Monday, August 11, 2008

Welcome to my world!

I was inspired today by a couple of fellow survivors who live with Mastocytosis, I ran across their blog on The Mastocytosis Society website. I thought to myself you know this could be very helpful to people like us. I decided to start my own blog sharing with anyone who felt my ramblings worthy of reading.

I can remember back when I had my first anaphylactic attack brought on my Systemic Mastocytosis. I was driving to work one morning when I started feeling very nervous. I looked into my rear view mirror and noticed my entire face, neck and arms had turned beat red. Talk about scaring the living crap out of me, I floored my accelerator and begin my 5 or so mile race to the hospital. After my 2 week stay and being treated like a lab rat I was released with no diagnoses.

I could share hundreds of stories over the past 11 years but that would take me weeks to write. I will say this The Mastocytosis Society and the people who founded that site saved my life literally. I have a great deal of respect and love for these people, there is NO place with more information regarding Mastocytosis.

I will be discussing my study with Dr. Srdan Verstovsek, MD, PhD at M.D. Anderson in my upcoming blogs.

I look forward to hearing from you so please leave a comment.




jennifer said...

hi, I have mastocytosis and u/ name is jennifer and I live in n.y..I know most of the trails are done at m.d anderson and your doctor runs most of them.IF you dont mind ,,which one are you in,,I was suppossed to start gleevec out here but my mutation testing came back i have the c-kit mutation and the jak 2,, I know your dr.v is running both trails for those mutations,,Im just hoping these trails look promising,,good luck with your treatment,,,,thanks

Kevin Zapatero said...

Hi Jennifer,

Sorry for taking so long to get back to you. I'm currently taking DASATANIB (da-SA-ti-nib)

As you are probably aware this is basically chemo in a pill. I'm currently taking 40mg in the morning and 40mg at night. I still have some severe stomach cramping in the morning from the chemo or it could just be part of our illness since it's so common among people like us. I also have the c-kit mutation. Thing you have to remember Jennifer is we are all different to some degree just because something may work for you it may not work for me, unfortunately we have to learn by trial and error. At least this is what I've found to be true in my 11 years of shocking with SM.

I've been recesitated 22 times over the past 11 years. The past 3 years I've had some complications along with some close calls, meaning I thought I may go into shock usually because of stress or something I ate winded up having preservatives in it when it should not have.

What I'm trying to say is I've been shock free for the first time in 11 years since I've been on this study at M.D. Anderson. Do I think I'm cured of course not am I still sick in the mornings yes, do I still have some affects caused from SM such as bone pain unfortunately yes. When you've learned to live with our illness as long as some of us (I have no idea how long you have lived with yours so this is not a punt towards you) you learn life is short and you have to make the best of what you have.

I'll be posting some more info on my main page of my blog regarding my life how I live day to day then and now. I'm also going to share my study at M.D. Anderson.

I look forward to talking with you more, please if you think I can help don't hesitate to ask.

Thanks for writing and reading my ramblings.


jennifer said...

Thanks Kevin, Im still holding my own ,,hope you are doing well and will check back,,,jennifer

Anonymous said...

Thanks for sharing your experiences. It helps to see that there are others out there fighting with masto and doing well. Take care, Bill in Michigan

robin said...

i am so glad i found you all my boyfriend has masto, he takes glevec, he has all the syptems you all do, he is also on different types of allergy meds he is also so depressed, he has had it about 6yrs now, all i want to know can you die from it please get back with me robin in virginia , e-mail robinknight649@GMAIL.COM

Kevin said...

Hi Robin,

I sent you a email, I hope it helps. I'm glad you found my blog if I can ever be of assistance just ask.

Take care,


Information Sheet for Patients and Caregivers!

The Mastocytosis Society,Inc. Information Sheet for Patients and Caregivers

This is a brief introduction for new patients and doctors who are unfamiliar with the management of mastocytosis. The information presented here, combined with that available in the cited references, provides a starting point from which to approach understanding, treating, and living with this rare disorder.

Mast cell disease, or mastocytosis, is characterized by the proliferation and accumulation of mast cells in a variety of tissues and can affect either sex at any age. Definite diagnosis is made by demonstrating an abnormal accumulation of mast cells in a biopsy, usually of the skin and/or bone marrow. Other causes for symptoms should be ruled out, and blood and urine testing for mast cell products may be suggestive of the diagnosis. When performed properly by experienced personnel with access to current information on recommended protocols the results of these tests will be useful in diagnosing and evaluating mastocytosis.

Mast cells are widely distributed in nearly every organ of the body, mainly close to blood and lymph vessels, nerve endings, and skin and mucous membrane surfaces. They develop from immature cells produced in the bone marrow, which migrate to the tissues where they mature.

Mast cells produce various chemicals which normally serve protective, inflammatory and regulatory functions as they interact with white blood cells and tissues. In mastocytosis these chemicals, or mediators, are abnormally abundant and cause symptoms.

Mast cell products (mediators)

Some mast cell products are stored in granules within the cell, and others are produced in response to stimulation by the immune system or by drugs, chemicals, or physical factors. Below is a table of some factors which can cause mast cells to release their products. Stress, strong emotions and estrogen can increase their effect.

The products present in granules and ready for immediate release on stimulation include histamine, heparin, tryptase, and chymase. These chemicals cause, and to some extent regulate, allergic and inflammatory changes, and are involved in tissue building or repair. In response to immune system activation of the mast cell, arachidonic acid within the cell is converted into prostaglandin D2 and leukotriene C4, which restrict air flow in the lungs, stimulate mucous formation, and attract some kinds of white blood cells.

Mast cells also generate several cytokines, which are proteins that interact with white blood cells and tissue cells to continue the allergic or inflammatory response.


The symptoms of mastocytosis vary from person to person and may occur as "attacks" or as simply fatigue and a feeling of ill health. Over time, symptoms may become more frequent and more severe. The rate of progression differs from person to person, and there may be an improvement in symptoms for long periods of time. The type and severity of symptoms can also vary greatly from person to person or from one episode to the next. Often, seemingly unrelated symptoms comprise an individual's personal pattern of mastocytosis. These may (or may not) include: flushing (temporary skin redness), itching, hives,bruising, and skin sensations such as tingling. Other symptoms experienced by many of the people with mastocytosis are nausea,vomiting, abdominal cramping, occasional or frequent diarrhea, and excess stomach acid or ulcers. The person may experience unexplained fractures, mild to severe pain in bones, joints, or muscles, enlargement of liver or spleen, bladder pain, heart palpitations or rapid heart beat, chest discomfort, shortness of breath, light-headedness, fatigue,weakness, weight loss, respiratory symptoms including asthma. There may be depression, poor memory or irritability; also intolerance to heat or cold or to a change in temperature. Other symptoms include headaches,fainting or near fainting, and recurrent anaphylaxis.


Because there is not yet a cure for mastocytosis, treatment is aimed at reducing the frequency and severity of the release of mast cell products and at countering the effects of mast cell products which are inevitably released. Most patients will achieve relief of symptoms only by employing measures in both areas.

Avoidance of the factors which are known to cause a reaction for the individual patient, and cautious evaluation of other factors, such as those listed in the Table below, are important in the management of mastocytosis.

In using drugs to counter the effects of mast cell products,treatment must be tailored to the individual patient. Most commonly, H1 antihistamines such as chlorpheniramine or hydroxyzine are used to decrease the skin symptoms, vasodilation and mucous secreting actions of histamine. Stomach symptoms generally respond well to H2 antihistamines, such as cimetadine or ranitidine, which can also help reduce skin symptoms.

Aspirin or other non-steroidal anti-inflammatory drugs, if tolerated by the patient, provide relief from flushing and lightheadedness by blocking the body's production of prostaglandin D2. These drugs can cause unexpected severe reactions, though, and their use must be instituted cautiously under careful medical supervision.

Cromolyn (disodium cromoglycate) is a mast cell stabilizing drug which is frequently effective in reducing skin and gastrointestinal symptoms as well as mental and other systemic systems.

For severe diarrhea or malabsorption, collection of fluid in the abdominal cavity, and continued anaphylaxis which fails to respond to other measures, systemic corticosteroids may be required.

People with mastocytosis should carry injectable epinephrine, and they should know how to inject themselves if necessary to treat anaphylaxis.

There are other medications which may provide symptomatic relief if the ones mentioned above are ineffective or undesirable for a particular patient. No new medication or remedy should be started without careful consideration and close supervision, in case of an unexpected reaction.


It is not yet possible to predict the course of mastocytosis in any individual person. When involvement is limited to the skin, symptoms may improve or clear entirely, but it is also possible for the disease to progress to the systemic form. In about half the young children affected, symptoms disappear as they reach adulthood.

When the mast cell infiltration is systemic, symptoms may progress slowly over many years or may suddenly increase temporarily or permanently. The patient may even progress to the more serious categories of disease.

For the small percentage of patients who develop an associated hematological disorder, the course varies, and the prognosis depends on the associated hematological disease. ( 2 )

It is very important for the patient and the doctor to maintain good communication and to work cooperatively to achieve the best possible symptom control. Communication with other doctors caring for mastocytosis patients, and with other persons who have the disease, is important in order to maintain a support and information network. The affected person, or the child's parents, should strive to become educated about mastocytosis and to be aware of their individual needs and responses to triggering factors. A recognized medical warning device, such as a MedicAlert bracelet, should be worn, and extra caution is needed when undergoing dental work or surgery.

The Mastocytosis Society provides education and support to people with all forms of mast cell disease and their doctors, encourages research, and hopes to help find a cure for the disease. The Society also maintains a list of consultants who are available to advise professionals caring for a mastocytosis patient. Because of the rare nature of mast cell disease, we encourage doctors and patients to register with the Society in order to facilitate the communication and information exchange which will hasten the achievement of a cure.

Classification (adapted from (1) )

The following is a useful classification of the kinds of mastocytosis.

Cutaneous mastocytosis: Skin involvement only. This may include:

Urticaria pigmentosa: The typical rash of mastocytosis in the skin.

Solitary mastocytoma - a clump of mast cells restricted to a small area of the skin.

Diffuse cutaneous mastocytosis - skin involvement without urticaria pigmentosa or telangiectasia macularis eruptiva perstans (distinctive patches of discolored skin with small blood vessels on a reddened background. If the skin is heavily infiltrated with mast cells, the release of large amounts of mast cell products may cause systemic symptoms.

Systemic mastocytosis with or without skin involvement: Mast cell infiltration of at least one internal organ (like bone marrow or gastrointestinal tract). (2)

Mastocytosis in association with hematological disorder, with or without skin involvement: For example: leukemia, lymphoma, and myelodysplastic or myeloproliferative disorders.

Lymphadenopathic mastocytosis with eosinophilia, with or without skin involvement: Enlarged spleen and lymph nodes, infiltrated with mast cells, along with a blood count high in eosinophils.

Mast cell leukemia: This is rare but is the most serious form of masocytosis. The treatment and course of the disease is dictated by the leukemia.

Table of mast cell degranulators (adapted from (3) )

Allergens and other immunologic stimuli


Physical stimuli (heat, cold, sunlight, friction, pressure, vibration)

Bacterial toxins

Venoms (snake and insect, especially bee and wasp stings)

Biologic polypeptides (released by intestinal roundworms, jellyfish,crayfish, and lobster)

Polymers (Compound 48/80, dextran)

Drugs: Aspirin, alcohol, narcotics (codeine, morphine), polymyxin B,amphotericin B, D-tubocurarine, quinine, iodine-containing radiographic dyes, scopolamine, gallamine,decamethonium, reserpine.


1. Dean D. Metcalfe. Classification and Diagnosis of Mastocytosis:Current Status. J Invest Dermatol 96: 2S-4S, 1991.

2. Linda Golkar, Jeffrey D Bernhard. Seminar: Mastocytosis. Lancet 1997; 349:1379-85

3. Jack Longley, Thomas P. Duffy, Steven Kohn. Continuing Medical Education: The mast cell and mast cell disease. J Am Acad Dermatol 1995;32:545-61

Last update 6th December,2004