Monday, 28 December 2015

Least favourite diagnoses

I have a little internal list in my head that I update from time to time whenever it strikes me.  The official title of this list is "My Least Favourite Diagnoses", but my pet name for it is "If I Ever Get This, Please Kill Me".  For your reading displeasure, I've decided to share that list with you fine folks:
  1. Penile cancer - duh
  2. Fournier gangrene - a necrotising infection of the scrotum, penis, and perineal area which I've warned you not to Google in the past.  No, I'm dead serious - DO NOT GOOGLE THIS.
  3. Fibrodysplasia ossificans progressiva - All the muscles and connective tissue in the body are progressively converted to bone, causing the appropriately-named Stone Man Syndrome
  4. Whatever the hell Joseph Merrick (the Elephant Man) had
For god's sake, please do NOT search for pictures of any of those things.  If you do, I hereby declare and avow that I will NOT be held responsible for any damage done to your computer, phone, tablet, etc from being coated with your own vomit.  And you probably shouldn't click any of those links that I accidentally provided on purpose.

There's one diagnosis that is currently not on the list that has been on there a few times, but it falls off and gets replaced from time to time (at one time it was replaced by Ebola).  I waver back and forth on whether or not this diagnosis qualifies, since it is technically treatable though not curable.  Regardless, even if it doesn't officially qualify for my list at this particular moment, it still qualifies as my least favourite surgical diagnosis for my patients:

CROHN'S DISEASE

There is precious little that can strike fear into the heart of a general surgeon (the good ones, at least).  But hearing the words "bowel obstruction" (or any other surgical catastrophe) in conjunction with "Crohn's disease" and "multiple prior surgeries" can make even the most war-hardened general surgeon soil his scrubs (or at least get really, really nervous).  In case you've never heard of the scourge which is Crohn's disease, it is an autoimmune disorder in which the body attacks its own digestive tract - any portion of it, from the mouth to the anus and anything in between.  This causes severe inflammation, resulting in chronic (often debilitating) abdominal pain, malnutrition, diarrhoea, and ano-rectal abscesses and fistulae (if you don't know what those are, I assure you they are exactly as unpleasant as they sound.  Another link you probably shouldn't click).  It can also cause life-threatening complications including bleeding, obstructions, and bowel perforations.

Sounds like tremendous fun, right?

WARNING: TERRIBLE SEGUE AHEAD

An experience with my bank teller (I warned you) got me thinking a few days ago.  I was trying to make a simple transaction, no talking necessary, but Mr. Chattypants was having none of that antisocial nonsense.  Somehow he managed to get me to divulge that I'm a surgeon, and he immediately asked the second question that pops into nearly everyone's head.  The first question, of course, is "What's this thing on my leg/face/arm/ass?"  I guess he decided to skip that one out of some sense of dignity and/or social grace, so he went directly to the second question which, regardless of the social situation, is always "What was your most difficult case?"  I've had several readers ask me the same question, so . . .

If you've put 2 and 2 together, you can see where this is going.

While knee-deep in my surgical training, I had the opportunity to treat Xavier (not his real name), a 50-ish year old gentleman who had been diagnosed with Crohn's disease about 30 years prior.  Since that day (which he eloquently described as "the worst fucking day of my miserable life"), he had been on every medication that researchers much smarter than I had been clever enough to invent, though none had provided any real (or long-lasting) improvement.  His chronic abdominal pain and rectal bleeding finally came to a head about 10 years before when he finally underwent an ileoc√¶cectomy (a surgical procedure to remove the end of the small intestine and beginning of the colon and reconnect the two ends), which looks something like this:
Surgeons know to avoid operating on Crohn's patients unless it is an absolute necessity, and in Xavier's case, it was.  His disease was isolated (at least back then) to the terminal ileum and caecum (the end of the small intestine and beginning of the large intestine), but this area had become so inflamed that it had ruptured.  

That was only the beginning of Xavier's problems.

Unfortunately there is no cure for Crohn's disease, and performing this life-saving surgery to remove a perforated segment of intestine did nothing to improve his overall process.  In Xavier's case it saved his life (for the time being) but opened him up to a whole spectrum of new problems.  Literally.  Over the course of the next several years, he was treated with multiple medicines to modulate his immune system, but despite the attempts to prevent further complications, he had further complications.

He had increasingly bad flares increasingly more often, and he eventually wound up with an entero-cutaneous fistula, an abnormal connection between the bowel and the skin, and bowel contents leak out.  In an attempt to prevent even more problems (read: death), the fistula was initially treated without surgery.  Unfortunately with each flare it seemed a new fistula popped up.  This non-operative approach predictably failed, and several years and several surgeries later, he was left with an abdomen that looked something like this:
Not Xavier's abdomen
By the time I met him he had an end jejunostomy, several entero-cutaneous fistulae contained within a blind-ended segment of ileum, and a mucus fistula at the proximal end of his remaining descending colon.  If that makes no sense to you, it made only slightly more to me.  Basically his intestine was a jigsaw puzzle with several missing pieces, several misshapen pieces from multiple other puzzles, and three Monopoly tokens, and the three of hearts.  Fortunately it made complete sense to Dr. Anderson (not his real name™), the surgeon responsible for putting Humpty Dumpty back together again.  The goal, he told Xavier, was to take everything apart and put everything back together again.  This was an extraordinarily risky surgery, fraught with potential dangers - leaks, more fistulae, and death.  Despite the risks, Xavier wanted all of his fistulae gone, and he pleaded with us not to create any new ostomies if at all possible.

"Risky" didn't even begin to describe it.

At precisely 7:45 AM, Xavier was put under anesthesia.  Dr. Anderson went over the game plan with me, took a deep breath, and we began.  It took us nearly an hour just to incise the skin from around the intestine.  As the case proceeded, we had to separate the bowel not only from the abdominal wall, but also from itself.  Years of chronic inflammation had caused everything to mat together into a giant clump of solid collagen.  We meticulously dissected his tissue, trying to cause as little trauma as possible, and failing often.  By noon we had finally gotten past the abdominal wall and into the abdominal cavity, and by 5PM we had delineated most of his internal anatomy.

That was the easy part.

The segment of small intestine containing the multiple fistulae had to be removed and the two ends reconnected, leaving a blind-ended segment.  One end of that segment then had to be connected to the small intestine, the other end to the large intestine.  In all we made three new anastomoses (connections between the bowel), any of which had the potential to leak or create new fistulae.  Dr. Anderson and I finished the case just before 10 PM, having taken one restroom break each, and no breaks to eat.

And we still hadn't cured his Crohn's disease.

We had told Xavier prior to surgery that, if he survived, he would have an extremely long recovery in the hospital, and an even longer one at home.  Unfortunately to make a long story short (too late), Xavier did not survive his surgery.  It's difficult to say what actually killed him, but the simplest explanation is that his chronically weakened system wasn't strong enough to endure a 14-hour surgery.  Xavier went into the procedure a broken and defeated man, unable and unwilling to endure his debilitating disease any further.  In his mind, death was preferable to continuing on as he was.  On the night he died, the last thing he told me before I went home for the evening was, "No regrets, Doc."

Which brings me back to my list.  After writing this story and thinking of what Xavier went through, Crohn's disease just made it back on.

NOTE: For more information on Crohn's disease, go to the Crohn's And Colitis Foundation of America website or the Mayo Clinic website.

22 comments:

  1. how about any disorder where the body turns on itself.

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    1. While it hasn't quite gotten to the "kill me now" phase, this suspected mast cell activation syndrome is the most challenging thing I've ever known. It's worse than the Fibromyalgia (although could be linked). The fact that i react to random things at random times and sometimes my body just reacts to itself is beyond frustrating. I can't avoid triggers that i don't know like people with allergies can.
      I am procrastinating on an official diagnosis because there's no test for MCAS -when the mast cells are activated by everything, only tests for mastocytosis where there are too many mast cells.
      It's a tricky place to be when you feel your body is betraying you and anything you are around or ingest will cause a reaction.

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    2. I mentioned elsewhere I once rolled on a patient with idiosyncratic anaphalaxis.

      that falls into the category of "and I thought I had problems." the basic explanation is that you are deathly allergic to things with no rhyme or reason. - anything the patient ingestes has the potential to make the patient stop breathing.

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  2. These things are one of the reasons I thank God that there are people in this world with a passion for medicine.

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  3. I had no Idea Crohn's was so...serious isn't the word, I kind of knew it was a nasty thing to have, but I never realised the extent of it.

    I definitely see why it's on your list.

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    1. Knowing someone with Crohn's I can assure you it is that serious. This person has endured multiple surgeries just like Doc's patient. They never know when a new bowel obstruction will send them back to the hospital.

      This is definitely on my list of diseases I would never want to have.

      Kudos to Xavier for enduring it as long as he did. RIP sir. If it were me I'd be saying just kill me now after the first fistula.

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    2. I'm not sure if you really meant the "kill me now" part of your comment, but that was one of the first things I thought of as I read the post. Isn't it amazing what different people are willing to tolerate to stay alive?

      I'm a quality of life kind of gal, and count myself lucky to have only suffered bad, chronic back/hip/leg pain for quite a few years, which has rectified itself with anti-anxiety medication, and one bout of breast cancer 10 years ago. However, I don't suffer suffering gladly (it makes sense if you read it a couple times), and am at the point where I have a DNR in place for any event where my heart stops or I'm not breathing.

      And yes, I also admire people who can endure chronic, debilitating pain and still want to live, even though I really don't understand how they do it.

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  4. I knew Crohn's disease was bad, but I had no idea. It makes me very sad for a young father I know who is battling the disease.

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  5. I don't think the pictures are THAT bad, even after googling them to see more. A bit cringeworthy and I'd hate to be in the situation myself but other than that they're fascinating to look at.

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  6. My dad has Crohn's and incidentally, I just did a research presentation on Crohn's for a genetics class. (Crohn's is definitely genetic, but it has a polygenic inheritance and....it's complicated. Even the NIH doesn't know.) My dad is very lucky in that he has a mild form, but even for him it sucks. He has bizarre eating habits which are somewhat controlled by medical marijuana and constantly fights weight loss.

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  7. Does anyone know someone with Crohn's who's tried the "biologic" medication? For the past few years, I've seen TV ads probably a dozen times a day for Humira (adalimubab) being touted as a treatment for everything from Crohn's to arthritis to plaque psoriasis to various other skin conditions. I also understand that using anything that suppresses these undesired immune system responses also exposes the user to a greater risk of *infections* that the immune system might ordinarily handle for us, so of course both patient and doctor would need to consider both the benefits and potential risks, given a particular patient's situation. Just wondering if anyone knew someone with Crohn's who had tried Humira and gotten some significant relief without also suffering some nasty side effects.

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    1. My partner's brother uses Humira for his crohn's and he is doing quite well at the moment, but I know he takes a lot of medications so I couldn't say whether it was specifically Humira which is helping. He also has Methotrexate injections which are fairly intense. He's terrified of surgery and I can see why, reading this article.

      Thanks Doc for the picture links! Awesome! In a horrific can't look away kind of way.

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  8. hello doc, im one of your readers since the famous coke bottle xray

    I actualy have crohn's since im 16 (im now 30) and i manage to live a pretty normal life with medication and a good diet. I guess if you can get over the chronic abdominal pain and spending alot of time on the toilet (thats where i read your blog most of the time) it's not that bad. My main problem is with the medication who give ALOT of side effect (fatigue and suppressed immune system). If someone got the flu around me im pretty sure to get it.

    I had surgery when i was 17 and got 60cm of my small intestine remove, as of this day the area of the operation still have some pain if i press on it.

    Yes it is a ''shitty'' disease but it has force me to take care of myself, eat healthy and do exercise to stay in good health. I guess the good outweight the bad in the end

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  9. On the night he died, the last thing he told me before I went home for the evening was, "No regrets, Doc."

    His last few years sound like a living hell and it doesn't sound as though he was lying to you that day. I'm sorry that he couldn't enjoy a nice long stretch of time without pain, particularly near the end. You're subject to the Call Gods, he was being batted around by all of them for sport.

    Wednesday

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  11. Thank you for this well thought blog entry, I have crohns disease myself and my 4 year old son has just been diagnosed with Ulcerative colitis I can assure you it is hell. The balance between medication to keep me in remission and my liver not signing out for the count is a fine art.

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  12. Thank you for this well thought blog entry, I have crohns disease myself and my 4 year old son has just been diagnosed with Ulcerative colitis I can assure you it is hell. The balance between medication to keep me in remission and my liver not signing out for the count is a fine art.

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  13. Good thing you provided those links so I didn't have to google it. ;)

    To echo other posters, I knew Crohn's was bad, I didn't realize it was this hellish.

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  14. Yesterday, we heard on the news of the passing of musician Glenn Frey, one of the founding members of the Eagles. It was said that he had suffered from a number of health issues in recent years, including rheumatoid arthritis, and that his death was due to complications of ulcerative colitis and pneumonia.

    On this morning's news, I heard some additional information. It seems that Glenn's family is considering taking a legal action against the manufacturer of the medication that he'd started to use fairly recently to treat the rheumatoid arthritis,which had originally been diagnosed about 15 years ago. Apparently they have reason to believe that this drug may have *caused* the ulcerative colitis and pneumonia. The family is not publicly releasing the name of the medication in question, pending their legal filing.

    I'm thinking that this medication, whatever it is, has to be one of these newer "biologics" that treats the targeted condition by blocking some aspects of the body's immune system responses. Apparently, Glenn didn't develop the ulcerative colitis *until* he began this new type of medication. And we all know that pneumonia, which is usually easily treatable if promptly diagnosed in an otherwise-healthy person, often ends up to be the primary factor in causing death in a person whose immune system is compromised and won't respond to the antibiotics.

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  15. I know I am totally late to the party, but i enjoy your blog as relatable comic relief. I love this post as I'm pretty sure every physician has their own list of "least favourite diagnoses," varying slightly based on subspecialty and experience.. Penile cancer isn't on my list (my pts are generally too young) but my current frontrunner is DIPG (Diffuse Intrinsic Pontine Glioma). It is immeasureably frustrating when people refer to certain cancers as "good" cancers, but in the world of childhood cancer this is "the worst of the worst." Google is fairly forgiving; no disgusting photos from ORs (these kids dont make it into the OR unless their families are selfless enough to donate their tumor postmortem), but an oncologic consultation is not: this disease strikes kids in their first decade of life, has only harsh palliative treatment options, leaves cognition intact but destroys the brainstem and kills within a year, BAR NONE. Treatment recommendation? Go home and make memories, you might have five days with them, but at most 12mos. You will experience emotional and economic devastation going to (literally) the ends of the earth trying in vain to save your baby. I know there are many adult diagnoses which are terminal, so I'll admit to bias, but this one just strikes at the very core of my being (the doctor one and the human one, though not mutually exclusive).

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  16. The condition John Merrick had was called Neurofibromatosis, learned that in English class of all places, never thought I'd remember

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    1. Joseph "John" Merrick's official diagnosis is, and will likely remain, speculative, though Neurofibromatosis and/or a condition known as Proteus Syndrome are currently thought to be the most likely cause of his deformities. I would place my bet on Proteus Syndrome based on the fact that his tumors were bony in nature as well as my limited knowledge other patients with both illnesses.

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