Mastocytosis Triggers/Mgt

What is Histamine?

Histamine is a natural substance produced by the body and is also present in many foods. It is released by the body during times of stress and allergy.

Histamine in Foods
There is quite a few foods that are known to contain histamine or can cause the body to release histamine when consumed. Histamine in food may be responsible for some cases of food intolerance.

No food should be excluded from your diet unless your dietician and doctor approve it first. This is to ensure your diet is not lacking in essential vitamins and minerals.

Foods that are rich in Histamines:

Anchovies
Aubergine
Avocados
Beer
Canned Foods
Cheeses
Cidars
Fermented Beverages
Fermented Foods
Fish
Herring
Jams and Preserves
Mackerel
Meats
Processed Meats
Salami
Sardines
Sauerkraut
Sausage
Some Oriental Foods
Sour Cream
Spinach
Tomatoes
Tuna
Vegetables
Vermouth
Vinegars
Wines
Yeast extract
Yogurt

Histamine Releasing Foods:

Alcohol
Bananas
Certain Nuts
Chocolate
Eggs
Fish
Milk
Papayas
Pineapple
Shellfish
Strawberries
Tomatoes 

What are Triggers?

Triggers are stimuli that can set off a mast cell response, potentially leading to a mast cell attack. Avoidance of various triggers (things that can set off a mast cell attack) can do much to improve quality of life and reduce the need for medication, but that is often easier said than done, as the triggers can be almost anything, including:

Alcohol
Friction

Anesthetic agents
Heat

Antibiotics
Infection with viruses, bacteria or fungi

Bacteria or fungi
Mold

Certain foods
MSG

Cold
Narcotics

Colors & flavorings in foods
Perfumes

Colors & flavorings in medicines
Pesticides

Emotional upset
Plasma expanders (i.e. dextran)

Environmental toxins
Preservatives

Exercise
Room freshener sprays

Fatigue
Stress

Fever
Sunlight

There is great variation from person to person in what is a trigger, and even within the same person. The triggers may change day-to-day - that is, heat may set off an attack on one day, but not on other days. The above list is not complete, but is meant to show the wide range of triggers that affect mast cells.

Some people with the indolent form of Mastocytosis, and/or people with Mast Cell Activation Disorder or Syndrome, have been told by some physicians that they can expect a nearly normal life expectancy, as long as they keep themselves as stable as possible by avoiding triggers and taking medications as prescribed. However, since it is not clear what is causing mast cell activation in patients with MCAD, this may not apply. Mast Cell Diseases are extremely unpredictable, and some people can very quickly develop acute symptoms that may require immediate medical attention. That is why it is advisable to stay within range of a medical facility, and to carry a written protocol from your Mast Cell Disease specialist for emergency care. 

Treatment/Management

Treatment of the symptoms occurring in pediatric mastocytosis consists of the prevention of exposure to agents that are known to cause symptoms in that child, and the use of medications if necessary. “Management” is at least as important as “medication”.

When symptoms are present in children with mastocytosis, it is often beneficial to treat them with medications at the time of symptoms, or with routine doses of medication if symptoms are frequent.

H1 antihistamines, such as are normally associated with relief of allergic symptoms, are the first treatment used, and can relieve most of the symptoms present in pediatric mastocytosis. It may be necessary to try several different H1 antihistamines, using each for several weeks before evaluating its effect.

H2 antihistamines, such as Pepcid (famotidine) and Zantac (ranitidine) may relieve skin symptoms that continue in spite of adequate treatment with H1 antihistamine. They also relieve symptoms from excessive stomach acid or gastric reflux.

If a child is taking both H1 and H2 antihistamines in adequate doses and still experiences frequent symptoms, there are several other medications used, depending on the symptoms.

Cromolyn (disodium cromoglycate) reduces mast cell activity. It can be used orally for GI symptoms, by inhalation for respiratory symptoms, as a nasal spray to reduce congestion, as eye drops to relieve itching and swelling of eyes, and as a topical cream to relieve skin symptoms. Cromolyn is absorbed into the system through both oral and inhalational use and, although absorption is minimal, children often experience a reduction in many of their symptoms.

Children with severe and symptomatic skin involvement (such as those with extensive urticaria pigmentosa and those with diffuse cutaneous mastocytosis) may be helped with light treatments in conjunction with taking a medication called psoralen. This treatment is referred to as PUVA, and reduces substantially the number of mast cells in the skin.

Leukotriene receptor blocking agent montelukast (Singulair) may be helpful when respiratory symptoms remain in spite of all of the above treatment, especially in children who have asthma along with their mastocytosis.

Other drugs are available for various symptoms and may be tried in consultation with a mastocytosis-experienced specialist. Aspirin and other non-steroidal anti-inflammatory drugs can cause sudden onset of severe symptoms in people with mastocytosis and are especially contraindicated in children with diffuse cutaneous mastocytosis because of their ability to cause gastrointestinal bleeding.

The emergency use of epinephrine (adrenaline) injection is controversial in very small children, for whom the available single-dose injectors would result in overdose. In children whose body weight is adequate for the use of EpiPen Jr., having one on hand and instructing the parent or caregiver in its use is an important safeguard if severe symptoms, such as loss of consciousness and shock, occur. These severe symptoms are possible in all forms of pediatric mastocytosis, and consultation with an experienced physician as to the use of EpiPen should be undertaken as soon as a child is diagnosed with mastocytosis.

Conclusion:

A diagnosis of pediatric mastocytosis may present challenges for the child and the family involved. Children with “spots” need to be reassured about social contacts, and children who experience obvious symptoms need the protection of having the adults around them educated in how to manage and treat these symptoms. Organizations such as Mastokids.Org (www.mastokids.org) can help families and affected children select useful techniques for coping with the disease while helping the child live as normal a life as possible.