Steampunk

The boy sits on the edge of his bed.  A metal frame surrounds his chest, plastic tubing covering him in tendrils of oscillating white.  A mask covers his face, flushing him with medicine laced steam.  The device rumbles and jerks, steam pulsing out of its joints with each movement.  He watches Law and Order quietly. 

He had come in with complications of cystic fibrosis, a disease that boiled down to a few faulty sodium transporters, most often a single faulty digit of genetic code.  Without this sodium transporter, his cells don't push water into mucus, creating a thickened, nasty product that makes the normal kind seem delightful. He cannot get rid of this mucus.  It accumulates, filling his lungs with a rising tide of congestion.   It serves as a platform for bacterial colonization, the thick substrate impeding his immune system from properly fighting back.  It clogs his intestines, it destroys his pancreas, his kidneys, whose ducts are as dependent on mucus as his nostrils.  It stunts his growth, ramping up his metabolism hugely with the physiologic effort of clearing mucus.  It will kill him, on average somewhere in his twenties or thirties.   He is 17.   His sister died of the disease at 18.  He is terrified.  

The device torturing him was a CF vest, a vibrating, oscillating, beating of a treatment, designed to free up mucus in the lungs, letting the child cough up some of the phlegm that was drowning him.  This contraption of steam and metal had him for hours each day, a shuddering, clanking mechanical replacement for a process his body could not do.   We doused his circulation with antibiotics, we gave him sprays of albuterol, steroids, and toxins designed to help fight his chronic infections.  The volume of his medication was so high we had cut a port into him, a ready made venous access normally meant for chemotherapy.   

He plays football for his high school.  He has a girlfriend. He wants to go to college, to med school, but thinks he will become a rad tech because it takes less time.  He doesn't feel like he has that much time.  I can't argue.  But he has a life, even inside the abusive steampunk hug of his vest.    

Nosebleed

I don't even touch his nose before it starts gushing.  I'd looked in his ears, an invasion that any four year old knows is worthy of the most potent vocal rage he can summon.  His tearful howl literally burst a vessel in his nostril, the sudden surge of pressure and agitation proving too much for a vessel that had assuredly been picked clean by his questing tiny fingers.  

The blood pours out in gushes that seem literally unimaginable for a child this tiny.  It sprays onto the ground in sartorial geysers, staining his pale freckled face with ghoulish warpaint. He stops bawling for a second, confused momentarily by this painless wet.  The blood slows down, the decreased pressure drawing the flow back to a trickle.  But he knows blood is bad. So he does the worst thing, he bawls more, ramping up the gusher in his sinuses once more.  Blood is pooling on the floor, a sticky mess of coagulation and snot running through corrugations in the tile.  

I sit there momentarily frozen, astonished by the sheer volume, stunned by the ultrasonic scream of a confused (but not wounded) child.  He grabs his mother, spreading gore across her shirt.  I hand her a paper towel for his nose, and think about what to do.  Tilting your head back is wrong, they say.  You just swallow blood, it doesn't actually stop the bleed.   I'd been told you use a tampon to stop nosebleeds.  I ask the nurses, but none of the available feminine hygiene products will fit up his nasal passage.   I tell her to tilt his head forwards, to put pressure on the outside of the nose.  I tell her to hold this position for ten minutes.

Those ten minutes are long for me.  He had come in for frequent nosebleeds.  Nosebleeds in kids can be nothing. But not always.   My mind stutters out fears like acute lymphoblastic leukemia, a possible death sentence for this friendly, albeit vocal, little man.   The bleed has to stop in 10 minutes.  Longer, and it begins to get worrying.  Shorter, and we can chalk it up to a youthful obsession with boogers.   A terrible fantasy sequence rolls out in my head, the guilt of possibly diagnosing this child with leukemia crushing, the idea of this mother losing her boy...but no, its just a nosebleed.  Its almost certainly just a nosebleed.  Right?

I look at the clock, begging for the right answer.    

Pseudo

The baby boy had horns. Two big round lumps growing from his brow, two cephalohematomas, bruises of the soft infantile skull that shrank with each post-natal day. His horns, a birth accident, but not one of any consequence, seemingly marked him from birth as a troublemaker. And in a way, he was trouble. Not that he behaved poorly, quite the opposite in fact. He was, to put it mildly, quite adorable. His round eyes took in the room with that innocence that rarely seems to last even weeks, and he rarely fussed. He took everything in stride, from the hospital crib he lived in to the tubes we fed him with. Even the two loops of his small bowel protruding from his side in a man-made point of access couldn't perturb this little guys day.

He'd been here for a month. He'd been on Earth for two. He was born happy and healthy, but with a distressing tendency to vomit. This isn't unheard of in babies, so his pediatricians weren't too worried. Not, at least, until each succesive test kept coming back negative. It wasn't the usual things, the pyloric stenosis, the duodenal atresia. There were no visible obstructions in his intestines, no problem we could point to, nothing to say "here. this is what we need to do." They pulled the ostomy (the loops of intestines) out to try to correct a problem, try to further diagnose. They ran test after test, all the while feeding him a mix of fats, sugars, proteins and salt through his minature veins. But every time they tried to feed him, every time they tried to get him back to normal, he threw up.

They took a sample of his intestines. Perhaps its an extremely early onset inflammatory bowel disease. Perhaps he has a strange infection. Either of these we could work with, work on. Instead, they found only an absence: he didn't have any nerves. You might think this wouldn't be a problem, but you need nerves in your intestines. They drive things forward, onward and downward, from stomach to the porcelain throne. Pseudo-obstruction, we said with a grimace, the word Pseudo belying the severity of the problem. Almost-obstruction, not-quite-obstruction, seems like it wouldnt be as bad as the real deal right?

But a blockage we can fix, a blockage we can clean out, set right. Even dead intestine can be removed and tied back together, leaving a shorter but functional system behind. He had no nerves, anywhere. His intestines would never work.

He lay there in his crib, wiggling happily in my powerless care. His parents asked question after question, hoping and praying for options, for prognosis. We tell them he will survive on TPN (IV feeding) for many years until his liver gives out. We tell them that Pittsburgh does a total small bowel transplant. We tell them that the outcomes have been improving. We mention, but do not focus on, the fact that the improvement is from 100% mortality to a 3-5 year life span post-op. And each day I check up on my newly alive and slowly dying patient, and each day he looks at me like I am something new and marvelous.