Cystic fibrosis (CF) is a genetic disease that every medical student becomes familiar with because it is common, causing by a single amino acid substitution, and has a clear link with severe lung (and other organ system) disease that can result in the need for transplantation,. LIfespan of patients with CF is severely curtailed and CF patients are plagued with infection after infection as their lungs are clogged with thickened secretions. It is the third leading cause of lung transplantation. Often, these infections involved highly drug resistant — as an ID fellow I vividly recall a twentysomething female die of a totally drug resistant Pseudomonas fluorescens infection — and ominous bacteria.
Infection with the Burkholderia cepacia complex, however, is the most significant of these infections. This gram negative bacteria targets those with cystic fibrosis and immunosuppressive conditions. When a cystic fibrosis patient is colonized with this complex, their chances of survival diminish. This fact leads some transplant centers to refuse to transplant patients with this recalcitrant infection.
The complex consists of a group of bacteria (one is even called B.metallica), with varying degrees of importance. Genoserovar III — B.cenocepacia — is the most dangerous and only two institutions in North America including one in the US (not surprisingly UPMC) will offer transplants to these individuals because the mortality post-transplant can be exceedingly high.
With these facts as context, I wanted to draw attention to a movie that, surprisingly to me, focused on this bacterial complex. Five Feet Apart is a 2019 movie that tells the story of young cystic fibrosis patients struggling with their disease, holding out for new treatments, and hoping for transplants. As part of their regimen they are forbidden to be close to other CF patients to avoid transmission of infections, which is difficult when there are romantic connections developing. One character suffers from B.cepacia and the movie details the efforts of medical professionals to try new treatments on him while also preventing it from spreading to other patients, particularly the protagonist. Not surprisingly, this story was inspired by a real life couple who were transplanted at — again no surprise — UPMC.
While the movie is not a medical documentary, the fact that it highlights the social aspect of infectious disease — the social (and emotional) distancing that can occur with an transmissible infection and how hard that can be to cope with for patients makes it particularly compelling to medical professionals. I think it is a film well worth watching for this aspect.
Hopefully, better CT treatments — drugs that modulate the function of the abnormal gene such as lumacaftor and ivacaftor — will diminish the need for transplantations and increase lifespans.