Day twelve of Cushing's Awareness: Released from my chains

On Sunday evening one of the Neurosurgeon's came in and told me it was time to remove the balloons (nasal pledgets) that had been placed in my nose after the surgery.  Basically they put these tampon looking things up there and inflate them so they keep pressure on the wound area so that it could seal itself shut and prevent a cerebral spinal fluid leak.  They are extremely annoying.  You can't breathe, they put intense pressure inside your sinuses and overall they just stink.  I was looking forward to getting them out.  I had heard it could be pretty painful and that sometimes a lot of blood and ooze would flow out.  He deflated them and pulled them right out.  It hurt for a half second, but the only thing I could honestly feel was relief!  I leaked out some blood and gunk, but really it wasn't much at all.  I still however had no sense of taste or smell. I also got to get two of my three I.V.'s taken out.  I had one on my left hand, one on my left forearm and also had an arterial line in my right wrist.  I am so glad I was asleep when they put that one in, as that is the same type they tried to put in when I had my IPSS the previous year and failed.  I have three scars in that area, so I'm guessing it took them three good tries before they finally were able to get it in. 

As time passed I felt a little bit stronger.  I was able to shower daily and get up a little more to do my rounds around the nurses station.  I had the most lovely nurse on one of the night shifts.  I will never forget her. She was an angel on earth.  We talked about everything I had been through and she was just the most sincere and beautiful person. She told me about the other patient who's story was almost identical to mine and that she felt it would be a disservice to humanity for us not to meet.  She asked if it would be okay if I popped in and they agreed.  So, I put on my robe and hubby and I ventured across the nurses station and there I met a new friend and her sister.  They both have Cushing's Disease and her sister had previously had this surgery with Dr. McCutcheon, but was not cured.  I was in awe of meeting not one, but two people with Cushing's and they were siblings.  I shall refer to my new friend as "B." ;)  She seemed to be doing pretty good recovering and was going to be released that day.  I should have been released that day as well, but while standing there talking to them I started to feel extremely sick.  I broke out in a cold sweat and thought I was going to pass out.  I apologized and told them I had to go lay down and to stop by before they left.  

I don't really even remember going back to my room, but I think what happened at that point was that my cortisol crashed.  After surgery they want to see your numbers drop drastically.  It doesn't always happen though.  Honestly, I still haven't seen any post op blood draw numbers except day 3 and 10 and neither of those were as low as I would have liked.  I believe at that point mine plummeted and put me into adrenal insufficiency.  I also started experiencing something called Diabetes insipidus.  I was basically having more urine output than the amount of fluid I was taking in.  I know I was constantly drinking something after the surgery and the need for ice chips the night of the surgery was intense.  I couldn't get enough.  They decided I needed to be kept at least another day for observation.  I was glad to be there another night.  I didn't feel like I was ready to go back to the hotel just yet.  They had the most comfortable hospital beds on earth.  I have never had a hospital bed be comfortable, but these things inflated air in different areas every time you moved so that it was always maximum comfort.  I still want one of these at home! LOL  

The following day which was Tuesday January, 24th they decided I could most likely be released.  I went ahead and got showered and ordered my lunch.  While sitting there eating I noticed if I ate really hot soup all of a sudden I could taste and smell.  I was excited that my sense of smell and taste were returning, even if it was only a small amount.  This was a good sign!  They said it could have taken a month or even longer, so I'll consider myself lucky.  An hour later I was released to go back to the hotel.  It felt weird to be free after having brain surgery.  We went to the hotel and I got on my laptop then I started feeling icky so I laid in bed.  My hubby the saint went to do some laundry while my mom babysat me. lol  I told her I wasn't feeling well and that I was starting to feel really, really cold.  I fell asleep for a while and when I woke up I was absolutely freezing.  I was shivering and chattering my teeth.  When I stood up some blood started dripping out of my nose.  It scared the crap out of me.  My mom said I was snoring really hard, so hard in fact she decided to take a video of me!!!  grrr mom! lol   What do you expect?  I couldn't breathe out of my nose yet! I looked at all my release papers and it said if I spiked a fever higher than 101 that I needed to go to the E.R.  My mom and her hubby left to go buy a thermometer.  I was pretty sure I had a fairly high temp.  They came back and I was running 101.9.  Ugh.  I was a bit worried I had some massive infection starting in my brain or something crazy!  No one really knows what to expect when things don't go as planned after such a major surgery.  We called the emergency hotline for the Neuro department, but no one ever responded so we packed up in the car and went to the E.R.  They got me in fairly quickly, took a ton of blood, did a urine test and a blood culture.  They gave me some pain meds and dosed me with two different types of very potent antibiotics. They had me sit forward to make sure I didn't have a cerebral spinal fluid  (CSF) leak, checked to see if I was overly sensitive to light.  I was a little sensitive, but nothing too bad.  Then they hauled me off in the middle of the night for a CT scan to make sure I wasn't hemmorhaging inside of my brain from the surgery.  Everything looked as expected for just having surgery four days prior. 

Apparently since I had left earlier in the day the whole hospital had filled up with patients.  They were going to readmit me, but had no beds.  I spent the entire night in the E.R. on this horrible E.R. bed.  My back was killing me!  This isn't exactly how I planned on spending my night.  My mom had to catch her flight back home that morning as well.  I had to say goodbye to her while I was still stuck in the E.R.. She didn't want to leave, but I told her to go ahead.  I would be okay!  Everything checked out okay, but no one knew why I was running this fever.  Finally later that morning or early afternoon they had a room for me.  They opened up a floor just for myself and overflow patients.  I got an awesome room with a nice view.  They just kept me there for observation, gave me more pain meds and kept me comfortable.  The following day I was released for good.  This was Thursday January, 26th.  I was actually feeling pretty darn good that day.  The best I had felt in a long time.  Hubby and I drove around Houston a little and I saw a Whole Foods Market. I have always wanted to go to one, so we stopped.  I don't know what the heck I was thinking, but within minutes of getting inside I thought I was going to pass out.  I clung on to the cart for dear life and made it through most of the store.  I was determined I was going to do this!  I got some awesome chapstick, some coffee to take home and we left.  Later that night we went back out.  I didn't want my last night in Houston to be stuck in the hotel.  I'm not stubborn or anything, really.  We ended up going back to the Cheesecake Factory.  I went there two nights prior to my surgery with my Uncle and my cousin.  They had valet and an elevator so I could get in easily without having to walk far.  It was nice to feel almost human again.  I was alive and eating at the Cheesecake Factory and I had brain surgery less than a week prior.  Awesome!

The following day we flew back to Illinois.  I was a little nervous about how I would handle the airport and the flight.  I had to be in a wheelchair.  I HATED being in a wheelchair, however I knew I was not capable of walking around the airport so I grudgingly did it.  The pressure in the plane wasn't too bad.  My head was hurting a little bit.  I stress dosed with some hydrocortisone and everything seemed to be fine.  We made it back into Chicago and my dad picked us up to take us back home.  It was good to be back in Illinois and especially great knowing my babies were only a few hours away!  We stopped to eat dinner and then headed home.  My dad had bought us some groceries and stocked my freezer with meat and all kinds of other goodies.  Once we got home and got our car we took off to my sister's house to get my babies.  We didn't ring the doorbell, we just walked in.  My babies lit up like a Christmas tree!  It was heaven.  I just held them in my arms and sniffed them.  Everything I just went through was worth it in that moment.  I was alive and I was back with my babies.  We packed up the kids, all of their belongings and headed back home as a family. :)  I figured that recovery wouldn't be too bad at this point.  I was feeling pretty good minus some headaches and being a bit nauseous.  

I am forever grateful to my sister for taking such good care of my kids while I was away.  We were able to Skype several times during my hospital stay, but I cannot tell you what a relief it was for me knowing that they were being taken care of and loved as if they were her own children when I couldn't be there.  Thank you, Ash!  

Day Eleven of Cushing's Awareness: I'm a survivor!

One of the first pics taken after my surgery.
Beautiful, eh?  

My next memories are of waking up with my family looking down on me.  They had a washcloth on my head and were telling me that they got it all and removed 25% of the left side of my pituitary and 5% off the right. They told me I didn't have a cerebral spinal fluid leak, which was a big worry due to my increased brain pressure issue.  I remember of all the things that happened to me,my right shoulder was absolutely killing me.  They gave me a shot of dilaudid and I immediately thought I was going to throw up.  They gave me some Zofran and kept me in recovery for a bit.  I had been told I would be going to the ICU, but apparently I was stable enough to go to my own private room on the Neuro floor.  I remember being wheeled to my room, nurses everywhere checking all my stats, getting me situated and checking my pain levels.  I was SO thirsty.  They would only let me have ice chips that night.  My poor husband and mom were up half the night feeding me ice chips.  It was horrible how thirsty I was.  I was also burning up.  I felt bad because they were both practically wearing winter coats and I was on fire!

The next day they wanted me to try and get up at some point.  I was in entirely too much pain and didn't think I could manage it at all.  I had tears constantly running down my face, yet I wasn't crying.  I had no idea why this was happening.  The pain was much more intense than I had anticipated.  I had read about other people who had already been on this journey through this surgery and most seemed okay with pain fairly quickly.  Why was I hurting so badly?  I have a very high tolerance to pain.  Finally, my mom decided to tell me the whole story.  Apparently I had a nose job in order to have the surgery.  They had to resect my middle turbinate in order to get access to my pituitary.  Apparently mine was quite prominent and they couldn't fit the instruments inside without a resection being done.  I wasn't just dealing with the pain of brain surgery, I had intense bone pain on top of it all.  The Neurosurgeon resident told me that the tears were from my pain level.  At least I didn't have to worry that I was going to be a freak with tears running down my face forever.  lol  That day was very rough for me, but I made it through.  I still did not manage to get out of bed though.  I think I would have dropped to the floor if I even attempted to stand on my shaky legs.  I was able to get pain meds roughly every two hours.  One being an injection of morphine and then 2 hours later they would give me percocets.  It helped somewhat, but after an hour and a half I was practically begging for more.  I would literally count down the minutes until I could beg for drugs.  Once again,  I have a very high tolerance to pain, but this pain was just so intense.  I was able to have a normal diet, but I mostly only wanted soup or jello.  The roof of my mouth, especially above my top two teeth was extremely numb and there was a flap at the back of my throat that had been sliced and hurt from being intubated.  One of the awesome things about MD Anderson is they have a 24 hour Starbucks!  Mind you, I couldn't taste or smell anything but just knowing that it was a Starbucks helped me feel at home. lol  I couldn't taste or smell for at least three days.  I was starting to get concerned that it wouldn't return.

I believe it was Saturday evening or early Sunday morning I finally felt strong enough to try and get out of bed.  The first thing I wanted was that darn catheter out and I wanted a shower.  My head felt and looked like it had been coated in vaseline.  It was disgusting and I cannot stand feeling greasy.  Hubby had to help me get showered and I had to sit for the majority of it because my legs were practically buckling under me.  My vision was also very blurry and I felt extremely dizzy.  However, that shower made me feel a hundred times better.  After my shower I rested a bit more and they wanted me to do a couple laps around the nurses station.  I clung to my husband and did my laps.  I couldn't believe how weak I was after this surgery.  It's like my body forgot how to work properly.  It was utterly annoying.  The pain was still very intense, but I felt like I was starting to come out of the woods a bit.  I'll go into more detail about the following days tomorrow.

Now for some diagrams about the surgery I had.  It was called a Transsphenoidal resection of pituitary microadenoma.  This website for the Mayfield Clinic has an amazing explanation and diagrams of what happens during this surgery.  Click on any of the pictures to be taken to their website.  The first two images have different links.

Day ten of Cushing's Awareness: Eviction Day!

January 20th, 2012 was here.  This was the day I had been fighting for since I realized I was sick and knew it was in my head, literally.  I woke up pretty early on my own, which was no surprise.  I didn't have to be at the hospital until later in the morning.  I was second on the surgery list.  Turns out the person going before me also had Cushing's Disease and was having a pituitary tumor or tumors removed as well.  It is a rare disease, but it's rarely diagnosed so I'm pretty sure there are so many more people out there dealing with Cushing's and pituitary issues.  I got myself showered, packed and was able to Skype with my babies and my sister one last time.  I was eerily calm.  Considering how nervous I had been up until that point, I was astounded by the amount of calm and comfort I felt.  I believe that the prayers that were with me played a huge part in it, I also believe the fact that I had faith in my surgeon was also a great deal as well.  I knew that the only step to get better was to get on that surgical table and let them rid me of my demons.  I was ready.

We left the hotel and headed to the hospital.  I was still so very calm.  I am not usually a freak out type of person, but I had my moments before I would go to sleep at night while still back in Illinois.  I would think about the surgery and my heart would start going crazy and I just had to take a deep breath to make it through the dark thoughts in my head.  I am my own worst enemy sometimes.  I got checked in and we all sat in the waiting area for quite a while.  Apparently they were running behind schedule.  I got to visit with my family some more and have some good laughs.  I then got called back.  I felt like I was having an out of body experience.  I didn't quite feel I was in control of myself, but felt I was watching everything happen through another person's eyes.  It was quite surreal.  I said goodbye to  my family and my husband and I headed back to the surgical staging unit.  I had to get undressed and put on my lovely gown.  Then they fitted me for my embolism stockings.  The ultimate in sexy wear, let me tell you.  lol  Once I was settled in I texted my mom and told her to come back as well.  I knew she was like a caged animal out there and I needed her with me anyways.  No matter what age your child may be, a mom never loses that instinct of needing to protect and nurture their young.  My husband went and got her and showed her back to me.  I was able to sneak in another call to my babies and I felt the first wave of tears and fear hit me.  Hearing my babies about did me in, but then I thought to myself I had to buck up.  I was doing this so I could be around for a very, very long time.  I was going to fight this beast head on and I was ready.  I told them I loved them and said goodbye.  Then the Anesethesiologist came back and asked a lot of questions and explained what they would be doing and if they had issues they couldn't use my nose because that was the route Dr. McCutcheon would be using to access my pituitary.  They put an I.V. in and gave me a little something to take the edge off and then they came to get me. I saw the look of panic on my mom and husband's face.  I knew they were trying so hard to remain strong, but I could see they were about to break.  I hate seeing my family scared and hurting.  I kissed them goodbye and off I went.

They brought me into the operating room and had me slide on to the surgical table.  On the wall were computer screens of my brain MRI.  It was pretty neat.  I remember them asking me some questions and they said they were going to give me something to put me to sleep.  I was out before I could even have another thought.

Goodbye Cushing's!

Day nine of Cushing's Awareness: My new birthday?

After getting my official diagnosis I continued researching Doctor's who specifically dealt with Cushing's tumors.  Not just any Neurosurgeon will do for a job like this.  Sometimes a Cushing's patient will have a nice well defined tumor.  Those are the ideal ones.  They are easy to remove, most likely leave behind no residual tumor cells and the patient is more likely to have a remission and possibly a cure.  Then there are the tumors that are more spread out.  Just by looking at my MRI it was pretty certain to me that I didn't have a well defined one.  I had three areas of interest and with that comes a term no patient with Cushing's disease wants to think of, hyperplasia.  There is a chance with hyperplasia that the bad cells are pretty much spread throughout the pituitary and mixed in with the good cells.  All it takes is one evil tumor cell to remain to keep us sick or start regrowing a new tumor.  I needed someone who knew the difference in appearance of these type of tumors, is experienced and had performed this surgery many, many times.  I also read a lot of experiences that others had with surgeons on the Cushings-help.com boards and other types of Cushing's groups.  

I made my decision to go with my first instinct which was Dr. Ian McCutcheon at the MD Anderson Cancer Center in Houston, Texas.  I still hadn't even had my MRI read, but Dr. Friedman was pretty confident it was all there.  Dr. Friedman went ahead and got my referral sent off and the very next day MD Anderson was calling me to set me up in the system.  Talk about excitement and nerves!  They took down all my demographic information and then told me that the person who booked the appointments would contact me the following day.  The following day came, and indeed they called.  My surgery was booked for Friday, January 20th, 2012.  Was this going to be my new birthday?  A new lease on life?  So many thoughts spinning through my head now that I officially had an eviction date for the "Three Stooges."  I decided to hold off until January because I didn't want to be recovering for my daughter's sixth birthday and also my little sister was getting married on New Year's Eve.  Everyone told me to go ahead and do it, but my gut reaction was to wait.  I am extremely glad I did.  Every single day I was getting worse, but at least I was physically able to be there for the holiday's and my sister's wedding.  Plus, at my sister's wedding during her maid of honor and best man speech decided she was going to do a speech herself.  This speech was about me, and how grateful she was that I was  able to be there for her special day and the fact that I put off my surgery to be there.  She had over two hundred people praying for my well being during the next few weeks ahead and also in my recovery.  It was one of the moments in my life no matter how bad my memory gets, I will never forget.  I was deeply touched and I'm still pissed at her for making me cry in front of people. ;) *Love you, Ash!*

I got everything finalized for the trip and on January 17th, we left for Houston, Texas.  It was a very difficult day for me.  I left behind my two babies with my sister and kissed and hugged them all goodbye.  I broke down when we left their house without my kids. I honestly wondered if it would be the last time I saw them. I know that sounds morbid, but having this disease and thinking about having brain surgery makes one think some very, very dark thoughts.  Would I be functional again?  Would my husband be able to handle them alone if I were to die? How would my daughter cope without having a mom as a teenager when she needed me most?  Just writing these things puts a lump in my throat and takes me back to those thoughts and I hate that I have even had to worry about such things at such a young age.

Our flight went smoothly and once again once the city lights came into view I got overcome with emotion.  This was where my life was going to change.  It was really happening.  I was going to finally get these bastards out of my head that had been making me sick for so, so long.  

The following day my Uncle and Cousin joined us. They were my own little angels during a very, very scary time for me.  They kept me in tears from laughing so hard.  I honestly don't know how I would have made it through those days prior to surgery without their comic relief.  I will forever be grateful for having them and the fact that they drove all the way to Texas to be with me.  I love you, Uncle G and little G!  My Mom  and her husband showed up later that night as well.  There is no way I could have managed this without my Momma.  However, I'm pretty sure she was more worked up and nervous than I was!  

On January 19th I had to be at the Clinic at 8 A.M. for blood work and then to meet with Dr. McCutcheon.  Now came my time for being nervous.  I was an absolute mess the hour before meeting with him.  I was about to meet the man who was going to be digging around inside my skull the following day.  That is a huge ordeal, let me tell you.  They called me back to take my blood pressure before meeting with him.  It was pretty darn high, scary high in fact.  I explained it was from stress and I am not normally running that high.  I went back into the waiting room and then they called my name.  I seriously thought I was going to throw up. My husband, mom and I all walked into a room and waited for Dr. McCutcheon to come in.  We didn't wait very long and then I heard the knock and then he entered the room with his assistant.  I was immediately overcome with a flood of emotions.  I had seen multiple pictures of him and had been researching him, yet here he was.  Right in front of me.  He started reading through all my notes and we started talking.  I instantly felt a sense of calm wash over me.  I knew once he began speaking that this man was 100% the right choice.  He was funny, super intelligent and extremely compassionate.  I have never in all of my years being sick, met a Doctor like him.  He loaded up my MRI into this machine and we read it together.  This was his first time seeing it as far as I know.  He would adjust the contrasts and showed me all the areas that had been seen before.  It truly was an experience watching a genius in motion.  He would stop and show me exactly what he saw and explain that these were not normal tumors.  He explained everything in such great detail so that I felt completely educated on the films myself.  We then discussed how he indeed felt this all needed to come out and that we would continue with the surgery as planned.   He then went over every detail of what would be done to me and he even personally read through every consent form and handed me his own pen to sign them with.  This was by far the most personable Doctor's appointment in my entire life.  I thanked him and told him to get plenty of sleep that night..LOL!  Immediately after the appointment they took my blood pressure again and it was completely normal.  That is the amount of comfort Dr. McCutcheon gave me.  

We were able to leave the clinic and we decided to go out and have a fun evening in Houston.  I picked out this lovely German restaurant to have my last meal at and of course had to hit up a Starbucks afterwards.  I love my family and am so grateful they were there with me during those days prior and after my surgery.  I am blessed to have them. I skyped with my kids and told them how much I loved them and tried to get some rest. I was surprised how easily sleep came to me that night.  The following day my entire world was going to change.  I was giving Cushing's Disease the boot!

National Cushing's Awareness Day!

Today is National Cushing's Awareness Day and it is also the birthday of Dr. Harvey Cushing.  He is the Neurosurgeon who first discovered this disease and was considered one of the pioneer's of Neurosurgery in the early 1900's.

What are Cushing’s syndrome and Cushing’s disease?

Cushing’s syndrome is a rare condition that is the result of too much of the hormone cortisol in the body. Cortisol is a hormone normally made by the adrenal glands and it is necessary for life. It allows people to respond to stressful situations such as illness, and has effects on almost all body tissues. It is produced in bursts, most in the early morning, with very few at night.

When too much cortisol is made by the body itself, it is called Cushing’s syndrome, regardless of the cause. Some patients have Cushing’s syndrome because the adrenal glands have a tumor(s) making too much cortisol. Other patients have Cushing’s syndrome because they make too much of the hormone ACTH, which causes the adrenal glands to make cortisol. When the ACTH comes from the pituitary gland it is called Cushing’s disease.

Cushing’s syndrome is fairly rare. It is more often found in women than in men and often occurs between the ages of 20 and 40.


What causes Cushing’s syndrome and Cushing’s disease?

Cushing’s syndrome can be caused by medication or by a tumor. Sometimes, there is a tumor of the adrenal gland that makes too much cortisol. It may also be caused by a tumor in the pituitary gland (a small gland under the brain that produces hormones that in turn regulate the body’s other hormone glands).

Some pituitary tumors produce a hormone called adrenocorticotropic hormone (ACTH), which stimulates the adrenal glands and causes them to make too much cortisol. This is termed Cushing’s disease. ACTH-producing tumors can also originate elsewhere in the body and these are referred to as ectopic tumors.


What are the symptoms of Cushing’s syndrome?

Not all people with the condition have all these signs and symptoms. Some people have few or “mild” symptoms – perhaps just weight gain and irregular menstrual periods. Other people with a more “severe” form of the disease may have nearly all the symptoms. The most common symptoms in adults are weight gain (especially in the trunk, and often not accompanied by weight gain in the arms and legs), high blood pressure (hypertension), and changes in memory, mood and concentration. Additional problems such as muscle weakness arise because of loss of protein in body tissues.

COMMON FEATURES	LESS COMMON FEATURES
Weight gain	Insomnia
Hypertension	Recurrent infection
Poor short-term memory	Thin skin and stretch marks
Irritability	Easy bruising
Excess hair growth (women)	Depression
Red, ruddy face	Weak bones
Extra fat around neck	Acne
Round face	Balding (women)
Fatigue	Hip and shoulder weakness
Poor concentration	Swelling of feet/legs
Menstrual irregularity	Diabetes

Information taken from http://www.pituitarysociety.org

Since it is National Cushing's Awareness day I find it fitting to go ahead and continue on with my story.  I left off that I was waiting for Dr. Friedman to call me on October 2nd, 2011 and discuss a plan of action for testing and to talk about all my test results.  I expected that I would have plenty of months ahead of me in which I would need to keep testing.

The phone rang and Dr. Friedman asked me how I was doing.  I told him I wasn't doing that great and in fact I felt worse than when I had seen him in June.  He told me he wasn't surprised and then he began telling me about my test results.  He told me I had several tests come back high and that I even had an 11P.M. Salivary test come back pretty darn high.  In all of my testing I have never, ever had a salivary cortisol test come back high.  He said that with all of these results he felt confident that I was ready to move on to surgery.  At this moment I about dropped the phone and then I said "Are you telling me that I have Cushing's Disease?"  His response was "Yes, I do believe you have Cushing's Disease."  At this moment I felt like every muscle in my body turned into gum.  I wanted to scream, cry, dance, you name it, but I had to keep my composure for the rest of the conversation.  Dr. Friedman asked me if I had a surgeon picked out.  I told him I had one in mind, but I hadn't officially made up my mind yet.  He told me to make sure Dr. McCutcheon read the MRI and that when I was ready he would get the referral going.  I thanked him profusely for believing in me and doing what no one else could do in almost twelve years of trying to get diagnosed.  ONE month of testing and I was diagnosed.  October 2nd, 2011.  A day I will never forget.

Once I hung up the phone I began to scream.  Screams of frustration, joy, validation, anger, every emotion from the past twelve years that could be mustered up came out of me at that moment.  My body was literally shaking from the amount of emotion that was pouring out of me.  FINALLY, I had my diagnosis.  I called my parents and let them know it was official.  My mom and sister soon showed up at my door to just hug me.  It was that pivotal moment in time where everything really was going to change.  No more dreaming about it, it was here and it was NOW!

I have Cushing's Disease and no one can ever dismiss me again.

Day Seven of Cushing's Awareness: Los Angeles, my city of Angels.

When I got home from that most devastating Endocrinologist appointment in St. Louis I put my research cap on.  There were two doctor's in the Cushing's community that were considered to be the experts in the country.  I decided Dr. Friedman was by far the one that would fit me the most in terms of his testing methods. I was sure that when I had highs they were at night and that I was bottoming out during the day.  I needed someone who believed in Cyclical Cushing's and knew how to test for it.  Dr. Friedman has tests set up perfectly for someone like myself.  Most Endo's only collect a 24 hour urine cortisol, where as Dr. Friedman does a 10 hour that is from 10 at night until 8 in the morning.  This is an excellent way for someone like myself to catch the highs.  Also he does other tests that most Endocrinologists don't do that check to see if recently your body had been high with cortisol.  This was one of the tests the I scored high on, even when my pee tests would come back normal.

Once we got our tax refund back I decided it was time to take action.   I booked the appointment and got everything set to go to Los Angeles on June 6th, 2011.  It never would have been possible if it weren't for the help from my family.  I am forever grateful to them.  I spent days working on getting all my past medical records together in a huge binder, collecting photos to show Dr. Friedman and just getting myself ready to go to L.A.  I was scared to death, yet I was hopeful.  What if he told me I didn't have this?  Was this my fault somehow?  All these questions running through my mind.  I was having a hard time just leaving my children behind for five days to head out there.  I had never been away from my babies before, but I knew if I didn't do this they may not have a mom around anymore.  I had to put on my big girl panties and do this.

I remember the feeling as we were starting to land in Los Angeles.  I was so overwhelmed with raw emotion.  I teared up and thought to myself, this is it.  Everything I have been through has led me to this city.  Would it live up to it's name as the city of Angels?  Everything went perfectly once we arrived.  Very odd things happened that I cannot explain, but I felt surrounded by Angels.  A song came on the radio that I associate with my Grandma who passed away.  I was very, very close to her.  This song was very old and there was no way it would just randomly come on without it being a sign from above.  I felt immediately at peace and knew that I was in the right place.  On June 7th, we arrived at Dr. Friedman's office.  My hubby took a pic of me in front of the sign with Dr. Friedman's name on it. Cheesy, I know but this was hopefully going to be my life changing experience.  We arrived way too early so we just hung out until it was time to go in.  I felt complete panic walking into his office. I was greeted by Dr. Z who immediately almost put me in tears.  He said, "Let me guess, you have been sick for at least 5 years, have seen multiple specialists and they have all told you it was in your head?"  I nodded as the tears began to well in my eyes.  He said "I can tell just by looking at you that you have this disease and that you will finally be taken care of."  As I write this I am still overcome with emotion from those words he spoke to me.  Someone finally could see what I had been trying to say for years.

He took all my vitals and wrote that my pulse was high.  Another symptom of Cushing's.  I rarely have a pulse below 100.  I then returned to the waiting area until Dr. Friedman was ready to see me.  He called me back into his office and immediately turned into this nervous mess.  How could I feel calm when my life was on the line and "The Wizard" was right in front of me asking me questions.  He asked me about my story.  I told him.  He looked through my files and of all the sheets in my file he took all my high results and my IPSS results.  He then wanted to see my MRI cd that I brought with. I watched him load it on to his laptop and then he said to me...."And they read this as normal?"  I instantly felt a lump in my throat.  He turned around his laptop and pointed to me on the screen two areas he believed were tumors.  Then a minute later he decided there was a third.  At this point my brain was racing.  I could hardly speak.  I was so overcome with emotions of anger, fear and validation.  He feared that the type of tumors I had may be aggressive because of the way they were showing up on film.  He also examined me and said that he believed I did have Cushing's and he also believed I was growth hormone deficient.  He gave me a battery of tests to do once I returned back to Illinois and a plan of attack for testing.  Someone BELIEVED in me!  The following day I was set to have another brain MRI at Cedars Sinai on a 3 Tesla MRI machine.  It's considered to be the best imaging source for MRI that is out there right now.  He also put me on some Vitamin D drops because mine is low and also wanted me to start taking L Glutamine twice daily to try and build up some of the muscle I had been losing from being sick with Cushing's.  After meeting with Dr. Friedman they drew some blood and I was free to go.  I walked outside and just started crying.  After I regained my composure I phoned my parents to let them know what had just taken place.  My mom, bless her heart was a mess.  No one wants to hear their child has brain tumors, but to me I was overjoyed.  I know that sounds absolutely crazy to a normal person, but to someone who has been sick and knew it was just a matter of time before it reared it's ugly head, it was a target.  A target to evict and hopefully give me my life back.

I did the MRI the next day and spent a few more days in Los Angeles before heading back home to Illinois to begin my testing.  The testing included multiple 24 hour urine free cortisol tests, multiple salivary tests to do at 11 at night or later, 10 hour Urine Free Cortisol tests and he would also check for hydroxycorticosteroids aka 17OHCS which is what would show if I had recently had a surge of cortisol in my system.

It took roughly a month of testing to complete all the tests he wanted me to do.  I would do tests basically 4-5 days out of the week.  I was tethered to the house for the majority of my testing, because collecting urine for 24 hours is not fun to do anywhere else.  Who am I kidding?  It's not fun EVER at any place!!

So in the meantime I waited for all my test results to come back in, got my MRI results which showed several areas where the contrast did not go through, but they couldn't say for sure there were tumors.  Dr. Friedman wanted me to send my MRI off to Dr. McCutcheon in Houston.  He is considered to be one of  the best Neurosurgeons in this field and amazing at seeing what most miss on the MRI's.  So I shipped off my MRI's and began the waiting game.

I  received an email from Dr. Friedman's office stating he would like to have a phone appointment to discuss my results and a plan.  I figured this meant how we were going to proceed with testing.  I had a sheet of paper with questions to ask.  Could I do midnight blood draws so that I could have those highs as well?  I know that I needed highs in at least three different testing areas for him to confirm I had Cushing's. As far as I knew I had only one test come back high so far, but had not seen all the results yet.  I set up the appointment and waited for the call with my questions in hand.  I was prepared to do as much testing as it took to prove I had Cushing's.  I was not prepared for what took place during that phone conversation.

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.