In the course of preparing a manuscript recently that focused on the perioperative management of patients with cystic fibrosis, I discovered a remarkable book that should be required reading for every physician who cares for patients afflicted with this devastating disease. The book, titled Breathing for a Living, is a poignant memoir written by a 21-yr-old woman living with, and dying from, cystic fibrosis. Although the book was written two decades ago, its lessons are timeless.
The author, Laura Elizabeth Rothenberg, was a gifted student at Brown University who valiantly fought the indignities of cystic fibrosis every day, with every fiber of her being. Diagnosed at 3 days of age when she required emergency surgery for meconium ileus, Laura led an extraordinary—and inspirational—life.
She chose her parents wisely. Mary Sinclair Rothenberg and Dr. Jon Anderson Rothenberg understood Laura’s illness both as a medical disease and as a personal tragedy. They gave Laura unconditional love, unwavering respect, and steadfast support, although Jon admitted, “It was hard, particularly for me, and for me as a doctor, to be enough in the background.” Laura dedicated her book to her parents, “who taught me how to live despite illness.” (At this point, I must disclose that Jon Rothenberg, a respected New York internist who worked mostly with disadvantaged populations, and I were both students at Cornell University Medical College in the early 1970s, although we were in different classes.)
The book does not dwell on the details of Laura’s early life, although we learn she lived on Manhattan’s Upper East Side and attended the Chapin School in Manhattan and then Brown University, where she wrote passionately about her life and its seemingly endless challenges. She had a compelling, expansive personality that brooked no inconsistencies, slights, or contradictions. Laura was scorchingly honest and open, autocratic, and strong. She was also witty, ironic, courageous, loyal, and generous. Despite the fact that chronic illness can be isolating, Laura had a remarkable talent—indeed, a genius—for friendship, treasuring each and every friend as a priceless gift.
Starting around age 12, Laura spent much of her life in hospitals, where she “always felt happy and safe.” For several years, she was managed by a compassionate and caring team of professionals at Columbia Presbyterian Babies’ and Children’s Hospital of New York. She formed enduring bonds with her physicians, nurses, therapists, and the other pediatric patients—especially those with cystic fibrosis—living most of her life with the inability to know what would happen next. Despite indomitable hope, she was forced to try to live in the eternal present.
Laura had high standards and was not shy about registering her objections when people did not meet her expectations. One disappointing incident involved a cardiology fellow who entered Laura’s hospital room without knocking or asking permission to enter. He also failed to introduce himself and state the reason for his visit, immediately going to the sink to wash his hands rather than communicating with his patient. Twice Laura attempted to engage the aloof fellow in conversation. “Excuse me. Can I help you?” No response. Next, “Hi yes, person at the sink, I’m talking to you! Excuse me, are you going to introduce yourself? Aren’t you at least going to ask permission to use my sink? My name’s Laura. I’m 19. I have CF, I go to Brown, and this is MY room.” Shocked by the blunt questioning and forceful narrative, the fellow exited the room! Laura relished telling everyone she encountered that day how she valiantly protected her space. Clearly, she was a force of nature to be reckoned with! Words were her sharpest weapons and best defense.
Her health inexorably deteriorating, at age 19 Laura made the difficult decision to have a bilateral lung transplant, well aware that the surgery could kill her or add even more medical problems to the ones caused by cystic fibrosis. She recognized that transplantation involves exchanging one medically challenging condition for another. She also understood that it was her only chance at a longer life.
Because Laura did not meet Columbia’s criteria for bilateral pulmonary transplantation, in February, 2001, during her sophomore year, Laura took a leave from Brown and moved to Boston to wait for new lungs. She waited for months to receive “the call.” (Characteristically, to relieve the boredom, Laura established a “transplant lottery,” with her friends proposing dates when they thought the surgery would occur.) Finally, on July 15, 2001, Laura received the bilateral transplant at Children’s Hospital in Boston, with her family and friends filling the surgical waiting room. Before she went to the operating room, Laura received a call from one of her greatest supporters, an anesthesiologist at Babies’ Hospital who had cared for her for years. (Indeed, he called her daily during her posttransplant struggles. These efforts meant the world to Laura and her parents.) T-shirts were made for her loyal coterie with “Team Laura” on the front and on the back. After 19 days, Laura left the hospital, breathing much more easily. Sadly, however, that improved breathing was to be a temporary respite.
Within weeks of the transplant, Laura developed small bowel obstruction that required surgery. Then posttransplant lymphoproliferative disease materialized from her immunosuppressant drugs. She also developed stenoses in the respiratory tract, necessitating almost weekly bronchoscopies under general anesthesia. Eventually, she received stents to keep the airways patent. Her thorax and abdomen covered in a lattice work of scars, Laura had approximately 40 surgical procedures in the 20 months that her transplant held. After repeated pneumonias and a cascade of other debilitating complications, her cystic fibrosis–related diabetes worsened, culminating in what Laura wryly described as “the cherry on the transplant sundae.”
By Thanksgiving 2002, Laura was diagnosed with chronic rejection, and there was no longer anything curative that could be done. She lived to correct the page proofs for her book, but she died at age 22 yr on March 20, 2003, several weeks before her book appeared in print.
For years preceding her death, she had worried that people would forget about her—that memories of her brief and fragile stay on earth might quickly fade. Laura succeeded in making her memory enduring through love, through courage, and through the words she used to reflect these noble emotions. The Laura Rothenberg Bronchoscopy and Endoscopy Center at New York–Presbyterian Morgan Stanley Children’s Hospital bears witness to her noble spirit and to her ability to humanize what was inhuman. Her beloved father, Jon, died seven years after Laura, from complications of valvular heart disease—literally from a broken heart.
Given the heterogeneity of its genotypic and phenotypic manifestations, cystic fibrosis is best considered as a disease spectrum rather than a distinct, single clinical entity. Although cystic fibrosis was first described almost 85 yr ago when 80% of affected infants died during their first year of life, there is still no cure for this dreadful illness; it remains the most common lethal genetic disease of Caucasians. Nonetheless, medical advances during the last two decades have impressively improved survival, longevity, and quality of life for cystic fibrosis patients. Owing largely to the introduction of cystic fibrosis transmembrane conductance regulator modulators in 2011, as well as better results after lung transplantation, current predictions indicate that half of babies born with cystic fibrosis in 2020 will live to age 48 yr when cared for in specialty clinics.
Although gene therapy has been explored as a potential cure for cystic fibrosis, results to date have shown limited success. Nevertheless, we can continue to hope for a breakthrough. As Emily Dickinson famously wrote, “Hope is the thing with feathers – That perches in the soul” and sings its song, no matter what. Hope is a precious resource that gives people the strength to carry on. Laura Rothenberg had that gift in abundance.
