RIP Fellow Cushie, Kalyn

Kalyn Allen, a fellow Cushie whose disease was finally diagnosed not that long ago (last year), lost her battle with Cushing's disease.  We've been blessed with all the resources now available to get to know and help fellow Cushing's, who are often quite alone in our journey as we are with families who often aren't that supportive.  Kalyn had a family who loved her and supported her through her treatment.  But sometimes even that is not enough.  It sounds like embolisms were the cause of death a reminder of how deadly they are.  I had a close call in 2002 with a pulmonary embolism which struck and nearly took me out 15 years ago.

The Announcement of Kalyn's death with a link to her story and a donation page for medical bills can be found Rest In Peace Kalyn.  The page contains a before and after photo of Kalyn.

RIP Dear Lady, Kalyn

Cushing's Blogger Challenge

Hello again!

Next month there will be a challenge to Cushie bloggers to post everyday in their blogs and I've accepted the challenge.  Four days in particular will be difficult to do since I will be out of town for a religious convention.  If you've read through my blog then you already know my faith is a major part of how I cope with the challenges of my disease.

Generally I put quite a bit of effort in my posts, to make sure the information I present is accurate, so plenty of research is often involved.  The challenge page offers a nice list of themes to blog on, so I'm going to see how many I can hit along the way.  I should also have my garden going for this season, I've already got some lettuce, spinach and onions up and running and they should be transplanted by then.

Since I mentioned my faith being important to how I cope and maintain some personal balance, I'm going to leave you with a devotional piece I try to read daily.   The last three parts are what I really like about it because it is a reminder of who is in control and the attitude I should work to have in the face of my problems.  It is called My Morning Resolve:

My Morning Resolve

My Earliest Thought I Desire Shall Be:

"What shall I render unto the Lord for all His benefits toward me? I will take the cup of salvation and call upon the name of the Lord [for grace to help]. I will pay my vows unto the Most High." – Psalm 116:12-14

Remembering the Divine call, "Gather My saints together unto Me; those who have made a covenant with Me by sacrifice" (Psalm 50:5), I resolve that by the Lord's assisting grace I will today, as a saint of God, fulfill my vows, continuing the work of sacrificing the flesh and its interests, that I may attain unto the Heavenly inheritance in joint-heirship with my Redeemer.

I will strive to be simple and sincere toward all.

I will seek not to please and honor self, but the Lord.

I will be careful to honor the Lord with my lips, that my words may be unctuous and blessed to all.

I will seek to be faithful to the Lord, the Truth, the brethren and all with whom I have to do, not only in great matters, but also in the little things of life.

Trusting myself to Divine care and the Providential overruling of all my interests for my highest welfare, I will seek not only to be pure in heart, but to repel all anxiety, all discontent, all discouragement.

I will neither murmur nor repine at what the Lord's providence may permit, because

"Faith can firmly trust Him,
      Come what may."  

Link to Cushie blogger's challenge:

 http://cushieblog.com/2015/03/01/fourth-annual-cushings-awareness-challenge/

 

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!

Update.

Hello my dear friends and readers:

I know it’s been awhile since my last post.  Several things have happened.  Because of continued back problems I went to my orthopedic doctor and it turns out my spinal deterioration has increased.  I now have three spinal joints in deterioration instead of just the one.  Those would be the last to lumbar joints and the first sacral.  This is not that unusual in patients with Cushing’s.  Both the leaching of calcium from the bones and the strain from the weight combine to cause the problems, which are pretty painful.  When we add the swings of my cortisol into Addison’s territory part of the time and I get a double whammy.

I also have another problem which I began to feel when I took my airplane trip back in April.  That would be bursitis in the hips.  Because of the Bursitis it is difficult to sit most of the time and it is also painful to rise from a sitting or lying position.  Given I weigh 366 pounds is it any wonder I developed inflammation of the bursas in that area?

Since then I’ve been looking into more research on Cushing’s, its causes and effects.  I’ve also looked into research suggesting the incidence of Cushing’s is much wider than the medical community realizes.  Some of the things I’ve learned will be presented in other posts.  However, I still do have to put up with the new problems and adapt, which is taking some time.

So keep an eye out for what’s ahead.

A tale of Stretch Marks!

Everybody hates stretch marks.  We get them when we gain weight.  We get them when we get pregnant.  And, oh, how unsightly they are!  But guess what?  They can be far worse as any Cushie knows.  Here is a meme I picked off of Face book which shows fairly normal postpartum stretch marks, the most typical for women:

And the worse a woman is likely to see if they maintain anything like normal weight is here:

Sorry about the language.

  Those are more typically called striae and there’s a reason a woman will end up with those during pregnancy and keep them post-partum.  When I sat through anatomy and physiology class the professor teaching the class revealed something a little unexpected at the time about pregnancy.  During pregnancy women typically experience a rise in cortisol levels and actually develop Cushing’s during their pregnancies.  The body’s response serves the purpose of providing tissue mass to support and protect the developing child during the pregnancy.  Consequently women will develop stretch marks during the pregnancy and keep them after.

However, for those who suffer from any variant of Cushing’s the situation typically becomes worse.  The stretch marks get bigger, wider and will often turn anything from pink to purple.  One of my friends let me use this image of her striae showing how wide they can get:

And here are links to more images of Cushing’s striae:

http://www.riversideonline.com/source/images/image_popup/sn7_stretchmarks3.jpg 

http://www.dermnetnz.org/systemic/img/s/cush1-s.jpg 

http://www.skinsight.com/images/dx/webChild/striaeStretchMarks_16044_lg.jpg 

http://o.quizlet.com/h0q1jDE1x0sv5LUECmjMzw_m.jpg 

What can you do about the striae, nothing much.  Put skin lotion on them and accept them for what they are, a symptom of a much bigger problem.  If you’re big enough that you will be losing a ton of weight when you’re cured you may be a candidate for having some skin removed, that will remove most of the striae with the trade-off of some surgical scarring unless a really skilled plastic surgeon handles the procedure.  The striae just make us resemble our namesakes, the zebras, more than most folks with rare diseases.

Cushing’s And the Immune System

Greetings again my friends and readers!

Well, it has come time to talk about another aspect of Cushing’s, compromised immune system response.  Most old time zebras know well enough how vulnerable they are to opportunistic infections.  That’s one of the things long term exposure to steroids do is to make the immune system weaker and less able to ward off disease and infections.  And what is Cushing’s syndrome but long term exposure to steroids?

I was forcibly reminded of that fact this past week.  To set the stage I was having some problems with what seemed to be a stomach bug of some sort.  So I went to a clinic not far down the highway from where I live.  After seeing the doctor and being told I was suffering from “food poisoning” I waited out in the waiting room for my wife to pick me up since she’d run out to run a couple of errands real quick while I was in.  I collapsed right there in the waiting room and was rushed to the hospital where I remained until yesterday.

Although there are still some questions since I didn’t test positive for the flu or bacterial infection, it was decided that I had been brought low by a viral infection which affected more than one system and also severely dehydrated me in the process.  Right up to the last minute before release I was pumped full of antibiotics and fluids.

That is one of the things those of us who suffer from Cushing’s have to be aware of and watch out for.  Our immune systems become less able to cope with invaders over time and one can easily get into in trouble if one doesn’t stay on top of things, like I didn’t.  That means not waiting until what seems like a small problem gets out of hand.  So one has to treat almost every sniffle as something serious, don’t wait around to treat it and be ready to get help at the first sign of trouble.  I know doctors and insurers get a little bent out of shape by us “hypochondriacs,”   But let me tell you three days stuck in a hospital bed eating the low calorie diets us fat folks inevitably get put on in the vain hope we’ll lose weight is no fun.

Another thing I found while checking the net for this post was multiple reports on some research done which is another reason to be careful.  It seems there is some evidence that infections can cause the production of cortisols to go out of control in a victim of Cushing’s with fatal results.  Since all I could access were abstracts I couldn’t get down to the details.  So there’s another reason for anybody with Cushing’s to be careful, very careful.

That doesn’t mean a Cushie can’t live a reasonable life on that score.  Just exercise common sense and care.  Do that and you can generally avoid ending up in as extreme a situation as I ended up in.

Cataracts and Cushing’s

Circumstances force me to interrupt my planned series for a short discussion on Cushies and cataracts.  I’ve been having growing problems with my sight lately and suffered from eye strain and headaches as well.  One day I noticed that when I closed one eye type on the monitor screen and in printed material simply disappeared.  I already knew I had cataracts in both eyes in the early stages and feared one of them had accelerated its development.  My fear was confirmed and my doctor wants me to have the cataract removed as soon as we can get it done while my eyes are stable.

Cataracts are a condition where the lens of the eyes becomes opaque.  As cataracts develop the patient experiences increasing difficulty seeing.  It will probably be things like not being able to read type which or an eye exam which will be the first clue that something’s wrong.  However, the cataract will eventually cause complete blindness in the eye if left untreated.

The formation of cataracts in cases of steroid treatment and exogenous Cushing’s syndrome (Cushing’s caused by medical therapy) is a well known side affect.  However, research seems to document that cataracts are not a common side effect in endogenous, or Cushing’s caused by tumors.  I didn’t find anything on it, but I imagine that cataract formation or acceleration would be a likely danger with the use of glucocortical steroids such as prednisone for the treatment of inflammation or injury with a patient who already has exposure to elevated levels of cortical steroids to begin with because of Cushing’s syndrome.  That is another reason steroids should be used with care to treat Cushies.

The treatment for advanced cataracts is removal and replacement with an artificial lens.   While reading through the brochure I was given I found out that great strides in advancement in both the surgery and the lenses have taken place since my days as a practicing nurse.  It is now possible in people with no astigmatism, or even certain cases of astigmatism to have such sight improvement after surgery that eyeglasses are no longer needed.  However, most cases of people with astigmatism will still require eyeglasses.

As with any other surgery there are risks.  According to the patient plan I was given they include cornea/retina injury, hemorrhaging, pain, blurred vision, double vision and loss of vision.  Since I’m on clotting therapy the risk of hemorrhaging is especially pronounced and I will probably have to go on a heparin bridge to reduce the likelihood of that complication.  That requires an added week before the surgery can be done.

Naturally I will not be in a position to write for awhile after the surgery and don’t know how long my recovery to the point I can resume writing will be.  But I will be back and will continue with the series I started.

Helpful links:

Do Steroids Cause Cataracts? 

http://ibdcrohns.about.com/od/prednisone/a/Cataracts.htm

Catarcts and Cushing's Syndrome

Truncal Obesity

Yes, that’s a brand new picture of me taken just this week.  That makes it among the most recent in ten years.  But let’s take a look at the rest of me:

As you can see I’m playing a guitar.  Flamenco guitar is one of my hobbies and I do enjoy playing it.  But the important thing to focus on is the fat.  My fat is concentrated mainly in my trunk area.  Look at how thin my arms are, almost like skinny sticks.  That is the classic portrait of what is called Truncal Obesity, the rather distinctive trait of Cushing’s syndrome.  Normally the morbidly obese have plenty of fat on their arms and legs as well as the trunk of the body.  However, those with Cushing’s syndrome, or a few other disorders such as PCOs (Polycystic Ovarian Tumors) will display truncal obesity to varying degrees.  Anytime one sees the pattern one should consider endocrine problems.

In the following picture you’ll see that my mother displayed the pattern as well:

Once again, we see morbid obesity combined with a relative lack of fat in the extremities.  With both my mother and me you could see the individual muscles in the forearm clearly.  It was the same with both our lower legs and my thigh muscles stand out really well.  In my case both my arms and legs are free of fat until I reach the joint with my torso, then some shows up.  Next I repost the picture of my friend:

I her case the differences in the trunk and extremities aren’t so pronounced.  Yet she does suffer the disease.  Look more closely at her forearms, though, and you will see the difference.  Finally I will repost another picture I’ve used:

In this picture which was posted on Facebook the woman shows the clearly distinct truncal obesity which is often the first big sign of Cushing’s.

This is the first in a series of posts on the things those with Cushing’s can see for themselves or which may be noticed by friends and family.  For some of those the clothes will come off, or the pictures themselves may be shocking.  I’m choosing the photos and getting permission to use them.  There are folks out there who are willing to expose themselves that those who are seeking to know what is going on with their own bodies, or those of loved ones.  They have my hearty thanks!