Thanks everyone for your words of comfort during these trying times for our family.... we greatly appreciate each and every one of you!

Brieann has an appointment to see to see Dr. Castell's on Tuesday June 28th !!!!!!!!!!!!!!!!!!!!!!!!!!!!!!!!!!!!!!!!!!!!!!!!!!!!!!!!!!!  

                                                                     Kim

I am sooo happy for you and Brie, Kim!!!! My prayers for your family.
Love
Ramona

Oh, Kim... so many worries, so many concerns, so many doubts and so many difficulties!!  

This is one, my dear, where I honestly think you have got to take a bit of a back seat and let your doctors be the doctors and trust them that they know what they are doing.  You've finally got yourself a team who are aggressively doing what they can and must for Brie and I feel certain that they are not going to let the ball fall anymore!   They're not going to relinquish their responsibilities to another doctor unless they're certain that the doctor can handle it.  And they aren't going to stop supporting the one who takes over either!   I'm certain that you'll have the other doctor right there on the phone wanting to know everything thing happening as it's going on if the pediatrician steps in!!   The ONLY thing that they could do to help with this is to call Dr. Castells and ask her advice on this and as to whether there is something extra that they need to do to care for her with this test.   Otherwise, I think they're going to make sure that all their Ts are crossed and I's are dotted!!!

So, Kim, although you can't fully relinquish your control and just let them have a hey day with Brie, I do think that this time you've got to let go and let them be the doctors here while we all keep in prayer for her!!!

I'm here - standing with you!!!!!   I know the others are too!!!

So, be at peace!  This is what you've been working so very hard to achieve - now let them do their work without being too afraid for OUR GIRL!

Hugs to you, my friend, at this trying time!!


Lisa

Hi Kim

I agree with Lisa but also sense you concern so wanted to share my experience of this with you

My adrenals are still off on holiday and doing very little . Low cortisol is termed addisons . Addisions can be primary or secondery . Primary is due to an autoimmune attack on the adrenals . The antibodies for this can be tested for . Steriods and other hormones can be replaced at levels the same as the body makes with no side effects of high levels commonly experienced .

Secondery is due to several reasons . Long term high dose steriods over greater than 2 yrs . It can also be because the pituatry is not signaling properly . The docs will ahve tested brie's pituatry stimulation .

So now they are going to give an artifical form of adrenal stimulant - ACTH . In the UK this is called a short snachten test .

I ahve had 2 of these .

It is , here , an injection of ACTH into muscule , a baseline cortisol (9am ) when it is normally released in the morning . Then 3-4 more samples at half hour intervals . This is an inpatient procedure here for anyone not only children .

The ACTH should stimulate the adrenals to create cortisol .

The 2 I ahve had made me a little nausated , but otherwise it was absolutely fine .

My first was in may09 , when my cortisol was NIL , but by the time they did the test it had sorted itself out . I had been on steriods full time , 25mg a day ( natural level 5mg a day of prednisalone for adult ) for 6 weeks . They were reduced slowly but my adrenals took a little time
My next one was may 10 . I had been on steriods again 25mg a day since august 09 -9 months . They were reduced and at 10mg I began bursting into tears for no reason . My cortisol was again nil and the ACTH test showed they were off on holiday . ( secondery addisions )This can be tempory or permenant . I was put on a very slow reducing regiemn .

Dr Grattan , masto specalist here in the UK , felt I had not been on steriods long enough to have this effect. ( secondery addisions ). he suspected this was also the source of my angiodeama . My compliment level C3 was also high which indicates lots of inflammation .

So he tested me for autoimmune (primary addisions ) but I came out negative .

My 9am cortisol remains nil , so I am now on replacement steriods daily which is suffiecent to replace my natural level . I have no stress response , which in ill health - viral , bacterial infection , means thet I do not produce enough steriod for that situation . So I get very sleepy ( unable to keep my eyes open ) and teary when a cold etc is starting .
When this happens I double my steriod dose and this gives my body enough to work with with its incresed demands .

So your encrinoogist is doing the right thing checking this to see if Brie's low adrenal response=low cortisol is due to them not reacting to stimulation . As it takes years of very high dose steriods to knock out the adrenals by this route . he knows this and is not blaming this problem on an unlikely cause .

Brie may be responding so well to LOW dose steriods as her body has not been making enough , especially when she has an extra like an infection anywhere or very busy days .

Primary addisions is normally coupled with salt changes esp sodium low , but mine have been absolutely fine . This was why it took an endocrinologist to spot my problem as I wasn't being obvious about my addisions

I am the exception that proves the rule .

idiopathic addisions xxxxxxxxxxxxxxxxxxxxxxxxxxxxxxxx

Once my steriods were replaced at a natural level I woke up spontaniously and stopped bursting into tears when i was not sad or depressed . I have 2 monthly 9 am cortisols off my steriod dose , to see if my adrenals are working . So far the remain on holiday .

I know I have an infection long before I am symptomatic as I can't stay awake . i awake take my steriods and wake up properly 1-2 hours later  .

I hope this helps
Josie

Hi Kim, I feel for you, but as Lisa said, maybe now, for your own sake,  it's time to relax a little in the knowledge that your doctors are right into it now, seriously looking for all of these possibilities. When this isn't the case we feel like we need to keep searching for answers, but it sounds like you have some very concerned thorough medical support now with the knowledge to figure out the unanswered questions. Sometimes the closer we get to an answer the more impatient we get to arrive, but the diagnostic wheels still have to turn.  

Just a side comment, I think in another thread you were asking about the puffy face, seems too obvious to suggest to all you nurses, but isn't it common for steroids to cause that 'moon face' ?  My nephew has had many courses of steroids for Crohns and he has that side effect, I was told because of the steroids.  Just wondering if this could be the cause for Brie.

Thinking of you, with all the challenges life is throwing at you right now.

Ruth

Hi Kim ,

Moon face comes with high steriods. How much brie is absorbing is so difficult as with poorly bowels , I , generally need higher doses to have the same effect .

Addisions causes certian symptoms whic do overlap with mcas . This is why they are making sure . Autoimmune conditions also open us up to two things .

1. it acts as a mignifying glass on mcas and when it is controlled the mcas has less to play with

2. it causes symptoms , like angiodeama because there are extra compliment cascades . Compliment is a cascade of chemicals which have the effect of attracting other immune cells and degranulating mast cells .


It may well be that Brie is having more systemic steriods than the doctors think - though it is unlikely at the dose

The acth test will tell you .

If Brie is negative for autoimmune addisions , and its not the steriods , she may just be like me . The exception that proves the rule .

She may well have addisions which is aggravating her mcas . But once the background problem is identified and stabilsed then it will give less ammunition  

In real terms , Now , I am having steriods at the same level as everyone else makes and I am having no steriod complications . I am like you I like to understand why things are happening to me . Sometimes we just don't know at the moment . BUT All that matters is Brie is treated and well For ages i felt this was not possible for me . But I am getting there My diagnosis is angiodeama - swelling - we dont know why . My autoimmune checks have come out negative . My heridatory angiodeama has come out negative . My tryptase is normal . BUT the anti histamines work . So we remain on that road for now

You have an amazing team of doctors who care genuinely about Brie being both well and diagnosed

How are you feeling in all this . I have spent time wanting it all to go away as we all do . Time kidding myself I was accepting it when I was still looking for the holy grail of a diagnosis in which I would go back to normal ;-( That was a hard day , the day I had to accept it wasn't going away but needed to be managed .

Then somehow it twigged . It would be managed , the epi pen worked . I may not go back to the old jose , but I would be ok  

How is trigger working out going ??? For a year I blamed everything on food , then I sussed one trigger , then another . Now very few of my reactions are foods as I am avoiding the ones that bother me .

For moon afce comparision - I ahve pics on my facebook which are me last may - classic moon face . Look up Josieevans in Bristol UK and ill add you so you can look at them . My face has gone right down now

Many hugs to you and brie

Josie

GREAT NEWS KIM!!!!!    

I will definitely be praying for her tomorrow morning!!!    

Let us know how it goes AS SOON AS YOU CAN!!    Try to be calm, I'm praying for you too!!

KISSES!!!  

ps  - Tell Brie that her FANS here are cheering her on!!  Give her a big hug for me Kim.  Let her know that I'm here praying for her!!!