"I can only take this in small doses," she said to me as I examined her heart and lungs with my stethoscope with reality TV playing on her hospital room television. I thought she was complaining about my examining her. "It's enjoyable for a few minutes, and then you really begin to question where these reality television people's values are."
My patient, age 22, could teach many people far older than she a thing or two about values and priorities in life. I started to tell her this during my morning rounds -- our daily 6 a.m. ritual in the hospital.
Kara had been slowly dying since the age of four. She was unlucky enough to be diagnosed with a rare tumor that most often occurs in the first two decades of life, inflammatory myofibroblastic tumor. Kara presented to her primary care doctor's office with complaints of pain when swallowing that was later shown to be tumor strangulating her esophagus and stomach. The tumor, which rarely spreads to distant areas of the body and rarely behaves aggressively, was doing just what it was not supposed to do. It next spread to her liver and invaded it so dramatically that she required a liver transplant.
In the next 18 years of her life, Kara's rare tumor continued to invade her body -- with massive lesions in her lungs, brain, pelvis and abdomen -- causing pain that was difficult for me to fathom. She would point to pain in areas around her body that corresponded to locations of serious tumor burden. With a stature of five feet, she only weighed 55 pounds when I met her, and she was on an aggressive enough pain regimen -- consisting of high doses of Dilaudid, methadone, Ativan, and gabapentin--that it quite possibly could have been enough to sedate a patient much larger than she. She also had pain down her legs from the pressure from tumors pressing on the nerves providing sensation to them and pain on urination, not from an infection, but from tumor pressing on her bladder. These barbaric tumors would eventually press on some vital organ that would slowly lead to the conclusion of my young patient's life.
In learning more about her medical history, I was also so eager to learn more about her passions and seemingly heroic coping skills. She had always hoped to become a caterer -- baking was a passion she never got to pursue. She loved art and creating multi-media canvases with photography and painting. She loved make up and jewelry. On certain days in the hospital, she spent up to an hour delicately applying lipstick, blush and mascara. She hated her legs -- "just too skinny; I could eat endlessly, and they'd still just be too skinny with my skin flaking off." She loved her mother, who stayed by her bedside during every hospitalization. Kara, after all, had spent more days of her life in the hospital than at home. As she faced death, she worried about her mother and how she would cope. Her artwork, so delicately and thoughtfully crafted, often featured photographs of her and her mother, created specifically for her. Composer Stephen Sondheim aptly wrote about children and art being the most profound ways by which we can leave a legacy for the world after we are gone in his extraordinary work, Sunday in the Park with George. Her murals were expansive, colorful, bright and moving similar to those of Georges Seurat. She used design contours in ways that made a central photograph pop out with great prominence -- she was rapidly creating her legacy of art as Sondheim described. In the field of pediatric palliative care, it has been noted that dying children are often exceptionally concerned about their parents and how they will cope. With such few years on earth, they often quickly develop perspective of how unnatural it is for a parent to lose a child. Kara certainly developed this appreciation early in life.
The gravity of such loss is timeless. In 1833-1834, the German poet Friedrich Rückert wrote Kindertotenlieder, a series of 428 poems on the death of children. The poems were in response to his own personal tragedy of having his two children become ill and pass away from scarlet fever, a bacterial infection that is now usually effectively treated with antibiotics and no longer a devastating cause of death. In one of those poems, he writes about the premature loss of a child, "In this weather, in this gale, in this windy storm, they rest as if in their mother's house: frightened by no storm, sheltered by the Hand of God." Kara's mother could have written the lines of that poem about losing her daughter.
The goal of this hospitalization was to start her on a new chemotherapeutic medication. If she could tolerate the medication, which would likely cause significant nausea and vomiting and slight vision loss that was not permanent, there was about a fifty percent chance that the symptoms due to her bulky tumors would improve. We read the article about the new drug, published just a few months ago, from the New England Journal of Medicine together.
"We just have to have hope, doc" she told me, "I'd really love to be able to bake if this drug works... I'd love to have my life back again."
She talked to me about her wishes as I held her hand. I hoped that one day those hands would be able to bake in addition to creating beautiful art. Kara not only provided me with hope, she showed me perspective on life and living in the present that I had never truly understood. Though she was slowing dying, she taught me so much about living.
Follow Up Note:
During Kara's hospitalization, I told her of my plan to blog about her and how my writing was progressing. She was very excited to read (and critique) it. When I came to the hospital on the morning of September 23, 2011, I had a draft of the blog to share with her and found out that she had passed away earlier that morning. She died in her sleep with her mother by her side. In the hospital that morning, all of us who had the honor of caring for this remarkable patient shared our thoughts and reflections during rounds and took a moment of silence together with all the physicians, nurses, social workers, patient care associates and staff on the floor. With the permission of her parents, I published this blog, dedicated in loving memory to Kara.
Robert E. Accordino MD, MSc
The views expressed in this blog are my own and do not reflect the views of The Mount Sinai Medical Center.