Unwrapping Lazarus

New Life for Cystic Fibrosis Patients
Giovanni di Paolo ‘The Resurrection of Lazarus,’ 1426 (Acquired by Henry Walters, 1911/Wikimedia Commons).

In the biblical story of Jesus raising Lazarus from the dead, the deceased receives a shock when he is unexpectedly ripped from the other world and returned to ours. So certain was Lazarus’s death—pronounced four days earlier—that the narrator of the story cannot even bring himself to call the revived man “Lazarus,” referring to him instead as “the dead one.” Apparently, no one else present at the miracle is quick to recognize Lazarus either, for Jesus has to command those attending the funeral to “release Lazarus from his grave clothes and let him go.” Wrapped fast in reeking bandages, Lazarus remains immobile in his tomb, like a mummy awaiting total reanimation.

Only recently have I begun to understand something of Lazarus’s situation in that pregnant moment at the tomb. I have cystic fibrosis (CF), a genetic disease that affects my entire body, but most seriously my lungs. For most CF patients, this condition makes a cruel companion in life, complicating and interrupting daily tasks with time-consuming treatment regimens, derailing plans with frequent and unpredictable hospitalizations, and, most ominously, threatening premature death. In 1980, the life expectancy for people living with CF was less than twenty years; before that, few made it past childhood.

But a new light has appeared on the horizon. Indeed, it would not be too much to call the past five years—and especially the past year—the critical juncture in the history of cystic fibrosis. Over the past thirty years, treatments for the symptoms of CF have steadily improved the quality of life and increased the life expectancy for people with the illness. But today, for the first time in our history, medicine is available that doesn’t just treat the symptoms of CF but addresses its root causes. These new drugs are classified as “Cystic Fibrosis Transmembrane Conductance Regulators” (CFTR or “modulators” for short), and their basic function is to enable the CFTR proteins to operate within the cell. When these proteins function inefficiently or not at all, thick mucus overruns the body, leading to organ malfunction, increased infection, and airway clogging. The ultimate result is death.

The first generation of CFTR modulators appeared in 2012 under the name Kalydeco, but that drug could treat only 4 to 5 percent of the seventy thousand people who suffer from CF worldwide. That changed in 2015, when the modulator underlying Kalydeco was combined with another to produce a drug known as Orkambi. With this new combination, more than 40 percent of CF patients could benefit from the medication—including me. Then, in October 2019, the FDA approved Trikafta, the first triple-combination modulator. Now 90 percent of CF patients can receive this life-altering treatment. These medicines have been greeted with something like a messianic fervor, and rightly so: for the CF community, this is our Lazarus moment.

Trikafta has been nothing less than a revolution for those suffering severely from this disease. Over the past months I have read hundreds of testimonials from fellow CFers who are living a new, resurrected life. Their lung function has skyrocketed by 20, 30, even 40 percent, allowing many of them to be removed from lung-transplant lists; mothers with CF now expect to live long enough to see their children grow into adulthood; students have resumed their college studies after being forced to drop out because of CF; newfound breathing capacity allows patients to run miles. One story that particularly struck me: a man shared that for the first time in years he was able to put fitted sheets on his bed without collapsing in exhaustion. Life—in all its texture, joy, and possibility—is being returned to those born with this disease.

 

Life—in all its texture, joy, and possibility—is being returned to those born with this disease.

In the biblical story of Jesus raising Lazarus from the dead, the deceased receives a shock when he is unexpectedly ripped from the other world and returned to ours. So certain was Lazarus’s death—pronounced four days earlier—that the narrator of the story cannot even bring himself to call the revived man “Lazarus,” referring to him instead as “the dead one.” Apparently, no one else present at the miracle is quick to recognize Lazarus either, for Jesus has to command those attending the funeral to “release Lazarus from his grave clothes and let him go.” Wrapped fast in reeking bandages, Lazarus remains immobile in his tomb, like a mummy awaiting total reanimation.

Only recently have I begun to understand something of Lazarus’s situation in that pregnant moment at the tomb. I have cystic fibrosis (CF), a genetic disease that affects my entire body, but most seriously my lungs. For most CF patients, this condition makes a cruel companion in life, complicating and interrupting daily tasks with time-consuming treatment regimens, derailing plans with frequent and unpredictable hospitalizations, and, most ominously, threatening premature death. In 1980, the life expectancy for people living with CF was less than twenty years; before that, few made it past childhood.

But a new light has appeared on the horizon. Indeed, it would not be too much to call the past five years—and especially the past year—the critical juncture in the history of cystic fibrosis. Over the past thirty years, treatments for the symptoms of CF have steadily improved the quality of life and increased the life expectancy for people with the illness. But today, for the first time in our history, medicine is available that doesn’t just treat the symptoms of CF but addresses its root causes. These new drugs are classified as “Cystic Fibrosis Transmembrane Conductance Regulators” (CFTR or “modulators” for short), and their basic function is to enable the CFTR proteins to operate within the cell. When these proteins function inefficiently or not at all, thick mucus overruns the body, leading to organ malfunction, increased infection, and airway clogging. The ultimate result is death.

The first generation of CFTR modulators appeared in 2012 under the name Kalydeco, but that drug could treat only 4 to 5 percent of the seventy thousand people who suffer from CF worldwide. That changed in 2015, when the modulator underlying Kalydeco was combined with another to produce a drug known as Orkambi. With this new combination, more than 40 percent of CF patients could benefit from the medication—including me. Then, in October 2019, the FDA approved Trikafta, the first triple-combination modulator. Now 90 percent of CF patients can receive this life-altering treatment. These medicines have been greeted with something like a messianic fervor, and rightly so: for the CF community, this is our Lazarus moment.

Trikafta has been nothing less than a revolution for those suffering severely from this disease. Over the past months I have read hundreds of testimonials from fellow CFers who are living a new, resurrected life. Their lung function has skyrocketed by 20, 30, even 40 percent, allowing many of them to be removed from lung-transplant lists; mothers with CF now expect to live long enough to see their children grow into adulthood; students have resumed their college studies after being forced to drop out because of CF; newfound breathing capacity allows patients to run miles. One story that particularly struck me: a man shared that for the first time in years he was able to put fitted sheets on his bed without collapsing in exhaustion. Life—in all its texture, joy, and possibility—is being returned to those born with this disease.

Published in the December 2020 issue: 

Roberto J. De La Noval recently received a PhD in theology from the University of Notre Dame.

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