Friday, April 26, 2013

Ectopic Cushing's Syndrome Secondary to Pulmonary Carcinoid Tumor

Shahriar Hashemzadeh, MD, Atabak Asvadi Kermani, MD, Akbar Ali-Asgharzadeh, MD, Moneireh Halimi, MD, Mina Soleimani, MD, Amirhosein Ladan, MD

The Annals of Thoracic Surgery, Volume 95, Issue 5, May 2013, Pages 1797-1799

Adrenocorticotropic hormone (ACTH) overproduction within the pituitary gland or ectopically leads to hypercortisolism.

In this study a case of Cushing's syndrome caused by an ectopic ACTH-secreting carcinoid tumor in lung is discussed, as are the available diagnostic procedures.

The patient was a 28-year-old woman with clinical features starting about 6 months previously. The results of her biochemical tests suggested ectopic Cushing's syndrome.

Full-body computed tomography revealed a single nodule in the inferior lobe of the right lung.

After removal of the nodule, the patient's symptoms subsided clinically, and laboratory tests confirmed remission of the hypercortisolism.

View Full Text

Wednesday, April 24, 2013

Adult growth hormone deficiency – benefits, side effects, and risks of growth hormone replacement

Frontiers in Endocrinology, 04/24/2013  

Reed ML et al. – Deficiency of growth hormone (GH) in adults results in a syndrome characterized by decreased muscle mass and exercise capacity, increased visceral fat, impaired quality of life, unfavorable alterations in lipid profile and markers of cardiovascular risk, decrease in bone mass and integrity and increased mortality.

The potential of GH to act as a mitogen has resulted in concern over the possibility of increased de novo tumors or recurrence of pre–existing malignancies in individuals treated with GH.

Though studies of adults who received GHRT in childhood have produced conflicting reports in this regard, long term surveillance of adult GHRT has not demonstrated increased cancer risk or mortality.

Read more:

Monday, April 22, 2013

A Show About Cushing's on TV

From my email:

We haven’t personally spoken for a while, but I wanted to share one of our recent efforts to increase awareness about Cushing’s.

To help spread the word about Cushing’s syndrome, we [Corcept Theraputics, makers of Korlym] coordinated with Lifetime TV to develop a short segment about Cushing’s syndrome. We were able to supply 2 guests to help bring the disease to life. Sara Cook, a patient with Cushing’s disease shares her personal story and her success with a medicine that blocks the activity of excess cortisol and Dr. James Findling shares his personal medical expertise. The Balancing Act segment will air tomorrow April 23 [note: it's showing several other times, as well.  Check your listings!] on Lifetime TV at 7:00 am EST and 7:00 am PST. After it airs, the episode will also be available on the Balancing Act website, . You can see the promotional segment at the following link: . The segment will initially be seen by the daily 500,000 mostly female audience… helping to spread the word about Cushing’s. It will get further viewership online and through online linking. We were hoping to have the segment air on Cushing’s Awareness Day, but they weren’t able to accommodate, so we got as close as we could.

In addition to the audience viewership, we hope this segment helps demonstrate our support and commitment to the Cushing’s community.

Sunday, April 21, 2013

Cushing’s syndrome increased risk for coronary arterial atherosclerosis

cushings-ladyNeary NM. Clin Endocrinol Metab. 2013;doi:10.1210/jc.2012-3754.

In a recent study supported by the NIH, researchers determined that patients with Cushing’s syndrome have a greater risk for developing coronary arterial atherosclerosis, increasing their rate of cardiovascular morbidity. These findings were published in the Journal of Clinical Endocrinology & Metabolism.

The researchers conducted a prospective case-control study of 15 consecutive patients with adrenocorticotropic hormone (ACTH)-dependent Cushing’s syndrome who were matched with 15 controls (aged 32 to 66 years) with at least one risk factor for cardiac disease (ie, diabetes, hypertension, hyperlipidemia, family history of early-onset coronary artery disease and previous or current smoking).

Researchers used a multidetector CT (MDCT) coronary angiogram scan to measure calcified and noncalcified coronary plaque volume and Agatston scores. Additional variables included fasting lipids, BP, history of hypertension or diabetes and 24-hour urine free cortisol excretion.

According to data, patients with Cushing’s syndrome had significantly greater noncalcified plaque volume and Agatston scores compared with controls (noncalcified plaque volume median [interquartile ranges]: 49.5 vs. 17.9,P<.001; Agatston score: 70.6 vs. 0, P<.05).

Patients with Cushing’s syndrome also demonstrated higher systolic (143 mm Hg) and diastolic (86 mm Hg) BP compared with controls (systolic: 134 mm Hg, diastolic: 76 mm Hg).

The limitations of the study include the small cohort of patients and potential selection bias due to ectopic ACTH secretion. However, the researchers wrote that these findings demonstrate a significant difference between the two groups included in the study.

“Overall, the findings point to the possible causes of cardiovascular morbidity in patients treated with exogenous steroids and indicate the need for further studies of that population,” they wrote.

Disclosure: The researchers report no relevant financial disclosures.
Alice C. Levine, MD Alice C. Levine
  • It has long been recognized that endogenous hypercortisolism (Cushing's syndrome) and administration of supraphysiologic doses of glucocorticoids are associated with increased mortality, primarily due to cardiovascular disease. Excess glucocorticoids induce all of the features of the metabolic syndrome including obesity with central weight gain, hypertension, impaired glucose tolerance/diabetes mellitus and dyslipidemia, all of which increase cardiovascular risk. In this small but well-designed study, the authors attempt to determine whether excess glucocorticoids have a direct adverse effect on the coronary vasculature. Utilizing multidetector computerized tomographic (MDCT) coronary angiography, a validated noninvasive method of assessing calcified and noncalcified coronary plaques, they compared measurements of coronary plaques (Agatston score) in 15 patients with ACTH-dependent Cushing's syndrome (CS) vs. 15 age-, sex- and body weight-matched controls with at least one risk factor for cardiac disease. They found significantly greater coronary calcifications and noncalcified coronary plaque volumes in patients with active or previous hypercortisolism.There are obvious limitations to the study; most notably the small sample size, the predominance of patients with CS due to ectopic ACTH (14/15) and significantly more hypertension in the CS vs. the control group. However, other than the HTN, the groups were well-matched and there was no statistical difference in the Framingham risk scores between groups. This is the first study to demonstrate direct effects of CS on coronary plaque burden.

    The findings, while unsurprising, underscore several important features of CS which endocrinologists need to consider. Firstly, as there were no statistical differences in plaque burden in patients with CS who were eucortisolemic (4/15) vs. those who were hypercortisolemic (11/15) at the time of study, the effects of CS on the coronary vasculature may persist even after biochemical cure. Many previous studies in larger cohorts have similarly demonstrated that the adverse effects of high glucocorticoids on cardiovascular, metabolic, psychiatric and neurocognitive function may be only partially reversible with disease remission. Secondly, even adjusting for all the confounding variables, hypercortisolism seems to be an independent risk factor for the development of coronary artery disease. The possible mechanisms underlying this observation are discussed and include increases in prothrombotic factors, circulating levels of vascular endothelial growth factor (VEGF) and angiogenesis. It is also plausible that cortisol increases atherosclerosis through the mineralocorticoid rather than the glucocorticoid receptor, suggesting the possibility of treating this particular deleterious effect of hypercortisolism with a mineralocorticoid-receptor blocker such as spironolactone.

    Within the CS group, no significant correlations were observed between the coronary plaque volumes and the duration of CS or urinary free cortisol (UFC) either at presentation or at the time of MDCT. Although this lack of correlation may also be attributable to the small sample size, it is well known that the onset of Cushing's syndrome is often insidious and it is impossible to pinpoint the exact duration of the abnormality in most patients. This study's finding of direct, adverse and possibly irreversible effects of hypercortisolism on the coronary vasculature should make endocrinologists even more vigilant in diagnosing and treating the disease as early as possible in its course.
    • Alice C. Levine, MD
    • Professor of medicine, division of endocrinology, diabetes and bone diseases
      Co-Director of The Adrenal Center
      Icahn School of Medicine
      Mount Sinai, New York, NY
  • Disclosures: Levine reports no relevant financial disclosures.

Friday, April 19, 2013

Cushing's on Capitol Hill

Earlier this year, I got this email:
Good morning Mary:
I hope everything is well.
I would like to invite you to join us at the Rare Disease Congressional Caucus briefing scheduled for April 2013. The final date is still being discussed but we are looking into two possible dates of either April 16th or April 18th. The meeting will take place in Washington, D.C. and will be attended by members of the Rare Disease Caucus including co-chairs Rep.Joseph Crowley and Rep. Leonard Lance.
As you may know Rare Disease Congressional Caucus is a forum for members of Congress to voice constituent concerns, share ideas, and build support for legislation that will improve the lives of people with rare diseases. The goal of the meeting in April is to educate the members of the Caucus about rare pituitary disorders, including Cushing’s Disease – area that has received little to no recognition among legislators. The meeting will serve as an opportunity to raise legislators’ awareness about multiple issues that patients with rare pituitary diseases, such as Cushing’s disease and Acromegaly, face in their everyday lives.
In preparation for the meeting we drafted a Resolution that addresses some of the key challenges for the patient community including long diagnostic delays, limited treatment options, difficulty finding physicians or treatment centers with expertise in their disease and as a result – a  diminished quality of life for patients. Would you be willing to have a look at the draft in the attachment and provide your feedback? Your opinion as a leader of the patient community and expert in Cushing’s disease would be highly appreciated.
I sincerely hope that you will be able to join us at the meeting to share your perspective and talk about the work that you are doing to help patient afflicted by Cushing’s disease live happier and healthier lives.
Please feel free to call or email anytime if you have questions or if you would like to discuss this further. I look forward to hearing back from you soon.
Attached to the email was the House of Congress Resolution.  Read it here.
I got back quite quickly and said that I would love to attend.  If it was on the 16th, I could go, no problem.  If it was the 18th, probably not because I had plane tickets that day to attend the Magic Foundation Conference in Las Vegas.
In late March, I needed to make my final decision on Las Vegas.  I had been waffling about that trip for a while since my husband had surprise triple bypass surgery in late January.  When I made the decision not to go, he still couldn’t drive or walk the dog – and I was just afraid to leave him alone for 5 days.
As it turned out, the date was a non-issue since the Congressional Caucus would be on the 16th.
April 15 was a terrible day as news of the Boston Marathon came in.  Security was stepped up in several cities, including Washington, DC.
I looked online to see if the Caucus would be cancelled and found out that the 16th was Emancipation Day in DC – and the main route that I would take to get there would be closed for a parade.
I was already getting very nervous about the whole thing and not knowing how to get there added to the stress levels.
I had my talk printed out with 3 different places to stop, depending on the time.
We left about 10AM for a noon meeting.  I’d decided to park at the train station and take a taxi to the Rayburn House Office Building.
When we got to the Rayburn Building, there was a long line of folks waiting to get in.  I don’t know if they only open the front door at certain times but when the line started to move, it went fairly quickly.  They took 5 at a time through security then we were on our own to find out where to go.
It turned out that our meeting room – 318 – is the room usually used for the Ways and Means Committee.  We got there just about 11:30.  Robert Knutzen from the Pituitary Network Association was already there as was Alexey from Novartis.  Alexey said “Mary?” and I said “Alexey?” and we introduced ourselves.  I already knew Bob from several past meetings so the four of us just chatted a bit while others started arriving.
I had brought quite a few Cushing’s brochures with me and had planned to hand them out to people but Julia from the RDLA (Rare Disease Legislative Advocates) showed me a table where I could leave them for folks to take on their own – and quite a few did.  If they read them, that’s another story!
Right around noontime, lots of people came in.  Some were staffers gathering information to take back to their offices, many others were from rare disease organizations, a few were legislators.  It was standing room only and we estimated there were maybe 120-140 people there.  Only two were known pituitary patients:  Bob with Acromegaly and me with Cushing’s.  Bob mentioned the statistic again “1 in 5″ so at least 24 others in that room should have had a pituitary tumor…
Representative Leonard Lance (NJ) spoke a bit about the need to recognize rare diseases in this country.  He mentioned that there were 7,000 rare diseases and it was important to focus on getting awareness for patients with them.  This Caucus focused on the pituitary, although only 2 pituitary diseases were represented.
Vijay Iyengar, Vice President the Rare Disease Franchise of Novartis oncology talked about their two drugs to either cure disease or improve quality of life through a  3-pronged approach:
  • Targeted research
  • Open collaboration
  • Patient inspired solutions
Novartis created the Rare Disease Franchise was recently created as a means of strengthening their involvement and has two drugs with FDA approvals, one for Cushing’s and one for Acromegaly. Their Acromegaly drug is 25 years old and their newest, Signifor, was approved on the anniversary of the discovery of Cushing’s Disease (December 2012) and three new applications are in the approval pipeline.
These diseases are rare because not many people have them and not much knowledge is available about them.
He also said he needs collaborative partners, particularly with Cushing’s.  He would like to have Clinical Trial centers.  However, usually enough patients are near one or two centers.  With Cushing’s, there would need to be 40 or more centers.  We talked to Vijay after the Caucus about this and connecting his company with Cushing’s patients.
Emily Acland, although not a Cushing’s patient, summed up some of the symptoms based on her contacts with patients through the Patient Access Network.
Alexey Salamakha, Manager of Rare Disorders for Novartis/Public Affairs and Communications,  read some thoughts on the need for disability benefits from Donna ofJohn’s Foundation for Cushing’s Awareness.  This included the the fact that veterinarians are more knowledgeable about Cushing’s than endocrinologists. He talked about patient advocacy.
Alexey specifically mentioned me and thanked me for my work.
Bob Knutzen was not diagnosed until the age of 52.  He is currently 75.  He expressed his desire to have Centers of Excellence for Hormonal Health with the funds coming from NIH’s budget.
Pituitary disease isn’t rare, just the diagnosis. He also pointed out that pituitary patients generally die 10 years early.  Without treatment, pituitary patients can’t have children.
If I didn’t know what acromegaly was before this meeting, I wouldn’t have known when I left, either.
Sean O’Neil, Vice President at Novartis made comments about his company and what was being done to help patients.
Other topics during this Caucus were:
  • The issues of Cortisol withdrawal
  • Congressmen Snyder and Runyon proposed H con resolution 31 ”Supporting Rare Pituitary Disease Awareness”.  Track this resolution through the Committee, House and Senate
  • The need for awareness of pituitary gland diseases
  • There are lifetime changes – people may be cured/in remission but they’re never the same
  • The possibility of a dipstick for cortisol similar to ones diabetics use
  • Faster diagnosis
My contribution to all this was speed of diagnosis.  I told a bit of my story, diagnosing myself in the pre-Internet 1980′s and how today, 26 years later, people are still having issues with diagnosis and wasting on average 6-20 years just getting to surgery.  I mentioned that I knew a few people who went for 20 years before getting diagnosed.
After the Caucus was over, there was a lot of discussion, and I talked with several people who had questions about my experiences, Cushing’s Help, what could be done to raise awareness…
Will anything come of it?  I don’t know but maybe some folks will start thinking a bit more.
From Tom, on Facebook:
Mary did a great job presenting the Cushings story at the April 16 hearing of the Congressional Caucus on Rare Diseases – Challenges our Country Must Address. Co- chairs Congressman Joe Crowley (D-NY) and Congressman Leonard Lance (R-NJ) both attended and endorsed the good work being done in this effort. Mary spoke with many of the sponsors and others both before and after the hearing discussing her personal experience. Mary has created multiple websites to get the message out on rare diseases especially Cushing’s Syndrome. That effort now extends to more than 40 countries and more than 10,000 participants. We will be doing follow ups with the Congressional Caucus on Rare Diseases and with Novartis, RDLA, EveryLife, Patient Access Network, the Pituitary Network Association and others to build on the gains.
And another email:
Dear Mary,
It was a pleasure to meet you and Tom today. Thank you for attending the Rare Disease Congressional Briefing. I think you did an excellent job by sharing your unique perspective on what a life with Cushing’s disease is like. I want to thank you for supporting our mission and educating general public about pituitary disorders. We at Novartis strongly believe that patient advocacy organizations such as Cushing’s Help and Support and passionate advocates like you are the future and the hope of the Cushing’s community.
As a follow up to our conversation I have reached out to my contacts at NORD and asked if they can help with filing for a 501(c)(3) status. I will keep you posted. Please stay in touch.

Monday, April 8, 2013

Cushing's Awareness Day, 2013


Today, April 8th, is Cushing's Awareness Day. Please wear your Cushing's ribbons, t-shirts, awareness bracelets or Cushing's colors (blue and yellow) and hand out Robin's wonderful Awareness Cards to get a discussion going with anyone who will listen.
And don't just raise awareness on April 8.  Any day is a good day to raise awareness.

What Can *YOU* Do to help?
Check out these ideas
1) status of the Cushing's Awareness Day legislation... who has signed, who do we need to get? I have contacts to the US congressfolks from Houston, and I wanted to get them to sign if they have not. Can we get someone to testify before Congress? Can we get any federal funding? Can we rally with other rare disease groups to get in front of that mic on CSPAN?
2) can we do a fund drive for the month prior to April 8th in order to fund all of our projects and this site? can we keep track of how much each board member raises with little thermometers that show how much is collected, in order to get people excited about how much money is being generated?
3) can those who have had surgery in the past year right up there story to be published on April 8th for awareness day? I hope that planning this far in advance will allow us to get more articles published. Perhaps those who already had surgery can ask the paper to re-run their story with an update and reminder about awareness day.
4) can someone can write a press release to the news channels to get coverage for awareness day
5) we get someone to create a very nice postcard that we all can print from our home at our expense on card stock. Then we each pay for the postage to send the cards to all of our friends and families. Perhaps we can ask our family and friends to donate money to us that we can then donate to this board...
6) I need a Cushing's bumper sticker! I know there are some for sale on the store site, but I was wondering if we can get some heads on this project to get something really catchy. I would put one on my card if we could get a really cool design going... any graphic artists wanna take this up?
7) I love the radio blog shows. I think they are so great. Perhaps we can add that icon to all of our Myspace, Facebook, and personal websites. Do we have an icon that we can post to get people to this site?
8) Have people set up care pages to keep friends and family informed about all the tests, doctors appts and then following us through surgery and post op. I have over 65 people subscribing to my care page, and I am confident that those 65 people didn't know anything about Cushing's until I told them about it. If we inform little circles of 50 all around the US and world, then we will be doing a great job increasing awareness. It is free, and I love mine. I control the tone of the site, and no one talks back to me there!
9). What can we do in the medical community? I'd like to see us patients doing more to lead the doctors down the proper path. ----- Do we get a booth at a national endocrinologist conference and pass out information we want them to know, with Cushies staffing the booth? ----- Do we develop a pamphlet specifically for doctors? ----- Do we start a partnership with the national association of endocrinologists (not sure if this really exists, but you know what I mean) and work together to get more research done on Cyclic Cushing's by offering ourselves up as research participants?
10) Perhaps we contact every Women's health magazine on the market and submit some articles in February for consideration of their April magazine.
11) Perhaps we buy the google ads based on key words that appears in email. The links pop up on the right side bar while people read their gmail or other online email. If the person says ponch, fat, or other key words, then the side bar will give a link to this site.
12) We can distribute DVDs of Kate's show and the two mystery diagnosis shows (Sam and Sharmyn) to doctors and endos? To new Cushies? This will take some money for DVDs and postage, but if we focus on raising some money, then we can allocated funds to these worthwhile expenses. Now, I am not sure if we need permission from Mystery Diagnosis or Nat Geo for that, but someone can take this project and get all the details.

I'd like to see us set up a goal-driven campaign for Cushing's Awareness Day. This will really help motivate us into seeing how much interest and good we are generating as a group.
# of newspaper articles submitted
# of newspaper articles publised
# of magazine articles submitted
# of magazine articles published
# of dollars raised
# of dollars allocated to outreach (keyword ads in gmail and others, etc)
# of carepages set up
# of care page followers (in friends and family.. I have 65 already) etc.

If we each decide to lead a project to get all of this taken care of, we could be in a very good position to launch a really big Cushing's Day Awareness in April. I am really looking forward to seeing all the creativity that exists of the minds of my brilliant Cushie friends!

This year, Novatis has made 2 TV commercials that will air this month to help with Cushing's Awareness.

View them in advance here:


Also, a bit late for Cushing's Awareness Day this year but a big hope for the future is the upcoming Congressional Caucus on Rare Diseases:

April 16th Rare Disease Congressional Caucus Briefing
Rare Disease Legislative Advocates and Novartis, in coordination with Rare Disease Caucus Co-Chairs Representatives Leonard Lance (R-NJ) and Joe Crowley (D-NY)  will host a briefing on “Medical Innovation for Rare Diseases: Challenges that Our Country Must Address.”  Tuesday, April 16, 2013 from 12:00 PM – 1:00 PM in Rayburn B-318

  • Advancements and new developments in the treatment of rare diseases
  • Advances in the rare pituitary disease space
  • Opportunities and challenges for those living with a rare disease
  • Opportunities to discover new treatment options
Speakers  include:   
  • Robert Knutzen – Pituitary Network Association; Chairman and CEO
  • Louis Pace – Cushing’s Support and Research Foundation; President
  • Dr. Vijay Iyengar – Novartis Rare Disease Franchise; Vice President
Food and Refreshments will be served.

General Public may RSVP by clicking here

Staffers may RSVP by contacting Helen Dwight in the office of Rep. Leonard Lance (202-225-5361) or Nicole Cohen in the office of Rep. Joe Crowley (202-225-3965).

Thursday, April 4, 2013

Latest Posts in the 2013 Cushing's Awareness Challenge

We have a new blogger today - Gail blogs at My Life as A Cushie

Please read these posts and comment on them, if just to give a bit of encouragement.

Wednesday, April 3, 2013

Cushing's Awareness Challenge - Day 1 Posts

Day One Posts: