Since my initial post, I've been in total denial. My allergist referred me to a hematologist about 6 weeks ago. I don't want to go. She was so severe that she was ready to drop me as a patient.

I've made my appt and now I just want to throw up. My father died last May from complications of Aggressive Systemic Masto with c-kit. I suffered a severed Ana. Shock in September and haven't been the same since. My new med regimen keeps everything in check but.......i have a variety of truggers

You poor woman. Try to remember that knowledge is power. If you are avoiding testing to save the stress you might be causing your body more harm in the long run. Once you know what you're up against, whatever it is, you CAN and WILL find a way to get through it and we will help you!

I would encourage you to see the hematologist.


I know this is nightmarish now, but it will get better, you are really at the scariest part, the unknown phase.

So sorry to hear about your dad


I'll need to look back for your other post, was your tryptase elevated?

Totally understand why you would want to pull your covers over your head. And knowing it may be fine doesn't always help. But new information is being discovered every day, so there is hope that IF you have an aggressive form of masto, something may be found to treat it.

Best wishes! Take good care of yourself during this time. Courage takes a lot of energy to maintain. Do everything you can to be kind to yourself, so that you have the resources you need to do what is necessary.

Any special treats that don't cause you to react? Some kind of reward for doing what is necessary? Whatever woudl do it for you, you deserve it!

Jane,

I am sorry to hear of how paralysed to move you are.  This is why this patient website exists, to help break that horrendous grip which fear has you in.

Fear, is by far, you very worst enemy and without a doubt, it can kill you.  That fear prevents you from moving and taking the decisions which are necessary to defend yourself.  That is because your ignorance and lack of undertanding has bound your mind to making the decisions you need to MOVE.  

Jane, this site is about learning.  Those patients who are most sucessful in controling their disease are those who want to learn and listen to the testamony of others who have indeed learned what to do and thus are able to defend themselves and keep themselves safe.

What you are really saying here, Jane is not that you are afraid of the disease, but that your real fear is from that of death.  And who amongst us can't relate to that?  

But the problem is, however, is that your doctor probably preciptated things for she may not be very knowledgeable about mast cell disorders and may only be moving upon the fact that your father had Agressive SM.  And your fear is that you have this and have convinced yourself that you're next.  

Jane, first of all, I've face death and my fears of it are now gone, so I understand your fears and what you are feeling and please let em reassure you with all that I can, your doctor maky hae moed too quickly and this is because you both know that there is a very serious form of masto and the patient must move quickly if they might be able to save their life.  However, what you may not know is that very very few of us ever develop that and if you aren't showing signs of it right now, then you most likely do not have that form of masto and never will.  Your doctor was doing her job, but, with a low or normal tryptase, this is definitely NOT YOUR CASE.    

So, in this case, your ignorance of mast cell disorders has bound you and kept you from understanding and thus moving.

Jane, I want you to give us some information about your case and the testing you've undergone and what results have been shown abnormal and let's CALMLY walk through this and learn with us so that we can help you find your feet again and lose some of this fear.  Then I want you to stay with us this time, learning amongst us so taht you can begin learniing about masto and what it does and how it works so taht we can give you some means of fighting against the fear of the unknown, for this is more deadly to all of us than any real fear we can have.  It paralyses us from living life.

So, please, breath deep, say a prayer for yourself that you can face your fears and overcome them and begin learning about this disease so that you can overcome these fears.  

I hope I've helped you!!  

  BEEN THERE, DONE THAT AND REFUSE TO DO IT AGAIN!!


LISA

Anybody with a NORMAL TRYPTASE has no reason to be concerned about ASM - period.  

The tryptase level reflects the MC burden within the marrow.  Aggreesive masto shows very high tryptase levels and the climb very quickly.  

Remember, the key in that diagnosis is the word AGGRESSIVE.  The disease behaves aggressively and that does NOT MEAN SYMPTOMS!!     It means that it will show horribly off CBCs   and horribly off tryptase and other markers!!  


Thus the word Aggressive.    


Aggressive masto will also show enlarged lymph nodes together with enlarged spleen or pancrease or liver and the MALFUNCTION OF THOSE ORGANS.     You can´t suspect aggressive masto just cause there is an enlarged organ or node, it also must accompany the MALFUNCTION of those organs and the malfunction of those organs will show up in other exams.


This is why ou CBCs are so important for our doctors.  The CBC shows the function of the bone marrow and the blood making organs.  If the CBCs are pretty and normal or close normal, then the bone marrow is not seriously compromised and is functioning properly.   The same will go with the other blood and urine tests which show how the pancreas, liver, kidneys, spleen and heart are all functions.   If your tests show normal or near normal levels, then THERE IS NOTHING TO WORRY ABOUT....

YOU DO NOT HAVE AGGRESSIVE MASTO!!!

Aggressive masto SCREAMS   at the doctors and is very easy to find!  It´s the easiest of all forms of mastocytosis to diagnose cause the patient is so obviously sick and their exams SHOW IT.


According to Dr. Castells and other authorities, you can not judge the severity of the disease and the damage it is doing by your symptoms.  This is the MAJOR MISTAKE that doctors and patients make when they are dealing with their disease.  This is because the majority of diseases work this way.   Masto is a disease which BREAKS RULES and it does what no other disease will do.    There are some masto patients who died from aggressive masto and never had a clue they were sick!!!  They are found only on autopsy.   And yet the contradiction is there are some patients who have MCAS and are housebound due to how very sick they are, and yet these patients DRIVE THEIR DOCTORS MAD   because they can´t find a single bit of PROOF to warrant the severity of the symptoms and are constantly being told by their doctors that it´s ALL IN THEIR MIND"!!!!!

The fault is NOT that of the doctors, for they are thinking within the box as to how almost every other disease out there behaves.  But the wacko masto   breaks rules and doesn´t follow the crowd and DOES IT´S OWN THING!!!!

So, Jane, please, please take a deep breath, let it out slowly and say to yourself,  I DO NOT HAVE AGGRESSIVE MASTO,   I AM DOING WELL!!!

Then, make an appointment for the hematologist so that you can take a further look into your illness and do a checkup on your body.   It is necessary to have the support of a hematologist, yes, for it is a blood disorder and to make sure that all remains fine and well with you it´s necessary to know what form you have and establish a baseline so that you can keep an eye on your health.  


I hope this helps!

Lisa

I had severe symptoms for 16 years and visited many well-respected doctors and hospitals before my SM was diagnosed, so I know that can happen. There are no words to describe that experience.  I just try not to look back.

You may already know this, but there are several treatments in clinical trials now that are showing promise for patients with C-Kit mutations and aggressive disease.  As you probably know, Gleevec is not an option with C-Kit.  One treatment, Midostaurin (PKC412), I know is helping some aggressive SM patients, although it is not a cure.

Don't know where you're located, but Dr. Jason Gotlib, at Stanford U. Cancer Center, is a hematologist who was involved in one of the earlier trials of Midostaurin.  I don't know if it's still a trial site or not.  He might be able to give you additional information.  
  My BMB was done there, and Dr. Akin highly recommends him as very knowledgeable about SM.  He's very nice as well.  He usually sees patients who are already diagnosed, but with your family history and symptoms, you might be able to see him if you want to.  I'm confident that the testing there is state of the art for genetic defects and blood disorders.

I would do both. Try to find a supportive doc and one that knows MCAS but maybe keep taking the meds you were on before for now

Docs that don't know MCAS have a hard time recognizing it.

I am very excited.  I sent an email to Dr. V @ MD Anderson in Houston who was my fathers SM doctor.  I asked him for a doctors referral for someone here in Texas who has experience with MCAD.  Lo and behold he sent me a referral.  I have an appointment with Dr. Henry Legere in Austin next week.  I'm excited, nervous, and grateful all at the same time.  My hopes are high.  I hope he is open minded and will listen to me and search for answers without judgment.