Happy Halloween

 

Happy Halloween!

This was Alli's first trick-or-treating, and she had loads of fun.

She dressed up as a ladybug, while Ava was a monkey (looks familiar?). Though it got a bit chilly later, she was a real trooper; knowing there was candy to be had, she wanted to go on.

Hope your kids collected a good stash, too! And hope you all stayed safe from Sandy.

An Interesting Case

During my last call week, I had a rather unusual list of patients. Not the usual diabetes management cases.

Of which it included a 58 year old woman who was admitted for confusion. She has an 80-pack-year smoking history. Her ER labs showed her potassium to be low at 2.6. During her stay, she underwent the usual host of screening tests, which eventually snowballed as more things were found. A chest X-ray showed some lung nodules. CT showed several lesions suspicious for malignancy, with probable mets in the liver. Concern was raised more when the PET showed these to be very FDG-avid, with some cranial foci, which was also seen on the head MRI. While awaiting tissue diagnosis, the oncologist then started her on dexamethasone for the brain mets.

Somewhere along the way, an astute clinician noted some she had some unusual exam features. She had a facial fullness and obesity, with numerous bruises as well. Family reported dramatic weight gain in the last 5 months too. Thus he ordered 24-hour urinary cortisol measurements.

This came back high. Over 40 times normal. Now, considering that cortisol is a stress hormone, it's not too unusual to see elevated levels in the sick patient. But to see levels that high, especially knowing that the patient was also on dexamethasone which tends to suppress endogenous cortisol, suggests Cushing syndrome (or Cushing's, depending on whether you want to be historically or politically accurate). After all, recall one of the screening tests we do is the dexamethasone suppression test. Hence, they requested my involvement.

I repeated the cortisol, this time on a morning serum sample, while she remained on 6 mg of dex every 6 hours. This again came back high at 48 (should be suppressed to less than 3).

Typically, once Cushing syndrome is confirmed, the next step is to determine the level of the problem. Is this primary (ie adrenal source) or ACTH-dependent (the distinction between Cushing disease and syndrome. Usually of a pituitary nature, but wasn't the case here)(hint hint).

As it turns out, her ACTH was indeed high. And the core biopsy of the liver metastases was consistent with adenocarcinoma, probably of lung origin. But by this point, the patient was doing poorly enough that the family had decided to put her in hospice and she was transferred.

It was a very tragic case. But from an academic standpoint, it was very interesting for my resident as this wasn't the usual consult we get, and had numerous learning points.

In this case, there were numerous clues that this was an ACTH-dependent Cushing syndrome. And very likely, of an ectopic source.

The very high cortisol levels and acute onset make it likely that this was an ectopic source of ACTH, not merely pituitary.

In addition, the hypokalemia that was noted on admission was another giveaway- cortisol has a weak mineralocorticoid activity, but at massive amounts, can exert the same effect as aldosterone.

In addition, her pituitary was normal on the MRI.

All suggesting that the source of the ACTH was the lung malignancy. I have to admit, I was expecting the pathologist to call this carcinoid, or small cell as this is usually the case. Adenocarcinoma of the lung would not be expected to secrete ACTH. Staining the tissue blocks for ACTH wasn't possible as there wasn't enough left; but I suppose it's more an academic question than clinically relevant to her, unfortunately. But if it was confirmed, this probably could be a publishable case (my hunch though is it wasn't adenocarcinoma, but small cell).

In these cases, the treatment of the Cushing should be resection of the source if possible. In a single focus with no extensive metastasis this might be achievable. In cases where the focus cannot be found and the prognosis is moderate then we have some medical options. This is sometimes the case for carcinoids where patients may expect to live years or decades even if metastatic.

And so in those cases, the options might be:

• Bilateral adrenalectomy with subsequent steroid replacement- but one has to be cautious of steroid withdrawal and start off with a supraphysiologic dose of Hydrocortisone. After all, the body has gotten used to having high levels of cortisol
• Medical adrenalectomy with Mitotane- though the onset of action can be slow
• Supression of glucocorticoid synthesis- using Ketoconazole, Aminoglutethimide, Metyrapone or even Etomidate. Some of these agents are hard to get- and though Metyrapone isn't commercially available I've been able to get it shipped from the makers on a compassionate-basis. And I've used Etomidate in a patient critically ill in the ICU so the sedative/anesthetic effect was useful.

Often, if this has been ongoing long enough, you'd also see bilateral adrenal enlargement from the stimulatory effects of the ACTH. It wasn't the case here, but it certainly was in my last ectopic.

Last I heard, she passed away last week, less than 2 weeks after I signed off. Sad, but I was at least heartened to meet the family and know that she got to meet her new grand daughter before she passed on.



EBM

I have to get this off my chest.

It's been bothering me somewhat for the last couple of weeks.
If you've been following that thread on Facebook, you probably know what's been going on. I belong to a FB group, name of which I won't divulge, that consists of Malaysian doctors. Primary care and specialists. Things started when another doctor asked me for my opinion as an endocrinologist about the numerous supplements he was taking. L-thyroxine, some compounded testosterone and adrenal androgens, as well as a host of other supplements. He called it his 'cocktail'. And so I shared that I personally wouldn't recommend it as the evidence out there does not suggest it should be done.

And this started a heated online debate about the topic of evidence-based medicine, not only related to endocrinology but medicine as a whole. While I fully acknowledge that individuals are for sure going to have different opinions and I respect that, what I wasn't prepared to see was the number of comments from people who were totally condemning the concept of EBM.

And perhaps this was a greater surprise to me because one of the integral foundations to my training at my alma mater is that we've been drilled to always question the data. To critically appraise a study and read between the lines; to not just accept the authors' conclusions but to find flaws and to make our own decisions about its validity. As residents, we regularly attended Journal Club conferences and presented at quite a few. When we suggested something, our attendings would often challenge us: "Cite your references..."

In fact in some of my papers you'd see me actually spelling out what the limitations of my studies were; not just presenting the strong points.

It's a principle I hold closely and I apply it to tests I might order, or medications I may prescribe.

"Prove to me that your medication results in better outcomes for my patient"

"Prove to me that your medication is safe"

It's something I've assumed the scientist in all physicians would question.

And so, it was surprising to see such a shift in thinking, to see my peers, just dismiss EBM as some voodoo. Or that EBM is flawed because it is manipulated by pharma. That "EBM is the worse form of science". Or that EBM is not reliable because no one studies natural supplements that are not patented because of the lack of profit.

The criticisms of the concept of Evidence-Based Medicine was staggering, and almost seemed to be bordering on ignorance or paranoia. And while they were dissing this, many seemed very open to recommending unconventional or alternative treatments. Quoting not textbooks or medical journals, but websites or books on spirituality, with generous statements like all lung cancer was due to "one of 30 mutations", or "this works because I felt better on it" and "where can I get some?"

The truth is, EBM is flawed. Yes, it's not a perfect science. And it can be intimidating- though I've published some papers and have done some research, I still consider myself a greenhorn and get overwhelmed sometimes (and I recall actually hating EBM as a medstudent). And science itself does not tell us everything. True, studies might be biased by monetary or nonmonetary factors. And the lack of evidence regarding efficacy of a treatment is not the same as the presence of evidence showing harm. But that's precisely it. The concept of EBM is not to say we should believe the publications out there. That all scientific studies are reliable. Far from it- the basis of evidence-based medicine is that it is meant to make us question the data. To force us to use our analytical brain and to make our own decisions on whether or not to believe the manuscript we are reading. To determine if a study was well-designed and free of bias. If the results are valid.

I realize that professional opinions differ, not only interregionally but even here. But I have to admit, I'm somewhat disturbed by the noise that was generated. I can't help to wonder, if doctors were not making treatment decisions based on the science, then what else is there to guide us?



Pumpkin Carving

Can't believe how time flies. Doesn't seem that long ago when Alli was a baby. Now, she has a younger sister, and she's 2 1/2 years old. So this year, she was much more into pumpkins than she was last year. She seemed like the fearless little tiger that she is, sticking her hand into the icky, sticky insides to get the seeds out. At the end, I'd say we had a pretty little pumpkin carved out (no, ours was the simpler one on the right; the one on the left was carved by some Malaysian students we hosted).

So, the pumpkin's all set. Now all we have to get in the candy...

Rambutans

Only a Malaysian will find this amusing. Two measly rambutans for one dollar?? With how many of these my neighbor Uncle Ang had in his rambutan tree, he'd be a millionaire!

Why We Need Women

And so, because my wife is on maternity leave, and I work, she has been gracious enough to do the nighttime feedings during the weekdays. Ava is 7 weeks old now, and feeds every 2-4 hours at night. It's not too long- she'd gulp down that 2-3 oz in 5 mins, but to have do wake up and clear your head enough to actually do it, and then clear the bottle and go back to bed, can be a pain in the butt.

And so, bless her heart, that amazing women does all that without complaining, providing nourishment to our little munchkin.

On weekends, I try to be more helpful with the feeding. But here's my observation: I'm up 3 times at night only on weekends, but I mope around all day whining about how sleepy I am. Or I give her that look, one that says "Lookee here, I fed the baby last night, don't I deserve a gold star?"

Heh, what would I do without my woman?

Next: Let's compare men and women when they're ill

Cooking Malaysian

One thing about being away from Malaysia is the food. So, one thing I tell new students they should do to ease the home sickness is to learn to cook Malaysian. Nothing special, but just pick a few of your favorite dishes from Mom's arsenal.
And so when I was in medical school in Canada, I learnt Mom's chicken curry and oyster sauce chicken. And I kinda got lazy and stopped there- learnt many more recipes, but not Mom's.

Naturally, I miss many of her dishes. But this trip, my wife made it a point to watch my Mom cook, and made lists of recipes.

And so this week, I've been treated with some of my favs: Honey Chicken and Fried Wantan! My wife whipped them up when I was at work. 

Maybe we need to extend her maternity leave- maybe I should get her pregnant again!

ADA Walk

It was a blustery fall day. Almost too cold, but we took part in the American Diabetes Association's Step Out- Walk to Stop Diabetes event. And yes, we were crazy enough to bring the two kids, though we we're expecting the morning temperatures to be below freezing so early in autumm.

The numbers however, are staggering. Almost 26,000,000 people in the country, or 8%, with over 6% of people about 65 years of age. Diabetes remains the leading cause of blindness and kidney failure, and is a major factor in vascular disease. And this number will only get higher in the future.

Though as an endocrinologist people say I shouldn't be complaining because of the job security, the truth is it's a worldwide problem that I wish we could do without. It's a (usually) chronic issue to which we have (usually- exception being something dramatic like bariatric surgery) no cure, only ways to treat it. And this usually requires tedious glucose testing and expensive medications. And so for patients and physicians alike, it's a very unsatisfying relationship. You are merely controlling the beast, usually not eliminating it.

The cause? Probably a very complex one, but in large part due to the ballooning of weight, obesity, that plagues modern society.

But it felt good to be out there. To see people united in their resolve. And perhaps someday, we can tame this beast.

Adoptions!

So, we've adopted a couple of kids.

Well, not in the way you might think.
We found out last year that the university here is very popular amongst Malaysian students- they have a pretty strong economics/acturial sciences program. And, as it turns out there is a program to sign up to be host families for any foreign students.

And so, Kris and I thought it would be neat to maintain that Malaysian connection, and to help any newcomers feel more welcome.

After all, I remember my first years being away from home, being in Canada. I remember the cultural shock, the home sickness. How much I appreciated that my mentor Dr. Baumber took us in and hosted the Malaysians numerous times. Or how nice it was for that random Malaysian woman to contact the U to get in touch with us students and how her family hosted us for that ski trip in Kananaskis. Admittedly, I thought it was strange for people to do that initially, though perhaps now that I've aged and matured somewhat, it seems to be the right thing to do.

We all remember how student life was. How most of us were so budget conscious that we ate miserably (a slice of ham on toasts was my staple weekday dinners!)(that being said, times seem to have changed; these students seem to be sporting the newest iPhone 5!).

And so, we signed up, and 'adopted' two kids (well, three if we included the guy's girlfriend)- a 20 year old 'boy' and 'girl'. So far, we've had them over a few times, and took them out for dinner once. And while I cringed when he called me "Mr. Vagus"-made me feel old- it's been nice hearing that familiar accent.

Someday, after they're more used to us, I'm going to start using them as my guinea pigs and start making some Malaysian food- stuff I haven't cooked in years because Kristin thinks they're nasty.

Remember the nasi lemak incident?

 

Fall

It's fall. And it's my wife's favorite season of the year. Me, I'm still not sure; I think every season has its pluses and minuses and I'm undecided if I actually have a favorite time. Maybe it's because I grew up in non-seasonal Malaysia, where things are the same year round (with the exception of the monsoon rains). And, as in my previous post- "autumm" has a different meaning in Malaysia, more so of a cultural event with the Moon Cake Festival.

But, that being said, I love the fall and what it brings.

Yellowing of the leaves, and how everything rustles under your feet

Those blustery cool days

Pumpkins, cinnamon

Halloween

Longer nights, shorter days

 

All leading up to Thanksgiving and the (dreaded) winter. For some reason, autumm sometimes makes me sad, too- I don't know why, but it seems to remind me of the end of things. That the summer, the year is wrapping up.

What is YOUR favorite season?

Ah the simple pleasures of life. Mom sneaked this in when she visited last month and thanks to her I get to relive childhood memories of the Mid autumn Festival. Perhaps someday I get to show my girls what it's like to light up candles in the tang lung and walk around the neighborhood at night.

Happy Moon Cake festival!