Destroyer of Worlds

Matthew Guess

Writer’s Comment: I love puzzles. Jigsaw puzzles, crossword puzzles, word puzzles, they all feel wonderful and adventurous and exciting, like some daring voyage through uncharted seas. Answers are not immediately obvious, the big picture remains hidden until the pieces are assembled, and, while clues are present to guide courageous explorers to paradise, the threat remains of stumbling off the correct path and into despair. Perhaps these notions are childish and fanciful, but they still enrapture me with mystery. For this very reason, I also love the collage style in writing. The collage is a mystery waiting to be pieced together by the reader, an argument begging to be substantiated. My hope for this piece is that the reader might be challenged to connect all the pieces and not just those which remain on the surface. In a puzzle, one must venture beyond the assembly of the edge pieces. He must strive to see the big picture.

—Matthew Guess

Instructor’s Comment: At first, “Destroyer of Worlds” may well leave you confused, even frustrated. Even while admiring the grace and fluidity of Matt Guess’s style, you may wonder what could have possessed contest readers to choose such a disorganized paper for publication. But choose it they did, and for very good reason. Matt’s piece is a fine example of a “collage” paper, a form much like the artistic collages many readers will have created at some time in their lives. Through the seemingly random ordering of unrelated material and events, Matt helps us understand the jerky rhythms and what he calls the “tone” of Huntington’s Disease. He does, then, what I hope all students will do in their collage papers: he uses the form as well as the content to help us see the connections and disconnections inherent in his subject, and in the broader world. After you’ve finished the collage and Matt’s insightful reflection on it, I hope you will agree that at center of the apparent chaos lies a deft illumination of what Huntington’s is and what it does.

—Marlene Clarke, University Writing Program

 

In the distance, a record hurls whining notes into the inanimate night.

“I got this thing called chorea in my head

wanna walk but I fall down instead

folks say “Woody, he’s just drunk again”

but I haven’t had a drink since I don’t know when

besides . . . I only drink when I’m alone . . . or with somebody”

The first breakthrough, spilling from the laboratory of Sir John Cockcroft and Ernest Walton in 1932, electrified the scientific community. For many years researchers had known of the incomprehensible amounts of energy residing in the cohesive forces of a nucleus. If the atom could be split, they theorized, its energy could be harvested for the good of mankind. Finally physicists had succeeded, the atom had been split, and many more labs around the world labored to perfect the science of nuclear physics. Leó Szilárd, a Hungarian-born physicist, proposed that if any nuclear process emitted more neutrons from the nucleus of an atom than were used to start the fission, a nuclear chain reaction might result, leading to an exponential increase in energy output. However, Szilárd also emphatically cautioned that, if such a reaction existed, it could be used as the catalyst in a new generation of weapons, atomic bombs.

Those last months were difficult for Sara. Though she wouldn’t dare let on, in fact, they were agony. Every free moment, Sara sat at her mother’s bedside in silence, watching helplessly as the disease sculpted its final cruel masterpiece. It had been 20 years since her mother had first been diagnosed, yet Sara still remembered exactly the way that day had progressed, from the hot pastrami sandwich she had devoured at lunch, to the uncontrollable deluge of tears that quieted her to sleep. How could she forget a day such as that?

Huntington’s disease, a degenerative neurological affliction, is caused by a mutation in the Huntingtin gene. This gene, which encodes for the protein HTT, contains a small repeated DNA sequence called a microsatellite. Three DNA bases constitute one of these microsatellites: Cytosine, Adenine, and Guanine, or CAG.

(-CAGCAGCAGCAGCAGCAG-)

Although every person carries microsatellites in the Huntingtin gene, the number of repeats dictates whether a person will be afflicted. Normal individuals have fewer than 27 -CAG- repeats, while affected individuals typically carry 35 or more.

Sara’s mother, Jane, had always been her daughter’s most dedicated advocate. Jane attended every one of her daughter’s Girl Scout meetings, dance recitals and piano lessons. When Sara was in junior high, Jane always volunteered to chaperone school dances, an idea that mortified her daughter. Despite Sara’s objections at the time, she felt safe knowing her mother was never too far from her.

Shortly after making his proposal, Szilárd discovered that the uranium fission reaction proceeded by a mechanism similar to the one he had warned about, a chain reaction. A single atom of uranium needed only one neutron to be split, but generated three free neutrons once the reaction occurred. “We turned the switch, saw the flashes, watched for ten minutes, then switched everything off and went home,” Szilárd reminisced. ”That night I knew the world was headed for sorrow.”

For want of a nail, the shoe was lost.

My arms felt funny moving all the time

and sometimes my head didn’t feel like mine

kept telling myself it was the Ballantine Ale

and them jugs of wine on the writing trail

I prefer a disease you can sober up from.

They needed to stick together. After Sara’s father died, Jane had made sure Sara never saw her cry. It was only the two of them, and they were undeniably close. Watching her mother’s jerky, repetitive movements, Sara remembered back to the times when the two of them engaged in food fights in the kitchen, each person diving as banana projectiles whizzed overhead, rolling in laughter as they hurled handfuls of chocolate chips. By the end of the battle, the kitchen was a landscape of war and the two rivals sat exhausted on the slick, chocolate-covered floor, completely content. Those food wars became less frequent, however, after Huntington’s Disease seized Jane’s body.

In a nuclear fission reaction, neutrons bombard an atom, in this case Uranium-235. When the nucleus of Uranium absorbs one of these neutrons, the atom momentarily becomes Uranium-236, an extremely unstable isotope. This instability causes the atom to break apart into two new atoms, Barium-141 and Krypton-92, and three free neutrons. These free neutrons are then able to bombard other Uranium-235 nuclei. If each neutron hits a target, those three nuclei will also split, each generating three more neutrons to add to the reaction. As this process continues, the number of neutrons increases exponentially. The process is, at first, quiet and unnoticeable, but it leads inexorably to enormous energy production.

A doctor, George Huntington . . . he gave it the name

and all these years later it’s still the same

no cure but the patience of the ones you love

and the busy schedule of the Lord above

you can usually count on him . . . but he’s mighty slow

For want of a shoe; the horse was lost.

The symptoms appeared trivial at first, a slight twitch in the elbow, a slurring of speech, the repetitive movement of an arm or leg. Slowly and painfully, the disease took over. Jane was a drowning swimmer, sliding effortlessly into the depths, surrendering to the crushing force that pulled her deeper. With time, she could barely control her movements. Instead of lovingly stroking her daughter’s smooth, amber hair, Jane’s once delicate hands became rigid, twisting into unnatural shapes under her brain’s incoherent direction. Her legs and arms seized rhythmically, jerking, flailing and slapping the air. The muscles on the left side of her face tightened in spasm, forcing large, clear droplets of saliva to slide mockingly from the right corner of her mouth. Only ten years after being diagnosed, Jane became confined to a wheelchair, her pale, withered legs shifting only at the direction of the disease’s pirated signal and, even then, only for a sudden jerk.

In August 1939, in response to fascist Germany’s invasion of neighboring Poland, Leo Szilárd drafted a letter to President Franklin D. Roosevelt. He convinced his friend and colleague Albert Einstein to sign the letter as well, knowing that Einstein’s name would lend credibility to the warnings contained within. The Einstein–Szilárd letter, as it was later termed, found its way to President Roosevelt in October 1939. In the letter Szilárd explained the inevitable outcome of the new research: nuclear weapons. He urged the President to develop this new technology before Nazi Germany had any chance.

When DNA from the Huntingtin gene is transcribed in the cell’s nucleus, the microsatellite faithfully remains coded in the messenger RNA sequence. The messenger RNA is then sent out of the nucleus into the cytoplasm of the cell where ribosomes translate it into an amino acid chain, a protein. Unfortunately, a ribosome cannot distinguish between RNA with the correct number of microsatellite repeats and mutated RNA with aberrant numbers. Therefore, the cell is unaware that it makes a dangerous product.

The Manhattan Engineering District, or the Manhattan project, was one of the government’s largest undertakings, employing over 130,000 people, including the world’s top scientists, and costing a total of two billion dollars. The weapon development took place at laboratories throughout the country, the researchers at each site being assigned specific roles. Los Alamos, New Mexico, considered by most to be the intellectual headquarters of the entire operation, became the site of final bomb assembly and testing. Richland, Washington, produced the plutonium used in “Fat Man,” the atomic bomb eventually dropped on Nagasaki, Japan. Oak Ridge, Tennessee, served as the main site of Uranium-235 extraction, Uranium serving as the payload of the Hiroshima bomb, “Little Boy.” During the development process, the Oak Ridge site consumed 1/6 of the country’s electrical power, and yet few knew of its existence. Even the Tennessee governor was unaware of his state’s newest city.

For want of a horse, the rider was lost.

Although the exact consequence of a mutation in the HTT protein is poorly understood, the overall effects of Huntington’s disease are well established. The disease first manifests itself as small changes in the cognitive and physical abilities of the afflicted person. Patients often complain of problems with coordination, speaking and thinking, as well as anxiety, depression, suicidal thoughts and compulsive behavior. Small repetitive movements, called chorea, also present. Due to their infrequent manifestation, the symptoms of Huntington’s disease, although serious, are often overlooked at first.

On August 6, 1945, at 8:15 a.m., the world became suddenly aware of the work of the Manhattan project when “Little Boy” exploded over Hiroshima, instantly killing 80,000 people. Three days later, on August 9th, “Fat Man” fell on Nagasaki, killing almost 74,000 people.

For want of a rider, the battle was lost.

As the body degenerates, a more noticeable pattern of dysfunction appears. Muscles become rigid and move repetitively, severely altering motor control, facial expressions, and verbal ability. Dysfunction in memory, abstract thinking, moral judgment and planning all contribute to overall dementia-like symptoms. The degeneration of Huntington’s disease, typically occurring over a period of decades, leads inevitably to complete destruction of the patient’s physical and mental capacities.

The Manhattan project’s leader, J. Robert Oppenheimer, later said:

We knew the world would not be the same. A few people laughed, a few people cried, most people were silent. I remembered the line from the Hindu scripture, the Bhagavad-Gita. Vishnu is trying to persuade the Prince that he should do his duty and to impress him takes on his multi-armed form and says, “Now, I am become Death, the destroyer of worlds.” I suppose we all thought that one way or another.

For Sara, the shape in the hospital bed was not Jane. That could not possibly be her mother, the same woman who had nurtured her, the same fortress of comfort. That body was a pillar of stone, some ancient beautiful arabesque straining outward with mineral eyes, breaking the granite only to swing out in repetitive jerks. Sara could barely relate to this mass. Perhaps her mother was somewhere still behind the façade, looking out on a world that ignored her. Or, perhaps she had already left.

The last few months were difficult for Sara, yet seeing that body waste into death presented a welcome relief. Her mother had suffered greatly throughout the previous months and years and she felt grateful that rest had finally come. She thought of Hamlet:

To die, to sleep, —

No more; and by a sleep to say we end

The heart-ache, and the thousand natural shocks

That flesh is heir to, — ‘tis a consummation

Devoutly to be wish’d. To die, to sleep; —

The most difficult reality of Huntington’s disease occurs as a result of the disease’s late onset. Huntington’s typically presents between the ages of 35 and 44 years old, after many sufferers have already begun families. Unfortunately, the disease passes to offspring as a genetically carried dominant trait, meaning fifty percent of the children of affected individuals will also have Huntington’s disease. This represents a death sentence because, as of yet, Huntington’s disease has no cure.

The beginning of her mother’s rest represented something altogether more terrifying for Sara. In time, her mother’s rest would become her own. She, herself, would one day become that pillar of stone because, just like her mother, she had Huntington’s.

In the years following the attacks on Hiroshima and Nagasaki, hundreds of thousands of people died due to nuclear fall-out and injuries sustained during the blasts. Leo Szilárd’s epiphany contained all the truth of that time; beginning with only a few neutrons exploding from an atom, the world came to recognize a truly bitter sorrow.

For want of a battle, the kingdom was lost!

If you can’t remember how I died remember how I lived

and if you can find it in your heart to forgive

know that the piece of brain that had to fall

never affected my love for you at all

I’m gonna play this thi . . .

The record skips

I’m gonna play this thi . . .

Again.

I’m gonna play this thi . . .

I’m gonna play this thi . . .

I’m gonna play this thi . . .

I’m gonna play this thing ‘till they find a cure . . .

Part 2: Examining “Destroyer of Worlds”

John Muir, the famous conservationist, once remarked, “When we try to pick out anything by itself, we find it hitched to everything else in the universe.” The simple beauty of the collage style is just this: every idea, every event has a thread of similarity with some other idea or event in the universe. All things are connected. Moreover, these ideas whisper back and forth to each other, each serving as a thesis to the other’s argument. Therefore, as I constructed my collage about Huntington’s disease, I hoped that the reader would dig in, shoveling through the layered ideas set before her. I hoped that she would eventually put down the paper understanding more of the tone of Huntington’s. To accomplish this I explored three main ideas in this collage: repetition, degeneration, and ignorance.

For a physician, the most noticeable symptoms presented by a patient with HD are the repetitive movements called chorea. Therefore, to capture the needed feel of the disease, I determined that much of the piece should also be repetitive. The short poem regarding the fall of a kingdom uses a repetitive style to illustrate the idea of the butterfly effect. While playing a song, a record player malfunctions, repeating a line of the lyrics several times before finally correcting itself. One can find repetition in a nuclear chain reaction, in the DNA sequence of the Huntingtin gene and in the labored movements of a dying mother. All these coalesce into one scattered description of chorea.

To illustrate in greater depth the disease’s degenerative processes, I juxtaposed the narrative thread with threads about the creation of the Manhattan Project. Like Jane’s heartbreakingly slow breakdown, a chain reaction is also degenerative. Nuclei break apart, rocketing out neutrons that break even more nuclei. Building momentum, the entire system snowballs out of control. Moreover, the whole history of the Manhattan Project describes deterioration, starting from small contained discoveries, building through fear of attack by the Germans, and finally exploding in a mushroom cloud above Hiroshima. Perhaps the reader should be asking the same questions about each thread. What happened to the control systems needed to keep such catastrophes in check? Indeed, in terms of Huntington’s, science continues to explore this question.

Finally, ignorance also plays a common note throughout the piece. Unfortunately people with Huntington’s often pass their dominant, affected allele to offspring before the late onset disease presents itself. Therefore, what I hoped to capture was the confusion, despair and guilt associated with this ignorant exchange. No doubt ignorant of the disease, Jane passed Huntington’s to her daughter. If she had known, surely things would have been different. What might history look like had the world been privy to the work of the Manhattan project? And what about the ribosome that innocently translates a mutated DNA sequence into a flawed protein product? Perhaps knowledge of its mistake could change the final outcome. The reader must exclaim, “if only it (he, she, we) would had known!” Such becomes the cost of ignorance.

While these connections are clear throughout the paper, they represent only the first layer of complexity. Many more associations lie directly beneath these, begging to be woven together with the rest of the story. That is the beauty of a collage; the writer need not explicitly state a thesis. He must only present seemingly disjointed ideas and rely on human creativity to sew together a statement of truth. As the reader changes, so also do the arguments. As the reader changes, the collage captures loftier observations.

 

List of Crots

1.    A record player plays the song “Talkin’ Woody Guthrie Huntington’s Chorea Blues” by Tom Flannery. Guthrie was a singer-songwriter who died of Huntington’s disease in 1967. At the end of the song, the record begins to skip.

Flannery, Tom. “Talkin’ Woody Guthrie Huntington’s Chorea Blues.” 2004. Lyrics accessed May 31, 2009. <http://www.songaweek.com/woody/songs/huntington.html>.

2.    The development of the atomic bomb during the 30s and 40s.

“Leó Szilárd.” Wikipedia: the Free Encyclopedia. May 28, 2009. May 29, 2009. <http://en.wikipedia.org/wiki/Szilard>.

“Manhattan Project.” Wikipedia: the Free Encyclopedia. May 28, 2009. May 28, 2009. <http://en.wikipedia.org/wiki/The_Manhattan_Project>.

Oppenheimer, J. Robert. Television interview. 1965. Retrieved May 28, 2009. <http://www.atomicarchive.com/Movies/Movie8.shtml>

3.    A fictional story of a woman whose mother is dying of Huntington’s.

4.    A short poem about the Butterfly Effect.

Gleick, James. Chaos: Making a New Science. New York: Penguin Books, 1987.

5.    A scientific explanation of Huntington’s disease.

“Huntington’s Disease.” Wikipedia: The Free Encyclopedia. May 27, 2009. May 29, 2009. <http://en.wikipedia.org/wiki/Huntington%27s_Disease>.