Day five of Cushing's Awareness: Visions of the future.

In May of 2010 I went to have my eyes examined.  I have to have them checked every year because I have something called Histoplasmosis which has left scars on the retina's of my eyes.  At any time they can become active and start bleeding which could cause me to go blind.  During my regular check up the doctor was asking me if I have had any changes in my vision.  I told him I had noticed some floaters that have been bothering me.  He also wanted to know if I had been experiencing more headaches than usual.  I had, but I was pretty used to headaches being a pretty normal part of my existence.  He said that he noticed I had something called papilledema which is optic disc swelling that is caused by increased intracranial pressure.  He seemed very concerned and said that I needed to have a brain MRI done and he also wanted a CT scan done of my orbits.  I asked him what could be causing this and he said there were several things, but the two most likely is a brain tumor or something called Pseudotumor Cerebri.  He explained that if it was the Pseudotumor Cerebri they would most likely put me on a diuretic type drug to reduce the amount of Cerebral spinal fluid that creates the extra pressure inside of the skull.

I left with an appointment for a brain MRI, a CT scan and something I hadn't felt in a while, hope.  I went home and started searching for some link to Cushing's Disease with all of this.  Upon looking up Pseudotumor Cerebri I immediately saw Cushing's in the list of causes.  I was in shock and I honestly felt giddy.  Who the heck feels giddy about such crappy news?  Someone who has been fighting to get a diagnosis with a rare disease, that's who.  I had my MRI and CT done and was told there was nothing found.  I decided to contact the hospital and get a copy of the report for my own records.  In the notes it said that I had Partial Empty Sella Syndrome.  I was in shock that I wasn't told about it or that it wasn't deemed important enough to tell me.  Basically the pressure inside my skull was so great with the excess fluid it was creating pressure on my pituitary and squishing it inside the bony structure which houses the pituitary.  So when looking at the MRI images it appears that my pituitary is squished down and only part of it is visible.  However, what was seen was considered normal to the "expert" radiologist.  I use that term very, very loosely.  Since the MRI and CT scan showed no "tumors," I was sent to a local Neurologist to discuss the possibility that I had Pseudotumor Cerebri.  Upon the first meeting with the Neurologist I explained my history with headaches, the possibility of having Cyclical Cushing's Disease and any other issues I had been having.  I should have known when he said "What is Cyclical Cushing's Disease?" that I should have ran for the hills.  However, I agreed to have a lumbar puncture done so that they could indeed verify that the pressure inside my skull was too high and that I had Pseudotumor Cerebri.

I had the lumbar puncture done at the local hospital.  They positioned me on my stomach, numbed my spine and then did the spinal tap aka lumbar puncture.  It turns out my opening pressure was roughly three times higher than what it should have been.  They withdrew around 11 cc's of cerebral spinal fluid and during that time I had a horrible Vasovagal response.  I immediately felt like I was going to die, throw up, have diarrhea and pass out.  I began sweating, but I was freezing cold.  The male nurse, bless his heart was trying to get me to talk and I couldn't hardly get a word out. My blood pressure plummeted as well.  He pushed fluids, held a wet washcloth against my forehead and I started to finally come out of it.  Once that was over they kept me in recovery flat on my back for several hours to make sure I wouldn't have a spinal leak.  I was sent home later that evening only to start having the worst headache of my life and only if I was upright.  I went to bed and when I went to get up the next morning I sat upright and thought my head was going to explode.  The pain was so intense.  I called the Neurologist and he said to wait it out and if it didn't get better by evening to go to the emergency room.  It didn't get better, in fact it got so much worse.  I had to get showered to go to the ER and had to literally lay down in the shower just to get myself presentable to go to the hospital.  I ended up throwing up while in the shower because the pain was just that intense.  My mom came over and got me and I had to lay reclined the entire ride there.  They gave me some pain medications and decided I needed a blood patch.  Once they injected my own blood into my spine I immediately felt better.

I was put on a drug called Diamox which is a diuretic, but I take it twice a day in a pretty strong strength of 500mg.  It is a horrible drug.  It makes me tingle all over, makes things taste horrible.  Drinking a carbonated drink while on this drug makes it taste like metal, but it decreases the amount of cerebral spinal fluid so it reduces the pressure in my skull.  After I started taking it I started noticing a lot of weird joint pain.  The doctor assumed I was having some type of reaction to it and thought perhaps I should see a Neurosurgeon about having a brain shunt put in to release the excess cerebral spinal fluid my body was creating.  I was not excited about this, but decided to go ahead and see a local Neurosurgeon who also happened to be a high school friend of my mom's and she also did a lot of the surgeries at the hospital I previously worked at.  I went to see her and explained the possibility of me having Cyclical Cushing's Disease.  She took me seriously and she also was upset that I was put on my stomach for the lumbar puncture.  She said that should have never happened and could have given a false reading on my opening pressure.  She wanted to do another lumbar puncture, but this time she was going to do it herself and in the OR with me sedated and on my side.  Later that night it is my understanding she went home and began researching Cushing's Disease.

The lumbar puncture went off without a hitch and she actually made me stay in the hospital overnight.  She didn't want me to have go through what  I did before with another leak.  She took a decent amount of spinal fluid out as well to help alleviate some of the pressure.  It was confirmed that I did indeed have Pseudotumor Cerebri, but she wasn't convinced that it was the only thing wrong with me.  She decided I needed to be seen at Barnes Jewish Hospital in St. Louis, MO and have something called an Inferior Petrosal Sinus Sampling done.  Looking back it was all done backwards, but it was this test that really changed my life and got people paying attention to me.