Went to bed feeling OK nothing but the normal aches and pains associated with this crappy illness, I woke up at 3:15 AM feeling flushed with my heart racing at Nascar speeds. I was also cramping pretty severely to my stomach, I knew that if I was not able to have a bowl movement soon I was going to be in trouble fast. When I’ve woken up like this out of the blue in the past normally I’m injecting Benadryl shots while calling the paramedics, my heart is still racing my face is beat red I’m beginning to worry so that makes matters worse. I take a few deep breaths going over in my head what I should do if this takes the turn for the worst. I have all my shots close at hand, phone, and cell phone (so I can call my wife’s cell phone because she is out of town) It’s hard to explain even though I’ve been through this so many times in my past it still scares the crap out of me, I can’t help but wonder will I wake up from this attack or is this going to be it. Experience tells me I’ll be OK don’t panic stay calm take a deep breath and try to relax. I’m able to finally release the histamines, I try to go lay down but my heart is still racing I’m short of breath I remember my oxygen bottle I decide to take a Xanax to try and calm my heart rate. I go back to the bedroom with my arsenal of drugs, oxygen, shots and my medical alert information card laying on my night stand so the paramedics will see it if it comes to that.
I woke up this morning feeling like I’ve been run over by a train!!! I feel like I’ve had an attack while in my sleep. I’ve done this many times to try and avoid going into anaphylactic shock only to wake up with the feeling I slept through it. I begin backtracking what I ate the night before or what might have set off the attack. This time I’m really not sure, I did eat a non preservative pizza last night of course there is always the stress!!!
This is just one story of one person living day to day with this crappy illness!!! I pray all the time they’ll find a cure, I’m so sick of always feeling like crap.
I will continue to smile, laugh and live the best life possible. I refuse to let this control my life!!!
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
26 comments:
This rings so true with my experiences. Fear is cumulative. I'm 47 years old and, despite all my well-rehearsed emergency strategies, I'm as frightened during an attack now as I was twenty years ago. Knowledge doesn't necessarily reassure. All best to you. Stay strong.
Thanks Mark,
I'm trying to get some work done but not have much success. I know it sounds crazy but it's nice knowing other people know what I'm going through!
Peace,
Kevin
I have spoke with you before, but never got a response. I too have this diease for 18 yrs now. Been though alot myself. Knowledge is good but attacks are scarey.
Very sorry about the no response! Glad to hear from you again, hope things are somewhat OK for you health wise. This illness is a rough roller-coaster at best! Actually the attack last night was probably a good wake up call for me I have been getting to relaxed. Sad thing is relaxed for us means trying to be normal.
Again I apologize about not responding in the past.
Paece,
Kevin
hi im eva and im 40... i was diagnosed with systemic mastocytosis a year and a half ago... im not feelin well at all i guess i was born with this disease and problems started happening two years ago... i am on a trial chemo drug..i would like to talk to people with this disease it is very depressing... my e-mail add. is softballmom4evr0004@yahoo.com
Hi Kevin!
I'm Ana, living in Lisbon, Portugal, Europe, and I was diagnosed with SM 5 years ago - fortunately the indolent type.
But even taking all the meds, there were still those nights to get through with a beating heart and exploding face - just like you describe them.
5 months ago, I started complementing my treatment with phytopharmaceuticals - and this is the reason I'm writing, because I'm really feeling much better, and I'm back to my old productivity (I'm a surgeon). You've probably allready tried them, but just in case you haven't, here are my tips: Quercetin Complex from Solgar and also Rutin from the same brand.
Hang in there, hope to have helped
Taking the pills suck! I agree. My pill collection has grown since I had a craniotomy in July.
I followed your blog a while ago and haven't read for a while. I hope you are still doing alirhgt:)
My 21 year old son has been having attacks just like you describe for the last three+ years. No diagnosis yet but lately systemic mastocytosis has been mentioned by three different doctors and it certainly seems to fit. His symptoms started out with flushing, hypertensive surges, tachycardia, diarrhea, headaches, muscular weakness. In the last year, more constant nausea, hypercalcemia, bone lesions, dramatic weight loss.
I found your blog while googling mastocytosis. Whether that is what my son has or not, I certainly understand what you are going through and it is really SO unfair. I wish so much that it was happening to me instead of my son; watching what he has gone through is simply unbearable.
My prayers are with you and all the others that are suffering from this.
• Anonymous – I apologize for not writing you sooner my life has been very crazy lately. I would love to talk with you. I’ll email you in a day or so, right now I have to finish some work and realized all these people have took the time to write me ;) so I wanted to take a second and let you know I’ll be emailing you really soon. Take care of yourself.
• Anandre – I’m not aware of phytopharmaceuticals I’ll be looking them up really soon, I’m always open to trying new things that may make my life easier thanks for taking the time to share this information. I’m glad you are back to your old self hang tough!!!
• Sara – I hope you are doing well or at least getting by the best we can! I’m glad you took a second and posted on my blog. I need to write more often. I tend to think that I’m just rambling and who really wants to read this stuff, I realized it’s nice to know other people in a weird kind of way are suffering and going through the same things I am. I guess it makes me feel a little more normal if you know what I’m trying to say. Your friend!!!
• Sheri – I’m sorry to hear about your 21 year old son and his struggles. Thank you for the prayers it’s a daily struggle/ battle for people with any type of illness but I’m not ready to quit yet Even though life has been hard and sometimes I have negative thoughts I have a lot to be grateful for. Hang in there hopefully your son will get on the right meds allowing him a somewhat normal life. Keep me posted!!!
Thank you everyone for reading my blog, I hope you all had a great Thanksgiving dinner! If you don’t celebrate Thanksgiving I hope you had a great day with someone you love ;) Your friend Kevin.
Hi Kevin,
I participate in a mast cell disorders forum that is incredibly supportive and helpful to people with all varieties of mast cell disorders. The web address is:
www.mastcelldisorders.lefora.com
Wondering if you are aware of the low histamine diet? You can Google it and there will be a site with a list of foods permitted and foods to avoid. It's a really helpful diet for people with mast cell disorders. It might take a a few days or even a week to notice a difference, but it might help keep you from having those major attacks.
Also, what chemo are you doing, if you don't mind my asking. Are you on H1 and H2 antihistamines? Gastrocrom?
Good luck! Joan
Hello, my name is Matthew and I am 19 years old and suffering from systemic mastocytosis. I have recently been diagnosed and am seeking advice. I suffer from extreme flushing and was wondering if you or anyone knows of medications to rid me of this burden.
I had contacted you with help regarding my father last year when he was very sick and had just been diagosed with masto. You had told me to contact your doctor in TX. My mom contacted him and he referred my mom to a trial for the disease in stanford. He was accepted in the trial and has been on a successful trial drug for a year now. He is feeling amazing and they said the disease is almost completely gone from his body! I just have to say THANK YOU for helping me save my dad's life....many many heartfelt thank you's...
chris bell
Matt, everyone is different in what types of medications work best for them I suggest you go to the http://www.tmsforacure.org/ you'll find a great deal of information regarding Masto.
Chris,
Thanks for letting me know how things turned out for your Dad. I'm glad I was able to help. I would love to know more about the study your Dad is on, can you find out what the pills are called he is taking? Best of luck to you :) Tell your Dad I said hello.
Kevin
Hi there...
Just been told I may have this and alto my symptoms are different to yours, I've had dermatographic urticaria for 18 years, and recently been on up to 9 anti histamines a day plus other drugs... And then more recently, asthma, bronchial hyperreactivity, weight gain, tiredness, high bp, gastric pain anyway what I'm getting at is that I was trying to find out What sm is and came across your blog..... Wanted to say thanks for sharing and hope if u don't get better, you at least have more good days than bad ;-)
Karen
I'm only 20 years old, and I have been diagnosed. When I was 11, I was told I had Mast Cell Hypersensitivity. I worked with that diagnosis for years. Recently, a new allergist followed up on tests and told me I have SM. Honestly, I'm lost and this gave me some hope. I'm constantly scared of sleeping because of the reactions. Just seeing your age and abilities made me relax! I just felt like I needed to connect. Thank you.
Hi there.
I think I may have this!
I have had a mystery "illness" for quite sometime. People thought it was parasites, and it may well be, but i don't know...
I have been treating it as that and the meds aren't really doing an awful lot. I feel like something is missing here...
We have no hard evidence of parasites, despite many many many blood tests. All we saw was a strange conglomeration (apolgies for my spelling) on my live blood analysis.
What struck me is my symptoms are exactly like yours! I get these "attacks" and my heart goes absolutely mental and all the same sort of symptoms as you. I've had my heart checked many times and been told it's fine, and have had lots of other tests run. Something is wrong here. How did you get diagnosed?
If you could email me, I would really be happy...I have some questions.
Please let me know if you're happy to and I'll send you my email.
Thanks! - Greta
Hi Kevin.
I really think I may have Systemic Mastocytosis.
I have the same symptoms as you exactly. I am 22 and no doctors can tell me what is wrong.
A nice naturopath I see thinks I may have parasites as we saw a conglomeration of cells on a blood anaylsis, but we didn't see anything other than that. No parasites have been found, despite many many many blood tests.
I feel like something is missing here in my story. Something is going on.
How did you get diagnosed? Doctors treat me like I am very crazy but I keep getting violently ill. Heart palpitations and weird beats are the worst for me! Muscle aches, vision issues. Lots and lots and lots and lots of symptoms with no firm answers.
I hope to hear from you.
Stay strong! <3
Greta
@Fraux,
Sorry for taking so long to get back to you. The best way to confirm you have Systemic Mastocytosis is by a Bone marrow biopsy.
The best advice I can give you is simply find the right Doctor. It is crucial you find a doctor who will continue look for the correct answer. You may need to see several specialist before finally finding the correct answer to your problem. I probably saw 8 different Doctors before finding someone who suggested it may be Masto.
Don't give up you'll find an answer you just have to keep searching and educating yourself.
I wish you the best,
Kevin
Hi Kevin-- nice to stumble on your blog. We live in The Woodlands! My 4 year old daughter has UP with gastric involvement. Your symptoms so eloquently describe what she must have gone through so many times in the night as a baby, before we got her stabilized. Thank you for sharing. We have had some pretty significant success with vitamin D therapy... we have a special tanning bed made by Dr. Mercola. Hannah gets a treatment for 4 minutes every few days. She sleeps MUCH better, her skin is clearing, and her stomach issues have subsided as well. We are also giving her probiotics, I use nasalcrom on her skin and I am trying to get her thyroid working better, as of right now she is borderline hypothyroid. Your commenter Fraux should have her thyroid checked. She is showing classic symptoms for Grave's disease. Kevin, have you had your vitamin D3 levels checked? Between that and a thyroid imbalance, it could explain your difficulty getting stabilized.
wishing you energy and peace! <3
I was reading you blog and saw that you have a doctor in TX. I am in Dallas and am in the process of getting diagnosed. Everything points to mastocytosis. Would you mind giving me the name of your doctor??
I am searching for a specialist that can help me!
Kevin,
Could you tell me a little more about the sleep reactions. I didn't realize this could happen. It makes sense but I just didn't think about it. Just a couple of days ago I woke from a very vivid dream. In it I was having an anaphylactic reaction. When I woke I had lost my voice and every joint felt like...well, you know how it felt. Are there any precautions you know about for this?
Thanks
Phyllis
I have had masto since I was 32 and now at 65, the attacks are worse than ever. I have sweats, mass itching, bright redness, etc, I feel stress brings on these attacksalso and I evcen take trans. but am still think suicide. My feelings are like yours, surived another day. I try to make every day count. Keep suriving.
Pat Paul IL
Hi Pat,
Don't give up keep trying different things (with Doctors advice of course) until you find something better that works for you. I've tried very hard to take anything that causes a great deal of stress out of my life. I hope you feel better soon my friend!
For everyone who is asking for the name of my doctor at M.D. Anderson his name is Dr. Srdan Verstovsek.
@GBailey if you would like more information shoot me an email at kzapatero@gmail.com.
@Phyllis I'm not really sure of anymore precautions to take while you are sleeping. Usually for me anyway I can go to sleep to help pass the attacks. However sometimes I have them during my sleep as you well know.
Thanks for following my blog!!! I know life can be a struggle while dealing with SM but I still value my life,my friends and my family.
-=Peace,
Kevin
Hello Mark,
I wanted to say thank you for starting this website. I have been suffering from this horrible disease since I was 14 years old, I am now 45. I was hoping that I would outgrow this, but lately it is worse than ever. When I read this blog, I felt like you were living my life. I couldn't believe there was another person out there with the same exact symptoms. I pray daily for a cure and will also keep you in my prayers. God Bless,
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