The saddest situations aren’t the 95-year-old grannies who’ve lived good lives. They’re not the people who’ve abused their bodies until the wheels fall off. They’re not even really the accidental stuff, the traumas that, in a split second, change a life forever. The saddest things are the young people with chronic illnesses, the healthy people whose bodies just up and betray them one day. Arguably, the worst of these illnesses is pulmonary fibrosis.
It’s fairly rare — the stats on Wikipedia say somewhere between 10-30 per hundred thousand people — but it’s devastating. It’s one of those diseases where you seriously wonder whose mom you ran over in another life to deserve it. Your normally very elastic lung tissue is replaced gradually with scar tissue — which doesn’t stretch. It’s like breathing with a belt around your chest, and every day the belt gets just a tiny bit tighter until finally, you suffocate to death. The treatments are generally just big-gun immunosuppressants. There’s no real specific treatment. Most people are dead within 5 years.
There’s no nice way to slice it. It sucks and it’s awful. Who can handle being given a death sentence and told “there’s nothing we can do”?
She’d been told that when she was in her 20s. She suffered through the shortness of breath, dragging an oxygen tank around with her. She was young and healthy otherwise, though, and so ended up on the transplant list. A lottery where if you win, you get to live. And lucky her — she won a pair.
I’ve heard it said that a lung transplant is trading one chronic disease for another — and it’s the truth. The antirejection drugs they give you after the transplant make you more likely to get infections that’ll wreck your lungs — it’s not uncommon to get colonized with something strange. Your new lungs aren’t connected to your nervous system so your cough reflex doesn’t exist. The drugs come with their own side effects — unpleasant ones. And sometimes your lungs come with pathogens of their own that you didn’t know about until after the recovery period.
She lived for months inside a hospital, slowly and tediously recovering from an illness that lay waste to the landscape of her body. She nearly died of CMV pneumonia. Her new lungs ended up getting destroyed and her shortness of breath came back. Again, she felt like she was suffocating to death. And then, the transplant center told her she was in full blown rejection, that there was nothing they could do, and that she should go home and get her affairs in order and be with her family.
I first met her because she had asked her family to help he commit suicide. She had suffered for years at this point. She remembered acutely what it was like to feel like she was suffocating to death, and she was terrified to die that way. So terrified, in fact, that she had begged her husband to kill her.
I provided no real respiratory therapy. Respiratory wise what could be done was being done. Instead, I sat on her bed and we talked, over her breakfast, while her husband snoozed in a cot by her bed. She described to me what she was afraid of: suffering. The reason she’d asked him to help her die was because she didn’t want to suffer; I ended up spending my visit explaining to he the concept of palliative care.
I explained that with diseases where you’re dying of shortness of breath, what we do is give medications to relieve shortness of breath. Ordinarily with respiratory disease we would not want to give medications that suppress the so-called “drive” to breathe, lest we worsen the situation. When we are palliating respiratory disease, on the other hand, the drive to breathe is what causes the sensation of shortness of breath — so we will in fact very deliberately suppress the drive to breathe, so as to relieve the shortness of breath. These medications are often sedating, so we only give you enough to make your time tolerable, so you can enjoy your time left with your family without suffering. Instead of increasing quantity of life, the goal is to increase quality of life.
She cried, cried and cried, her relief pouring off of her with her tears. She had so anticipated suffocating to death that to be told she doesn’t have to suffer was very relieving. Now, instead of feeling like her only way out was an act of desperation done in secret, she instead could pass away in a safe place, in relative comfort, with her family by her side seeing that she didn’t have to suffer. I saw her again a couple of days later and she seemed at peace. She’d spent some time touring the palliative care ward, talking to the palliative care coordinator. It seemed the relief had settled on her.
I was not surprised to find out that she had passed away only a few days later.
I think about how I got into respiratory to save lives, but in all honesty, I spend a good chunk of time helping people end them. I can’t say I find this disagreeable… I feel much, much better at the end of the day knowing I helped give a very sick lady what she wanted — a good death.