Debunking the Myth about Cyclical Cushing’s
By Dr. N. Karen Thames
Licensed Clinical Psychologist
Founder and Executive Director of The EPIC Foundation
There is a myth that “Cyclical Cushing’s does NOT exist!” Once I realized how prevalent this myth is, even within the medical community, I felt compelled to address this myth.
“Cyclic Cushing’s syndrome (CS) is a rare disorder, characterized by repeated episodes of cortisol excess interspersed by periods of normal cortisol secretion. The so-called cycles of hypercortisolism can occur regularly or irregularly with intercyclic phases ranging from days to years” (Meinardi et. al, 2007). Adequate and appropriate testing requires multiple test measures over an extended period of time. For this reason, testing can take months and sometimes years. Florid Cushing’s refers to Cushing’s where the patient has consistent and constant high levels of cortisol. For these patients, they will always have abnormal test results. This is not the case for Cyclical Cushing’s patients.
The fact is that Cyclical Cushing’s does exist! The prevalence of Cushing’s patients who cycle is much higher than was once thought. In fact, in a study done in 2007, it was found that Cyclical Cushing’s cases made up 54% (more than half) of the cases of Cushing’s originating from a pituitary tumor, (adenoma), in 26% from an ectopic ACTH-producing tumor, and adrenal tumor cases accounted for 11% of Cyclical Cushing’s cases (Meinardi et al.)
The old school way of thinking was that patients with Cushing’s had consistent and constantly high cortisol levels, causing their Cushing’s symptoms. Now, we know that many patients cycle, which means that they have PERIODS of excessive cortisol levels causing Cushing’s. However, the same patients may also have periods where their cortisol is seemingly normal or even low. There are multiple factors involved with patients’ test results showing for no abnormalities including possible external variables (Friedman et al., 2010).
Some doctors, even experts in the field, will dismiss a patient and suspend testing from Cushing’s if the patient has even one “normal” test result. There is a myth that just one normal test results warrants the suspension of testing, even when a patient presents with symptoms of Cushing’s. Well, now we know that a number of variables can contribute to a result that is not truly reflective of the patient’s medical state, including possible external test error (Friedman et al.) Also, assuming that those external variables were not a factor in a patient with a normal test for Cushing’s, we know that it is, in fact possible, for a patient with Cushing’s to cycle, but many people argue that Cyclical Cushing’s is an even more rare form of Cushing’s Syndrome.
There is a misconception that the disease is not as serious for these patients. This is simply not true! Patients with Cyclical Cushing’s experience the same symptoms and are very sick, particularly during times when their cortisol is high. Ironically, these patients can also experience Adrenal Insufficiency, which means that the body is not producing enough cortisol and can go into shock. Even the patients who experience Adrenal Insufficiency (AI) are still sick from their Cushing’s. The sad part is that, though there is a wealth of research on Cyclical Cushing’s, there are even doctors who do not believe it exists. Either they claim they have never seen it or they refuse to diagnose anyone with fluctuating cortisol levels, even IF that patient has periods when they have higher than normal cortisol levels.
Again, it is very important to test adequately enough to discover your pattern of “highs”. “The tests results used to document episodes of hypercortisolemia in cyclic Cushing’s Syndrome will be similar to those of traditional Cushing’s Syndrome except one will observe periodic episodes of “normal” test results. The duration of the cycles has been described as being 12 hours to 86 days. This range is in contrast to the condition referred to as intermittent Cushing’s Syndrome, in which the cycle of cortisol production is irregular and ranges from 10 episodes of relapse in 3 months to two episodes of exacerbation in 5 years” (Velez et. al, 2007). According to my great friend and Cushing’s advocate, Melissa Moxie, “You must figure out when your body is in a cortisol high and only test then” (www.Cushingsmoxie.blogspot.com, 2016). Everyone’s cycles are different and there is a pattern of high periods. It is up to the patient and his/her medical team to discover/determine what those high patterns are.
Let me be very clear. Cushing’s Syndrome/Disease IS life threatening! We have lost people in the community to this dreadful disease both before and after treatment! Cyclical Cushing’s patients are at just as much risk as those who have consistently high cortisol levels. If a Cyclical Cushing’s patient does not receive adequate help and treatment, this will lead to death! In my book, there is no such thing as a “milder case”! Cushing’s is Cushing’s and WILL kill its sufferers if left untreated.
If you suspect Cyclical Cushing’s, it is important to find a doctor who can follow an adequate testing and diagnostic protocol. Some people who have Cushing’s symptoms get dismissed because they come up normal on ONE test! This is not appropriate at all! If you happen to be in a low or normal cycle period, that ONE test did not pick up your high levels. Being dismissed and ignoring it will not make the symptoms go away. Only a Cushing’s EXPERT will know when it is appropriate to rule out Cushing’s!
There are doctors who will only diagnose a patient with Cushing’s if their pathology is Florid in nature and if their cortisol levels are at least 3X the normal range. You may feel discouraged if you have visited such a doctor and you don’t meet their criteria. However, I encourage you to keep fighting. YOU know your body! Find someone with expertise in Cyclical Cushing’s and who knows the appropriate way of assessing and treating it. Remember, if left untreated, Cushing’s Disease can and will lead to a fatality! You have our support! You are a warrior!
References
- Friedman, T. C., Ghods, D. E., Shahinian, H. K., Zachery, L., Shayesteh, N., Seasholtz, S, Zuckerbraun, E., Lee, M. L. , McCutcheon, I. E. (2010). High Prevalence of Normal Tests Assessing Hypercortisolism in Subjects with Mild and Episodic Cushing ’ s Syndrome Suggests that the Paradigm for Diagnosis and Exclusion of Cushing ’ s Syndrome Requires Multiple Testing. Horm Metab Res, 42, 874 – 881.
- Meinardi1, J R , Wolffenbuttel, B H R, Dullaart, R P F. Cyclic Cushing’s syndrome: a clinical challenge. Eur J Endocrinol, 157, 245-254.
- Velez, D.A., Mayberg, M. R., Ludlam, W.H. Diagnosis and Management of Cushing’s Disease: Cyclic Cushing syndrome: definitions and treatment implications. Neurosurgical Focus 23 (3), 1-3.
- www.Cushingsmoxie.blogspot.com