“YOU! DO! NOT! HAVE! CUSHING’S!” My endocrinologist yelled after I requested more testing despite three negative 24 Hour Urine Free Cortisol tests and a below normal Low Dose Dexamethasone test. “YOU! CAN’T! PROVE! THAT!” I yelled back while my mind desperately reeled for more arguments, more statistics, more anything to get another test for Cushing’s. I stuttered as I rapidly fired off findings by Cushing’s researchers who had reported patients with Episodic or Cyclic Cushing’s. “As long as the entity of Cyclic Cushing’s exists, and I continue to have Cushing’s symptoms, you HAVE to keep testing!” I ended. During my tirade he had turned around and leaned against the counter in the exam room, head down with hands on either side of my open file. He stood silently for so long I flinched slightly when he took a breath, turned around, looked me straight in the eye and said quietly, “Do you know how rare that is?” My eyes watered and I looked away. “That still doesn’t mean I don’t have it.”
Rewind about six weeks to my first visit, same doctor, same exam room. As the endocrinologist sat down I started relaying my list of symptoms (at this point the onset had been about 4 and 1/2 years), current and past medications, family history, surgeries and hospitalizations, all known by heart from the many, many other times I had been in a new doctor’s office. Before I was even halfway through my recital, he held his hand up to stop me and asked, “Have you ever heard of Cushing’s Disease? I haven’t run any tests on you yet, but you have it.” He continued to explain what Cushing’s Disease was — ACTH, pituitary, adrenal, but all I heard was the word “TUMOR”. No. Absolutely not. I do not have this. I cannot have this. I came here because my sister and mother were both just diagnosed with Hypothyroidism. That’s what I have, no way did I have a damn tumor! This guy is a quack. I’ll make a follow-up appointment to be nice and cancel it by telephone tomorrow.
But as he began asking questions, it was as if he was telling me my own story: “Do you have any purple markings? Have you noticed your arms or legs becoming weak? Has your face changed shape along the jaw line?” The list goes on. I did indeed have all the symptoms he asked about, but never thought them relevant enough to report to a doctor, nor did any doctor ever bother to ask me about them. Our eyes met as the feeling in the room shifted from the boring, routine office visit to an eerie, yet exhilarating event. I was terrified, but fascinated. Could there really be one cause to all my symptoms? If so, could I be fixed? And most importantly, I pushed through the shock to ask, “Is this…um…fatal?” “Yes,” he said and smiled, “But it’s completely curable.”
The next year and a half was filled with 24 hour Urine Free Cortisol collections, Midnight Salivary Cortisols, MRI’s, CT’s, etc. I became increasingly debilitated, making multiple trips to the Emergency Room with kidney stones and being hospitalized with what should have been minor infections. A doctor in the hospital declared me “the sickest 26 year old he’s ever seen!” Yet no Endocrinologist, Cushing’s Specialist or Neurosurgeon would agree to treat me because my results were all over the board. Some tests came back definitively negative for Cushing’s, some tests came back toward the high normal or slightly above normal limits for Cushing’s, and some came back soaringly high positive for Cushing’s. I had a mathematician run statistics on my test results using conservative estimates of sensitivity and specificity. The result? I could prove with over 99% accuracy that I both did and did not have Cushing’s Disease. Great. I had the dreaded Cyclic Cushing’s Disease. Now all I needed was a doctor who would treat it.
After multiple arguments with my local Endocrinologist for a referral, I finally ended up in the office of a Neuroendocrinologist at Mt. Sinai School of Medicine. She ran her own tests with the same results — negative, borderline, positive. I even ended up with a negative 24 hour Urine Free Cortisol and a positive Midnight Salivary Cortisol on the same day! Apparently, I had a tumor that produced cortisol at night, but not enough cortisol to show up consistently on 24 hour Urine Free Cortisol tests. However, the Neuroendocrinologist and Neurosurgeon were still hesitant to treat my condition.
I pulled every stunt I could possibly think of to convince those doctors to treat me. I argued, I threw journal articles at them, I gave them statistics, I did and said outrageous things to make them laugh, I offered to sign away all my rights to the point that they could walk into the operating room roaring drunk and I couldn’t sue them. I so doggedly pursued them that they probably could have had me put away for harassment. I knew I was pushing it — with every move a voice in my head screamed “What are you doing, crazy woman?!?!”, but something in my gut would not allow me to stop. It was as though my brain was put on autopilot while some kind of strange intuition in my gut took over — it told me I was dying quickly and I needed to do whatever it took to get treatment as soon as possible. Nothing else mattered. Nothing.
About a year and a half after first hearing the word “Cushing’s” I walked into the Neuroendocrinologist’s office again carrying test results with the same pattern as always — one negative, one borderline, one positive. I felt like crap and didn’t have the energy to fight that day. She made some notes about my blood pressure being higher than the last time she saw me, my hair loss, and the few pounds I had gained. She started the same speech about her hesitancy to treat me because I had such inconsistent results. To this, I sat back up from where I was laying on the exam table and wearily responded by naming the risks involved in the surgery along with their respective statistical likelihood of occurrence, and said that I understood I could die and/or be disabled by this, and that it was my choice to take this risk.
I saw her face change as she reconsidered. “What would you do if we go ahead with surgery and you remain sick?” she asked. “I would be disappointed, but at least then we’ll know we need to look somewhere else for a cause,” I replied, “But we can’t do nothing while I sit here and waste away! I’m 26 years old and almost completely disabled. No one can say why, but I have a good hypothesis and if it’s wrong we have to know so we can start looking for the real problem!”
Exactly one month later my parents and I entered the pre-surgical intake lobby at Mt. Sinai Hospital. After the usual paperwork and insurance business, a nurse took me back and I changed into a hospital gown and put the hospital socks on and got set in a hospital bed. This was the real deal. No going back now. I was terrified, but hopeful they would find the tumor that had, up to this day, been elusive. The next thing I remember is hearing the neurosurgeon’s excited voice saying “Are you awake? We opened you up and the tumor was right there! We got every bit of it! We got it!” If I didn’t hurt so badly, I would have cried for relief. Instead, I tried to convince them to increase the painkillers because I was absolutely overwhelmed with pain, but I couldn’t talk so it just ended up sounding like E.T. saying “Ooooooouch! Ooooooooouch!”
The next few months are a blur of indescribable pain. I was no stranger to pain before — kidney stones, aura migraines, etc. But there was nothing to prepare me for the pain of cortisol withdrawal and there was nothing to prepare me for just how long the pain would last. I thought about pounding myself on the head and knocking myself out. I thought about begging for some doctor to put me in a coma for a few months. I even thought about asking for the tumor back! Somehow, I managed to get through the first couple months’ worth of pain. Around the third month or so the pain got to a tolerable level. I am only 9 months post-surgery as of this writing, and still experience some pain and other Cushing’s symptoms. However, I am at the point now where I can see the other side — life without Cushing’s — and get excited about it.
Please feel free to contact me with questions or concerns!
Newsletter: Winter, 2010
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