Hi Kim, nice to 'see' you back here! Glad to hear Brie is doing OK, OK is good, when she has been through so much not OK. Praying for more of the same, or better. I hope you are also doing ok, with all that life has thrown at you this year. What a blessing kids can be, when we see their strength, resilience,  and sweetness in spite of all they are going through. Best wishes for health and stability, and time to enjoy the sweetness of your children.

Ruth

Kim

I never "met" you before but feel for what your young Brie has gone through, I can't imagine a child experiencing this,

I'm falling into the adrenal insufficency (maybe from steroids for anaphylaxis) and ?MCAS category so can sympathize about the confounding situation.


the only thing I noticed and I'm sure you've gone over it, but I noticed she's also on an inhaled steroid.....in my case the inhaled steroid was causing a lot of adrenal suppression and i had to wean off very slowly decreasing by one puff every few weeks, but it seems to be improving my situation. I wasn't on entocort though I was on prednisone and am not on hydrocoritsone..

I REALLY hope they sort things out for Brie. She is so lucky to have a Mom like you to fight for her

Hey Kim, we know about rarities with masto, don´t we? !!


Yet, what's really so important here is that even though Brie's still got troubles, hers and your circumstances are so much better than what they used to be!!   All your hard work has really paid off and I'm so very proud of you for being so persistant in pushing for those answers!!!  

You know, Kim, perhaps if you look at Brie's activity from a different viewpoint it may help you and her as well in having more patience with her situation.   I understand your discouragement in wishing her to be a 100% normal, healthy child and I know that she gets discouraged too for her masto really gives her a terrible beating.   But I've been noticing my own situation and considering those of some of the others of us who also take a beating.   I can't help but begin to see that there are phases of activity we get into.   I'm not like Deborah or Heather or some others who can find a reprieve and pretty much remain stable.  I have phases.   Some where I'm taking more of a beating than others.  Perhaps I can say that I go through lull's of activity instead and that it's in these lulls that life is more like it used to be where I get an entire day of feeling normal.   I've yet to have two days together where there isn't something or other acting up, but at least I'm not feeling so awful as I used to!!   I have had an improvement and with masto, any improvement is a victory!

If you can try to keep this perspective, of bearing out the phases of more activity, focusing more on the certainty of a lull coming up to give  you a break, it may help the phases of more activity seem a bit less trying and help to enjoy more the lulls.  Knowing that these are phases, they are temporary, that they will improve is what has helped me deal with so many unpleasant things in my life.   It's been crucial in dealing with pain, for pain is often a very psychological process in that the more you tense up and get anxious with pain, the more intense you feel it.   By emotionally and mentally being more pateint, waiting it out, bearing with it, trying not to focus on it, you can get through it better.   This was never better shown to me than following my open heart surgery for again, all I had to dull the intense pain was an NSAID no stronger than Ibuprophen and what really got me through it was my attitude, that of repeating to myself - this will be over soon, in a moment the worst will be past and I can breath free for a bit longer.

By working with my pain and difficult circumstances as nothing more than a phase, by only allowing for it to be a moment and work with it during that moment, once the moment passed, I was free and able to relax.  

So, helping Brie to see that this is temporary, a phase, that in a bit she'll be in a lull from the reacting and that when that lull comes she can relax and take more advantage of life, this will help her find resources within herself to deal with these things.    It will also give you the break from having to deal with a soon to be pre-teen who is tired of taking a beating all the time.  If you can teach her now how to find these resources within herself, it will give her the tools to overcome many circumstances beyond just her masto.  

I hope this helps a bit, Kim!!

Lisa

PS - it is SOOOOOOOOO GOOD to hear from you.   I always have loved the tone of what you write and can't help think of how blessed Brie is to have you for her mom!!!

Wow!!! Can't believe it's been a couple months since I have posted on Brieann's status...  that's a good thing I guess  Her Mast Cell disease is more stable than it has been in several years!  I think they have finally found the best med regime for her and that for the most part keeps things to a minimum.  She still has symptoms.... but mostly GI related.  Her skin outbreaks and very minimal at this point and the same with the neurological, cardiac, etc.  She has had a couple more episodes of the NEH....  still have no idea how this all ties into it if it does at all.  Her Adrenal Insufficiency still bares it's ugly head now and then.... but this has improved greatly also.  She has been off ALL steroids... even the emergency Solu-Cortef shots since last October.  She has had one significant episode of illness in that time and did not require the emergency injection.  I really hoped that meant her Adrenal Glands were up and functioning..... but she was admitted to ICU last week for a repeat test of the Adrenals.  She was given the 250mcg (vs. the 1mcg) and surprisingly she did quite well.  She reacted for 2 days but nothing too scary... just miserable for her
  For this test they "don't pay attention to the baseline Cortisol level" and only consider the importance of doubling her level at 60 minutes."  This is a definite concern of mine as her baseline was still only 2.6 (way to low).... and she was 18 in 30 minutes and 23 in 60 minutes.  Sooooo...... not only did she double but she went 8 fold!!!!  This, from any literature I read means her Adrenals CAN function and went hog wild with the cortisol injection..... which may mean the parathyroid just isn't stimulating to produce the cortisone... which would be secondary versus primary Adrenal Insufficiency.  During her initial test a year ago she only got up to a 9 at her 60 minute level.... so that reflected Primary Adrenal Insufficiency.  So I am really confused.....    When I mentioned all of this to her Dr. she seemed a little perturbed that I ask so many questions or that I know more than they think I should... and she then said that she is not out of the woods and we will continue to follow her and may need a 3rd test down the road.  Some Dr.'s are AWESOME (as she is) but don't like it when the parent or patient know a little something and question it.  This can be really frustrating for me.  So now that she is doing fairly well at this point I am confused as to whether or not I should be concerned for the possibility of Primary or Secondary Adrenal Insufficiency and still how this may or may not be related to MCAD.   I guess we wait it out like we have been doing for the last few years and see what happens.  But if any of you can relate to this I would love any input into this.     Thanks a Bunch,   Kim

This really is good news, Kim!!   I´m rejoicing with you!!!!!!    YEAH BRIE!!!!!

Tell me, Kim, has she begun GROWING more now that she´s settled down a bit, or is she still on the small size?


Another thing - I don´t see why the need for steroids at all, Kim.  I know that the steroids help to settle down the allergic reacting that gets stirred up with the anaphylaxis and crises that we go through, however, I´ve found that steroids are not really so essencial.   The reason why I say this is because I RARELY will have them, even with my worst events.  I have only recently used steroids following huge reactions to perfume, but that was because we refused to go to the hospital!!   I´m still not able to run to the hospital with big events because I´ve got to get the paperwork finalized and this has been complicated because my doctor who must sign it all is in Rio and I don´t get to go there often.   She´s got to put her stamp on it all and without it my local doctor won´t put hers.  So without that, it makes little sense to go to the ER.   But these big reactions need more powerful meds to pull me out of the reacting which means steroids.  

But, for example, following my open heart surgery, which was a huge surgery, they didn´t put me on steroids to help with the reacting afterwards, for that kind of situations creates prolonged allergic reacting for a while afterwards.   We had to combat it with IV antihistamines and IV infusion of epinephrine while I was in hospital and then higher doses of oral antihistamines once I was home.

This is how my doctors have always dealt with this and I personally think that in the US and Canada the doctors use this much more than necessary.   I don´t believe that either Dr. Escribano or Dr. Castells uses the steroids all that much, but then I´m not certain.    I do know that the use of the steroids are on the REMA protocols as they are also on Dr. Castell´s pre-medication protocols, however, they never mention a continuation of use of them.   And when Dr. Castells adjusted my meds for my surgery, there were no instructions for using steroids afterwards.

So, what do my doctors do?

Well, what we´ve finally discovered to do with me is when I have a crisis event and need the ER, or I´m hospitlized for a procedure, we have found that IN MY CASE, I must be put on an IV infusion of either antihistamines or a mixture of antihistamines and epinephrine, depending on the situation.  The more acute the reacting, the more the need for epinephrine.

I would suggest that for Brie that her doctors consider doing this.   The constant infusion of medications for at least 24 hours will help her system calm down.  It also will force her to keep quiet and in bed and this forced bed rest also helps her to keep her body quiet and recuperate.  

I think, Kim, that Brie´s situation warrents just such an approach and recommend your speaking with your doctors as to this option.  

Lisa

Kim,

One of the things I was trying to stress in my post was that steroids really don't need to be intruduced at all, at least from my own experience of dealing with reactions.  My doctors and I avoid them due to the fact that they will push up the BP and I've already had a hypertensive crisis while they were involved.  So it's something that I will use only when I have no other option.    

I ve used them recently with severe reactions to perfume, but this was only because going to the ER is still VERY complicated for me and I've no written instructions for my doctors as to how to deal with me.   It's still a matter of go to the ER and fight with the doctors to have them do what they need to and probably not get them to do it and thus go home hours later frustrated....OR  try to deal with it ourselves at home with heavy duty medication.   I know it sounds foolish and I personally hate this situaiton, but this is what we've been doing while I'm fighting to get this document prepared and signed for by my doctors, both local and distant.   My recent experience in the operating room confirmed that I'm right for the doctors can't recognize this syncope and when they hear the word syncope they automatically presume that it's SYNCOPE the vaso-vagal kind which is no big deal.    The anesthesiologist was young and had never heard of masto and she decided to completely ignore my doctors written instructions due to seeing this word syncope!  I was left 3 hours in syncope before another doctor was called in and he had to work with me to bring me to full consciousness so I could explain the situation to him and then he listened and used epinephrine on me and saw immediate improvement.  But being it was only a bolus shot, which is short lived, 15 minutes later I was back in the grips of the syncope!  It took another 5 hours of foolishness before one of my doctors was called on the scene and they gave me the infusion and THEN, FINALLY things turned around for me!!

This is what I'm trying to say is that this INFUSION form of both antihistamines and epinephrine are EFFECTIVE and there's NO NEED FOR STEROIDS for this is for a 12 - 24 hour drip and in that time period, the forced bed rest also works to settle the reacting down.   By the time Brie would return home, she would be better, more recuperated without the need to attack her adrenals.  

This form of medication is based upon an article by a Dr. Simon Brown from Australia in a report he wrote on Cardiovascular Anaphylaxis.   His report cites how some patients don't respond well to the bolus or IM use of epinephrine and he investigates this issue and his findings are that infusions are much more effective with this patient.  He also deals with this same supposition regarding antihistamines and the experience I had with my open heart surgery post-op syncope and reacting was supporting of this paper.  

So, I think that with Brie, she should never really need to have steroids introduced but instead  just be on the anti-mediator meds of leukotreine blockers (singulair) antihistamines and epinephrine and then when she gets home, keep her antihistamine levels up and taking singulair until she calms back down again.   This should deal with it all nicely.  


Lisa

I am so glad to see this site is still helping so many people. It seems like forever that I have been back here. But I think of all of you often! Life  has just been so full of obstacles that I have been unable to make the time to frequent this wonderful site and Brie has remained about the same regarding medical stability for almost 2 years now.  Her medication regime, since seeing Dr. Castelle's in Boston 2 summers ago, is what changed her life around. She hasn't improved or worsened much since that time.....  But is stable and sooo much better than she's ever been.  Now that she is 11 years old and the body, etc is starting to change with hormones and puberty things are picking up just a little..... not sure what that all means. And even Dr. Castell's was unable to shed light on what would happen at this time when we saw her since Brie is such an unknown entity with being a young child with systemic mastocytosis...... and this is almost unheard of. If anybody has gone through a similar situation with a child or knows someone who has I would really appreciate communicating with you.  Thank You!

I've read every page of this so far. (It took 18 days of reading one page per day, and I looked forward to that one page every day.)  Please keep us updated on Brie's case and how she is progressing.   This story is very informative and compelling.   I wish the best for Brie and her loved ones.

Todd in Tenn.