Friday, March 16, 2007


(UPDATE: Chef Boy's mom is home now and doing fine. They never did find anything obviously wrong with her; I'm thinking she had a TIA or was simply dehydrated. Thanks for your prayers and thoughts.)

There is such a thing in nursing as Having Seen Too Much of a particular syndrome, disease, or situation. I realized that I had Seen Too Much of Creutzfeld-Jakob disease (CJD) the other day when Footer started his report with "Well, he's a typical CJD-er" and I knew exactly what he meant.

CJD is *not* "mad-cow" disease. That's variant CJD, or vCJD to those who sling the lingo. Creutzfeld-Jakob, non-cow type, is a steadily and usually rapidly progressive neurological disorder that essentially turns your brain into mush. We're not sure what starts it off. If I were to oversimplify the cause and process, I'd say this:

There's a type of protein called a prion that lives in your brain. As long as this prion is happy, everybody's happy...but sometimes, the prion will flip over into its mirror-image (molecularly speaking) and become its own evil twin. This causes your brain to stop working in a particularly nasty way.

It starts with balance problems, memory loss, and speech difficulties. Within a few weeks to months, you're stuck in bed, with your arms and legs at unpleasant cattywampus angles and your hands all clenched. Your eyes don't track, you don't sleep, you can't eat, and you make weird meowing noises almost constantly. You can't control your movements or your bowels, nothing much medicinally helps your contractures or weird choreic motions, and eventually you die from pneumonia, since you can't clear the crap out of your lungs.

Given a choice of ways to die, I recommend not going with what's behind the door labelled "prion diseases".

How do we diagnose it? Definitively, through brain biopsy or on autopsy. The trouble with those is this: prions can be inactivated only with extremely expensive and difficult sterilization processes. Normal autoclaving won't cut it; you can't burn the suckers up. So any time we start messing around with the brain of somebody who might have a prion disease, we have to toss all the stuff that touches their neural tissue. Reason being, prion diseases can be transmitted by one person ingesting or otherwise ending up with the prions from the infected person in their bodies.

However, there are pretty-close methods we use. The most reliable is known as the Tired Old Nurse test: if the most-experienced nurse on the floor sighs and says, "Dammit" upon sight of the patient, it's a near-sure thing it's CJD. We also do EEGs; there are particular brain waves that are diagnostic along with other clinical signs. And, of course, there are the rule-outs that get done early: poisons, drugs, weird encephalopathies caused by viruses or bacteria. It's basically a What Every Young Neurologist Should Know disease, in that you learn about everything else that's not causing it.

Nursing care for the patient with CJD focuses mainly on safety (I've had patients dance themselves out of bed before or hang up in their own restraints), clearing the airway of secretions, keeping the skin intact, and keeping the patient as comfortable as possible. We do a lot of stuff that doesn't seem like normal nursing care at first glance: for instance, nails can be a problem.

When a patient is in decorticate posturing (scrunched up), their hands many times will end up around their necks or in their armpits. Long nails mean dozens of tiny infected wounds in tender skin, especially in the groin when legs contract. So we keep their nails really short and blunt.

Another thing we do is lots and lots of aggressive mouth care. People with CJD tend to clench their teeth hard enough to grind off pieces of enamel, so you try to suction and scrub those bits out of their mouths before they swallow 'em. That is, if they can unclench. I often thank Frog for missing teeth in a CJD-er, so I can slide a small suction cath into the space.

And then there's the whole eye thing. If you're not really sleeping or blinking, you tend to get corneal ulcers, so we irrigate the CJD-er's eyes and lubricate them with oily goo several times a day.

The most important part of the nursing care of the CJD patient, to my mind, involves the unaffected family members. They've watched the person they love go from being a happy, normal, laughing person to something that's not quite human, often in the space of a couple of weeks. I encourage them to talk to the person. I look at the pictures they bring in and admire the grandkids. Most of all, I do what I have to do to the patient (most of it unpleasant) as gently as possible, explaining what I'm doing all along.

After all, I don't know if some part of that person can still hear and understand me. I doubt it, but if it were me, I would want to know what was going on outside my head, even if I couldn't control any of it.


babe said...

how many of these patients do you see? in 35 years of neuro nursing i think i've taken care of about a dozen CJD patients. admittedly most of that time was in critical care and not at the floor. but still, i'm impressed in kinda a sick way.

Jo said...

Babe, I've seen (and here I had to go back and check with coworkers) probably ten or eleven in just under five years. We're a research hospital, though, so we tend to get all the GOKs from a zillion-mile radius.

But yeah, we have three right now--two on the floor and one in the unit.