“We invite you to join us for our opening 12 Days of Cortisol Christmas event, “Overcoming Fatophobia” with Dr. Karen Thames.
Dr. Karen, a licensed clinical psychologist and founder of the EPIC Foundation, has battled Cushing’s disease and is now adrenal insufficient as a result. She has faced the battle of weight stigma both professionally and personally. In this interview, she bravely shares her story and advice on how to overcome #fatphobia in #chronicillness.
Adrenal Alternative’s 12 days of Cortisol Christmas is an event where we give back to the adrenal community during the Holiday Season.
Throughout the 12 days, we will be giving away adrenal awareness merch, hosting interviews with patients and professionals, and sharing helpful resources.
Even though patients must fight the war that is #adrenalinsufficiency, we want to let you know that you are supported by an army of adrenal warriors and you are not alone.”
People sometimes ask me how I found out I had Cushing’s Disease. Theoretically, it was easy. In practice, it was very difficult.
In 1983 I came across a little article in the Ladies Home Journal which said: “If you have these symptoms…”
I found the row with my symptoms and the answer read “…ask your doctor about Cushing’s”.
After that article, I started reading everything I could on Cushing’s, I bought books that mentioned Cushing’s. I asked and asked my doctors for many years and all of them said that I couldn’t have it. It was too rare. I was rejected each time.
Due to all my reading at the library, I was sure I had Cushing’s but no one would believe me. My doctors would say that Cushing’s Disease is too rare, that I was making this up and that I couldn’t have it.
In med school, student doctors are told “When you hear hoofbeats, think horses, not zebras“.
According to Wikipedia: “Zebra is a medical slang term for a surprising diagnosis. Although rare diseases are, in general, surprising when they are encountered, other diseases can be surprising in a particular person and time, and so “zebra” is the broader concept.
The term derives from the aphorism “When you hear hoofbeats behind you, don’t expect to see a zebra”, which was coined in a slightly modified form in the late 1940s by Dr. Theodore Woodward, a former professor at the University of Maryland School of Medicine in Baltimore. Since horses are the most commonly encountered hoofed animal and zebras are very rare, logically you could confidently guess that the animal making the hoofbeats is probably a horse. By 1960, the aphorism was widely known in medical circles.”
So, doctors typically go for the easily diagnosed, common diseases. Just because something is rare doesn’t mean that no one gets it. We shouldn’t be dismissed because we’re too hard to diagnose.
When I was finally diagnosed in 1987, 4 years later, it was only because I started bleeding under the skin. My husband made circles around the outside perimeter each hour with a marker so my leg looked like a cut log with rings.
When I went to my Internist the next day he was shocked at the size of the rings. He now thought I had a blood disorder so he sent me to a Hematologist/Oncologist.
Fortunately, that new doctor ran a twenty-four-hour urine test and really looked at me and listened to me. Both he and his partner recognized that I had Cushing’s but, of course, couldn’t do anything further with me. They packed me off to an endo where the process started again.
My final diagnosis was in October 1987. Quite a long time to simply “…ask your doctor about Cushing’s”.
Looking back, I can see Cushing’s symptoms much earlier than 1983. But, that ‘s for a different post.
Myth: “All Cushing’s patients have the exact same symptoms and the level of illness is the same for everyone. If you do not have ALL of the classic symptoms of Cushing’s, then you must NOT have Cushing’s Syndrome/Disease!”
Fact: Everyone does NOT have the exact same symptoms. Not all Cushing’s patients are exactly the same. This is one mistake that non experts tend to make in terms of categorizing patients by whether they meet the exact same classic symptoms or not. Experts have come to learn that each patient should be treated individually. Though there are symptoms that are more prominent in the Cushing’s population, not every patient has to meet every single symptom in order to meet criteria for Cushing’s.
For instance, not all Cushing’s patients become overweight. Everyone does not gain the same amount of weight. There are various theories as to why. One issue is that different patients are diagnosed at different stages of the illness. We know that patients tend not to be diagnosed at the onset of the illness because of doctors’ misconception that Cushing’s patients must be extremely obese to have the disease. So, patients who have not gained as much weight may not be listened to until after the weight has gotten out of control. However, there ARE patients who are diagnosed early enough where there has not been a tremendous amount of weight gain.
I (Karen Ternier Thames), for one, started trying to get help after gaining my first 30 pounds because I KNEW that something was wrong with my body. Had I received an appropriate diagnosis, I probably would not have gained the 150 pounds I ended up gaining in 5 years.
Regardless of the reason, it is a myth that all Cushing’s patients gain the same amount of weight. The following are other additional reasons that an endocrinologist gave me for supposedly not meeting the criteria for Cushing’s when I was misdiagnosed: “1. Your stretch marks are not purple enough”, 2. “Your buffalo hump is not large enough”, 3. “You are not THAT fat!”, 4. “Cushing’s patients do NOT have children”, and 5. ” your face does not look like a classic moon face”. These are some of the reasons why, 2 years earlier, this same doctor dismissed apparently high cortisol levels, and didn’t even tell me, leading to several more years of suffering!
So, not all Cushing’s patients are obese, not all Cushing’s patients gain the same weight at the same rate, not all Cushing’s patients have the same size buffalo hump or the same round moon face. There are variations in these symptoms. IF you are experiencing extreme changes in your body regardless of diet and exercise and its not influenced by external factors, then it is time to speak up!
It is important to raise concern with your doctor if you do have ANY Cushing’s symptoms. Please do not be inhibited if you do not show every single symptom!
I wrote parts of this in 2008, so all the “yesterdays” and “last weeks” are a little off.
Wow. That’s about all I can say. Yesterday was possibly the best day of my life since I started getting Cushing’s symptoms, and that was over 30 years ago. More than a quarter of a century of feeling exhausted, fatigued. A quarter of my life spent taking naps and sleeping.
I went to the endo yesterday. Nothing has changed for me. Nothing will. He wants me to take more cortef. I don’t want to gain weight again. He looked up Provigil and it’s not indicated for panhypopituitarism. So he won’t prescribe it. My kidney surgeon probably won’t let me take, anyway, but it was worth a try.
…
He did mention that in “only” 2.5 years maybe I can go back on growth hormone. I don’t want to live like this another year let alone 2.5. But then, when I was on GH before it didn’t help me like it helps most everyone else.
I’m tired of catering to a kidney that may or may not fail sometime anyway, tired of being so exhausted all the time. I feel like I’ve lost nearly half my life to this Cushing’s stuff already.
So, yesterday I was supposed to go to a conference on web design for churches. My church sent me because they want me to spiff up their site and make them a new one for Christmas. I wanted to go because, well, I like learning new stuff about the web. I figured that I would learn stuff that would also be useful to me in others of my sites.
And I did!
But the amazing thing is this. My son had told me about a medication that was very similar to Provigil, that he had tried it while he was writing his doctoral thesis and it had helped him.
So, having tried the official doctor route and being rebuffed – again – I had decided to try this stuff on my own.
I was just talking to a friend today about how I’d try nearly anything – even if it ruined my one remaining kidney – to have a few days where I felt good, normal, where I could wake up in the morning rested and be able to have energy for the day.
I want to go out and have fun, to be able to drive for more than 45 minutes without needing to rest, to be have people over for dinner, whatever. I hate being restricted by my lack of energy.
My endo says to cheer up. In two and a half years I can try the growth hormone again. Whoopee. Didn’t work the first time and maybe gave me, or contributed to, cancer growth. Why would I want to look forward to trying that again?
I want to feel good now. Today.
I hate that this disease kills but I also hate that it’s robbed me of half my life already.
I wish doctors would understand that even though we’ve “survived”, there’s no quality of life there.
I hate Cushing’s. It robs so much from so many of us. 🙁
As I said earlier, I have a history of daily naps of at least 3 hours a day. It cuts into everything and prevents me from doing many things. I have to schedule my life around these naps and it’s awful.
A few years ago I went on a Cushie trip to Rockford. I’ve been there a few times and it’s always so much fun. But this first year, we were going to another Cushie’s home for barbecue. I didn’t drive, I rested in the back of the car during the drive. We got there and I managed to stay awake for a little while. Them I put my head down on the dining room table and fell asleep. Our hostess kindly suggested that I move over to the sofa.
So, I have a long history of daily naps, not getting through the day, yadda, yadda.
So, I was a little nervous about yesterday. I really wanted to go to this conference, and was afraid I’d have to go nap in my car.
I got up at 5:30 am yesterday. Before I left at 7:15, I took my Cortef and then I took my non-FDA approved simulated Provigil. (Although it’s not FDA approved, it is not illegal to possess without a prescription and can be imported privately by citizens)
I stayed awake for the whole conference, went to a bell rehearsal, did Stacey’s interview, had dinner and went to bed about 10:30PM. NO NAP! I did close my eyes a little during the 4:00PM session but it was also b-o-r-i-n-g.
I stayed awake, I enjoyed myself, I learned stuff, I participated in conversations (completely unlike shy me!).
I felt like I think normal people feel. I was amazed. Half my life wasted and I finally (thank you Michael!) had a good day.
My kidney doctor and my endo would probably be appalled but it’s about time that I had some life again! Maybe in another 25 years, I’ll take another pill. LOL
Well, the energy from the Adrafinil was a one day thing. I felt great on Thursday. Friday and Saturday I slept more than usual. Saturday, today, was one of those days where I sleep nearly all day. Maybe if I took the drug more it would build up in my system, maybe not. But it was still worth having that one day where I felt what I imagine normal to be.
While I was being a slug today, my husband painted the entire house.
I’m not sure if I would have been this tired today or if I was somehow making up for the nap I didn’t get on Thursday. Whatever the case, I’m glad that I had the opportunity to try this and to experience the wonderful effects, if only for one day.
Information from a site that sells this:
Alertness Without Stimulation
Adrafinil is the prototype of a new class of smart drug – the eugeroics (ie, “good arousal”) designed to promote vigilance and alertness. Developed by the French pharmaceutical company Lafon Laboratories, adrafinil (brand name, Olmifon) has been approved in many European countries for treating narcolepsy, a condition characterized by excessive daytime sleepiness and other unusual symptoms.
Non-narcoleptic users generally find that adrafinil gives them increased energy and reduces fatigue, while improving cognitive function, mental focus, concentration, and memory. It has been reported that quiet people who take adrafinil become more talkative, reserved people become more open, and passive people become more active.
Of course, many stimulant drugs, ranging from caffeine to methamphetamine, are known to produce similar alerting/energizing effects. Adrafinil has been described by some users as a “kinder, gentler” stimulant, because it provides these benefits but usually with much less of the anxiety, agitation, insomnia, associated with conventional stimulants.
Adrafinil’s effects are more subtle than those of the stimulants you may be used to, building over a period of days to months. They appear to be based on its ability to selectively stimulate 1-adrenergic receptors in the brain.2 These receptors normally respond to norepinephrine (noradrenaline), a neurotransmitter linked to alertness, learning, and memory. This is in contrast to conventional stimulants, which stimulate a broader spectrum of brain receptors, including those involving dopamine. Its more focused activity profile may account for adrafinil’s relative lack of adverse side effects.
It’s interesting that that snipped report that people become more talkative. I reported that in the original post, too, even though I didn’t realize that this was a possibility.
A good quote that I wish I could relate to better:
“Time is limited, so I better wake up every morning fresh and know that I have just one chance to live this particular day right, and to string my days together into a life of action and purpose.”
Lance Armstrong (1971 – )
Cyclist, seven-time Tour de France champion and cancer survivor
2011 stuff starts here:
Awhile ago I went to a handbell festival. I took a bit of adrafinil on the main day to try to stay awake for the whole day. It didn’t seem to keep me as on as it did before. I can’t be used to it already. Maybe I’m just that much more tired than I was before.
Our son lives in New York and every few years he gives us tickets to see a Broadway show. A couple years ago we took the train to NY to see Wicked. Usually my DH wants to go out and see sights while we’re there. I usually want to nap.
This time we got up on Saturday morning, went out for breakfast. I wanted to take in the whole day and enjoy Wicked so I took some Adrafinil. We got back to the hotel and got ready to go to a museum or other point of interest.
But, DH wanted to rest a bit first. Then our son closed his eyes for a bit…
So, I found myself the only one awake for the afternoon. They both work up in time for the show…
Sigh It was a great show, though.
A recent Christmas I was going to get my son some Adrafinil as a gift. The original place we bought it didn’t have any more stock so I tracked it down as a surprise. He was going to give me some, as well, but couldn’t get it from the original source, either. So he found something very similar called Modafinil. GMTA!
And 2016..
Saturday, 4/23/16 really was one of the best days I’ve had in a long time.
Cushing’s Conventions have always been special times for me – we learn a lot, get to meet other Cushies, even get referrals to endos!
As early as 2001 (or before) my pituitary function was dropping. My former endo tested annually but did nothing to help me with the symptoms.
In the fall of 2002 my endo refused to discuss my fatigue or anything at all with me until I lost 10 pounds. He said I wasn’t worth treating in my overweight condition and that I was setting myself up for a heart attack. He gave me 3 months to lose this weight. Those 3 months included Thanksgiving, Christmas and New Years. Needless to say, I left his office in tears, again.
Fast forward 2 years to 2004. I had tried for a while to get my records from this endo. He wouldn’t send them, even at doctors’ or my requests.
I wanted to go see Dr. Vance at UVa but I had no records so she wouldn’t see me until I could get them.
Finally, my husband went to the former endo’s office and threatened him with a court order. The office manager managed to come up with about 13 pages of records. For going to him from 1986 to 2001 including weeks and weeks at NIH and pituitary surgery, that didn’t seem like enough records to me.
In April of 2004, many of us from the message boards went to the UVa Pituitary Days Convention. That’s where the picture above comes in. Other pictures from that convention are here.
By chance, we met a wonderful woman named Barbara Craven. She sat at our table for lunch on the last day and, after we learned that she was a dietitian who had had Cushing’s, one of us jokingly asked her if she’d do a guest chat for us. I didn’t follow through on this until she emailed me later. In the email, she asked how I was doing. Usually I say “fine” or “ok” but for some reason, I told her exactly how awful I was feeling.
Barbara emailed me back and said I should see a doctor at Johns Hopkins. I said I didn’t think I could get a recommendation to there, so SHE referred me. The doctor got right back to me, set up an appointment. Between his vacation and mine, that first appointment turned out to be Tuesday, Sept 14, 2004.
Just getting through the maze at Johns Hopkins was amazing. They have the whole system down to a science, moving from one place to another to sign in, then go here, then window 6, then… But it was very efficient.
My new doctor was wonderful. Understanding, knowledgeable. He never once said that I was “too fat” or “depressed” or that all this was my own fault. I feel so validated, finally.
He looked through my records, especially at my 2 previous Insulin Tolerance Tests (ITT). From those, he determined that my growth hormone has been low since at least August 2001 and I’ve been adrenal insufficient since at least Fall, 1999 – possibly as much as 17 years! I was amazed to hear all this, and astounded that my former endo not only didn’t tell me any of this, he did nothing. He had known both of these things – they were in the past records that I took with me. Perhaps that was why he had been so reluctant to share copies of those records. He had given me Cortef in the fall of 1999 to take just in case I had “stress” and that was it.
The new endo took a lot of blood (no urine!) for cortisol and thyroid stuff. I went back on Sept. 28, 2004 for arginine, cortrosyn and IGF testing.
He said that I would end up on daily cortisone – a “sprinkling” – and some form of GH, based on the testing the 28th.
For those who are interested, my new endo is Roberto Salvatori, M.D.
Assistant Professor of Medicine at Johns Hopkins
Medical School: Catholic University School of Medicine, Rome, Italy
Residency: Montefiore Medical Center
Fellowship: Cornell University, Johns Hopkins University
Board Certification: Endocrinology and Metabolism, Internal Medicine
Research Interests: Control of growth hormone secretion, genetic causes of growth hormone deficiency, consequences of growth hormone deficiency.
Although I have this wonderful doctor, a specialist in growth hormone deficiency at Johns Hopkins, in November, 2004, my insurance company saw fit to over-ride his opinions and his test results based on my past pharmaceutical history! Hello??? How could I have a history of taking GH when I’ve never taken it before?
Of course, I found out late on a Friday afternoon. By then it was too late to call my case worker at the drug company, so we had to appeal on Monday. My local insurance person also worked on an appeal, but the whole thing was just another long ordeal of finding paperwork, calling people, FedExing stuff, too much work when I just wanted to start feeling better by Thanksgiving.
As it turned out the insurance company rejected the brand of hGH that was prescribed for me. They gave me the ok for a growth hormone was just FDA-approved for adults on 11/4/04. The day this medication was approved for adults was the day after my insurance said that’s what is preferred for me. In the past, this form of hGH was only approved for children with height issues. Was I going to be a guinea pig again?
The new GH company assigned a rep for me, submitted info to pharmacy, and waited for insurance approval, again.
I finally started the Growth Hormone December 7, 2004.
Myth: Cushing’s Syndrome/Disease can be healed or cured through change in diet or exercise.
Fact: NO! Caloric intake or lack of exercise has NO impact on weight gain and/ or loss in persons with Cushing’s.
Saying that someone “cheated” on their diet may seem reasonable to some as a reason for weight gain but I assure you that a candy bar or a piece of pie does not make a person with Cushing’s gain weight or get sick. Excess cortisol is the reason for Cushing’s symptoms. Treating the disease is the only way to alleviate symptoms.
The first line of treatment with the highest rate of remission is currently surgery to remove the tumor (s) from the pituitary, adrenal gland, or ectopic source.
This is kind of a “cheat” post since it’s a compilation of other posts, web pages, message board posts and some original thoughts. I wrote it to submit to Robin’s Grand Rounds, hosted on her blog.
For all of my early life, I was the good, compliant, patient. I took whatever pills the doctor prescribed, did whatever tests h/she (most always a he) wrote for. Believed that whatever he said was the absolute truth. He had been to med school. He knew what was wrong with me even though he didn’t live in my body 24/7 and experience what I did.
I know a lot of people are still like this. Their doctor is like a god to them. He can do no wrong – even if they don’t feel any better after treatment, even if they feel worse. “But the doctor said…”
Anyway, I digress.
All this changed for me in 1983.
At first I noticed I’d stopped having my periods and, of course, I thought I was pregnant. I went to my Gynecologist who had no explanation. Lots of women lose their periods for a variety of reasons so no one thought that this was really significant.
Then I got really tired, overly tired. I would take my son to a half hour Choir rehearsal and could not stay awake for the whole time. I would lie down in the back of the van, set an alarm and sleep for the 30 minutes.
A whole raft of other symptoms started appearing – I grew a beard (Hirsuitism), gained weight even though I was on Weight Watchers and working out at the gym nearly every day, lost my period, everything hurt, got what is called a “moon face” and a “buffalo hump” on the back of my neck. I also got stretch marks. I was very depressed but it’s hard to say if that was because of the hormone imbalance or because I felt so bad and no one would listen to me.
I came across a little article in the Ladies Home Journal magazine which said “If you have these symptoms…ask your doctor about Cushing’s”. After that, I started reading everything I could on Cushing’s and asking my doctors. Due to all my reading at the library and medical books I bought, I was sure I had Cushing’s but no one would believe me. Doctors would say that Cushing’s Disease is too rare, that I was making this up and that I couldn’t have it.
I asked doctors for three years – PCP, gynecologist, neurologist, podiatrist – all said the now-famous refrain. It’s too rare. You couldn’t have Cushing’s. I kept persisting in my reading, making copies of library texts even when I didn’t understand them, keeping notes. I just knew that someone, somewhere would “discover” that I had Cushing’s.
My husband was on the doctors’ sides. He was sure it was all in my mind (as opposed to all in my head!) and he told me to just think “happy thoughts” and it would all go away.
A Neurologist gave me Xanax. Since he couldn’t see my tumor with his Magnetic Resonance Imaging (MRI) machine there was “no possibility” that it existed. Boy was he wrong!
Later in 1986 I started bruising incredibly easily. I could touch my skin and get a bruise. On New Year’s Day of 1987 I started bleeding under the skin. My husband made circles around the outside perimeter each hour with a marker, like the rings of a tree. When I went to my Internist the next day he was shocked at the size. He now thought I had a blood disorder so he sent me to a Hematologist/Oncologist.
Fortunately, the Hematologist/Oncologist ran a twenty-four hour urine test and really looked at me. Both he and his partner recognized that I had Cushing’s. Of course, he was sure that he did the diagnosis. No matter that I had been pursuing this with other doctors for 3 years.
It was not yet determined if it was Cushing’s Disease (Pituitary) or Syndrome (Adrenal). However, he couldn’t help me any further so the Hematologist referred me to an Endocrinologist.
The Endocrinologist, of course, didn’t trust the other tests I had had done so I was back to square one. He ran his own multitude of tests. He had to draw blood at certain times like 9 AM. and 5 PM. There was a dexamethasone suppression test where I took a pill at 10 p.m. and gave blood at 9 am the next day. I collected gallons of urine in BIG boxes (Fun in the fridge!). Those were from 6 a.m. to 6 a.m. to be delivered to his office by 9 a.m. same day. I was always worried that I’d be stopped in rush hour and the police would ask about what was in that big container. I think I did those for a week. He also did standard neurological tests and asked lots of questions.
When the endo confirmed that I had Cushing’s in 1987 he sent me to a local hospital where they repeated all those same tests for another week and decided that it was not my adrenal gland (Cushing’s Syndrome) creating the problem. The doctors and nurses had no idea what to do with me, so they put me on the brain cancer ward.
When I left this hospital after a week, we didn’t know any more than we had before.
As luck would have it, NIH (National Institutes of Health, Bethesda, Maryland) was doing a clinical trial of Cushing’s. I live in the same area as NIH so it was not too inconvenient but very scary at first to think of being tested there. At that time I only had a choice of NIH, Mayo Clinic and a place in Quebec to do this then-rare pituitary surgery called a Transsphenoidal Resection. I chose NIH – closest and free. After I was interviewed by the Doctors there, I got a letter that I had been accepted into the clinical trial. The first time I was there was for 6 weeks as an inpatient. More of the same tests.
There were about 12 of us there and it was nice not to be alone with this mystery disease. Many of these Cushies (mostly women) were getting bald, couldn’t walk, having strokes, had diabetes. One was blind, one had a heart attack while I was there. Towards the end of my testing period, I was looking forward to the surgery just to get this whole mess over with. While I was at NIH, I was gaining about a pound a day!
The MRI still showed nothing, so they did a Petrosal Sinus Sampling Test. That scared me more than the prospect of surgery. (This test carries the risk of stroke and uncontrollable bleeding from the incision points.) Catheters were fed from my groin area to my pituitary gland and dye was injected. I could watch the whole procedure on monitors. I could not move during this test or for several hours afterwards to prevent uncontrolable bleeding from a major artery. The test did show where the tumor probably was located. Also done were more sophisticated dexamethasone suppression tests where drugs were administered by IV and blood was drawn every hour (they put a heplock in my arm so they don’t have to keep sticking me). I got to go home for a weekend and then went back for the surgery – the Transsphenoidal Resection. I fully expected to die during surgery (and didn’t care if I did) so I signed my will and wrote last letters to those I wanted to say goodbye to. During the time I was home just before surgery, a college classmate of mine (I didn’t know her) did die at NIH of a Cushing’s-related problem. I’m so glad I didn’t find out until a couple months later!
November 3, 1987, the surgeon, Dr. Ed Oldfield, cut the gum above my front teeth under my upper lip so there is no scar. He used tiny tools and microscopes. My tumor was removed successfully. In some cases (not mine) the surgeon uses a plug of fat from the abdomen to help seal the cut. Afterwards, I was in intensive care overnight and went to a neurology ward for a few days until I could walk without being dizzy. I had some major headaches for a day or two but they gave me drugs (morphine) for those. Also, I had cotton plugs in my nostrils. It was a big day when they came out. I had diabetes insipidus (DI) for a little while, but that went away by itself – thank goodness!
I had to use a foam product called “Toothies” to brush my teeth without hitting the incision. Before they let me go home, I had to learn to give myself an injection in my thigh. They sent me home with a supply of injectible cortisone in case my level ever fell too low (it didn’t). I was weaned gradually off cortisone pills (scary). I now take no medications. I had to get a Medic Alert bracelet. I will always need to tell medical staff when I have any kind of procedure – the effects of my excess cortisone will remain forever.
I went back to the NIH for several follow-up visits of a week each where they did all the blood and urine testing again. After a few years NIH set me free. Now I go to my “outside” endocrinologist every year for the dexamethasone suppression test, 24-hour urine and regular blood testing.
As I get further away from my surgery, I have less and less chance that my tumor will grow back. I have never lost all the weight I gained and I still have the hair on my chin but most of my other symptoms are gone. I am still and always tired and need a nap most days. I do not, however, still need to take whole days off just to sleep.
I consider myself very lucky that I was treated before I got as bad as some of the others on my floor at NIH but think it is crazy that these symptoms are not taken seriously by doctors.
My story goes on and if you’re interested some is on this blog and some is here:
Because of this experience in getting a Cushing’s diagnosis – and later, a prescription for growth hormone – I was concerned that there were probably other people not being diagnosed with Cushing’s. When I searched online for Cushing’s, all the sites that came up were for dogs and horses with Cushing’s. Not what I was looking for!
In July of 2000, I was talking with my dear friend Alice, who ran a wonderful menopause site, Power Surge, wondering why there weren’t many support groups online (OR off!) for Cushing’s. This thought percolated through my mind for a few hours and I realized that maybe this was my calling. Maybe I should be the one to start a network of support for other “Cushies” to help them empower themselves.
I wanted to educate others about the awful disease that took doctors years of my life to diagnose and treat – even after I gave them the information to diagnose me. I didn’t want anyone else to suffer for years like I did. I wanted doctors to pay more attention to Cushing’s disease.
The first website (http://www.cushings-help.com) went “live” July 21, 2000. It was just a single page of information. The message boards began September 30, 2000 with a simple message board which then led to a larger one, and a larger. Today, in 2022, we have over 73 thousand members and many others on Facebook. Some “rare disease”!
People are becoming more empowered and participating in their own diagnoses, testing and treatment. This have changed a lot since 1983!
When I had my Cushing’s over 30 years ago, I never thought that I would meet another Cushing’s patient in real life or online. Back then, I’d never even been aware that there was anything like an “online”. I’m so glad that people struggling with Cushing’s today don’t have to suffer anymore thinking that they’re the only one who deals with this.
Because of my work on the websites – and, believe me it is a ton of work! – I have had the honor of meeting over a hundred other Cushies personally at local meetings, conferences, at NIH (the National Institutes of Health in Bethesda, MD where I had my final diagnosis and surgery). It occurred to me once that this is probably more than most endocrinologists will ever see in their entire career. I’ve also talked to countless others on the phone. Amazing for a “rare” disease!
I don’t know what pushed me in 1983, how I got the confidence and self-empowerment to challenge these doctors and their non-diagnoses over the years. I’m glad that I didn’t suffer any longer than I did and I’m glad that I have a role in helping others to find the medical help that they need.
What do *YOU* think? How are you becoming empowered?
So, these are only seven of the many, many symptoms of Cushing’s. I had those above – and I often felt like I looked like one of those little bearded dwarves.
Cushing’s affects every part of the body. It’s not like when I had kidney cancer and only the kidney was affected.
Here are some of the many areas affected.
Progressive obesity and skin changes
Weight gain and fatty tissue deposits, particularly around the midsection and upper back, in the face (moon face) and between the shoulders (buffalo hump). Some symptoms such as sudden weight gain, are caused by excess cortisol. The excess cortisol in the body does not increase protein and carbohydrate metabolism. It slows or nearly disables metabolism function, which can cause weight gain (fat accumulation) in the buttocks, abdomen, cheeks, neck, or upper back.
Loss of muscle mass. Some areas of the body, such as the arms and legs, will remain thin.
Pink or purple stretch marks (striae) on the skin of the abdomen, thighs, breasts and arms
Thinning, fragile skin that bruises easily
Slow healing of cuts, insect bites and infections
Acne
Women with Cushing’s syndrome may experience:
Thicker or more visible body and facial hair (hirsutism)
Irregular or absent menstrual periods
Men with Cushing’s syndrome may experience:
Decreased libido
Decreased fertility
Erectile dysfunction
Other signs and symptoms include:
Fatigue
Muscle weakness
Depression, anxiety and irritability
Loss of emotional control
Cognitive difficulties
New or worsened high blood pressure
Glucose intolerance that may lead to diabetes
Headache
Bone loss, leading to fractures over time
Hyperlipidemia (elevated lipids – cholesterol – in the blood stream)
Recurrent opportunistic or bacterial infections
Think you have Cushing’s? Get to a doctor and don’t give up!
April is always Cushing’s Awareness Challenge month because Dr. Harvey Cushing was born on April 8th, 1869.
Thanks to Robin for this wonderful past logo! I’ve participated in these 30 days for Cushing’s Awareness several times so I’m not quite sure what is left to say this year but I always want to get the word out when I can.
As I see it, there have been some strides the diagnosis or treatment of Cushing’s since last year. More drug companies are getting involved, more doctors seem to be willing to test, a bit more awareness, maybe.
How fitting that this challenge should begin on April Fool’s Day. So much of Cushing’s Syndrome/Disease makes us Cushies seem like we’re the April Fool. Maybe, just maybe, it’s the doctors who are the April Fools…
Doctors tell us Cushing’s is too rare – you couldn’t possibly have it. April Fools!
All you have to do is exercise and diet. You’ll feel better. April Fools!
Those bruises on your legs? You’re just clumsy. April Fools!
Sorry you’re growing all that hair on your chin. That happens as you age, you know. April Fools!
Did you say you sleep all day? You’re just lazy. If you exercised more, you’d have more energy. April Fools!
You don’t have stretch marks. April Fools!
You have stretch marks but they are the wrong [color/length/direction] April Fools!
The hump on the back of your neck is from your poor posture. April Fools!
Your MRI didn’t show a tumor. You couldn’t have Cushing’s. April Fools!
This is all in your mind. Take this prescription for antidepressants and go home. April Fools!
If you have this one surgery, your life will get back to normal within a few months. April Fools!
What? You had transsphenoidal surgery for Cushing’s? You wasted your time and money. April Fools!
I am the doctor. I know everything. Do not try to find out any information online. You could not have Cushing’s. It’s too rare… April FOOL!
All this reminds me of a wonderful video a message board member posted a while ago:
So now – who is the April Fool? It wasn’t me. Don’t let it be you, either!
Jessica Rotham, National Center for Health Research
What is it?
Cushing’s syndrome is a condition you probably have never heard of, but for those who have it, the symptoms can be quite scary. Worse still, getting it diagnosed can take a while. Cushing’s syndrome occurs when the tissues of the body are exposed to high levels of cortisol for an extended amount of time. Cortisol is the hormone the body produces to help you in times of stress. It is good to have cortisol at normal levels, but when those levels get too high it causes health problems. Although cortisol is related to stress, there is no evidence that Cushing’s syndrome is directly or indirectly caused by stress.
Cushing’s syndrome is considered rare, but that may be because it is under-reported. As a result, we don’t have good estimates for how many people have it, which is why the estimates for the actual number of cases vary so much–from 5 to 28 million people.[1] The most common age group that Cushing’s affects are those 20 to 50 years old. It is thought that obesity, type 2 diabetes, and high blood pressure may increase your risk of developing this syndrome.[2]
What causes Cushing’s Syndrome?
Cushing’s syndrome is caused by high cortisol levels. Cushing’s disease is a specific form of Cushing’s syndrome. People with Cushing’s disease have high levels of cortisol because they have a non-cancerous (benign) tumor in the pituitary gland. The tumor releases adrenocorticotropin hormone (ACTH), which causes the adrenal glands to produce excessive cortisol.
Cushing’s syndrome that is not Cushing’s disease can be also caused by high cortisol levels that result from tumors in other parts of the body. One of the causes is “ectopic ACTH syndrome.” This means that the hormone-releasing tumor is growing in an abnormal place, such as the lungs or elsewhere. The tumors can be benign, but most frequently they are cancerous. Other causes of Cushing’s syndrome are benign tumors on the adrenal gland (adrenal adenomas) and less commonly, cancerous adrenal tumors (adrenocortical carcinomas). Both secrete cortisol, causing cortisol levels to get too high.
In some cases, a person can develop Cushing’s syndrome from taking steroid medications, such as prednisone. These drugs, known as corticosteroids, mimic the cortisol produced by the body. People who have Cushing’s syndrome from steroid medications do not develop a tumor.[3]
What are the signs and symptoms of Cushing’s Syndrome?
The appearance of people with Cushing’s syndrome starts to change as cortisol levels build up. Regardless of what kind of tumor they have or where the tumor is located, people tend to put on weight in the upper body and abdomen, with their arms and legs remaining thin; their face grows rounder (“moon face”); they develop fat around the neck; and purple or pink stretch marks appear on the abdomen, thighs, buttocks or arms. Individuals with the syndrome usually experience one or more of the following symptoms: fatigue, muscle weakness, high glucose levels, anxiety, depression, and high blood pressure. Women are more likely than men to develop Cushing’s syndrome, and when they do they may have excess hair growth, irregular or absent periods, and decreased fertility.[4]
Why is Cushing’s Syndrome so frequently misdiagnosed?
These symptoms seem distinctive, yet it is often difficult for those with Cushing’s syndrome to get an accurate diagnosis. Why? While Cushing’s is relatively rare, the signs and symptoms are common to many other diseases. For instance, females with excess hair growth, irregular or absent periods, decreased fertility, and high glucose levels could have polycystic ovarian syndrome, a disease that affects many more women than Cushing’s. Also, people with metabolism problems (metabolic syndrome), who are at higher than average risk for diabetes and heart disease, also tend to have abdominal fat, high glucose levels and high blood pressure.[5]
Problems in testing for Cushing’s
When Cushing’s syndrome is suspected, a test is given to measure cortisol in the urine. This test measures the amount of free or unbound cortisol filtered by the kidneys and then released over a 24 hour period through the urine. Since the amount of urinary free cortisol (UFC) can vary a lot from one test to another—even in people who don’t have Cushing’s—experts recommend that the test be repeated 3 times. A diagnosis of Cushing’s is given when a person’s UFC level is 4 times the upper limit of normal. One study found this test to be highly accurate, with a sensitivity of 95% (meaning that 95% of people who have the disease will be correctly diagnosed by this test) and a specificity of 98% (meaning that 98% of people who do not have the disease will have a test score confirming that).[6] However, a more 2010 study estimated the sensitivity as only between 45%-71%, but with 100% specificity.[7] This means that the test is very accurate at telling people who don’t have Cushing’s that they don’t have it, but not so good at identifying the people who really do have Cushing’s. The authors that have analyzed these studies advise that patients use the UFC test together with other tests to confirm the diagnosis, but not as the initial screening test.[8]
Other common tests that may be used to diagnose Cushing’s syndrome are: 1) the midnight plasma cortisol and late-night salivary cortisol measurements, and 2) the low-dose dexamethasone suppression test (LDDST). The first test measures the amount of cortisol levels in the blood and saliva at night. For most people, their cortisol levels drop at night, but people with Cushing’s syndrome have cortisol levels that remain high all night. In the LDDST, dexamethasone is given to stop the production of ACTH. Since ACTH produces cortisol, people who don’t have Cushing’s syndrome will get lower cortisol levels in the blood and urine. If after giving dexamethasone, the person’s cortisol levels remain high, then they are diagnosed with Cushing’s.[9]
Even when these tests, alone or in combination, are used to diagnose Cushing’s, they don’t explain the cause. They also don’t distinguish between Cushing’s syndrome, and something called pseudo-Cushing state.
Pseudo-Cushing state
Some people have an abnormal amount of cortisol that is caused by something unrelated to Cushing’s syndrome such as polycystic ovarian syndrome, depression, pregnancy, and obesity. This is called pseudo-Cushing state. Their high levels of cortisol and resulting Cushing-like symptoms can be reversed by treating whatever disease is causing the abnormal cortisol levels. In their study, Dr. Giacomo Tirabassi and colleagues recommend using the desmopressin (DDAVP) test to differentiate between pseudo-Cushing state and Cushing’s. The DDAVP test is especially helpful in people who, after being given dexamethasone to stop cortisol production, continue to have moderate levels of urinary free cortisol (UFC) and midnight serum cortisol.[10]
An additional test that is often used to determine if one has pseudo-Cushing state or Cushing’s syndrome is the dexamethasone-corticotropin-releasing hormone (CRH) test. Patients are injected with a hormone that causes cortisol to be produced while also being given another hormone to stop cortisol from being produced. This combination of hormones should make the patient have low cortisol levels, and this is what happens in people with pseudo-Cushing state. People with Cushing’s syndrome, however, will still have high levels of cortisol after being given this combination of hormones.[11]
How can Cushing’s be treated?
Perhaps because Cushing’s is rare or under-diagnosed, few treatments are available. There are several medications that are typically the first line of treatment. None of the medications can cure Cushing’s, so they are usually taken until other treatments are given to cure Cushing’s, and only after that if the other treatment fails.
The most common treatment for Cushing’s disease is transsphenoidal surgery, which requires the surgeon to reach the pituitary gland through the nostril or upper lip and remove the tumor. Radiation may also be used instead of surgery to shrink the tumor. In patients whose Cushing’s is caused by ectopic ACTH syndrome, all cancerous cells need to be wiped out through surgery, chemotherapy, radiation or a variety of other methods, depending on the location of the tumor. Surgery is also recommended for adrenal tumors. If Cushing’s syndrome is being caused by corticosteroid (steroid medications) usage, the treatment is to stop or lower your dosage.[12]
Medications to control Cushing’s (before treatment or if treatment fails)
According to a 2014 study in the Journal of Clinical Endocrinology and Metabolism, almost no new treatment options have been introduced in the last decade. Researchers and doctors have focused most of their efforts on improving existing treatments aimed at curing Cushing’s. Unfortunately, medications used to control Cushing’s prior to treatment and when treatment fails are not very effective.
Many of the medications approved by the FDA for Cushing’s syndrome and Cushing’s disease, such as pasireotide, metyrapone, and mitotane, have not been extensively studied. The research presented to the FDA by the makers of these three drugs did not even make clear what an optimal dose was.[13] In another 2014 study, published in Clinical Epidemiology, researchers examined these three same drugs, along with ten others, and found that only pasireotide had moderate evidence to support its approval. The other drugs, many of which are not FDA approved for Cushing’s patients, had little or no available evidence to show that they work.[14] They can be sold, however, because the FDA has approved them for other diseases. Unfortunately, that means that neither the FDA nor anyone else has proven the drugs are safe or effective for Cushing patients.
Pasireotide, the one medication with moderate evidence supporting its approval, caused hyperglycemia (high blood sugar) in 75% of patients who participated in the main study for the medication’s approval for Cushing’s. As a result of developing hyperglycemia, almost half (46%) of the participants had to go on blood-sugar lowering medications. The drug was approved by the FDA for Cushing’s anyway because of the lack of other effective treatments.
Other treatments used for Cushing’s have other risks. Ketoconazole, believed to be the most commonly prescribed medications for Cushing’s syndrome, has a black box warning due to its effect on the liver that can lead to a liver transplant or death. Other side effects include: headache, nausea, irregular periods, impotence, and decreased libido. Metyrapone can cause acne, hirsutism, and hypertension. Mitotane can cause neurological and gastrointestinal symptoms such as dizziness, nausea, and diarrhea and can cause an abortion in pregnant women.[15]
So, what should you do if you suspect you have Cushing’s Syndrome?
Cushing’s syndrome is a serious disease that needs to be treated, but there are treatment options available for you if you are diagnosed with the disease. If the symptoms in this article sound familiar, it’s time for you to go see your doctor. Make an appointment with your general practitioner, and explain your symptoms to him or her. You will most likely be referred to an endocrinologist, who will be able to better understand your symptoms and recommend an appropriate course of action.
All articles are reviewed and approved by Dr. Diana Zuckerman and other senior staff.
Nieman, Lynette K. Epidemiology and clinical manifestations of Cushing’s syndrome, 2014. UpToDate: Wolters Kluwer Health
Newell-Price, John, Peter Trainer, Michael Besser and Ashley Grossman. The diagnosis and differential diagnosis of Cushing’s syndrome and pseudo-Cushing’s states, 1998. Endocrine Reviews: Endocrine Society
Carroll, TB and JW Findling. The diagnosis of Cushing’s syndrome, 2010. Reviews in Endocrinology and Metabolic Disorders: Springer
Ifedayo, AO and AF Olufemi. Urinary free cortisol in the diagnosis of Cushing’s syndrome: How useful?, 2013. Nigerian Journal of Clinical Practice: Medknow.
Tirabassi, Giacomo, Emanuela Faloia, Roberta Papa, Giorgio Furlani, Marco Boscaro, and Giorgio Arnaldi. Use of the Desmopressin test in the differential diagnosis of pseudo-Cushing state from Cushing’s disease, 2013. The Journal of Clinical Endocrinology & Metabolism: Endocrine Society.
Tirabassi, Giacomo, Emanuela Faloia, Roberta Papa, Giorgio Furlani, Marco Boscaro, and Giorgio Arnaldi. Use of the Desmopressin test in the differential diagnosis of pseudo-Cushing state from Cushing’s disease, 2013. The Journal of Clinical Endocrinology & Metabolism: Endocrine Society.
Galdelha, Monica R. and Leonardo Vieira Neto. Efficacy of medical treatment in Cushing’s disease: a systematic review, 2014. Clinical Endocrinology: John Wiley & Sons.
In 1983 I came across a little article in the Ladies Home Journal which said: “If you have these symptoms…”
In med school, student doctors are told “When you hear hoofbeats, think horses, not zebras“.
Our son lives in New York and every few years he gives us tickets to see a Broadway show. A couple years ago we took the train to NY to see 
