TILIR #26-30: Catch-up, part 1

Rapid-fire catch-up madness!!!

LastFridayIL: There's a 2-3 inch wide circular hole in the wall of the super secure room that houses the MRI machines that's there to allow for medications to be given to the patient

A bronchoscopy is a great way to visualize how well a jaw thrust can open up an airway. While you've already got a camera in the patient's throat, just pause a moment and pull their chin up and out. Voila! The larynx becomes twice as large. No wonder the patient can breath better when positioned properly.

Bardet-Biedl syndrome is a genetic disease that causes visual problems, kidney problems, obesity, infertility, and intellectual disability, amongst other problems. All of these widely varied effects result from a defect in the tiny finger-like cellular projections known as cilia.

LastMondayIL: ...some more basic things about manipulating IV tubing. It's amazing that no one teaches medical students some of the very basic aspects of the delivery medicine because nurses and medical assistants are nearly always the one's interacting with it. Sure, we've probably all placed an IV... once... a few years ago... but how do you flush it? How do you hook up the IV pump? How do you keep air out of the system as you hook up and disconnect various syringes.

TuesdayIL: Ketamine is a sedative that is not our first choice because it can cause kids to acts pretty crazy or aggressive when waking up. It also causes hypertension (high blood pressure), tachycardia (high heart rate), myoclonus (muscle twitching), and nystagmus (eye twitching). The blood pressure changes can actually be a good thing in patients that are hemodynamically unstable, especially since our go-to sedative, propofol, tends to cause low blood pressures, which can be very dangerous if a patient is in shock.

St. Jude's sucks at treating medulloblastoma.

WednesdayIL: CPVT stands for catecholamine-induced polymorphic ventricular tachycardia. It's an interesting if scary genetic condition where a person can be totally normal from a cardiac standpoint until they get excited or have some form of stress. Suddenly their heart starts beating so fast that it can't pump blood to the body effectively. Often people will faint as there suddenly isn't enough oxygen getting to the brain.

ThursdayIL: Delirium is much more common in ICU patients than people typically think. It comes in three varieties: hyperactive, hypoactive, and mixed. Hyperactive is what most people think of, where patients get confused, start yelling, forget things immediately, etc. But the hypoactive version can be easily confused with just sick or sedated. There is a big push to combat delirium by limiting medications known to be associated with the condition and re-establishing normal daily routines as soon as possible, even in ICU patients.

You can apparently continue sobbing while completely sedated. We had a very upset younger child that was not happy about getting the IV placed that allowed us to give her sedation medication. We took pains to make it not painful, but it doesn't matter if you're scared. When we finally just threw in the towel and went to put her to sleep, she continued to cry for nearly 5 minutes even after she was no longer conscious. T'was a little traumatic for the parents.

TILIR #24 & 25: Hearts and poisons

Wednesday morning marked a milestone for our program. It was the first multidisciplinary conference to feature a cardiac patient since we started taking care of some of the cardiac service at the beginning of the academic year.  I had actually presented a cardiac patient for one of my multi-disc conferences last year and it was noticeably difficult for our residents and even attendings to work through what was going on with that patient because we simply never saw that pathology on the services we managed. Now we are actually starting to interact with this important subset of pathology regularly and our program can only be better for it.

WednesdayIL: DiGeorge (or 22q11.2 deletion) syndrome is a genetic condition associated with numerous cardiac problems, but a particularly pathognomonic one (a symptom that is very characteristic of a certain condition) is an interrupted aortic arch. This is like an extreme form of a coarctation where the aorta not only narrows but pinches entirely off. This is obviously bad, but not immediately incompatible with life as the body will force blood through collateral smaller arteries which widen in caliber as much as they can to accommodate the extra blood flow.

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Thursday we finally got to do a well known pediatrics mainstay: baby formula taste test! A couple of the hospital's registered dieticians gave a brief lecture on the numerous different types of formulas and dietary supplements for babies and toddlers. They then lead us in groups to the adjacent room to try small samples of most kinds of formula. We of course had to run upstairs afterwards for an ice cream chaser.

ThursdayIL: Alimentum tastes like poison. This is one of the least natural formulas in that it is basically pre-digested and thus marketed as hypoallergenic (along with the other big brand version, called Nutramigen). Nearly any baby is able to tolerate this formula so it's kind of our last line of defense before things get much more difficult (IV nutrition, etc). However, these formulas are crazy expensive, very physiologically dissimilar from regular breast milk, and they tastes awful, even to babies. Seriously, though, tastes horrendous. Stay away.

TILIR #22 & 23: Gotta walk before you can... RUN! NOW!

Last Monday, I started a new block. I am trading in my collared shirt and khakis for some comfy scrubs and heading into the basement of the hospital to hang out with the sedation folks. Sedation is a weird place. It's a very pediatric specific concept where an entire department is created to provide a space for children to have minor procedures and time-intensive imaging. Most adults can handle a lumbar puncture with only local anesthetic or sitting through an MRI with, at most, a little anti-anxiety medication.

Kids, on the other hand often don't understand why they need a procedure, can't sit still enough for a scan, and have a very low pain tolerance. So most pediatric hospitals have a sedation department to help get kids through these little experiences that cause them big anxiety.

MondayIL: The Mallampati score is a way to estimate ease of intubation prior to a sedation procedure. Basically, a score of I says that you can see the back of the throat easily through the mouth. II and III indicate that you can see either most of or just the base of the uvula (the dangly thingy in the back of your throat) respectively. IV means the patient's mouth shape and tongue size make seeing the throat very difficult, and accordingly, they would be a more difficult intubation.

Another metric we use is the ASA (American Society of Anesthesiology) scale, which categorizes patients by how generally healthy or sick they are:

• ASA I - Healthy
• ASA II - Mild sytemic disease or major disease under good control (mild asthma, cancer in remission, etc.)
• ASA III - Major disease/illness, not controlled (influenza, cancer not in remission, etc.)
• ASA IV - Major disease/illness that is a constant threat to life (ICU patients)
• ASA V - Unstable patients that will die without the procedure
• ASA VI - Brain-dead patient awaiting organ donation

Well that escalated quickly, huh?

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On Tuesday I got to experience a bit of the sink-or-swim method of teaching. The physicians that administer monitored anesthesia care (MAC) (formerly known as conscious sedation) in my hospital are all pediatric intensivists (ICU doctors). As this level of sedation is by far the most common in the sedation unit, it's most of what I get to see. After one day observing various intensivists administer sedatives, generally in the form of Propofol +/- Fentanyl, I was getting somewhat comfortable with the procedure of "pushing meds," as they call it.

On day two, I was rushing out of one procedure and into another for which sedation was being provided by an attending I had yet to work with. I walk into the room, apologize for not being there at the start, and am immediately beckoned over to the head of the bed. 

"This is what, your second day?" asks the intensivist. 

"Yep!" I assured him. 

"And you've been pushing meds, right?" 

"Nope! Not yet!" I reply nervously. 

"Well, there's no better time to start!" he says without missing a beat, and hands me the syringe of death milk (it's white and opaque, much like milk, actually for the same reason as milk (it's a colloidal suspension)).

While this may seem horrifyingly reckless, it's a good bet that a nervous resident is going to err far on the side of pushing too little anesthesia than too much. And in fact, that first time, I didn't push a single milliliter of sedative without the attending telling me to.

TuesdayIL: If a patient's oxygen saturation starts plummeting during a procedure, there are several things that can be going on. First, check that the pulse ox sensor is actually still on their finger. Of course they'll have a low reading if the little red light isn't shining through their finger/toe/earlobe/etc.

But say it's real. The patient's oxygen saturation actually is plummeting. What's happening and what do you do? The two most likely answers to what's going on are that:

a) you pushed too much medication and now they aren't breathing, or
b) something irritated the middle part of the throat (larynx) and the patient is trying to breath, but can't because the airway is spasming closed.

So, what do you do? Actually the exact opposite thing. In the first case stop pushing sedatives. You also may need to put a mask on them and bag a few breaths until they start breathing on their own again.

In the second case, it's far easier. Push more sedative medication, and quickly. This will relax those spasming muscles and allow air to pass again.

TILIR #21: No more, fingers crossed

[A little behind, but I'm taking notes and will catch up over the next few days! Sleep is sometimes more important than blogging (gasp!)]

On Sunday I had to go into the hospital for the the first part of the last 24 hour newborn call shift my program will ever have. They recently changed up the distribution and responsibilities of senior pediatric residents at my program. We used to have a rotating call pool that had to takeover the newborn nursery service a few weeknights for a month at a time and then work at least one weekend 24hr call shift, all while on another rotation on weekdays. Now, one of the inpatient senior residents will always take call overnight.

Anyways, I had to go in at 7am to round on a handful of babies with the attendings who were working that day. If, from 7am to 7am the next day, there were any calls on any of the ~30 babies (a very below average number), I got the page and either took care of the problem or asked the on-call attending for help. Was actually a pretty mild day/night. My biggest gripe is of being notified way after I should have been about a problem. I can't fix a problem I don't know about and sometimes I can no longer fix problems that I hear about too late.

SundayIL: Syndactyly, or webbed and conjoined fingers and toes, is surprisingly common. It's often easy to fix at a very early age, which is why you don;t see many adults walking around with double thick fingers or flipper feet. But what may not be obvious is that conjoined fingers are not caused by fingers getting stuck together in utero, but rather because a part of our embryonic flippers fail to separate.

There are numerous inheritable forms of syndactyly and it is associated with many different genetic syndromes. Without other findings, however, it's not really worth it ot do a big genetic work-up on a baby with a few conjoined toes and fingers. Have orthopedics fix them (or not) and just let them go along with their lives.

TILIR #19-20: Tenacious D

Today we had a set of parents come in to their pediatrician with their newborn. We talked about vitamin D supplementation while breastfeeding, among many other things. They remember their older child taking the older version of this medication that came as a liquid with a small dropper to dispense the not-insignificant volume of medicine into the baby's mouth. It wasn't much fun because it tasted bad and babies tend to spit it out. But thankfully the newer preparations of this supplement are concentrated enough that you only need one drop.

So, we turn to mom and explain that the easiest way to give the medication is just put a drop on the breast before a feed. Simple!

Dad chimes in: "So you just spread it across the baby's chest like a VapoRub?"

Mom shoots him a looks that plainly says, "Are you an idiot?" But the other doctor and I laugh and explain that, no, the drop is placed on mom's breast so that the baby swallows it when breastfeeding.

ThursdayIL: There is a charter school near the airport in Grand Rapids called the Aviation Academy, where kids who want to be pilots (or just get a really STEM education) can go to high school instead of a normal public high school. Weird niche schools like this are touted as examples of how charter schools (the pet project of our Grand Rapids native Secretary of Education Betsy DeVos) can expand the model of public education.

However, I'm less impressed with the system when I see kids from other charter schools that are falling behind without repercussions thanks to loose regulations. And when resources that should be helping all public schools provide a great education are diverted to niche elite schools like the Aviation Academy, it makes me sad. Strangely enough you don't see many of the inner-city population that I tend to see in my clinic attending the Aviation Academy.

TodayIL: If you are testing an eye for HSV or chlamydia (as are concerns in a newborn with a red, swollen eye) you need to culture the eyelid not just some eye discharge, because both of these organisms are intracellular. There aren't any skin cells in tears or eye discharge, so you need to scrape some off the interior of the eyelid.

If a neonate does develop a suspected chlamydial infection of the eye, you should always treat it with systemic antibiotics, rather than with just eye drops. This is because the other major area where neonates can get chlamydia is in the lungs as pneumonia. If they were exposed in the eye, they were likely exposed in the lungs, so treat now regardless of respiratory symptoms and avoid the dangerous sequelae of a newborn with pneumonia.

TILIR #18: Ota, Ito, and blue milk

Blue flour-water
from Wikipedia

TIL: Nevus of Ota and nevus of Ito are two lesser know types of dermal melanocytosis, or gray-blue patches of skin. The more common form is the gray birthmark often found on the lower back or butt called congenital dermal melanocytosis, but more commonly known as Mongolian spots. The medical field is trying to shy away from this latter terminology... in case it offends Mongolians? It's not good or bad so not sure why it would offend. I think it might be more that the name is just arbitrary and inaccurate, with the condition having nothing to do with Mongolia or its people.

Anyways, nevi of Ota and Ito are gray-blue patched of skin in the distribution of the first/second branches of the trigeminal nerve (top half of the face) and the posterior supraclavicular and lateral brachiocutaneous nerves (neck, upper arm, upper back).

The Tyndall effect is what gives these lesions a bluish hue. This is the same effect that gives rise to blue eyes and turns milk with flour in it blue. In the milk, flour particles scatter light with the energetic blue light preferentially scattering over the red wavelengths.

In both eye color and the above skin lesions, the flour-like light-scattering particle is melanin, the dark pigment that colors skin. The bluish gray skin color with any of the dermal melanocytoses happens just like the blue milk, with melanin scattering the blue light and absorbing the red. Eye color is far more interesting. Irides with lots of melanin absorb almost all the light coming in because any blue scattered light from all but the surface-most layers of the iris just get caught by the melanin in front of it when trying to bounce out. These eyes tend to look dark brown.

Iris color diagram (made by me!)

The less melanin in the iris, the more blue light is able to escape back out of the eye after scattering. Bright blue eyes have the perfect goldilocks amount of melanin to scatter light but not get in its own way. Greens and hazels are somewhere in between. Albino persons have no melanin in their iris and so neither absorb nor scatter light coming in. The iris is basically see-through so the red of the retina behind can shine through giving the appearance of red eyes.

TILIR #17: Balto and diphtheria serum

One of the attendings today was opining about how flippant parents have gotten with vaccines now that most of the diseases that vaccines prevent are rarely if ever seen anymore. However, she has been practicing so long that she can pull stories from her own experience of most of the diseases. She's seen many kids die of the various types of meningitis that are basically nonexistent in the US. She's seen measles, mumps, rubella. Rotavirus used to be everywhere. The list goes on and on. However, she does admit that there are a few diseases that were gone even before her time. Polio is the first, but thanks to FDR and the countless other survivors, everyone still remembers the horrors of that disease.

The statue of Balto in Central Park, NYC

Diptheria is the second. But, unlike polio, people don't remember this nasty respiratory illness. So the example she used is actually the story of Balto, the Iditarod husky. The story was popularized (and heavily fictionalized) during my childhood by the animated movie named after the real dog.

The real tale is of the famous passage of a series of dog sled teams along the harrowing Iditarod trail to deliver diphtheria serum to Nome, Alaska during an major outbreak of the disease with a blizzard blocking any other transport by air, train, or sea. Diphtheria toxin consists of antibodies against the bacterium, and was the only treatment at the time.

Nowadays no one training in medicine in the US has even seen a case of diphtheria thanks to vaccination. There were 5 reported cases in the US in the past 10 years (compared to over 1700 cases of the measles and well over 100,000,000 cases of the flu).