Cushing’s syndrome is a rare disorder that occurs when the body is exposed to too much cortisol.Cortisol is produced by the body and is also used in corticosteroid drugs. Cushing’s syndrome can occur either because cortisol is being overproduced by the body or from the use of drugs that contain cortisol (like prednisone ).
Cortisol is the body’s main stress hormone. Cortisol is secreted by the adrenal glands in response to the secretion of adrenocorticotropic hormone (ACTH) by the pituitary. One form of Cushing’s syndrome may be caused by an oversecretion of ACTH by the pituitary leading to an excess of cortisol.
Cortisol has several functions, including the regulation of inflammation and controlling how the body uses carbohydrates, fats, and proteins. Corticosteroids such as prednisone, which are often used to treat inflammatory conditions, mimic the effects of cortisol.
I’ve been searching every possible alternative explanation. I really hoped I just had a Lipoma but GP was pretty confident no.
I’ve gained about 40lbs in the past year, I bruise easily and I have a Buffalo Hump. Cycle is regular, my mood is good, well ya know pandemic, home schooling, owning a business … Lol I don’t think I’m suffering any unusual stress or anxiety though. I am anxious over the time it’s taking to get any info.
I had blood drawn and an upcoming sono, date TBD, but don’t see my GP for 5 wks!
Can I get blood results over ph and skip right to Endocrinologist if Cortisol is high? Any input of speeding this along?
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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.
In March of 1987, after the endo finally confirmed that I had Cushing’s, I was sent 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.
My husband asked my endo if it were his wife, if he would recommend this surgery. The endo responded that he was divorcing his wife – he didn’t care what happened to her. Oh, my!
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 night before I was admitted, I signed my will. I was sure I was going to die there. If not during testing, as a result of surgery.
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. Several were from Greece.
My first roommate was a nurse. She spent the entire first night screaming in pain. I was very glad when they moved me to a new room!
Towards the end of my testing period, I was looking forward to the surgery just to get this whole mess over with – either a cure or dying. While I was at NIH, I was gaining about a pound a day!
During the time I was home the weekend 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 reading the alumnae magazine a couple months later! She was the same class, same major, same home-town, same disease…
We have a Scottish doctor named James Lind to thank for the clinical trial. He conducted the first ever clinical trial in 1747 and developed the theory that citrus fruits cured scurvy. Lind compared the effects of various different acidic substances, ranging from vinegar to cider, on groups of afflicted sailors, and found that the group who were given oranges and lemons had largely recovered from scurvy after 6 days.
I’d like to think that I advanced the knowledge of Cushing’s at least a little bit by being a guinea pig in 1987-1989.
Several components of the National Institutes of Health (NIH) conduct and support research on Cushing’s syndrome and other disorders of the endocrine system, including the National Institute of Diabetes and Digestive and Kidney Diseases, the National Institute of Child Health and Human Development (NICHD), the National Institute of Neurological Disorders and Stroke, the National Cancer Institute, and the National Center for Research Resources.
NIH-supported scientists are conducting intensive research into the normal and abnormal function of the major endocrine glands and the many hormones of the endocrine system. Researchers continue to study the effects of excess cortisol, including its effect on brain structure and function. To refine the diagnostic process, studies are under way to assess the accuracy of existing screening tests and the effectiveness of new imaging techniques to evaluate patients with ectopic ACTH syndrome. Researchers are also investigating jugular vein sampling as a less invasive alternative to petrosal sinus sampling. Research into treatment options includes study of a new drug to treat the symptoms of Cushing’s syndrome caused by ectopic ACTH secretion.
Studies are under way to understand the causes of benign endocrine tumor formation, such as those that cause most cases of Cushing’s syndrome. In a few pituitary adenomas, specific gene defects have been identified and may provide important clues to understanding tumor formation. Endocrine factors may also play a role. Increasing evidence suggests that tumor formation is a multistep process. Understanding the basis of Cushing’s syndrome will yield new approaches to therapy.
The NIH supports research related to Cushing’s syndrome at medical centers throughout the United States. Scientists are also treating patients with Cushing’s syndrome at the NIH Clinical Center in Bethesda, MD. Physicians who are interested in referring an adult patient may contact Lynnette Nieman, M.D., at NICHD, 10 Center Drive, Room 1-3140, Bethesda, MD 20892-1109, or by phone at 301-496-8935. Physicians interested in referring a child or adolescent may contact Constantine Stratakis, M.D., D.Sc., at NICHD, 10 Center Drive, Room 1-3330, Bethesda, MD 20892-1103, or by phone at 301-402-1998.
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
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:
Other signs and symptoms include:
Depression, anxiety and irritability
Loss of emotional control
New or worsened high blood pressure
Glucose intolerance that may lead to diabetes
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!
Myth: Even though you are chronically ill, you should have the same amount of energy every day. “You look SO good and you went to that party last month! Why can’t you come to MY party?!” When you say you are not well enough to do something, you are just making excuses. You could do it, just like you did that other thing; you are just choosing not to!
Fact: You may have heard me talk about “The Spoon Theory”. It was created by someone named Christine Miserandino, to explain the experience of someone with chronic illness in terms of using energy to live and to complete tasks every day. Though the myth assumes that one should have the same amount of energy all the time; the fact is that energy levels fluctuate and people who are chronically ill must make conscious decisions about what they can spend their energy on.
Christine Miserandino (2010) uses the spoon theory to answer the question, “What does it feel like to be sick?” The spoons serve as a symbol for resources available and energy spent to get through every moment of every day. Miserandino states that “The difference in being sick and being healthy is having to make choices or to consciously think about things when the rest of the world doesn’t have to”. Most people who get sick feel a loss of a life they once knew. When you are healthy, you expect to have a never ending supply of spoons. But, when you are not well, you need to count your spoons to keep track and you can never forget about it or take it for granted. Each task costs a spoon and each spoon is not to be taken for granted. Miserandino (2010) asks, “Do you know how many spoons people waste every day?”
Patients use the metaphor of a banking system. In this system, patients must make a withdrawal of a spoon every time they complete a task. Cushing’s and Adrenal Insufficiency patients talk about the “Cortisol Bank” metaphor. The concept is the same and the idea is that certain stressors and/or tasks cause one’s body to make a cortisol withdrawal from the body. Bad things happen when there is a cortisol deficit, meaning that there is not enough cortisol in the body for one to live everyday because of the amount of cortisol that has already been used up. If a person continues to draw from the bank on an account that is already negative, the situation can become worse and worse as each day passes.
Something needs to happen in order to start making appropriate deposits. This can include, taking more medication (stress dosing or an emergency shot), resting, getting adequate physical and emotional support and help, and saying “NO!”. Even when in a deficit, many patients have a difficult time saying “no” to an invitation to an event, completing a task, or engaging someone in a way that will use up more energy because of their fear of their loved one’s reactions. Much of the time, this fear is warranted because of the actual reactions they have received. Ever heard, “But you volunteered for the bake sale last week! You must be better! Why can’t you come to church this week?!”. You may have heard something similar.
It is important for loved ones to understand the amount of “spoons” it takes for a chronically ill person just to get through every single day. EVERYTHING costs spoons! The amount of spoons paid by each person varies from person to person. It all depends on that individual’s situation, body, level of illness, etc. What is common for all, though, is that spoons must be used and eventually those spoons run out. In order to avoid becoming sick or to recuperate from getting sick, the chronically ill patient must evaluate how he/she will use spoons and what tasks can be feasibly completed that day or week. Please understand that when the chronically ill patient says, “YES” to you; he/she is making a conscious choice to use up spoons to meet your need, request, or demand. Talking on the phone, going out to lunch, making dinner, coming to your event all required a sacrifice of another task that day or week. Your friend may have come to lunch with you but that required that she skipped washing the dishes that day or washing her hair, or is even giving up doing something important the next day. Instead of being angry at your friend, please consider why the request is denied at times.
Refer to the attached picture. This is not an exact science but gives some idea of the spoon bank. If you have time, try doing this exercise: Lay out 8-12 physical spoons. As you complete certain tasks throughout the day, use this chart to subtract spoons from your pile.
Each and every thing requires a spoon. Taking a shower, washing your hair, cooking, cleaning, watching a movie, going out to lunch, working, writing this post (Ha)! When you are done with your day, notice how many spoons you have left. Observe your feelings after this exercise. You can even do it for a week. Lay out a certain amount of spoons for every day for seven days. If you go into a deficit, borrow spoons from the following day. However, if you do borrow spoons; you must take away a task that you WERE planning to originally do that day. Notice what happens and notice how you feel at the end of the week.
Can you think of any other tasks that are not on this chart? Help our friends who are doing the activity. List those tasks and assign how many spoons each task will require. Spoon Bank
Get out of bed- 1 Spoon
Shower- 2 Spoons
Attend Special Event- 5 Spoons
Go out for Coffee- 4 Spoons
Drive- 4 Spoons
Make a Phone Call- 3 Spoons
Work- 5 spoons
Play Games-3 Spoons
Clean the House- 5 Spoons
Have a Meal- 2 Spoons
Walk the Dog- 4 Spoons
Study- 5 Spoons
Watch TV- 3 Spoons
Ironing- 5 Spoons
Exercise- 4 Spoons
Shopping- 4 Spoons
Read- 2 Spoons
Catch Public Transport- 4 Spoons
Cook- 4 Spoons
Myth: “Each person requires the same dose of steroid in order to survive with Secondary or Primary Adrenal Insufficiency”
Fact: In simple terms, Adrenal Insufficiency occurs when the body does not have enough cortisol in it. You see, cortisol is life sustaining and we actually do need cortisol to survive. You have probably seen the commercials about “getting rid of extra belly fat” by lowering your cortisol. These advertisements make it hard for people to actually understand the importance of the function of cortisol.
After a Cushing’s patient has surgery, he/she goes from having very high levels of cortisol to no cortisol at all. For pituitary patients, the pituitary, in theory, should start working eventually again and cause the adrenal glands to produce enough cortisol. However, in many cases; the pituitary gland does not resume normal functioning and leaves a person adrenally insufficient. The first year after pit surgery is spent trying to get that hormone to regulate on its own normally again. For a patient who has had a Bilateral Adrenalectomy (BLA), where both adrenal glands are removed as a last resort to “cure” Cushing’s; his/her body will not produce cortisol at all for his/her life. This causes Primary Adrenal Insufficiency.
All Cushing’s patients spend time after surgery adjusting medications and weaning slowly from steroid (cortisol) to get the body to a maintenance dose, which is the dose that a “normal” body produces. This process can be a very long one. Once on maintenance, a patient’s job is not over. He/She has to learn what situations require even more cortisol. You see, cortisol is the stress hormone and also known as the Fight or Flight hormone. Its function is to help a person respond effectively to stress and cortisol helps the body compensate for both physical and emotional stress. So, when faced with a stressor, the body will produce 10X the baseline levels in order to compensate. When a person can not produce adequate amounts of cortisol to compensate, we call that Adrenal Insufficiency. If it gets to the point of an “Adrenal Crisis”, this means that the body can no longer deal and will go into shock unless introduced to extremely high levels of cortisol, usually administered through an emergency shot of steroid.
There are ways to help prevent a crisis, by taking more steroid than the maintenance dose during times of stress. This can be anything from going to a family function (good stress counts too) to fighting an infection or illness. Acute stressors such as getting into a car accident or sometimes even having a really bad fight require more cortisol as well.
It was once believed that everyone responded to every stressor in the exact same way. So, there are general guidelines about how much more cortisol to introduce to the body during certain stressors. For instance, during infection, a patient should take 2-3X the maintenance dose of steroid (cortisol). Also, even the maintenance dose was considered the same for everyone. Now a days, most doctors will say that 20 mg of Hydrocortisone (Steroid/Cortisol) is the appropriate maintenance dose for EVERYONE. Now, we know that neither is necessarily true. Although the required maintenance dose is about the same for everyone; some patients require less and some require more. I have friends who will go into an adrenal crisis if they take LESS than 30 mg of daily steroid. On the other hand, 30 mg may be way too much for some and those folks may even require LESS daily steroid, like 15 mg. Also, I want to stress (no pun intended) that different stressors affect different people differently. For some, for instance, an acute scare may not affect them. However, for others, receiving bad news or being in shock WILL put their bodies into crisis. That person must then figure out how much additional steroid is needed.
Each situation is different and each time may be different. Depending on the stressor, a person may need just a little more cortisol or a lot. Every person must, therefore, learn their own bodies when dealing with Adrenal Insufficiency. This is VERY important! I learned this the hard way. As a Clinical Psychologist; I assumed that my “coping skills” would be enough to prevent a stressor from putting me into crisis. That was FAR from the truth! I have learned that I can not necessarily prevent my body’s physiological response to stress. People often ask me, “BUT you are a psychologist! Shouldn’t you be able to deal with stress?!!!!” What they don’t realize is that my BODY is the one that has to do the job of compensating. Since my body can not produce cortisol at all, my job is to pay close attention to it so that I can take enough steroid to respond to any given situation. We all have to do that. We all have to learn our own bodies. This is vitally important and will save our lives!
To those we have lost in our community to Adrenal Insufficiency after treatment of Cushing’s, Rest in Peace my friends! Your legacies live on forever!