Greater Expectations: How The Lazarus Effect Has Been Resurrected

Happy New Year to all of our readers! This past year has been such a whirlwind and we hope that you all have enjoyed reading our articles as much as we like writing them. Though our work generally focuses on politics or current events, the article this week – in honor of new beginnings –  focuses on something good, even miraculous, even when that good comes with its own complications.

In 2019, a medicine known as Trikafta first hit the market for cystic fibrosis patients. For those who don’t know, cystic fibrosis is a fatal genetic disease, caused by an overproduction of sticky mucus that slowly destroys the lungs and other organs.

Once upon a time, cystic fibrosis was a death sentence. In 1954, the life expectancy for a child born with the disease was just four to five years. Medical advancements made some progress, but it was still limited; a child born with CF between the years of 1993-1997 was not expected to live beyond 31, and those three decades would generally be filled with hospital stays and illnesses.

But Trikafta changed all that. Hailed as a “miracle drug,” the medicine not only effectively stops CF’s progression, but also helps restore lung function in those who have dealt with the effects of CF for years. Doctors have reportedly stated that the life expectancy of a child or baby born with cystic fibrosis today will have the same life expectancy as a non-affected peer, though studies have obviously not yet been able to verify this. That said, the drug is not perfect; reportedly ten percent of the CF population has a variation of the disease that makes it impervious to the medicine. 

But for the most part, Trikafta has been a wonder drug for an illness that has historically been inevitably fatal, so it might be surprising to learn that those taking it have complicated emotions surrounding their new medication. 

Escaping Death

Those who are unacquainted with the reality of a fatal disease may assume (as I did), that the development of a life-saving drug would be met with nothing but total elation. However, the truth is a lot more complicated. As articulated in a November essay featured in Bari Weiss’s Free Press, a patient poignantly discusses the complex emotional and psychological dimensions of seeing one’s identity and future change so quickly:

“Some CFers don’t know who we are without dying. Next year is a blank page. I’ve never experienced that before . . .I am incredibly grateful but also incredibly confused . . . Now that I’m not dying anymore, I don’t know who I am.”

On a CF forum on Reddit, many users similarly express a new set of challenges following the release of Trikafta. One discusses the daunting task of entering the workforce after years of illness left him with no experience to put on a resume, while another grapples with the unfamiliar territory of building credit and relationships, having never anticipated a life beyond 30.

“I never dated seriously because I thought I’d die. I never figured out what I wanted to do career wise. I’m 29 now and lonely and lost as f*ck. Trikafta has me living longer so now I have to figure it all out,” wrote a third user.

Sadly, for many patients, Trikafta has been associated with worsening symptoms of depression and anxiety.¹ Though it is possible that the mechanism that loosens mucus may also have a negative psychiatric effect, some doctors have theorized that the decline in mental health may be attributed to the fact that patients are suddenly faced with a future they never imagined would be possible.

But this phenomenon isn't unique to cystic fibrosis: this scenario echoes the seismic shifts in the HIV/AIDS landscape during the late 1980s and 1990s, where patients similarly navigated the uncharted waters of extended lifespans.

The Lazarus Effect

The HIV/AIDS epidemic in the United States during the 1980s and early 1990s was a period of profound crisis and, later, transformation. The first cases of what would later be known as AIDS were reported in 1981, initially among gay men in Los Angeles and New York. This mysterious new illness was characterized by a rapid deterioration of the immune system, which in turn led to other health issues like infections and tumors. Doctors were flummoxed by the cause, even believing it was a type of cancer rather than a viral affliction. 

It did not help that at the time, the disease was stigmatized as a "gay plague,” and the bigotry surrounding homosexuality meant that many people who were infected preferred to stay hidden, keeping the true scope of the epidemic, as well as its symptoms, opaque. Prejudice also played a part in the muted societal discussion and overall view of the disease.

In its early years, an HIV/AIDS diagnosis meant a life expectancy of just 1-2 years. The epidemic's toll was staggering – in 1990, AIDS accounted for 61% of all deaths of men aged 25-44 in San Francisco, 51% in Ft. Lauderdale, and 35% in New York.²

With such grim numbers, many of those afflicted with the disease took drastic measures to live their lives to the fullest. It was not unusual for HIV/AIDS patients to spend their life savings – and even sell their life insurance payouts – to afford either treatments or a lavish last hurrah.

Then, in 1996, a series of new antiviral medicines, known as protease inhibitors, hit the scene. This drug meant that a diagnosis was no longer a death sentence. The reaction to these drugs was much like that to Trikafta today. In fact, a quote from one man in a 1998 New York Times article are almost identical to the Reddit comments today:

''On the surface it's wonderful to walk down the street, see myself in a store window and think, God that's me . . . But there's an awful lot below that surface. All my friends are dead. I haven't worked since 1992. I haven't had sex in six years. How do you begin being a human being again?''

The psychological impacts were so severe that it was even given a name: the Lazarus Syndrome, named after the biblical figure that Jesus Christ raised from the dead. As explained by Dr. Robert Remien, a clinical psychologist and researcher at the H.I.V. Center for Clinical and Behavioral Studies at the New York State Psychiatric Institute:

“When you suddenly find yourself facing a much longer-term future, people are left with this feeling of, ‘What do I do now? This isn't what I was planning. I have to worry about retirement benefits? I may have to go back to work? I may have to pay off those credit cards?' That is tremendously traumatic and disruptive.''

The CF Buyers’ Club

Another striking similarity is the emergence of buyers’ clubs for both diseases. Trikafta is still under patent by pharmaceutical company Vertex, and the cost per patient is estimated at around $300,000 a year. Such a hefty price tag has left some countries, such as India, South Africa, and many in the developing world, unable to come to financial terms with Vertex. As a result, those suffering with CF in those places have found themselves without access to Trikafta.

However, enterprising CF individuals and their family members have found a way to get their hands on the miracle drug another way: by creating Cystic Fibrosis Buyers’ Clubs and traveling to a lone pharmacy in Argentina that offers the only generic option in the world. Argentina does not recognize patents, and so a single Argentinian pharmaceutical company has managed to reverse engineer Trikafta. The price of this generic drug is still expensive: an estimated $10,000 to $15,000 a year, though it is undoubtedly less when using the buyer’s club. Still, many CF patients understandably believe that the cost is worth it. Sadly, many are unable to afford the steep price, and crowdfunding websites like GoFundMe often have campaigns to raise money so either they or their family members can access this medicine.

Similarly, the HIV/AIDS epidemic spawned many similar operations, a fact made famous by the Matthew McConaughey movie The Dallas Buyers Club, which in turn was based on the life of Ron Woodroof. Woodroof, after being diagnosed with AIDS, sought out alternative medicines and would sell his finds to other HIV/AIDS sufferers in the Dallas area, creating a little club for his unapproved drugs.

Of course, one major difference is that Trikafta, and its generic, actually work. As noted by The Washington Post, Woodroof’s medicines were not effective against HIV, and those afflicted were better off continuing with the officially prescribed medicine at the time, despite its unsavory side effects.³

Looking to the Future

Interestingly, both HIV/AIDS and cystic fibrosis still lack a cure, but even that prospect is on the horizon. Gene therapy was recently developed to cure sickle cell anemia, and there is at least one gene therapy medication currently going under trial in the United States. A remedy for HIV/AIDS is likely even closer, as at least three people (and some sources even claim five) are confirmed to have been cured of HIV following stem cell transplants.

However, CF and HIV/AIDS are not the only diseases or health concerns that will likely be overhauled in the coming decades, raising its own questions about the future of society.  If, tomorrow, scientists were able to create a drug that upped the average lifespan to 150 years, the societal impacts would likely mirror the Lazarus pattern, especially for the older generations. At a minimum, it would spell financial disaster, as most retirees would be unable to fund an additional five decades of living expenses. Moreover, the American economy would be close to collapse unless it reneged on its Medicare and Social Security responsibilities; many countries in Western Europe, with socialized healthcare and generous safety nets, would also likewise feel enormous financial pressure.

However, the effects would also likely apply to younger individuals as well. For example, the median age of marriage for women in the United States is 28.6 years. Would this change with the knowledge that the commitment would be 120 years versus 50 or 60? Or even workers in their 40s and 50s – if the traditional retirement age of 65 becomes unrealistic, how many employees will change professions in light of decades more of work? How many will face burnout?

Though these consequences may raise uncomfortable hypotheticals, we hope that the focus remains on the amazing prospect of defeating illnesses and even old age itself. As we step into a new year, stories like these remind us of the power of medical science not just to extend life, but also to challenge our perspectives on living it fully. Happy 2024!Thank you for reading History, Rinse, & Repeat. This post is public so feel free to share it.

1. https://www.ncbi.nlm.nih.gov/pmc/articles/PMC9793010/

2. https://www.thebritishacademy.ac.uk/blog/aids-epidemic-lasting-impact-gay-men/

3. https://archive.is/v6LF6#selection-461.152-461.164