How to catch a virus: Flu 101

NPR has a great video on how flu viruses spread and multiply.  In this video, "NPR's Robert Krulwich and medical animator David Bolinsky explain how a flu virus can trick a single cell into making a million more viruses".





Although this isn't really Cushing's related, it is pertinent to all of us.  We are all immunocompromised with this disease, and everything we can do to prevent infection is important to us!  Besides, this is wonderful animation and I'm a real David Bolinsky fan.  I know you'll enjoy him.

For more interesting reading/viewing:

http://xvivo.net/
http://multimedia.mcb.harvard.edu/

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More on taking Ketoconazole to lower cortisol

Well, the past week has been very interesting with the Ketoconazole.  I've learned some things by taking it:

• I definitely cycle. 
• I have very low cortisol in the mid-afternoon (worse on Keto) and it is difficult to function without some additional hydrocortisone.  I come home from work and crash each day.
• Even taking the keto in the evenings, I still get more "wired" and less tired around 9 p.m., so my body/tumor is producing more ACTH/cortisol then.  And it gets even more wired around midnight or so.
• Taking the Keto makes me low in cortisol most of the time, and I have to replace some right now in the morning by taking hydrocortisone.  But I don't think I'm low enough at night. However, this is working to simulate a normal diurnal pattern.  Thus, I'm sleeping better.

I haven't lost much weight, but I have lost inches in my stomach area.  I go up and down in weight, so it's hard to know if what I have lost is from cycling or from the keto. 

This isn't easy. I'd be lying if I said it was.  On Wednesday, I posted this on the Cushing's Help message boards:

Oh man...whew.... this is hard on a person. I definitely know what my lows feel like. From 3-9 (I'm assuming I'll do the same around 9 tonight) I feel like I just can't walk another step or even stay awake. I absolutely had to go to the grocery store after work, and I just did not think I'd make it. My cupboards and refrigerator were bare. It was so hard to do, but I did it. Once I got home, I thought I'd never unload the minimum amount I managed to get. And only the cold stuff is put away. The rest are on the table or right inside the door where they were dropped.

I took another 2.5 [of hydrocortison] at 1 p.m. today because I had such a hard time standing up. I felt it coming. Every joint, every muscle hurts. My knees are horrible. If I stay awake 'til 9, I'll let you know how it goes. I suspect I'll perk up then.

I think I feel my cycle even more on the keto...not less.

Nausea is also bad in the afternoon/evening, but then I get really hungry around the 8-9 time.   It's really the only time of day I'm hungry.  Eating at night and not during the day is not a good habit.  I'm trying to eat something in the mornings and then at lunch.  Actually, lunch is ok if I eat it early enough before I start feeling bad.  And if I eat dinner (called supper around here), I am eating something light and low-carb because it's late when I do want to eat.

Again, I sure wish I had a "cortisolometer" to be able to monitor my cortisol levels.  That sure would help me know whether to take hydrocortisone and how much to take in the mornings and early afternoon.  I don't want to take it if I don't need it because then I'm just replacing what I'm trying so hard to block.  I do want to emulate a normal diurnal level all day long.

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I've started Ketoconazole...

...and it's a crap-shoot....

Well, I bit the bullet and started a course of Ketoconazole to see how I do. Keto works by blocking the secretion of cortisol by inhibition of adrenal 11 beta-hydroxylase. Since no two people respond the same, it's a guessing game at first.


My endo started me on 200 mg of the Keto at 6 p.m. and another 200 mg at 10 p.m. In a "normal" person, cortisol is the highest around 8 a.m. and decreases to about half that value around 4 p.m. By midnight (give or take an hour each way), cortisol should be about zero or close to it with blood and salivary levels. This is a normal diurnal variation. It is also called the circadian rhythm.


When one has Cushing's, this circadian rhythm is lost, and the normal diurnal variation changes. Cushing's patients have "flat" diurnal levels of cortisol, or even higher levels at night instead of in the morning. So, taking the keto at those "high" times lowers it to more natural levels, which is what I want.


But, the flip side of this is I don't produce the normal levels in the morning. So, I essentially have a type of "morning sickness" a lot of mornings with low cortisol until my body (aka...tumor) kicks in and makes some. (Read more about the Cushing's diurnal variation here.) So, I already don't make it in the mornings (or very well), and now I'm lowering it in the afternoon and evening.


Here is where the guessing game comes in. If I need to replace/add "cortisol" in the form of hydrocortisone in the morning, I also have a prescription of that. But how do I know? It varies by day, and there is no meter to help me. I so wish there was. I'd give my eyeteeth for a meter similar to a glucometer so I could figure out how much I need. The only thing I have to go by, except for regular serum cortisol checks which can't be done daily , is how I feel.


And what is so concerning about that? Cortisol is a corticosteroid hormone produced by the adrenal cortex, the outer layer of the adrenal glands which are essentially lying on top of the kidneys. Without it we die. It is the hormone that responds to "stress", both good and bad. It affects levels of multiple other hormones and electrolytes, and is very important in the homeostasis of the body. I need ENOUGH. Not too much and not at the wrong times. If I don't have enough, I'll end up with adrenal insufficiency, which is life-threatening.


Why this instead of other treatment? We are working toward a long-term solution for me. I explained it in the Medscape interview with Dr. Colin Son. It's not an easy choice.


I don't know how this is going to go. I'll let you know. I hope I can read my body right.

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I am sad....

We lost another of our Cushies. I am sad. Mary O'Connor posted about Bonnie at one of her blogs. "Another Cushing's Patient has Died" talks about Bonnie and what we know about her short life.

Grand Rounds Volume 6, Number 4

When I chose the theme for this week's Grand Rounds, I chose it because it is near and dear to my heart. Before I knew what it was called, I was looking for a "participatory medicine" model. Long before I heard the term "ePatient", I was one.

Since the Society for Participatory Medicine is the next evolution of the group at http://e-patients.net/, it makes sense to start by having them spell it out. Their new Journal of Participatory Medicine is launching next week at Connected Health, and Co-Editors-In-Chief (a physician and a patient, of course) explain the purpose clearly on the Society's blog, in Why the Journal of Participatory Medicine?

If you still aren't certain what an ePatient is, in The e-patient white paper: Seven Preliminary Conclusions, Dave deBronkart (better known as ePatient Dave) points out ePatients are not "anti-doctor" or "anti-medicine". Rather, they "use the internet...to access content, connect with others, and collaborate with others in ways never possible before."

John Sharp of the Cleveland Clinic wrote a guest-post called Journal of Participatory Medicine and e-Patients .

If you have not read the e-Patient White Paper, you do not understand the future of medicine...Being an e-Patient is beyond being empowered. The subtitle of the paper, “How they can help us heal healthcare,” describes the potential for a revolution of change....

In Participatory Medicine: Text of my speech at the Connected Health symposium, Susannah Fox of the Pew Research Center’s Internet & American Life Project hits the nail on the head in her response to this:

In December 2001 the American Medical Association put out a press release suggesting that Americans make a New Year’s resolution to “trust your physician, not a chat room” since the information found online puts “lives at risk.”

Recently, Susannah presented the latest data on social media and health care with Social Media's Promise for Public Health . If you want some eye-opening data, check out the slide show on this one. (Did you know 79% of adults use the Internet, up from 46% in 2000?)

"I asked doctors for three years - PCP, gynecologist, neurologist, podiatrist - all said the now-famous refrain. It's too rare", says Mary O'Connor in her Participatory Medicine post. Her riveting story exemplifies the move from patient to ePatient and the need for participatory medicine.

A thousand kudos to blogger Brian Ahier for showing us this wonderful Seinfeld clip in which Elaine has the chutzpah to look at her folder in the doctor's office.




Of all the submissions, Brian's is the only one to point out that October is Patient-Centered Care Awareness month. His October 1 post, Patient at the Center, mentions the principle "Access to understandable health information can empower individuals to participate in their health care" and contrasts that with the words of Hippocrates and Oliver Wendell Holmes that discouraged physicians from letting patients know what's going on. It makes one wonder if the physicians Elaine visited were trained by Holmes.

Amy Romano, a nurse-midwife and advocate for mother-friendly maternity care illustrates the need for patients to "become empowered, engaged, equipped, and enabled...before they face life threatening illnesses or need to manage chronic conditions" in A Lifetime of Participatory Medicine Can Start With Maternity Care. She originally posted a version of this at her own blog, Science and Sensibility.

How does one become an ePatient? An empowered, engaged, equipped, and enabled patient? Trisha Torrey gives some great guidelines in her article Tools for the Savvy Patient. This is what PM should look like to a patient who has never heard of it. She includes clear, simple, sensible advice on how to play an active role and have good communication with one's practitioners.

Speaking of good communication, Dr. Rob Lamberts practices participatory medicine every day with his patients. In this post he identifies with his patients and their Anxiety. He talks about how he works with his patients honestly and openly to deal with this and other life issues.

Charles Smith MD, co-editor of the JPM, tells a story of a specialist who responded to a patient's request by looking, not ignoring, and found a new medical association he'd never known. He talks about how doctors can learn from patients:

"...patient outcomes will be improved if we encourage them to ask questions,
and to enlighten us when they come across something that may be relevant.
...Equally important, it is critical for providers to remain open to being guided and educated by our patients."

Chris, of the Man-Nurse Diaries, wrote to me "As a nursing student, I find the idea of participatory medicine akin to the idea of patient advocacy, and I think both ideas need to be spread to patients/individuals and their families." He talks about how this works in his post Participatory Medicine.

Barbara Olson at Florence dot com goes even further. She says, "Since we're all seated at the grown-ups' table, let's get this on it: Healthcare providers are fallible humans. It's not 'if' we make mistakes, it's when." She takes participatory medicine a step further with thoughts about how patient safety can be made better by participatory processes in Participatory Safety.

Society president Alan Greene, MD of DrGreene.com likes to quote Thomas Jefferson, citing Jefferson's view that "When the people are well-informed, they can be trusted to govern themselves." He talks about how at one time this didn't apply to women or slaves, and how it still does not to patients. A provocative Glimpse of American Healthcare of the Future , this reminds us of the premise on which our nation was founded.

Heathcare is not something done ‘to’ us or ‘for’ us. Health is something we
foster ‘with’ our family, our friends, our community, and with other people
dedicated to fostering health.

Hugo Campos, in his post My Journey from Patient to Consumer of Health Care, echoes this sentiment. At risk for sudden cardiac death (SCD), he was misdiagnosed at least 3 times before he became an ePatient and started advocating for himself and then for others.

David Harlow at his Health Care Law Blog, Healthblawg, has some wise advice for providers. His post A Declaration of Health Data Rights: Can't argue with it, but it's only a first step includes a proposal for participatory-medicine-minded providers. Announced less than four months ago, the Declaration of Health Data Rights has over 1200 endorsements by individuals and organizations. Can you say "handwriting on the wall"? We sure have a long way to go, though, and we all must work together to make this happen.

Speaking of health data rights, no discussion of participatory data sharing would be complete without a mention of e-Patient Dave's earnest attempt to move his PHR data from his hospital to Google Health last March. The mess that resulted (and his articulate post about it) had unforeseen effects. It landed him on the front page of the Boston Globe, brought him to Washington for policy meetings, and led to his delivering the opening keynote, "Gimme My Damn Data," at last month's Medicine 2.0 Congress in Toronto. Data sharing is a vital part of participatory medicine, but again, we have a long way to go.

A key factor in PM is the altered relationship - not just providers "letting patients in"- but the shift in responsibility. As every physician knows, the physician may advise, but then the patient does or does not take the advice. This issue was discussed in a trilogy of posts:

• "Physicians are Coaches, Patients are Players" (quoting Stanley Feld MD)
• Sequel: "Embrace Knowledge Symmetry"
• "The Ideal Doctor/Patient Relationship" (ICSI President Kent Bottles)

The Health 2.0 conference in San Francisco last week was covered by an empowered patient, Amy Tenderich of the Diabetes Mine blog. In “Health 2.0″ - The Buzzword You Hate to Love she gives her patient's view coverage. In her post she says, "...in this entire national dialogue about healthcare reform, there’s simply not enough patient representation!" I agree, Amy!

Another patient, Lauren, talks about how tough it is to be My Own Advocate as she deals with multiple autoimmune diseases. She highlights the conundrum with ill patients who need to advocate for themselves and yet are too sick to do that.

I always tell people that you have to be your own advocate, but it can be downright exhausting under the best of circumstances let alone when you are sick.

And last, but not least, Dr. Charles will close this edition for us with Watching my Grandfather Watching the Sky. Although not exactly on topic, it still fits as it explores the melancholy satisfaction a physician feels at the end of the day, and the heroism of patients dealing with life's struggles.

And as an addendum: While researching this issue of Grand Rounds, I came across this incredibly moving post. It doesn't address participatory medicine but it does a powerful job of reminding us all of why we care: lives are at stake, and when healthcare fails, there are real human consequences. Please read artist Regina Holliday's detailed description of the symbolism and meaning embedded in her now-famous mural of her husband Fred's death last June

Thank you for reading this edition, and be sure to catch next week's edition at Sharp Brains.

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Stars Go Blue

"It happened in early August, and quite frankly I haven't had to strength, spirit, energy, to give an update. I was away at NIH with Jordan for her yearly
follow up... I left Sam with her dad and 14 year old sister. "



This is how Jackie, Sam’s mother who posts on the cushings-help.com message boards as Samsmom, started a post. My heart sank when I read it.

At these message boards, we all know Sam (short for Samantha) Edgar even if we’ve never met her. She is near and dear to our hearts. Featured on the Mystery Diagnosis Season 1 segment, “Grasping for Straws”, Sam’s early childhood story paved the way for many of us to even hear the word “Cushing’s”.

The youngest of Jackie’s three girls, Sam was born on March 22, 1999 with “enormous chipmunk cheeks and was bright red” according the biography written by her mother. Immediately sent to the NICU for breathing difficulties, at day three she was diagnosed with hypertension. Increasing doses of medicines to decrease the hypertension did not work, and by day 18 Sam was transported to Seattle Children’s Hospital.

In her biography, Jackie describes the nightmare that she lived while watching her daughter get sicker and sicker with the high blood pressure, necrosis and more. No one was helping. By the second month of Sam’s life, she says “we realized we were on our own”, speaking of herself and her husband, Bill. Because Sam was born 5 weeks early, the countless doctors they saw all contributed her problems to the premature birth.

During the course of the first three years of Sam’s life, she cycled between being “small, thin, and crabby” to chubby, red, and agitated. She would gain 10 pounds in 10 days, have a high cortisol reading, and doctors would attribute that to overeating and high stress. The only thing that seemed to soothe Sam through all this was the Ryan Adams song "Stars go Blue". Jackie recounts playing it over and over to get her to sleep. Never did she sleep more than 2 hours at a time .

Still seeking answers, Jackie first heard the words “cyclical Cushing’s Syndrome” while visiting an endocrinologist at Arizona Children’s Hospital in Scottsdale. Shortly afterwards, Sam gained 15 pounds in as many days. Her round face covered in acne and her round tummy with stretchmarks, all she could do was rock and cry. Nothing consoled her but “that damned Ryan Adams song and her black cat Max”.

Jackie said, “About that time I posted my first post on [the cushings-help.com message boards]. I was desperate, angry, terrified and sick with grief. I KNEW Sam was going to die. My posting was answered by the angels [there]... all agreed Sam had Cushing's, all gave me strength and validated me... all were rooting for Sam.”

Accused of Munchausen’s by proxy, of overfeeding Sam, and more, Jackie had the fight of her life. No, of Sam’s life. The Mystery Diagnosis segment tells parts of it. Jackie recounts it this way:

Dr. [Dan] Gunther admitted her for testing. He contacted Dr. Stratakis at [the] NIH and followed the testing protocal recommended by him. All of Sam's tests revealed a Cushing's diagnosis. On day 3, Dr. Dan told me there was some suspicion among the hospital that Sam had been given mega doses of steroids and was being poisoned. I looked at him as though he had gone mad. He said that some docs felt I was giving her steroids. They thought I had Munchausen by proxy. I assaulted Dr. Dan verbally and he took it. Then he told me that he disagreed with them and kept Sam another day to prove that Sam's adrenals were the ones making the steroids, not Sam's mother.

The tests showed that Sam's adrenals were in fact making the cortisol. I was cleared of any wrongdoing.

Sam and Jackie made several trips to the NIH for testing. Ultimately, Sam was diagnosed with primary pigmented nodular adrenocortical disease (PPNAD), a disease which causes the adrenal glands to make too much cortisol. On April 8, 2003 (Harvey Cushing’s birthday and Cushing’s Awareness Day), Sam had a bilateral adrenalectomy (BLA) to remove both adrenal glands. She would be on steroid replacement for the rest of her life. Adrenal crisis and emergency/stress dosing would be a regimen she and her family would always have to follow carefully.

Two years later, the Mystery Diagnosis segment was filmed. A little over a year later, Dr. Stratakis of the NIH contacted Jackie to tell her they found out what caused Sam’s problem. It was a gene handed down to Sam and her two sisters by her Dad. One sister and her dad remain symptomless. The older of her two sisters, Jordan, was not as lucky. She has had multiple trips of her own to the NIH and subsequent pituitary surgery and two unilateral adrenalectomies. She and Jackie were at the NIH for her yearly follow-up this past August when it all began. Here is a portion of what Jackie wrote:

Sam started vomiting early one evening after farm (day) camp. Her father gave her a 15 mg stress dose, then LEFT HER ALONE to go pick up our 14 year old from cheerleader practice. Sam continued to vomit and lose consciousness... he wisely realized she was in trouble and took her to Seattle Children's. ... He did not give her the IM injection and informed Seattle Children's that her 'stress dose' was 15 mg- which they proceeded to give my unconscious baby through an IV. He did NOT bring the emergency bag (labeled clearly and full of everything we need for AI + directions that is in each and every one of our cars). He also foolishly decided NOT to call me in DC OR call my mother, sister, or neighbor that are all fully aware of Sam's need for a 100 MG hydrocortisone injection in the event of adrenal crisis.

Sam lay dying for 13 hours with Seattle Children's endocrinologists standing around watching and wondering why the continual 15 mg they were giving her wasn't working.

Finally. he called me. I was in O'Hare airport waiting for our plane to come home. I screamed she needed 100 MG NOW! He called Sam's endocrinologist at the hospital across town who immediately ordered 100 mg... it did nothing... her bp fell to 60/22. He ordered 100 more.... nothing....my plane was boarding... I asked the endo on the phone "has it gone too far? I know there is only a small window to be treated--can they bring her back?" I was told "I don't know". And THEN I boarded my plane for Seattle.

I'll spare all the details of my 5 hour incommunicado trip home.

I arrived in Seattle near midnight. I called from the plane asking is she was still alive. She was. She had had another 100MG of hydrocortisone at this point. But her organs might be failing; they weren't.

I endured the longest night of my life (and those who know me know just how scary that statement is)...and Sam pulled through. She was not coherent much when I got
there... but burst into tears when she finally recognized me.

Sam has had no residual damage from this event. We all wonder how and why.

My nerves of steel have rusted...I am beating myself up for not properly educating
this man (her father) as to the seriousness of our child's disease. We have dealt with Addison's for 7 years; but I have handled everything. Apparently the vials of solu-cortef with step-by-step instructions hanging on the bulletin board in the kitchen, medicine cabinet and in every vehicle somehow missed his attention.

My 14 year old has written an essay- "if I could change one thing" about this incident for her English class.

My heart is broken. I never want to see that again. I never want to be away from her again. I can't trust anyone with her--I am anxious sending her to school. I have taken a step 10 years back. I wish I believed in God. I don't think I have ever said that before, but boy, I do now.

This isn’t the end of the story. For page and pages of posts, Jackie and her family got support. But more than that. Folks brainstormed. How can we prevent this again? What are options we can use for ourselves, for Sam, for Jackie, for Jackie’s family? Everything from drills (similar to fire drills) to tattoos and more was discussed. And we all learned from this. Our Cushie family has many children, teenagers, and adults who could have a similar situation. We realize we all need advocates and we all still need to educate our medical helpers. We still must participate in our own treatment. Sometimes we really do know more than the doctors treating us.
Sam is now armed with a cell phone of her own with Jackie’s number and 911 programmed into it.

Jackie says in her biography, “I often wonder what happened in my other lives that dealt me such a tragic hand... whatever it was I hope it was fun. Sometimes I pretend I am a character in a Robin Cook novel... it isn't that far of a stretch.”

And she says, “I can hear Sam's CD still playing upstairs where she left it on... I swear to God it's playing Ryan Adams ‘Stars Go Blue’.”

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To hear Jackie and Jordan interviewed by Mary O'Connor and Robin, listen on iTunes or on BlogTalkRadio

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Grand Rounds Volume 6, Number 4 -- Call for Submissions

I will be hosting Grand Rounds next week for the first time. The theme is "Participatory Medicine". If you need ideas, the "parameters for participatory medicine" are an interesting read.

Please send your best posts to me at survivethejourney@gmail.com by midnight EDT, Sunday, October 11 with "Grand Rounds" in the subject line. It will help if you include the following:

• URL:
• Author:
• Why this submission illustrates participatory medicine

I look forward to hearing from you!

SurgeXperiences 307

Welcome to the October 4, 2009 edition of SurgeXperiences. There is quite a variety of posts. I thought I'd give you a snapshot view of each blog just in case you haven't already seen them. Enjoy!



Posted at Aggravated DocSurg you will find The Not-So-Accidental Tourist and Hic Sunt Dracones.


Øystein Horgmo presents No Scrubs and The Party Bag posted at The Sterile Eye. The former is "A little anecdote concerning scrubs" and the latter "My little stoma bag spoof" , he says. I think he has a sense of humor and likes a joke.



bongi presents the baby story posted at other things amanzi, which he says are "the very old days". Also on his blog is selfish bastard with the comment "some things we see are not so cool" .



Ramona Bates presents Historic Treatment of Burns posted at Suture for a Living. As always, this is a wonderfully documented article with plenty of quotes.



Karen Little submitted three fine posts at Just Up The Dose: Time versus Money, Happy Hour, and Biggest Lipoma Ever! (with a picture to go with it!).



For some good Medical Humor check out It's a wonderful life.



The story, Surgeon attacks cataract surgery funding cut, is posted at ICNN.



Showing life as a patient, I'm back is posted at Althouse. The comments make the post!



Surgery Day, Again posted at Baby Steps is a scary story about a reporter's patient husband's surgery after swallowing something in his chicken finger.



Surgery at Mount Meru Hospital, posted at The Lancet Student, explores a hospital in Tanzania. "Patients had to pay for operations and there were several occasions when a patient had not provided adequate funds and their operation had to be postponed".

Why Does Everyone Love a Brain Surgeon? is also posted at The Lancet Student.



Venturing into Pediatric Surgery posted at Eisha'z Inner world. "I was transplanted to an entirely different place, where the patients are smaller, have rare congenital anomalies and typically can't communicate directly with me. "



Finally, What was surgery like in the 1930s? posted at Kevin, M.D. shows old youtube videos on surgery in the 1930's.

That concludes this edition of SurgeXperiences. Submit your blog article to the next edition of using this form. Past posts and future hosts can be found on here.

I enjoyed hosting. Thank you for visiting.