Baffled in Brooklyn

To clarify my earlier post

The baby made it through the code and seems to be doing well. Post-code labs don’t show any signs of end organ damage.

Bad Shift Was Bad

It all started when I ran a code in the NICU 20 minutes after arriving at work.  Ventricular tachycardia is no a rhythm we see often in the NICU.  Luckily it was all done with 1 push of Epi.  No shocking necessary.  I did get to brush up on my chest compression technique.  this baby has some sort of yet unnamed x-linked genetic neuromuscular disorder.  His older brother has the same thing.  The parents are first cousins.

11 fingers and 11 toes, would you like to have a cheap cigar?

Many babies yesterday were born with many digits yesterday. I had one baby with an extra finger on one hand, one with an extra finger and toe, and one with 6 digits on all four extremities. To balance this out the prankster God Loki saw fit to give one baby only 9 toes.

Our unit record for most digits on a baby with 4 limbs is 28. Our record for the fewest is 8.

Best Patient/Worst Patient: Episode 8 - Natal Teeth

I forgot to do a BP/WP for one of my February shifts so I’m gonna throw this in a bonus on my off day.  Every once in a while I see babies who are born with a tooth.  This unusual tooth is called a natal tooth (a.k.a. fetal tooth).  Natal teeth are typically poorly formed, flat, and have a poor root structure.  They are often not well anchored to the gum and are very wobbly.  While natal teeth can be present with certain syndromes, the overwhelming majority are an incidental finding in an otherwise healthy baby.  Natal teeth are often removed after birth because they cause difficulties with breastfeeding and their poor attachment to the gum make them an aspiration risk.  This means the tooth can easily detach and get lodged in the infant’s lung where it can cause severe infection and respiratory problems.

Natal tooth (Not my patient)

My patient had one natal tooth on the top that gave him a cute buck tooth appearance that the parents thought was adorable.  In the end, pediatric dentistry removed the tooth because it was very loose.

Removal of natal teeth can cause crowding of permanent teeth when they erupt.

End Note:  Not to be confused with natal teeth, neonatal teeth erupt in the first 30 days of life but are not present at birth.

Best Patient/Worst Patient: Episode 7 - Jaundice & Exchange Transfusion

Jaundice is a very common problem in newborns.  Without going into the gory details (Click here for gory details), it is a build up of bilirubin in the blood.  Bilirubin is the result of the breakdown of fetal red blood cells.  Everyone breaks down red blood cells all the time.  Various processes can speed up the rate of red blood cell breakdown and make jaundice worse, but we’ll skip that for now.  Bilirubin can be toxic to the brain in high concentrations but your liver converts it into a form that’s safer which can then be removed from the body via pee and poop (Again, congenital liver abnormalities can effect this process).  The newborn liver isn’t very good at converting bilirubin into the safer form so it takes a while.  Slower breakdown leads to a backlog.  Jaundice is a log jam of bilirubin in the bloodstream.  A certain amount of jaundice is considered acceptable in newborns and requires no treatment.  

If the level gets too high, or begins to rise too quickly, we help the newborn liver convert the bilirubin to the safer form.  The easiest way to help the infant in this process is by using phototherapy.  Phototherapy as a treatment for jaundice was discovered when a charge nurse at Rochford Hospital in Essex, England decided babies enjoyed sunlight and fresh air.  It was noticed that the babies who went outside for the sunlight had less jaundice.  Later, a vial of blood accidentally left on a lab window sill too long lead to the discovery of why sunlight helped the babies.  Modern ‘bili-lights’ use a wavelength between 460-490nm, the specific wavelengths of light that aid in bilirubin conversion.  How much bilirubin  is too much?  This is best answered by using a table such as the one below.  (Our baby’s bilirubin was too high to fit on this chart.)

Note:  The above table is only for babies older than 35 weeks gestation.  Jaundice in small premies is a much more complex issue that we’ll ignore for now.

As I mentioned above, we treat jaundice to avoid the buildup of bilirubin in the brain (called kernicterus).  Because modern phototherapy is so efficient, kernicterus is extremely rare.  Sometimes the biliruibin level is too high or the body is producing new bilirubin too quickly for the phototherapy to be effective.  In these cases we do what’s called an double exchange transfusion.  Very simply stated, we replace the baby’s entire blood volume 2 times over.  The overall result is an 88% reduction in bilirubin. 

For details of the exchange transfusion process, click below.  Suffice it to say, it takes a very long time and involves a lot of standing around in sterile gown, gloves, hat, and mask.

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Best Patient/Worst Patient: Episode 6

This week on BP/WP I’m going to give you some sage advice.  Most of you won’t need to hear this advice, but I’m going to tell it to you anyway.

Never bang your first cousin.

Aside from societal and religious norms, why is consanguinity a bad thing?  Genetics.  We all have mutations in our genetic code.  Most go unnoticed because we have 2 copies of each gene.  If you have a mutation in one copy of a gene, the other copy picks up the slack.  this type of genetic inheritance is called autosomal recessive.  Many AR mutations are passed down through generations unnoticed because there is only a problem if you reproduce with someone with the same exact mutation.  The odds of 2 people having the same exact mutation is extremely low.  If you bang your first cousin however, the chance you both share a rare mutation increases.  This concept is known as pedigree collapse.  This is a gross oversimplification of a very complicated topic.  

Why am I bringing it up today?  Because the worst patient from last shift has an extremely rare genetic condition due to the fact that his parents are second generation first cousins (Think French royalty without all the guillotines).  They are first cousins who’s parents were also first cousins.  2 of their 4 children have the same, as yet undefined, genetic syndrome.  They also have 2 healthy children.  To explain how they can have healthy children, I will refer to a picture.

As a physician, it’s important to note that consanguinity is not considered taboo in some countries.  There are areas of Bangladesh where this is the case.

For information on how this is relevant in Brooklyn and in your area, click the read more…

On a tangent, pedigree collapse is also relevant small, isolated communities such as the Doma people of Zimbabwe.  In Brooklyn, it is also a factor in the hassidic Jewish community.  Brooklyn is filled with many small sects of hassidic Jews.  Most will only marry people from within the same hassidic sect.  The sects small size with relatively few outside influences also leads to a situation similar to pedigree collapse (Only to a lesser degree).  To hear how Hassidic communities combat this problem (It’s actually quire neat) click the read more.

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Best Patient/Worst Patient: Episode 5

Polycythemia

We had a 33 week twin born with polycythemia.  Polycythemia is when your blood count is too high.  This is dangerous for the baby and can lead to a whole host of problems including strokes, seizures, necrotizing enterocolitis, as well as a kidney and liver problems related to blood clots.    A normal hematocrit for a 33 week newborn is somewhere between 35-45.  This baby’s hematocrit was 85.  Almost double.  Too many red blood cells and elevated hematocrit cause the blood to become thick.  This can cause it to sludge in the small vessels and cause problems.  Our baby had dark purple color on the soles of his feet from the viscous blood pooling in the small vessels of the feet.

In this case, the extremely high blood count was due to twin-to-twin transfusion.  Briefly, twin-to-twin transfusion is when the blood vessels of the twin’s placenta(s) become connected and blood is shunted from one fetus to the other.  This can cause a whole host of problems that we won’t discuss here, but one of those problems is polycythemia.

The baby on the left has polycythemia from a twin-to-twin transfusion.

Treatment for polycythemia is a partial volume exchange transfusion.  This is a long, tedious procedure that involves removing some of the excess blood from the newborn and replacing it with normal saline.  Since you can’t just take all the blood out at once, during an exchange transfusion blood is removed in 5mL aliquots until you reach your target.   This results in lowering the baby’s hematocrit without reducing overall blood volume.

8 Admissions in Half a Shift

‘cause someone’s gotta pay the bills in this shit hole.

Best Patient/Worst Patient: Episode 4

Worst Patient

I went on a transport to pick up a baby from another hospital.  It was a 35 week twin who was the donor in a twin-twin transfusion.  The result of this is that the baby is very small (1500g).  The baby’s abdomen got very distended and he developed necrotizing enterocolitis (NEC).  NEC is one of the big enemies in neonatology.  Babies can get very sick.  NEC can cause severe damage to the intestine and can even require surgery to remove dead intestine.  The mortality rate is around 50%.

X-rays on arrival to our NICU indicated the NEC had caused spontaneous perforation in the small intestine (A hole).  This is a sign that the infection is severe.  The only treatment for a perforation in NEC is to have a surgeon place a drain.  Our surgeon came to the unit and placed a small drain that allowed all of the infectious and necrotic fluid to drain from the abdomen.

If the baby recovers from the infection, testing will be done to determine if the areas of intestine involved in the infection have recovered.  Occasionally, sections do not recover and must be removed.

Best Baby

Just to end things on a lighter note, the best baby in the ICU was a little guy with Down Syndrome who came from the regular nursery because he didn’t really want to eat so much.  He sat under the warmer all day waving his arms and legs around with the cutest smile on his face.

My poor resident…

Just had to accompany a baby that we transferred to a hospital 3.5 hours away. On his weekend off. Turns out they don’t do the fancy PET scan we need anywhere closer to us.