Replacing Doctors.

Hello again:

Once again I pen a personal note.  The other day I went to my PCP, Primary Care Provider, for what was supposed to be a routine follow-up visit after all this mess I’ve been through only to have her terminate me as a patient.  It just seemed we couldn’t see eye to eye on my care.  So the patient doctor relationship was ended.  It happens, in fact this was the second doctor this year to end the relationship, and the first was a specialist.  It happens.

Let’s face it, people with rare chronic disorders are difficult patients and many doctors just aren’t up to the challenge or even want it.  Last year when the PCP I had stopped accepting the form of Medicaid I was on I had to search for another PCP.  The search took weeks simply because doctors weren’t willing to take my insurance, or they didn’t want the responsibility of taking care of a rare disease patient along with all its challenges.  This is the lot of a zebra as many of my fellow Cushies well know.  And it’s getting worse, but that’s a whole other topic all by itself and fraught with peril because politics has much to do with it.

I think I’ve already mentioned that I’ve been through five endocrinologists in the last twelve years.  Add to that Four PCP’s and a pulmonologist and that’s a lot of doctors.  I know of other Cushies who’ve been through even more.  The reasons are legion, they don’t get enough money from the insurance provider to make it worth their while, and they refuse to see what’s right in front of their face or whatever.  And more often than not it is somehow our fault.  It just seems to come with the territory.  So what is the Cushie to do in the face of the loss of a doctor, especially a PCP, the gatekeeper to our care?

Well, after we calm down from being royally pissed whether they ended it or we had to a positive attitude becomes a must.  That’s especially needed for the search or one can easily become discouraged.  In my case I had to call Medicaid to request a list of providers in my area approved for Medicaid and directions to their site.  In my case I found out that significant changes had been made in the policies and system since my last search a year ago.  Those changes were welcome, not the least of all that the Medicaid plan I was under had been discontinued and I’d been folded into the main Medicaid population.  That made some things easier.

It was about the time I started the search that my computer went down.  So my search for the day seemed pretty short.  But when my wife got home and I told her of the changes she suggested I call my last PCP’s office and see if they would take me back.  So I did and now have an appointment for next week.

So my next point, patience wasn’t necessary this time.  But it usually is when one loses a doctor and has to search for another given the reluctance of doctors now to take either those with chronic diseases or who are poor.  When one has both strikes against one; patience becomes a necessity.

Persistence is a key in the face of rejection.  Remember it took me several weeks to find a doctor’s office who would accept me the last time around.  One simply has to keep at it, just as one has to persist to get their diagnosis and care in the first place.  But if one hangs in there like a bulldog and doesn’t give up one will succeed.

That being said, don’t let the prospect of having to find another doctor intimidate you.  If your doctor is either incapable or unwilling to do their job you are better off finding one who is.  The search may be hard, but the rewards are worth it at the end when, not if, but when you find the right doctor for you.

Recent doings.

Hello again!

As I write this the first big rain from Subtropical Storm Beryl is beating against my bedroom window.  Since the storm is near hurricane strength it promises to get worse as the night wears on.  And the predictions are that I’m stuck with it for a few days.  I live on the coast of Georgia.

But as the storm gets going another one is winding down for me.   Since this is a personal blog about my personal journey with Cushing’s as well I’m going to tell the story.  The story in some ways is something most Cushies can relate to, as those who followed me on Facebook during this ordeal reminded me as they were there for me.  This isn’t a bitching session; some of my Cushie friends kindly let me get that out of the way, like any good herd of zebras.  This is for your information and insight on what those of us with chronic diseases go through if you don’t have one.

This one started when I found a tumor.  Cushing’s is caused by tumors, mostly tumors which run deep and require special imaging to find.  However, in rare cases, often the really rare familial Cushing’s they can occur in a place where it is possible to find them on your own if they’re not micro tumors.   So when I found a tumor on a routine self-examination of that personal area, oh well, my scrotum and testicles, I thought maybe   I found the tumor.

Now the first thing to understand is that  anytime one finds a lump in a man’s jewels  it needs to go to a doctor and most times will likely be removed.   So the next step was one I would’ve taken without Cushing’s being a factor.  However Cushing’s did make for a complication.  I would be examined three times before the tumor was removed.   Each time about two days after the examination I went in to a cortisol crash.  I know what those are like because I go into them every time some doctor orders up what is known as a Dex suppression test.  So I mention this to the surgeon who was to remove the tumor and  to  head off any problems he gives me an injection of hydrocotisef  while I’m under  in the operating room and  gives me instruction  for taking some prednisone for the next three days which were reasonable.

The day of the surgery, and the day after I’m fine.  But everything starts to go downhill after that and by Sunday (the surgery was on Wednesday) I had to go to the emergency room with acute respiratory problems.  While the ER doctor is examining me I go into extreme cramping all over my body.  It was one of the worse days of my life.   Needless to say I was in a critical situation.  After a ton of morphine and other medications were given me I was sent home instead of admitted with instructions to take massive doses of prednisone until I see my surgeon at my scheduled follow-up.

That follow up was the next Friday.  That day my morning blood sugar was 490.  Since I was to see the doctor I decided to keep the appointment as scheduled and let him send me down to the ER, the likely course of action since his office is in the hospital I also took more of the medication I use to control my diabetes to hopefully bring the sugar down.  Just before I leave to see him I check my blood sugar again and the machine reads “Hi.”  Blood glucose testing devices generally won’t go above 500, which are considered lethal.

By the time I get to the ER and they finally pull blood to check my blood sugar I was going into a diabetic coma with a blood sugar of 632. The culprit was the massive doses of prednisone which had been prescribed me.  Once again at the ER I’m pumped full of insulin until my blood sugar went down to 302 and then sent home for my family to deal with.  When I call my surgeon Monday and tell him the details the first words out his mouth were “and they admitted you?”  As you know, they didn’t.  He couldn’t believe that and the doctor he sent me to begin to wean me off the prednisone wasn’t too pleased with it either.

Now I’m being weaned off the prednisone slowly being given medication to prevent cramping and insulin to control my blood sugar during the weaning process.  I think the doctor wanted to have me admitted  to do it under more controlled conditions, but considering the  reluctance of the hospital to admit me when I was in critical condition not just once, but twice in a week he opted to go the route we’re taking now.   What is the matter with my local hospital that they seem to be acting in a way which invites a lawsuit is beyond me, but  this situation isn’t by any means  all that strange to those of us who suffer from Cushing’s and its affects.

Everyday Cushing’s patients who have had their adrenal glands removed will develop the symptoms of Adrenal insufficiency (AI)   and have a hard time at the ER.  Many now carry letters from their endocrinologists for emergency room staff when they go.  So it would seem my experience is by no means unique.  Medical zebras aren’t so easily seen as their wild namesakes.  Our diseases often look like other things.  So we don’t get the care we rally need in more than just the ER setting.  It still boils down to that old saying about hoof beats.

My Story.

I haven’t heard back on the biography I wanted to use, so:

My name is Stanley Loper and I’m a 56 year old male living in Brunswick, Georgia USA.  I was born in Jacksonville, Florida and have lived in my Dad’s home state of Michigan as well as Georgia, my mother’s home state.  I’m the second of four children.  And I’m the face of Cushing’s Syndrome.

I was the sickly one from the get go.   Something wasn’t right with my lungs when I was born and several times as a toddler I was found in respiratory arrest, already blue by the time I was found and revived.  I suffered often from chronic lung disease, largely bronchitis and asthma and have been told my parents were told not to expect me to survive childhood.  But I’m a fighter who just doesn’t give up.

I was also overweight as a child, very overweight, the only one of us kids to be so.  Folks like to say I take after Mom’s side of the family more than Dad’s, they don’t know the half of it, but more on that later.

When adolescence came things seemed to get better.  By that time Dad had divorced Mother and sent us all down to Georgia where she grew up.   I gained my height, slimmed up some, though even at my lightest weight I still looked like a stick man who swallowed a cannonball.  I was still, physically weak, but still one tough kid as anybody who ever stood on the other side of a grass field football line learned pretty quickly.  I always played for keeps.

I graduated high school, the only one of us kids to do so.  Put myself through a trade school and went to work supporting my mother and youngest brother until I married a divorced woman with two children in 1981 at age 25.  That is also when my health trouble began again.  My lung problems returned and I started gaining weight, about forty pounds overnight.   That was also when  the economy really bottomed out in the South and I lost my job and   spent the next sixteen  years  hustling up whatever work I could find to support my family in  small town rural Georgia .  Still, I kept gaining weight and nothing would take it off.  I often walked miles to work without losing a pound.

Two years after I married I entered LPN school and it was there that I first heard about a rare disease called Cushing’s Syndrome.  As I sat in class and   listened to the list of symptoms, the distinctive fat concentrated in the body trunk, the “buffalo hump,” and the other symptoms I realized the teacher was describing my mother!  Mother had suffered from those symptoms for as long as I can remember and it was her sickness which broke up her and Dad’s marriage.   Excited, I went home to Debra, my wife, and told her I now knew what mom’s problem was.   Debra asked me to   tell her the symptoms, which I did.  Then Debra floored me when she said, “but Stanley, except for the mental sickness that’s you!”  That was December of 1983.

It didn’t take me long to find out how hard it is to get a diagnosis.  The first doctor I went to ordered a serum cortisol, I already knew from that he wasn’t serious.  Sure enough, when it didn’t come back positive he smirkingly told me I didn’t have Cushing’s and simply needed to have some self control at the table.  He did the same to mother.  As far as I know she never mentioned Cushing’s to a doctor again so long as she lived.   But I’m a fighter.

I learned to bide my time until I could find the right doctor.   But my health deteriorated over the next seventeen years until that time came.  By that time I was disabled by the progress of the Cushing’s.   One day my doctor at the time called me into his private office to suggest I have a radical panniculectomy, a procedure to slice the body fat off surgically, to take off about a hundred pounds.   I agreed to the procedure on the condition that a test be run first for a specific problem I thought I had just to make sure we weren’t wasting the cost of the operation.

After listening to me present my case the doctor agreed and ordered the first real test for Cushing’s, a 24hr urinary cortisol test.  Since we’ve since established my Cushing’s is the rare cyclical Cushing’s I’m lucky the test, and the follow-up test the doctor did for confirmation both came back positive.  He also did some other testing and told me I likely had an ectopic tumor, that is a tumor somewhere other than the pituitary or Adrenal glands driving my Cushing’s Syndrome.  Since Cushing’s is supposed to be treated by endocrinologists, the doctor referred me to one.  She would be the first of five endocrinologists I’ve seen over the next twelve years and I have yet to be treated for Cushing’s syndrome.

First, most have tried to prove the diagnosing doctor wrong.    In the process it was established that I’m cyclical, and that my cycle is extreme.  It swings all the way from Cushing’s Syndrome to Addison’s disease and back again.   So I have many of the symptoms and characteristics of both diseases, such as the tan skin and degenerating back of Addison’s and the morbid obesity, etc. of Cushing’s.  I have yet to see so extreme a cycle described in any medical journal yet.  For years the treatment protocol with cyclical Cushing’s which has no obvious tumor is to leave it untreated until it becomes full-blown, or florid Cushing’s.  So I’ve been in a medical holding pattern while the doctors concentrated on my secondary conditions, such as my diabetes.

Socially Cushing’s has been a nightmare.  Society already stigmatized morbid obesity, so as my weight went up, my social life disappeared.    More and more my weigh was used as an excuse by my “friends” and even family to exclude me. The most common excuse was I was too heavy for their furniture.  Right now I’m banned by my stepson, who married into wealth, from his house on both that, and my low social status grounds.  The excuses currently in use are irrelevant.   This is all too often the experience of those who suffer from Cushing’s.

As this disease has progressed I’ve grown weaker, more sickly as the progressive weakening allows other problems to emerge, or are even caused by the disease.  I almost died from a pulmonary embolism in 2002.  I spent ten days in the hospital that time.  I’ve also been hospitalized again for blood clots and had an umbrella device put in the major vein which leads to the Vena Cava.  I’ve had my gallbladder removed, a bone spur removed, and another surgery to remove a tumor we hoped would be the tumor driving my Cushing’s.   I have a rare form of Rheumatoid Arthritis, whose attacks appear to be linked to my Cushing’s cycle.  In my case it attacks my eyes and is quite painful.

I’m in and out of the ER too often now days. For things like opportunistic infections of my lungs, which don’t need the abuse to begin with.   My back has degenerated to the point that I can no longer walk as well as I used to.  I often use a cane, a TENs unit, and even narcotics at times.  My stomach can no longer handle certain foods and I’m on medicine for that, along with diabetes, problems with gout, and I can go on and on.   About the only bright spot in my health is that my heart is in good shape and my circulation is alright.  We keep a close watch on that.  But even it will only be able to handle so much.

I basically live my life in a room and have friendship through the internet. I mainly leave it to go to one of my doctors, or the emergency room.  It is hard being a zebra.

There you have it, the story of me.  Now, life isn’t all glum however dark what I’ve written may make it seem.  But I’ll examine that in another post on how we Cushies can have meaningful lives with some bright spots in them.

Cushie Appetite!

Hello again:

I’d wanted to go next with a bio I wrote for a nationally published  newsletter for  Cushing’s suffers,  but I’m waiting on permission since I don’t own the copyright.  However, a conversation some of us Cushies are having online got me thinking on another topic, one which is touchy for us.  Here is a family picture taken some years ago of me and some of my family:

I’m not hard to spot, I’m the fat one.  At the time I weighed around 390 to 400lbs.  I know, you’re probably saying “My god that’s a really fat man!  How did he let himself get in that kind of shape?”  I didn’t.  That’s what years of Cushing’s Syndrome does to its victims.  And for the ones who have it in the extreme it doesn’t take all that long to do it.  Now you’re wondering if we can’t control our appetite and that’s only natural.  So I’m going to discuss that facet of Cushie life and lay it all bare for you that you may understand us and our problems a little more.

First off please understand that if we eat normally, and most of us do most or even all of the time,  cushies will still gain weight, lots of weight.  That’s because cortisols encourage the storage of food as fat.  In fact it is the reason why women gain fat during pregnancy they enter a cushingoid state during pregnancy which goes away after birth.  I know of a lady who is a runner.  She started gaining weight out of the blue and decided to simply go on a diet and increase the distance of she ran.  She still kept gaining weight.  She’s a Registered nurse and knew that simply didn’t happen unless something serious was wrong.  So she went to the doctors and didn’t take no for an answer until they found the problem.  She had pituitary Cushing’s.

Surgery cured her Cushing’s, but she didn’t stop there.  She started one of the first organizations to bring cushies together and start pushing for better research into our disease.

However, that isn’t all there is to how we gain weight.  Among the things high cortisols do are they ramp up the appetite.  We don’t like to admit it, but there it is.  But it’s not even as simple as that.  There is no such thing in the human body as constant hormone levels.  Every Cushie is cyclical to some extent.  It just that the “Florid” cushies’ (that’s a term I just saw today and may be the new one for what we call full-blown) cycle is always in the higher ranges and never enters lows anything like normal.  Because of societal pressures over our weight and appetites most of us fight our appetites and win that game when they are on the low side.  Some few win it all the time and my hat is off to them!

However, when the cortisol goes up we get hungry, insanely hungry.  And some of us just can’t help eating, even in the face of familial criticism.  The thing to understand is that it isn’t a mental thing, it is an organic imperative like that sudden itch couples get and scratching it is not anywhere near as satisfying.  I have stuffed myself until I couldn’t hold another bite and still had that overwhelming hunger driving me up the walls.   I’ve learned to indulge only a little, and then grit my teeth and suffer.  Most cushies do.

You see society and family shame us for our size. And when the appetite goes up the shame is heaped on in even larger measure.  And we feel the shame very keenly.  So we are touchy on the subject.  We wish family and friends were more understanding instead of critical.  We’re tired of hearing them and doctors tell us it’s all in our heads and we just need to exercise some discipline.  We need family to stop throwing it up in our faces that we “aren’t aware of how much we eat.”  We are when we do and we’re ashamed of it.  And saddest of all we need encouragement and understanding because, guess what, most of us diet when the hunger isn’t on us to try to undo the damage.  And almost all of us fight it  anyway, even when we’re losing that particular battle.

So what can family do?  Be understanding and supportive.  Try to have the sorts of food around which don’t go to fat so easily, whichever ones your Cushie family member may like.  Listen to them and if their hunger is on the rise provide that kind of snacking in as healthy an amount as you can compromise on.  But listening is the most important thing and giving them the love they need to find the strength will go much further in helping them lessen the damage a drive will cause and such love will  do much to keep your relationships strong and help them have a life worth living.

It is hard being a zebra after all.  And we need our friends and family more than ever until we each find our own cure.                                                     

Hello From A Zebra!

Hello:

I’m the Original Zebra.  That means I started something which folks on Facebook with the debilitating disease of Cushing’s Syndrome and Disease latched onto and made their own.  There is a medical axiom taught budding new doctors in many medical schools.  The saying is “when you hear hoof beats think horses not zebras.”  The idea is that a given set of symptoms are most likely to be the more common or obvious problems than the rare.  Well I’m a Zebra.  I’m the face of Cushing’s syndrome.

What is Cushing’s Syndrome?

Cushing’s Syndrome is a rare disease with an occurrence of one in millions.  Here in the united State we number a few thousand.  We are so rare that   even specialists in endocrinology are often taught they may practice their entire careers without seeing one of us.  And that is a big   problem for us.

With Cushing’s Syndrome the body produces too much of the hormone cortisol than the body needs or can handle.  That will cause the following symptoms:

·       Profound or even morbid truncal obesity (the most obvious symptom)

·       Round, or “moon” face

·       A fat pad on the upper back (often called a buffalo hump)

·       Stretch marks which are purple when fresh or may even continue to  stay purple of pink

·       Excessive sweating

·       Weakness and muscle mass loss

·       Hirsutism (large amounts of body hair)

·       Baldness and or dry and  brittle hair

·       Thinning of the skin

·       Insomnia

·       Tiredness, irritability and even mental problems

·       Reduced libidity, or sexual desire

·       Problems with periods in women

·       Excessive urination and thirst

·       Hypertension

·       Type II diabetes secondarily

·       Weakening of the bones due to osteoporosis

And this list is by no means complete as anyone with Cushing’s can tell you.  One also doesn’t need to have all or even most of the symptoms together to have the disease.

What causes Cushing’s Syndrome?

Simply put Cushing’s Syndrome is caused by tumors.  And the location of the tumor is the difference which determines whether doctors call it Cushing’s Syndrome, or Cushing’s disease.  The two most common places to have the tumors which cause Cushing’s are the pituitary gland at the base of the brain and the Adrenal glands, glands which sit on top of the kidneys.  In both cases the tumors produce a hormone messenger for the Adrenal glands to make more cortisols, ACTH.  The runaway production of ACTH causes the Adrenals to pump out massive doses of cortisols and the games begin.  The treatment for these tumors is to find them and surgically remove them.

However, there is a third category of tumors, ectopic tumors.  That is my problem in particular.  Those tumors can be anywhere in the body, literally.  However the most common place to find them is in the lungs as a form of cancer.  That’s not where mine is and it has defied all efforts to locate it so far.  Traditionally the treatment for ectopic Cushing’s if the tumor cannot be found is to remove both Adrenal glands and place the patient on hormone replacement therapy (HRT).  However that isn’t indicated in my case.  Why?

My Cushing’s Syndrome is an even rarer kind.  My overproduction of cortisols is only some of the time and even practically stops altogether part of the time.  This is known as Cyclical Cushing’s Syndrome, what used to be called “mild” Cushing’s Syndrome.  I get both the problems of Cushing’s Syndrome and its opposite disease, Addison’s Disease, the disease John F. Kennedy suffered from, but in what are considered milder ways.  Never mind I’m 353lbs as I write this and have back degeneration to the point my sciatic nerves is seriously pinched on both sides at times.  The end is the same, it’s just that the road to death is longer and drawn out.  Both diseases, by the way, are lethal if left untreated as mine is.

Generally speaking Cushing’s Syndrome isn’t some thing you can inherit.  However an inherited version called Familial Cushing’s Syndrome is known and extremely rare.  One of my brothers has Cushing’s Syndrome with and ectopic tumor like mine and there is evidence my mother, an Aunt and their Mother all may have suffered from this disease.  So mine is triply rare, which is why my Facebook Avatar is a zebra striped Rhinoceros.

The most common form of Cushing’s, though, is what is called exogenous, or medically induced Cushing’s Syndrome.  It simply comes from long-term use of high doses of steroids, most commonly cortisone, prednisone and other similar medication.  That’s the kind most easily accepted by doctors and one simply has to weight the benefits of continued therapy against the problems   with Cushing’s and take the lesser of the two evils.

Diagnosis

If your doctor agrees your symptoms suggest Cushing’s you get sent to a specialist in endocrine disorders, and Endocrinologist.  The endocrinologist will order testing of your hormones to try to determine if you have Cushing’s.  However, don’t forget the ditty about the hoof beats.  Doctors often won’t think outside the box and will look for more common problems such as PCOs (Polycystic Ovarian tumors) in women.  I’ve personally seen both General practitioners and Endocrinologists look right at testing results indicating Cushing’s and deny what they were seeing.  It’s hard being a zebra.    I’ve known about Cushing’s Syndrome and that I had the symptoms since 1983.  It took me until 2000 to get a diagnosis and the only treatment for the disease I’ve had since then was a recent removal of a tumor which could’ve been the cause.  It wasn’t.  Disappointment and frustration often goes with the territory when one has a rare disease. 

In the meantime I’ve been hospitalized with a pulmonary embolism, to have my gallbladder removed, for blood clots and to have a bone spur removed, all likely due to the Cushing’s.  I’ve been socially shunned by friends and family because of my large size.  And my closest family ties are strained as I grow physically weaker and am in and out of the Emergency room for other growing problems as my body literally comes apart right around me as the ravages of this disease advances.

This blog will be about Cushing’s Syndrome and about my journey with this disease until I either find the cure, or reach the end of the journey.  It is my earnest hope that those who find this blog will be informed on the disease, especially in the midst of this new “war on obesity” which threatens to make the Journey even harder and that some will find inspiration as well from a long-term suffer of this disease who is also a fighter.