ondine's curse

he lay quietly, the rhythmic breaths from the vent lulling him deeper into sleep. his mother, weary from lack of sleep, sat in a chair at the bedside, unwilling to leave him. if he woke, he would want her.
before this night, i did not know the extent of their journey together, this young boy and his mother. as it became clear that he would sleep soundly, the tension drained from her face. i asked her how it all began. as she eagerly began to recount their story, i was struck by how young she looked, after having been through so much.
there were multiple, frustrating trips to emergency rooms, doctors who told her that there was nothing wrong. once it became clear to all who knew him that there was, indeed, something wrong, an excruciatingly long stay in the PICU. yet, still, no diagnosis.
finally, after much searching and many tests, the diagnosis was clear. ondine's curse. congenital central hypoventilation sydrome (CCHS), a rare disorder that causes sleep apnea and usually requires tracheostomy and life-long mechanical ventilation, but only at night.

the name of the disorder, ondine's curse, comes from the myth of ondine. in the current rendition of the story, ondine was a water nymph, stunningly beautiful, who fell in love with a human. when she married him and bore his child, she lost her immortality. as she aged, her husband lost interest in her and was unfaithful to her. she discovered him in the arms of another woman and cursed him, telling him that as long as he remained awake, he would live, but as soon as he slept, his breath would desert him.

in the original tale, "Undine" by Friedrich de la Motte, undine's father, a prince of the mediterranean sea, wishes for her to possess a soul, as undines' do not have souls. (undines are spirits of the elements, earth, water, wind, fire.) he sends her to live with a fisherman and his wife who have lost their own daughter, bertalda, after she wandered away (lured by undine's uncle, kuhleborn).

when undine is 18, a knight, huldbrand von ringstetten, comes to the fisherman's cottage and falls in love with her. only once they are married does she tell him of her heritage. she tells him that if he is to reject her, to do it then and she will return to the crystal palaces of her father, "a soul-endowed woman". huldbrand does not reject her, but vows his never-ending love for her and takes her to live with him in his castle near the danube.

bertalda, who finally discovers her true identity, lives with undine and huldbrand. undine's water-spirit uncle, kuhleborn, will not let them live in peace. he haunts them by emerging from the fountain of the castle and taking human form. to prevent him from doing so, undine places an enchanted stone over the fountain to keep kuhleborn away. they live peacefully for a time.

however, when the three of them attempt to travel to vienna, bertalda drops her necklace, given to her by huldbrand, into the water. undine summons a beautiful red coral necklace out of the watery depths. huldbrand is enraged by this, realizing that his beloved wife is still connected to her water-spirit family. despite undine's warning that should he ever be angry with her that her family will recall her to her watery home, huldbrand expresses his anger and undine is taken back beneath the water where she lives in sorrow. as long as huldbrand is faithful to her, he can continue to draw breath. if he is not, he will die.

at first, huldbrand is grief-stricken and weeps and weeps for his beautiful undine. however, his grief for his wife fades and he eventually plans to wed bertalda, even though his love for undine remains true. bertalda agrees to marry him even though she knows he is still in love with undine. the night before their wedding, bertalda foolishly removes the stone from the fountain, allowing undine to rise from the water to fulfill her curse:

There was a gentle tap at his door. Undine used to tap thus when she wanted playfully to tease him "It is all fancy," said he to himself; "I must seek my
nuptial bed."

"So you must, but it must be a cold one!" he heard a tearful voice say from without, and then he saw in the mirror his door opening slowly--slowly--and the white figure entered, carefully closing it behind her. "They have opened the spring," said she softly, "and now I am here, and you must die."

He felt in his paralyzed heart that it could not be otherwise, but covering his eyes with his hands he said: "Do not make me mad with terror in my hour of death. If you wear a hideous face behind that veil, do not raise it, but take my life, and let me see you not."

"Alas!" replied the figure, "will you then not look upon me once more? I am as fair as when you wooed me on the promontory."

"Oh, if it were so!" sighed Huldbrand, "and if I might die in your fond embrace!"

"Most gladly, my loved one," said she; and throwing her veil back, her lovely face smiled forth divinely beautiful. Trembling with love and with the approach of death, she kissed him with a holy kiss; but not relaxing her hold she pressed him fervently to her, and as if she would weep away her soul. Tears rushed into the knight's eyes, and seemed to surge through his heaving breast, till at length his breathing ceased, and he fell softly back from the beautiful arms of Undine, upon the pillows of his couch--a corpse.

the story has been immortalized in many different forms, ballet, theater, animation (disney's "the little mermaid" is a veiled re-telling of the myth), painting and sculpture (the above painting, "Ondine", is by Paul Gaughin). time has changed the essence of the story, which, it turns out, is quite beautiful and tragic.

the boy slept soundly all night. after telling me their story, his mother slept soundly, as well. the reason they are in the PICU? he had phrenic nerve pacemakers placed. once they are turned on next month, controlled by a remote control box, the pacemakers will stimulate his phrenic nerves, which stimulate his diaphragm to move and, thus, to breathe. eventually, we will be able to remove his trach and he will be able to talk and laugh and breathe, even as he sleeps. amazing.