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Authors

  1. Brown, Theresa PhD, RN

Abstract

Cystic fibrosis and an unfinished life.

 

Article Content

Mallory Smith's Salt in My Soul: An Unfinished Life (Spiegel and Grau, 2019), an account of the author's struggle with cystic fibrosis (CF), will pull at your heartstrings. Smith kept a diary from the age of 15 years until she died in 2017 at age 25. This book, published posthumously, is an edited version of those 2,500 pages. Smith's honesty about the difficulty of balancing normal life with having an incurable disease offers nurses valuable insights into the patient experience. Perhaps inadvertently, it also raises important questions about the allocation of health care resources in the United States.

  
Figure. Theresa Brow... - Click to enlarge in new windowFigure. Theresa Brown

I found the "Introduction" most poignant. Smith must have written it when she was fairly sure she would die soon, stating, "I write to leave something behind . . . so I won't just end up as ashes scattered in the ocean and nothing else." She adds,

 

Cystic fibrosis is a disease that does a lot of taking-of dreams, of time, of travel, of friendships, of freedom, of potential, of plans, of lives. . . . More than that, it's easy to think about all the things I want for my future that might no longer be possible, the will-be-takens.

 

This broke my heart. I can't imagine how painful it must be to know from the age of three years on that one had a genetic disease likely to cause early death from respiratory failure.

 

In writing about her childhood, Smith also chronicles her parents' pain. At age nine, she decided to stop doing her daily chest physiotherapy treatments, and although her mother "tried pleading, bribing, punishing, and yelling," Smith continued to refuse. Her dad intervened, laying out the hard truth: "Your mother and I do not ask you to do them so you'll feel better. . . . We ask you to do them so you won't die. If you don't do your treatments, you will die." Years later he explained his "extreme" approach: "Sometimes . . . tough love and brutal honesty are the only arrows left in our quiver."

 

Smith grew up in Los Angeles, with parents who were financially well-off. Economic privilege is evident in her stories of succeeding at Beverly Hills High School and Stanford University; and in her descriptions of family trips to Hawaii, a place she found healing. But her sun-drenched memories stand in jarring contrast to her illness status, as she sadly realizes: "That summer of 2014 was freedom. It was fantasy. My life was a movie that I didn't want to end. No perception of my body being limited in any way until my very last night on the island." That night Smith coughed up so much blood that she had to be hospitalized: "Hemoptysis. I plunged, free-fall. The fabric of my perfect life unraveled. . . . When you're falling, there's only one direction you can go."

 

Smith belonged to a small subgroup of CF patients whose lungs become colonized with Burkholderia cepacia, a type of bacteria resistant to most antibiotics. This colonization made Smith's disease harder to treat and led her to develop cepacia syndrome, which causes high fever, an elevated white blood cell count, and severe pulmonary decline.

 

Her only chance at a reprieve from the progressive nature of CF was a lung transplant; but because of her B. cepacia colonization, no transplant center would take her. Finally, the University of Pittsburgh Medical Center's transplant program accepted Smith, and she received a double-lung transplant-only to die two months later, when her new lungs became infected with B. cepacia.

 

Smith's transplant and its failure gave me pause, especially in light of ongoing debates in this country about how to best meet the economic burdens of health care. Her double-lung transplant probably cost half a million dollars. Smith acknowledged how lucky she was to receive excellent health care, which was largely covered by her family's insurance. But according to recent Gallup poll data, nearly 14% of the U.S. population currently lacks health insurance, and millions more have plans with high deductibles and copays they can't afford. This gross lack of fairness in how we apportion care is perhaps the salt in our collective soul.

 

Mallory Smith was a remarkable young woman. If telling her tragic story helps to foster a larger discussion about how to ensure that all Americans get the health care they need, I think she'd find that a worthwhile outcome.