Inheritance.

Posted on April 15, 2012 by MaryO

Inheritance..

Do pituitary issues run in families?

I responded to the above post with:

I posted the link above on my FB in the hopes that some of the folks I know with familial Cushing’s will respond to you. I know of several families like this.

After I had my pituitary surgery and my son started having some similar symptoms, I had him tested. Of course, at the time, the doctor said that there was no way that Cushing’s would run in families. Another thing we now know not to be true.

My son failed his Cushing’s testing at the time but even now he has symptoms of other endocrine issues. I have learned that my father was seeing a specialist for his endocrine issues when I was a child. He’s long gone now and my mother doesn’t remember, or won’t tell, what he was being tested for.

I wish you the best for you and your daughter.

via Inheritance..

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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 »

Stand Up and Be Counted!

Posted on April 10, 2012 by MaryO

For all Cushies, diagnosed or not, friends and family – add your name  and whatever info you want to share to this map.  The directions are similar to those for AI, below.

Cushing’s MemberMap

If you have Adrenal Insufficiency, a friend of mine from Adrenal Insufficiency United has a similar map.  Please add your info to her map, as well.

Adrenal Insufficiency Map

Directions for the AI map:

LET’S FIND EACH OTHER! (please read the instructions)

Use ONE MARKER PER PERSON WITH AI (to ensure an accurate representation do not add yourself as a parent or family member) However, If you have lost a loved one to AI, feel free to add them just indicate it in the description box.

After navigating to the map

CLICK “ADD” on top right of map

ENTRY NAME: Initials, first name, City…any of these are fine..or just leave it blank and it will say anonymous

LOCATION: if you don’t want your address known just “click on a map location”

I put my daughter in a park near our home :o) zoom in or out to find your location.

DESCRIPTION: Age of affected, or any other description you’d like

PHOTO: not required

MARKER Addison’s is the default so make sure you look at the whole list. If you have more than one condition which causes your or your childs’ AI, just pick the one you feel is the most relevant and then feel free to add the others in the Description box.

Please do not use ADRENAL INSUFFICIENCY UNITED’S marker. Right now it’s for our main office, but we’ll add more locations/contacts in the future.

CLICK SUBMIT

After you submit, write down the url link for future edits. Then click your marker, and then again click the BLUE initials, city, or anonymous…this will open up a more detailed window to add DOB and Diagnosis info plus anything else you like. Just remember if you put any personal email it will be able to be viewed by anyone.

If you mess up don’t fret, just contact me, as the admin of the map I can fix your entry.

 

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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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Remission of Cushing’s disease may improve body composition, reduce cardiovascular risk markers

Posted on April 5, 2012 by MaryO

Geer EB.  J Clin Endocrin Metab.2012;doi:10.1210/jc.2011-3123.

Patients in remission of Cushing’s disease may experience decreased total fat and improved cardiovascular risk factors, according to data from a prospective study.   

Researchers examined 14 participants (12 women, 2 men) with active Cushing’s disease before transsphenoidal surgery and 6 to 8 months after glucocorticoids (remission), using whole-body MRI to pinpoint patients’ lean and fat distributions. One patient required two transsphenoidal surgeries before entering remission.

Of the 14 participants, 13 had hypertension, which was confirmed by a resting blood pressure higher than 140/90 mm Hg or the use of antihypertensive medications. At follow-up, only three participants had persistent hypertension.

Most participants remained in the overweight (BMI >25 ) or obese (BMI >30) category.

Eliza B. Geer, MD, researcher for the division of endocrinology at the Mount Sinai School of Medicine, and colleagues, investigated the link between body composition and CV risk in the altered bodies of patients with Cushing’s disease.

According to the study, remission decreased visceral, pelvic bone marrow, subcutaneous (including trunk and limb) and total fat; waist circumference; and weight (P<.05). The researchers found that remission altered fat distribution, which resulted in decreased visceral/total fat (P=.04) and visceral fat/skeletal muscle ratios (P=.006). In addition, CV risk factors such as insulin resistance, leptin and total cholesterol decreased (P<.05).

“Prospective studies of CD [Cushing’s disease] are needed to understand possible associations between previous or persistent abnormalities in adipose tissue distribution and cardiovascular risk markers in patients successfully treated for CD [Cushing’s disease],” the researchers wrote.

Further studies are needed to examine the lack of change in adiponectin, C-reactive protein and other lipid measures.            

Disclosure: The researchers report no relevant financial disclosures.

 

 

Cushing’s disease alters fat distribution, muscle mass, adipokine profile and increases cardiovascular risk factors (serum insulin, glucose, leptin, high-molecular-weight adiponectin, C-reactive protein and lipid profile). Even though only a small group of 14 patients were studied, this is difficult to overcome in a rare disease like this.

They demonstrate that even though disease remission reduced most fat depots, dramatically improving body composition abnormalities, not all CV risk markers normalized completely; namely, adiponectin, C-reactive protein, and some lipid measures did not change after control of hypercortisolism. Furthermore, a decrease in skeletal muscle also persisted. This is interesting since one of the main complaints in patients “successfully” treated for Cushing’s disease is tiredness and fatigability, which persist beyond 1 year after surgery. This limits the patient’s exercise capacity and negatively affects their everyday life and subjective feeling of well-being, impairing their health-related quality of life.

Susan M. Webb, MD, PhD
Professor, Department of Medicine/Endocrinology
CIBER-ER, Unit 747 on Pituitary Diseases Hospital de Sant Pau
Universitat Autònoma de Barcelona, Spain

 

From http://www.endocrinetoday.com/view.aspx?rid=96175

 

 

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