TILIR #9-10: Feast and famine

Some days is truly difficult to come up with a single concrete thing that I've learned. I'm obviously still learning how to be better at my job, how to be more efficient, how to play nice with others, etc. but rarely can those intangibles be made solid enough to write about. That was Thursday; I had to scrape together a tidbit about a rare disease that I just don't know as well as I should.

Friday I was working with a different doctor and our patient mix just happened to lend itself to a veritable deluge of teaching moments. Buckle up! I promise most of them are pretty interesting!

ThIL: Ehlers-Danlos syndrome is a condition marked by too much elasticity - of the skin, of the joints, and (here's where we get into trouble) of the walls of some blood vessels. There are currently 13 subtypes of this syndrome, varying by different presentations. For instance, there are some subtypes that also have eye problems (known as "brittle cornea syndrome"), and others that have bony changes including bowed legs and scoliosis. Unsurprisingly, there are many different genetic mutations that can cause this condition.

FIL:

• Grand Rapids was where the first pertussis (whooping cough) vaccine was developed. In the early 1930s, two scientists by the name of Pearl Kendrick and Grace Elderington did basic research to help learn about the disease and then developed a vaccine. Most vaccine testing at that time was done on orphan or institutionalized children because it was easier than convincing parents to test a vaccine on their children. But Kendrick and Elderington built a coalition of health department officials, doctors, schools, and parents in Kent County to trial the vaccine on a diverse group of kids from throughout the community. These two women should be as well know as Salk who developed the polio vaccine, but they always refused publicity despite saving untold hundreds of thousands of lives.
  
• There is a newly approved treatment for hyperhidrosis (sweating a lot (like a lot, a lot (like your-hands-are-always-dripping-and-your-shirt-is-never-dry a lot)). It's a topical anticholinergic, which is great because you get all the benefits of the systemic drug with far fewer side-effects. Up until now, we basically had intense powered antiperspirant or an invasive neurosurgical procedure to cut the nerve chain that controls sweating.
  
• A halo nevus (aka leukoderma acquisitum centrifugum 'cause dermatologists are horrible) is a mole that the body eats from the outside resulting in a progressively shrinking dark spot with an inwardly thickening ring of light skin. The mole disappears pretty quickly and then the white area gradually fades. It's nice to have an immune system that will attack moles, however it comes with a risk. These same antibodies that attack the pigment cells of the mole can sometimes go crazy and attack all the pigment in all of the skin, leading to vitiligo where large patches of skin will turn stark white.

  

  A rough diagram of the progression of a halo nevus

• Singulair (montelukast) is a common asthma medication of the leukotriene inhibitor class. It actually has a competitor that isn't used very often called Accolate (zafirlukast). I had never even heard of it. It's probably less well known because it must be given twice a day rather than one a day, and it is significantly more expensive (not that Singulair is all that cheap anyway).
  
• One "small" section of the large ethnically Dutch population in West Michigan is the Frisians. They originate from the Friesland province in the Netherlands, which is the only province to have it's own language and is historically known for the exceptional height of its population. This does throw a kink in using US national averages for growth charts during well-child visits. Telling someone he is at the 60th percentile in height nationally is awfully misleading, since in certain high schools he is likely one of the shorter guys in his grade.
  
• Paroxysmal kinesigenic dyskinesia is a very rare neurological disorder that actually describes itself fairly frankly (for medicine). Symptoms consist of occasional weird movements caused by quick, voluntary movements. For example, jumping out of bed can trigger dance-like movements of the legs. There are some antiepileptics medications that can lessen the frequency of episodes but eventually, most people just grow out of it over time.
  
• And then finally we have something that, confusingly, I learned through fact-checking for this blog. Or rather, I learned that I already knew something and that my attending was trying to teach me something incorrectly. Prolonged use of a pacifier, continued bottle-feeding, and thumb-sucking do indeed have long term effects on orthodontia. Word to the wise: if someone tries to teach you something by prefacing with "just think about it..." instead of "studies have shown...," be dubius.

TILIR #8: The Clinic Strikes Back

Today was my first day of clinic since becoming a second-year resident. A lot changes from the first week of first year to the first week of second year in regards to our clinic workload. Initially we don't have much idea what we're doing, both in terms of general logistics, navigating the electronic medical record, and - ya know - practicing medicine. It is, after all, a somewhat shocking experience to suddenly be the primary care provider for children.

We start out with only one patient for an entire morning or afternoon shift in the clinic. By the second week, we are up to two patients and it stays there for a couple weeks. After that for morning clinic we see three patients and afternoon clinic we can see up to four. As second year however, we have clinic all day long, and we also increase the number of patients so we can see up to four in the morning and 6 in the afternoon. All of this means we have to be more efficient with our time or we end up having a lot of extra work at the end of the day to catch up on.

One of the more fun things that I got to experience for the first time today was working with the new interns (the first-year residents). Since I've been on an outpatient rotation the rest of the month, I haven't actually gotten to work with any of them since seeing them throughout their orientation. It was nice little dose of schadenfreude seeing these scared new doctors figure out the ropes. As we helped them throughout the day, it was a pleasant reminder how much my classmates and I have grown in the past year.

TIL: There is an old psychiatric diagnosis called the Cotard delusion or "Walking Corpse Syndrome." It is no longer a official diagnosis in the DSM-5 (the official diagnostic manual of psychiatry), but I stumbled across an old ICD-9 code in the computer for this diagnosis and I just had to look it up. Seems the vague idea used to be that this particular delusion was that the patient believed that their some or all of their body was dead or decaying. Descriptions are rather vague though, and with such a broad grouping of delusions it makes sense that they were just lumped into other somatic delusions.

TILIR #7: Got an itch

I feel bad having been assigned to my current rotation site. It is a wonderful outpatient pediatrics office. Stellar doctors, great staff, lovely patients and families. About half of my program wants to go into general pediatrics and would kill to rotate here. It's the sort of place that you would dream of someday working at. 

I am so bored! Today I was working with my favorite attending, who is 30 years my senior and yet I feel like we could be best friends. But every patient we saw was either totally healthy or had super non-specific symptoms that we had to basically say we'd wait and watch. I want to see sick kids!

Don't get me wrong; it is a very relaxed rotation. I get to work around 9, get over an hour for lunch and am done by around 5:15. But I know many residents in my shoes would come in early to see more patients, or rush to try out the electronic medical record, or see patients on their own. I like hanging out with most of these attendings, but mostly just want to be done with this rotation. The grass is always greener, I suppose.

TIL: Sand fleas are tiny crustaceans than can cause small itchy bites on the skin. Rarely, certain species will bite the skin and then burrow into it to give their eggs time to mature and hatch. Seriously! There isn't really anything to do from a medical perspective but wait and treat the itchiness.

The beach and TILIR #6: Putting a name to it

Yesterday, Jenni and I jumped in to the typical Michigan activity of... going to the beach! As a California native and former bicoastal resident, I never associated Michigan with sunny, sandy beaches. Snow: yes. Cars: sure. Beaches: not even in my top 10 beachy states.

The path to our secret little beach
on the coast of Lake Michigan

But it turns Michigan with it's two enormous peninsular halves, is chalk full of beautiful beaches. Bordering four of the five great lakes will do that. And while it is pretty chilly and snowy in the winter, it's fiercely hot and humid in the summer.

The waves aren't much to write home about, but the water isn't salty, the sand is clean (due to the dearth of both plant life and petroleum wells), and the water level stays the same (no tides). There are giant state beaches that thousands of Michiganders flock to each weekend, as well as tiny off-the-beaten-trail beaches that are quieter and more intimate. Yesterday, Jenni and I ventured to one of the latter and had a wonderfully peaceful time.

TIL: Pleonasm! You ever know that feeling that there must be a word for a thing but you neither know the word nor how to describe it well enough to find the word?  Well a couple months ago an attending and I were (apparently) trying to think of "the use of more words or parts of words than are necessary or sufficient for clear expression: for example black darkness or burning fire."¹ The best we could think of was tautology, which does have one similar definition, but I mostly hear tautology used in the context of logic, which is a subtly but distinctly different concept.

And, just to make sure a dab of medicine ends up in this post: I also learned that we don't have a great idea how many kids "grow out" of ADHD. Somewhere 15 and 50% of ADHD patients no longer qualify for that diagnosis by their mid 20s. However a significant portion of these young adults are able to be reclassified as having other behavioral concerns such as an autism spectrum disorder or anxiety.

TILIR #4-5: On call

Oof. That was painful. So painful it took two days to recover enough to post this.

Last night I took call for the first time in my medical career. There's some confusing nomenclature here. When we cross-cover and work in the hospital over a weekend, we sometime refer to those shifts as call shifts. If we are working nights, then we might get Monday off because we are "post-call."

But what I did last night was more of what the average person thinks of as being on call. I was at home. I had a phone. If anything happened in the hospital's newborn nursery and required a doctor, they called me. If I can handle it from home, I can give advice or put in orders from my computer. However, if a baby were sick enough, I would have to throw on some scrubs and drive in.

As is often the case in medicine, this could result in wildly different nights for me. Either I get a couple of calls but otherwise a pretty normal night's sleep, or I'm basically working a full night shift between two normal day shifts. There is a rule in place that if you have a rough night, you can ask an attending physician to excuse you from your morning clinical activities to get some sleep. But we try not to use that excuse unless we really have to.

Last night I got 7 different calls, plus two other times I got up on my own to check on a lab result. I  slept maybe 5 hours but broken up so that the longest stretch was a little over an hour. I was so tired today that, for the first time since my surgery rotation in med school, I briefly fell asleep standing up. 

It's neat that I have advanced enough to be trusted with this responsibility. But if I'm going to stay up all night, I think I'd rather it just be in the hospital working. Thankfully, most of my top choices for careers after residency, do not include home call.

YIL: There is no current treatment for RSV infections other than supportive care, but there used to be. Ribavirin is an antiviral chair used to treat numerous viral diseases including hepatitis C, various viral hemorrhagic fevers, and, until recently, RSV. Studies done on the drug showed that it was not super effective at decreasing symptoms or hospital stays due to RSV, it was crazy expensive, and there was some concern about occupational exposure to nurses, with this drug having some association with birth defects in pregnant women. The big factor however, seemed to be the extreme cost for little benefit. It is still FDA approved, but only rarely used for immunocompromised kids with known exposures to RSV.

TIL: Adenovirus is the main virus being researched for use in general therapy. It was picked because it is one of the few viruses that targets multiple, disparate organ systems. Unlike for example RSV which targets the lungs and airways, or rotavirus which goes after the GI tract, adenovirus hits both those major systems plus smaller ones like the eyes. The biggest hiccup in this plan is that it has proven really hard to separate the infectiousness of the virus (which we want) from the pathogenicity (which we don't).

West Michigan Fourth of July

The Fourth of July in Michigan is weird. Well, it's probably totally normal to everyone around here, but to a fella that hails from both coasts, this is all wrong.

First of all, fireworks are legal here. Neither of my previous home states had legal fireworks. Of course people smuggled them in pretty easily from neighboring states but there was an exciting naughtiness to seeing any elicit fireworks go off in your neighborhood. I once played with a sparkler that was smuggled into California from Arizona. It was thrilling!

In Michigan, not only are fireworks legal, they are ubiquitous. There is a pop-up tent in every parking lot selling arsenals of near-professional grade pyrotechnics. Around here, you are only supposed to set off fireworks in the few days before or after a relevant holiday. You might hear a couple distant pops and crackles in the week leading up to the Fourth, but once the sanctioned timespan begins, there are multiple large fireworks going off every second in every direction for a good three hours per night, with aftershocks of drunken shenanigans through 3am.

Last year, Jenni and I were living out of a third floor room in an extended-stay hotel during the Fourth so we had a great view of the dozens of private little fireworks shows going off at any given time stretching out to the horizon.

But the more annoying difference with fireworks here, compared to other places I've lived, is the time it gets dark. West Michigan is way over on the extreme western end of the eastern time zone. Between that and our modestly northern latitude it means that in early July, sunset isn't until 9:30 and it isn't dark until about 10:30. When you work early the following day, how can you stay up for that?! Little kids stand no chance of staying up for a fireworks show.  And if you do decide to just go to sleep, you're bound to be woken up repeatedly when your neighbors decide to fire off their own private artillery barrage 20 feet from your bedroom window.

I love fireworks, but I long for the relatively subdued and controlled celebrations of some of the other, more sensible places I've lived. Ah well, here's to the wild freedoms of 'Murica.

TILIR #3: Mentors

Through the course of my medical career, I've met and worked with many very talented and  accomplished doctors. The old adage of "those who can't do, teach," does not generally hold up in medicine. In pediatrics, mediocre physicians can plod along just fine in an urgent care or private office, but if you're teaching medical students, residents and/or fellows, you need to be on top of your game. We question everything and have the entirety of medical literature at our fingertips via our phones and computers.

What we are really hoping to glean from our mentors is the wisdom and experience that is more difficult to codify into guidelines and algorithms. Some of our younger attendings seem to know every relevant paper published in the past 5 years in pediatrics, no matter how obscure. But some of the older attendings can reliably call that a seemingly stable patient will be in the ICU by morning just by glancing at them. The former talent we can learn from reading books and going through practice questions, but the latter just takes time and purposeful observation.

Today I got to work with another one of those mentors that simultaneously make me proud to be in this profession, afraid that I'll never be good enough, and hopeful that someday I might be. It's remarkable how much has changed since her generation of physicians were in my position. The hospital here in Grand Rapids only had a couple pediatric floors, the residency only had a quarter the number of residents, the residents drew their own patient's blood, there were no intensivists or neonatologists on call overnight, and they saw countless children hospitalized or dead from illnesses that we never even see today thanks to vaccines. I often wonder what medicine will be like 30 years down the road when I have been practicing as long as she has.

TIL: Stickler syndrome is one of the broader constellations of symptoms that includes the Pierre Robin sequence. Pierre Robin (pronounced "Pee-air Row-ban"), consists of a small, receded jaw, protruding tongue, and cleft palate. It is non-specific and can have numerous causes and several distinct associations. With Stickler syndrome, the Pierre Robin findings are joined by hearing loss, arthritis, severe myopia, and retinal detachment.