Valves go with ventricles

Hypoplastic Left Heart Syndrome (HLHS).

HLHS is a congenital cardiac malformation that falls under the heading of "single ventricle" defects. Essentially, the baby is born with only one functioning ventricle that must supply (actively or passively) both the body and the lungs. They also fall under the coloquial heading of "blue babies."

Typically HLHS babies will have a poorly formed left ventricle, mitral valve, aortic valve and proximal aorta. In order to promote delivery of blood to the body, the ductus is kept open by administering prostaglandins and in some cases the foramen ovale will be enlarged (in the cath lab or the OR).

Surgical repair for this condition is done in three stages: a Norwood procedure (with central shunt) shortly after birth, a hemi-fontan or bi-direction Glenn a few months later and eventually a full fontan at 2-3 years. Pictures and explanations of those in a later post. Sometimes the right heart cannot cope with the strain and transplantation needs to be considered.

HLHS occurs more frequently in boys than girls and has an overall prevelence of roughly 4 in 10,000 live births.

On a more personal note, a child I visited often in the hospital made it to her hemi-fontan, but her heart couldn't cope with the stress. She was on the transplant list for months and recently received her new heart. I was able to watch them close her chest three days post-op (in major cardiac surgery on infants they generally delay sternal closure) and she is growing stronger daily.

On feeling stupid

I have been cramming physiology, anatomy, histology, pathology, biochemistry and pharmacology into my head since August 4th 2008 and I am not one tangible iota closer to being able to effectively treat patients. It's not that I haven't learned anything, it's just that most of what I've learned is foundational. It's like building a house: when you drive by a newly laid foundation it doesn't look like much progress despite a terrific amount of work.

Yesterday I shadowed on the pediatric cardiology ward. This is a leading candidate for the kind of physician I want to be in the future and a field in which I have been published for clinical research. I didn't expect to keep up with the residents and fellows, but I thought I'd get some of it... maybe 10%?

Try less than 1%. About the best I could manage was knowing what the acronyms stood for. I knew why they wanted albumin levels and I correctly identified respiratory acidosis. But I couldn't tell you that the pleural effusion was protein or why, what the difference between a surgical and pigtail chest tube was, why you would withhold anti-fungals on the febrile baby with mediasteinitis, why a triple lumen catheter was better than a double, which port you put the guide wire through when changing a central line or what a 3/4 Fontan was. It was intense, thrilling and terrifying. How am I supposed to get from what I know now to being responsible for a pod full of patients like that?

Plenty of people dumber than me have become good physicians. I find myself repeating that a lot lately. I too will learn it and master it. But it was certainly a hip check to see just how long the road ahead is.

To end on a high note... I loved the day on the PTCU anyway. Despite feeling cluess most of the time, I loved it there. It's a great mix of medicine and procedures, acute care with repeat patients, interesting problems and enough good outcomes. One day my life is going to be amazing.

Watch where you're mowing

We recently had a lecture on bioterrorism within our infectious diseases sequence. One of the potential diseases that could be used as a weapon is Tularemia, a not-too-deadily infection caused by Francisella tularensis. It has a low infective dose, does not spread human-human, but you would feel like crap for a while. The Soviets were accused of using it and the US even researched its use as a weapon in the '50s.

Anyway, what I think is much more interesting is the outbreak that occurred in Martha's Vineyard in 2000. The CDC documented cases of people getting sick from lawn-mowing. Apparently, they mowed over nests of infected rabbits, aerosolizing the infected rabbits and inhaling the bug. That's right. The people of Martha's vineyard got sick from aerosolized bunnies.

It was then published in the Journal of Clinical Microbiology.
http://www.pubmedcentral.nih.gov/articlerender.fcgi?artid=1233993

A spirochete song

My professor sang this to us in lab. Then he played a CD of his son's band performing it. It was originally printed in JAMA on Jan 31, 1942. Can you guess the disease?

There was a young man of Black Bay
Who thought syphilis just went away,
  And felt that a chancre
  Was merely a canker
Acquired in lascivious play.

Now first he got acne vulgaris,
The kind that is rampant in Paris,
  It covered his skin
  From forehead to shin,
And now people ask where his hair is.

With symptoms increasing in number,
His aorta's in need of a plumber,
  His heart is cavorting,
  His wife is aborting,
And now he's acquired a gumma.

Consider his terrible plight--
His eyes won't react to to the light
  His hands are apraxic,
  His gait is ataxic,
He's developing gun-barrel sight.

His passions are strong, as before,
But his penis is flaccid, and sore,
  His wife now has tabes
  And sabre-shinned babies--
She's really worse off than a whore.

He aches from his head to his toes,
His sphincters have gone where who knows,
  Paroxysmal incontinence
  With all its concomitants,
Brings forth unpredictable flows.

Though treated in every known way,
His spirochetes grow day by day,
  He's developed paresis
  Converses with Jesus
And thinks he's the Queen of the May.

It's syphilis.

spilled milk

I've alluded to the idea that the last month hasn't been the happiest in my personal life, which is true. And while I'm now managing to be honestly happy most of the time, I still have hours/days when I just feel achingly sad. I have found though, that when I get into one of those moods that stepping back a bit helps. Yes, it is appropriate and natural that I am sad, but the man I met who lost his arm, his son and his wife in the same week has much more to bear. The parents who had to withdraw care on their 3.5 year old son have lost so much more than me. It doesn't invalidate my own grief, but it certainly puts it in perspective for me.