Wednesday, April 22, 2009
Saturday, April 18, 2009
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.
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.
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.
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.
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 Hurtdoes 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
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.
Sunday, April 12, 2009
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.
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,
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.