My name is Kathleen and I presently have Cushing’s disease due to an inoperable ACTH secreting pituitary tumor. I was born in 1950 and am married with two great sons and a wonderful one and a half year old grandson. My husband and I have lived in the small, rural town of Mt. Shasta, CA at the foot of the mountain with the same name since 1979.
In 1995, I was working as a school nurse when I began experiencing an intense headache. This was unlike a migraine as it was an extreme burning pain behind my left eye. After about 7 days I couldn’t focus my left eye so I went to a walk-in clinic. Fortunately my family doctor, Dr. Sternberg, was on call. It was a Sunday, and he immediately sent me to the emergency room in Redding, about an hour away, where I was seen by a neurologist. A CAT scan showed something suspicious. I was hospitalized, and an MRI the next day revealed a growth on my pituitary. Amazingly, Dr. Sternberg’s nurse had a history of a pituitary adenoma so he knew to make an immediate referral to Dr. Charles Wilson, an experienced pituitary neurosurgeon at the University of California San Francisco Medical Center (UCSF). After another MRI and complete physical with lab tests, Dr. Wilson performed a transsphenoidal resection and I was discharged two days later. I had a macroadenoma that at the time was not secreting excessive ACTH. Dr. Wilson was unable to remove all of the tumor, so I had a series of 23 radiation treatments. Afterwards, I was followed by my family physician, Dr. Sternberg, and an endocrinologist, until the endocrinologist retired. Since I was doing so well, I decided not to go to another endocrinologist and my family physician was willing to order the 24 hour urines and other lab work.
After about 6 months, I recovered to close to my preoperative health and thought that was the end of the problem. I was fine until November of 2008. Early on I didn’t realize what was happening; I was feeling fatigued, my hip joint ached, I gained some weight from the waist up, started getting the round facial features, and my hair was falling out. One of the school nurses said after I was diagnosed that she thought I looked like I had Cushing’s. Then I began to feel very dizzy and weak and to bruise easily. I also had a metallic taste in my mouth, blurred vision, and trouble concentrating. After seeing marked swelling in my ankles, I saw my family physician, who suggested congestive heart failure. He ordered some blood tests and a 24 hour urine cortisol. When my blood work came back, my potassium was very low at 2. Amazed that I was still walking around, he had me hospitalized for 3 days to receive intravenous (IV) potassium. My 24 hour urine cortisol was very high at 6500.
Dr. Sternberg again referred me to UCSF which is about 300 miles from Mt Shasta and is also where I attended nursing school. Since the first tumor did not cause elevated cortisol levels, the endocrinologist wanted to be sure the source of the ACTH secretion was the pituitary. Dr. Christopher Dowd performed an inferior petrosal sinus sampling (IPSS) which confirmed the ACTH was coming from the pituitary. I was surprised to read in the newsletter that some patients are awake through this procedure and find it uncomfortable. I was put to sleep and woke up pain free so for me it was OK. A CAT scan of my adrenal glands showed them to be hypertrophied.
By the time I had the surgery, I was very sick and weak with hardly any muscle in my arms and legs. In addition to the symptoms mentioned earlier, I felt like there was a constant buzz surrounding me, my blood pressure and blood sugars were elevated, and I had the facial hair. Dr. Sandeep Kunwar performed an endonasal transsphenoidal resection. When I woke up, I could immediately feel that the buzz was gone and my husband noticed that my voice was stronger. Dr. Kunwar was not able to remove all of the tumor as it was very invasive. I was discharged the day after the surgery. Even though the outward signs of the disease were gone and my cortisol was normal within a couple of months, I never returned to how I felt before Cushing’s.
I was the administrator for the school nurses of Siskiyou County. I went back to work about a month after the operation and tried working the following 2009/2010 school year, but I found I did not have the energy, concentration, or ability to handle the stress that goes along with the position. So in June of 2010 at the age of 60, I retired even though I loved my work and my coworkers. I feel very fortunate that I have a decent retirement plan and was able to keep my health insurance. It is quite expensive, but has great coverage.
I was frustrated that following surgery, endocrinologists did not seem to want to follow me long term. I saw a local endocrinologist who only seemed interested in following my hypothyroid condition. So again, I just saw my family physician, Dr. Sternberg, who is a wonderful and caring doctor.
It was a good thing I was able to keep my insurance, because in November of 2013, five years after my last surgery, I again began to exhibit signs of Cushing’s and again, it snuck up on me. Then I fell and hit my head, and the symptoms began to snowball. I had headaches, rounding of the face, a strong metallic taste in my mouth, weight gain from the waist up, joint pain, progressive weakness, that buzzing surrounding me, bruising, blurred vision, and inability to concentrate.
I went to my physician and he ordered lab tests and a 24 hour urine. He called me back when he received the results and said my potassium was 1.9. So he again admitted me to the hospital for IV potassium. This time it took 4 days and 50 bags of potassium to normalize my levels. In the hospital, one is followed by hospitalists. It was then I realized that some doctors are not interested in learning about or treating Cushing’s disease. One doctor said I didn’t need the 24 hour urine, and I had to insist on continuing it while in the hospital. Another doctor looked at me and said in an irritating manner that he didn’t want to hear about “my Cushing’s.” I was trying to explain why my potassium dropped so low. When I was discharged, I was prescribed 20 mEq/day of oral potassium when I really needed 160 mEq/day to maintain a normal level. The urine cortisol “I didn’t need” was 13,000 (normal </=50).
I did some research with the help of a couple of night nurses while I was hospitalized. Several of them didn’t know much about Cushing’s and to their credit, they went online to find out more. One nurse gave me some information on the UCLA Pituitary Tumor Program that includes a very experienced pituitary neurosurgeon , Dr. Marvin Bergsneider, and an endocrinologist, Dr. Anthony Heaney, who is on the CSRF Medical Advisory Board. Even though UCLA is 600 miles from Mt Shasta, my husband and I decided we would try their program. My doctor promptly did a referral, and they accepted me into their program.
I was seen in two weeks. Transferring medical records from the local hospital and UCSF to UCLA was not easy, especially since I was very sick, but everything got there on time. Dr. Heaney did more lab tests; 3-24 hour urines, 3 salivary cortisol tests, an MRI and an octreotide scan. My urinary cortisol was down to 6500. I was still very weak, and the symptoms were severe. My hair was falling out at an alarming rate.
After all the tests were done, my husband and I met with both doctors. Their consensus was that the tumor was inoperable, too small and invasive into critical structures. Radiation was not an option as there was no specific target. The doctors were alarmed by my cortisol level and physical state. They considered a bilateral adrenalectomy, but I was at risk of developing Nelson’s Syndrome. And once the adrenal glands are gone, they’re gone for good.
We then looked at medication options. Since I needed something to bring my cortisol levels down very soon, clinical trials that included time on a placebo rather than the medication being studied were not an option. Also, we were so far away from UCLA that frequent trips would be difficult. One study was evaluating the effectiveness of combining cabergoline and pasireotide (Signifor), a drug that has only been available by prescription for about two years. Both drugs were available on the market, so Dr. Heaney decided to try cabergoline (0.5mg, 3 tabs twice a week) and pasireotide (0.9 mg injection subcutaneously twice a day).
We went home feeling pretty depressed as pasireotide returned cortisol levels to normal in only about 25% of the cases. However, within six weeks, my cortisol level dropped to the lower end of normal, and I was starting to feel better except for the metallic taste and persistent nausea. Also, a side effect of pasireotide is high blood sugar. We tried metformin, but it didn’t really lower my blood sugar, and seemed to make my nausea worse. After gaining about 10 lbs. with the Cushing’s, I lost 20 lbs. due to not being able to eat. Dr. Heaney then tried nateglinide, and the metallic taste and nausea went away; but it also didn’t help lower my blood sugar.
On my next visit, Dr. Heaney was amazed!. He had never seen cortisol levels as high as mine, nor had he ever seen them drop so fast. I was definitely a pasireotide success story. I was much stronger and alert, and my Cushing’s features were disappearing. He reduced the pasireotide to 0.6 mg subcutaneously twice daily, still with the cabergoline. I just had my check-up at the end of January and since my cortisol was still at the low end of normal, Dr. Heaney has again reduced the dose of pasireotide. My husband and I have found that taking the shot at least one hour after eating keeps my hemoglobin A1c at high normal, and I am not taking any diabetic medication. We are hoping that the once a month shot will be available soon. I find the injections painful, but, hey, it’s doing the job.
My road to recovery is slow. Every time I have a bout with the high cortisol levels, it takes a chunk out of me that never seems to return. I live with a nagging fear that my cortisol levels may rise again, and I don’t think I could survive it. My doctor said that the only reason I did this last time was that I was in such good shape to start with. When I have days when I don’t feel well, I wonder… is my cortisol going up, is it too low, a medication side effect, am I being paranoid, or could it be I’m getting old? Many of the symptoms are the same.
I am exercising and building muscle mass and strength. My husband and I can go woodcutting and on hikes. We are enjoying our new grandson, so life is good. I never would have made it through these episodes without the help, love, and support of Kevin. It has been very hard on him, but he is always there for me.
I would love to hear from others.
Newsletter: Spring, 2015