Friday was another one of those days... like the one that prompted PICU Lesson #1, the one that really inspired the revival of this blog. 

On Thursday night before I went home, we got a call from a hospital on the west side of the state saying that they had a 4 year old with what they thought was Hemolytic Uremic Syndrome (HUS).  They could not provide dialysis to treat his kidney failure, so they wanted to transfer him to us for better care.  When I left, they were heading out to pick him up and bring him back, something around a 4 hour trip.  I would pick him up as my patient when I came in in the morning.

When I got in Friday morning around 6:45, I got right to looking up what had happened with him since he'd come in overnight.  Throughout the morning as we were "prerounding" on our patients (going around and examining all the patients, getting their vital signs, seeing what happened overnight), he had a couple episodes of worsening blood pressure and unstable heart rate.  By the time we'd finished prerounding and were ready to round (seeing all the patients AGAIN as a whole team and making decisions for the day), he'd been seen by infectious disease also, because we were very much concerned that what he had was NOT HUS, but Rocky Mountain Spotted Fever.

When I presented this patient to the team, this 4 year old little boy who'd been healthy the week before, this is what I had to say:

When he was sent from the outside hospital yesterday, he came already on two pressors, medications to keep his blood pressure high enough to get blood to his body.  He'd recieved many liters of fluid.  He was on Vancomycin, Rocephin, and Doxycycline, three different antibiotics.  When he arrived here, he was intubated without sedation.  His blood pressure was dangerously low and he was started on another pressor, making the total three.  We considered bypassing his heart and lungs and putting him on ECMO (extracorporeal membrane oxygenation), but he was too sick for even that.  His infection had spread throughout his body, causing his capillaries to leak fluid into his tissues and causing swelling all over his body.  It also caused bleeding, which showed itself with a purpuric rash all over his legs, arms, torso, back, and neck.  He was bleeding out of his nose, his endotrachial tube, and his bladder catheter.  He also had blood in his belly, chest, around his heart, and possible in his head.  His feet were white and cold with no pulses.  His right hand was black, tense, and necrotic.  His kidneys were failing.  His liver enzymes were elevated, evidence that his liver cells were dying and releasing toxins into his blood.  He'd received over 5 liters of IV fluids, 4 units of blood, 4 units of platelets, 4 units of fresh frozen plasma, and 2 units of cryoprecipitate, all in an effort to keep his blood volume up... to no avail.  There was not an inch of this child that looked normal.  He was the sickest human being I have ever seen. 

His disease, presumably Rocky Mountain Spotted, raged through his body causing disseminated intravascular coagulation, multisystem organ failure, and irreversible septic shock.  He did not have a single organ system that was unaffected.  They say that each failing organ system gives you a 20% chance of dying.  Kidney, liver, heart, lungs, skin, brain.  He had at least 6.  That's a 120% chance of dying.  Despite our efforts, which were swift, sincere, and vast, there was nothing we could do.  One of the neurologists came down and we performed an exam that revealed no brain activity.  At that point, there was nothing left to save.  His parents, who from the beginning said they did not want him to suffer, quickly said they wanted to withdraw care.  They cut a lock of his hair from his head.  A priest came and blessed him, anointed him with oil.  His mother held him.  His father couldn't bear to see his son, now bleeding and bloated, looking nothing like the child he'd raised and loved.  When we disconnected the ventillator, it only took two minutes for him to be gone.  He had notihng left to fight with. 

Our team was devastated by his death.  Not only because we couldn't save him, but because a week ago, he could have been saved.  He didn't have to die.  The week before, he'd gotten a fever and his mother had taken him to the ER.  They sent him home, calling it a virus.  The next day, his fever was higher, and his mother took him back.  They sent him home again.  Later in the day, he developed a rash on his feet and still had the fever.  His mother knew something was wrong, and took him back.  They gave him amoxicillin and sent him home.  Wednesday (two days before he died), he was still not feeling well, he still had the fever, and his mom sensed something was wrong.  She got a ride to another hospital, where they gave him a shot of antibiotic and admitted him to the hospital to investigate.  Within hours, he'd become lethargic, hypotensive, and very very sick.  They intubated him, gave him fluids, tried to stabilize him, and on Thursday decided they needed help.  You know the story from there.

Now, if you're from North Carolina, you've heard of Rocky Mountain Spotted Fever.  It's transmitted through tick bites, although frequently you never see the tick.  It presents with fever, headache, and a rash that usually starts on the feet and hands.  In North Carolina, if you see a patient with fever, rash, and headache, it's RMSF until proven otherwise, and you treat with Doxycycline.  If you treat within the first 4 days, virtually everyone is fine.  If you wait, there's no guarantee.  If untreated, RMSF kills about 20% of its victims.  This young boy did not receive treatment at the first hospital he was seen at.  I was not there and I don't know what they saw or what they were told.  I can't say for sure that they should have known to treat him for RMSF, but I know they didn't.  And if they had, he'd probably be alive today.  I'm sure the doctor who treated him knows that now.  And I'm sure, it's killing him inside that he didn't treat it.  But still, I can't help but think that he didn't have to die.

This will probably be one of those patients that changes my prescribing practices for the rest of my career.  I expect that it will do that for the other students, residents, and physicians who also treated this patient.  More kids that necessary might get doxycycline because of this boy.  But maybe, one will get treated who otherwise wouldn't have.