Interview with Deborah March 30, 2016

Deborah has many symptoms but is not yet diagnosed.

interview

Deborah will be our guest in an interview on BlogTalk Radio  Wednesday, March 30 at 6:00 PM eastern.  The Call-In number for questions or comments is (845) 241-9850.

The archived interview will be available after 7:00 PM Eastern through iTunes Podcasts (Cushie Chats) or BlogTalkRadio.  While you’re waiting, there are currently 89 other past interviews to listen to!

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Deborah’s Bio:

Hello all,

I do not know where to begin. For many years I have been struggling with these symptoms. I have proximal weakness, intolerance to stress, blood pressure fluctuations, hyperpigmentation, reactive hypoglycemia, sweating, severe dehydration, very bad confusion, vision, memory problems, physical body changes (hump, bruises), carb intolerance, and inability to exercise.

My endocrinologist did a workup for Cushing’s disease and the midnight saliva test was high. She brushed it off as “stress”. I am seeing a doctor now that says I have POTS and Dysautonomia. My doctor says I have inappropriate adrenaline rushes.

My body is falling apart because I haven’t found a doctor who will take my symptoms and test results serious. I would like to talk to others who are having trouble getting diagnosed and also to those who have gotten diagnosed who have a good doctor.

God Bless and Thank You,
Deborah

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In Production: Quick and Cheap Bedside Test for Cortisol Uses Smartphone

An innovative method of measuring the stress hormone cortisol is being developed by researchers in Utah. Requiring just a simple kit and a smartphone to read results, this new approach should allow quick, affordable, and accurate testing of cortisol levels, enabling rapid diagnosis of adrenal diseases, the investigators say.

“A lab charges about $25 to $50 for a quantitative salivary cortisol test and has a turnaround time of days to a week,” said lead researcher Joel Ehrenkranz, MD, director of diabetes and endocrinology at Intermountain Medical Center, Murray, Utah. “This test, taken in a medical office or at home, will cost less than $5 and take less than 10 minutes,” he noted.

Dr. Ehrenkranz reported the details of the new test kit, developed at his institution, at ICE/ENDO 2014 week. He said he and his fellow researchers are now collating clinical data for a Food and Drug Administration (FDA) submission and hope to gain approval of the test as a class 2 medical device in the United States in 2015.

Chair of the session, Jeremy Tomlinson, MD, of University of Birmingham, United Kingdom, said the new approach employs “great technology and is an interesting innovation, but there are a few concerns. For example, how well will it perform against the state-of-the-art technique for measuring salivary cortisol, which is mass spectrometry — is it as sensitive?”

Also there is a possibility the immunoassay in the new test will cross react with another steroid hormone, prednisolone, that people might be taking for a whole range of inflammatory conditions, so “you would want to make sure it’s measuring what you want it to,” he noted.

And finally, there is the question of exactly how this would be used.

Cortisol levels are needed when conditions are suspected where too much or too little cortisol is produced, but the diagnosis for most of these doesn’t really need to be immediate, Dr. Tomlinson explained to Medscape Medical News. However, he conceded there might be a role for the assay in patients presenting to the emergency room or in developing nations.

No More Presumptive Treatment of Adrenal Insufficiency

At the meeting, Dr. Ehrenkranz said that adrenal diseases are commonly overlooked because current methods of measuring salivary cortisol require instrumentation and technical personnel and so are costly and unable to deliver timely results.

He noted also that a stint in the developing world convinced him that a simpler test was needed, so he and his colleagues set about developing an assay that would be inexpensive and easy to perform — they came up with disposable cortisol immunoassay strips containing a glass fiber element with colloidal gold-labeled murine anticortisol antibodies and a saliva collection pad.

The person being tested inserts a strawlike saliva collector under the tongue, which wicks the saliva to the immunoassay test strip housed in a cassette, which is then inserted into a reader in the device.

“The device…includes a case, a light pipe, and a lens and costs about a dollar to make. There is no battery power, and it’s unbreakable, passive, and reusable,” Dr Ehrenkranz said.

Because of the physical properties of the gold nanoparticles, a smartphone flash can illuminate and camera-image the color generated by the colloidal gold-labeled anticortisol antibodies, he explained.

The color subsequently generated is “read” by an app on the smartphone to give a cortisol reading, based on an algorithm derived from observed vs reference salivary cortisol values. The R value of this curve was 0.996 for salivary cortisol in the range of 0.012-3.0 µg/dL, Dr Ehrenkranz noted.

The new technology can therefore measure cortisol in a range sufficient to diagnose adrenal insufficiency and hypercortisolism and monitor physiologic variations in cortisol concentration, he said.

And the software is “operating-system agnostic,” he added, meaning the device can be used on all platforms, including iOS, Android, Windows, and BlackBerry, and it has a universal form factor that works with all smartphones.

“Measuring salivary cortisol at the point of care in 5 minutes using an inexpensive immunochromatographic assay, reader, and smartphone may obviate the need to presumptively treat patients for adrenal insufficiency and makes cortisol assays available to regions of the world that currently lack access to this diagnostic test,” he concluded.

Test of Use in Emergency Room, in Developing Countries

Dr. Tomlinson explained that diagnosis of Cushing’s syndrome — caused either by tumors of the pituitary gland producing too much ACTH or tumors of the adrenal gland producing too much cortisol — or alternatively, diagnosis of conditions where it’s thought too little cortisol is being secreted, such as Addison’s disease — an autoimmune process whereby the adrenal gland is destroyed — are not conditions “you necessarily have to diagnose in a few minutes by the bedside,” and therefore it is better to use the “gold standard” of diagnosis, mass spectrometry, in these cases.

But the new test “might be of use in determining whether people have enough of their own natural corticosteroid, in terms of deciding whether you need to give supplemental cortisol to people in an emergency situation,” he explained.

This could include patients presenting with suspected or underlying pituitary or adrenal disease or in people who have been on large doses of steroids who have then stopped taking them, so there will be a resulting suppression of their natural steroid production, he noted.

“That’s not an uncommon situation that we see in the emergency room. At the moment, if there’s suspicion, we might take a test but it takes a day or 2 to come back from the laboratory, and in the meantime we will give patients [presumptive] steroids. But you could do this test by the bedside,” he acknowledged.

And in developing countries, use of this test “is feasible, where cost comes into the equation and you might not have access to mass spectrometry; this could be an alternative and would help you to exclude or make these diagnoses,” he concluded.

This study was privately funded. Dr. Ehrenkranz and colleagues report no relevant financial relationships.

Joint Meeting of the International Society of Endocrinology and the Endocrine Society: ICE/ENDO 2014; June 24, 2014. Abstract OR48-2

From http://www.medscape.com/viewarticle/827580

Does a Normal Urine Free Cortisol Result Rule out Cushing’s Syndrome?

Endocrine Society’s 97th Annual Meeting and Expo, March 5–8, 2015 – San Diego
SAT-384:
Does a Normal Urine Free Cortisol Result Rule out Cushing’s Syndrome?
1 and 2

  • 1Eunice Kennedy Shriver National Institute of Child Health and Human Development, National Institutes of Health, Bethesda, MD
  • 2National Institutes of Health, Bethesda, MD
Presentation Number: SAT-384
Date of Presentation: March 7, 2015
Abstract:Background: Urine free cortisol (UFC) has been traditionally used as one of the first steps in the diagnostic evaluation of Cushing’s syndrome (CS) (1). False positive results, especially values less than twice the upper limit of normal (ULN), can be seen in uncontrolled diabetes, obesity, depression, alcoholism, increased fluid intake, overcollection and stress. False negative results have also been reported with incomplete collection, in mild or cyclic CS and in patients with renal insufficiency (2-3). We evaluated the diagnostic accuracy of UFC and 24-hour urine 17-hydroxycorticosteroids (17OHCS) in patients with CS.Methods: Retrospective study of all CS patients evaluated at the National Institutes of Health (NIH) from 2009 to 2014. Screening tests used for CS included UFC, 17OHCS, late night salivary cortisol (LNSC), midnight serum cortisol and low dose (1mg overnight or 2-day 2mg/day) dexamethasone suppression test (DST). Values above reference range for UFC, 17OHCS and LNSC, a midnight serum cortisol ≥ 7.5 mcg/dL, and post-dexamethasone cortisol values ≥ 1.8 mcg/dL were considered abnormal. Hourly 24-hour sampling for cortisol was performed in a few cases with a mild clinical phenotype and equivocal test results. UFC was measured using liquid chromatography/tandem mass spectrometry (LC-MS/MS). 17OHCS was measured using colorimetric methodology with Porter-Silber reaction (reported as mg/g of creatinine). Mean of the first two UFC and 17OHCS values (appropriate collection by urine volume and creatinine) obtained within 30 days of initial NIH presentation were used for the purpose of this study.

Results: Seventy-two patients were diagnosed with CS (aged 18-77 years, 51 females). Of these, 51 had Cushing’s disease (CD), 10 had ectopic CS while 2 had an adrenal source of Cushing’s based on pathology. Biochemical tests including inferior petrosal sinus sampling (IPSS) suggested ectopic CS but no tumor was found (occult) in 6 patients. IPSS was indicative of a pituitary source in 2 patients with failed transsphenoidal surgery while one patient did not complete evaluation for ACTH-dependent CS. UFC results were available in all, 17OHCS in 70, LNSC in 21, midnight serum cortisol in 68 and DST results in 37 patients. UFC was falsely normal in six and only minimally elevated (< 2 x ULN) in 13 patients (normal renal function, no history of cyclicity, all had CD). Of these 19 patients, 24h 17OHCS was abnormal in all, LNSC was abnormal in 12, midnight serum cortisol was abnormal in 18 and DST was abnormal in 12 patients. Hourly 24-hour sampling for cortisol performed in 3 of these patients revealed abnormal nadir (> 7.5 mcg/dL) and mean daily serum cortisol (> 9 mcg/dL) levels.

Conclusion: UFC can be falsely normal or only minimally elevated in mild CS. Multiple collections and use of complimentary screening tests including 24-hour urine 17OHCS and LNSC can help make a diagnosis and prevent delay in treatment.

(1) Newell-Price J, et al. Cushing’s syndrome. Lancet. 2006;367(9522):1605-17.  (2) Alexandraki KI, et al. Is urinary free cortisol of value in the diagnosis of Cushing’s syndrome. Curr Opin Endocrinol Diabetes Obes. 2011;18:259–63.  (3) Kidambi S, et al. Limitations of nocturnal salivary cortisol and urine free cortisol in the diagnosis of mild Cushing’s syndrome. Eur J Endocrinol. 2007;157(6):725-31

Nothing to Disclose: STS, LKN

Sources of Research Support: This research was in part supported by the intramural research program of NICHD/NIH

Read the entire article at http://press.endocrine.org/doi/abs/10.1210/endo-meetings.2015.ahpaa.9.sat-384

Experts recommend tumor removal as first-line treatment for Cushing’s syndrome

The Endocrine Society today issued a Clinical Practice Guideline (CPG) on strategies for treating Cushing’s syndrome, a condition caused by overexposure to the hormone cortisol.

The CPG, entitled “Treatment of Cushing’s Syndrome: An Endocrine Society Clinical Practice Guideline,” was published online and will appear in the August 2015 print issue of the Journal of Clinical Endocrinology and Metabolism (JCEM), a publication of the Endocrine Society.

Cushing’s syndrome occurs when a person has excess cortisol in the blood for an extended period, according to the Hormone Health Network. When it is present in normal amounts, cortisol is involved in the body’s response to stress, maintains blood pressure and cardiovascular function, keeps the immune system in check, and converts fat, carbohydrates and proteins into energy. Chronic overexposure to the hormone can contribute to the development of cardiovascular disease, infections and blood clots in veins.

People who take cortisol-like medications such as prednisone to treat inflammatory conditions, including asthma and rheumatoid arthritis, can develop Cushing’s syndrome. The high cortisol levels return to normal when they stop taking the medication. This is called exogenous Cushing’s syndrome.

In other cases, tumors found on the adrenal or pituitary glands or elsewhere in the body cause the overproduction of cortisol and lead to the development of Cushing’s syndrome. The Clinical Practice Guidelines focus on this form of the condition, known as endogenous Cushing’s syndrome.

“People who have active Cushing’s syndrome face a greater risk of death – anywhere from nearly twice as high to nearly five times higher – than the general population,” said Lynnette K. Nieman, MD, of the National Institutes of Health’s Eunice Kennedy Shriver National Institute of Child Health and Human Development in Bethesda, MD, and chair of the task force that authored the guideline. “To reduce the risk of fatal cardiovascular disease, infections or blood clots, it is critical to identify the cause of the Cushing’s syndrome and restore cortisol levels to the normal range.”

In the CPG, the Endocrine Society recommends that the first-line treatment for endogenous Cushing’s syndrome be the removal of the tumor unless surgery is not possible or unlikely to address the excess cortisol. Surgical removal of the tumor is optimal because it leaves intact the hypothalamic-pituitary-adrenal axis, which is integral to the body’s central stress response.

Other recommendations from the CPG include:

  • Tumors should be removed by experienced surgeons in the following situations:— A tumor has formed on one or both of the two adrenal glands.— A tumor that secretes adrenocorticotropic hormone (ACTH) – the hormone that signals the adrenal glands to produce cortisol – has formed somewhere in the body other than the adrenal or pituitary gland.

    — A tumor has formed on the pituitary gland itself.

  • Patients who continue to have high levels of cortisol in the blood after surgery should undergo additional treatment.
  • People who had an ACTH-producing tumor should be screened regularly for the rest of their lives for high cortisol levels to spot recurrences.
  • If patients’ cortisol levels are too low following surgery, they should receive glucocorticoid replacement medications and be educated about adrenal insufficiency, a condition where the adrenal glands produce too little cortisol. This condition often resolves in 1-2 years.
  • Morning cortisol and/or ACTH stimulation tests, or insulin-induced hypoglycemia, can be used to test for the recovery of the hypothalamic-pituitary-adrenal axis in people who have low cortisol levels after surgery. Once the tests results return to normal, glucocorticoid replacement can be stopped.
  • People who have undergone pituitary surgery should be re-evaluated for other pituitary hormone deficiencies during the post-operative period.
  • Patients who have a pituitary tumor and have undergone surgery to remove both adrenal glands should be regularly evaluated for tumor progression using pituitary MRIs and tests for ACTH levels.
  • Radiation therapy may be used to treat a pituitary tumor, especially if it is growing. While awaiting the effect of radiation, which may take months to years, treatment with medication is advised.
  • To assess the effect of radiation therapy, the patient’s cortisol levels should be measured at 6- to 12-month intervals.
  • Medications may be used to control cortisol levels as a second-line treatment after surgery for a pituitary gland tumor, as a primary treatment for ACTH-secreting tumors that have spread to other parts of the body or suspected ACTH-secreting tumors that cannot be detected on scans. Medications also can be used as adjunctive treatment to reduce cortisol levels in people with adrenal cortical carcinoma, a rare condition where a cancerous growth develops in the adrenal gland.
  • People with Cushing’s syndrome should be treated for conditions associated with the disease, such as cardiovascular disease risk factors, osteoporosis and psychiatric symptoms.
  • Patients should be tested for recurrence throughout their lives except in cases where the person had a benign adrenal tumor removed.
  • Patients should undergo urgent treatment within 24 to 72 hours of detecting excess cortisol if life-threatening complications such as serious infection, pulmonary thromboembolism, cardiovascular complications and acute psychosis are present.

More information: The Hormone Health Network offers resources on Cushing’s syndrome at www.hormone.org/questions-and-answers/2012/cushing-syndrome

Hydrocortisone Replacement Patient Information

steroids
Patient Information

What is Hydrocortisone?
Hydrocortisone is a steriod hormone produced by the adrenal gland.  It regulates many of the bodies functions and is essential for life.
Hydrocortisone is taken as a replacement for the natural hormone where this is deficient, either because of pituitary deficieny of ACTH (the hormone that stimulates the production of hydrocortisone by the adrenal gland) or failure of hydrocortisone production by the adrenal gland.

How do I take it?
A common dose is 15-20mg orally split over two or three times daily, and depending on your individual Endocrinologist’s recommendations, e.g., 10mg before rising, 5mg at mid-day and 5mg at 4 p.m.

When would I need to take more hydrocortisone?

If you become ill then the body would naturally increase the output of steroid from your adrenals.  Therefore if you are taking replacement steroid (hydrocortisone) it is essential to mimic the natural response by increasing your dose appropriately.

How can I let others know I take replacement hydrocortisone?
When you are prescribed your medication you will be given a ‘blue steroid card’ from the hospital to carry.  You should also purchase and wear a medical necklace or bracelet, such as MedicAlert, to show your Cortisol replacement therapy.

Emergency Injections – should I have these at home?
It is advisable for all patients on hydrocortisone replacement to have a 100mg injection pack at home and for them or their partners to be taught how to administer it.  If you don’t have one of these already, you can ask your GP or endocrinologist if they will prescribe this for you.  Please check regularly that these preparations are not expired.  Some endocrine clinics will help to show you how to inject in an emergency.

When do I know that I would need an emergency injection?

If you cannot absorb your tablets, or your usual replacement wasn’t sufficient for an acute shock or illness, then gradually or perhaps quite quickly you would feel weak, sickly and light-headed.

Recommendations for changes in oral dose ‘The Sick Rules’
If you become unwell you should take additional hydrocortisone. The amount depends on how unwell you are and the type of illness. The pituitary foundation provides some sensible examples:

If a patient is unwell they should take additional hydrocortisone. The amount depends on how unwell they are and the type of illness. Some examples:

Situtation  Increase in dose Duration  Emergency?
Cold without fever   none
Fever, flu, infection         double dose duration of illness see GP after 48 hours
Vomiting > once, diarrhoea and severe illness Emergency 100mg injection if extra dose of 10-20mg cannot be kept down restart usual dose once stable Phone GP or go to A&E. Administer injection prior to this if emergency pack available (but still seek help)
Minor surgical procedure e.g. tooth extraction     20mg hydrocortisone before procedure resume on usual dose immediately afterward
Minor operation e.g. hernia repair  100mg im every 6 hours for 24 hours  resume on usual dose immediately afterward
Major operation e.g. abdomen or chest 100mg im injection every 6 hours for 24 -72 hours and eating and drinking reduce rapidly to usual dose tell the surgeon and anaesthetist before the operation
Endoscopy Double the dose the day before during bowel prep. For colonoscopy 100mg im before procedure take usual dose on the morning of the procedure drink lots of water to prevent dehydration. Inform your doctor.
Cystoscopy Double dose on the day of procedure. resume as normal inform your doctor.
Severe shock e.g. bereavment or road traffic accident 20mg as tablet or 100mg intramuscular injection See GP or hospital for further advice Sudden and severe shock may be classed as an emergency – seek advice
Long haul flight > 12 hours double dose on the day of the flight extra dose every 6-8 hours when the day is legnthened. Usual regimen in timing with sleep / wake cycle when day is shortened. Speak to your consultant before travel.
General stress, exams etc  not usually required ask GP if concerned

How do I cope if I’m travelling away from home?

You should travel with a 100mg injection kit  in case of emergency.  This injection should be placed in a small cool bag, labelled with your name and kept with you at all times during your journey. You should ask your GP or endocrinologist for a letter about your injection kit, medication and your doses prescribed.  This letter is essential to travel through security checks and will be helpful should you become unwell and have to see a doctor. It is wise to take an extra two weeks supply of hydrocortisone tablets with you in case you need to increase your usual dose whilst away.  All medication should be kept in your hand luggage.

Printable patient information

From http://www.imperialendo.com/for-doctors/hydrocortisone-replacement/hydrocortisone-replacement-patient-information

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