The Ordeal part 5

I laid like a good little patient on my back for the next 5 days. I watched movies when I felt up to it and slept a lot. (I was having some serious back spasms and was on Valium so I could count on a couple of good naps every day.) My nurses were fabulous, and at St. Raphael's unlike Yale, they give you the same nurses every day they are available. What a wonderful group of nurses! They kept me clean, happy, well-drugged, and snuck my kids in a couple of times to see me. (No one under 12 was allowed on the floor.)

On the 6th day they sat me up about 40 degrees (which to me felt like I was standing up) and gave me a cup and said let's see if you leak....Sadly enough.. I did. One of the Neuro PA's took the cup and things started rolling for another surgery. This time a CSF (Cerebral Spinal Fluid) Revision. My darling nurse Brittany came and sat with me while I cried a little bit, and she got teary, and then said, "Well, at least we get you for longer."

In some ways I was lucky because the best ENT was available that day and wasn't for the rest of the week. The other good thing was that as soon as my nurses heard I would be around for quite a while longer, they switched my room to one that had a TV in good repair. Sounds lame, but every little thing is exciting when you are on bed rest.

I called my mom and Jason and both sounded pretty dejected and resigned. Jason rearranged patients for the rest of the afternoon and he and my mom came over to be with me while everything was prepped for a 2:00 surgery. As we were told the problem with a revision is that the surgeon never really know what the prior one did and they don't like going into an unknown.
This neurosurgical group decided that they wanted as much of a visual field as possible so they decided they would cut the skin right under my nose and peel the skin back so they could see a lot more. Pretty gross, but they thought they would have better luck that way.

Around 2:00 I was wheeled back into the surgical waiting room. My mom and Jason were able to stay there with me right up to surgery time. We were all a little weepy at having to do this again. Jason was promised that this time someone would come and talk to him about the surgery immediately following the surgery. They gave him a 4 hour time frame. I was anxious about the CSF headaches coming back, but otherwise in as good of shape as possible.

I was wheeled into the ER and the ENT came in and put on "Phantom of the Opera" and was singing along to it. I told him that was my least favorite musical ever. He chuckled and said if I could sing to it he would change it. So I sang right along with him and he asked me what musical I preferred. I told him "The Fantastiks" and I fell asleep to "Try to Remember." Much better.

I woke up briefly in a CT room. Apparently my pulse had dropped very low unexpectedly during surgery and they were checking for blood clots. The next thing I remember was waking up in my room in the ICU with my nurse Brittany and Jason. My nose was packed and oozing and I had a whopper of a headache, but the surgeon came in and said everything looked OK. They found a secondary leak to the one I originally had and so to fix everything they took a piece of my septum and wedged it in between 2 pieces of cartilage, took a piece of fat, some duraseal (basically surgical glue) another piece of fat, and more duraseal and packed my sphynoid sinus completely. I am told I will never miss the back of my septum and I can't say I have to this point. I was still plugged up to the drain and would be flat for another 5 days.

Pain management was much better this time around. when I wasn't getting a lot of relief from Morphine they had me take Percocet and that helped immensely. The next 5 days passed as slowly as the previous 5, but adding in the pain medication I slept a lot more.

The ICU became fairly homey in its way. These are the things I will remember about my various stays. Someone is always up, the respirators make sounds like someone is playing the oboe, tiny taps of people walking quickly up and down the halls, that darn curtain being thrown open so someone can come in to take more blood, check your levels, or answer your call light, quiet conversations, gentle nurses hands, and wanting to go home so badly.

5 days after surgery I had my nasal packing removed. I have been told by many a friend and relative whose noses have been broken that this is the worst experience ever. I demanded that I be doped up before they did it. The on call ENT said, "I don't really hear complaints about it." I asked her if she had ever had it done. She admitted she had not, so I said, "I'll wait for some drugs." My darling nurse Cindy gave me some morphine through my IV and we waited a few minutes and then had the packing pulled. I'm so glad I wasn't all the way "with it" for that. Pretty horrid.

6 days after surgery, I was sat up again to watch for leaks. I was petrified I would leak again. But we waited and waited and the day passed with everyone watching and waiting. On the 7 day, the Neurosurgeon decided we were successful and in a highly anti climatic moment they pulled out the drain. Of course, they then told me I had to stay on my back for the rest of the day to keep pressure on the drain site! I stayed in the ICU for one more day as they monitored and got me up to walk (that was fun!) and then I was transferred to the regular floor for one day to irritate me!

I'll post about my homecoming next, but the medical saga for now is over. I'm hopeful we don't have any more adventure for a long time coming. Recovery is slow and frustrating for someone who wants to run a million miles an hour in every direction, but I am trying to pace myself, and when I don't, my body does it for me.

People keep asking me if I would do it again, knowing this all would happen. Of course I would. What choice did I have? I could live with a disease that slowly killed me or I could be brave enough to jump at a chance to live my life in a healthy body. I took my chance, and it wasn't the easiest course, but it is still my first best chance. My Aunt Kristie told me that one of my Grandpa's favorite things to say was, "When I get something, I really get it!" I am his granddaughter after all!

The ordeal-part 4

When last I posted I was headed home for some much needed and anticipated rest. My mom and Aunt Kristie had headed to their various homes to do a few things that needed doing in their lives. I was left in the very capable hands of my cousins Lacey and Ashley. They marched in and took over all child care, house cleaning, fun having, and they made me rest a lot. We had a fairly quiet weekend and I was even starting to get pretty stable walking around.

My mom and Kristie returned on Monday and I mentioned that I had a lot of sinus pressure and was worried I might be getting a sinus infection. (A fairly normal complication of pit surgery) I had an ENT appointment scheduled that Monday morning with a local and respected ENT.
My mom and I went to see the ENT who squirted some numbing solution into my sinus and cleaned out some of my sinus cavity. He said everything looked fairly normal, but he gave me some antibiotics to take just in case.

Later that evening I started tasting a lot of salty liquid dripping down the back of my throat. The next morning, I sat up and salt liquid was dripping out of my nose. I took a deep breath and called the ENT again. I told them I thought I was dripping cerebral spinal fluid from my nose and I needed an appointment as soon as possible. Kristie drove me to the appointment a few hours later. Aidan was not too keen on me going to the doctor and he cried a bit. I told him, "It's not the hospital, I'm just going to a regular doctor, then I'll be home." He looked at me suspiciously and said, "Mom, you've got one hour!"

The same nurse tried to spray the numbing solution in my nose, but I told her no I wasn't going to do that. She kept telling me they had to and I said, I wouldn't have anything up my nose until I saw the Doctor. When the doctor came in he had me bend forward and try to drip fluid into a cup. I got a drip within a minute. He swore and said he needed to make some phone calls. He was back about 5 minutes later and told me I needed to go directly to St. Raphael's hospital in New Haven and I would be a direct admit to the ICU and would meet with a team of Neurosurgeons there. They were so anxious to get us out of there that they said, they would call our cell phones along the way if they needed anymore info.

I was a little bit teary eyed on the way to the hospital. Kristie was so sweet and kept telling me "It's OK to cry and be upset." I just felt like I had to keep it together. We called my mom along the way and she said, "No, I don't want that....just don't go!" We were all pretty bummed. I had only been home for 3 1/2 days.

I arrived at the hospital and was sent directly to the surgical ICU. Kristie and I had to wait a number of minutes before anyone paid any attention to us at all. Finally someone looked at me and I told her my name, and she laughed saying they didn't realize I was a patient because they never have patients walking and talking to be admitted. I was taken to a room slightly smaller than the ICU at Yale and the flurry of activity began. I was asked a million boring and personal questions, I had to change into a gown and get in bed. (However, before I did, I told my nurse I was walking to the bathroom like a normal human. She said, "Well we don't really have a protocol for that so you should wait for your catheter." I told her, she could walk to the bathroom with me or watch me go by myself. All the nurses decided I was "fiesty" I told them I was just grumpy.

I told the nurses over and over again that I needed my hydrocortisol at 7:00 AM and at 2:00 PM and that if they weren't on top of it, I did have some in my bag and I would be dosing myself. I told them I did it at Yale and I would tell them I did it, but I wasn't waiting for any medication. They cringed a bit. (Once again, labeled fiesty.)

I was taken on a gurney to have a CAT scan just to make sure there was no bleeding anywhere, and then taken back to my sterile prison. (Kristie was still there and decided it was the ugliest hospital ever, and they wouldn't allow live flowers, my TV was fuzzy, and they were very strict about visitors.) I was keeping in fairly good spirits, but kept telling my nurse I was tough until I saw my husband and then I always cried. Since Kristie was still keeping me in good humor we were fine.

About an hour later I met with the first neurosurgeon who told me the "good news". They were going to place a tube to drain spinal fluid in my spinal cord and let the pressure equalize for 5 days to see if the dripping would stop on its own. They would let 10 ml an hour drip (same as before) and I would have to stay flat on my back. "What if that doesn't work?" I asked. "Well, at that point we would have to do a revision on your original surgery." he said. So I found the good news a little less depressing. I asked why I would start leaking now, and he had a couple of theories... the pressure from the airplane flight home was too much, I lifted something to heavy, it was a bad patch to begin with. Then he quickly and quietly asked if I had any leaking at all before going to see the ENT. I told him, "No". He was really quiet and asked me not to see that doctor again. I kind of think I got his real opinion....

The nurses came in to prep me for my "small" surgical procedure. Jason had shown up by this time. As soon as he showed up, I started crying. I think sometimes you have to be strong until the real strong one shows up. I started crying because I had told my darling little 5 year old that I would be right back and I was done with the hospital, and here I was committed to at least 5 more days.

Those crazy anesthesiologists insisted on doing a pregnancy test before the surgery. I told them, I had been in a hospital for the past 2 1/2 weeks and my husband wasn't stupid. They laughed and made me do it anyway.

I had another small blessing right before surgery. A phlebotomist named Carol Ann stopped by to take blood levels fore the surgery. She peeked in my room and said, "Alicia, what are you doing in here?" She had been the phlebotomist who worked midnights for the past year and we got to be friendly when I would come in for midnight blood serums during the diagnosis process. She had just been moved to evenings and she told me about how she had been sick for over a year and couldn't walk and how she had an angelic friend who bathed her every day and got her kids ready for school.

She then turned to Jason and asked, "How are you doing darlin'?" I don't think anyone had ever actually asked him that. We were both so touched. She said, it is so hard to see someone you love like this and to be in charge and have to keep up a "normal" life. She assured me I would be fine and to come and visit when I was better. I didn't see her again my entire hospital stay, but I truly believe I saw her then because we needed her.

I was wheeled into a freezing operating room, given something to help me sleep and I woke up with a headache and back spasms. Once again, Jason had been left in the waiting room for much too long a time. They told him it would take about an hour and 2 and half hours later the surgery was over. (Apparently, I have a strangely narrow spinal column and they told me they had to "beat me up" quite a bit to get the drain in.)
It was almost 10 pm by now and Jason wasn't allowed to stay the night, so he headed home and I began my 5 day vigil.

The Ordeal- Part 3

I came home from Houston and still had some very severe headaches. I emailed the surgeon who quickly responded that quite often CSF leaks will cause headaches for a week or so after being resolved. I pretty much took pain medication and slept for the next day. The day after I sat downstairs for a while and then took more pain medication. I still couldn't eat a bite. I was tempted with all kinds of yummy foods (even a diet coke) and I wasn't slightly hungry.
That evening (Saturday night) I was feeling really anxious and couldn't sleep. I started feeling nauseus and scared when I couldn't feel the tips of my fingers. I roused Jason at about 1:30 AM and said I thought we should go to the ER. We left the kids with Kristie, Mom, and Dad who was up for the weekend and headed to Yale.

When we arrived there wasn't anyone around to help us, so Jason went back into the ER and said, "My wife had brain surgery a week ago and isn't feeling great." They had me back in an examination room so quickly it was unreal. Initially the doctors asked a lot of questions about the surgery and seemed to think that I might have adrenal insufficiency. They tried to take blood from about 5 different veins before they finally got some. I went in for a CT scan, they checked out my neurological responses (which were fine).

After about an hour, the resident returned and told me I was being admitted because my sodium level was 119. (Normal levels are between 130-145) I was then asked a million questions about what had gone on between my surgery and coming to the ER, because in their minds there was no way sodium could go that low in the day and a half I was home. They also kept asking why I came in to the hospital and I told them it was because I couldn't feel my fingertips. (They didn't seem to think this was a good enough answer...) They kept asking if I had headaches and I said, "Of course I have headaches, I just had part of my pituitary removed!"

I was admitted to a normal floor at Yale and was seen rather quickly by a endocrinologist resident. She asked all of my medical history and then tried to have me drink some liquid form of sodium. One sip made me throw up all over the place, so we didn't do that again. She then said I was to be on a water restriction to see if that would raise my sodium levels.
In the morning a team of endos came in and questioned me about my surgery and if I truly had had Cushing's because they didn't see any red stretch marks or a "buffalo hump." They then said they were trying to get a handle on my sodium levels so they were going to raise my cortisol supplements to 4o mg in the AM and 20 mg in the PM. I still wasn't eating and I felt miserable. People kept asking where I hurt and I couldn't tell them. All I can say is that everything felt wrong and everything hurt.
I asked the endocrinologist to page Dr. Friedman to let him in on the situation and get his opinion. She said she would, but it was too early in the morning there. I asked again about noon our time and she said she would but didn't. I asked again around 4:00 pm and she said they really had a good team here, and they would keep him informed. At 4:30 pm Jason paged him himself, gave him the run down on what was going on and then walked into the endo conference room where 7-8 doctors were talking about my situation and he handed the phone to the chief. He told me if looks could kill, he would have been a dead man.

The endos all came in about 20 minutes later and told me that Dr. Friedman was a very well respected doctor and that he agreed with their treatment plans. (Dr. Friedman also called Jason back, and said he knew the chief of endocrinology personally and that they were doing what he would do.) Unfortunately, my sodium was continuing to drop so I needed to be moved to the Medical ICU and be monitored more closely. I was moved upstairs to a lovely room around 11:00 pm. As soon as I arrived I was told that I needed to have an IV port put in. The best way I can describe it is that they needed an IV that went directly to my heart because the saline they were going to try to put in my body would burn the rest of my veins.

At that point my Mom called Jason and he came over to the hospital to be with me. I was told by the doctor that this would be really uncomfortable, but that it had to be done. ( I have now learned this is code for "this will hurt like crazy".) Jason had to go into the waiting room and was told that it was about a 40 minute procedure. I was laid on the operating table and they tried to get the port put into my right jugular. They would numb the area with Novocaine and were using an ultrasound to make sure they were in the right place. I had a sheet put over my head to prevent infection and I held on to one of the nurses hands as hard as I could. After about an hour of being poked and prodded I had the most horrific pain. Apparently part of my carotid artery was punctured and the nerve shot pain down my neck all the way into my arm. I laid there as tears just rolled down my face. They immediately stopped and put as much pressure on my throat as possible.

No one really moved or said much for about 10 minutes. Then one technician asked if I was crying because I was scared or in pain. He told me, "I told you this would be uncomfortable." I looked at him and said, "I have give birth naturally 2 different times, that was a picnic compared to this." I did get him to smile.

After that small disaster, they decided to try to thread that catheter up from my groin. Not much more fun, but bearable. Finally, Jason was able to come back into the room. (Poor guy didn't know why a 45 min. procedure had taken 2 and half hours.) He told me I looked like I had been bitten by a vampire. I was immediately given some form of saline directly into the port in my groin. I was told that it had to be done a very little bit at a time to prevent seizures. By now it was almost 4 in the morning and I did sleep a very little bit.

The head of the ICU came in around 7:00 AM and told me that my sodium had been down to 111 last night. ( I was later told that the reason I wasn't put out for the port insertion was that with sodium that low, I wouldn't have made it through the surgery.) By that morning they had raised my sodium to 113 (slow and steady just like they wanted). Even with that little bit of sodium raised I felt better than I had in days. My headaches were almost gone and I even ate a little bit.
The worst part was that I had to stay on my back to keep the catheter from rupturing any internal organs.

I stayed in the ICU until my sodium levels were over 130 (6 days) and then was returned to the main floor for another 2 days. I had wonderful nurses in the ICU. They were so attentive and caring. One angelic nurse named Heather came on at midnight and noticed that I couldn't sleep and she asked if she could give me a little spa treatment. She bathed me, rubbed lotion all over, and bless her heart...she even shaved my legs! She stayed with me talking until I was able to go to sleep. Truly a tender mercy!

Now for those of you who are as depressed as I was at this point I want to point out my truly great blessings!!!

The evening I had the port put in, I truly was feeling so alone and so abandoned. I remember thinking, I have people praying for me, fasting for me, wishing me well all over the world and there isn't any point. I am having horrid complications and more pain that I had ever had. I must say I was feeling bitter for a couple of days. However, on the day I was released from the ICU my nurse was telling me what a happy day it was. I agreed, but asked him why. He said, "It isn't often people leave this floor without some aspect of their lives being totally altered forever. You are going to be able to resume your life as you knew it."

I joked with him about needing my room for the "sick" people and he touched my face and said, "Honey, you were as sick as they come." He told me that there had been 5-6 doctors constantly talking about my case. They had never seen someone with sodium as low as mine who didn't end up with permanent brain damage. They couldn't understand why I didn't have seizures, and they couldn't explain it when they spoke to colleagues. I was sent down to a room on the main floor and had a lot of family call me. Kari told me she had just heard that a congregation in Lesotho (where she travels often for work) had been praying for me that week. Lacey told me she had put me on a prayer roll at her church. My lovely Uncle Dan who has so many of his own problems, had put my name on the rolls at the temple. A congregation of Jehovah's Witnesses that are patients of Jason's had a prayer meeting for me. Various cousins and in-laws told me they had been fasting a praying for me.

I cried in thankfulness and humility for most of the day.
Here I was feeling abandoned and alone and I truly had a miracle worked for me. The doctors just think that I am a medical marvel, but I know better. I know that our Heavenly Father loves us individually and that even though things are often very hard our prayers are answered. He hears our prayers whether offered in whatever faith or language they are given and I grateful for those of you who have and continue to pray for me and my family.

The Ordeal -Part 2

As I prepared for surgery, I looked at a a number of different surgeons with my primary goal being cure rates. Cyclical Cushings has a lower cure rate than florid Cushings and depending on the literature the cure rate of a first surgery ranges from 40%-70%. There are a number of studies published that prove that cure rates go up dramatically with an experienced (over 1000) pituitary surgeon. Those statistics really only leave 3-4 surgeons in the US. I decided on Dr. Ian McCutcheon who does surgery at MD Anderson Cancer Center in Houston. He does a Cushing's adenoma every week or so (actually a very large amount considering the rarity of the disease) and is the unequivocal expert on the pituitary gland. One of the things that really endeared him to me is that he reads MRI films for free in his spare time for people trying to be diagnosed.
Jason and I flew to Houston Monday Feb. 23rd and left the kids in the superb care of my mom and my Aunt Kristie.

I had my appointment with Dr. McCutcheon at 9:30 the next morning and was more impressed than I can say with him. He spent about 45 minutes with me and Jason going over hundreds of views of my latest MRI and explaining why he saw a tumor or suspicious areas of the pituitary even though some read the film as normal. His knowledge was truly staggering, but he never spoke down to us or made us feel as though we weren't part of the process.

He estimated the tumor to be around 5 mm but stressed that until he actually got inside the pit, he wouldn't know. He explained that the risks and how he planned to minimize them. For example, he said there was a risk of damage to the optic nerve, but in all his many surgeries he had yet to blind anyone! He said that there was a risk of a cerebral spinal fluid leak because of the proximity of the dura (this is similar to the plastic bag that surrounds your brain with spinal fluid and keeps it hydrated and less weighty.) If that happened he would plug the leak with a piece of abdominal fat and I would have a lumbar drain in my spine coming out of surgery.
He would approach the pituitary through my nostrils and carve out little bits of my sinuses to make room from his instruments. The surgery itself would take anywhere from 3-4 hours and I could expect to feel tired and have a bit of a headache for a couple of days. I asked him about seeing an in-house endocrinologist and he gave me a little smile and said, "We don't do that anymore." When I asked him why, he said that too often the local endos would try to convince the patient scheduled for surgery the next day that they needed more testing. He said by not seeing them "we minimize unpleasant feelings." (Very diplomatic!)

After seeing Dr. McCutcheon we were sent to billing, the lab, and anesthesiology. I will say that MD Anderson is a truly staggering hospital. They have more than 20,000 employees and it is run with an efficiency that is remarkable. I was in and out of the lab in less than 10 minutes despite the waiting room being full. Twenty minutes later in anesthesiology my labs were already available on the anesthesiologist's computer! We were out of the hospital with a surgery call time of 6:00 AM by 1:30 in the afternoon.

We went back to our hotel and took a nap, ate at Fuddruckers and headed for the mall. I made Jason make friends with a salesperson at Sephora and made him buy some new cologne. (He never likes what I pick out for him.) We then went back to the hotel and slept.
I was up and not overly anxious by 5:00 and we arrived in plenty of time. I was ushered to a surgical waiting room of sorts where I had to gown up and wait. Jason took an ugly picture, and we joked around. A little before 6:00 my team came, I kissed Jason, and they drove my gurney to the operating room... from there everything is a little bit fuzzy except for the pain....that I remember in great detail!

I remember hearing that my tumor was actually larger than expected (10 mm which is a larger microadenoma) and I had experienced a CSF leak during surgery. They had patched it up and inserted a drain in my spine to drain off approximately 10 ml every hour so that the pressure wouldn't be too great on the patch. Dr. McCutcheon was confident he removed all of the tumor. He said that as soon as he made an incision the tumor actually started squirting like toothpaste. Gross! The tumor wrapped around the pit and actually was close to causing some visual disturbances due to its location near the optic nerve.

Jason stayed in Houston until Thursday night, but to be honest I don't remember much about it. My fabulous sister Kari came on Thursday and spent what she has called "the worst week of her life" taking care of me. For some reason, we had a really hard time getting rid of pain. The tried morphine, dilaudid, codeine, and I can't remember much beyond shaking in pain.

One of the most comforting things was a cool wet washcloth that Kari kept putting on my head. I was really out of it most of the time...couldn't watch TV, read, knit, do any of the things I had prepared for. Due to the drain in my back I had to remain at 30 degrees in my bed for 5 days straight. I couldn't eat a thing and I had these yucky balloons in my nose to keep pressure on the surgical site.

Poor Kari couldn't do anything either. She finally got them to hook up a morphine drip that I could push every 15 minutes and that helped a bit. However, I guess I overdid it a bit and I stopped breathing. I remember a doctor coming in and screaming at the nurses that he didn't like to be called in because his patients weren't breathing and unresponsive. He asked where my little breathing toy was (can't remember the name) and when I didn't know how to use it, he threw it at the nurse. Don't know who he was, but kind of a jerk. Of course, I was then terrified I would die in my sleep and I begged Kari to sit and watch me breathe all night. Bless her heart, she did!!!!

The next day we found out that "someone" had been weaning me off cortef (a synthetic cortisol that your body needs to stay alive after this surgery) from 100 mg the day of the surgery to 10 mg by the 4th and 5th day. This was a potentially fatal slip. I wonder if a lot of the pain I felt was due to my body screaming for cortisol or not. I kept telling Kari in my delerium that I felt weird and I needed to be having 30 mg of cortef a day. I kept chanting it like a mantra. Kari asked every nurse, doctor, aide, and cleaning person who came in how much cortef I had had that day and where it showed in the computer. No one would give her a straight answer so she called Jason who called Dr. McCutcheon and contacted some dear people on my support boards to get a hold of Dr. Freidman. Dr. Freidman spoke to the on staff endo who finally admitted to him and my sister that "someone" had changed my chart and I was going down to a complete wean. I got some more cortef at that point.

My drain came out on day 5 and my headaches eased a bit. I stayed 3 more days in the hospital and then Kari got me on a direct flight to New York. I don't remember a bit of it, but she said I talked to her and carried on a conversation! Jason picked us up at the airport and Kari headed home to Virginia.

The kids were so sweet when I came home. Carter had decorated the entire bedroom with streamers and balloons and flowers. I got lots of love and got into bed ready to recover and put the ordeal behind me. Would have been nice.
Check back soon for the continuing saga.

The Ordeal

I have been putting off writing this post because I feel like anytime I write about being better I jinx myself, but I figure in this digital age, I can always revise.
As most of you knowI was finally given the official diagnosis of Cushing's Disease in December of 2008 and after much back and forth and waiting on labs and MRIs was scheduled for surgery of Feb. 25th of this year.

What is Cushing's Disease?
Cushing's Disease is a disease of the endocrine system and it is not widely understood or diagnosed. It is estimated that 2-3 people per million are diagnosed every year. Due to its unique nature it is hard to be diagnosed and hard to maintain a cure.

For one reason or another my pituitary gland decided it would like to have a tumor take over its function. This very small tumor (10 mm) would constantly tell my adrenal glands to make a hormone called cortisol. Cortisol is good in moderated amounts. It is the hormone that stimulates the fight or flight response. It allows us to deal with emotional and physical stress. Too much of it causes problems that really suck!

Symptoms vary, but most people have upper body obesity (central obesity), rounded face ("moon face"), increased fat around the neck and on the back of the neck (buffalo hump), and thinning arms and legs.

Other symptoms appear in the skin, which becomes fragile and thin. It bruises easily and heals poorly. Purplish pink stretch marks (straie) may appear on the abdomen, thighs, buttocks, arms and breasts. Boils often occur on the stomach and thighs. The bones are weakened, and routine activities such as bending, lifting or rising from a chair may lead to backaches, rib and spinal column fractures.

Most people have severe fatigue, weak muscles, persistent hypertension (due to the aldosterone-like effects) and insulin resistance, leading to hyperglycemia (high blood sugars) which can lead to diabetes mellitus. Patients frequently suffer various psychological disturbances, ranging from euphoria to frank psychosis. Depression and anxiety, including panic attacks, are common.

Women usually have excess hair growth (hirsutism) on their faces, necks, chests, abdomens, and thighs. Their menstrual periods may become irregular or stop (amenorrhoea). Men have decreased fertility with diminished or absent desire for sex.

Other symptoms include excess sweating, telangiectasia (dilation of capillaries, spider veins), atrophy of the skin (which gets thin and bruises easily) and other mucous membranes, proximal muscle weakness (hips, shoulders).

Untreated Cushing's syndrome leads to heart disease and increased mortality.

Not a fun list, huh?

Getting a diagnosis

You would think that with all that litany of problems doctors would be all over me ready to treat me. Well as I have heard again and again, "Cushing's is too rare, you don't have it!" My first inclination that something was wrong was in 1997. I gained 60 pounds in 3 months without changing my diet, excercise or anything. I got labeled as a closet eater, was told I was getting older and my metabolism was slowing down, was told I had chronic fatigue syndrome, I was depressed, I went to sleep too late at night. I knew something was wrong because the weight kept coming on, and I was so tired I would pull over while driving and wake up 2 hours later.

Jason was in his second year of chiropractic school and came home one night and said, "I know what you have!" They

had been studying the endocrine system and he saw a picture of stretch marks known as "striae". He also saw a picture of a cushing's patient with a "moon face." Aren't they lovely?

Once I had a name, I became a crazy researching woman. I read every article I could find in every journal and online. I joined a support group for cushing sufferers and asked question after question.
I soon found out that although I was convinced about the diagnosis, most of the doctors I saw were unconvinced.
I made the rounds in Connecticut for about 3 years seeing various endocrinolgists. Most would run one test or the better ones 2 or 3 and if any came back normal, I was dismissed as an attention seeking overeater. I was getting more and more frustrated because the weight kept coming on, I was having horrible panic attacks in the middle of the night. My muscles were so weak I could carry my babies for more than a minute at a time. (The weird thing was that during my pregnancies my symptoms would go away. I would lose weight, my face would get slimmer, my stretch marks would fade. But as soon as I had the baby, everything would come back.)

After doing some more research and with some encouragement from my support group (and some help from Bob and Barbara.) I went to LA in May of 2005. Kari flew out with me and I went to see the world's leading researcher on Cushing's disease. Dr. Friedman is primarily a researcher, but he does have one evening a week he sees patients. He met me in his very crowded office and immediatly asked me to do a squat. I tried and fell on the floor. He told me, "If more doctors diagnosed by believing what they could see with their eyes, I would have much less work." (He told me proximal muslce wasting is a hallmark sign of Cushings.) After looking at my labs, he told me we had a lot more testing to go.

He believed that I had a type of Cushing's called Cyclical Cushings which was unfortunately even harder to diagnose. The reason being, that unlike some cases of Cushing's, my tumor would turn on and off and my job was to learn to recognize a cortisol high and low and only test during the highs. I had to chart things like blood pressure which tend to go up during a high, but the confusing thing is that some people have a lot of energy during highs, others don't. Some people are depressed during highs, other's aren't. I began a symptom journal and looked for patterns based on my symptoms and the results I was getting.

I was given open orders for 24 hour urine tests ( a rather annoying test where you have to collect all your urine for 24 hours and keep it in a jug in the fridge.) Midnight blood draws, (you have to drive to the emergency room at midnight to have your blood drawn. Salivary cortisols (you have to spit into a little vial at midnight for a number of days, by far the easiest, but I never got any good results from them!)
I was also given an MRI and it showed a 2-3 mm tumor on my pituitary. Not enough to make a case for surgery, but a step in the right direction.

I tested at home for a few months with moderate success, but because we are talking about brain surgery, Dr. F requires a lot of chemical evidence. Jason and I decided we wanted to have another baby and Dr. Friedman said there was no reason not to try. I stopped with the testing and got pregnant right away with Keaton, waited until I was finished breastfeeding and began testing again like crazy.
It took 5 months of testing, but I finally figured out my cycles and got some cortisol numbers 15x what a normal person should have. I was finally diagnosed.

For those of you interested in the continuing saga, check back soon for more exciting chapters!