Day six of Cushing's Awareness. A horse or a Zebra?

Towards the end of September 2010 we headed to Barnes Jewish Hospital early in the morning so that I could get my Inferior Petrosal Sinus Sampling done.  Before heading down there a research portion of the Washington University wanted to know if I would be willing to let them draw blood from an arterial line during the procedure to help with Alzheimer's research.  I agreed.  I lost my grandfather to Alzheimer's and loved the idea of thinking that my blood could somehow play a part in researching and possibly finding a cure for Alzheimer's.

I arrived, got checked in promptly and got prepped for the procedure.  I wasn't too nervous as I was told they would give me drugs to keep me loopy, but not really sedated.  There wasn't enough sedation in the world other than being knocked out cold that could have made this procedure any better.  The research team tried for over an hour to get an arterial line in my left wrist.  They kept numbing it and digging around.  It was horribly painful and finally I said I couldn't keep doing it, plus they agreed that they were not going to be able to get the line going.  One of the lovely side effects of Cushing's is we have horribly small veins.  Usually it requires someone who is pretty skilled and using a butterfly needle to get blood from me.  Finally they started the IPSS. It felt as if they shoved a javelin inside of my groin and then I felt a wire moving up through my abdomen and they moved it up further.  However, when they tried to access my left side they had an issue with the positioning.  I was stabbed a good five times, felt them move the wire up then pull it back out.  It was absolutely horrifying and so painful.  I kept begging for more drugs and for them to knock me out, but I had to be awake.  It was like being wide awake and not numbed for a massive surgical procedure.  I think I would have been fine had they not encountered the issue on the left side.  Finally they got it positioned and I felt the wires moving through my neck then into the sinus cavities on each side of my pituitary.  It hurt a little, but it felt so nasty.  I heard crackling and fizzing type noises.  They then started taking blood samples from each side of my pituitary, then gave me an injection to stimulate my pituitary and took more blood at specific timed intervals.  Once they were done with that they cleaned me up, and had to hold pressure on the puncture wounds for quite a while so that they would clot.  I went to recovery for a few hours then was sent home.  I had to take it easy as to avoid the wounds from starting to bleed.  I was extremely sore for a good week after that.  My abdomen hurt and my neck was killing me from the guide wires going up.  Other than that I healed up nicely from it all.  It was just a very horrific experience and if I have to do it again I will find a place that will completely sedate me.

A week or so later I got a call that my results were in.  What this test does is look to see if there is an overproduction of ACTH to determine if I have a pituitary source of Cushing's.   Someone with a normal pituitary who doesn't have Cushing's would have fairly low numbers.  Mine came back extremely high.  Even before stimulation I was at an 11:1 ratio, but at the peak of the test I tested at a 65:1.  At a 2:1 ratio it is determined that you have a pituitary source for Cushing's Disease.  Needless to say I had some very impressive numbers.  The local Neurosurgeon decided to send me to a new Endocrinologist who had recently taken up residence in our community.  I had high hopes that since I had this new evidence I would finally be on my way to getting surgery and hopefully getting better.

I met with the new local Endocrinologist and immediately was not impressed and knew it would be a dead end.  The problem that arises with most Endocrinologists is they are trained to deal with Diabetes and Thyroid issues.  When someone comes in with a truly rare disease, they believe that disease is just too rare for them to actually see in front of them.  He said I had some symptoms, but the kicker was that my stretch marks were not red or purple enough to be Cushing's and that a normal person could have those kind of numbers from an IPSS.  What?!!  I wanted to slap him.  However, he wasn't going to miss a chance at the possibility that I could have it so he went ahead and ordered me some tests.  I was able to do a few 24 hour Urine Free Cortisol's, a couple 11P.M. Salivary cortisol tests and he also had me do another dex suppression test, plus some 8 AM blood draws to check my ACTH and cortisol numbers.  I had several tests come back below normal which showed I was in a severe low, and I had some come back at the very high end of normal, but nothing that screamed out I was sick.  However it did show massive fluctuations from one end of the spectrum to the other.  The Neurosurgeon asked this local Endocrinologist to set up another Pituitary MRI.  He went ahead and got it scheduled on a newer 1.5 magnet machine.  The results came back that there was nothing remarkable on the films at all.  Oddly my partially empty sella syndrome disappeared as well.  These things do not just go away overnight, so either I had a miracle performed inside my brain or someone misread my MRI.  However, I went along with it and was discouraged again.  The local Endocrinologist said that even if I did have Cyclical Cushing's there was nothing that could be done about it. . They don't do brain surgery for someone who cycles, they only do it for full blown cases.  Once again, REALLY?

At this point he decided I should go back to Barnes Jewish Hospital in St. Louis, MO and see another Endocrinologist who was considered to be more of an expert in the field.  Once again we headed to St. Louis.  I brought along photo's of myself, my records and I was ready to tell my story.  A lovely woman went over everything with me.  She was an Endocrinologist resident there and she truly felt I had this disease.  She seemed excited to be interviewing someone with this rare disease.  She left the room for a few minutes to fill in the Endocrinologist I was there to see.  He came into the room and I immediately had that sinking feeling in the pit of my stomach.  He was extremely old and with being old in this field you always think of old school medicine.  I explained to him my symptoms and testing and was crying to him that I just wanted my life back.  I wanted to be able to be a good mother to my children.  His reply...."You need to get this out of your head that you have Cushing's."  He said he would give me two, TWO... 24 hour Urine Free Cortisol tests to do over a six month period of time.  If I was cyclical then by golly we would catch them in that six month period of time.  I wanted to scream!!!  I was absolutely humiliated and horrified that I had hit another road block.  When we left we walked out to the parking lot and I lost it.  I had a melt down and I'm pretty sure a panic attack.  I couldn't breathe.  I felt like everyone I was begging to help me was sending me to death row to die.

On the trip home I made up my mind that I was done with these "experts."  I was going to do whatever it took to get out to Los Angeles and see Dr. Friedman.  He was my last hope.  If he told me I didn't have Cushing's then I would believe him and I would try and figure out what else was wrong with me.  I knew deep in my heart that I had this disease and even though it was going to be expensive, I couldn't continue on dealing with people who knew absolutely nothing about this disease, yet were what stood in the way of me having a chance at living again.

"Zebra:  In medicine, a very unlikely diagnostic possibility. It comes from an old saying used in teaching medical students about how to think logically in regard to the differential diagnosis: 'When you hear hoof beats, think of horses, not zebras. 'For example, when someone develops a mild transient cough, a virus infection is the most logical and likely cause, and tuberculosis is a zebra."

I am a Zebra and I want my stripes to be seen for the first time in my life.