27 Haziran 2012 Çarşamba

Day Nineteen, Cushing's Awareness Challenge

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In Day 10 on April 10, 2012, I wrote about how we got the Cushing's colors of blue and yellow.  This post is going to be about the first Cushing's ribbons.

 

I was on vacation  in September, 2001 when SuziQ called me to let me know that we had had our first Cushie casualty (that we knew about).

On the message boards, Lorrie wrote: Our dear friend, Janice died this past Tuesday, September 4, 2001. I received an IM from her best friend Janine, tonight. Janine had been reading the boards, as Janice had told her about this site, and she came upon my name and decided to IM me. I am grateful that she did. She said that she knew that Janice would want all of us to know that she didn't just stop posting.

For all of the newcomers to the board that did not know Janice, she was a very caring individual. She always had something positive to say. Janice was 36 years old, was married and had no children. She had a miscarriage in December and began to have symptoms of Cushing's during that pregnancy. After the pregnancy, she continued to have symptoms. When discussing this with her doctor, she was told that her symptoms were just related to her D&C. She did not buy this and continued until she received the accurate diagnosis of Cushing's Syndrome (adrenal) in March of 2001. Tragically, Janice's tumor was cancerous, a very rare form of Cushing's.

Janice then had her tumor and adrenal gland removed by open adrenalectomy, a few months ago. She then began chemotherapy. She was very brave through this even though she experienced severe side effects, including weakness and dizziness. She continued to post on this board at times and even though she was going through so much, she continued with a positive attitude. She even gave me a referral to a doctor a few weeks ago. She was my inspiration. Whenever I thought I had it bad, I thought of what she was dealing with, and I gained more perspective.

Janice was having difficulty with low potassium levels and difficulty breathing. She was admitted to the hospital, a CT scan was done and showed tumor metastasis to the lungs. She then was begun on a more aggressive regimen of chemo. She was discharged and apparently seemed to be doing well.

The potassium then began to drop again, she spiked a temp and she was again admitted to the hospital. She improved and was set to be discharged and then she threw a blood clot into her lungs. She was required to be put on a ventilator. She apparently was at high risk for a heart attack. Her husband did not want her to suffer anymore and did not want her to suffer the pain of a heart attack and so chose for the doctors to discontinue the ventilator on Tuesday. She died shortly thereafter.

Janice was our friend. She was a Cushie sister. I will always remember her. Janine asked me to let her know when we get the Cushing's ribbons made as she and the rest of Janice's family would like to wear them in her memory. She said that Janice would want to do anything she could to make others more aware of Cushing's.

The image at the top of the page shows the first blue and yellow ribbon which were worn at Janice's funeral.  When we had our "official ribbons" made, we sent several to Janice's family.

Janice was the first of us to die but there have been more, way too many more, over the years.  I'll write a bit more about that on Day 21.

 

Day Twenty, Cushing's Awareness Challenge

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This is one of the suggestions from the Cushing's Awareness Challenge post:

What have you learned about the medical community since you have become sick?

This one is so easy.  I've said it a thousand times - you know your own body better than any doctor will.  Most doctors have never seen a Cushing's patient, few ever will in the future.

If you believe you have Cushing's (or any other rare disease), learn what you can about it, connect with other patients, make a timeline of symptoms and photographs. Read, take notes, save all your doctors notes, keep your lab findings, get second/third/ten or more opinions.

This is your life, your one and only shot (no pun intended!) at it.  Make it the best and healthiest that you can.

When my friend and fellow e-patient Dave deBronkart learned he had a rare and terminal kidney cancer, he turned to a group of fellow patients online  and found a medical treatment that even his own doctors didn't know. It saved his life.

In this video he calls on all patients to talk with one another, know their own health data, and make health care better one e-Patient at a time.

 

Have You Learned About Cushing's At a Health Fair?

To contact us Click HERE

I cannot imagine this myself, since Cushing's is so hard to diagnose but an article at yourdailyjournal.com claims: 

The blood profiles provide a comprehensive look at several physiological systems in the body at a cost that is very reasonable for the patients, all in one panel,” Laboratory Manager Rhonda Outlaw said. “The cost savings would amount to anywhere from $185 to $1,100, depending on the tests done and whether they were drawn at a physician’s office or on an outpatient basis.

 

“The panel itself will give indication of possible problems with kidney functions, hematological functions, like anemia, platelet function and infection; cardiovascular disease, thyroid functions, diabetes detection, liver functions and electrolyte function, like Cushings Syndrome, potassium regulation and dehydration detection.”

What do you think about this?


 

 

Cortendo Receives Positive Orphan Drug Opinion from EMA for NormoCort for Cushing’s Disease

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Cortendo AB with support from their preclinical development partner, PharmaDirections, Inc. received a positive opinion from the European Medicines Agency for NormoCort.

Radnor, PA (PRWEB) June 26, 2012

Cortendo AB [ticker: CORT on the Norwegian NOTC-A], a biopharmaceutical Corporation focused on the development of new therapies in the field of Metabolic Diseases, obtained a positive opinion by the European Medicines Agency's Committee for Orphan Medicinal Products, on its application for orphan drug designation for NormoCort (COR-003) for the treatment of hypercortisolism (Cushing’s Syndrome). The positive opinion of the COMP for NormoCort has now been forwarded to the EU commission for final approval and publication in the EU community register. With orphan drug designation granted in the US by the FDA in March and now with this positive opinion from the EU’s COMP, Cortendo is well positioned to move NormoCort into pivotal global clinical trials in Cushing’s Syndrome.

Cortendo is a biopharmaceutical company that relies in part on quality consultants and CRO’s to support the research and development of its pipeline. For the past year, Cortendo has contracted with PharmaDirections for a number of key services ranging from CMC to US and European Regulatory support. PharmaDirections’ regulatory services have ranged from the successful preparation and support to orphan drug designation applications in both the US and Europe to support with both IND and CTA preparation. “Cortendo has appreciated the high quality of support particularly in the areas of regulatory, CMC, and project management services offered by PharmaDirections”, said Dr. Ted Koziol, COO of Cortendo.

“Our Cortendo relationship is a great example of a virtual company using outsourced resources to their maximum advantage” said Dr. Richard Soltero, President of PharmaDirections.

About Cortendo:

Cortendo is a pioneer in the field of cortisol inhibition. The development of the lead drug candidate NormoCort (COR-003), the 2S,4R-enantiomer of ketoconazole, has been directed to Cushing’s Syndrome. The company’s strategy is to focus its resources to opportunities where the path to commercialization or partnership is clear and relatively near-term. Strategically, Cortendo’s business model is to commercialize relevant opportunities in the United States while partnering its assets ex-US. Backed by a highly experienced leadership team Cortendo has plans to continue to implement its pipeline expansion efforts in osteoarthritis and diabetes, as well as other near term revenue opportunities.

About PharmaDirections:

PharmaDirections, Inc. provides pharmaceutical consulting and project management services with a focus on preclinical development, formulation development and CMC, and regulatory affairs. The company was founded in 2003 and is based in Cary, North Carolina.

From PRWeb

25 Haziran 2012 Pazartesi

MEN1 and pituitary adenomas

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Abstract

MEN1 gene mutations predispose carriers to pituitary tumors. Molecular pathways involved in the development of these tumors seem different to what is known in sporadic tumors. Clinical studies showed that all types of adenomas can be found with a predominance of prolactinoma and macroadenoma compared to a control population.

These MEN1 tumors seem more aggressive, invasive and resistant to treatment requiring a very careful long-life follow-up. Occurrence of these tumors can be described in the pediatric population and it can be the first and only manifestation of MEN1 for some years asking the question of the systematic screening for MEN1 gene mutation in pediatric population with pituitary adenoma.

More at http://www.sciencedirect.com/science/article/pii/S0003426612000625

Psychological Manifestations of Pituitary Disease

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From the May 2012 PNA Newsletter:

 

Psychological Manifestations of Pituitary Disease

Editor’s note: This is an introduction to a lecture given by Dr. Michael Weitzner. It makes many of the points that the PNA strives to promote.

The objectives of this lecture are to provide an overview of the psychological and neuropsychiatric problems faced by patients with pituitary disease, the impact on family, and the options for treatment.

Cushing, himself, believed that there was a need to differentiate the psychological effects that resulted from the pituitary tumor from those that resulted from the stress of illness. It is now recognized that the hypothalamic-pituitary axis is not only an integral element in the expression of behavior, but also an essential part of the limbic system which controls our emotions.

Many patients with pituitary tumors develop an apathy syndrome which is the result of this interplay between the limbic system and the hypothalamic-pituitary axis. An important task is the differentiation of this apathy syndrome from other psychiatric disturbances which are also seen in patients with pituitary disease. It is well recognized that depression and anxiety are present in many patients with hyperprolactinemia and Cushing’s disease. Personality change and anxiety are commonly seen in patients with acromegaly and hypopituitarism. There are several options for treatment, both pharmacological and psychological. One element that is unfortunately ignored in this illness is the effect on the family. Effective treatment of the patient with pituitary disease included treatment of the family.

Michael A. Weitzner, M.D., Department of Psychiatry,University of South Florida, Tampa, Florida

From www.pituitary.org

Have You Learned About Cushing's At a Health Fair?

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I cannot imagine this myself, since Cushing's is so hard to diagnose but an article at yourdailyjournal.com claims: 

The blood profiles provide a comprehensive look at several physiological systems in the body at a cost that is very reasonable for the patients, all in one panel,” Laboratory Manager Rhonda Outlaw said. “The cost savings would amount to anywhere from $185 to $1,100, depending on the tests done and whether they were drawn at a physician’s office or on an outpatient basis.

 

“The panel itself will give indication of possible problems with kidney functions, hematological functions, like anemia, platelet function and infection; cardiovascular disease, thyroid functions, diabetes detection, liver functions and electrolyte function, like Cushings Syndrome, potassium regulation and dehydration detection.”

What do you think about this?


 

 

Pasireotide in Cushing's Disease

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N Engl J Med 2012; 366:2134-2135 May 31, 2012

 

To the Editor:

In their study, Colao et al. (March 8 issue)1 examined the clinical efficacy and safety of two different doses of subcutaneous pasireotide in patients with newly diagnosed, persistent, or recurrent Cushing's disease. Since alternative therapies (including bilateral adrenalectomy) are available for patients with persistent or recurrent Cushing's disease, it would be important to consider all options before embarking on what might turn out to be many years of medication.

Giovanni Targher, M.D.
University of Verona, Verona, Italy 
giovanni.targher@univr.it

No potential conflict of interest relevant to this letter was reported.

1 References

To the Editor:

The phase 3 trial by Colao et al. showed the efficacy of 12 months of treatment with subcutaneous pasireotide (600 or 900 μg twice daily) in patients with Cushing's disease. We now report results after 7 years of treatment with pasireotide administered as part of a phase 2 study.1 In July 2004, a 43-year-old woman with Cushing's disease, whose 24-hour urinary free cortisol level was 9.2 times the upper limit of normal, began 15 days of treatment with subcutaneous pasireotide (600 μg twice daily) that resulted in normalization of these levels (Figure 1AFIGURE 1Effects of Pasireotide Treatment on 24-Hour Urinary Free Cortisol Levels and on Adrenocorticotropin Hormone Levels during Desmopressin-Stimulation Testing.). When treatment was halted for 35 days, urinary free cortisol levels increased. In September 2004, she resumed treatment with pasireotide (600 μg twice daily), which led to clinical improvement (i.e., a weight loss of 13 kg, regular menstrual cycles, and reduced hirsutism). Hyperglycemia ensued (glycated hemoglobin, 5.7 to 7.7%), and weakness necessitated a temporary reduction in the dose to 450 μg twice daily (November 2004 to October 2005). Since November 2005, when the patient resumed taking the 600-μg dose twice daily, urinary free cortisol levels have remained in the normal range at most monthly assessments. Basal and desmopressin-stimulated adrenocorticotropin levels also decreased as a result of treatment with pasireotide (Figure 1B). To date, she has not had any serious adverse events. This case illustrates the long-term efficacy of pasireotide without the development of resistance to the drug's effects.

Rossella Libé, M.D.
INSERM Unité 1016, Paris, France

Lionel Groussin, M.D., Ph.D.
Université Paris Descartes, Paris, France

Jérôme Bertherat, M.D., Ph.D.
Hôpital Cochin, Paris, France 
jerome.bertherat@cch.aphp.fr

Drs. Libé and Bertherat report being investigators for studies of pasireotide in Cushing's disease funded by Novartis. No other potential conflict of interest relevant to this letter was reported.

1 References

Author/Editor Response

We concur with Targher's implication that the advantages and disadvantages of all management options should be considered for each patient before a specific treatment is advised.

Libé and colleagues present a very interesting case of a patient with Cushing's disease in the extension of a phase 2 study of pasireotide. This patient was treated with pasireotide for a much longer duration than the 1 year reported in the phase 3 study.

Annamaria Colao, M.D., Ph.D.
University of Naples Federico II, Naples, Italy

Mario Maldonado, M.D.
Novartis Pharma, Basel, Switzerland

Since publication of their article, the authors report no further potential conflict of interest.

 

From http://www.nejm.org/doi/full/10.1056/NEJMc1204078

This blog post is posted from Cushing's & Cancer at http://cushingshelp.blogspot.com/

Medical management of Cushing's disease: what is the future?

To contact us Click HERE

Fleseriu M, Petersenn S.

Source

Departments of Medicine and Neurological Surgery, Northwest Pituitary Center, Oregon Health & Science University, Portland, OR, USA.

Abstract

Cushing's disease (CD) is caused by a corticotroph, adrenocorticotropic-hormone (ACTH)-secreting pituitary adenoma resulting in significant morbidity and mortality. Transsphenoidal surgery is the initial treatment of choice in almost all cases.

Remission rates for microadenomas are good at 65-90 % (with an experienced neurosurgeon) but remission rates are much lower for macroadenomas. However, even after postoperative remission, recurrence rates are high and can be seen up to decades after an initial diagnosis. Repeat surgery or radiation can be useful in these cases, although both have clear limitations with respect to efficacy and/or side effects.

Hence, there is a clear unmet need for an effective medical treatment. Currently, most drugs act by inhibiting steroidogenesis in the adrenal glands. Most is known about the effects of ketoconazole and metyrapone. While effective, access to ketoconazole and metyrapone is limited in many countries, experience with long-term use is limited, and side effects can be significant. Recent studies have suggested a role for a pituitary-directed therapy with new multireceptor ligand somatostatin analogs (e.g., pasireotide, recently approved in Europe for treatment of CD), second-generation dopamine agonists, or a combination of both.

Mifepristone (a glucocorticoid receptor antagonist) is another promising drug, recently approved by the FDA for treatment of hyperglycemia associated with Cushing's syndrome. We review available medical treatments for CD with a focus on the two most recent compounds referenced above.

Our aim is to expand awareness of current research, and the possibilities afforded by available medical treatments for this mesmerizing, but often frightful disease.

PMID: 22674211 [PubMed - as supplied by publisher]

From http://www.ncbi.nlm.nih.gov/pubmed/22674211

This blog post is posted from Cushing's & Cancer at http://cushingshelp.blogspot.com/

24 Haziran 2012 Pazar

A New Blogging Challenge

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Blog If any other Cushie bloggers are interested in this challenge, let me know and I'll promote your blog on http://www.cushie-blogger.blogspot.com/

Attention bloggers! WEGO Health has found another writing challenge in honor of National Women's Health Week, The Fitness & Health Bloggers Conference (the same one that one of our Health Activists will be attending!) is hosting a week long blogger challenge in honor of National Women's Health Week. The theme for National Women's Health Week is "It's Your Time" National Women’s Health Week empowers women to make their health a top priority. It also encourages women to take the following steps to improve their physical and mental health and lower their risks of certain diseases

Have You Learned About Cushing's At a Health Fair?

To contact us Click HERE

I cannot imagine this myself, since Cushing's is so hard to diagnose but an article at yourdailyjournal.com claims: 

The blood profiles provide a comprehensive look at several physiological systems in the body at a cost that is very reasonable for the patients, all in one panel,” Laboratory Manager Rhonda Outlaw said. “The cost savings would amount to anywhere from $185 to $1,100, depending on the tests done and whether they were drawn at a physician’s office or on an outpatient basis.

 

“The panel itself will give indication of possible problems with kidney functions, hematological functions, like anemia, platelet function and infection; cardiovascular disease, thyroid functions, diabetes detection, liver functions and electrolyte function, like Cushings Syndrome, potassium regulation and dehydration detection.”

What do you think about this?


 

 

Course of pregnancies in women with Cushing’s disease treated by gamma-knife

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(doi:10.3109/09513590.2012.683057)

Francesco Ferraù1, Marco Losa2, Oana Ruxandra Cotta1, Maria Luisa Torre1, Marta Ragonese1, Francesco Trimarchi1, Salvatore Cannavò1

1Department of Medicine and Pharmacology, Section of Endocrinology, University of Messina, Messina, Italy

2Department of Neurosurgery, Istituto Scientifico San Raffaele, Milan, Italy

Correspondence: Francesco Ferraù, MD, Department of Medicine and Pharmacology, Section of Endocrinology, University of Messina, AOU Policlinico “G. Martino” (Pad. H, floor 4), Via Consolare Valeria 1, 98125 Messina, Italy. Tel: +39 090 2213507. Fax: +39 090 2213945. E-mail: ferrau1@interfree.it

 

Data concerning pregnancy in women with Cushing’s disease treated by gamma-knife (GK) are scanty. We present and discuss the course and outcome of five pregnancies in two women with Cushing’s disease (CD), the first of whom was treated only by GK, and the second one treated by surgery, GK and ketoconazole.

In the first patient, pregnancy was uneventful and full-term. During gestation, plasma ACTH, serum cortisol and 24-h urinary free cortisol (UFC) levels were steady, and always in the normal range for healthy non-pregnant individuals. The newborn was healthy and normal-weight.

In the second woman, two pregnancies, occurring 3 years after GK and few months after ketoconazole withdrawal, were interrupted by spontaneous abortion or placental disruption despite normal cortisol levels. This patient became again pregnant 3 years later and delivered vaginally a healthy full-term infant.

Seven months after the delivery, the patient became pregnant again and at the 39th week of gestation delivered vaginally a healthy male. Hypoprolactinemia and/or central hypothyroidism occurred in both cases. In women with CD treated by GK, pregnancy can occur. However, pregnancy is at risk even when ACTH and cortisol levels are normalized by treatment. After GK, evaluation of pituitary function is mandatory due to the risk of hypopituitarism.

Read More: http://informahealthcare.com/doi/abs/10.3109/09513590.2012.683057

Pasireotide in Cushing's Disease

To contact us Click HERE

N Engl J Med 2012; 366:2134-2135 May 31, 2012

 

To the Editor:

In their study, Colao et al. (March 8 issue)1 examined the clinical efficacy and safety of two different doses of subcutaneous pasireotide in patients with newly diagnosed, persistent, or recurrent Cushing's disease. Since alternative therapies (including bilateral adrenalectomy) are available for patients with persistent or recurrent Cushing's disease, it would be important to consider all options before embarking on what might turn out to be many years of medication.

Giovanni Targher, M.D.
University of Verona, Verona, Italy 
giovanni.targher@univr.it

No potential conflict of interest relevant to this letter was reported.

1 References

To the Editor:

The phase 3 trial by Colao et al. showed the efficacy of 12 months of treatment with subcutaneous pasireotide (600 or 900 μg twice daily) in patients with Cushing's disease. We now report results after 7 years of treatment with pasireotide administered as part of a phase 2 study.1 In July 2004, a 43-year-old woman with Cushing's disease, whose 24-hour urinary free cortisol level was 9.2 times the upper limit of normal, began 15 days of treatment with subcutaneous pasireotide (600 μg twice daily) that resulted in normalization of these levels (Figure 1AFIGURE 1Effects of Pasireotide Treatment on 24-Hour Urinary Free Cortisol Levels and on Adrenocorticotropin Hormone Levels during Desmopressin-Stimulation Testing.). When treatment was halted for 35 days, urinary free cortisol levels increased. In September 2004, she resumed treatment with pasireotide (600 μg twice daily), which led to clinical improvement (i.e., a weight loss of 13 kg, regular menstrual cycles, and reduced hirsutism). Hyperglycemia ensued (glycated hemoglobin, 5.7 to 7.7%), and weakness necessitated a temporary reduction in the dose to 450 μg twice daily (November 2004 to October 2005). Since November 2005, when the patient resumed taking the 600-μg dose twice daily, urinary free cortisol levels have remained in the normal range at most monthly assessments. Basal and desmopressin-stimulated adrenocorticotropin levels also decreased as a result of treatment with pasireotide (Figure 1B). To date, she has not had any serious adverse events. This case illustrates the long-term efficacy of pasireotide without the development of resistance to the drug's effects.

Rossella Libé, M.D.
INSERM Unité 1016, Paris, France

Lionel Groussin, M.D., Ph.D.
Université Paris Descartes, Paris, France

Jérôme Bertherat, M.D., Ph.D.
Hôpital Cochin, Paris, France 
jerome.bertherat@cch.aphp.fr

Drs. Libé and Bertherat report being investigators for studies of pasireotide in Cushing's disease funded by Novartis. No other potential conflict of interest relevant to this letter was reported.

1 References

Author/Editor Response

We concur with Targher's implication that the advantages and disadvantages of all management options should be considered for each patient before a specific treatment is advised.

Libé and colleagues present a very interesting case of a patient with Cushing's disease in the extension of a phase 2 study of pasireotide. This patient was treated with pasireotide for a much longer duration than the 1 year reported in the phase 3 study.

Annamaria Colao, M.D., Ph.D.
University of Naples Federico II, Naples, Italy

Mario Maldonado, M.D.
Novartis Pharma, Basel, Switzerland

Since publication of their article, the authors report no further potential conflict of interest.

 

From http://www.nejm.org/doi/full/10.1056/NEJMc1204078

Medical management of Cushing's disease: what is the future?

To contact us Click HERE

Fleseriu M, Petersenn S.

Source

Departments of Medicine and Neurological Surgery, Northwest Pituitary Center, Oregon Health & Science University, Portland, OR, USA.

Abstract

Cushing's disease (CD) is caused by a corticotroph, adrenocorticotropic-hormone (ACTH)-secreting pituitary adenoma resulting in significant morbidity and mortality. Transsphenoidal surgery is the initial treatment of choice in almost all cases.

Remission rates for microadenomas are good at 65-90 % (with an experienced neurosurgeon) but remission rates are much lower for macroadenomas. However, even after postoperative remission, recurrence rates are high and can be seen up to decades after an initial diagnosis. Repeat surgery or radiation can be useful in these cases, although both have clear limitations with respect to efficacy and/or side effects.

Hence, there is a clear unmet need for an effective medical treatment. Currently, most drugs act by inhibiting steroidogenesis in the adrenal glands. Most is known about the effects of ketoconazole and metyrapone. While effective, access to ketoconazole and metyrapone is limited in many countries, experience with long-term use is limited, and side effects can be significant. Recent studies have suggested a role for a pituitary-directed therapy with new multireceptor ligand somatostatin analogs (e.g., pasireotide, recently approved in Europe for treatment of CD), second-generation dopamine agonists, or a combination of both.

Mifepristone (a glucocorticoid receptor antagonist) is another promising drug, recently approved by the FDA for treatment of hyperglycemia associated with Cushing's syndrome. We review available medical treatments for CD with a focus on the two most recent compounds referenced above.

Our aim is to expand awareness of current research, and the possibilities afforded by available medical treatments for this mesmerizing, but often frightful disease.

PMID: 22674211 [PubMed - as supplied by publisher]

From http://www.ncbi.nlm.nih.gov/pubmed/22674211

23 Haziran 2012 Cumartesi

Day Ten, Cushing's Awareness Challenge

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Blue and Yellow - we have those colors on ribbons, websites, tshirts, Cushing's Awareness Challenge logos and even cars.

This is the yellow PT cruiser I had rented for the Columbus, OH meeting in 2007. It was when we all met at Hoggy's for dinner although some of us travellers stayed at this hotel.

I'm the one in yellow and blue.

 

 

Later in 2007, I bought my own truly Cushie Car.  I even managed to get a butterfly on the tags.


So, where did all this blue and yellow come from, anyway?  The answer is so easy and without any thought that it will amaze you!

In July of 2000, I was talking with my dear friend Alice, who runs a wonderful menopause site, Power Surge, wondering why there weren't many support groups online (OR off!) for Cushing's and I wondered if I could start one myself and we decided that I could. This website (http://www.cushings-help.com) first went "live" July 21, 2000.  It was a one-page bit of information about Cushing's.  Nothing fancy.

I didn't know much about HTML (yet!) but I knew a little from what Alice had taught me and I used on my music studio site.  I didn't want to put as much work <COUGH!> into the Cushing's site as I had on the music studio site so I used a WYSIWYG web editor called Microsoft FrontPage.

One of their standard templates was - you guessed it! - blue and yellow.

TaDa!  Instant Cushie color scheme forever.  Turns out that the HTML that this software churned out was really awful and had to be entirely redone as the site grew.  But the colors stuck.

Day Eleven, Cushing's Awareness Challenge

To contact us Click HERE

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.

 

Day Nineteen, Cushing's Awareness Challenge

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In Day 10 on April 10, 2012, I wrote about how we got the Cushing's colors of blue and yellow.  This post is going to be about the first Cushing's ribbons.

 

I was on vacation  in September, 2001 when SuziQ called me to let me know that we had had our first Cushie casualty (that we knew about).

On the message boards, Lorrie wrote: Our dear friend, Janice died this past Tuesday, September 4, 2001. I received an IM from her best friend Janine, tonight. Janine had been reading the boards, as Janice had told her about this site, and she came upon my name and decided to IM me. I am grateful that she did. She said that she knew that Janice would want all of us to know that she didn't just stop posting.

For all of the newcomers to the board that did not know Janice, she was a very caring individual. She always had something positive to say. Janice was 36 years old, was married and had no children. She had a miscarriage in December and began to have symptoms of Cushing's during that pregnancy. After the pregnancy, she continued to have symptoms. When discussing this with her doctor, she was told that her symptoms were just related to her D&C. She did not buy this and continued until she received the accurate diagnosis of Cushing's Syndrome (adrenal) in March of 2001. Tragically, Janice's tumor was cancerous, a very rare form of Cushing's.

Janice then had her tumor and adrenal gland removed by open adrenalectomy, a few months ago. She then began chemotherapy. She was very brave through this even though she experienced severe side effects, including weakness and dizziness. She continued to post on this board at times and even though she was going through so much, she continued with a positive attitude. She even gave me a referral to a doctor a few weeks ago. She was my inspiration. Whenever I thought I had it bad, I thought of what she was dealing with, and I gained more perspective.

Janice was having difficulty with low potassium levels and difficulty breathing. She was admitted to the hospital, a CT scan was done and showed tumor metastasis to the lungs. She then was begun on a more aggressive regimen of chemo. She was discharged and apparently seemed to be doing well.

The potassium then began to drop again, she spiked a temp and she was again admitted to the hospital. She improved and was set to be discharged and then she threw a blood clot into her lungs. She was required to be put on a ventilator. She apparently was at high risk for a heart attack. Her husband did not want her to suffer anymore and did not want her to suffer the pain of a heart attack and so chose for the doctors to discontinue the ventilator on Tuesday. She died shortly thereafter.

Janice was our friend. She was a Cushie sister. I will always remember her. Janine asked me to let her know when we get the Cushing's ribbons made as she and the rest of Janice's family would like to wear them in her memory. She said that Janice would want to do anything she could to make others more aware of Cushing's.

The image at the top of the page shows the first blue and yellow ribbon which were worn at Janice's funeral.  When we had our "official ribbons" made, we sent several to Janice's family.

Janice was the first of us to die but there have been more, way too many more, over the years.  I'll write a bit more about that on Day 21.

 

Day Twenty, Cushing's Awareness Challenge

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This is one of the suggestions from the Cushing's Awareness Challenge post:

What have you learned about the medical community since you have become sick?

This one is so easy.  I've said it a thousand times - you know your own body better than any doctor will.  Most doctors have never seen a Cushing's patient, few ever will in the future.

If you believe you have Cushing's (or any other rare disease), learn what you can about it, connect with other patients, make a timeline of symptoms and photographs. Read, take notes, save all your doctors notes, keep your lab findings, get second/third/ten or more opinions.

This is your life, your one and only shot (no pun intended!) at it.  Make it the best and healthiest that you can.

When my friend and fellow e-patient Dave deBronkart learned he had a rare and terminal kidney cancer, he turned to a group of fellow patients online  and found a medical treatment that even his own doctors didn't know. It saved his life.

In this video he calls on all patients to talk with one another, know their own health data, and make health care better one e-Patient at a time.

 

Have You Learned About Cushing's At a Health Fair?

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I cannot imagine this myself, since Cushing's is so hard to diagnose but an article at yourdailyjournal.com claims: 

The blood profiles provide a comprehensive look at several physiological systems in the body at a cost that is very reasonable for the patients, all in one panel,” Laboratory Manager Rhonda Outlaw said. “The cost savings would amount to anywhere from $185 to $1,100, depending on the tests done and whether they were drawn at a physician’s office or on an outpatient basis.

 

“The panel itself will give indication of possible problems with kidney functions, hematological functions, like anemia, platelet function and infection; cardiovascular disease, thyroid functions, diabetes detection, liver functions and electrolyte function, like Cushings Syndrome, potassium regulation and dehydration detection.”

What do you think about this?


 

 

21 Haziran 2012 Perşembe

Day 18 of the Cushing's Awareness Challenge: Fiona Apple may just have it right...

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He said 'It's all in your head' and I said 'So's everything' but he didn't get it --Fiona Apple in "Paperbag".

Pituitary tumors have a large spectrum pathology. These "little buggers" may cause large problems, also. Dr. Shereen Ezzat of the Toronto Cancer Institute has been studying pituitary tumors for several years, and presents the case for epigenetic disruption of gene expression which causes alterations of normal pituitary cells. Using the information he and others have gathered, he is hopeful treatments for these lesions/tumors can be developed. Since the morbidity/mortality of most with pituitary tumors is greatly increased, and the only long-term therapies so far are surgical or radioactive, improved treatment is anxiously sought by those of us with Cushing's or presently in remission.
According to Dr. Ezzat, pituitary tumors comprise 10% of the tumors surgically removed intercranially with increased morbidity due to invasion of surrounding structures. As a Cushie community, we know this only too well first-hand. All too often the only recourse for the hypercortisolemia due to ACTH producing tumor cells is a bilateral adrenalectomy. Radiation is often a secondary treatment to decrease tumor growth or Nelson's syndrome.
In another article, Dr. Ezzat says, "Some pituitary adenomas grow rapidly, producing symptoms of an intracranial mass, loss of normal anterior pituitary hormone production, and visual-field disturbances due to stretching of the overlying optic chiasm. They can invade downward into paranasal sinuses, laterally into the cavernous sinuses (thereby disrupting coordinated eye movement) and upwards into the brain. They can cause death by invasion of the brain."
But size does NOT always matter with these tumors. Dr. Ezzat also says "they can cause mood disorders, sexual dysfunction, infertility, obesity and disfigurement, hypertension, diabetes mellitus and accelerated heart disease. If untreated, hormone-excess syndromes can be lethal."
In the pre-print, recent article, Epigenetic Control in Pituitary Tumors , Dr. Ezzat discusses epigenetically-mediated gene dysregulation as a cause for the production of pituitary tumors. He explores the role of fibroblast growth factors and histone gene silencing and discusses the implications of the need to find the process or mechanism involved in order to develop new therapies.




Shereen Ezzat, M.D. (2008). Epigenetic Control in Pituitary Tumors Endocrinology Journal DOI: http://dx.doi.org/http://www.jstage.jst.go.jp/article/endocrj/advpub/0/0804240101/_pdf

Day 21 of the Cushing's Awareness Challenge: eHow doesn't know how...

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A must read on another blog, Brain Tumors can make you Fat!, points out the problem with eHow's information on Cushing's Disease/Syndrome.

The title of your article is "How to Avoid Cushing's Disease."  So, in your expert opinion, how does reducing your salt intake prevent a disease that is caused by an overproduction of cortisol?  Could you please cite your source for your readers on what tells you avoiding salt will help a person avoid hypercortisolemia?
You don't want to miss this.

Day 28 of the Cushing's Awareness Challenge: Getting it right...

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The Cushing's Awareness Challenge is winding down, and I haven't posted every day.  I have tried to post at least twice a week.  I have been so busy with work plus dealing with allergies I haven't had time or felt like posting when I have time.

I do believe my allergies are worse since my BLA.  Perhaps the high cortisol treated them?  I don't know.  I do know this spring allergens are worse in my area than they usually are.  Everything seemed to bloom and spout pollen all at once.

Someone asked me the other day why we are so concerned about awareness for Cushing's.  "Isn't is a really rare disease?"

"No", I said, "It's just rarely diagnosed."  

And there is research to back up my statement.  One recent research article is one you should take to your doctor if you believe you have Cushing's.  It talks about the reality of testing for Cushing's Disease/Syndrome and that it requires a lot of testing.  One can have a lot of normal tests and still have Cushing's.

As I go through my daily life, I see a lot of people who have the signs of Cushing's.  It's a daily conundrum deciding whether to approach a person about it or not.  Many times when I have, I've been met with cynicism or been ignored totally.  Other times, folks want information.  A few times, I've been contacted by these saying either a)  my doctor thinks I'm full of it or b) my doctor thinks you may be right but doesn't know what to do from here.  It's tough, having this disease.  Although there are a lot of textbooks for doctors describing how to test and diagnose, so many of us aren't truly textbook cases.  That's the problem with textbooks.  They are a "one size fits all" type of diagnosis/testing.  We come in all sizes, shapes, and genders.  We don't fit the textbook mold.   Slowly, the textbooks are changing.  Recent research is changing how doctors test and diagnose.  In my opinion, it's going to take another generation or two of doctors to really get it right. Until then, many people won't be diagnosed and treated.

Day 29 of the Cushing's Awareness Challenge: Life goes on

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Life goes on...

Life doesn't stop because one gets a rare illness or is diagnosed with a disease.  However, mine seems to be delineated by before Cushings, after Cushing's,  before BLA and after BLA.  Before Cushing's is a gray area.  I'm not sure exactly when I started getting symptoms.  Some of my symptoms went as far back as childhood but others were more recent when I realized what was wrong with me.  I was 47-48 at that time.  I'm sure I had symptoms of Cushing's (verified by my photo evidence) from the age of 24.

Skipping ahead past those years between ages 47 and 52 when I was going through testing, diagnosis, pituitary surgery to remove the tumor, recurrence, and re-testing/diagnosis though my BLA, I am in the after BLA era.  Does anyone else see her life this way?  I know most folks look at graduation, job, marriage, children, etc. as the defining moments of their lives.  And my children, plus my grand-child, are definitely more important to me, but I still categorize them in the pre-BLA/post-BLA eras.

Isn't it crazy that one event can be so momentous in one's life?  I sit here typing this after a day of being lonely and wishing I was closer to my family and my grandson.  Part of me wants to make the big leap and just "do it". Life is short.  Just do it.   The other, conservative part of me says, "You have to make it to retirement.  You have to have something to live on and you don't want to lose this money."  And once I do this, which I will someday, I know it will be a defining moment and I'll classify it post-move.  I think that's a good thing.  I'm tired of living my life around a disease.

Korlym: New drug to treat Cushing's Disease

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I know several folks who have recently started taking the new FDA-approved drug, Korlym, to treat Cushing's Disease.  Korlym is a new name for the "old" drug mifepristone and was developed by Corcept Therapeutics Incorporated. 
Korlym blocks the activity of cortisol and is proven to reduce high blood sugar (hyperglycemia), a key symptom of Cushing's. Korlym has a unique way of working. Instead of reducing cortisol levels, it blocks the action of cortisol, thus preventing the effects of excess cortisol.1
Korlym has many side effects and cannot be taken by everyone.  Once the patient stops taking Korlym, she will continue to have Cushing's.  The biologic half-life of Korlym is approximately 85 hours.  If a patient suffers adrenal insufficiency or crisis, massive amounts of hydrocortisone or dexamethasone are needed to alleviate these and will have to be continued for the duration of the drug in his system.

To follow a patient who has just started taking Korlym, you will find her blog here:  Cushing's Disease

20 Haziran 2012 Çarşamba

Adrenal cavernous hemangioma with subclinical Cushing’s syndrome: report of a case

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Masaharu Oishi, Shugo Ueda, Sachiko Honjo, Hiroyuki Koshiyama, Yoshiaki Yuba and Arimichi Takabayashi

 

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Cavernous hemangioma of the adrenal gland is a rare tumor, which does not usually have endocrinological function. We report to our knowledge, the third documented case of a functioning adrenal hemangioma.

Interestingly, this tumor indicated glucocorticoid hypersecretion, whereas the two previous cases showed mineralocorticoid hypersecretion. The tumor was 5 cm in diameter with typical computed tomography and magnetic resonance imaging findings.

Subclinical Cushing’s syndrome was diagnosed preoperatively, as there was insufficient suppression of cortisol by low-dose dexamethasone, a low adrenocorticotropic hormone (ACTH) concentration, and diminished ACTH and cortisol circadian rhythms without the typical clinical manifestation and symptoms of hypercortisolism.

Intraoperative hypotension occurred immediately after tumor removal and following postoperative adrenal insufficiency, which support that the tumor was hyperfunctioning. The postoperative adrenal insufficiency had recovered completely by 12 months after the operation.


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Read more at http://www.springerlink.com/content/5mv23480j870462m/

 

This blog post is posted from Cushing's & Cancer at http://cushingshelp.blogspot.com/

Correlation Between Histological Subtypes and MRI Findings in Clinically Nonfunctioning Pituitary Adenomas

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Hiroshi Nishioka, Naoko Inoshita, Toshiaki Sano, Noriaki Fukuhara and Shozo Yamada

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Clinically nonfunctioning pituitary adenomas (CNFPAs) consist of several histological subtypes, including null cell adenoma (NCA), silent gonadotroph cell adenoma (SGA), silent corticotroph adenoma (SCA), and other silent adenomas (OSA) (i.e., GH, TSH, and prolactin adenomas).

To detect possible correlations between MRI findings and the subtypes, we retrospectively studied 390 consecutive patients with CNFPA who underwent surgery between 2008 and 2010. They were classified into three groups: NCA/SGA (313 cases), SCA (39 cases), and OSA (36 cases); in addition there were two unusual cases of plurihormonal adenoma.

Three MRI findings were less common in NCA/SGA than in the other groups (P < 0.0001): giant adenoma (>40 mm), marked cavernous sinus invasion (Knosp grade 4), and lobulated configuration of the suprasellar tumor. When these MRI findings were negative in patients older than 40 years old, 91.0 % (212/233) were NCA/SGA.

These MRI findings were frequently noted despite a low MIB-1 index in SCA. OSA showed a high MIB-1 index and a preponderance in younger patients. In conclusion, although SCA and OSA consisted of only 20 % of CNFPAs, their frequency significantly increased when the tumor was large, invasive, and lobulated, and the patient was younger than 40 years old.

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  • From http://www.springerlink.com/content/4r466139264616q2/

This blog post is posted from Cushing's & Cancer at http://cushingshelp.blogspot.com/