24-hour period may be ordered instead to evaluate histamine production over a longer time frame. In some cases, the metabolite N-methylhistamine may be measured in urine instead of histamine.

Histamine testing may sometimes be ordered along with a tryptase test to help diagnose mastocytosis or mast cell activation disorder. Cutaneous mastocytosis typically only causes skin problems (particularly hives). People with systemic mastocytosis or mast cell activation disorder may experience anaphylaxis and its associated symptoms.

When is it ordered?

Histamine is not a frequently ordered test. Anaphylaxis is usually diagnosed without testing for histamine or tryptase, and mastocytosis is rare. Histamine and tryptase tests are sometimes ordered when a person has symptoms that suggest anaphylaxis, especially when the diagnosis is not clear and/or the symptoms are recurrent. Symptoms of anaphylaxis include:

• Difficulty breathing, wheezing
• Flushing
• Itching, often with visible hives
• Light-headedness or dizziness
• Low blood pressure
• Swelling of the throat, face, tongue, and/or eyes

Many of these symptoms are also seen with other conditions.

Testing may also be ordered when a doctor suspects that a person may have mastocytosis or a mast cell activation disorder. People with these disorders have many of the same symptoms and signs as persons with severe allergies, but without any specific trigger, such as exposure to certain foods (e.g., peanuts) or a bee sting. Persons with systemic mastocytosis may have signs and symptoms such as peptic ulcers, chronic diarrhea, joint pain, enlarged liver, spleen or lymph nodes, rashes or characteristic red, blistering lesions that may be present singly or by the hundreds.

What does the test result mean?

Acutely elevated histamine and/or tryptase levels in a person with symptoms ofanaphylaxis are strong evidence for that diagnosis.

Normal histamine results may indicate that a person's symptoms are due to another cause, or that the sample was not collected at the right time. With anaphylaxis, blood histamine levels rise rapidly and can fall back to normal within about 30-60 minutes. If a sample is drawn too late, results may be normal. If a tryptase test is also performed, its value can be compared to the histamine levels. Tryptase levels rise and fall more slowly than histamine levels, peaking within 1 to 2 hours of symptom development.

If the timing of sample collection was appropriate and neither the blood histamine or tryptase concentration is elevated, it is less likely that a person had anaphylaxis. However, a person can have anaphylaxis or mastocytosis without elevated histamine levels, so the diagnosis cannot be ruled out just because the test is negative.

Increased levels of histamine and/or N-methylhistamine in a 24-hour urine sample indicate an event associated with mast cellactivation. Persistently elevated histamine and/or tryptase levels in a person with mastocytosis symptoms make it likely that the person has this condition. The diagnosis must still be confirmed with other testing.

Is there anything else I should know?

Histamine may be elevated with any condition that activates mast cells, and the release of histamine may be triggered by a wide variety of substances.

An allergic reaction to a food is thought to be the most common cause of anaphylaxis.

In some people, histamine-related symptoms, such as flushing, headache, diarrhea, itching, etc., may develop after eating histamine-rich foods. Histamine can be found in a variety of foods, especially those that are "aged" such as cheese, wine, and sauerkraut. Symptoms may also be caused by ingesting alcohol, or by drugs that either stimulate the release of histamine or block its metabolism.

Rarely, histamine poisoning can occur by eating fish that has spoiled (e.g., tuna, mackerel) and has high quantities of bacteria-produced histamine. Called scombroid fish poisoning, this condition can cause flushing, sweating, vomiting, headache, and diarrhea.

Some gastrointestinal carcinoid tumors produce excess histamine.

Common Questions

1. Does histamine do anything besides cause allergic reaction symptoms?
2. If I think I have an allergy, should I have a histamine test done?
3. How is anaphylaxis treated?
4. Can histamine testing be done in my doctor's office?

1.  Does histamine do anything besides cause allergic reaction symptoms?

Yes. In addition to allergic reactions, histamine plays a role in inflammatory processes, stimulates gastric acid secretion, acts as a neurotransmitter, dilates blood vessels, increases vascular permeability (allows fluids to move through blood vessel walls), affects smooth muscle contraction in the intestines and lungs, and affects heart rate and contraction. Medications have been developed to block some of the actions of histamine, including antihistamines and drugs that reduce stomach acid secretion.

2.  If I think I have an allergy, should I have a histamine test done?

Your doctor may order specific allergy tests to help determine the substances you are allergic to, but a histamine test would usually be done only if severe allergic symptoms (such as those in anaphylaxis) are present. Most people with allergies will never need to have a histamine test performed.

3.  How is anaphylaxis treated?

Anaphylaxis can be rapidly fatal and requires immediate medical treatment with injections of epinephrine and other medications. This is followed by careful monitoring as it is not uncommon for anaphylaxis to recur within a couple of days of the initial episode. Those who are known to have severe allergic reactions are encouraged to carry a kit that contains an emergency injection of epinephrine with them at all times.

4.  Can histamine testing be done in my doctor's office?

No. Though your sample may be collected at your doctor's office, it will be sent to a laboratory for testing. Histamine is a specialized test that is only performed in large or specialized laboratories.

Food Intolerance Network FactSheet

Food Intolerance Network FactSheet

Mast cells, leaky gut, gluten, and IBS linked?

Mast cells, leaky gut, gluten, and IBS linked?

Mast cells, leaky gut, gluten, and IBS linked?

Digestive tract is the largest barrier to outside attack and only one cell thick.



Our digestive tract is the largest defensive barrier to outside attack. It is vulnerable to stress, foreign food proteins and bad bacteria. The lining of the digestive tract is protected by a single layer of epithelial cells. In the small intestine these are called enterocytes and in the colon they are called colonocytes. The small intestine is long enough to wrap around your waist about eight times whereas your colon is about as long as you are tall.


Irritable bowel syndrome (IBS), the most common digestive disorder, may be an inflammatory disease of the bowel caused by stress and leaky gut.


Irritable bowel syndrome is reported to be the most common gastrointestinal disorder. We need to start thinking of diarrhea predominant irritable bowel syndrome (D-IBS) as a disease caused by stress, food and or bad gut bacteria induced intestinal irritation resulting in leaky gut. In Gut, Guilarte et al. from Barcelona, Spain report finding mildly increased intra-epithelial lymphocytes (IELs) and marked increased mast cells in patients with diarrhea predominant IBS who also had higher levels of psychological stress than normal volunteers.


Allergy cell, the mast cell, may be the link to many causes of IBS but requires special intestinal stains.


A stress-mast cell axis has been proposed for possible cause of IBS. Mast cells release chemicals in response to triggers such as allergy and parasites typically in the body but have been linked to stress. Increased mast cells have been found in the large and small bowel of patients with IBS especially when a stain for tryptase, an enzyme specific for mast cells, is performed.


Mast cells can cause leaky gut resulting in increased pain and diarrhea in IBS.


Mast cells can increase intestinal permeability (cause leaky gut), increase visceral sensitivity (increased pain sensation and intensity) and increase motility (contractions of the intestine). This results in diarrhea and abdominal pain.


Increase in lymphocytes noted possibly explain the link to gluten noted in IBS.


The finding of increased lymphocytes or intraepithelial lymphocytosis in the patients of this most recent study was of particular interest to me. I am seeing this very commonly in my patients who do not meet criteria for celiac disease yet respond to a gluten-free diet who have previously been labeled IBS. Sometimes I find this in small intestine biopsies and sometimes in the colon of patients. Some have had a colonoscopy in the past but no biopsies were done because the colon "looked normal". In a recent post I discussed a study that noted IBS defining symptoms in over 50% of patients with microscopic colitis.


Is your digestive tract under attack and causing you ill health?


If you are not experiencing optimal health, consider the proposition that our digestive tract is now under constant attack and is frequently failing as a defensive barrier. Our enemies increasingly appear to be genetically modified or prepared foods and bad bacteria. The bad bacteria have taken over due to excess hygiene practices and liberal use of antibiotics. Our defense must include consideration of dietary changes such as reduction or elimination of gluten, eating organic foods, and the liberal use of probiotic supplements.


Copyright © 2007, The Food Doc, LLC, All Rights Reserved.


Reference:
Diarrhoea-predominant IBS patients show mast cell activation and hyperplasia in the jejunum. Guilarte, M et al. Gut February 2007; 56:203-209.

Mastocytosis Awareness Video

Our youngest daughter, Candice, was born with Mastocytosis.  It's a rare orphan disease.  Her older sister, Zoë, created this awareness video in hope for a cure!

Mastocytosis - An Orphan Disease Awareness Video

A dear friend made this mastocytosis awareness video!
My daughter Candice is in this video because she has this rare orphan disease.

Be Safe from Anaphylaxis-Mayo Clinic Video

Not all anaphylactic reactions are severe, they can be mild with subtler symptoms.  Most people don't know that if you've had a mild reaction in the past you are at risk of having a life threatening one in the future.

International Chronic Urticaria Society FAQ

International Chronic Urticaria Society FAQ

Frequently Asked Questions

• What is chronic urticaria? (CU or CIU)?
• How long does CU last?
• What produces hives?
• What causes CU?
• How can I figure out the cause of my CU?
• How do I find out if it's autoimmune?
• What if I am not autoimmune?
• How can CU be controlled?
• What are H1 and H2?
• What other treatments are there?
• Will I have to stay on these medications forever?
• What can I do for immediate relief of itching?
• Are there any natural remedies for CU?
• How can I help manage my own care?
• How can I live with this?

What is chronic urticaria (CU or CIU)?

Chronic urticaria (CU) is a type of hives, also called nettle rash, that lasts for more than six weeks. Shorter bouts of hives are considered acute urticaria, and are treated differently.

Urticaria can be a manifestation of many conditions and illnesses, rather than one illness.

CIU stands for chronic idiopathic urticaria. The term idiopathic simply means 'of unknown cause'. The diagnosis is often made by ruling out known causes, but some causes, like mastocytosis, cannot absolutely be ruled out. How much your physician rules out depends on his or her personal diagnostic talent, knowledge and willingness to test. 
Back to top of page

How long does CU last?

There's no easy answer to this question. Some people have it for a year or so, after which it goes away, never to return. Some people have remissions of as much as thirty years between episodes. Some people suffer from it their whole lives. The answer also lies in finding out possible causes. Some people are lucky enough to discover and treat the underlying cause well enough to make the hives go away. 
Back to top of page

What produces hives?

Mast cell degranulation is the mechanism behind hives. In simple terms, certain little white blood cells are going bonkers and firing histamine. Mast cells fire in response to antigens (invaders, real or perceived) that come into your body either by contact (skin), inhalation or digestion. Antigens are also produced within the body by other factors, such as inflammation or systemic illnesses such as cancer, thyroid disease or lupus.

Besides histamine, mast cells also fire heparin, chrondroitin sulfates, neutral proteases, acid hydrolases and other enzymes. On the surface of these mast cells are little receptors for IgE (immunoglobulin E antibody). Think Velcro®. When an antigen comes into the body it "sticks" to the IgE receptors. When the mast cell gets loaded it starts firing its weapons(degranulating) and it encourages other mast cells as well as other inflammatory cells (basophils and leukotrienes) to fire also.

As the mast cells fire, they release histamine. Histamine and other mast cell byproducts cause
vasodilation (where the capillaries increase in diameter), which in turn causes the blood vessels to leak fluid into surrounding tissues. The histamine infusion into the tissues produces hives. 
Back to top of page

What causes CU?

CU can be caused by a great many different things. Generally, chronic urticaria is either autoimmune (a primary autoimmune disease all its own) or a symptom of something, it is just a matter of finding what that something is. Approximately half of CU cases are autoimmune.

CU may be related to an autoimmune problem such as lupus, thyroid disease, or multiple sclerosis. It may be an allergic reaction to something (although if the hives are chronic this is unlikely). If you have had hives for longer than six weeks, you may not discover the cause. This doesn't mean you should stop looking for the cause, but knowing this can help you focus on managing your symptoms and living with the condition on a daily basis. 
Back to top of page

How can I figure out the cause of my CU?

If you want to pursue finding the cause, keep in mind it may take a long time to pinpoint, and it probably won't be easy to do. And you may never find the underlying cause. You will have to be your own detective and advocate to find it. If it means changing doctors, so be it. Listen to your body and keep a detailed diary of what goes into your body, what you are exposed to, and what your symptoms are like each day. A pattern may emerge. Several people with mastocytosis/mast cell activation syndrome have also reported that their symptoms began with an 'event' or trauma, like that seen in autoimmunity. 
Back to top of page

How do I find out if it's autoimmune?

One of the first tests you should have is something called the autologous serum skin test (ASST). This test is still not widely available, so it may take some hunting around to find a local clinic or hospital that offers the test. The ASST involves taking a sample of your blood, spinning it down in a centrifuge to separate the serum, and then injecting the serum back into your arm. A wheal/flare response to the injection is considered positive for autoimmune CU.

Some things many people with autoimmune disease have in common:

• For women, a worsening of symptoms during periods
• Remissions during pregnancy
• Development of other autoimmune diseases
• Family history of various autoimmune problems (one family member may have rheumatoid arthritis, another may have thyroid disease, still another may have endometriosis)
• Onset of symptoms with an event or trauma such as accident, illness, surgery, or infection within approximately 6 months—something that kicks the immune system into high gear.

If you've experienced any of these, you may indeed have autoimmune CU, and this warrants further investigation. 
Back to top of page

What if I am not autoimmune?

Here are a few things to consider:

• Can you take aspirin or other NSAIDs (non-steroidal anti-inflammatory drugs)? If not, you may be salicylate sensitive. Salicylates are the active ingredient in aspirin, and are found in all plant matter to some extent (fruits, vegetables, herbal supplements, etc). If you find that taking aspirin makes your hives worse, you may have discovered your cause. This is also true if you cannot tolerate other NSAIDs such as Ibuprofen or Aleve, because they are cross-reactive with salicylates. A low salicylate diet may help.
• A few people have found that a yeast-elimination program (diet and medications) has helped reduce their flares.
• You may be sensitive to additives or dyes or preservatives. Again, you have to be your own detective. Pay attention to what is happening, and your body may give you clues.
• Have you had a complete thyroid workup? It seems to be quite common that people with several autoimmune diseases also have thyroid trouble. If you have a thyroid disorder, you may find that the CU improves with appropriate thyroid medications.
• Have you ever had a root canal done? Some people in our group have had CU go away after being treated for infected root canalled teeth.
• Do you have any other possible sources of infection? Potential culprits are kidney infections, sinus infections and gallbladder infection.
• Hepatitis B and C have also been implicated in CU.
• Have you been tested for other autoimmune conditions? While the majority of autoimmune CU is a primary illness, hives can also be a symptom of some other autoimmune diseases, such as lupus. The most common screening test is ANA, which looks for some specific autoimmune diseases, such as lupus. However, it is possible to have a negative ANA and still be autoimmune. A diagnosis is made based on a combination of symptoms and lab results. One excellent starting place to learn more about autoimmune diseases is the American Autoimmune Related Diseases Association website.
• Do you have any other symptoms besides hiving? Stomach problems, slow healing, sinus problems, headaches, normally low temperature (less than 98) or low blood pressure, anything at all, whether you think it is related or not? The more you know about your symptoms and the medications you are currently taking, the better able you will be to manage your CU.
  Back to top of page

How can CU be controlled?

The principal approach is to control mast cells, by controlling either the IgE stimulation or the histamine being leaked. Granted, this treats the symptom and not the cause, but until a cause is determined, it is in your best interest to try to gain some control over the symptoms. It's a matter of working with your doctor to find the right combination of medications for you.

Because skin contains receptors for H1 and H2, treatment frequently involves taking both an H1 and H2 antihistamine.

Back to top of page

What are H1 and H2?

Mast cells release 3 known types of histamine, H1, H2, H3. It is believed there are also H4 and H5 histamine. Skin has receptors at least for H1 and H2. Airways have receptors for H1, the gastrointestinal (GI) tract has receptors for H2, and the brain is believed to have H3
receptors.

The H1 drugs are those commonly thought of as antihistamines, such as Zyrtec, Benadryl, Claritin, Allegra, and Atarax. The H2's are normally thought of as ulcer medications, but are actually histamine 2 blockers. Zantac, Pepcid, Axid and Tagamet are the most common. 
Back to top of page

What other treatments are there?

Some people also benefit from the tricyclic antidepressant Doxepin. For CU, it is prescribed at a much lower dose than for depression, and works as both an H1 and H2 blocker.

Still others find that drugs like Gastrocrom, Zyflo and Accolate help. Gastrocrom can also be made into a cream that helps the itching for many people.

Ephedrine sulphate or inhaled epinephrine can help some symptoms, especially for those with mast cell disease. Inhaled epi (Primatene Mist) is also used by some shockers as a first line of defense during anaphylaxis.

Another drug some people respond to is Procardia (which is actually a heart medication).

If your hives are due to autoimmunity, immunosuppressant drugs such as colchicine or Imuran (Azathioprine) may help.

And if you have ever experienced symptoms of anaphylatic shock (especially drop in blood pressure, throat swelling/tightness, trouble breathing), an Epipen® (emergency shot of epinephrine)—best to have at least two—is a MUST. 
Back to top of page

Will I have to stay on these medications forever?

You may find that over time, the drugs you are taking aren't as effective. Talk to your doctor about switching to something else, or changing the combinations you are taking. 
Back to top of page

What can I do for immediate relief of itching?

Here is a list of a few things that may help you. Before you slather your whole body with any of these, test it on a small area of skin away from your face, preferably for about three days. Many people are more reactive to any new substance when they are in a flare, so be cautious!

• Cromolyn cream is something you can make yourself using any form of cromolyn (Gastrocrom, Nasalcrom, Intal or cromolyn eyedrops). The recipe for it was developed by a nurse and pharmacist who are both mastocytosis sufferers, and many people swear by it.
• In a pinch, you can spray some Nasalcrom directly on an especially itchy spot of skin for instant relief.
• MSM lotion or cream works very well for the itching for some people.
• Other lotions and creams that have helped many include Sarna™ Lotion, Aveeno™ Lotion in the green bottle, Aveeno™ Oatmeal Bath, tea tree gel, and several other lotions which contain menthol.
  Back to top of page

Are there any natural remedies for CU?

A number of people with CU have experienced relief using a variety of natural treatments. the list of possible treatments and remedies is quite long and results have been variable. Remember that many people with CU are very sensitive to certain chemicals, such as salicylates, and some of these chemicals may be found in natural remedies. Always proceed with caution when trying any new treatment, and collaborate with your medical professional.

Note: This post to the Urticaria Yahoo group reviews several possibilities. (You must be a member of the group to open this link. )

Back to top of page

How can I help manage my own care?

Every time you see your doctor, ask for a photocopy of every test and his notes. This is your right as a patient. If you have to change doctors, get a second opinion, or try to keep the facts straight, having your own file will help.

If you find a medical journal article about a kind of hiving you think might be the same as yours, copy it and take it to your doctor. He has to include it in your medical file at your request—another of your rights as a patient. 
Back to top of page

How can I live with this?

Never give up. You are going to be either your best friend or your worst enemy in this thing. Sometimes that choice will be made daily, hourly or by the minute. And take a look at our Useful Informationpages for some practical help.

The ICUS email list is a wonderful support group where you will find many others who may be able to help and will certainly listen to your joys and sorrows. 

23andMe Myeloproliferative Neoplasms Research Initiative

I was contacted some time ago by a person from 23andMe that had seen this blog and sent me an email regarding Candice having Mastocytosis and asking if I would be interested in having her participate in their Research Initiative for Myeloproliferative Neoplasms.  I was hesitant to have Candice participate but after some discussions with my family and other mastomoms I decided it couldn't hurt for her to participate, after all we are all in hopes for a cure!

Now, several months later, I have been contacted by a few family members via 23andMe family finder.   I am still amazed that thing works! It is getting quite interesting.  There seems to be the possibility of some family members having mastocytosis.  There are many symptoms that are in common with one another.  I hope that with us all working together we can help each other find some much needed results for medical and other family issues.

I am thankful that Candice participated in 23andMe.

A little about the program:
23andMe, a personal genomics company, recruited patients with myeloproliferative neoplasms (MPNs), including all forms of mastocytosis, for a research study.  The project aimed to identify differences in the human genome that might predispose a person to a MPN.  23andMe provided eligible participants explanations of how their genomic data relates to a list of currently analyzed diseases and traits and to explore their ancestry.  It's a lifetime membership.

The research project info: https://www.23andme.com/mpn

Emergency Medical Binder

Candice has an emergency medical binder.  She has one at school that the nurse keeps in her office for reference and for anyone that comes into contact with Candice to read (i.e. teachers, substitute teachers, etc).  I have one for myself that I take to doctor appts and keep readily available in case we have to go to the Emergency Room so that anyone that will treat her will have access to.  All of Candice's doctors and dentists have copies of all important information that is in the binder and pertinent to her treatment and/or prevention of a flare up.

In this binder I have:

*A cover page that says Emergency Medical Information and her name.
*On the inside cover I have a picture of Candice for in case she has a substitute the sub will know who she is by sight.
*Letter from her allergist that treats her for mastocytosis stating that she is treating Candice for mastocytosis, explaining what mastocytosis is, possible and known triggers for Candice, medications Candice takes in an attempt to prevent a flare up, also other preventatives that Candice needs to have access to at all times such as: cool mist fan, cool vest, cool packs, cool cloths (i.e. frog togs), and iced water bottles or thermos, etc.  Her doctor also provided an Action Plan for the school and Emergency Room doctor with signs of a reaction/flare up, action for initial minor reaction/flare up, action for major reaction/flare up, and her emergency number.
*Candice's medic alert information.
*Information provided by Candice's allergist stating Treatment and prognosis of mastocytosis.  This gives information on what mastocytosis is and general management of mastoytosis.
*Emergency medical information such as her name, date of birth, and that vaccines are up to date.  Along with her diagnosis, medications, known allergies and triggers, possible degranulators/triggers, prior history such as illnesses and hospitalizations, pediatrician information, allergist information treating her for mastocytosis, and all emergency contacts.
*Type of mastocytosis she has, categories of her reactions such as mild, moderate, and severe and what to do during each of these.  Stating that she usually feels when a reaction is coming on and will inform someone and to please send her to the nurse asap or if anyone notices anything not "right" with her to send her to the nurse.
*A little about Candice and how a typical reaction for Candice may look and when to send her to the nurse and what medications are to be administered and when.
*Pictures of reactions she has had in the past such as lesions swelling, filling with fluid, bursting and flushing.
*A link to my blog about Candice and mastocytosis.
*Description of Mastocytosis, Symptoms, Treatment, and link to TMS and NIH websites regarding mastocytosis.
*Dental management of a pediatric patient with mastocytosis that I printed off from the TMS website.
*Emergency Room Protocol that I printed off from the TMS website.
*Any and all doctor letters regarding Candice and mastocytosis.

Cool Vest

Candice started Kindergarten last year and I knew that outdoor activities such as recess and P.E. would be an issue, especially here in hot and humid Houston, TX.  I wanted her to have a cool vest to use so that she could still participate most of the time and not feel left out.  At the time we were trying to get our insurance to help cover some of the cost as they are pretty expensive.  It was an ongoing long process.  So while I was still trying to get them to help I decided to make a temporary one.

I purchased a child size life preserver. I took out the flotation devices and put velcro in the bottom of the vests back and sides where the flotation devices had been to create pockets.  She could wear the preserver over her clothing and it had fasteners that could be tightened and/or loosened if needed.  I also purchased some cool packs that could be frozen and last up to about an hour at a time.  The cool packs could be inserted and removed from the pockets because of the velcro.  She was able to wear this and participate in outdoor activities for her Kindergarten year, unless the heat was extreme and then she would participate with another class in either Art or Music class.  She didn't feel left out and had a great year!  Below is a picture of the homemade cool vest.

Unfortunately we were not able to get our insurance company to help out with expenses and Candice outgrew her cool vest therefore needing a new cool vest.  Candice's grandma Marion and grandpa Joe bought her a cool vest this past year that she should be able to wear for some time and came with sets of cool packs that last up to about 8 hours at at time!  She is using it this year at school, her first grade year, and is able to participate in recess and P.E. even with it being very hot and humid here.  She really loves it!  Below is the link to the cool vest.  We are truly thankful for this cool vest!


Cool Vest