Wow Do I have a lot to learn !!!  Lisa I hate to think of how many hours of research you have into this.  I have been trying to read and read, and when I think I might understand, I realize I dont .  However, I love to research new things so this is one big challenge for me.  
I ended back at the Dr. today with what I was sure was appendisitis.  The pain started around my belly button, and by the end of the day was in the lower right pelvic region of my abdomen.  I hardly got any sleep because it just gradually got worse.  I waited until morning and called my hemotoligist to see if this could be related to either my bmb or the barium contrast I had to drink for my ct scan and he didnt seem to think so.  I went to see my regular Dr. and she took another cbc and said that all my blood work came back perfetly normal.  Dont get me wrong, this is very good news, however I still have the pain.  Has anyone else experienced this ?  this is not the same gut wrenching pains I get with severe loose stools if I eat something I shouldnt have.  This is a pain I have never had before dull and persistant.  Any way, other than this new thing I have been feeling very well.  Just tired alot.  As I say with every new post, I am so very greatful to have met such a wonderful group of peaple so dedicated to helping one another.  Keep smiling every one   ~Jodi~ Oh and I did get the REMA protocall to print for me.

Thank you Joan.  I have a follow up appointment tomorrow, I guess its so I can let my Dr. know if I still have the pain or not (I do)  If I take 3 200 mg Ibuprofin and 2 tylonol at the same time it will pretty much deaden the pain, only to come back later.  my bowels are moving well so I am not sure what the pain is all about.  We will see what the Dr. has to say.  thank the lord I have insurance !!!  God Bless ~Jodi~

I will ask my Dr about that today when I go Lisa, thank you.  It doesnt hurt when I walk really (only a little bit) but if I touch or press on any of that area ohhh it really hurts  .  Right now all I am taking is 2 tylonol, 600 mg of ibuprofin, and prilosec otc.  I wont take more than 2 tylonol at a time because of the worry of liver damage., but I have taken up to 800 mg of ibuprofin.  i do not have a fever right now which is good, but the pain now radiates almost my whole right side.  Something is going on, now just to get to the bottom of it.  I will check in again after the Dr.  Have a great day everyone !!! ~Jodi~

Well here I am frustrated to say the least !!!  Didnt get any results at the Dr.  dont know why I bothered.  She took more labs not sure why and did an x-ray ?  I dont know what she thought they would find in the x-ray but I feel as though I wasted my time and money.  She said she would call me with results as they were getting ready to call it a day, well I never got the call.  She said before I left that she was going to refer me to the gastric Dr. and that maybe he can give me some answers.  I cant get in with him until the 15th !!  She said to keep in mind that I can use the e.r. in between if I felt things were going that way.  I think she thinks I am making it all up.  I have been going to her for the last few years and I really like her, but I think she just doesnt know what to do with me so she is going to pass me off on some one else.  I still have not seen the hemotologist to find out hte results of my 24 hour urine and my bmb.  I am sorry to vent, I know a lot of you have far more issues than I do,  I think the stress of it all if getting to me.   will let you all know when all my results come in.  Pray for my sanity and health !!!

Thank You Joan, I must say I fealt a lot better after I got that off my cheast !!!!    still smiling !!! ~Jodi~

Jodi,

This response that you have received is exactly the reason that we always advise going to Boston for testing and diagnosis if possible. The bottom line is that they are more open minded there as well as knowledgable on more current trends than the guidelines from 2008. Mayo and NIH tend to be very restrictive in considering MCAS cases, which you may very well have. You should definitely have your results sent to Boston and have them guide your doctor along.

Jodi,

I know you're frustrated, but don't be.  This is actually GREAT NEWS!!   It is not saying you don't have an MC disorder.  In fact, it is indeed confirming that you do, you have MMAS/Monoclonal MCAS.   It is considered a possible pre-SM state.  They aren't sure yet if it is its own specific subset, or if it is SM in its infancy.   Yet because you don't fulfill the WHO criteria for SM it doesn't mean you don't have a Mast Cell disorder.   You do.   You just don't have the more damaging form of it, not yet anyway.   This is also my case.   So, don't be frustrated, REJOICE!   You and your doctor have finally found the disease and you should be treated accordingly.  He's not got you on enough meds!  He obviously doesn't know that the severity of our symptoms has nothing to do with the severity of the disease.  This is typical behavior with doctors who don't know masto.  The majority of diseases out there work that the sicker you feel and are, the more progressed the disease is.   Masto is a rule breaking disease and it won't follow the leader with the way other illnesses work.   You need to be on twice as much medicine, two doses of those meds AT LEAST and even adding singulair and gastrocrom or Ketotifen.

Your doctor has given you instructions and the freedom to seek out higher authorities and that he will work with them!  Oh how I wish I had doctors like that!!  Take him up on this.  He's admitting that he doesn't know what he's doing with you and needs help to continue treating you.  I other words, he's interested in keeping you as his patient and yet letting you know he needs HELP.   So, get him some help!!   I think his suggestion is perfect - send your tests up to Boston and if you can GO TO BOSTON!   Then, when you get back home, he'll take care of everything else and any issues which come up in the future, he will refer back to them for guidance!!!   This is EXACTLY what you need!!!

If you (or anybody else)  wants a copy of this document, send me you email and I'll be glad to give it to you.  This is a document your doctors need to have for this is one of several recent articles which give the doctors the INFORMATION they crave to understand our disease and diagnosis.


Here, I'm going to copy a bit of information which I think will help you see what I'm talking about.   This is from a recent article on the Diagnostic perameters for MCAS by Valent, Horny and et al.  They are MAJOR authorities in mastocytosis.



There are a number of clinical features that may provide
direct or indirect evidence for MC activation. One
such feature is the presence of a primary MC disease (MC
neoplasm = mastocytosis) often manifesting with skin
lesions. MC activation with mild or severe symptoms may
occur in all variants of mastocytosis independent of the
burden of MC and the presence or absence of skin lesions
[11–17] . Even in cases with ‘subdiagnostic’ accumulations
of monoclonal and thus neoplastic MC in the tissues, MC
activation may occur and may represent a relevant clinical
problem [12, 13, 18–20] . This condition, which may
represent a prephase of SM, has recently been termed
‘monoclonal MC activation syndrome’ (monoclonal
MCAS) [18, 19] . In other cases, MC activation is the first
and only clinical symptom of an otherwise occult SM,
which may be a diagnostic challenge in patients who have
no skin lesions [18–21] .



Here, this is a great table that was written up by these doctors as to the difference between the different variants of mast cell disorders.   You will see that you come clearly into the Monoclonal MCAS diagnosis perameters.


Table 3. Major discriminative features in mastocytosis, MCAS,
and allergic disorders

Diagnosis         Typical clinical                 Key laboratory findings
                    features

CM                  skin lesions (UP),              positive skin histology,
                     +/– mediator                    SM criteria not met
                     symptoms1


SM                  +/– skin lesions                 basal serum tryptase >20
                     (UP-like), +/– mediator       ng/ml (screen), SM
                     symptoms1                       criteria fulfilled


Monoclonal        mediator symptoms1,          KIT D816V+ cells found
MCAS               no UP and no signs of          in the bone marrow or
                      SM, +/– allergy2                 blood; 1 or 2 minor SM
                                                             criteria, but no major
                                                             SM criterion and increase
                                                             in tryptase during
                                                             an attack/event


Idiopathic          mediator symptoms1,           no KIT D816V found,
MCAS               no UP and no signs of           no SM criteria, no specific
                      SM, no allergy (negative       IgE, but increase in
                      allergy test)                       tryptase during an attack/
                                                             event


Allergy              allergic reaction to               specific IgE detecable
(secondary        defined allergens,                 and +/– an increase in
MCAS3)             no signs of UP or SM,           tryptase during an allergic
                      mediator symptoms1             reaction3 (CM or SM
                                                              is rarely found)


MCAS = MC activation syndrome; UP = urticaria pigmentosa;
CM = cutaneous mastocytosis; SM = systemic mastocytosis;
IgE = immunoglobulin E.
1 In the context of MCAS, symptoms are considered to be
caused by mediators released by MCs.
2 In patients with monoclonal MCAS, allergy to hymenoptera
venoms is found quite frequently.
3 A transient and substantial increase in tryptase in an allergic
reaction will argue for involvement of the MC lineage and thus for
secondary MCAS. If no increase in tryptase is found during an
anaphylactic reaction, basophil activation has to be considered (is
the most likely explanation).

Joan, this isn´t exactly my strong point either.   I believe that the CD2+ and CD25+ expression is abnormal and this abnormality is only seen on mastocytosis patients, which is why it is considered diagnostic for mastocytosis.   I believe the genetic mutation is a seperate issue but from what I understand the majority of the CD2+ and CD25+ MCs are also positive for the mutation as well.  

Yet the issue here is that of the SM diagnostic requirements.  The requirement of CD2+ and CD25+ expression is one of the requirements for the SM diagnosis.  That diagnosis is very strict and unless the patient fulfills all of that criteria, they fall outside of the SM diagnosis.  However, that doesn´t mean that they don´t have mastocytosis.  

What it means is that they do indeed have a mast cell disorder, but it´s either not progressed enough to fulfill the WHO criteria for SM  OR it is another form of the disease.   There still lacks research to say exactly what the situation is here.  

So, Jodi does indeed have a diagnosis and it would be MMAS or Monoclonal MCAS for she has proof of the neoplasm, only not enough of it.  A few years down the line her doctors will have to redo her testing to figure out where she is in all of this.  Masto yes!  What form?  That remains to be answered.    

This is why she should be properly treated as a masto patient for this is INDEPENDANT upon the form of masto!

Does that make sense?

I hope so!


Lisa