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Even after death, a part of her fights on in battle against Lou Gehrig's disease

LOS ANGELES

If Elizabeth Uyehara were alive, she would be pleased. Everything was working out as she had hoped. The terrible banality of her illness had ended, and she was about to make her final journey. • Two hours before she died, David Jones' pager went off. He canceled his lunch and made a few phone calls to sort out the details with the hospital and the family. He went to his lab and picked up the paperwork and, before heading over the Sepulveda Pass, stopped at a 7-Eleven for some ice that he tossed into a cooler.

He parked in the back of the mortuary in Northridge and let himself into the prep room. Her body lay on the porcelain table. He walked over to the cabinet where he kept his instruments, pulled on two pairs of size 8 1/2 surgical gloves and zipped open the white bag.

He still remembers seeing her there. She was 75 years old, 5 feet 5, a little less than 111 pounds, brown hair, brown eyes. He read her toe tag and wrist bracelet and double-checked her name and date of birth.

Schooled in mortuary science, Jones had been performing autopsies and harvesting organs for 30 years; by his count more than 11,000 bodies. Whether by habit or instinct — he wasn't sure — he always paused at this moment.

Here was someone's loved one, he thought; you were brought to me for a reason: that someday we might help someone else.

Uyehara would have returned the sentiment.

• • •

She had always been sensitive about her health. Nothing was too extreme to worry about, so four years ago, when she found herself slurring her words and her vision suddenly blurry, she made an appointment with a neurologist.

It could be a number of things, he suggested: cancer, stroke, progressive bulbar palsy, neuromuscular dystrophy, even Lou Gehrig's disease. She listened, dumbstruck. He's measuring me for a coffin, she thought, and she went looking for a second, then a third opinion.

She was tested and rediagnosed. She was told she had Stephen Hawking's disease. She was told she had Dudley Moore's disease. One doctor admitted he didn't know what she had. At least he was honest, she thought.

Nearly a year and a half went by. Someone had mentioned myasthenia gravis. Its description — slurred speech, blurred vision, fatigue — matched her symptoms. She felt encouraged; typically it wasn't fatal. She made an appointment for another test.

This time a needle electrode was inserted an inch into her muscles, and as she tensed and relaxed, a machine recorded the activity. When it was over, the news wasn't good.

A relaxed muscle results in a reading close to zero and is almost silent; in her case, the reading was 3-plus and accompanied by a cacophonous and irregular sound, an indication that the nerves leading to the muscles were beginning to fail. When she flexed, the results were more dramatic.

She found herself in the company of yet another celebrity.

"Lou Gehrig's disease," she heard the neurologist say. Amyotrophic lateral sclerosis, ALS.

She started to cry. It would take a few weeks to find a comeback: "Does this mean I can finally hit a major league fastball?"

• • •

Beneath the fluorescent lights in the windowless room, Jones pulled on shoe covers, a gown and a face shield and laid out his instruments. He cued his CD player to the gospel strains of the Edwin Hawkins Singers and placed a small rubber block beneath her head. If he felt any emotions, he kept them in check.

With a Stryker saw, he cut a circle around her cranium and removed the cap. He used a syringe to extract the spinal fluid between the hemispheres, and afterward he cradled her head with one hand, reached around the brain and with a scalpel freed it from the stem. He lifted out both hemispheres and placed them in a plastic bag and put the bag on ice.

He opened her chest with a deep Y-shaped incision and sawed through her ribs. He withdrew about 60 milliliters of blood from the inferior vena cava and then removed her heart. He excised her liver, spleen, pancreas, lungs, kidneys, adrenal and thyroid glands, uterus, bladder and large and small bowels, and cut samples from each.

The spine was now exposed, and he sawed through the vertebrae and freed the cord, a half-inch-diameter bundle of nerves, roughly 2 feet long, soft and white. He coiled it into a round plastic container.

By late afternoon, he began positioning the organs back inside her, and with nylon thread and a baseball stitch, he sewed her up. He didn't know whether she would be cremated or whether there would be a viewing, but that didn't matter. He always made sure his work was as inconspicuous as possible.

With water, soap and a soft towel, he washed her off and pulled the body bag around her.

• • •

She feared ALS but never considered it a possibility. It wasn't her style, so dramatic: no treatment, no remission, just muscles shutting down to the point of suffocation.

She was certain her death would be less flashy — a heart attack or a pile-up on the freeway — and until then, she'd live at home in Reseda, Calif., painting and taking care of her cats and husband, Paul. He was nine years older and showing signs of Parkinson's. They never had children and managed on their Social Security and a pension from her years working in the marketing department at the Los Angeles Times.

But when she Googled "ALS bulbar onset," she learned that patients with her form of the disease typically live 18 months after the first symptoms emerge. She did the math. Her expiration date had come and gone, she thought, which could mean only one thing: She was never going to know how Lost ended.

She started seeing a doctor at UCLA, Martina Wiedau-Pazos, and arrived to each appointment with typed notes and questions.

ALS was unlike anything she had experienced. The more moderate symptoms and sensations — the yawning, sneezing, hiccupping — she could handle, but the involuntary fits of laughing and crying were a bit much. Tears were hard to explain to the clerk who simply wanted to know "Paper or plastic?"

One day she put a key in a lock and couldn't understand why it didn't turn. She pulled it out. It was the right key, wasn't it? She tried again. The lock had to be broken. Then it dawned on her: She had lost the strength in her fingers.

The disease became a hundred little battles waged in a losing war. She taught herself a new way to use a paper clip and light a match, and with the help of a speaking device attached to her computer, she could type and it would talk.

Eating was her first major defeat. As her tongue stopped working, she changed her diet to smoothies and oatmeal. Still she choked, and her doctor worried she might aspirate. A feeding tube was prescribed.

She missed her salads but made do, injecting a regular diet of Ensure and protein supplements into her stomach. Life, she explained to a friend, does not have to be lived in the extreme. "You can be fully alive by just being alive in your body."

Chocolate was a clear victory. If she tucked a Hershey's Kiss into her right cheek, lay down on her side and let it melt, she could still savor its sweetness.

• • •

Saturday evening traffic was light as Jones drove into West L.A. He had skipped lunch and now he'd miss dinner, but he needed to finish.

The National Neurologic AIDS Bank is a UCLA facility that archives brain and spinal tissue for researchers studying AIDS-related neurological disorders. Although the bank is funded for the study of AIDS, its collection has come to include tissue from HIV-negative patients and individuals with ALS and brain cancer.

Jones unlocked the door to the lab. He laid the heart and the smaller tissues on the counter and cut them in two, and divided the brain by its hemispheres. He sectioned the spine into thirds — thoracic, lumbar and cervical — and sliced each length into 1-centimeter increments. Half the samples went into formalin preservative, the other half into the freezer. Its frosty air purled into the room as Jones opened the door.

He glanced at the clock, a little past midnight. He went to his computer and sent an e-mail to the lab's staff. Elizabeth Uyehara, he typed, "passed away on 3/6/2010 at 2:27 p.m. The brain was on ice at 6:30 p.m."

• • •

A friend suggested that she start a blog, but she was reluctant. Jeering at ALS was fine if done in private, but she worried that her irreverence might offend those further along with the disease.

Still she knew the value of words, and on June 23, 2008, posted her first entry. "I might as well join the rest of the human race," she wrote, "and start blogging to kill a little time, since time is busily returning the favor."

She also made friends on an ALS forum, venting frustrations, discussing medications and cracking jokes. ("Q: When you're lying in your casket, what do you most want your friends to say? A: Look, he's moving.")

There were times when she felt suicidal. But they passed, and what remained was a cobbled-together spiritualism based on the 12-step program, the Upanishads, Buddhism, yoga, Pink Floyd, Leonard Cohen and Freud. It was enough to sustain her.

"We are not really humans looking for spiritual experiences," she paraphrased a mentor. "We are spiritual beings looking for human experiences."

Now if only those experiences had less to do with trying to find a skilled nursing facility for herself and Paul, paying income taxes and fighting her insurance company for a power wheelchair.

After she had decided to give her body to UCLA, she tacked on one provision in a blog post: that the university, once done "ooo-ing and ahh-ing" over her spine, "donate it to the Democratic Party."

Wiedau-Pazos sees patients for only a few hours each week. Most of her time is spent in her office or the lab on the fourth floor of UCLA's Neuroscience Research Building, where she has been studying ALS since 1995.

About 35,000 people in the country have the disease. Some cases are hereditary; most are sporadic. In each, motor neurons — long thin fibers that run from the brain through the spine to the muscles — degenerate, leaving the muscles to atrophy and the patient paralyzed.

No one knows why. Wiedau-Pazos is studying the possibility that ALS is a disease of the immune system, like multiple sclerosis and lupus. She and other researchers have uncovered evidence — an elevated number of white blood cells in the spinal cords of ALS patients — but they are uncertain whether the proliferation of these cells causes ALS or is the result of ALS.

This spring the doctor began a new round of studies and needed samples from five brains and spinal cords.

• • •

ALS was writing the final chapter to her life. What had once seemed so important — her ambition as a painter, her identity as a wife, an aunt, a cousin and a friend — mattered less as she grew weaker.

"We sit in empty rooms," she wrote her cousin one rainy afternoon in January, "but between us and the walls, there is endless chemical and electrical activity, molecules [and] the air is filled with star dust. . . . There is no emptiness, only endless pulsing energy."

Perhaps in death, released from her body, she would join that energy. She began to look forward to dying. She found a Vedic prayer to be spoken at the end: ". . . let my life now merge with the all-pervading life . . . ashes are my body's end." But it was too long to memorize.

She wondered instead whether her final words would be more like aaaaaeeeeeiiiiiii.

If she did utter a prayer or scream one morning last March, no one heard. She was having difficulty breathing and arrived unconscious at the emergency room. The doctors had intubated her by the time two friends and her niece arrived. The niece went home to get Paul. He shuffled up to her hospital bed.

"I love you," he said.

There was little else to be done, and he was taken home.

After the breathing tube was removed, the niece held her aunt's hand and watched and listened as the heart rate slowed.

"Keep a place in line for me," her niece said, and the room grew silent.

• • •

Wiedau-Pazos opened up the green slide carrier and pulled out eight slides. Each contained a nickel-sized cross-section of the cervical spinal cord, sliced by a microtome, as fine as an eyelash, preserved and embedded in paraffin.

She held up No. 3038 and kept her memories in check. In the lab, there was no past, no personal history, just the present moment and what it might reveal.

After the tissue was properly stained, Wiedau-Pazos placed the slide under the microscope and peered through the eyepiece. The anterior horn of the spinal cord was brilliantly colored. The sliver of tissue looked like the night sky seen through a red filter. The motor neurons were the stars, and a cluster arcing through it, the Milky Way.

Elizabeth Uyehara was now a portrait of proteins, sugars, phosphates and amino acids, and they belonged to the future, a story waiting to be told.

Even after death, a part of her fights on in battle against Lou Gehrig's disease 09/11/10 [Last modified: Friday, September 10, 2010 6:31pm]

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