Ondine's Curse and Other Unusual Causes for Respiratory Failure

There's a short list of really common reasons patients may need a mechanical ventilator in the intensive care unit: pneumonia, acute respiratory distress syndrome, pulmonary edema, hypercarbic respiratory failure, altered mental status. But at a tertiary referral center like Stanford, we get a few rare causes of respiratory failure. A few months ago, I admitted a patient who was simply too weak to take a deep breath. She was young, but she had a rhematologic illness that infiltrated her central (proximal) muscles. She went from working full time to being completely bedbound and gasping for air within months. After confirming the diagnosis with nerve and muscle conduction studies and a muscle biopsy, we started high dose immunosuppressants. Soon she was walking around the ICU on a ventilator. With good rehabilitation and rheumatologic followup, we hope that she will regain the ability to breathe.

Another patient has a more unfortunate disease. She has a tumor in her brainstem right at the center that controls subconscious breathing. This is a big problem; she is effectively cursed by Ondine. Ondine was a water nymph who had a unfaithful mortal lover, so she cursed him so that if he fell asleep, he would stop breathing (he had previously vowed that every waking breath was a testimony of his love). This patient suffered two cardiac arrests from apnea - she stopped breathing, and there was no automatic stimulus or drive to breathe. Normally, our brainstem regulates our breathing pattern while we are asleep, but the location of her brain tumor disrupts those signals. During the daytime, she is fine, but when she falls asleep, she needs a backup rate to breathe for her through a tracheostomy. She, too, will do fine; her brain cancer is benign, but she will need to be in a long term ventilator facility, and we need to see if chemotherapy and steroids shrink the tumor such that she remembers to breathe again.

First image of a mechanical ventilator shown under Creative Commons Attribution Share-Alike License, from Wikipedia. Second image of John William Waterhouse's painting of Ondine is in the public domain, from Wikipedia.