Could you Shed Some Light on Cushing’s Disease?

Dear Dr. Roach: Could you shed some light on Cushing’s disease? Four people in the same family have it. The doctors say it has something to do with the thyroid gland.

— Anon.

A: Cushing’s syndrome, which is different from Cushing’s disease, is an excess of cortisone or similar corticosteroids. It can be caused by taking too much steroid for too long, usually as treatment for a serious medical condition. Cushing’s disease is a special case of Cushing’s syndrome, when the excess cortisone is caused by a tumor in the pituitary gland, which spurs the adrenal gland to make excess amounts of hormone. Weight gain, almost exclusively in the abdomen, a striking round “moon” face, a fat pad on the back of the neck and upper back (“buffalo hump”), diabetes, pigmented stretch marks and high blood pressure are common findings in any form of Cushing’s syndrome.

It is very unusual for Cushing’s disease to run in families. Also, it does not affect the thyroid, although thyroid conditions can sometimes mimic Cushing’s (and vice versa). I suspect that what this might be is a rare condition called multiple endocrine neoplasia type I (MEN-1). This does run in families, and combines risk for pituitary, parathyroid and pancreatic islet cell tumors. (The parathyroid glands sit on top of the thyroid gland and secrete parathyroid hormone, responsible for calcium metabolism. The pancreatic islet cells are where insulin is made.) Not everybody with MEN-1 will have tumors in all of these glands. Parathyroid tumors are the most common.

An endocrinologist is the expert in Cushing’s and the MEN syndromes.

​Dr. Keith Roach writes for North America Syndicate. Send letters to Box 536475, Orlando, FL 32853-6475 or email ToYourGoodHealth@med.cornell.edu.

From http://herald-review.com/news/opinion/editorial/columnists/roach/dr-keith-roach-teeth-grinding-is-common-in-the-elderly/article_bef63ba4-9b5e-5bff-b66a-3530be158857.html

Cushing’s Awareness Challenge: Day 13

robin-complications

What can I say?  Robin’s images area always right on!

My only addition to this list would be the ongoing daily fatigue.  Maybe that’s not really a complication but more of an annoyance.

My bone loss has stopped  (or  slowed down some) over  the years since my pituitary surgery in 1987 but I doubt that I’ll ever gain back any height!

Luckily, I haven’t had a stroke – knock on wood!

Cushing’s – the “gift” that keeps on giving!

maryo colorful zebra

 

Cushing’s Awareness Challenge: Day 12

robin-head

 

Mail!  I get all kinds of email asking questions about a variety of Cushing’s issues.  I’m not a doctor and I don’t play one on TV.  I don’t even play one on the internet.  People are desperate for answers, though, so the questions keep coming and I try to answer the best I can.

Here’s a recent question and answer.  Note that you have to be logged into the message boards to view the links in this post.

 

Question: My daughter was diagnosed w/ cushings in 2001 at the age of 20 & had the pituitary surgery.

In late 2013 she was diagnosed with a recurrence. I’ve read that that usually happens within 5 years, not a dozen years.

Regardless, there is a new research program but she was told she doesn’t qualify for it. The other medications offered are either exhorbitant ($100-200,000/year), another causes liver damage, another causes uterine problems. A 2nd surgery is not recommended according to  the surgeon (because there would be only a 50% rate of success due to the scar tissue from the original surgery), and radiation is being vetoed as well, being recommended ONLY as a very last possible resort.

Are there other parents who chat & share experience here? Will I find help as a parent here with my frustration over this disease? Are there other patients who communicate here that are from Michigan?  Are there other patients here who are suffering from the recurrence? Don’t get me wrong, I’m happy to find on several sites online today that there are so many success stories; I would just like to know what other options there are that perhaps our Dr. is missing.  Thanks.

My response:

S, since you have a Board Name, I assume that you are a member of the message boards.

There are areas specifically for recurrence – http://cushings.invisionzone.com/index.php?/forum/35-recurrences/

People in Michigan: http://cushings.invisionzone.com/index.php?/topic/13696-michigan/

Parents of patients: http://cushings.invisionzone.com/index.php?/forum/31-parents-spouses-children-and-friends-of-patients/

The more you read, the more you will learn.  Many patients with a recurrence  have a second pituitary surgery.  She might need to get another opinion from another surgeon.

Another option is a BLA – or have her adrenal glands out.  That can cause other issues, though.

The 2 drugs you  mentioned are Signifor and Korlym.  Although both are expensive, each has a patient assistance plan which lowers the cost dramatically.  Doses can vary dramatically so that they don’t necessarily cause liver or uterine issues.

Ketoconazole is another drug that’s sometimes used.

I did a search on the boards and there are 69 topics for Mifepristone (generic Korlym), 51 topics discussing the brand name Korlym, 40 for pasireotide (generic Signifor), 13 for the brand name Signifor, and 69 for keto (the common abbreviation on the boards for ketoconazole)

Here’s a personal experience from a woman on Korlym who likes it: http://cushings.invisionzone.com/index.php?/topic/53342-i-like-korlym/?hl=korlym

So – the information is out there.

I know it’s hard to process all this and make decisions.

I know it’s hard to process all this and make decisions. I had my one pituitary surgery in 1987, before the Internet was available so I had to really research all this in medical texts.

At that time, there weren’t any drug options. Just surgery and radiation. I decided off the bat if I should have a recurrence, I would not do radiation. I’d go for another pituitary surgery first, then a BLA if needed.

But that was then and this is now.  There is way more information which is much easier to find.  There are better surgical options and even some more medical ones.

Good luck!

8e1d2-maryo_colorful_zebra

Cushing’s Awareness Challenge: Day 11

robin-uncontrolled

Robin has shared this quote from Dr. Prevedallo.  You can read more at the link at http://brainsurgery.upmc.com/_pdf/Review-of-Endocrin-Cushings.pdf

Over the years, I have seen that this is true, sometimes even for controlled Cushing’s.  Far too many Cushies have died.

Here are some of those that I know of:

Cushing’s is a terrible disease.

There is another Cushie I should add to this list. During the time I was home from NIH just before pituitary surgery, a college classmate of mine (I didn’t know her) did die at NIH of a Cushing’s-related problem. I’m so glad I didn’t find out until a couple months later!  I still have the college alumni magazine that mentioned this.  I’ll have to find that and add it to the In Memory list.

My husband shared a bit about her in my bio:

During the same time Mary was at NIH, another woman had the same operation. She came from Mary’s home town. They were class mates at college. They had the same major. They were the same age. They had the same surgical and medical team. Mary recovered. The other woman died during surgery.

 

I know we’re always fighting with doctors to get diagnosed, to get treated but reading the stories of these people will hopefully inspire people to fight even harder to be heard.

Stay safe – don’t get added to this list!

8e1d2-maryo_colorful_zebra

 

Cushing’s Awareness Challenge: Day 10

robin-tests

Gee, I’m an underachiever. LOL I only had one IPSS and one pituitary surgery.

While I was at NIH, my MRIs still showed nothing, so they did an Inferior Petrosal Sinus Sampling Test. That scared me more than the prospect of surgery. (This test carries the risk of stroke and uncontrollable bleeding from the incision points.) Catheters were fed from my groin area to my pituitary gland and dye was injected. I could watch the whole procedure on monitors.

I could not move during this test or for several hours afterwards to prevent uncontrollable bleeding from a major artery. The test did show where the tumor probably was located.

Also done were more sophisticated dexamethasone suppression tests where drugs were administered by IV and blood was drawn every hour (they put a heplock in my arm so they didn’t have to keep sticking me). I got to go home for a weekend and then went back for the surgery – the Transsphenoidal Resection. I fully expected to die during surgery (and didn’t care if I did) so I signed my will and wrote last letters to those I wanted to say goodbye to.

During the time I was home just before surgery, a college classmate of mine (I didn’t know her) did die at NIH of a Cushing’s-related problem. I’m so glad I didn’t find out until a couple months later!

maryo colorful zebra

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