A New Discovery About Cushing's!

One of my Facebook friends and a fellow Cushies posted the following to her blog and then posted the link to one of the Cushing's forums today.  Instead of simply posting my own write-up of the material I felt it best she gets the credit she deserves for both bringing this to our attention and the well-written post she put together.  So here is the link to her post:

What We Know About Cushing's just changed!

One of my Sister-in-laws has Cushing's and had the surgery.  Her tumor was in the Pituitary gland and there is no evidence the last I heard of a return of the tumor though there has been a return of the Cushing's Disease.  Could this be the answer?

Catch you later, my friends and readers!

So, How's That Treatment Going?

Since it's been awhile since we talked about our treatment we thought our online friends might want to know how things are going.  We went to the doctor just last Friday, 01/30/2015.  I'll get to what we found out in a minute, but first a review of the treatment we started.

Back in October of 2014 we reported among a number of things that we began a course of treatment with a medication of the same class as one which is being evaluated to treat Cushing's, that is Cabergoline.  Specifically it is not cabergoline used in my case, but another D9 agonist which is also a wider acting drug.  a couple of years back we underwent surgery to remove a tumor we hoped might be the culprit for our Cushing's though it is sited in a place more typical for a Cushing's like disease known as MENS, or Multiple Endocrine Neoplasia.  MENS is a Disease in which the victim presents with multiple tumors which secrete excessive amounts of hormones, cortisols being the most common.  It's not a nice disease to contemplate having as in many cases the tumors are cancers.

Within days we entered the local emergency room with agonizing muscle spasms only to find out that though we had removed a tumor suspected of causing Cushing's and prophylactically been given some corticef to head AI off, the local ER wasn't allowed to test for AI (Adrenal Insufficiency) because it wasn't considered an emergency situation, a really dumb idea given this patient had informed the staff that the possibility was on the table because of the surgery and that AI can kill in a hurry.  So the attending doctor pumped us full of another kind of steroids and muscle relaxers until the cramping stopped.  Then we were sent home on a very high dosage of prednisone and told to see the attending surgeon in  couple of days.

On the day of our appontment with our surgeon we tested our blood sugar and the home tesotr informed us that our blood sugar was high, no figure, just too high for the machine to test.  Since the appointment was an hour away and the office in the same building as the hospital's ER, we kept the appointment.  Naturally the doctor sent us down to the ER, where we were found disoriented on the way by a nurse who was off duty on break and she rounded up a wheelchair and somebody to take us down to the ER where we were admitted with a blood sugar of 608, a deadly situation.  When our condition was stabilized we got a referral to the only local physician we trusted enough to deal with the situation though it was outside his specialty.

Because that doctor felt our neurological system had been compromised he started us on two medications to deal with the situation, Gabapentin and Ropinerole.  We are sure the Ropinerole was a good call because we'd already started having some trouble with some mild restless legs style symptoms but hadn't reported them yet.  So we were on the way there and the ER treatment just probably hurried the situation along.  That was back in 2012 and I haven't had a high cortisol test since.  I've had a couple of very low ones, but no high one.  What made that really frustrating is that during 2013 we did an evaluation with the object of putting us on one of the two new medications to teat Cushing's and all of our test results came back on the high side of normal.  What is more certain symptoms of Addison's I have increased since.  So we couldn't get the medication to treat Cushing's, yet our cortisols were not at a level low enough to begin reversl of my condition.

While a friend and I were on the phone one day looking into a personal theory he had which he felt might explain our Cushing's, we stumbled on a paper about a trial of a medication being explored to treat Cushing's, the D9 agonist used to treat Parkinson's disease, Cabergoline.  It hit us one of our new medications, Ropinerole, is also used to treat Parkinson's, along with Restless Leg's Syndrome.  So we checked and fund out it is also a D9 agonist, along with acting on some other receptors as well, which would explain why it wasn't being evaluated as well.  So we talked with our endocrinologist on our next visit who felt we might be on to something and looked into Cabergoline and the possibility that tweaking our dose of Ropinerole might be a strategy for treating our Cushing's.  As a research physician as well as a professor of endocrinology the possibility struck a note with the doctor.  After six months of research and forming a plan, our dosage was slightly increase to see if the Ropinerole was indeed suppressing our cortisols and a treatment stratagem established.  By the way, the doctor who at the time was prescribing the Ronpinerole approved of our little experiment, tomorrow I'll tell him how it is turning out.

Now for the money part of the post. The doctor who started me on the Ropinerole will probably like what I have to report.  Technically I'm still at the small dosage range, small enough that increasing the dosage as we did probably wouldn't have hurt a thing.  Yet, my endocrinologist is pleased with results.  Our cortisols are now at a level he feels comfortable with for treating the disease.  Something to remember is that we are cyclical  and our cortisols do drop far enough on their own to cause problems, and may already have done so once since treatment began.  So the doctor wants to go slow enough to avoid that problem as much as possible.  While we don't blame him, we do have a problem with patience and are willing to risk much to gain much.

Our heamogobin A1C is stabilized a 7.0 and we have a demonstratable, if slow weight loss going.  We've gone from 368 lbs, at the beginning of treatment to 358 lbs, so the weight slope is downward.  Although we are still hypertensive, and that worries the Doctor, our blood sugars are well into normal range in the mornings.  So everything points toward success at this point.

We've also improved our health in other ways.  We are now off of pain medications and getting around better.  My back Doctor would probably be pleased with that and I might want to schedule an appointment with her for a new evaluation for that.  

If we continue to improve and could get our experience into the medical literature it would add another bit of evidence for the efficacy for treating Cushing's with D9 agonists safely.  With more treatment options open for Cushies more personal stratagems for the treatment of this disease be available to personalize the treatment to what works best for the individual.  That can't but help those of us who've waited so long for this day where more treatment options open up for us.

So I just thought my friends might want to the update.  I have a few more topics cooking and will hopefully have something more for you in a bit.

Chiao!

BLA

Thanks for waiting dear friends and readers.  Since this is also a chronicle of my journey with Cushing’s syndrome I’ll take just a moment for an update.  My sonogram came up negative for any tumors.  That doesn’t mean there weren’t any micro tumors there, just that if there were they were too small for the scan to visualize.  They did find another problem which will require me to go back to my urologist.  So my endocrinologist wants to do more testing to see where things are at.

Surgery is out, of course, since they couldn’t see anything to justify it.  There is only one surgery which is often done in cases where adenomas can’t be located, that’s the bi-lateral adrenalectomy or BLA for short.  The BLA is performed when they find tumors in both adrenal glands, in the case of recurring pituitary tumors and in the case of ectopic tumors.  The BLA is simply the removal of both adrenal glands.  The idea in the case of ectopic Cushing’s and recurring pituitary tumors is to shut of the production of the cortisols at their source.  Since tumors generally produce ACTH, the hormone which signals the adrenals to produce cortisols that is considered the logical way to deal with the problem in many cases.  After the two glands are taken out the patient is put on hormone replacement therapy or HRT for short.

The adrenal glands sit on top of the kidneys and are protected by the lower ribs.  Not many years ago the surgeon had to break the ribs over the adrenal glands to get at them for removal.  But now days, at least here in the United States of America, a new method is used to remove them.  The surgeon uses a laparoscope which requires relatively small incisions and which snakes up under the ribs to get to the adrenal glands.  Another great aspect of this kind of surgery is that the patient isn’t placed on their stomach for the procedure, which is contraindicated in my case.  The advantages of this kind of surgery are low recovery time with less pain and less time in the hospital.

After the surgery the hormones which the adrenal glands produce have to be replaced or there will be trouble.  So the patient goes on HRT which involves testing and adjusting the hormone levels through time.  The patient is also subject to going into Adrenal failure (AI), which I’ve discussed in two other posts, and having to seek emergency treatment for that problem.  So a BLA is a surgery with serious consequences and the patient and their family have to be familiar with what to watch out for and always ready to take them for emergency treatment.

Although one has to be watchful, the surgery does cure the Cushing’s and the patient will generally lose weight, even achieving normal weight in some cases and they will have a more normal life when compared to what they had with Cushing’s.  So while it is a procedure with serious consequences, including death in some cases, it is a surgery to consider in many cases.  According to a study done by doctors in Oregon, it is a surgery which can result in a good quality of life for most.  I’ll link the study at the bottom.

http://home.comcast.net/~staticnrg/Cushings/QualityOfLifeBLA.pdf