Dr. Friedman was guest speaker on Cushings-Help BlogTalkRadio Show

Dr. Ted Friedman, an e-patient advocate, vibrantly answered a boatload of questions on the Cushings Help BlogTalkRadio show on January 29. He discussed everything from Cushing's Syndrome to new technologies for testing. Because we ran out of time, he is going to return soon and the date will be posted.

Take time to listen as Dr. Friedman untangles some of the mysteries surronding testing and diagnosis for so many Cushing's patients and as he advocates for patient awareness and education. It's refreshing and enlightening to hear.

Blogging his BLA, Steve shares his experience...

"We are the orphans. We are the zebras in a world of horses. The ones who have a rare disease many have never heard of. We have no famous speakers to bring life to our voices. We have no sports stars to put a face on Cushing’s. We have no pink ribbons. We have no simple tests to tell us if we have it. If no one cures us, our lives will be shortened at best and ended at worst." --Steve Owens on "Should Have Seen It"

Steve is a Cushing's survivor who has already had surgery to remove a pituitary adenoma. He still has full blown Cushing's, and is going to have a bilateral adrenalectomy (BLA) on Friday, January 30. He has chosen to blog about his surgery and you can read all about it at his blog "Should Have Seen It".

Steve also blogs at Thankful for the Journey and Herding Zebras.

Watch as Steve shares his experience, good and bad, happy and sad. We are pulling for you, Steve!!

Company develops medical breakthrough

A small company is working on a medical diagnostic device that can instantaneously detect the presence of disease in a single drop of body fluid.

The Aurora-based company, Beacon Biotechnology, says future disease diagnosis could revolutionize medicine by making complex disease detection as simple as a trip to the local drug store. "I've been involved in diagnostics for a long time and when I saw this I recognized what it could do to help change health care. We're all very excited about it," said CEO Fred Mitchell.

To read more: Company develops medical breakthrough

Their website: http://www.beaconbiotechnology.com/

Transsphenoidal Surgery: Comparison of Techniques

Most of the time, treatment for a pituitary adenoma involves surgery to remove the adenoma. There are three different techniques for transsphenoidal pituitary surgery: (1) sublabial transseptal approach with microscopic resection, (2) transnasal transseptal approach with endoscopic resection, and (3) endoscopic approach with endoscopic resection.



Sublabial transseptal approach with microscopic resection:

An incision is made under the lip and the nasal tissue and a section of the central partition of the nose is removed to form a large opening through which the remainder of the procedure is conducted. An adjustable retractor is placed into space whose blades reach to the sphenoid sinus. An operating microscope is used to resect the anterior wall of the sphenoid sinus, remove the floor of the sella, and incise the dura. The tumor is then removed from the pituitary. Any tumor in the sinuses and on the floor of the sella is also removed. The floor of the sella is then "plugged", usually with a fat graft from the abdomen. The microscope and retractor are removed, and all tissue, including nose and lip, are replaced and the incision is sutured. The nasal cavities are packed with some form of packing. Balloons or tampons are often used, although gauze is still used by some.

Transnasal transseptal approach with endoscopic resection:

An incision is made just inside one or both nostrils and an opening is made in the septum. There are two approaches, one using both nostrils (one for microscope, the other for the endoscope) and one where only one nostril is used. The bone that is removed is usually kept to be used later in the operation. The floor of the sella is again removed and the pituitary tumor is extracted in small fragments using the endoscope. When all tumor has been removed the saved bone is used to refashion the normal housing of the pituitary gland. The incision is sutured and the nostrils are packed similarly to the sublabial approach.

A modification that some surgeons use involves a septal pushover/direct sphenoidotomy: The incision is deeper within the nasal cavity where the cartilage meets the bony part of the septum. Entry to the sinuses are made on either side of the bony septum until the sphenoid sinus is reached.

With yet another possible modification, the posterior part of septum just in front of the sphenoid sinus is moved to one side and the sphenoid sinus can be reached more directly. There are several advantages to these techniques. Usually, packing is not needed with this approach.



Endoscopic approach with endoscopic resection:

No incision is made with this procedure. An endoscope is used alone (no microscope), and is maneuvered through one nostril, forcing the cartilage to separate from the septum allowing passage to the sinuses. The endoscope is used to proceed much like the other approaches. A fat graft is not always needed due to the small size of the opening with a pure endoscope approach. No packing is needed, either.

Watch while a minimally invasive endoscopic surgery is performed at Thomas Jefferson University Hospital (click here).

MedPage Today Blogs: Grand Rounds 5:18: Ten Suggestions For Healthcare Reform - Medical News plus CME

Ten Suggestions For Healthcare Reform

If you are serious about healthcare reform, then you need to read this. Multiple medical bloggers, including patients, are featured with links to pertinent articles. A well-written and timely presentation by Dr. Val Jones, and I highly recommend reading it all. Don't miss a link.

(And yes, yours truly has an article linked there, but if you are reading here, you've read the article!)

Healthcare: Is it really about me, the patient?

Because of the way the healthcare system is now, few doctors have the time to see a patient as a whole. It isn't profitable when inequitable reimbursements for primary care physicians (PCPs) force them to see a certain number of patients a day to remain a viable business. In turn, many are turning to specialties rather than primary care. But as a "zebra" patient, I see poor healthcare because of this. No one is there to see me as a whole.

Recently, when communicating this online with a physician in a discussion about "zebras" in healthcare, he made several points which eventually led to a discussion of reform. Each made sense from his point of view and I understood what he was saying. But I wanted him to see my point of view as a patient.

First Point: "An unusual case is more likely to be an unusual presentation of a common disease, rather than a rare disease itself."

Perhaps that is true. As patients with Cushing's (and I believe other "zebra" diseases), we are more a conglomeration of "usual" presentations which are treated piecemeal rather than taken as a whole. Together, these should give a physician an "aha" moment. But most of the time, in our experience, they do not. And part if this is due to the way each symptom is farmed out to a specialist. Many PCP's don't take the time to see the "whole" and put it all together.

Second Point: "As a patient, you can provide information for other patients."

Mary O'Conner had Cushing's in the 80's with no support whatsoever; she had no help or information other than what she gleaned at her library. Thanks to her hard work and dedication, she founded the cushings-help.com website and message boards, which she still single-handedly runs with it's thousands of members. Without those message boards (forums), many of us would be dead or suffering with the disease. (In fact, several we have known have died from it.) It's strong, vibrant, and supportive community. We know how to test, where to test and when to test due to that. We know who the specialists are (and I don't mean those who profess to be, but those who actually practice like they are), and they know us.

Mary Shomon has been instrumental in sharing and coordinating information about thyroid disease with patients. She hosts an active support board as well as several web sites. Other groups with other diseases do the same.

There is a growing group of patients providing support and information through blogs. Our Cushing's group is one. I know there are many others about other zebra diseases. Each group will tell you that they have gleaned much more support there than in a doctor's office.

What influence does this have on healthcare? We take in recent journal articles, research, and whatever else we can find to educate our doctors. We "fire" some, and "hire" others. We spread the word about both sets of doctors. Yes, the internet is a fabulous tool. Which brings me to the next point.

Third Point: "The internet itself is helpful in dispensing useful information for patients."

I did a reader's take for KevinMD a while back on that very thing. I often fight that battle with doctors who say I "read too much on the internet" as if they are the only smart folks in the world. (I realize not all doctors are like this.) I am able to find and download recent research about my disease. Sadly, though, not all patients have the resources or the background to do that. This means those of us who do need to share with those who do not.

Fourth Point: "As a doctor, the biggest obstacle to thoroughly investigating some cases is simply having enough time to do so."

Some doctors have overcome the time restraints by going to coincierge medicine. Kevin Kelleher is using a business model which reduces the volume of patients he must treat to be a viable business. While he says he can give patients more adequate care with more time for each, this is no assurance of quality just because he is a doctor who wants a guaranteed income stream with less work. If enough doctors did this, however, perhaps the competition would guarantee better healthcare. I think the fifth point, below, will help with this if/when we can do it.

Fifth Point: "Reform the system."

This same physician with whom I was communicating had ideas about how to reform the system: Health Savings plans, changes in tax codes, eliminating government purchased healthcare (i.e. Medicaid and Medicare), etc. In other words, get rid of a controlled, third-party system.

I'm a fiscal conservative, and I would very much love to choose how and where I spend my money, since I now have to spend it where I'm told I can by insurance, plus spend out-of-pocket because the "where I'm told" isn't cutting it. If I choose to travel to those who truly do understand my disease, I have to pay. I made a conscientious and life-saving choice to do just that. That has financially limited other things in my life due to my adjusted priorities, but it has been worth it. Sadly, not everyone has that choice due to circumstances in his/her life.

I often ask, "When does the business of taking care of patients really become about the patient?" I realize it's only possible if those providing it get something from it, too. Yes, they need to make a decent living. I know that. Otherwise, I jokingly say sometimes I forsee a nation of pharmacists with no one to prescribe the medications.


I recently wrote an article about profitability in the business of healthcare. I said:

Where does this put those who cannot afford health care? That's the ethical quandary...Other folks really do have a dilemma between eating and shelter vs. insurance and health care monies. Where will they go? In a "for profit" system, seeing them isn't profitable. What happens then? In my naive eyes, I would hope there is some charity left for them. But even if not, it looks like offering tax breaks to those who serve the indigent may be a cheaper, win-win situation for all involved. Yes, that includes Uncle Sam. Besides, it doesn't seem fair to expect charity for nothing from a tax-paying business just because it's a healthcare industry when we wouldn't do that from our auto-repair folks.

Our Cushing's blogs, including the two with which I'm associated, are to help those veering down the same path as we have gone or are going. Even more, they have a second objective: to hopefully make more folks in the medical community aware that we are people who suffer when we aren't tested, diagnosed, and treated correctly. Finally, it seems they are also being used for a third objective--the need for change in our healthcare system. This was not an intended objective, but I'm realizing it comes with the territory of the other two.

To read more:

From a patient's eyes: Healthcare for profit?

Dear Doctor, I can help....

Healthcare Crisis? Reform? What the medical blogosphere has to say.

It’s not our fault , says Dr. Rob....

IN THE NEWS: Vitamin D: Research appears to support greater daily intake

Vitamin D: Research appears to support greater daily intake

This article in Endocrine Today highlights some of the recent studies on Vitamin D intake. Included are previous articles about which I've blogged, Vitamin D and the thyroid gland... and Vitamin D: "Normal" isn't normal anymore.

Running a hospital: What does it take?

Running a hospital: What does it take?

Well worth the read and in desperate need of patient input. Thanks to e-Patient Dave for leading me to this. I've read Paul Levy's blog before, but hadn't seen this post.

So, take the time to read it and comment. Folks need to hear what patients think.

IN THE NEWS: Subclinical Thyroid Disease: Scientific Review and Guidelines for Diagnosis and Management

JAMA -- Subclinical Thyroid Disease: Scientific Review and Guidelines for Diagnosis and Management, January 14, 2004, Surks et al. 291 (2): 228

(UPDATE: Not just released...oops. But still well-worth reading. I had the wrong document open. )

The basic questions that are asked (and answered) are:

• What is the definition of subclinical thyroid disease?
  
• What is the epidemiology of subclinical thyroid disease?
  
• What are the consequences of untreated subclinical thyroid disease? How should it be evaluated?
  
• What are the risks and benefits of treatment for subclinical thyroid disease?
  Is screening for subclinical thyroid disease warranted?

It also uses a rubric to determine the following:

Strength of the Overall Evidence
Strength of Panelists' Recommendations Based on Available Evidence

Subclincial hypothyroidism is defined and differentiated from central hypothyroidism. Short and long-term consequences are also delineated.

Protein That Regulates Hormones Critical To Women’s Health Found In Pituitary

Protein That Regulates Hormones Critical To Women’s Health Found In Pituitary

The scientists found abundant amounts of the puzzling protein - whose main location and function were unknown until now - in a specific area of the pituitary gland. Like someone at a control knob, the protein may adjust the release of the two hormones that come almost exclusively from the posterior pituitary: oxytocin, which controls many reproductive functions, and vasopressin, which controls fluid balance.

New! Chronically Me's Photostream

Chronically Me's Photostream is a fresh look at Cushing's and it's aftereffects. I can't wait to see more!

Here is just one of her pictures which so eloquently say so much. Be sure to visit because you don't want to miss the commentary with the photo.

Future Treatment Strategies in Managing Aggressive Pituitary Tumors

Within this article, the authors discuss possible future treatments of pituitary tumors. The other day I discussed what is presently done in Current Treatment Strategies in Managing Aggressive Pituitary Tumors. Most of the time transsphenoidal endoscopic surgery is the first-line approach.

According to the authors, dopamine agonists (DAs) will continue to be the best treatment for prolactinomas, with new somatostatin receptor subtype 5 (SSTR5) possible alternatives in the future. The somatostatin analogs, octreotide and lanreotide, will continue to be the treatment of choice for acromegaly.

With Cushing's disease and incidentalomas, there really aren't any long-term pharmacological treatments. The authors offer hope for SSTR5 analogs within the next few years which will effectively treat the Cushing's. A combination of SSTR5 and SSTR2 combinations for treating the incidentalomas and preventing recurring tumor growth is a future possibility.

The authors conclude:

SSTR5 analogs, alone or in combination with DAs, may play a role in the
future medical management of Cushing’s disease, but not in the most aggressive
tumors, and ongoing trials may elucidate the future role of such agents. Until
then, surgery and radiotherapy will remain the mainstay of treatment for these
tumors.

Steven W. J. Lamberts, Leo J. Hofland (2008). Future treatment strategies of aggressive pituitary tumors Pituitary DOI: 10.1007/s11102-008-0154-y

In the News

Thyroid hormone might repair MS damage - 07 January 2009 - New Scientist

Mice, treated to cause MS-type symptoms, were treated with injections of the thyroid hormone triiodothyronine for three weeks and symptoms improved.

Current Treatment Strategies in Managing Aggressive Pituitary Tumors

Michael Buchfelder pulls together recent studies with a good synopsis of current treatments for aggressive pituitary tumors in this article. However, he doesn't address ACTH-producing adenomas which cause Cushing's Disease. He does, however, talk about some of the treatments that are being used for other types of tumors.

Buchfelder discusses the use of dopamine agents to control prolactinomas, and focuses on cabergoline and bromocriptine. Cabergoline is the preferred agent due to its ability to normalize prolactin levels as well as shrink tumor size with few side effects.

In the article, surgery is always the front-line approach for other tumors with secondary treatments to control recurrence and/or regrowth. Transsphenoidal endoscopic surgery is the first-line approach, but he does say surgical techniques can remove the bulk of the tumor "but do nothing to reduce the recurrence of aggressive tumors."

The somatostatin analogs (SSAs) lanreotide and octreotide are mentioned as effective agents for "other kinds of tumors", but only treatment of those causing acromegaly are described in depth.

SSAs can be used as primary therapy, pre-operatively to reduce the tumor volume
and make it more amenable to surgical removal, or post-surgery to control
re-expansion.

He also mentions the recent studies using the chemotherapy agent, temozolomide (another research blog I did recently), to control aggressive pituitary tumors.

Radiotherapy is a non-pharmacological treatment outlined in the article, with stereotactic radiation is only working for small tumors with the "precise location" known. Any type of radiotherapy works best in conjunction with surgical intervention.

He really articulates well the frustrations and complications with treating pituitary tumors with his closing remarks:

...we should not be reticent about using all options available (even in a single patient if necessary) to get the best possible control of symptoms.

Michael Buchfelder (2008). Management of aggressive pituitary adenomas: current treatment strategies Pituitary DOI: 10.1007/s11102-008-0153-z

There is more research on future treatment strategies which I hope to share in the next few days.

To learn more:

Is Temozolomide a new treatment for aggressive pituitary tumors?

A novel pituitary tumor transforming gene identified

A Cushie Champion: Dr. Ian McCutcheon (MD Anderson) (talks about tumors and treatments)

Recent Advances in Neuroendocrine Imaging Lead to Improved Diagnosis

In the News: Lab Acknowledges Problem With Vitamin D Test - NYTimes.com

Lab Acknowledges Problem With Vitamin D Test - NYTimes.com

Since so many of us with or recovering from Cushing's have very low Vitamin D, this is (oh geez...Cushie brain...word for "impactful"...) weighty. (Does that word work?)

Seriously, I had Vitamin D testing done through my local lab and it was sent to Quest in October. One value came back high. Thankfully, my endo didn't trust it and ordered another. It was not sent to Quest, and it came back very low, which is my "normal". By the time I did it a few weeks had passed, but the difference was huge.

An erroneously high result may mean patients will not take vitamin D
supplements when perhaps they should, doctors said. And an erroneously low test
result might lead in rare instances to a toxic overdose of vitamin D. When the
Quest tests have been inaccurate, the reading has typically been too high,
although not in all cases.

From a patient's eyes: Healthcare for profit?

In October, I wrote about our healthcare system in Healthcare Crisis? Reform? What the medical blogosphere has to say. As a patient, understanding what doctors and other healthcare providers face with the deepening and widening regulations is difficult.

It's not that I don't want to help. I do. It's to my benefit. I wrote about that in Dear Doctor, I can help.... But I find it disheartening to read more and more about the problems in various medical blogs with no guidance on how to "fix" it. Yes, I very much understand THERE IS A PROBLEM. Now, why isn't someone (anyone) in the medical arena (i.e. doctors) organizing and using the huge patient base to help?

So, what do I do? I peruse the blogs, read the news, and try to discern what is important. I've found some interesting information. I need to know what you, the doctor/healthcare provider thinks about it.

Businessweek highlighted the Institute for Healthcare in Donald Berwick: Curing the Healthcare System. According to this article, Berwick is a huge proponent of applying business best practices to health care.

"Other industries had long ago started managing for continual improvement in
products, services, cost structures," says Dr. Berwick, who toured Bell Labs, NASA, and Toyota (TM) in the late '80s to see how those organizations approached quality control. "I soon realized that there was a need for this knowledge on a national level."

Does what he says help us? Does this make a profitable business? Is making the practice of medicine "profitable" what we want or need? If it means that a doctor who does better, is better, works harder, and cares more is more profitable, then by all means, YES!

What does that mean, then? If I understand the economics correctly (and feel free to correct me!), then this will lower waste and decrease cost. Technologies which are expensive but often unnecessary won't be implemented without due thought.

Where does this put those who cannot afford health care? That's the ethical quandary. If you watch the video in my previous post (mentioned above), Healthcare Crisis? Reform? What the medical blogosphere has to say, you'll see that a lot of folks don't prioritize spending. They put health insurance at the bottom of the list of spending priorities rather than nearer the top.

Other folks really do have a dilemma between eating and shelter vs. insurance and health care monies. Where will they go? In a "for profit" system, seeing them isn't profitable. What happens then? In my naive eyes, I would hope there is some charity left for them. But even if not, it looks like offering tax breaks to those who serve the indigent may be a cheaper, win-win situation for all involved. Yes, that includes Uncle Sam. Besides, it doesn't seem fair to expect charity for nothing from a tax-paying business just because it's a healthcare industry when we wouldn't do that from our auto-repair folks.

(The big question I have as I write this is how in the heck do we spell healthcare (or health-care or health care)? I've seen it written umpteen ways and even looked it up. Is there a rule? Yes, there is! Healthcare and health care are synonymous as nouns. Health-care is an adjective but may also be written as healthcare. Please don't hold me to this. I'll inadvertently err when I write.)

Comments, rebuttals, teachings, and such are very welcome. I'm all ears...uh, eyes....

A novel pituitary tumor transforming gene identified

Over the next few days I'll take a look at several recent articles about pituitary tumors. This first post highlights The molecular biology of pituitary tumors: a personal perspective by Ashley B. Grossman, renowned Professor of Neuroendocrinology at St. Bartholomew's in the UK. Prof. Grossman draws on his extensive experience with pituitary tumors to highlight what is known about them and what needs to be further explored.

Efforts to establish the ‘cause’ of pituitary tumors have, therefore, focussed on three main areas:

• The mutations involved in genetic syndromes associated with pituitary tumors have been identified and assessed in sporadic adenoma;
• Abnormalities in pituitary-specific signaling pathways have been studied;
• Any candidate genes that could potentially be involved have been studied in animal models of pituitary tumor formation.

In this short report, the findings of this research are summarised, using some of our own studies to look for a commonality of theme.

The behavior of pituitary tumors is notable because they offer information about more aggressive tumors. Genetic studies have offered a lot of information about Carney's Complex, MEN1, and isolated familial somatotropinomas (IFS) but have failed to give much information about "sporadic" pituitary tumors.

Although "abnormalities in the signaling and feedback pathways have been demonstrated in pituitary tumors" causing Cushing's Disease and acromegaly, the evidence currently available does not show they are the cause of the tumors. Conversely, these are probably acquired due to the disease.

There are, however, some pathways which are interesting. According to the article:

• There is abundant evidence that many cell cycle inhibitors involved in the normal regulation of the cell cycle are under-expressed in sporadic adenomas,including p16, p18 and p27 [23], and such dysregulation can be shown to cause chromosomal destabilisation...

  Other cell cycle inhibitors are also under-expressed in pituitary adenomas, but in many cases these are at the level of transcriptional control...

  A novel gene transcript PTTG has been identified in rat GH4 pituitary tumors, and this has been shown to be overexpressed in a large proportion of pituitary tumors compared to the normal pituitary [34]. Furthermore, expression appears to be correlated with invasiveness...

  Despite much excellent research into pituitary adenomas, all that can be stated for sure is that secretory tumors have defective feedback, pituitary tumors are characterised by over-activity of both the Akt pathway and the MAPK pathway, and we can speculate that this may relate in some way to aberrant methylation of tumor suppressor gene promoters.

Professor Grossman concludes:

Little information can be gained from the data presented here to explain the pathogenesis of more aggressive pituitary tumors, since these generally exhibit the same changes described here but to an exaggerated degree. The potential role of PTTG in tumorigenesis and invasiveness has yet to be determined but this gene could play an important role in aggressive tumors.

This is just a brief synopsis of the whole article with much of the technical detail weeded out for ease of understanding by the general reader. However, it outlines some interesting research with reference to the same.

Ashley B. Grossman (2008). The molecular biology of pituitary tumors: a personal perspective Pituitary DOI: 10.1007/s11102-008-0158-7