Day 14, Cushing’s Awareness Challenge 2016

Way back when we first got married, my husband thought we might have a big family with a lot of kids.  He was from a family of 6 siblings, so that’s what he was accustomed to.  I am an only child so I wasn’t sure about having so many.

I needn’t have worried.

In January, 1974 I had a miscarriage.  I was devastated. My father revealed that my mother had also had a miscarriage.  I had no idea.

At some point after this I tried fertility drugs.  Clomid and another drug.  One or both drugs made me very angry/depressed/bitchy (one dwarf I left off the image)  Little did I know that these meds were a waste of time.

Eventually,  I did get pregnant and our wonderful son, Michael was born.  It wasn’t until he was seven that I was finally, actually diagnosed with Cushing’s.

When I had my early Cushing’s symptoms, I thought I was pregnant again but it was not to be.

I’ll never forget the fall when he was in second grade.  He was leaving for school and I said goodbye to him.  I knew I was going into NIH that day for at least 6 weeks and my future was very iffy.  The night before, I had signed my will – just in case.  He just turned and headed off with his friends…and I felt a little betrayed.

Michael wrote this paper on Cushing’s when he was in the 7th grade. From the quality of the pages, he typed this on typing paper – no computers yet!

Click on each page to enlarge.

When Michael started having headache issues in middle school, I had him tested for Cushing’s.  I had no idea yet if it could be familial but I wasn’t taking any chances.  It turned out that my father had also had some unnamed endocrine issues.  Hmmm…

I survived my time and surgery at NIH and Michael grew up to be a wonderful young man, if an only child.  🙂

After I survived kidney cancer (Day Twelve, Cushing’s Awareness Challenge 2015) Michael and I went zip-lining – a goal of mine after surviving that surgery.  This photo was taken in a treetop restaurant in Belize.

For the mathematically inclined, this is his blog.  Xor’s Hammer.  I understand none of it.  He also has a page of Math and Music, which I also don’t understand.

I know it doesn’t fit into a Cushing’s awareness post but just because I’m a very proud mama – Michael got a PhD in math from Cornell and his thesis was Using Tree Automata to Investigate Intuitionistic Propositional Logic

 

proud-mom

 

Straight Talk Cushing’s

awareness

 

On April 8th, people from around the world unite in support of Cushing’s Disease Awareness Day.

In this animated video, learn more about Cushing’s disease, including the mechanism of disease, clinical manifestations, symptoms and diagnosis challenges.

VIDEO: Cushing’s syndrome still difficult to diagnose

endo2016

 

In this video exclusive, Hershel Raff, PhD, professor of medicine, surgery and physiology at the Medical College of Wisconsin and scientific director and clinical research supervisor at Aurora St. Luke’s Medical Center, discusses his presentation on the laboratory diagnosis of Cushing’s syndrome.

According to Raff, Cushing’s syndrome is the most enigmatic disease in endocrinology and one of the most difficult to diagnose.

The use of late-night salivary cortisol for diagnosis has recently become popular although the test has been around for many decades. Patients can send samples by mail to have their cortisol measured in a laboratory to determine whether they have Cushing’s. According to Raff, the test is about 95% accurate in making a diagnosis.

View video at http://www.healio.com/endocrinology/adrenal/news/online/%7B5545e9a6-7475-454a-94d5-3994ac8beec5%7D/video-cushings-syndrome-still-difficult-to-diagnose

Morning Cortisol Rules Out Adrenal Insufficiency

endo2016

 

Key clinical point: Skip ACTH stimulation if morning serum cortisol is above 11.1 mcg/dL.

Major finding: A morning serum cortisol above 11.1 mcg/dL is a test of adrenal function with 99% sensitivity.

Data source: Review of 3,300 adrenal insufficiency work-ups.

Disclosures: There was no outside funding for the work, and the investigators had no disclosures.

BOSTON – A random morning serum cortisol above 11.1 mcg/dL safely rules out adrenal insufficiency in both inpatients and outpatients, according to a review of 3,300 adrenal insufficiency work-ups at the Edinburgh Centre for Endocrinology and Diabetes.

The finding could help eliminate the cost and hassle of unnecessary adrenocorticotropic hormone (ACTH) stimulation tests; the investigators estimated that the cut point would eliminate almost half of them without any ill effects. “You can be very confident that patients aren’t insufficient if they are above that line,” with more than 99% sensitivity. If they are below it, “they may be normal, and they may be abnormal.” Below 1.8 mcg/dL, adrenal insufficiency is almost certain, but between the cutoffs, ACTH stimulation is necessary, said lead investigator Dr. Scott Mackenzie, a trainee at the center.

In short, “basal serum cortisol as a screening test … offers a convenient and accessible means of identifying patients who require further assessment,” he said at the annual meeting of the Endocrine Society.

Similar cut points have been suggested by previous studies, but the Scottish investigation is the first to validate its findings both inside and outside of the hospital.

The team arrived at the 11.1 mcg/dL morning cortisol cut point by comparing basal cortisol levels and synacthen results in 1,628 outpatients. They predefined a sensitivity of more than 99% for adrenal sufficiency to avoid missing anyone with true disease. The cut point’s predictive power was then validated in 875 outpatients and 797 inpatients. Morning basal cortisol levels proved superior to afternoon levels.

The investigators were thinking about cost-effectiveness, but they also wanted to increase screening. “We may be able to reduce the number of adrenal insufficiency cases we are missing because [primary care is] reluctant to send people to the clinic for synacthen tests” due to the cost and inconvenience. As with many locations in the United States, “our practice is to do [ACTH on] everyone.” If there was “a quick and easy 9 a.m. blood test” instead, it would help, Dr. Mackenzie said.

Adrenal insufficiency was on the differential for a wide variety of reasons, including hypogonadism, pituitary issues, prolactinemia, fatigue, hypoglycemia, postural hypotension, and hyponatremia. Most of the patients were middle aged, and they were about evenly split between men and women.

There was no outside funding for the work, and the investigators had no disclosures.

aotto@frontlinemedcom.com

From http://www.clinicalendocrinologynews.com/specialty-focus/pituitary-thyroid-adrenal-disorders/single-article-page/morning-cortisol-rules-out-adrenal-insufficiency/af59bab2bb014ca9d352c792f9d41653.html

Mortality in adults with hypopituitarism

endo2016

 

April 04, 2016

Oral Session: Pituitary Patients and Outcomes

Mortality in adults with hypopituitarism: A systematic review and meta-analysis

S Jasim, F Alahdab, A Ahmed, S Tamhane, TB Nippoldt, H Murad

Summary: The purpose of this study was to review existing literature on mortality in hypopituitarism and determine possible predictors of this outcome to help create clinical practice guidelines. Premature mortality may be associated with hypopituitarism, and the risk of hypopituitarism is higher in women and patients diagnosed at a younger age, concluded researchers.

Methods:

  • Researchers conducted a comprehensive search of multiple databases: MEDLINE, EMBASE, Cochrane Central Register of Controlled Trials, Cochrane Database of Systematic Reviews, and Scopus.
  • Using a predefined protocol, they selected eligible studies that evaluated patients with hypopituitarism and reported mortality estimates.
  • Reviewers, independently and in duplicate, extracted data and assessed the risk of bias.

Results:

  • A total of 12 studies (published 1996 to 2015) that reported on 23515 patients were included in this review.
  • Compared to the general population, hypopituitarism was associated with an overall excess mortality (weighted SMR of 1.52; 95% CI: 1.14- 2.04), P=0.000.
  • The following were independent risk factors for increased mortality: younger age at diagnosis, female gender, diagnosis of craniopharyngioma, radiation therapy, transcranial surgery, DI, and hypogonadism.

From http://www.mdlinx.com/endocrinology/conference-abstract.cfm/ZZ6AA1CEC190F5428EA690616DAA054518/57001/?utm_source=confcoveragenl&utm_medium=newsletter&utm_content=abstract-list&utm_campaign=abstract-ENDO2016&nonus=0