Tough Week, Not Much Left In The Tank, Very Confused...input Please???

[CharmedLinz]

I have to keep this short because I'm plumb worn out, but I've had a tough week that was bearable until an appointment topped off Friday afternoon with pure confusion.

Tuesday I had a follow up with my Cardio to let him know about my ER episode....outcome.... the ER did Nothing right....... bigger shocker.

The Cardio is blowing things off and pushing things on other docs. Though he was shocked that I was passing out while laying flat during the episode.

So he asked me to try Midodrine again as PRN since he thinks I might tolerate it better now that I'm on Cortef.

He is agreeing with my other Docs to send me East to see Grubb and a bunch of docs at Cleveland Clinic.

Rheumy appt on Wednesday that was 2 hours, much more productive, and surprising considering we had almost dumped this Doctor. Hmm the power of him thinking we were switching care.

He had lots of ideas and wants to get me tried on IVIG.

He was going to call a Mast Cell expert to make sure I will be ok with the IVIG.

Fast Forward to Friday....... Hematology appointment..... let the confusion begin.

Oh and I should say, last Friday was Thyroid Biopsy day.... test results today..... Benign...YAY but what happens to watch it???

The first time I saw this hematologist was in February before I saw a specialty Neurologist who did skin biopsies for Small Fiber Neuropathy, which was Positive at both sites.

The opinion of the Hematologist before that, I'm going to call him Dr. B for ease, had the opinion that I fit the criteria for Polymyositis or some of other type of muscle disease and was sure the Neuro would do a Muscle biopsy.

At that time he had ruled out Carcinoid because of the negative ocreotide scan. We were also awaiting a thyroid biopsy that he felt could cause some skewed results if I had thyroid cancer which was beleived because of family history and the type of nodule.

Fast forward to now.

The Neuro did not do a muscle biopsy he was waiting to do that maybe in the future.

I had my thyroid biopsy which came back negative.

I've had an AIRE gene test that is pending to look for MEN or APECED because my PCP and Thyroid Surgeon feel I have some form of Polyglandular Failure.

I know this can kind of fall under the umbrella with Carcinoid.

My Immunologist ordered the AIRE gene test.

He is the one who initially tested my methylhistamine and prostaglandins and did the Chronic Urticaria Index.

Based on the CU Index he said I had some type of mast cell thing going on but he wasn't sure how to classify it.

This was in December of 2010.

Fast forward again to a month ago. My immunologist had spoken with Dr. Afrin after I had emailed him about sending some colonoscopy tissue. Dr. Afrin felt by my labs and symtpoms he didn't need my tissue I had MCAS.

He sent info to my Immune doc who called me in and said the CU Index showed the autoimmune form of MCAS where I am attacking and causing my Mast Cells to degranulate.

We then ordered my Complement Levels and Further Eosinophil evaluation because my Eosinophils have always been high.

My Eosinophilic Cationic Protein came back HIGH showing that something is causing my Eosinophils to degranulate into their toxic form.

My Complement C5A came back HIGH, it's the most potent Analphatoxin and causes Basophils and Mast Cells to degranulate.

Back to the Hematologist, Dr. B.

He briefly looks at all the weird labs, and I mean briefly.

Hears my story of my recent ER visit that easily could have been a Mast Cell Attack and anaphalaxis, or an Adrenal Crisis, or a Carcinoid crisis, or a POTS related episode.

He skimmed the Primer from Dr. Afrin but wouldn't even look at my emails from Dr. Afrin.

He looked at my Chromagranin A level of 47 which had not been done before. He said that level was normal.

He then said to him this all seems Autoimmune and everything has to be tied together somewhere. This can't be 10 diseases that are seperate. I agree.

He doesn't feel I have Mast Cell Issues or Carcinoid or MEN.

He realizes my Platelets have always been a bit high that can happen with auto immune or inflammatory.

During the ER however the platelets and WBC were high which concerns him more.

So what's the answer........ he orders a Bone Marrow Biopsy!!!

Fine, great, at this point I'll take anything, and at least if he doesn't beleive the mast cell thing he will have to look for it.

I asked if he would look for mast cells in the marrow and he says in Bone Marrow they look for everything not just specific tests.

So I feel he's ordering it for the wrong reasons, but at least my Marrow will finally be looked at for not only mast cell but for Mito issues that have needed addressing as well.

His best bet after that is to send me to NIH. We've already figured this won't work for so many reasons and are planning the trip to Cleveland Clinic and to hopefully Dr. Grubb.

How exactly does Carcinoid get ruled out over Mast Cell stuff??

Would my CU Index be high in Carcinoid?

I know the Seratonin really points to Carcinoid. I will ask to have it redrawn this week along with Tryptophan as suggested.

My Histamine has been high in whole blood but normal in serum and normal in urine.

My insulin is strange in that the fasting level is always normal but during a 3 hour GTT it was always triple the high range.

Over the past 2 years I've had Gastrin, VIP, Glucagon, Ceruloplasm, Calcitonin, and they've been normal.

But I know things can change and they've only been tested once.

I have had my Catecholamines and Metanephrines tested numerous times and they have always been normal. Though I haven't had the sitting and standing, lab here in not capable of that.

My 5IHAA (I always get that wrong) has been tested 3 times I think and has tested normal.

Prostaglandins and Methylhistamine just once at the same time as CU Index in December 2010.

Tryptase 3 times I think, levels were 4, 6, and 7.

As for imaging I've had the Ocreotide, a CT enterography, gastric emptying, Hida Scan, MRCP, abdominal MRI.

The only thing that showed up was some weird fatty liver, and my gallbladder doesn't work.

During a surgery last year they found multiple adhesions and fibrotic tissue throughout my abdomen of unknown cause.

This was a hysterectomy and my pathology report was clean.

So what rules what in or out???

I'm confused more than anything, and scared only of what they don't find at this point. I'm soooo sick and rapidly declining that I've reached the stage where I just want answers, I don't even care what they are.

I'm more scared that they are over looking things because I'm so complex and they don't have enough time and they aren't expert enough to know what they are looking for.

Boy I hope some of that made sense, I'm exhausted.

I've cross posted a bit of this on a mast cell forum as well.

I know some of this is off topic for POTS, but I know a lot of people here have Mast Cell issues and some have Carcinoid.

I also found it very interesting that the Complement levels, especially the C5A cause Basophil and Mast Cell Degranulation and can negative impact on smooth muscle like heart function.

Soooo any input????????

[sue1234]

First, how did you get an ocreotide scan when your 5-HIAA(?) scan was normal?? My endo wanted a PET scan of my pancreas, but insurance continually denies it. My poor doctor has tried three separate times!

Second, how high was your insulin when you say it was triple? I am finding more of us with high insulin. Why do we have high insulin???

[Christy_D]

If I'm not mistaken, with MCAS there won't necessarily be too many mast cells which is what they will be looking for in the bone marrow. It is just that the mast cells 'behave' badly. So they still might rule out a mast cell issue if they don't know much about MCAS. Is there any way you could get to see Dr Afrin? He is so knowledgable and thorough.

[Clairefmartin]

Sounds like you need to get to a specialized center ASAP (in my opinion), like Cleveland or Mayo or Vanderbilt. Somewhere that has multiple doctors across specialties that can work together to dx you. I'd say skip Dr. Grubb for now, he is WONDERFUL, but he is one man and his wait list is long. Have they looked into MIto for you? Mito causes issues in so many areas, its just the first thing I thought of. Either way, I'm sorry you are dealing with so much - and I hope you get answers!

[RichGotsPots]

@Linz- can you make a signature with some of your dx's and meds?

[CharmedLinz]

Hello All,

Sorry to have ignored my own post for a couple of days, just needed to be away and not type. I did read your posts. Will answer them now......

Sue--- My GI Doc was able to order the Ocreotide scan off the result of a high Whole Blood Serotonin Level. He backed it up with a High Histamine Whole Blood, and the 3 hour GTT Insulin Levels.

The 5HIAA urine is only accurate 50% of the time.

Here are my levels from my GTT test..

So here goes.....

Fasting Insulin 9 uIU/Ml range is 3-25, my insulin fasting has been as high as 19

Fasting Glucose 85 mg/ml

Fasting Growth Hormone .68 ng/ml range is 0-10

30 minute

Insulin 205 range 20-112

Glucose 66

GH 1.17

1 Hour

Insulin 176 range 29-88

Glucose 143

GH 0.53

90 Minute

Insulin 195 range 26-84

GH 0.21

2 Hour

Insulin 126 range 22-79

Glucose 126

GH 0.14

150 Minute

Insulin 29 range 3-25

GH 0.14

3 HOUR

Insulin 19 range 3-25

Glucose 61

GH 1.53

Christy-- We are trying to get into see Dr. Afrin in December on our trip or possibly another high up MCAS Doctor in Boston that may be more feasible for the trip and with some of the weirder markers I've had lately.

My Mast Cells won't show up in Bone Marrow unless I have systemic. Which is still possible as I have never been able to get a Tryptase drawn at the right time.

This Hematologist though said he doesn't even think I have Mast Cell problems, which has been proven that I do. So I don't even know that he would look for Mast Cells if we do this Biopsy.

Claire-- You are BRILLIANT. Mito is on the short list for what is the root of my problems. A Mito Specialist is on the list for the December trip and I have Docs here supporting that theory.

Rich-- I will try and do that, I haven't figured out how you guys get it all in the signature without it being too many characters. I will try again in a few minutes.