Surgery in the time of THE ELEPHANT MAN

What kind of a cinematologist would I be if I didn't reference the surgery scene at the top of The Elephant Man? A mediocre one, I'd say.

As it happens, just for fun, I've been reading The Greatest Benefit to Mankind, a history of medicine by the late (great!) Roy Porter. The Greatest... can get a little encyclopedic, but Porter's other book, Blood & Guts: A short history of medicine has a lot of entertainment value, especially the chapter in which Porter describes catheterizing a penis with mercury as a cure for syphilis. Off topic I know, but the book is fascinating.

Where was I? Oh yes... When Fredrick Treves met J. Merrick in 1884, Treves was a surgeon at London Hospital. At the beginning of the film we see him (Anthony Hopkins) performing surgery on a man who has apparently been in an "industrial accident." In graphic black and white we see the man's open bloody abdomen rising and falling with his breath as the hospital staff work to save his life. The surgeons wear smocks, with cuffs protecting their shirtsleeves. They are bare-handed, they aren't wearing masks, and their breath and their hair can go anywhere, including into the patient's abdomen. Come to think of it, the patient's own hair from his chest or abdomen can get in there too. Brrr! If the man doesn't bleed to death, he still has a good chance of dying from infection.

A LITTLE ASEPSIS GOES A LONG WAY

By the 1880s, in Glasgow, Joseph Lister (think Listerine) had been working out his theories involving bacteria and aseptic(1) practices, but the idea of doctors being a source of infection themselves (via transmission of microorganisms) was still controversial.

At this point in history, nurses had been trained (by Florence Nightingale and others) to scrub every surface within an inch of its life. But, there was still a popular idea held by doctors and nurses alike, that miasma caused infection and death, not bacteria. Many medical people were willing to believe in bad air, but not in invisible little creatures like bacteria which could be on a seemingly clean surface or even blown around on the breath.

We can hope that in this surgery scene from the movie, some form of antiseptic(2) practice is in place. But at this time, it was common practice to roll up your shirt sleeves and don a surgical smock that was stiff with blood, having been worn by other surgeons previously and not laundered. Facemasks didn't come into vogue until the late 1890s. Rubber gloves were popular in dissection, but didn't get a grip in surgery until the late 80s or early 90s. Antiseptic and aseptic practices were also inconsistent. Lister sprayed carbolic acid all over himself, the patient, and his surgical team to cut down on germs, but he didn't make it a practice to scrub his hands, just dip them in carbolic.

ANESTHESIA

Here we see a shot in the scene with a breathing mask and what we must assume is chloroform, since ether was used in the 1840s for surgery, but replaced by chloroform pretty quickly. Apparently the stuff is safer to use, but I don't know why.

Can you believe that before the 1840s, surgeries (when they were done) were done without anesthesia??? Tumor removal, amputations, gallbladder and bladder stone removal, mastectomies... my various body parts are shrinking at the thought of getting cut into without the benefit of pain killers stronger than gin.

DO YOU SMELL SOMETHING BURNING?

Treves calmly takes a hot poker and cauterizes the patient's arteries, as needed. I thought the smoke in the scene particularly well done. I imagine there would have been a substantial smell to the procedure. I imagine there still is a smell, however these days I believe cautery is performed with an electrical charge or some kind of chemical on a stick, depending on the type of surgery, its location, and the amount of bleeding.

All in all, David Lynch managed to stay true to his own dream state trippy-ness and layer some very interesting medical history into a very poignant story. I highly recommend it.

NEXT TIME

I've had a request from one of my students: he asked me to describe the mechanism by which Spider-Man can squirt web out of his wrists. Young men and their curiosity about sticky palms! As we all know(3) Peter Parker in the comic strip had a mechanical web-squirting device. In the movie Peter is an organic squirter, so... Look out! ... Here comes the Spider-Man!

1. Asepsis means to exclude all microbes. Something that is sterilized has nothing at all living on it. As a colleague of mine often points out to his students, there is no such thing as partial sterilization.

2. Antisepsis means to prevent microbes from multiplying and getting out of hand, but they are still present, sometimes in a weakened condition.

3. Thanks to my cartoonist husband, R. Sikoryak.

THE ELEPHANT MAN part 3- Pain

J. HURT AS J. MERRICK

This will be the last entry about Proteus syndrome and The Elephant Man. Brace yourself: the entry is a little long and complicated because I'm still working through the diagnostic criteria chart from the Leslie Biesecker article from The European Journal of Human Genetics, mentioned in my April 2 post. Yes, I know that some people may be ready to go on to other things, like antiseptic practices in the late 1800s, or a description of how Spiderman could grow web-squirting organs in his palms. To them I say, "All In Good Time." I assume that most of you are willing to go the distance with David Lynch and J. Merrick. So here we go...

WHAT WOULD IT FEEL LIKE TO LIVE WITH PROTEUS SYNDROME?

Merrick's defomities probably threatened his life by crowding and compressing other tissues and structures in his body. It may be possible that his growths invaded other tissues, but that seems unlikely. Cancer is invasive. Benign tumors like fatty lipomas (more on these later) are not invasive, but when these growths and tumors reach a certain size, they can wreak a lot of havoc on the body.

Section of specific criteria for diagnosis of Proteus Syndrome

As we continue to compare David Lynch's J. Merrick to the Specific Criteria(1) for diagnosis of Proteus syndrome, I will also talk about the types of pain that it is likely Merrick was experiencing from his deformities. Because I'm a teacher specializing in pathology for massage therapists and I'm not a doctor (although I watch one on TV)(2), I'll be focussing mainly on pain Merrick may have felt from over-crowding tissues and musculoskeletal complications, although he certainly may have had many other sources of pain and discomfort.

Look at this picture of Merrick’s skeleton.

As we hear in the film, only his left arm and genitals escape deformity. We can see the bony hypertrophy(3) in Merrick’s right femur (thigh bone), upper extremity, and skull. Muscles attach to those bones and rub across them as they shorten and lengthen. Every time a muscle contracts, it presses into the bone beneath it. The pressure and movement generated by the muscle on the bone would be rubbing it "raw" continuously. Man, that's gotta hurt! The degeneration of his joints would make it severely painful to move those limbs and he would have extremely limited range of motion.

J. MERRICK

The deformity in his joints would cause uneven weight distribution, leading to inflammation and a painful arthritis. As if that weren't enough, those skin growths aren't as flexible as regular skin. They would be heavy, and restrict joint movement by not being able to stretch as Merrick lifted his arm or flexed his elbow. Come to think of it, the bulk of those growths could also get compressed as a joint moved, restricting motion. In the film Merrick limps and rarely uses his right arm and hand. When in public, he also covers his right arm and hand entirely, which means the task of make up would be easier.

RUB SOME SALT IN THOSE WOUNDS

In The Elephant Man, Merrick is beaten by the side show manager. Whether this really happened or not, the violence is off screen, but still very disturbing. I study stage combat as a hobby(4) and I appreciate effective violence. I found myself swept up in the scene and although I couldn't see it, I imagined poor Merrick with everything he'd gone through being caned by his manager. Merrick probably had chronic painful muscle spasm and joint pain from arthritis and postural deformity, he's horribly disfiguring by skin and connective tissue growths, and he's beaten! I envisioned that cane pummeling those cerebriform growths, which would have a pretty good blood supply, and the amount of bruising and pain those blows would cause. It makes my neck tense just to think about it.

Since Merrick was beaten in the film, the next logical question is: who would have spent time keeping Merrick's wounds clean if he weren't at the hospital? He couldn't do it himself. Even if those crevices on his back weren't injured, hygiene is extremely important for preventing skin infection and this was a time when hygienic practices weren't that popular.

Crevices in healthy skin are still susceptible to infection. Fungi love dark, warm, creases in the skin and Merrick certainly had a lot of them. Here's a normal armpit with a simple tinea (fungal) infection. ARMPIT

Even if Merrick's skin was intact, regular bathing and clean clothing would be imperative for maintaining skin health. It's not like he could just powder himself with Tinactin whenever there was a problem. Not only do we have pain, but itching with this scenario.

HEADACHE

J. MERRICK

J. HURT AS J. MERRICK

Merrick’s skull and facial deformities could be credited to “hyperostosis of the skull(6),” from Category B, above. The swellings on his forehead could be partially from bony growth but also from Category C's, “Dysregulated adipose tissue.” Adipose is fat and the swellings on the head could very likely be fatty tumors (lipomas).

Other diseases such as osteitis deformans (3), that cause enlargement of the skull often lead to severe headache because of the stress on the connective tissues surrounding the brain and possible pressure on the brain and cerebrospinal fluid. Merrick probably also had strain on his muscles from the weight of the head. This isn’t in the movie, but I would imagine that Merrick would be subject to some raging headaches caused by both his deformity and severe muscle tension.

BACKACHE

MERRICK'S SCOLIOSIS

Look at the curvature of Merrick's vertebral column in the skeleton photo. In the film we can also see this scoliosis(8) in the naked sillouette of Merrick, as Treves presents his case to his peers at the hospital. When we look at the photo of Merrick’s actual skeleton, it’s hard to tell if there is severe bony deformity causing his curvature, or if it’s a functional deformity as his vertebral column tries to compensate for the incredible stresses put on it from other deformed bones, plus the pounds of tumors distributed throughout his body. I would think this kind of condition would cause debilitating back pain.

In the movie, and reported elsewhere, Merrick had to sleep sitting up because the weight of his head was too great. In the film Merrick chooses suicide by sleeping supine as a normal person would(9). Think about the way you felt the last time you slept sitting up, then imagine having to do that every night.

AND HE’S A NICE GUY

Throughout the film, Merrick is a pure soul. He is grateful for Treve’s support, treats everyone with courtesy and kindness, and never complains about physical discomfort. Is that possible in a person? As a massage therapist, I’ve worked with clients in chronic, incurable, debilitating pain who had to live most of their lives tanked on pain killers and hooked up to TENS (transcutaneous electric nerve stimuation) machines(10). Some of them were complainers, and frankly, who could possibly blame them? Some of them were the most pleasant, giving, non-complaining people you’d ever hope to meet.

Could Joseph Merrick, a grossly deformed person, probably in constant chronic pain, living in a world without Tylenol or other effective non-addictive pain-killers really be that type of person? If I had to cope with his incurable condition, I’d probably get super drunk all the time, then eventually go on a killing spree and throw myself out a window.

WHAT COULD I, CLAUDIUS POSSIBLY HAVE TO DO WITH THE ELEPHANT MAN?

Merrick has a speech impediment from his facial deformities. Did he have the “parotid monomorphic adenoma(11)” from Category B, number 3b? John Hurt

does a rather wet and drippy sounding voice for Merrick, which I would normally find admirable. But as I was watching the movie, I kept thinking of Derek Jacobi in I, Claudius. I couldn’t figure out why Hurt would sound so much like Jacobi until my husband pointed out that Hurt played Caligula in the same BBC production from 1978. Hurt's version of Jacobi's version of the Emperor Claudius' speech impediment was a little distracting. If encourage you to watch The Elephant Man then watch a later episode of I, Claudius and you will know what I'm talking about.

HURT AS CALIGULA (left)

JACOBI AS CLAUDIUS (right)

If you made it through this entire post in one sitting, you have some highly developed attention endurance skills! This one got away from me. But that’s it for Merrick and Proteus syndrome. Next time I’ll do a little Elephant Man mop up (ha!) about the surgery scene at the top of the film and antiseptic practices in the days of surgeon Fredrick Treves.

1. See post for April 2.

2. Yes, this is the second time I've used that joke, but it's good to keep reminding people that I'm not a doctor and can't do what they do.

3. Over-growth of bone

4. For great information about stage combat classes go to www.safd.org, or if you're in New York, try Joe Travers and Swordplay workshops.

5. Tinea is a common type fungus that is responsible for athletes foot, ringworm, nail fungal infections, etc.

6. Increased bone growth.

7. Osteitis deformans is a chronic condition usually affecting men in middle/late age in which the bones of the head, trunk, and legs become enlarged. The bone will also weaken as it grows, leading to deformities, pain, arthritis, and fractures. The condition is occasionally called "Tight Hat Disease," because your hat would get tighter as your skull got larger. Another popular name for this condition is Paget's disease, named after Sir James Paget.

8. Scoliosis is a lateral curvature of the spine, often accompanied by a rotation. The condition can vary greatly, and can be caused by lots and lots of things including having a head that weighs three times what it should.

9. I have read two different descriptions of how Merrick's supine position killed him. Wikipedia currently says that he dislocated his neck this way. Other sources say that he crushed his trachea, or if he didn't crush it, compressed it to the point which he couldn't breathe. The film doesn't get into the details here.

10. The TENS machine is an apparatus that applies an electrical current through the skin of a patient. This current helps to control pain. Here's a link to Wikipedia, if you're curious. Be warned that the article still needs some work.

11. A parotid monomorphic adenoma is a type of tumor of a salivary gland.

DIAGNOSING THE ELEPHANT MAN part 2 - Some Specific criteria

Hey folks! I'm having a series of IRRITATING formatting issues, so bear with me while I get some stuff figured out. Thanks! -K

RECAP

When last I blogged, we examined Lynch's depiction of Merrick's fulfillment for the general criteria of Proteus syndrome, which included a mosaic distribution of lesions, sporadic occurrence, and a progressive course the condition. Now, let's take some time to look at Merrick's relationship to the specific criteria for PS.

THERE'S A LOT TO CHOOSE FROM...

Here is a close up of half of the diagnostic chart we used last time.

Now, in The Elephant Man, Lynch has to show us that Merrick claims either the single condition from "Category A" (Cerebriform connective tissue nevus,) or two conditions from "Category B" (Linear epidermal nevus, asymmetric disproportionate overgrowth, specific tumors before the second decade), or three from "Category C" (Disregulated adipose tissue, vascular malformations, lungcysts, facial phenotype.)

How can you do that in a movie? Not very easily. But before we glaze over from the long words, let’s look at Category A - cerebriform connective tissue nevus. If Merrick is qualified through this single category, then Lynch's work is over (ha!)

NEVI, NEVI, AND MORE NEVI

First of all a “nevus” is usually a congenital(1) pigmented skin growth like a mole or a birthmark, or it can be a skin growth with a lot of blood vessels in it, or any one of a bunch of benign skin growths out there. Nevi can also be any size. Moles are nevi. Small nevi are not usually a big deal. Large nevi can be very disfiguring. Some types of nevi can have systemic effects(2), but most do not.

Nevi

In relation to Proteus syndrome, a connective tissue nevus is a skin growth made of hyper-active connective tissue cells (as opposed to skin cells), and “cerebriform” means that it has contours that make it look like the surface of a brain.

Look at a picture of this foot with CCTN,

CCTN

and look at the film version of Merrick’s back. The texture is very close and very brain-like towards the left side of Merrick's back. These make up guys are really good.

Technically, we could stop here with the diagnosis, since Merrick only has to have the single condition in category A. If we look at Category B, we see another type of nevus called a linear epidermal nevus, which (Yes, you guessed it!) are a series of nevi distributed in a linear pattern.

The two conditions from Category B that would be easy to depict on film are the linear epidermal nevus, which Merrick doesn't display, and the asymmetric, disproportional over growth of the limbs and/or head, which he does. Hypertosis of the skull means increase bone growth. We will look at this along with megaspondylodysplasia, next time.

THE TUMOR THING

As for tumor formation from Category B, parotid monomorphic adenoma is a tumor of the parotid gland, which is a salivary gland. Merrick's face is so deformed with bony hypertrophy and connective tissue overgrowth, that it's entirely possible he had a tumor inside his cheek. On the other hand, we can be pretty darn sure he didn't have ovaries, so those tumors are out.

Just to linger on the tumor thing for a bit longer, surgery and medicine was not very advanced in the late 1800s. With everything else that Merrick had going on it was unlikely that internal tumors would be addressed unless they were extreme and/or treatable. Tumors were usually only diagnosed and treated when they were big enough to cause pain and threaten one's life. Antiseptic practices were still controversial and not only did one have to survive potential sepsis post-surgery(3), but you had to survive surgery itself, which wasn't always easy. Although Merrick's "tusk-like" tooth is mentioned in the film, it would be hard to credibly bring up a parotid tumor.

Next time, I'd like to continue to look at some of the criteria in categories B and C for their expression in Merrick's body in the film and in real life, as well as some of the symptoms Merrick might have felt as a result of this tissue growth.

1. Congenital: Disease or condition present at birth or that manifests within the first five years of life. Congenital conditions may be hereditary, caused by disease, birth trauma, poisoning in utero, etc. Some people will also use the term congenital to describe any genetic condition, even if it manifests in adulthood. As far as I know, Congenital Liars have not been recognized as a medically diagnosable group, nor are there studies out there looking for genetic markers that may pre-dispose someone to being a congenital liar.

2. Other organs or systems in the body may be affected.

3. Once in later posts I'll discuss the surgery scene at the opening of the film, and antiseptic practices of the day.

Diagnosing The Elephant Man, part 1 - General criteria

Because Joseph Merrick is called "John" Merrick in Lynch's film(1) I will try to avoid confusion by referring to Mr. Merrick by his first initial and last name.

The Elephant Man

MERRICK'S CONDITION AS A SOURCE OF CONTROVERSY

The Elephant Man tells the story of J. Merrick, a patient of the surgeon Fredrick Treves in the 1880s. Merrick had severe physical congenital deformities and made a livign for a number of years as a sideshow performer billed as The Elephant Man.

According to Wikipedia, Merrick has been diagnosed with a number of different conditions from elephantiasis(2) to neurofibromatosis(3), to Proteus syndrome, and back again. Proteus syndrome is an extremely rare condition characterized by overgrowths of a number of different types of tissues. Sufferers of this condition will experience accelerated growth in skin and connective tissues, including bone (this is useful to us later.) Tumors can also form in the ovaries and/or parotid (salivary) gland. Other fatty tumors may also be present. The resulting deformities can be moderate to severe(4).

The Wiki entry about Merrick and Proteus syndrome states, "During 2003, DNA tests conducted by Dr. Charis Eng on sample of Merrick's hair and bone showed no mutation in the PTEN gene (only present in some Proteus syndrome sufferers.) Hence, there is as of yet, no physical evidence to support the theory that Merrick suffered from Proteus syndrome."

It seems that not everyone agrees as to what Merrick's exact condition was. You'll notice that the entry above does not say that the gene mutation is present in all PS patients. Additionally, presence of a genetic mutation is usually not considered a criteria for a number of pathologies that have genetic markers. While researching Proteus syndrome, I found a 2006 article from the European Journal of Human Genetics(5), setting diagnostic criteria for this extremely rare condition. The PTEN mutation isn't mentioned anywhere and Merrick is named as having the syndrome.

As we launch into this analysis, let's pour a little gas on the fire of amateur medical practice, and see if we can diagnose J. Merrick's Proteus syndrome from Lynch's movie.

DIAGNOSING MERRICK

To diagnose John Hurt's portrayal of J. Merrick in Lynch's film, we will look at photos of Hurt as Merrick:

Hurt as Merrick

and Merrick, himself:

Merrick

We should also use the photo of Merrick's skeleton, since Hurt's skeletal x-rays would be normal. ($5 to anyone who can get me a bona fide x-ray of John Hurt's distal femur to post on this site. Well okay, any x-ray of Hurt would be quite a coup!)

Merrick

And here is a chart of criteria for diagnosis of Proteus syndrome, from The European Journal of Human Genetics article:

Sorry it's so small, folks! You may want to check it out with this link.

Bisecker article

GENERAL CRITERIA

Wow! There's a lot here! Maybe we'll just hit the highlights. Let's see... As he made this film, Lynch had to show us that Merrick's signs(6) followed the general criteria of a mosaic distribution, sporadic occurrence, and progressive course.

A "mosaic pattern" can mean alternating stripes of affected and unaffected cells if we're talking about skin growths, or a mix of mutated and unmutated cells of the same type if we're talking about deeper growths. Either way, the tissue overgrowth is described as patchy, irregular, and distorting, in the article.

If we look at these photos of Hurt playing Merrick, we can definitely see distorting irregular overgrowth. It seems to me that there is indeed a mosaic distribution to those overgrowths.

Merrick in the side show

I'd like to take a moment to compliment Christopher Tucker on his make up design and Wally Schneiderman on his make up application in this film. Do these lesions resemble Merrick's exactly? No, but they're really darn close, and the texture is so... texturey!

Does Merrick show signs of, or claim sporadic flare-ups in the film? No, but that might be too much to ask. Let's face it, talking about disease minutiae can really slow down a plot. So for the sake of pacing, let's pass him on this anyway.

Treves presents Merrick at the hospital

A normal birth with signs developing between 6-18 months of age are common with this disease. Treves tells us that Merrick was a normal infant who began showing signs of his condition around three years of age. This information doesn't fit the usual picture, but patient history is often subjective. Merrick or his parents may have misremembered when he started showing signs of deformity. Or it's possible that no one noticed changes on Merrick's skin until they became very pronounced. However old he was when the overgrowths of his skin began to manifest, his appearance was normal initially, and the condition was clearly progressive from early childhood.

Thus far, through assessment of the film, Merrick has confirmed the general criteria for the disease. Next time we look at the specific criteria.

1. See last blog comments for further details.

2. A chronic condition characterized by pronounced hypertrophy of teh skin and subcutaneous tissues resulting from obstruction of the lymphatic vessels. This condition can result from infection or be congenital.

3. Tumor growths of various sizes on peripheral nerves.

4. Adsen, Esra, Meltem Onder, and Mehmet Ali Gurer. "A Mild form of Proteus Syndrome." Pediatric Dermatology 24.6 (Nov. 2007):660-662.

5. Biesecker, Leslie. "The challenges of Proteus syndrome: diagnosis and management." European Journal of Human Genetics 14.11 (Nov. 2006):1151-1157.

6. Signs are objective data from the patient such as tumor growth, blood pressure, fever, etc. Symptoms are subjective data such as pain, nausea, dizziness, etc.