Dr. Theodore Friedman will be back in Denver, Colorado and will host a free patient support group on Sunday November 10, 2013 at 10 am at his sister’s house.
152 S Trenton
Denver CO 80230
It’s in Lowry
Please bring your questions.
Feel free to mention this to other Colorado patients.
Filed under: Cushing's, FAQ, Meetings and Conferences | Tagged: Colorado, Denver, Dr. Theodore Friedman, meeting, support group | Leave a comment »
Have you ever noticed that when you are “stressed” you can feel either emotionally/physically depleted or energized? When our body is under stress the brain responds by producing epheniphrine or adrenaline, sending signals to our adrenal glands, increasing the rate at which our heart beats while releasing oxygen to our muscles. The long term response to this process produces cortisol (aka the stress hormone) facilitating the release of energy throughout our body. However, when our body isn’t properly balanced these hormones can wreak havoc on our wellness possibly resulting in one of three conditions: Cushing’s syndrome, Cushing’s disease or Addison’s disease.
The actual Adrenal glands sit physically atop both kidneys, taking on a triangular shape and a roundish rectangular type shape. These glands are responsible for our sex hormones and cortisol, helping us respond to stress amongst other functions. When our body is under stress, physically and/or nutritionally, it responds one of two ways: Produces too much or too little of the cortisol hormone. Our Adrenal glands also contribute to regulating our blood sugar, blood pressure, salt and water.
Adrenal disorders can cause our body to make too much or not enough of these hormones, bringing about adrenal gland related syndromes and disease. Cushing’s syndrome results from our body making too much versus Addison’s disease produces too little.
Cushing’s syndrome vs. Cushing’s disease
Glucocorticoids (naturally produced in our body or received through medicine) are groups of corticosteroids (cortisol or dexamethasone) involved in metabolizing our carbohydrate and protein. When taken synthetically (i.e. treatment of allergies, skin problems, and respiratory problems) or over-produced naturally, the side effects can result in “Cushing’s syndrome”.
Cushing’s syndrome can occur one of two ways: Endogenous or Exogenous. Endogenous is caused by the body (usually through tumors). Exogenous is caused by medication. In both cases, the body produces too much cortisol.
Symptoms: Severe fatigue/muscle weakness, high blood sugar and high blood pressure, upper body obesity, thin arms/legs, bruising easily
Treatment: The cure and treatment for Cushing’s disease can come through medicine, surgery, or by lowering the dosage of your current synthetic hormone treatment. Cushing’s syndrome can be cured.
Cushing’s disease is the most common form of endogenous Cushing’s syndrome and is likely treatable. Caused by a tumor in the pituitary gland secreting too much Adrenocorticotropic hormone (ACTH), this type of tumor does not spread and can be removed through surgery.
Nutrition: See a nutritionist or dietician for your condition. Mostly, avoid excess sodium. High blood sugar (hyperglycemia) and high blood pressure can easily occur with this condition. Bone loss density is common with this condition, so be extra aware of your calcium (800 – 1200 mg per day, based upon age) and Vitamin D intake (5mcg from age 0-50, increasing up to 10 mcg 50-71, and 15 mcg after 71). Eating healthy, balanced and whole food (versus processed) is extremely important.
(Resource: http://www.aboutcushings.com/understanding-cushings-disease/causes-and-differences.jsp)
Addison’s disease
Opposite from Cushing’s syndrome, Addison’s disease doesn’t make “enough” of the sex hormones and cortisol. The result of this disease causes our immune system to attack our tissue, damaging our adrenal glands.
Symptoms: Weight loss, muscle weakness, increasingly worse fatigue, low blood pressure and patchy or dark skin.
Treatment: If left untreated, the condition can be fatal. Lifetime hormone treatment is usually required. Addison disease patients should always carry medical/emergency ID on them, listing their medication, dosage and disease
Lab tests can confirm that you have Addison’s disease. If you don’t treat it, it can be fatal. You will need to take hormone pills for the rest of your life. If you have Addison’s disease, you should carry an emergency ID. It should say that you have the disease, list your medicines and say how much you need in an emergency.”
(Ref: http://www.nlm.nih.gov/medlineplus/cushingssyndrome.html, NIH: National Institute of Neurological Disorders and Stroke) Learning how to balance our stress-filled lives is extremely important to our overall health. Healthy nutrition always contributes benefits to our overall wellness. We can overwhelm our endocrine system by simply not eating nutritionally. Understanding that “Food is a drug” is vitally important to how we help our body naturally heal itself. The above two conditions are the result of our body not handling the stress we are putting it through, causing our body to producing too much or too little of the sex hormones and cortisol.
Unless we first address what we can do naturally through nutrition, the medicine we consume will only do so much in helping our body heal completely. You simply cannot continue doing the same thing over and over again, expecting the medicine to do all the work. Some diseases are brought upon us through our environment (emotionally as well as physically) as well as our diet/nutrition. Reviewing our entire wellness is always wisdom whenever we’re diagnosed with anything.
Certainly listen to your doctor and their advice. But also ask your doctor to refer you to a nutritionist or clinical/registered dietician for a complete evaluation that includes a review of your nutritional diet/wellness. Too often we reach for a pill or a procedure to “fix” our health problems, ignoring what we should be doing on our own to help our body heal. Medical intervention is the result of providing our body with what it cannot produce on its own. Nutrition should always be the “natural” medicine we take, as well as what we might need through prescribed medication.
Adapted from (Spelling errors corrected) http://hamptonroads.com/2013/10/remaining-calm-reducing-illness
Filed under: adrenal, Cushing's, pituitary, Treatments | Tagged: ACTH, Addison's disease, adrenal, Adrenocorticotropic hormone, blood pressure, blood sugar, bruising, cortisol, Cushing's Disease, Cushing's Syndrome, dexamethasone, endogenous, epin, exogenous, fatigue, medic alert, muscle weakness, obesity, patchy skin, pituitary, sex hormones, weight loss | 2 Comments »
–Author Affiliations
Background: The outcome of bilateral adrenalectomy (BADx) in patients with Cushing’s syndrome (CS) is not well characterized.
Methods: A literature search was performed with the search terms “bilateral adrenalectomy” or “total adrenalectomy” and “Cushing’s” or “Cushing.” Immediate and long-term outcomes after BADx in CS were analyzed using descriptive statistics (median [range]).
Results: From 549 screened publications, 37 studies met inclusion criteria (1320 patients, 82% having Cushing’s disease [CD], 13% having ectopic CS, and 5% having primary adrenal hyperplasia).
Surgical morbidity and mortality of BADx (23 studies, 739 patients) were 18% (6–31) and 3% (0–15), respectively. In patients with CD, surgical mortality was below 1%. Although residual cortisol secretion due to accessory adrenal tissue or adrenal remnants was found in 3–34% (5 studies, 236 patients), less than 2% had a relapse of CS.
Symptoms of hypercortisolism (eg, hypertension, obesity, or depression) improved in the majority of the patients after BADx (7 studies, 195 patients). The number of adrenal crises per 100 patient-years was 9.3 (6 studies, 203 patients).
Nelson’s syndrome occurred in 21% (0–47) of the patients (24 studies, 768 patients). Mortality (23 studies, 690 patients) was 17% (0–88) at a follow-up of 41 months (14–294). Remarkably, 46% of the patients died in the first year after surgery. The median ages at death were 62 years (CD) and 53 years (ectopic CS).
Conclusion: BADx is relatively safe and provides adequate success. Excess mortality within the first year after surgery suggests that intensive clinical care for patients after BADx is warranted.
For editorial see page 3974
Abbreviations:
From http://jcem.endojournals.org/content/98/10/3939.abstract
Filed under: adrenal, Cushing's, pituitary, Treatments | Tagged: abstract, adrenal, bilateral laparoscopic adrenalectomy, BLA, cortisol, Cushing's, depression, ectopic, hypercortisolism, hypertension, mortality, Nelson's syndrome, obesity, primary adrenal hyperplasia, transsphenoidal | Leave a comment »
By Salynn Boyles, Contributing Writer, MedPage Today
There is a paucity of clinical trial data supporting the efficacy of most drugs used to treat Cushing’s disease, researchers reported.
Just one drug — pasireotide — has been evaluated in a randomized, double-blind trial, but even it was judged by the researchers to have only a ‘moderate’ level of evidence supporting its effectiveness and safety.
The review of the literature evaluating drug treatments for Cushing’s disease, a rare pituitary disorder, is the first to employ a rigorous systematic approach with strict, predefined inclusion criteria and formal analysis of the quality of evidence using an established standard, researcher Monica Gadelha, MD, PhD, of Brazil’s Federal University of Rio de Janeiro, and colleagues wrote in the journal Clinical Endocrinology.
“This systematic review indicates that the majority of medical therapies currently used in the treatment of Cushing’s disease are supported by a low level of evidence,” the researchers wrote. “Further well-designed prospective studies of medications in Cushing’s disease would help to inform clinical practice further.”
Cushing’s disease is the most common form of endogenous Cushing’s syndrome, a hormonal disorder resulting from persistent exposure to abnormally high levels of the hormone cortisol. In the case of Cushing’s disease, the cortisol is secreted by a pituitary adenoma.
Prolonged exposure to high levels of cortisol raises the risk for diabetes mellitus, cardiovascular disease, osteoporosis and nephrolithiasis. Patients with persistent Cushing’s disease have a 3- to 5-fold higher mortality than the general population.
Surgery to remove the pituitary adenoma is the first-line treatment for Cushing’s disease in the U.S., and when the procedure is performed by an experienced surgeon, remission rates in patients with smaller tumors range from 65% to 90%. The long-term remission rate is lower, however, because many patients develop recurrent disease.
Several medical therapies are widely used to treat patients who are not candidates for surgery or who experience relapse following surgery.
Novartis Oncology’s somatostatin analog drug pasireotide (Signifor) became the only drug approved for this indication in December of last year. And the progesterone-blocking drug mifepristone, best known as the abortion pill once called RU-486, was approved in February of 2012 for the treatment of Cushing’s disease-associated hyperglycemia.
Other drugs — including metyrapone, mitotane, cabergoline, and ketoconazole — are also used off-label in the treatment of Cushing’s, and several have shown better response rates than pasireotide in small studies.
In their systematic review, Gadelha and colleagues identified 15 studies that included at least 10 adults with Cushing’s disease and reported treatment responses as the proportion of patients reaching a specified definition of response. Studies examining combinations of medications were excluded from the analysis, as were studies with indefinite diagnoses of Cushing’s disease.
For medications other than mifepristone, studies had to report the proportion of patients with normalized urinary free cortisol (UFC), midnight salivary cortisol or midnight serum cortisol.
The studies were scored according to the Grading of Recommendations, Assessment, Development and Evaluation (GRADE) system for rating quality of evidence.
Ten of the 15 included studies reported outcomes specifically for patients with Cushing’s disease and the remaining five included patients with other forms of Cushing’s syndrome.
The researchers reported that:
But the researchers urged caution in comparing the drugs, citing the variability in the study designs and patient selection endpoints, among other limitations in the research literature.
“The wide variation in the time-frames over which response to treatment was measured makes comparison a challenge,” they wrote. “Comparison of response rates reported in the included studies is also complicated by the variation in methodology used to assess response.”
They noted that well-designed clinical trials are needed to determine which drugs or drug combinations are most effective in the treatment of Cushing’s disease patients.
“Combinations of medical therapies with different modes of action might aid in optimizing the balance of efficacy and safety,” they wrote. “Investigational medications, such as bexarotene, LC1699 and retinoic acid, may help to expand the range of future therapeutic options.”
Maria Fleseriu, MD, who was not involved in the review, agreed that more drug treatments are needed. But she added that Cushing’s patients today have many more drug options than they did just a few years ago.
Fleseriu directs the Pituitary Center at Oregon Health & Science University, where she is an associate professor of medicine and endocrinology.
In a recently published analysis, Fleseriu wrote that pituitary-targeted medical therapies should soon play a more prominent role in treating Cushing’s disease, and may become first-line treatments when surgery fails or is contraindicated.
“We now have one drug approved for Cushing’s and another approved for diabetes symptoms associated with the disease,” she told MedPage Today. “We are moving forward, but we are not where we would like to be. Combination therapy is probably where we are heading, but further studies are needed.”
Financial support for this research was provided by Novartis Pharmaceuticals.
Researcher Monica Gadelha reports receiving speaker fees and participating on advisory boards for Novartis. Gadelha and co-author Leonardo Vieira Neto were investigators in Novartis’ clinical trials of pasireotide.
From http://www.medpagetoday.com/Endocrinology/GeneralEndocrinology/42043
Filed under: Clinical trials, Cushing's, pituitary | Tagged: 24-hour urine free cortisol test, adenoma, cabergoline, cardiovascular, clinical trial, diabetes mellitus, Dr. Maria Fleseriu, drugs, ketoconazole, Metyrapone, midnight serum cortisol, mifepristone, mitotane, nephrolithiasis, Novartis, Oregon Health & Science University, osteoporosis, pasireotide, pituitary, RU-486, salivary cortisol, Signifor, tumor, UFC | Leave a comment »
Greg Dohler/The Gazette
Lanham resident Stacey L. Hardy, a survivor of Cushing’s disease, will speak about her experience with the pituitary disorder at an upcoming event at Johns Hopkins Hospital.
Stacy L. Hardy of Lanham described herself as athletic, which is why she became concerned when in 1996 she mysteriously gained 240 pounds that took five doctors 14 years to determine she had a potentially fatal disease.
Now Hardy said she wants to raise awareness among others who may unknowingly have Cushing’s disease, but are unaware of the symptoms and treatment.
It wasn’t until 2010 that Hardy, now 43, was diagnosed with the disease, a rare disorder that causes the body to release too much cortisol, the body’s stress or “fight or flight” hormone, said Gary Wand, a pituitary gland specialist at The Johns Hopkins Hospital in Baltimore.
Excess cortisol causes weight gain, especially in the stomach, extreme fatigue, muscle aches, anxiety and depression, he said.
“I didn’t even know what Cushing’s was. I was ready to just live with [the symptoms],” Hardy said, adding that by the time she was diagnosed she felt so tired she could barely move.
At 5 feet, 4 inches tall, Hardy said she reached 365 pounds during her struggle with the disease.
“We knew something for a while wasn’t right, but I never thought it would be something like that,” said Hardy’s daughter, Paij Hardy, 21, a student at Baltimore City Community College.
Just three out of every one million people are diagnosed with Cushing’s each year, said Wand, who estimates he sees 30 patients per year worldwide.
In 2011, Hardy underwent 16 hours of surgery at Sinai Hospital in Baltimore to remove four tumors from her pituitary gland, located at the base of the brain that controls the release of cortisol.
Today, she is 100 pounds lighter, with the weight still rapidly coming off, and said she is determined to serve as a lifelong support and education source for her fellow “cushies” — others with Cushing’s disease.
Hardy will speak Saturday at the Johns Hopkins Pituitary Gland Center’s fifth annual Patient Education Day, an event to raise awareness about the disease, Wand said.
Since the pituitary gland is the size of a kidney bean, Hardy underwent several brain scans before doctors, who previously suggested she might have leukemia or needed to diet and exercise more, could tell there were tumors on her gland, she said.
Hardy’s experience with delayed diagnosis and misdiagnosis is not unique, Wand said.
Cushing’s is a “subtle” disease, which is difficult to diagnose, and not everyone exhibits the same symptoms, he said.
If left untreated for more than a decade, the disease is fatal, but removing the pituitary gland tumors has proved extremely successful, Wand said.
“I’m evidence that there’s help out there,” Hardy said. “I can move. I can almost run. I can bend over and pick up a box. Oh my goodness, I can dance.”
Filed under: Cushing's, Inspiration, Meetings and Conferences, pituitary, Rare Diseases | Tagged: anxiety, Baltimore, Baltimore City Community College, cortisol, Cushing Disease, Cushing Syndrome, depression, Dr. Gary Wand, fatigue, Johns Hopkins Hospital, muscles, Patient Education Day, pituitary, Pituitary Day, Pituitary gland, Sinai Hospital, surgery, weight | Leave a comment »
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