Adrenal Insufficiency - Female Patient found Unconscious with Syringe Nearby

Crew’s misdiagnosis of drug overdose could have cost patient her life

Kimberly Doran | From the October 2012 Issue |

A call comes in to 9-1-1 dispatch. “Help” is all that’s spoken before the operator hears the phone hit the floor. The 9-1-1 dispatcher calls back only to get a busy signal. Police and EMS are dispatched for a well-being call.

On arrival, the front door is found to be slightly ajar. The crew knocks, but there’s no reply. They find a young woman  lying on the floor, naked, and in a pool of vomit. A syringe with an unknown substance is on the ground nearby. Suspecting a drug overdose, the EMS crew begins treating the patient for this condition. The patient is unconscious with emesis around her head and face. Her vital signs are blood pressure 60/45, heart rate of 130 bpm and respiratory rate of 10.

The patient shows no signs of waking. The crew clears the airway and administers oxygen. An IV is established, and the patient is readied for transport. As the crew leaves the scene, one of the medics turns to shut the door and sees a vial under a chair. He retrieves it and notes that the label says Solu-Cortef (a glucocorticoid). He bags it for the emergency department (ED). Following his instinct, he looks around the area for medications and finds two bottles. One is labeled dexamethosone and the other is labeled fludrocortisone. He takes his findings and rushes out the door into the awaiting ambulance. During transport, the patient continues to deteriorate.

The medic administers 0.5 mg of narcan and a 500mL bolus of normal saline with no response. He radios ahead to let the hospital know that they’re en route. Now questioning the original diagnosis of drug overdose, he reports the medications he found on the scene in hopes it will help the receiving physician determine the cause of the patient’s condition.

Arrival at the ED
On arrival to the ED, the medic hands over the loaded syringe containing 2mL of unidentified solution, as well as the empty vial of Solu-Cortef and the bottles of dexamethosone and fludrocortisones.

As the crew arrives at the hospital, the ED physician meets the crew and informs them that he’s familiar with the medications. He says they're all used for people who have various forms of adrenal insufficiency (AI). The symptoms seen in this patient coincide with life-threatening adrenal crisis. The physician administers 100 mg of Solu-Cortef via IV and within minutes, the patient rouses. In 30 minutes, she can explain what happened in the desperate moments before her crisis.

Adrenal Insufficiency
Adrenal Insufficiency (AI) is a life-threatening in which the body is unable to produce enough cortisol to sustain life. In other words, their adrenal cortex is “asleep.” People suffering from AI take daily cortisol/glucocorticoid steroid replacement because whatever adrenal function is depleted. These patients are glucocorticoid dependent. In times of injury, dehydration, illness or surgery, they require an injection of Solu-Cortef. Solu-Cortef contains both glucocorticoid and mineralocorticoid properties, helping the body to compensate during a stress event.

The adrenal medulla (inside of the adrenal gland) secretes epinephrine and norepinephrine. The adrenal cortex (outer layer of the adrenal gland) secretes cortisol and aldosterone. Cortisol, a glucocorticoid, is often called the “stress” hormone. One of cortisol's functions is elevating blood glucose levels in times of stress. It also functions as a mediator for several inflammatory pathways.

Absence of cortisol can result in hypotension, hypoglycemia and death. Aldosterone, a mineralocorticoid, is responsible for the regulation of sodium and water. Absence of aldosterone can result in hypotension and electrolyte imbalance. AI in the prehospital setting may be difficult to recognize in the absence of a good history, including medications, to point providers in the cause of the problem. Two life-threatening conditions associated with AI include hypotension and hypoglycemia.

If not managed, these two conditions are life threatening. Prehospital treatment should include management of the patient’s airway, vascular access and fluid resuscitation. If blood glucose levels are low, the patient should receive dextrose per local protocol. It’s important to complete a thorough physical assessment and obtain a complete patient history before treating patients with this condition. Providers may confuse patients having an adrenal crisis with drug overdose patients because of their similar symptoms. Although AI is rare, it should still be considered as a potential diagnosis.

Authors’ note: Parts of the above case are taken from a true story. However, the difference is that there was no syringe on the floor, no vial under the chair and no one found the medications. The patient was treated with charcoal and diagnosed as a drug-overdose patient. She likely would have died, but her mother charged into the ED and expressed the need for Solu-Cortef. Security was called, but luckily someone listened, researched and called the patient’s treating physician. The patient was treated and released. 

From Journal of Emergency Medical Services

Day Nine, Cushing's Awareness Challenge

Cushing's Conventions have always been special times for me - we learn a lot, get to meet other Cushies, even get referrals to endos!

As early as 2001 (or before) my pituitary function was dropping.  My former endo tested annually but did nothing to help me with the symptoms.

In the fall of 2002 my endo refused to discuss my fatigue or anything at all with me until I lost 10 pounds. He said I wasn't worth treating in my overweight condition and that I was setting myself up for a heart attack. He gave me 3 months to lose this weight. Those 3 months included Thanksgiving, Christmas and New Years.  Needless to say, I left his office in tears, again.

Fast forward 2 years to 2004.  I had tried for awhile to get my records from this endo. He wouldn't send them, even at doctors' or my requests.

I wanted to go see Dr. Vance at UVa but I had no records so she would't see me until I could get them.

Finally, my husband went to the former endo's office and threatened him with a court order, The office manager managed to come up with about 13 pages of records. For going to him from 1986 to 2001 including weeks and weeks at NIH and pituitary surgery, that didn't seem like enough records to me.

In April of 2004, many of us from the message boards went to the UVa Pituitary Days Convention. That's where the picture above comes in.  Other pictures from that convention are here.

By chance, we met a wonderful woman named Barbara Craven. She sat at our table for lunch on the last day and, after we learned that she was a dietitian who had had Cushing's, one of us jokingly asked her if she'd do a guest chat for us. I didn't follow through on this until she emailed me later. In the email, she asked how I was doing. Usually I say "fine" or "ok" but for some reason, I told her exactly how awful I was feeling.

Barbara emailed me back and said I should see a doctor at Johns Hopkins. I said I didn't think I could get a recommendation to there, so SHE referred me. The doctor got right back to me, set up an appointment. Between his vacation and mine, that first appointment turned out to be Tuesday, Sept 14, 2004.

Just getting through the maze at Johns Hopkins was amazing. They have the whole system down to a science, moving from one place to another to sign in, then go here, then window 6, then... But it was very efficient.

My new doctor was wonderful. Understanding, knowledgeable. He never once said that I was "too fat" or "depressed" or that all this was my own fault. I feel so validated, finally.

He looked through my records, especially at my 2 previous Insulin Tolerance Tests. From those, he determined that my growth hormone has been low since at least August 2001 and I've been adrenal insufficient since at least Fall, 1999 - possibly as much as 10 years! I was amazed to hear all this, and astounded that my former endo not only didn't tell me any of this, he did nothing. He had known both of these things - they were in the past records that I took with me. Perhaps that was why he had been so reluctant to share copies of those records. He had given me Cortef in the fall of 1999 to take just in case I had "stress" and that was it.

The new endo took a lot of blood (no urine!) for cortisol and thyroid stuff. I went back on Sept. 28, 2004 for arginine, cortrosyn and IGF testing.

He said that I would end up on daily cortisone - a "sprinkling" - and some form of GH, based on the testing the 28th.

For those who are interested, my new endo is Roberto Salvatori, M.D.
Assistant Professor of Medicine at Johns Hopkins

Medical School: Catholic University School of Medicine, Rome, Italy
Residency: Montefiore Medical Center
Fellowship: Cornell University, Johns Hopkins University
Board Certification: Endocrinology and Metabolism, Internal Medicine

Clinical Interests: Neuroendocrinology, pituitary disorders, adrenal disorders

Research Interests: Control of growth hormone secretion, genetic causes of growth hormone deficiency, consequences of growth hormone deficiency.

Although I have this wonderful doctor, a specialist in growth hormone deficiency at Johns Hopkins, in November, 2004, my insurance company saw fit to over-ride his opinions and his test results based on my past pharmaceutical history! Hello??? How could I have a history of taking GH when I've never taken it before?

Of course, I found out late on a Friday afternoon. By then it was too late to call my case worker at the drug company, so we had to appeal on Monday. My local insurance person also worked on an appeal, but the whole thing was  just another long ordeal of finding paperwork, calling people, FedExing stuff, too much work when I just wanted to start feeling better by Thanksgiving.

As it turned out the insurance company rejected the brand of hGH that was prescribed for me. They gave me the ok for a growth hormone was just FDA-approved for adults on 11/4/04. The day this medication was approved for adults was the day after my insurance said that's what is preferred for me. In the past, this form of hGH was only approved for children with height issues. Was I going to be a ginuea pig again?

The new GH company assigned a rep for me, submitted info to pharmacy, and waited for insurance approval, again.

I finally started the Growth Hormone December 7, 2004.

Was the hassle and 3 year wait worth it?

Stay tuned for Day 12, April 12, 2012 when all will be revealed.

 

Read Dr. Barbara Craven's Guest Chat, October 27, 2004

Thanks for reading :)

 

 

Interview with Leiana, Adrenal Patient

Leiana was diagnosed with autoimmune adrenal insufficiency  in 2009 and put on 30mg of Cortef for the rest of her life. Her cortisol levels were below normal of -1.    She has been trying to wean off the steroids with no success. 

She had a 3.9 adenoma on the right adrenal gland removed in Sept 2010 is waiting for the left adrenal gland to kick in. She is extremely skinny and bony and eats around 3000 to 4000 calories a day.

Read Leiana's bio here.

Listen Live at http://www.blogtalkradio.com/CushingsHelp

The Call-In number for questions or comments is (646) 200-0162.

Archives will be available after the interview

Adrenal Crisis Tips

From Ellen, on the Cushing's Help Message Boards

I have a very good friend who has had more adrenal crises than anyone care to count (more than 20). She has tried hard to teach me some important things for the day I should ever have a crisis. Among them is the reality that (as before surgery) success of your care depends on YOU getting everything prepared for the worst as best you can. We can no more depend on the ER staff than any other doc out there who isn't a specialist in pituitary medicine. You all have already done much of preparing by having your medic alert bracelets on, your injectable Cortef (bring it with you in case they don't have it there) and your letter from Dr. F. But that isn't enough much of the time as you have painfully discovered.

1) Prevention is the key. You, Mary, are SO overdoing it, I don't know what to say. You shouldn't even be leaving your house right now, let alone taking on the care of small children. You need a good talking to, missy. Perhaps you can choose to do ONE easy task a day but overall-you should be bored out of your gourd sitting on your tuckus. The more you do, the more you risk events like this. The hardest part is understanding that recovery is not a linear improvement every day. You are going to have weeks or maybe months where you can do no more than you did the first week after surgery. This recovery takes a long time when surgery works. Each tiny task you accomplish depletes you in an additive way. It might not have seemed much at the time to unload the dishwasher but you better believe it counts when you add in each additional task you want to accomplish.

2) Everything is additive. It isn't just what you did today but also what you did the last three, four or five days. You may have felt good the first day but each successive day my guess is you could feel your body pushing a bit. I find I say things like, "If I could just get this ONE more job done, then I will rest" before I am off to the next job. Before I know it, it is too late. Think hard about the twinges you feel the days before this happened this time around, when you were tired. How do you feel in the evenings after a day of activity? Those are the signs to look for and treat early the next time. You are having to listen to your body in a whole new way. Learn your earliest signs.

3) Take more Cortef when you first get those twinges above--the days before a crisis might strike.

4) Everyone in your household needs to be trained to give you Cortef. Teach them that confusion on your part indicates a crisis coming on-if you aren't making sense they need to understand that YOU are not able to help yourself. In most cases the oral cortef will keep you out of the ER if someone else makes sure you take it. Don't hesitate, don't let yourself talk them out of helping you-just take it-it is better to err high than low with your history of crises right now.

5) Knowing that in spite of all of this you need ER care potentially, consider calling the liason in person and talking real-time about your needs for future visits-explaining how quickly things become life threatening. They need to have something about your history in the computer already-a copy of that letter from Dr. F plus their own notes that it is on the up and up along with the note to please page your doctor. Give them a recipe to follow that they have pre-approved and it will help greatly. Most ER doctors will never see an adrenal crisis patient in their ER. Doctors have limits on their abilities just as everyone else does. I can read English really well but if you handed me a book written by someone in 1610, I would likely take longer to get through it because I am not as familiar with the format...the 'wherefore art thous' are English but they sure slow you down. That is what happens in the ER to Addisonians...doctors eventually get there but it takes longer because it is unfamiliar. Help them out by giving them the Cliff-Notes before you ever get there.

5A) Also insist that for now they order and keep Solu-Cortef on the shelves for you. Many (most?) hospitals do NOT have it stocked, as my friend discovered over and over again when she went to the ER. It took hours for them to track some down and give it to her. In the meantime, she was getting sicker and sicker. She finally asked the liason to help be certain they had it for her, ready to go. Now, they have it ready for her, they know her and they know what to do. She is often out of there in about 3 hours.

6) If you are vomiting/collapsed clearly in serious trouble, by the time you head off to the ER, call 911/ambulance so you get taken in and cared for without waiting. Your life is at risk by that stage and you need immediate care. It is justified and potentially life-saving.

7) Always have a trained advocate with you, have several back ups in your life. My friend has her husband but she also has me ready to go-have the hospital list several people to call ahead of time in case you arrive on your own and they can't reach your primary person. Your advocates need to know what to do independently of you. They need to know what to say and how to push the ER staff to get things done on your behalf. We, as patients struggling with Cushing's, are used to having to push but most people are very intimidated by medical personnel and often hang back, figuring they must know what to do. Make sure they understand this just isn't the case sometimes and that your life may depend on what they say or get the staff to do. It is critical they contact your endocrinologist-have your advocate INSIST they do this. If they won't, have your advocate page the doctor for you. Be sure you go over a plan with your advocate periodically or answer their questions about what may happen. I went over much of my plan with my husband prior to my surgery but discovered that within days of surgery, he had forgotten most of what I said. He really wanted to help but just hadn't taken in the medical stuff because it was overwhelming and scary. Keep going over it until they are comfortable.

I really hope these are the last ER visits for you all. I have agonized each time my friend goes into another crisis. I know that in spite of everything you do (or don't do) to prevent a crisis, they still happen. Hopefully the next time around, everything will be in place for you all to have a smooth experience.

So, sit down, turn on that television and get comfy girls. TAKE IT EASY!!

Cushing’s 22 Years, part 2

I just had my endo appointment on New Year’s eve, 2009.

Actually, it was kind of good having an appointment that day since there was no waiting, we good a great parking space in the garage, it was easy getting lunch afterwards at Baltimore’s Inner Harbor.

But, it’s always so disappointing.  If you look at other posts you’ll know I’m always checking the abstracts and news items to see if there’s anything that can help me.  There never is.

The big surprise was that my blood pressure was high which was really unusual for me. It has been very low since my cancer surgery. That, coupled with my enlarged lymph nodes was a red flag that something's not right. I'm supposed to do another CT scan in February to see if the lymph node has shrunk or gotten bigger - it's just staying the same, so it keeps getting scanned every 3 months. My only other option is a biopsy which sounds most unpleasant.

The main thing, which I knew anyway, was that there was nothing that could be done for my energy levels, since I can't take GH, except take more Cortef.

So, I'll still be tired and start gaining more weight.

I hate this! I feel like I'm half dead all the time :(

Part one of this post is here.

Cushie 12-Step Program

I “acquired” this from another site and changed the names to protect the innocent…

As you know many of us Cushies have had or have an addiction to Googling.

I suggest anyone who feels compelled to google symptoms go to the message boards to ask for support instead of typing these or any words such as "buffalo hump" in the google search engine. When this is done all roads lead to one thing...... You Eat Too Much!; You're Depressed! 

So, anyone who is about to hit enter on their computer which feeds their Google addiction.... go to the message boards to ask for help. One of the other Cushies who have achieved "Google sobriety" will help you down of the ledge.

1. Admit for now that you are powerless over your urge to Google.
2. Believe that a power greater than you (that would be other Cushies) can help you off that ledge
3. Decide to turn your "fingers" over to a higher power as you understand it.
4. Make a searching and fearless inventory of what you hope to gain from googling.
5. Admit to yourself and to another human being the exact nature of your addiction to Google.
6. Allow other Cushies to assist you when you are so scared you are thinking about googling symptoms.
7. Humbly ask for Xanax, Klonopin or Cortef in order to calm yourself from googling symptoms
8. Make a list of all the diseases you think you have or have had and survived and have a burning ceremony and then.... take a nap.
9. Cite all Google sites you have searched diseases on and delete them from your favorites/history.
10. When you start thinking....STOP..... do something else, like take an inventory of your test results.
11. Seek through prayer and/or meditation to improve your thought process. Do not travel to "OMG' or "What if" land (this is a serious suggestion).
12. Having had a spiritual awakening as a result of these steps, carry this message to all your Cushie friends and anyone else you know who has a Google addiction.

13. Now I know there isn't a 13th step, however, if you have noticed you are drinking too much wine or taking too many pills, please remember there are other 12 step programs for that, lol...

So, what about it - what are *your* suggestions to cure Google Addiction?

Why my house is a mess!

I always knew it wasn't my fault (entirely!)

From an email from The Organizer Lady:

THE BOREDOM OF ROUTINE TASKS

What's the matter with me? Am I just lazy? That is the question many women ask themselves as they see their friends who are quick to clean the cabinets, fold the laundry, wash the dishes, file the papers, or whatever dreaded mundane task they procrastinate doing.

The answer may lie in research which shows that some who dread these type of jobs have bodies that are low in adrenaline. These people can focus and accomplish jobs they find exciting or interesting so they often say they can do what they are interested in. But if they are not interested, the jobs go undone. This is because excitement stimulates the production of adrenaline and, voila, they suddenly feel like doing the job. Sometimes they lose interest before the job is completely done, the adrenaline drops, and the stuff sits around the house half done. Cleaning up after the job is completed is definitely boring. No fun in that! So the ebb and flow of interest regulates whether the job gets done. And the house shows the effect of it.

(Tomorrow - What to do about it.)

I wonder if tomorrow will tell me to up my Cortef to get a cleaner house.

My Life With Cushing's Disease

From http://lpn.advanceweb.com/editorial/content/editorial.aspx?cc=191157

My Life With Cushing's Disease
A healthcare provider shares her story.
By Kathie Harrington, MA, CCC-SLP

Cushing's might sound like something soft and fluffy; but it's quite the contrary. 

A rare condition that presents itself with many physical characteristics, Cushing's occurs most often in women ages 20-40, but can occur in males or in children and older adults, such as was the case for me.

I was diagnosed at 58.

I'd never heard of the disease until January 2007, when my endocrinologist confirmed some strange changes I was noticing with my body during a routine visit. He noted my "moon face" and the "buffalo hump" on the back of my neck as well as redness in my face and neck. He also realized my blood sugar levels were high even though I had been on 105 units of insulin per day due to my diabetes mellitus.  My extreme fatigue was also a concern, as was my weight gain and lack of muscle strength in my arms and legs. 

John Carmichael, MD, and Kathie at Cedars-Sinai, June 14, 2007.

Facing The Realization
When I was diagnosed, I was scared. After an MRI revealed a tumor on my pituitary gland it didn't take me long to realize that Cushing's is a very serious, life-threatening condition. But I also learned it often goes undetected and, thus, untreated. All healthcare professionals should know the signs of Cushing's disease in order to make/assist in an early diagnosis. 

Disease Prevalence
According to the Cushing's Support and Research Foundation (CSRF), an estimated 10-15 people are affected by the condition per every 1 million births. While Cushing's may never be a major portion of an LPN's patient caseload, it is important for nurses to be aware of its characteristics because it can co-exist with any disability, disease, or syndrome. As such, LPNs may find themselves in positions to assist in recognizing the disease.

Both Cushing's disease and Cushing's syndrome exist, both of which are the result of incorrect production of the steroid hormone cortisol, a hormone normally made by the adrenal glands and referred to as the "stress hormone" because it's typically produced by the body in response to stress. When produced it increases blood pressure and blood sugar, and reduces immune responses.

According to CSRF, Cushing's syndrome occurs when a tumor on the adrenal glands causes secretion of too much cortisol. Cushing's syndrome can also be caused by medications such as prednisone, which can limit and/or halt the level of cortisol the body is able to produce.

Cushing's disease occurs when one produces too much of the adrenocorticotropic (ACTH) hormone, which stimulates the adrenal glands and causes them to make too much cortisol. ACTH-producing tumors can originate elsewhere in the body (such as on glands, chest, thyroid or pancreas), but are most often found on the pituitary gland in both men and women. These tumors are almost never cancerous.

My Surgery
I had transsphenoidal surgery to remove the tumor and the gland at the Cedars-Sinai Medical Center, Los Angeles, in spring 2007. The procedure is very delicate in nature but is most often recommended, according to the Pituitary Network Association. It's carried out through the use of microscopic instruments that are inserted into the nose or upper lip and through the sphenoid sinus. I now take hydrocortisone, synthroid and desmopressin acetate.  The hydrocortisone is needed to replace cortisol in the absence of my pituitary gland. I take the synthroid for hypothyroid and desmopressin acetate for diabetes insipidus, which is a result of losing the pituitary.  I also wear a medical alert bracelet and carry a medical identification card in my purse. 

Despite the surgeries, I was back at work on a limited basis within 4 months. In spite of how all of this may sound, I never once experienced any pain. I never even had headaches (as some Cushing's patients report).   

Ongoing Recovery
According to my doctors, total recovery has been documented to take up to 3 years. Now, 14 months post surgery, I still experience muscle weakness, fatigue and dry skin, but I consider myself healthy.

I do get very cold easily, so I always make sure I have a sweater when I go out, and I had lost 30 pounds 2 months post-surgery because I had no appetite, but I've since gotten 10 back. Additionally, my insulin intake has gone from 105 units per day to 8 units and my blood pressure medication has been cut in half, which brings me within normal limits. I've lost my moon face and buffalo hump. As a bonus, I rarely have to shave my legs. (Thick, course hair is characteristic of Cushing's, but with the surgery comes normal conditions.) 

As an SLP of more than 25 years, I have found some language deficits in myself due to the surgery.  These deficits include word retrieval, memory and "language of confusion."  This confusion is worse on some days than on others and is characterized by interchanging the beginning or ending consonants of single words such as saying, "ford" or "chord" but meaning "board." It's weird, and I recognize it as it is happening and am sometimes able to self-correct it most of the time. I also practice many word-retrieval exercises, puzzles and watch old movies (which I love) to write down the actors, scenes, dialogue, etc., I can identify.

My concentration and word retrieval are also getting better and, today, I would judge these language deficits within 95 percent of where I was presurgery, unless I am very fatigued and it's difficult to concentrate. 

Relating To LPNs
I share this life experience with you and your fellow nurses because I want to implore you to be aware of your total patient. The root of someone's condition may not be what it appears to be. I was lucky because my Cushing's was originally suspected, not because of one individual characteristic but due to several seen together. I'm living proof that it can be cured.

Characteristics of Cushing's:

•           Abdominal weight gain

•           Hypertension

•           Poor concentration

•           Poor short-term memory

•           Irritability

•           Acne

•           Excessive hair growth (women)

•           Red, ruddy face and neck

•           Extra fat around neck (buffalo hump on back of neck)

•           Round face (moon face)

•           Fatigue/muscle weakness

•           Menstrual irregularity

•           Stretch marks on abdomen/thighs (purple)

•           Insomnia

•           Balding (women)

•           Hip and shoulder weakness

•           Swelling of feet/legs

•           Excessive hunger

•           Excessive thirst

•           Frequent urination

•           Diabetes

•           Depression

(Not all characteristics are found in any one person.)

Sleep, tired, nap, Sleep...

Seems like an endless cycle of either sleeping or being sleepy, napping or dozing off.  I miss the olden days when we used to have "project weekends" and get so much done, things built, rooms organized, going places, doing things, living.

My life now seems like it's a prelude to death.  I mostly can't stay awake for anything.

Yesterday I just slept most of the time.

I got up this morning and went to church - my kiddies sang at the first service, then another group rang at the second.  I left right after the bells and was home by 11:15AM.  And I napped until I had to leave for rehearsal at 4:45.  Home by 6:15 and - napped until dinner.  Ate and slept during TV. 

What a bore I must be.  Can't go anywhere, or do anything because I'm too tired. 

I cancel piano students because I'm too tired to clean stuff off  the piano before they come, don't call friends because I don't have the energy to talk. 

We run out of stuff because I'm to tired to go to the grocery and I can't remember when I vacuumed last.  And dust is everywhere.

Not that I'd spend my time cleaning if I had it but I'd like to make the choice myself what to do and not just depend on my lack of energy.

Maybe sometime I'll take a pile of Cortef and be done with it.  I'll gain my Cushie weight back again but maybe it would be worth it.

A Completely Different "Yesterday" Post

Wow.  That's about all I can say.  Yesterday was possibly the best day of my life since I started getting Cushing's symptoms, and that was over 25 years ago.  A quarter of a century of feeling exhausted, fatigued.  A quarter of my life spent taking naps and sleeping.

Last week  in this post I wrote in part: 

I went to the endo yesterday.  Nothing has changed for me.  Nothing will.  He wants me to take more cortef.  I don't want to gain weight again.  He looked up Provigil and it's not indicated for panhypopituitarism.  So he won't prescribe it.  My kidney surgeon probably won't let me take, anyway, but it was worth a try.

...

He did mention that in "only" 2.5 years maybe I can go back on growth hormone.  I don't want to live like this another year let alone 2.5.  But then, when I was on GH before it didn't help me like it helps most everyone else.

I'm tired of catering to a kidney that may or may not fail sometime anyway, tired of being so exhausted all the time.  I feel like I've lost nearly half my life to this Cushing's stuff already.

So, yesterday I was supposed to go to a conference on web design for churches.  My church sent me because they want me to spiff up their site and make them a new one for Christmas.  I wanted to go because, well, I like learning new stuff about the web.  I figured that I would learn stuff that would also be useful to me in others of my sites.

And I did!

But the amazing thing is this.  My son had told me  about a medication that was very similar to Provigil, that he had tried it while he was writing his doctoral thesis and it had helped him.

So, having tried the official doctor route and being rebuffed - again - I had decided to try this stuff on my own.

Just the night before I had written a response on Robin's wonderful blog that reads in part:

I hate this disease, too.

I was just talking to a friend today about how I'd try nearly anything - even if it ruined my one remaining kidney - to have a few days where I felt good, normal, where I could wake up in the morning rested and be able to have energy for the day.

I want to go out and have fun, to be able to drive for more than 45 minutes without needing to rest, to be have people over for dinner, whatever. I hate being restricted by my lack of energy.

My endo says to cheer up. In two and a half years I can try the growth hormone again. Whoopee. Didn't work the first time and maybe gave me, or contributed to, cancer growth. Why would I want to look forward to trying that again?

I want to feel good now. Today.

I hate that this disease kills but I also hate that it's robbed me of half my life already.

I wish doctors would understand that even though we've "survived", there's no quality of life there.

I hate Cushing's. It robs so much from so many of us. :(

As I said earlier, I have a history of daily naps of at least 3 hours a day.  It cuts into everything and prevents me from doing many things.  I have to schedule my life around these naps and it's awful.

A few years ago I went on a Cushie trip to Rockford.  I've been there a few times and it's always so much fun.  But this first year, we were going to another Cushie's home for barbecue.  I didn't drive, I rested in the back of the car during the drive.  We got there and I managed to stay awake for a little while.  Them I put my head down on the dining room table and fell asleep. Our hostess kindly suggested that I move over to the sofa. 

So, I have a long history of daily naps, not getting through the day, yadda, yadda.

So, I was a little nervous about yesterday.  I really wanted to go to this conference, and was afraid I'd have to go nap in my car.

I got up at 5:30 am yesterday.  Before I left at 7:15, I took my Cortef and then I took my non-FDA approved simulated Provigil.  (Although it's not FDA approved, it is not illegal to possess without a prescription and can be imported privately by citizens)

I stayed awake for the whole conference, went to a bell rehearsal, did Stacey's interview, had dinner and went to bed about 10:30PM.  NO NAP!  I did close my eyes a little during the 4:00PM session but it was also b-o-r-i-n-g.

I stayed awake, I enjoyed myself, I learned stuff, I participated in conversations (completely unlike shy me!).

I felt like I think normal people feel.  I was amazed.  Half my life wasted and I finally (thank you Michael!) had a good day.

My kidney doctor and my endo would probably be appalled but it's about time that I had some life again!  Maybe in another 25 years, I'll take another pill.  LOL

Jake's Cushing's Disease Story

http://jakesilvershow.blogspot.com/2008/07/my-cushings-disease-story.html

Hi. My name on the internet is Jake Silver. Some of you know me. Some of you know this Blog. Some of you have never visited before, and that's okay. Today I am going to tell you about my battle with CUSHING'S DISEASE.

It probably all started in 2005. I began to gain a lot of weight very rapidly and only on my face and stomach. I gained about 30 pounds and weighed about 170 at one point. I started this Blog NOV 2005 and throughout I struggled with my weight. I didn't know it was a precursor to more insidious problems to come. My blood pressure rose and I got stretch marks on my thighs and butt.

In the summer of 2006 I began to have serious back pain. I thought maybe my workout routine was imbalanced so I tried to do more back exercises. I also thought carrying my new son (now 2 1/2) contributed to the problem. But it did not go away and in OCT '06 I went to see a Doctor who said I wasn't stretching right. He sent me to Physical Therapy and prescribed Motrin. They gave me a stretching routine to do which felt nice but didn't alleviate my back problems. I returned to the Doctor in DEC '06 and he prescribed more stretching, Motrin and muscle relaxers.

In FEB I suffered a staph infection after shaving...and in March my chest popped while doing dips.

In the summer of 2007 my back pain continued to get worse and now my feet were killing me as well. They sent me to a Spine Specialist who said I had some disc deteroration and some kind of early onset arthritis. At this point my main doctors ran tests for Rhematoid Arthritis and some other nasty things. One nurse suggested it might be Fibromyalgia.

In fall 2007 I was pushing on some wolling equipment at work we call AGE and I was using my chest for leverge and my chest popped in. OUCH!!! I went to the doctor and they discovered I had also fractured 7 ribs in the back at some point earlier.

That is when they questioned me about my blood pressure and my weight...

I mean, here is another photo from my Cushing's Dayz:

and then me a few months after my surgery:

In November 2007 they finally did a 24 Urine test and discovred I had way too high levels of Cortisol, which indicated Cushing's Syndrome.

They referred me to an Endocrinologist, who is brilliant, and he repeated the test to confirm it. Then we had to figure out what was cauing it. I wasn't on any steroids... Well in Nov around Thanksgiving I completely threw my back out and laid on the couch for two days. Then it happened again.

I had to take leave from work... and it turned out that leave lasted for 6 MONTHS. My back was just "gone". It was too painful to stand or sit up so I laid on the couch and crwled everywhere. I tried to do exercise and Physical Therapy but after a month or so I could no longer get in th car at all. I had a walker and a wheelchair.

My wife was a saint and an angel the whole time... encouraging me and taking care of everything.. Tathan, the chores, my medical stuff and my professional stuff. My unit and Squadron was excellent the whole time. At first I didn't tell them what was going on but after my doctor's had misdiagnosed my symptoms and wrote me off as another airman trying to get out of work I was upset. My tests came back showing I had severe osteoporosis and my bones were extremely fragile. One I told my squadron about my problems, my first shirts literally came to my rescue. I will never ever be able to repay all that they did and continue to do for me and my family.

Also my Mom moved in for a few months to help with the house and to boost my spirit. Her company was very valued during this trial. And my Dad and step-mom also came in and helped me with transporation and morale.

I was on muscle relaxers and, after a nasty reaction to Tramadol AKA Ultram, I was on Lortab. (Vicoden)

In Jan 08 I was sent to Eglin for tests. My Dad took me and they said they could not find any growths on my adrenal glands (one of the causes of Cushing's)... and my brain scans were inconclusive. But I do have to say that Eglin Medical is the epitome of AF "Excellence".

In Feb 08 I went to the University of Alabama MedicAL center and they did this weird test where they send tubes through your groin to your brain and take blood or something to figure out IF there is a tumor in your Pituitary Gland and if so, which side.

Well the first time they jacked it up, which they graciously admitted. The second time (which they paid for) they detected the tumor, so my surgeon scheduled me for surgery... which by the way on the MORNING of my surgery TRICARE had not agreed to pay for but my First Sgt. saved the day again and fixed it within the hour.

After the surgery I thought everything would be back to normal but that didn't happen... instead I was in so much pain I was barely mobile at all. THEN in March I suffered a seizure and had to be hospitalized. After I woke up from my seizure I was in more pain than ever. So they sent me to an inpatient rehab facility run by HealthSouth. They helped me roll over, sit up and start walking again.

All those things you take for granted like getting up to go get a soda or use the bathroom on your own... believe me I am very grateful for.

After my release frm HealthSouth, I was trying to get off steroids to fast and I got really sick. I could barely eat andI started throwing up all day. So I went to the hospital two weekends in a row.

The weekend of April 26 was my birthday and I decided my best present would be to go back to work ... so on May 1st I went back to work with a cane and a profile. I have only stayed home once... the next day!! From being so sore.

On July 8th I returned to the flightline and to AMU Physical Training. My current profile says I can't run... (I can only jog for a few minutes before the jarring is too much.) No Sit-Ups (crunches are fine) and I can't lift anything really heavy. About 45 pounds is my limit. I am working and fixing planes everyday.

I am off all drugs and medications... I take Tylenol maybe 3x a week. I am taking vitamins and supplements like Calcium and Glucosamine and MSM. I did try stopping my Cortef completely, but I started getting really tired and very itchy skin and more aches. So I am taking it again.

The AF Informal Physical Evaluation Board returned a decision to Temp Retire me for 15 months with 100 percent pay and benefits with a Re-Eval in 15 months. But I am appealing it on principle. I can fix jets right now with no problems so I might as well do my job the taxpayers pay me to do instead of have a free vacation.

Stay tuned? and any questions ask me : jakesilvershow@yahoo.com

Comments: http://jakesilvershow.blogspot.com/2008/07/my-cushings-disease-story.html

Exhaustion, again

Today, we went out to "our" farm again. We've been members of this CSA (consumer supported agriculture) farm for 13 years now.

It's wonderful! They deliver fresh, in season, no pesticides veggies, various fruits and berries from May through October, once a week. The other days, we can go out anytime we want and pick whatever is available.

I love just going out there, even if I don't feel like picking. It's wonderful to get away, out to "our" farm, and see the animals, and the veggies...very soothing and healing.

The first year we belonged, I did a comparison study of several of the local stores, and carefully weighed everything. The farm was a bargain.

In addition to getting all this stuff, we've made some new friends, I've tried some new veggies and herbs - and I think it's important to support the small local farmer like this - and to have a source of food that hasn't been sprayed with chemicals.

Anyway, we went out again today. We haven't been so much since our son went away to college. But just driving out into the country is so refreshing.

For the last several years, I've had trouble getting back from picking stuff. I could hike out to the fields ok and pick but coming back, I'd have to stop and sit by the side of the dirt road, sometimes even lying down. After a while of this, I found that taking an extra Cortef would help. I found that eating a raw green pepper would sometimes help, too. I don't know if it was the heat, the walk, the 'work" of picking or some combo that caused this to happen.

Today seemed better. We went to the peach orchard and picked about 10 pounds of peaches which we took back to the car. My husband wanted to stop by the blackberries - just a little out of our way. I wanted to take the farm wagon out for the blackberries. In addition to the field we knew, there was supposed to be a new one, further away.

So...we got back to the car and put our peaches away. We waited a while for the wagon but my husband got impatient and wanted to walk back to the berries. I gave him the boxes and told him I'd wait on the porch, in a rocking chair, in the shade, thank you.

I finally summoned up some more energy and went out and helped him finish the blackberry picking. On the way home, we were talking about how I seemed to have more energy, that I had made it back after the peaches but I reminded him that it was cooler than it often is and that the peaches were about halfway to the fields where we've usually picked vegetables. So, maybe my energy level isn't really any better.

We got home about 5. And I napped until 9:45. Some improvement - HA!

Music Camp is done!

I posted last week about my energy levels and music camp teaching. I came out of it better than I thought I would but I ended up having a headache from Friday until Sunday. And, I'm not supposed to take any meds without approval from my kidney surgeon. So, I toughed out the headache.

The first day was a nightmare - one of the 6th graders started crying during the first handbell rehearsal. I don't blame her, either. She was the only one in the group who had never rung before. Her class had also come directly from Orff class and I guess that some experience would have helped there, too.

The second day our church's assistant music director got sick with a high fever and didn't come in. Actually, she didn't make it for the whole rest of the week. So, there was a lot of shuffling around of classes and some students were moved from one class to another. Somehow, we got through the day. I had to take a bit of extra Cortef to make it until naptime.

By Wednesday I figured that I could have 2 groups ready to play something on Friday, both with a water theme. So, the second graders would play Lightly Row and the sixth (even the cryer!) would play The Water is Wide, both by that famous composer - Traditional.

Thursday I was finally getting the hang of all these different classes. There was virtually no time to set up between class which made it a little tough since every class had different pieces and some had chimes instead of bells, some doubled different parts, some had TAs filling in. But it worked out!

Friday was mostly spent on the dress rehearsal and show. My kids did great playing their pieces before the show. My mom even came to see!

I left Friday with such a headache. It started in the right side of the back of my neck, then worked its way up to the left side of my forehead by Sunday afternoon.

Sunday, the kids performed a couple songs from the musical at all 3 church services. My husband even came to the first service. Even rarer than my mom coming to the performance. Since the assistant director was still out sick, I stayed for all 3 services as a second director in case the kids looked my way.

All in all, a success!

Things must be getting better. When I first started music camp about 5 years ago I would sleep until about 11:30am, go to camp, get home about 4:30 and sleep and sleep. I think I was on growth hormone by then, too.

I was definitely on cortef again at that point. I had been off for something like 15 years after my pituitary surgery, then the scar tissue caused the pituitary to not work so well so my endo had me go back on the cortisone. He wanted me to take a lot more but when I did, the Cushing's symptoms - including weight gain and that hump - started coming back.

So, I'm on the smallest possible dose. It's not enough to help me feel better but not enough to bring on Cushing's. When I'm "stressed" I'm supposed to up the dose. The growth hormone was supposed to help with my energy and weight issues but it did neither.

Then 2 years ago I had just had my kidney cancer surgery and I was still tired from that experience - and newly off the growth hormone. (Because of the cancer, I can't take growth hormone) I got through camp, just barely.

So, maybe next year, if I still get "volunteered" I'll be able to do this without any extra cortisone and no headaches.