In the myth of Ondine, a water nymph falls in love with a mortal, giving up her immortality for him. When he betrays her, she curses him: he will continue breathing only as long as he stays awake. The moment he falls asleep, his body will stop breathing, leading to his death. This curse mirrors the rare medical condition called Congenital Central Hypoventilation Syndrome (CCHS), where affected individuals must consciously breathe or rely on ventilators, especially during sleep, as their bodies “forget” to breathe automatically.
CCHS is primarily caused by mutations in the PHOX2B gene, which plays a crucial role in the development of the autonomic nervous system (ANS). The ANS regulates involuntary bodily functions like heart rate, digestion, and breathing. In healthy individuals, the ANS monitors carbon dioxide (CO₂) levels in the blood and adjusts breathing rates accordingly to maintain homeostasis.
Although CCHS is usually present at birth, individuals can acquire Central Hypoventilation Syndrome (CHS) from trauma to the brainstem. In a case study I found, a patient acquired CHS after undergoing a posterior fossa craniectomy to remove a hemangioblastoma in the cerebellomedullary cistern. The surgery affected the brainstem, which controls breathing, leading to problems after the operation. Although the patient breathes normally while awake, they had difficulty breathing during sleep, resulting in apneic episodes. Continuous monitoring confirmed the diagnosis of acquired CHS as a result from the surgery. The patient underwent a tracheostomy on the fifth day and was started on respiratory stimulants, but there was no improvement. After 14 days in the hospital, they were discharged against medical advice due to financial reasons. This case study poses several ethical dilemmas. What do you think is the most important ethical consideration in this situation?
Karn M, Mahato BK, Sah R, Kandel D, Sapkota S. "You Sleep, You Die": A Rare Clinical Case of Ondine's Curse after Posterior Fossa Surgery. Case Rep Surg. 2023 Sep 11;2023:3113428. doi: 10.1155/2023/3113428. PMID: 37727799; PMCID: PMC10506874.
I wont lie josh I was going to write about this but ya beat me too it. But my answer is YES I would wish this on my worst enemy *** cough cough Eric.
ReplyDeleteBut on a more academic approach, I do believe there has been some real good advancements in the treatment of this condition, if you have good enough insurance I presume. But they use a diaphragm pacing stimulation system (DPSS). Electrodes are implanted on the diaphragm and cause contraction on stimulation. Usually this was used for patients with amyotrophic lateral sclerosis (ALS), But it is really cool how they found a way to try and extend patients quality of life suffering from Ondine's curse.
Niazi, A. U., Mocon, A., Varadi, R. G., Chan, V. W., & Okrainec, A. (2011). Ondine's curse: anesthesia for laparoscopic implantation of a diaphragm pacing stimulation system. Canadian journal of anaesthesia = Journal canadien d'anesthesie, 58(11), 1034–1038. https://doi.org/10.1007/s12630-011-9580-9