Best. Decision. Ever.

Where I used to work:













Where I work now:

Employee appreciation day

It was Caribbean-themed employee appreciation day at the hospital today, complete with activities, free food, and dancing. We started by donning flowered necklaces (I wore mine as an armband), moved on to decorating flip flops, ate some fried plantains and mango popsicles and the daring among us got spray-on tattoos. The award definitely goes to Alana, for tattooing Gray's name on her leg - fabulous.

Clinic redux

Last Friday I shadowed an outpatient neurology clinic as I do once or twice a month; here are the highlights.

1. Childhood narcolepsy. This is diagnosed using a sleep latency test; abnormally quick decent into slow wave sleep indicated narcolepsy. Unfortunately, the normative data for children doesn't exist so it can be hard to definitively diagnose. An EEG is also a good idea to rule out seizures.

2. Autism. I've never seen autism to this degree before; the appointment was because of an increase in obstinate behavior. The child was barking intermittently and would lunge for any paper he saw (to eat it). He was pulling the threads out of his sweater and eating those too. The parents looked haggard and completely worn out; dad would jump at the slightest noise. Three clinicians saw the boy together to determine whether antipsychotics should be started or whether the current medications should simply have their doses tweaked.

3. Refractory status epilepticus. An adopted child who has failed five different kinds of anticonvulsants. In the last two weeks there have been no seizures, but the week prior there were two: 75 and 90 minutes in duration. An MRI and overnight EEG are on the table as the first step towards consideration of neurosurgery.

4. Cerebral palsy. Not usually something we see unless it's part of a larger issue, which, in this case, it was. We just don't know what the larger issue is. The part I want to bring up is that the leg muscles can get tight and force the knees to turn inwards. This realigns the hips; if left uncorrected, the hips can become painfully and permanently displaced.

5. Absence seizures with syncope. I have now met two children with this seizure type: they lose consciousness for the duration of their seizure. Invariably they are worked up by cardiology first and when they don't find anything, they send them to us and we hook them up to an EEG.

Neonatal pre-op MRIs

One of the research studies I am involved in at the hospital includes performing an MRI immediately prior to the heart repair surgery of TGA and HLHS babies*. The baby we studied yesterday was oversized (due to mom's diabetes), but otherwise stable. The MRI revealed PVL,** which we have seen in 20% of patients prior to their surgery, and also a temporal lobe hemorrhage. We immediately called the surgeon to the MRI suite and a discussion ensued: what is the bigger danger - delaying the heart repair or potentially enlarging the bleed (the surgery would involved heparin, a blood thinner)?

We decided to delay the surgery and re-image the child in a few days to re-evaluate.

Sitting in on that discussion reminded me why I want to be a doctor. I've been losing the faith a bit with all the paperwork that is my job recently and this year long illogical waiting game of frustration that is applying. It was nice to have a reminder; hopefully I will get another one this Friday in clinic.

- - -
*TGA = Transposition of the great arteries. The aorta is connected to the right ventricle and the pulmonary artery is connected to the left ventricle, creating two independent circulations.
HLHS = Hypoplastic left heart syndrome. The left ventricle fails to form and the aorta is small and insufficient. Once the ductus closes, there is no systemic circulation.
**PVL = Periventricular leukomalacia, a white matter injury that occurs in pre-mature babies and is thought to be associated with mild cognitive problems.

Bummer

I had a little abstract prepared to submit to the Pediatric Academic Societies annual meeting (in Hawaii). It's customary to run these things by the Director(s) of the department(s) relevant to the abstract. Well, one of them killed my abstract. He won't let me submit it. Boo. And not because it's bad science, but because he's afraid we'll lose referral business.

I deserve a cookie.

A moment of victorious immodesty: I just cranked out a 115 page IRB protocol for constraint-induced movement therapy in 4.5 days.


update: It was pushed to the October 3rd review. : (

My very own brain

This, to the left, is my brain. As in, the one in my head - the one I use every second of every day. Cool.

For those of you with no neuro background, let's name some structures.

1 Cerebellum
2 Medulla oblongata
3 Pons
4 Pituitary (right above the point of the 4)
5 Cortex
6 Splenium
7 Fornix
8 Septum pellucidum
9 Corpus callosum
10 Meninges: dura, arachnoid, pia

For the record, I have a normal brain for someone my age. The funny dark patch on the top of my head is cerebral spinal fluid (CSF), which is visible because the interhemispheric fissure was not fully perpendicular.

Interventional Radiology

I shadowed a third year fellow in the cardiac cath lab today, watching two interventional procedures. The first was the expansion of a stenotic bicuspid aortic valve and the second was the closure of an atrial septal defect (ASD).

Note: The cath lab pictured is at Columbus Children's, not where I work. They look the same, though.

During the first case it took almost two hours to get access - meaning to establish a catheter in a femoral artery and femoral vein. In this case it was important to have both because we wanted to measure the blood pressure in the left ventricle and in the aorta. Ideally there is no difference; in our patient there was a 100 mm Hg gradient. This indicates that the aortic valve is very stenotic (narrow) - a condition that eventually requires a valve replacement. Valve replacements in children are to be avoided so there are two ways to buy some time: 1) expand the valve with a balloon in a catheter procedure or 2) open the chest and scrape the valve in surgery. The procedures carry approximately the same rate of complication, but each carries a different complication. Surgery generally leaves residual stenosis and interventional radiology tends to produce aortic insufficiency (backwards flow through the valve from the aorta into the ventricle).

The second case involved using a yo-yo looking instrument to plug a hole in the septum separating the two atria. If you look closely at the picture to the right you can see it in the top, just right of center. Once the catheter enters the heart, it is threaded through the hole (technically called a patent forman ovale, patent indicating open) and the first half of the yo-yo is deployed. The catheter is retracted through the hole and the second half of the yo-yo is deployed. It's very important that the placement is correct because otherwise it could loosen and go bumping around the heart or even enter the systemic circulation (depending on the size and type of closure device used).

Interestingly, you cannot actually see the outlines of the heart when doing a cath procedure. All the monitors carry continuous x-ray images and if you've seen an x-ray before you know that it's nearly impossible to see tissue with any resolution. That's how well these guys know the heart. They can tell by the ribs and chest cavity around it exactly where they are inside it. When appropriate, they will use a simultaneous ultrasound though (on which you can see tissue and blood flow).

At the end of the day, I'm not sure this is my new specialty of choice. It was incredibly cool to see and the people who work in the group were really fun (the atmosphere resembled a sports team pre and post game), but the patients are sedated the whole time you are with them and man, those lead aprons/vests/thyroid glands are heavy and hot!

Monthly clinic

On Friday I shadowed the outpatient neurology clinic, as I do once a month, and observed a days worth of follow-up visits. Most clinic days seem to show a theme - more likely because something registers in my subconscious than any trick of patient scheduling. Considering my recent review of genetics, it's perhaps not overly mysterious that this month impressed upon me the phenotypic markers that can signal an underlying neurologic process.

Most of you are probably familiar with some of the more famous neurologic phenotypes. For example, the Down's syndrome features of a single transverse palmar crease (simian crease), epicanthic eyelid fold, flattened nasal bridge, shortened limbs, proruding tongue, and white spots on the iris (Brushfield spots). The genetic correlate of Down's is whole or partial trisomy 21.

There are, in fact, a large number of genetic or neurologic processes that can be identified by phenotypic markers. An non-genetic example would be a perinatal (near time of birth) stroke patient. His stroke included part of the internal capsule and thus affected the neuronal tract that includes motor neurons. His stroke was right-sided, so one would expect left-sided motor affects. Indeed, because the stroke was not immediately noticed during his infancy (most infants do not get MRIs) his left side failed to develop properly. His left arm is shorter than his right (atrophy) and displays abnormal tone and fine motor control. In this case, the morphologic presentation of a shortened arm with abnormal tone allows a neurologist to begin localising his stoke before ever seeing an image.

A note about fine motor control - I get a lot of questions about this. Gross motor contol is large movements: walking, throwing, and lifting. Fine motor is things like tieing shoes, buttoning buttons, and writing. One test you might see a neurologist perform is asking a patient to touch their first finger to their thumb, then their second, third, and fourth. Another is to tap their first finger and thumb together as rapidly as possible.

Regarding motor development in infants... like anything else it's hardest to assess in younger children. Babies have little to no purposeful motor control so assessment usually consists of examining involuntery movement for abnormal posturing, tone or lack of movement. Mild deficits can be difficult to note until a child fails to develop on a normal timeline (ie, cannot stand independently or sit-up on time).

And now a genetic example. I should mention that this patient has not yet been conclusively diagnosed, we simply added Wolf-Hirschhorn to the differenital because of the phenotype. There were two children who came into clinic after having been neglected by their biological parents. It was impossible to tell whether they were delayed due to lack of input or whether there was genuine neurologic impairment of some kind. The children were placed in foster care and came back speaking 3 word sentences (they are 3 and 4 yrs old), but still underweight and incapable of tasks such as putting on clothing or drinking from normal cups. The younger child displayed tremors (attributed to more than being nervous because it affected her head as well) and the older child has "greek-helmet head", microcephaly (small head), and shortened stature. Greek helmet head is characterised by a high hairline and a broad, flat nose.

If the older child does have Wolf-Hirschhorn (deletion on chromosome 4) he's both lucky and unlucky. Unlucky because it's associated with fairly profound mental retardation, and lucky because his phenotype is (apparently) relatively mild. More extreme symptoms can include cardiac septal defects, poor development of secondary sex characteristics (genitals), renal (kidney) malformation, malrotation of the intenstines, and hand/foot contractures.

One of the important notes about these phenotypic varients is that in order to dianose anything they generally occur in clusters. For example, just having wide-set eyes or a high hairline doesn't mean you have a genetic defect or a neurologic symdrome. Cleft palate is a midline closure defect - a characteristic of Wolf-Hirschhorn syndrome. However, far more babies are born with cleft palate than Wolf-Hirschhorn: about 1 in 600-800 vs. 1 in 50,000.

Not-so-dead language

This morning I was told that I would have to be at work at 6:15am tomorrow morning in order to do the research part of a pre-op research MRI.

This generally involves preparing the MRI suite by stocking the bed with hot packs, warm blankets, assorted pressure cuffs and blood gas syringes and programming the monitors for a neonate (baby less than 28 days old). Then, if all goes well (it's about 7am), the baby is brought down from the NICU (neonatal intensive care unit) to the scanner by the anesthesiologist who will participate in the baby's impending heart surgery and the baby is placed in the scanner for an hour. I spend most of this hour recording vitals, sedation doses, running blood gas samples and results slips back and forth to the cath lab (catheterisation lab) and, of course, eating donuts. Then the baby goes off to surgery and I go back to my usual job (it's now about 8am).

Later this afternoon, however, the scan was called off because we couldn't get consent from the parents. They were not unwilling, so far as we could tell, but unfortunately no one speaks their primary language and I honestly haven't the faintest clue where to get ahold of a translator that speaks Aramaic. Yes, that's right, Aramaic. As in, lanuage commonly believe to be the one Jesus used and is generally thought to be less prevalent than Latin. I mean, how many high school Aramaic clubs have you heard of?

In the few hours that I was seriously pursuing the idea of getting a translation made of our consent form, I learned a little about the Aramaic language. It is, rather logically, an Afro-Asianic, Semetic language whose status is not actually "dead" but "endagered". It is the original language of the books of Ezra and Daniel and is the primary language of the Talmud. 400,000+ people speak modern Aramaic, mostly Assynians, though the communities are small and geographically scattered. It is frequently confused with the Ethopian language Amharic (also a Semetic language).

In case you are curious, the baby needed heart surgery for a transposition of great arteries (TGA), which is exactly what it sounds like. The pulmonary artery is joined to the left atrium and the aorta is attached to the right atrium (in a healthy person it is the reverse). The net effect is that the blood in the pulmonary (lung) circulation does not get to the body and the systemic (body) blood doesn't get to the lungs to be oxygentated. There is a small amount of mixing, facilitated by the ductus arteriosus (a small duct between the two arteries) that is artificially kept open through the administration of prostaglandins.