I went back to M.D. Anderson for my yearly checkup, bone marrow + aspiration. Things are about the same so I consider that good news considering the alternative! I’m still taking the chemo by pill twice a day. I also started taking blood pressure medicine again so far so good.
I apologize to everyone for not getting back with you or posting more often. I’ve been so busy lately with different things life has thrown my way hence the high blood pressure. I still have most of the same symptoms from before that people with SM have learned to just live with but it’s been awhile since I’ve been in anaphylactic shock. I keep the shots handy because we all know how fast things can change.
I recently joined Face book so if you like look me up: Kevin Shoemake
If you read this say hello and let me know how you are doing.
Kevin
I was diagnosed with Systemic Mastocytosis in 1997 I'm also known as a shocker. I'm a 58 year happily married man with three beautiful girls and two step sons and 11 beautiful grandchildren!
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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
10 comments:
Glad to see you are so upbeat. I was diagnosed with it about 6 years ago but had it longer. It's great that you were able to tolerate alcohol. Even the tiniest sip causes me such intense ulcerative pain and flushing that I have to avoid it. What a drag. I was not a candidate for the Geevac so I get by with antihistamines and PUVA. Wish there was something better.
I was not always able to tolerate the alcohol, I think I'm doing better because of the new drug / chemo I'm on that enables me to tolerate things better. I could also just be going through a good phase if you know what I mean. I'm glad you commented, wish you the best please keep in touch.
Kevin
Hi Kevin, I just wanted to let you know that I'm reading a bit. My husband is about to go for a bone marrow biopsy to find out if this is indeed his diagnosis. Thanks for the great blog.
You are very welcome Momin. I wish you and your husband the very best!!! Let me know how the biopsy goes.
Kevin
Hi Kevin
Great news on the "no change" but one for the better would have been welcome.
My mom was recently diagnosed with SM after 20years of suffering from this "idiopathic flushing syndrome" as they've been calling it. It's a long story but I'll give you the short version for now.
My mom is fantastic, a strong woman! She started with the attacks Feb 1990. Through the years she's experienced anaphylactic shock 4 times and was lucky to be brought back each time.
Last year in March she had a severe attack and was hospitalized (once again) and the specialist sent a blood sample to America to test for Masto. We never heard from them again until a month ago when she ended up in hospital with a bad attack again (ambulance as usual) I was infuriated when the doctor casually informed us that the results for Masto were positive!!!
Then about 6 weeks ago, mom started hitting out in welts all over her body and face and this was diagnosed (biopsy) as Urticaria. As if she needed any further complications!!
We have since been to see an oncologist in Pretoria. They did a bone marrow test 3 weeks ago and we went to see her partner (as she was off sick) who informed us that mom has Mast Cell Leukemia. What a shocker!! The prognosis for MCL is weeks to 6 months! Then today I managed to see moms doc and she confirmed that she only has SM. I guess if you don't have SM or are close to someone who has SM you can say "only" but it's most definitely not an "only" disease.
Sorry I am rather frustrated and angry with the medical profession by now.
Mom started on Gleevic today, with a mild reaction, but they have assured us that it's normal.
I am not sure what is normal and what is not anymore. All the information I read is just too much to deal with. But we will keep spirits high for her and take it one day at a time for now.
I wrote the above yesterday and did not hit send.....
Just found out that mom had a bad attack in the night. I will now have to find out if this Gleevic must be continued or stopped. I do not trust doctors anymore. Not after 20 years of all these frustrations.
Must run but will chat soon again.
Take care and thank for your blog, it helps to have someone out there who knows what this is like.
Will look for you on FaceBook
Belinda
Hi Kevin,i was diagnosed with SM just recently in may 2010.I had a tumor in my femur that was mast cells.They removed the part of the femur and put supports on my femur bone.I had the same operation 24 yrs ago in the millitary.Just a quick update but il post more. thx..
I'm glad to see so many people still reading my blog. I hope it has helped with questions you may have had regarding our illness. In some strange way it's nice to know that we are not alone!
Kevin
Hi my names,Michele and i've been living with this for over 10years.I've never met or been in contact with anyone who has the same illness as me maybe i'm was scared of knowing too much going back a few years.I lived well with this and i would like to share what i have found out what helps me thats all
Take care
I was recently diagnosed with sm and now they are checking for aggression. I am scared and worried. I never feel good and have been in pain for a couple of years. Its nice to know there are other people to talk too about it . I tried looking kevin up on facebook and couldn't find him .
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