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In Memoriam

Unexpected Tragedy in a Little Girl’s Expected Death

Front Page

Unexpected Tragedy in a Little Girl’s Expected Death

Photo by Graham Ramsay Ellen Rothman

The church parking lot looked out over the red plain to the still snow-covered mesa be-hind. A tired gray hearse was parked in front, glinting in the morning sun. The thin wheels were covered with a film of red dust, and the gray curtain in the rear window was moth-eaten.

The front doors of the church opened, and a few Navajo boys in pressed jeans with black leather vests and red rose boutonnieres walked out and lined the walkway. It only took a few men to bear the small white coffin out of the church and deposit it in the rear of the hearse. I recognized the mother, the grandmother, and the auntie among the family that spilled out of the church behind the coffin.

Valrena died in our small emergency room on the Navajo Reservation, where my husband Carlos and I work as pediatricians. She had turned 8 at the beginning of the winter. At age 4, when we first met her, she was already debilitated by a progressive neurologic disorder. Her disease was exceedingly rare, caused by iron deposition in the same area of the brainstem affected by Parkinson’s disease. Her limbs were contorted by tense muscle contractures, requiring her to be strapped in her wheelchair at all times. She drooled constantly, and she was unable to speak.

Brighter Moments
While the disease ravaged her body, it was unclear how it affected her mind. Her mother always felt that she was locked into a body that had failed her. I saw Valrena on her sixth birthday for a routine checkup. She sat proudly in her high-backed wheelchair, wearing a plaid Western-style button-down shirt with shiny snap details and brand new jeans. Her grandmother, who had brought her in that morning, was in high spirits, planning a family celebration for later that evening.

Her muscle disease caused her to aspirate food into her lungs. This caused recurrent pneumonias, and these repeated bouts began to cause scarring in her lungs. At first, we were able to treat her simply with IV fluids and antibiotics, then short hospital stays, then longer hospital stays. She required a ventilator for the first time, to support her breathing, the winter after she turned 6.

This was a difficult winter for Valrena and her family. Her lung disease had progressed and her muscle rigidity worsened. Now, every time she was sick she needed the ventilator. Our small clinic has no inpatient beds, and our local hospitals were unable to respond to her increasing medical needs. Each time now we had to fly her to Phoenix for admission. Her lungs were weak and her muscles were tired from the extra work of breathing. It took longer to recover from each illness.

Fleeting Health
By early March we had put her on the ventilator four times. We openly wondered how much longer she could survive. It hardly felt worth it, these snatches of recovery squeezed between periods of devastating illness.

I was in the emergency department when Valrena died. By the time the ambulance brought her in, Valrena was blue around the lips and her fingers and toes were already cool. Her eyes were rolled back in her head and her pulse was thready. Her mother, following close behind, was quiet as we made our initial assessment. A few moments later, Valrena’s heart stopped. We initiated CPR, but within a few moments, her mother asked us to stop.

I sat with her mother as she waited with Valrena’s shrouded body for the coroner to arrive. “I’ve been expecting this moment. But I always thought it would be different. For all the times we brought her in so sick, today she seemed well. She slept in, and I was telling the other kids to be quiet, that Valrena needs her sleep. I checked her once, and she was breathing easily, just sleeping. I checked her oxygen level, and it was good. I went in a little later to fix her blankets. Then, the last time, she seemed to be a little blue, so I checked her oxygen and it was low. That’s when I called the ambulance.”

At her funeral, I saw pictures for the first time of Valrena from before she was ill—standing in the hallway, dressed as a butterfly in diaphanous wings. To the careful eye, there were hints of the future—a slightly gaping mouth, a stance just slightly too wide. Her death was not a surprise. It offered release and even relief. In her life, I saw oxygen levels, fevers, antibiotics. But Valrena’s death made me see the tragedy.

—Ellen Rothman, HMS ’98,
practices in northern Arizona
on the Navajo reservation.

The names used in this column are pseudonyms, and the opinions expressed are not necessarily those of Harvard Medical School, its affiliated institutions, or Harvard University.

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