Cushies can die after exhausting all treatment options

This makes me incredibly sad.  I have had two unsuccessful pituitary surgeries, and I am currently doing medication therapy (pm ketoconazole with am Cortef) until the tumor culprit comes out of hiding and presents itself on the pituitary MRI for a third pituitary surgery.  No one knows how long that will take.

After my first pituitary surgery, I developed ptosis, or drooping eyelid.

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Cushing’s: the worst case scenario

Viv Thornton-Jones

Churchill Hospital, Oxford, UK.
Endocrine Abstracts (2008) 15 S58

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We present the case of a 40-year-old female who was referred to our Department in 1993, for further management following the diagnosis of Cushing’s disease. She proceeded to a transsphenoidal adenenomatectomy (TSA, note: pituitary surgery) which resulted in a biochemical cure.

In 1998 she presented with recurrence of Cushing’s Disease, which was managed by a 2nd TSA (pituitary surgery) followed by external beam irradiation.

Bilateral adrenalectomy followed a year later, due to the inability to control her disease.

In 2001 she presented with Nelson’s Syndrome managed by a 3rd TSA (pituitary surgery) followed this time with Gamma Knife surgery.

In 2004 she presented with manifestations consistent with recurrence of Nelson’s Syndrome and proceeded to a 4th TSA (pituitary surgery). 

Despite the risk of blindness, the patient agreed to a second course of Gamma Knife treatment for the possibility of tumour control.

Over the next 2 years her clinical picture deteriorated, resulting in a right partial ptosis and a sixth nerve palsy.

She was referred to an Oncologist who offered her Chemotherapy, but she refused treatment.

The patient was then in the care of the Palliative Care Team and she died peacefully at home in 2006.

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Endocrine Abstracts (2008) 15 S58

Thank you to Ami and MaryO for finding this abstract.

Less Fat, Still Weak after Cushing's

Good news. 

Fat decreases after wild, errant cortisol overproduction from Cushing's is stopped. Just like we Cushies told everyone it would. See, it's not just from over-eating and not exercising.

Bad news.  

Muscle weakness persists. Cardiovascular risk associated with unfair fat symptom may remain.  So do cognitive issues. 

Shoot. This disease just won't let up.

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Body composition and cardiovascular risk markers after remission of Cushing's disease: a prospective study using whole-body MRI

J Clin Endocrinol Metab. 2012 May;97(5):1702-11. Epub 2012 Mar 14.

http://www.ncbi.nlm.nih.gov/pubmed/22419708

Geer EB, Shen W, Strohmayer E, Post KD, Freda PU.

Source

Department of Medicine, Division of Endocrinology, Mount Sinai School of Medicine, One Gustave Levy Place, Box 1055, New York, New York 10029, USA. eliza.geer@mssm.edu

Abstract

CONTEXT:

Cushing's Disease (CD) alters fat distribution, muscle mass, adipokine profile, and cardiovascular risk factors. It is not known whether remission entirely reverses these changes.

OBJECTIVES:

Our objective was to determine whether the adverse body composition and cardiovascular risk profile in CD change after remission.

DESIGN, SETTING, AND PATIENTS:

Fourteen CD patients were studied prospectively: before surgery (active disease) and again postoperatively 6 months after discontinuing oral glucocorticoids (remission). Whole-body magnetic resonance imaging was used to examine lean and fat tissue distributions.

OUTCOME MEASURES:

Body composition (skeletal muscle and fat in the visceral, bone marrow, sc, and inter-muscular compartments) and cardiovascular risk factors (serum insulin, glucose, leptin, high-molecular-weight adiponectin, C-reactive protein, and lipid profile) were measured in active CD and remission (mean 20 months after surgery).

RESULTS:

Remission decreased visceral, pelvic bone marrow, sc (including trunk and limb sc), and total fat; waist circumference; and weight (P < 0.05). Remission altered fat distribution, resulting in decreased visceral/total fat (P = 0.04) and visceral fat/skeletal muscle ratios (P = 0.006). Remission decreased the absolute muscle mass (P = 0.015). Cardiovascular risk factors changed: insulin resistance, leptin, and total cholesterol decreased (P < 0.05), but adiponectin, C-reactive protein, and other lipid measures did not change.

CONCLUSIONS:

CD remission reduced nearly all fat depots and reverted fat to a distribution more consistent with favorable cardiovascular risk but decreased skeletal muscle. Remission improved some but not all cardiovascular risk markers. Remission from CD dramatically improves body composition abnormalities but may still be associated with persistent cardiovascular risk.

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Thanks to MaryO for the post.

Day 20: Surgiversary

DATE:  
APRIL 20, 2011.


TARGET:  
3 MILLIMETER TUMOR ON THE RIGHT SIDE OF PITUITARY.


OFFENSE:  

UNLAWFUL PRODUCTION OF EXCESS ACTH, SPURRED EXCESS PRODUCTION OF CORTISOL BY ADRENAL GLANDS.


ACCOMPLICE:  

ADRENAL GLANDS FOR AIDING AND ABETTING.


STRATEGY:  

TRANSSPHENOIDAL TRANSNASAL RESECTION OF PITUITARY TUMOR.


OUTCOME:  

1) SUCCESS. PATIENT ALIVE. TUMOR REMOVED.


2) FAIL. PITUITARY HORMONE ACTH STILL HIGH ON POST OP DAYS 5 & 16. TUMOR CELLS PLAYING LETHAL GAME OF PEEK-A-BOO. 


3) FAIL. NOT ONE DAY OF RELIEF FROM CUSHING'S SYMPTOMS. PATIENT CONTINUES STRUGGLES TO FIGHT DAMAGE TO BODY, MIND & SOUL CAUSED BY EXCESS ACTH & CORTISOL PRODUCTION.

4) PATIENT STRUGGLES TO RE-ENGAGE IN LIFE AND SOCIETY WITH MULTIPLE HORMONE- AND VITAMIN-DEFICIENCIES (GROWTH HORMONE DEFICIENCY, VITAMIN D DEFICIENCY, OSTEOPENIA, THYROID DEFICIENCY, FERRITIN (IRON) DEFICIENCY, ACTH EXCESS, CORTISOL EXCESS). 

5) PATIENT DECIDES ON NEXT COURSE OF TREATMENT: 

3RD PITUITARY SURGERY vs. BILATERAL ADRENALECTOMY vs. KORLYM

Structures surrounding pituitary. Important things nearby include carotid artery and optic nerve.

Arrow points to 3 millimeter tumor, right side pituitary; black indented area on opposite side shows space where tumor removed in 1st surgery (6/2009).

Recovery. Swelling but not too bad. Rocking those eye brows and ACTH shadow (acanthosis nigricans). 

Post op day 4 with Lola's blanket. Neurosurgeon deflated,

removed balloons from nose. Balloons measured 4-6 inches.

Crazy nurses insisted on placing an IV here. Btw--my veins are good.

Day 1: Cushing's on my Mind

There is no real way to explain how devastating Cushing's is to a patient unlucky enough to suffer from high cortisol. In nearly four years of blogging, I find that this word cloud I made does the best job so far. 

Click on the image to enlarge it.

Yes, it is really this bad, and isn't that so sad for me and other Cushies.

  

I can’t believe I have lived in that cloud for over 60 months now. To be a patient suffering with Cushing’s is to feel and experience all that word swirl of emotion and more. That word cloud isn’t just my disease. Cushing's is the monkey on my back that I cannot shake. Hours and days, then months and years pass without feeling better, looking better, or being better.  When the days carrying moments of relief arrive for a short surprise visit, I am pleased. Looking back, I realize the strength I have in just walking through it, never seeing any light at the end of the tunnel.  May I and the many other Cushing’s patients continue to find the strength to keep going.

Cushing's has altered who I have become, but I can't let it change who I can become anymore.

Korlym: New Medication in Clinical Trials to treat Cushing's

Wouldn't this be interesting if this could really help some people. ~m


Corcept Therapeutics Nears Completion of Enrollment in Phase 3 Cushing's Syndrome Study -- Data Announcement Anticipated by Year End

MENLO PARK, CA, Jun 25, 2010
(MARKETWIRE via COMTEX) --

Corcept Therapeutics Incorporated ("Corcept"), a pharmaceutical company engaged in the discovery and development of drugs for the treatment of severe metabolic and psychiatric disorders, today announced that it had enrolled 49 of the planned 50 patients in its Phase 3 trial of CORLUX(R) for the treatment of Cushing's Syndrome and expects the fiftieth patient to begin dosing next week.

"We are near our target of dosing 50 patients in our Phase 3 study of CORLUX for the treatment of Cushing's Syndrome. We remain on track to announce top line results from the trial by the end of this year," said Joseph Belanoff, M.D., Chief Executive Officer of Corcept.

Cushing's Syndrome Phase 3 Trial Nears Completion of Planned Enrollment of 50 Patients

We have dosed 49 of the planned 50 patients in our open-label Phase 3 trial of CORLUX in patients with endogenous Cushing's Syndrome, which is being conducted at 20 leading medical facilities throughout the United States. The final patient is expected to be dosed next week.

The FDA has indicated that this single 50-patient open-label Phase 3 study of CORLUX may provide a reasonable basis for the submission of a New Drug Application ("NDA") for Cushing's Syndrome. In the study, each patient's dose is titrated to clinical benefit by their study investigator and the primary endpoints (either an improvement in glucose tolerance or blood pressure) are measured at the end of 24 weeks.

We expect to announce the top-line results of this study by the end of 2010 and to submit our NDA to the FDA in the first quarter of 2011.

About Cushing's Syndrome

Endogenous Cushing's Syndrome is caused by prolonged exposure of the body's tissues to high levels of the hormone cortisol due to either cortisol or adrenocorticotropic hormone (ACTH) production by tumors. Cushing's Syndrome is an orphan indication which most commonly affects adults aged 20 to 50. An estimated 10 to 15 of every one million people are newly diagnosed with this syndrome each year, resulting in over 3,000 new patients in the United States. An estimated 20,000 patients in the United States have Cushing's Syndrome. Symptoms vary, but most people have one or more of the following manifestations: high blood sugar, diabetes, high blood pressure, upper body obesity, rounded face, increased fat around the neck, thinning arms and legs, severe fatigue and weak muscles. Irritability, anxiety, cognitive disturbances and depression are also common. Cushing's Syndrome can affect every organ system in the body and can be lethal if not treated effectively.

About CORLUX

Corcept's first-generation compound, CORLUX, also known as mifepristone, directly blocks the cortisol (GR-II) receptor and the progesterone (PR) receptor. Intellectual property protection is in place to protect important methods of use for CORLUX. Corcept retains worldwide rights to its intellectual property related to CORLUX.

About Corcept Therapeutics Incorporated

Corcept is a pharmaceutical company engaged in the discovery and development of drugs for the treatment of severe metabolic and psychiatric disorders. The company has two ongoing Phase 3 programs: CORLUX for the treatment of Cushing's Syndrome, and CORLUX for the treatment of the psychotic features of psychotic depression. Corcept also has a Phase 1 program for CORT 108297, a selective cortisol receptor antagonist. Corcept has developed an extensive intellectual property portfolio that covers the use of GR-II antagonists in the treatment of a wide variety of psychiatric and metabolic disorders, including the prevention of weight gain caused by the use of antipsychotic medication, as well as composition of matter patents for our selective GR-II antagonists.

CONTACT:
Caroline Loewy
Chief Financial Officer
Corcept Therapeutics
650-688-8783

SOURCE: Corcept Therapeutics

Original Marketwire article