Day Eleven, Cushing’s Awareness Challenge

Posted on April 11, 2012 by MaryO

In March of 1987, after the endo finally  confirmed that I had Cushing’s, I saw sent to a local hospital where they repeated all those same tests for another week and decided that it was not my adrenal gland (Cushing’s Syndrome) creating the problem. The doctors and nurses had no idea what to do with me, so they put me on the brain cancer ward.

When I left this hospital after a week, we didn’t know any more than we had before.

As luck would have it, NIH (National Institutes of Health, Bethesda, Maryland) was doing a clinical trial of Cushing’s. I live in the same area as NIH so it was not too inconvenient but very scary at first to think of being tested there. At that time I only had a choice of NIH, Mayo Clinic and a place in Quebec to do this then-rare pituitary surgery called a Transsphenoidal Resection.

My husband asked my endo if it were his wife, if he would recommend this surgery.  The endo responded that he was divorcing his wife – he didn’t care what happened to her.  Oh, my!

I chose NIH – closest and free. After I was interviewed by the Doctors there, I got a letter that I had been accepted into the clinical trial.

The night before I was admitted, I signed my will.  I was sure I was going to die there.  If not during testing, as a result of surgery.

The first time I was there was for 6 weeks as an inpatient. More of the same tests.

There were about 12 of us there and it was nice not to be alone with this mystery disease. Many of these Cushies (mostly women) were getting bald, couldn’t walk, having strokes, had diabetes. One was blind, one had a heart attack while I was there. Several were from Greece.

Towards the end of my testing period, I was looking forward to the surgery just to get this whole mess over with – either a cure or dying. While I was at NIH, I was gaining about a pound a day!

During the time I was home the weekend  before surgery, a college classmate of mine (I didn’t know her) DID die at NIH of a Cushing’s-related problem. I’m so glad I didn’t find out until reading the alumnae magazine a couple months later!  She was the same class, same major, same home-town, same disease…

We have a Scottish doctor named James Lind to thank for the clinical trial.  He  conducted the first ever clinical trial in 1747 and developed the theory that citrus fruits cured scurvy.  Lind  compared the effects of various different acidic substances, ranging from vinegar to cider, on groups of afflicted sailors, and found that the group who were given oranges and lemons had largely recovered from scurvy after 6 days.

I’d like to think that I advanced the knowledge of Cushing’s at least a little bit by being a guinea  pig in 1987-1989.

From the NIH: http://endocrine.niddk.nih.gov/pubs/cushings/cushings.aspx

Hope through Research

Several components of the National Institutes of Health (NIH) conduct and support research on Cushing’s syndrome and other disorders of the endocrine system, including the National Institute of Diabetes and Digestive and Kidney Diseases, the National Institute of Child Health and Human Development (NICHD), the National Institute of Neurological Disorders and Stroke, the National Cancer Institute, and the National Center for Research Resources.

NIH-supported scientists are conducting intensive research into the normal and abnormal function of the major endocrine glands and the many hormones of the endocrine system. Researchers continue to study the effects of excess cortisol, including its effect on brain structure and function. To refine the diagnostic process, studies are under way to assess the accuracy of existing screening tests and the effectiveness of new imaging techniques to evaluate patients with ectopic ACTH syndrome. Researchers are also investigating jugular vein sampling as a less invasive alternative to petrosal sinus sampling. Research into treatment options includes study of a new drug to treat the symptoms of Cushing’s syndrome caused by ectopic ACTH secretion.

Studies are under way to understand the causes of benign endocrine tumor formation, such as those that cause most cases of Cushing’s syndrome. In a few pituitary adenomas, specific gene defects have been identified and may provide important clues to understanding tumor formation. Endocrine factors may also play a role. Increasing evidence suggests that tumor formation is a multistep process. Understanding the basis of Cushing’s syndrome will yield new approaches to therapy.

The NIH supports research related to Cushing’s syndrome at medical centers throughout the United States. Scientists are also treating patients with Cushing’s syndrome at the NIH Clinical Center in Bethesda, MD. Physicians who are interested in referring an adult patient may contact Lynnette Nieman, M.D., at NICHD, 10 Center Drive, Room 1-3140, Bethesda, MD 20892-1109, or by phone at 301-496-8935. Physicians interested in referring a child or adolescent may contact Constantine Stratakis, M.D., D.Sc., at NICHD, 10 Center Drive, Room 1-3330, Bethesda, MD 20892-1103, or by phone at 301-402-1998.

 

Filed under: Clinical trials, Cushing's, pituitary | Tagged: , , , , , , , , , , , | 1 Comment »

Day Nine, Cushing’s Awareness Challenge

Posted on April 9, 2012 by MaryO

UVA 2004
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 🙂

 

MaryO

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Day Four, Cushing’s Awareness Challenge

Posted on April 4, 2012 by MaryO

The Seven Dwarves of Cushing's

So, these are only seven of the many, many symptoms of Cushing’s.  I had those above – and I often felt like I looked like one of those little bearded dwarves.

Cushing’s affects every part of the body.  It’s not like when I had kidney cancer and only the kidney was affected.

Here’ are some of the many areas affected.

  • Progressive obesity and skin changes
  • Weight gain and fatty tissue deposits, particularly around the midsection and upper back, in the face (moon face) and between the shoulders (buffalo hump). Some symptoms such as sudden weight gain, are caused by excess cortisol. The excess cortisol in the body does not increase protein and carbohydrate metabolism. It slows or nearly disables metabolism function, which can cause weight gain (fat accumulation) in the buttocks, abdomen, cheeks, neck, or upper back.
  • Loss of muscle mass. Some areas of the body, such as the arms and legs, will remain thin.
  • Pink or purple stretch marks (striae) on the skin of the abdomen, thighs, breasts and arms
  • Thinning, fragile skin that bruises easily
  • Slow healing of cuts, insect bites and infections
  • Acne

Women with Cushing’s syndrome may experience:

  • Thicker or more visible body and facial hair (hirsutism)
  • Irregular or absent menstrual periods

Men with Cushing’s syndrome may experience:

  • Decreased libido
  • Decreased fertility
  • Erectile dysfunction

Other signs and symptoms include:

  • Fatigue
  • Muscle weakness
  • Depression, anxiety and irritability
  • Loss of emotional control
  • Cognitive difficulties
  • New or worsened high blood pressure
  • Glucose intolerance that may lead to diabetes
  • Headache
  • Bone loss, leading to fractures over time
  • Hyperlipidemia (elevated lipids – cholesterol – in the blood stream)
  • Recurrent opportunistic or bacterial infections
Think you have Cushing’s?  Get to a doctor and don’t give up!

MaryO
         MaryO

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Cushing’s FAQ

Posted on May 21, 2011 by MaryO

A FAQ (Frequently Asked Questions).  Directions are in each category for adding your own question.

IMPORTANT: The information and material posted on this Web site is intended as general reference information only. Specific facts and circumstances may alter the concepts and applications of materials and information described herein. The information provided is not a substitute for professional advice and should not be relied upon in the absence of such professional advice specific to whatever facts and circumstances are presented in any given situation.

Please note that there are several questions waiting to be answered at this time. Your question will be answered as soon as possible.

This is a different website than the message boards and requires a different log-in, although you may use the same log-in name and password.

 

Adrenal Insufficiency

Adrenal insufficiency is a life threatening chronic illness. An active and vigorous lifestyle with normal life expectancy is possible as long as the prescribed medications are taken regularly and adjusted when indicated. As with most chronic diseases, adrenal insufficiency demands that the patients take responsibility and develop self-management skills and techniques.

Read an article on Adrenal insufficiency

Cushing’s Types

Cushing’s Disease/ Cushing’s Syndrome: Cushing’s is a hormonal disorder caused by prolonged exposure of the body’s tissues to high levels of the hormone cortisol. Your adrenal glands, which are right above your kidneys, release cortisol when they receive a chemical message from your pituitary gland. The message comes in the form of adrenocorticotrophic hormone (ACTH), which travels through the bloodstream.

Cushing’s Disease Is the result of a pituitary tumor which causesthe emergence of secondary male characteristics (like hair growth, acne, etc.), and ovarian failure. Other symptoms usually include high blood pressure and water retention.

Cushing’s Syndrome: Causes the same symptoms, but is a disorder marked by overproduction of adrenal hormones, which can cause a drop in LH and FSH.

An estimated 10 to 15 of every million people are affected each year. Cushing’s is an increased concentration of glucocorticoid hormone (ACTH) in the bloodstream that is being produced by an adrenal gland tumor (adenoma). Ectopic Cushing syndrome refers to the production of ACTH in a location other than the pituitary gland or adrenalgland. Examples of ectopic sites include thymoma, medullary carcinoma of the thyroid, pheochromocytoma, islet cell tumors of the pancreas, and oat cell carcinoma of the lung.Symptoms include weight gain, central obesity, moon face, weakness, fatigue, backache, headache, increased thirst, increased urination, impotence, mental status changes, and muscle atrophy.

Treatment varies with cause. If an ACTH secreting tumor is involved then it must be removed surgically.

More about Cushing’s.

Talk about Cushing’s with people who understand.

Subcategories:

Growth Hormone

Human Growth Hormone (hGH) is produced in the pituitary gland of humans, and the hormone is secreted throughout a person’s lifetime. It promotes growth in children and plays an important role in adult metabolism.

More about HgH

Where Can I Find…?

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Medical treatment of Cushing’s disease: Overview and recent findings

Posted on October 21, 2009 by MaryO

Published Date October 2009

Journal: International Journal of General Medicine

Stephanie Smooke Praw1, Anthony P Heaney1,2

1Department of Medicine, 2Department of Neurosurgery, David Geffen School of Medicine at UCLA, Los Angeles, CA, USA

Abstract: Cushing’s disease, due to pituitary adrenocorticotropic hormone (ACTH) hypersecretion, is the most common etiology of spontaneous excess cortisol production. The majority of pituitary tumors causing Cushing’s disease measure <1 cm and the excess morbidity associated with these tumors is mostly due to the effects of elevated, nonsuppressible, ACTH levels leading to adrenal steroid hypersecretion. Elevated circulating cortisol levels lead to abnormal fat deposition, hypertension, diabetes, coronary artery disease, osteoporosis, muscle weakness and psychological disturbances. At experienced centers, initial surgical remission rate via transnasal, transphenoidal resection approaches 80% for tumors less than 1 cm, but may be as low as 30% for larger lesions and long-term recurrence in all groups approaches 25%. Residual disease may be managed with more radical surgery, pituitary-directed radiation, bilateral adrenalectomy, or medical therapy. This paper addresses current and novel therapies in various stages of development for Cushing’s disease.

Keywords: Cushing’s disease, treatment, pasireotide, PPAR-γ, 11 β-hydroxysteroid dehydrogenase inhibitors, dopamine agonists

Download article (Free!): http://dovepress.com/getfile.php?fileID=5388

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