Monday, November 9, 2009

Small update – Combo Post: Friends / Problems

When I first started this blog I felt kind of silly posting about my illness and problems. I was like who cares about my problems, who in the world is going to actually read this stuff? To my surprise I’ve had the pleasure of talking to half a dozen wonderful people regarding Masto, not only are they awesome people and I feel better for knowing them it’s made myself worth shoot through the roof to feel like I’m actually helping people. I find that a lot of you are facing the same problems I faced early on with this disease. You have no clue in the beginning as to what is happening you just know this is not in your head like so many Doctors, Family and even friends may try to make you think. They’ll say things like you look fine to me stop being lazy, they may say things like it’s all in your head go see a shrink, I’ve had one Doctor recommend I read this book to name just a few.

I’ve been feeling really well for about 3 years now since I started the Dasatinib (chemo in a pill form those that do not know) a study Dr. Verstovsek allowed me to take part in. Now don’t get the wrong impression I still have some problems. Recently I’ve had about 3 near misses where I was concerned I may go into anaphylactic shock. I’ve been battling with what appears to be gout, it’s the ball of my foot around my big toe and boy is it painful. I went in for it about two weeks ago and they put me on steroids masto patients best friend, even after taking all the pills it began to get worse, sharp pain along with lots of swelling, I could hardly walk on my right foot. After coming home Sunday from deer hunting, I went back to see my Doctor she needed to deaden the area and then poke a large needle kind of like how they drain a knee of fluid. Let me tell you something call me a wuss that was some serious pain. She drained a very large amount and I should get the results tomorrow. I let out a holler that the entire clinic took notice. I was stung in three different areas by some unknown insect that left me itching like a mad man, that all seems to be clearing up now.

I hope everyone is doing well I apologize for not writing something sooner I’ve just been very busy with going to court, taking my girls to volleyball practice, out of town games and now Basketball has begun along with the rest of deer season. You women like to shop and we country boys love to hunt.

I would love to hear from everyone just to see how you are all doing.

Your friend,

Kevin

16 comments:

Faith Berry said...

Kevin, I love your blog! I am so happy to see a man writing about Masto! I always know where to send other men that pop up with masto issues from time to time.

Stay well and keep inspiring us all.

Gypsy Princessa said...

Me too....Faith said it well!

And so do you...((hugs))

Amanda said...

Hi Kevin,

I found your blog and have recently met many others (mostly women) with Masto. I have SM and was diagnosed this year around Christmas time. So far, it's been no big deal for me and symptoms, but I worry about my future. Things that I read about keep me worried I guess. I seem to be able to eat what ever I want, drink a Mike's Hard (the only drink I like) and do many things that others with this disease can't do. I'm confused about my diagnosis, but know that I have it after a Bone Marrow Biopsy was done to prove it. Anyway, I'm rambling. You are the first man I've come across with this disease. I would love keep in touch. Amanda (ajjb1020@yahoo.com)

Sara said...

Hi Kevin, I have masto too. I've had it since I was young, but just got diagnosed about ten years ago. I recently started doing lots of research about it and trying to reach out to other people with it. I wrote about it on my own blog a couple of weeks ago.

http://whatsarasays.blogspot.com/2009/11/mastocytosis-my-story.html#comments

My email is lagringuitablog at gmail dot com and i would love to "talk" to you sometime.

CAROL said...

Kevin,

I am in the process of being diagnosed with SM. The next step is the bone marrow biopsy. However, I have what I would call minimal symptoms. I thought my symptoms were all part of getting older. I only went in to the dermatologist for a preventive checkup and pointed out the "freckles" that had appeared earlier in the year. What is the progression of this disease? Do some people take a long time to have significant symptoms and others get them quickly? I feel fairly healthy and I want to stay that way, but I find myself thinking about the future with SM almost continuously.

Anonymous said...

Kevin - I was on the computer looking up information about Masto and i found your blog. I agree great to see. I would like to talk to you more - what type of meds do you take, what are your symptoms? I just found out i have this a little over a year ago. We think I've had for about 8 - but did not know it was masto. I have pain in my side, feet, flashes etc. I feels good to know i'm not the only one. my email is barbreynolds828@yahoo.com - please respond if you have time. thanks barb

Anonymous said...

Hy Sara:

I'm woman from Spain with a long story of different kind of affections, pain and of course suffering.I've been recently diagnosed with a Sistemic Masto, this extrange ilness, I had hardly heard about it one month ago.

I felt so scared the first time I learnt about it. Looking bakward in my past I can see I have suffered it since I was eleven.

Of course I'm worried about my future but there are many illness degrees. I got a little bit worst along years but I've been ill for more than 30 years as I'm 45 now. I've got three kids (one under suspicious) and I had a "normal" life.

Most of the doctors I visited looked at me as If I was crazy, even my own familily did it.

Just a few words to say that is conforting to find people like you and thanks to contac with all of us in the WEB.

I'd like to join the group you mentioned in facebook, would you mind to post the adress?.

Thanks very much

Maria.

Anonymous said...

Kevin,

I am also a 43 year old father and would love to have a chance to chat with you directly. Please email me at bgross@ugl-equis.com.

Brad

Anonymous said...

Hi! Kevin,

I have been on chemo for almost a year now, only I have to go get it 5 days in a row. The type your on, is it avaiable to everyone now? Please respond if you could, my e-mail is cahoericson@yahoo.com

thanks, Cindy

Anonymous said...

Hey Kevin- Reading your blog and knowing that people are out their continuing to live their lives with this disease is conforting to me. My family has been searching for 20 years to gain a confirmed diagnosis for the symptoms Daddy/Papoo has been living. He has suffered with vasal-vagal synope,stomach cramps, low-high blood pressure,lumps on elbows&knees,red skin nodules, and aching all over his body.His body would produce episodes of low blood pressure,cold sweats,vomiting,diarhea, and then he would pass out. The EMT would treat him as if he was having a heart attack due to his blood pressure being so low. I could not began to count how many times he was transported to the hospital from his place of employment due to these episodes.Leaving every time with and unknown diagnosis. Fifteen years Papoo's was given a diagnosis by his cardiologist ( Vasal-Vegal Synope) This condition was not life-threateniing, treatable but no cure. He would have to learn to live with the systoms. In 2005 Papoo's symptoms became so severe that he was placed on short-term disability. Initially we worked with local doctors with no relief. By begging and pleading he was sent to a world renoun doctor in Toledo Ohio. He reviewed all records and suggested he was symptomatic of carinoid tumors. He was sent for a battery of testing.Nothing in the testing proved he had carinoid tumors. He is now on long-term disability due to him being systomatic of carnoid tumors. He takes monthly sandostation(otretide) injections to suppress the severity of his systems. Now that the gastrointestional and blood pressure are under control, new symptoms are surfacing. Lumps on elbows on knees and elbows. Open skin lesions on his scalp. Newest symptom is a growth on his ankle. I have a cousin in the medical field and graduated from Harvard. When he reviewed all the medical records he suggested mystosis. On monday we are going to have a surgeon look a the growth on his ankle. Hopefully the biopsy will reveal something that can be treated. Questions Who did your inital diagnosis and how do we rule this disease in or out?
Take Care & God Bless you & your family.
Hollywood

Anonymous said...

Hi Kevin, first of all thanks for your blog... My mum died 2 mths ago in masto which has never been diagnosed... I am grateful to gain some info about this disease and would be happy to chat with you direcly. My address is violabog@yahoo.com

Lots of love to you and your family,
viola

Sara said...

Maria, My favorite faceboook group in the Mastocytosis (mast cell disorders) group. Hablo español por si acaso ;)

Hua said...

Hello,

I'm new to your blog and I have really enjoyed reading your posts. You have some great information! I hope all is well with you and your family.

Best,
Hua
Director of Blogger Relations
wellsphere.com

Kathy Donald said...

Hi Kevin,
I (like others)came upon your blog while researching my masto. I can't seem to find anyone else who has it (as I do) ON the bone vs in the marrow etc. I was wondering if you or anyone else who reads your blog has. I had 2 very large bone tumors on my right femur. I had surgery in 2002 and was diagnosed with masto then. I continue with typical symptoms ie stomach issues, itching, hives bone pain blah, blah blah. My docs don't seem to know much about it so I am trying to research it myself. If you know of others with bone involvement, would you let me know? I would greatly appreciate it.
My email is kathydonald@comcast.net

Kevin said...

Hi Kathy,

I highly suggest you contact my Doctor at M.D. Anderson his name is Dr. Srdan Verstovsek. You can email him at: sverstov@mdanderson.org he may be able to help answer some questions for you. He has seen a great deal of people with masto such as ourselves. Let me know what you find out.

Hope this helps,
Kevin

Amanda said...

Hello all,

I too am researching. I have had many random health issues that have turned out normal test results all along. I had my first known symptom when I was 17. It was only an occasional flushing during public speaking.

Last year it progressed to become quite intrusive. At new years I turned bright red in the face and chest after drinking a margarita. By the end of the year I was flushing, burning, and itching to card games as simple as Go-Fish and during conversations with strangers.

What I'm curious about is what other symptoms could be explained by Mastocytosis. I suffer from varying degrees of stomachaches and headaches on a weekly basis and the last three months have had moving abdominal pains. These seemed more like the symptoms of the systemic version but I've read that you can have systemic symptoms but still only have the cutaneous version.

Has anyone heard of a similar set of symptoms?

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.

Symptoms

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.

Treatment

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.

Prognosis

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

Exercise

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.

References:

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