Little Boy Blue
By Dr. Robert C. Reiser
The baby was blue. Not sad but actually blue in color. Deep dark blue. Cyanotic. Things that turn babies blue tend to be bad, and they tend to worsen very quickly. The referring hospital wanted the baby transferred stat via helicopter.
His name was Abraham and he was 10 days old. His father was a surgical resident in his fifth year of training. Abraham had been born healthy and done well up until the day before when he had begun to turn ever more blue. His only medical history was a circumcision performed when he was eight days old. Most circumcisions are performed in the first day or two of life while the baby is still in the hospital, so this timing stood out to me. I learned that Abraham was born into an orthodox Jewish family and had been circumcised at home by a type of rabbi known as a mohel. This custom is over 3,500 years old and is called a bris. It signifies a covenant between the child and God.
Like any good physician, my first diagnostic move was to Google up “bluebaby.com” which advised that time was critical. I must retain an attorney immediately. Whoops! Wrong website. I am not an obstetrician.
Back on track, I began to review the causes of cyanosis in a newborn. Normal babies are pink because that is the color of richly oxygenated blood cells. Blue is the color of blood cells deprived of oxygen. The most common reason for this is a defect or hole in the infant’s heart that shunts blood away from the lungs. Babies can do okay with this for a brief period after birth because there is normally a temporary hole from the aorta into the pulmonary artery, while the fetus is in the uterus, which can shunt blood back into the lungs. This hole closes in the days and weeks after birth. When it closes it closes fast and some of these babies suddenly get into a great deal of trouble rapidly. Ten days old would be about the right timeframe for this temporary hole to be closing, leaving the baby’s blood without access to the lungs and oxygen.
Yet the more I heard about this baby the less it sounded like he was in trouble. Abraham was feeding and pooping normally and seemed alert with stable vital signs. His sole abnormality was his striking blue color. Additionally a blood test to measure his arterial oxygen level was normal. This meant Abraham had a problem with his hemoglobin, not his heart.
Hemoglobin is a twisty molecule, a pigment really, in our red blood cells that carries oxygen. The pigment changes color when it binds oxygen: red when fully saturated with oxygen and blue when the oxygen is released to the cells that need it. At birth, Abraham had had normal hemoglobin. Now something had changed and the hemoglobin could no longer bind oxygen. It remained blue even after traversing the lungs, and so did Abraham.
The list of chemicals that can change hemoglobin in this way is short, but Abraham had no known exposures to any of these chemicals.
I went back on the Internet to research this bris business, since that seemed to directly precede the sudden change in Abraham’s coloration.
Bris is a highly ritualized circumcision, commanded directly by God in the biblical book of Genesis to be performed on the eighth day of life (and to any newly purchased adult slaves—ouch!). As a sacred covenant, it has not changed substantially in 3,500 years, with one peculiar and very recent exception. It seems that mohels are now advertising their services on the Internet and, in an effort to stay competitive, some are offering a topical anesthetic called EMLA cream to be placed on the surgical site 10 minutes prior to circumcision to numb it up.
The FDA in 1998 recommended against this practice because EMLA has been known in rare cases to mutate hemoglobin into a related pigment called methemoglobin. Methemoglobin cannot bind oxygen and so the color of the blood cells remains blue or, more accurately, brownish blue. Abraham had acquired methemoglobinemia, a hemoglobin-based cyanosis caused by the EMLA cream. Mystery solved, “House MD” style, and I let the referring physicians know of my conclusion. The cyanosis would reverse as soon as the EMLA wore off. I also offered my unsolicited opinion that one shouldn’t mess with 3,500 years of tradition (or the FDA).
We all agreed the helicopter could stand down.
There was just one problem: the EMLA cream should have worn off two days ago. Inquiries to the nursing staff revealed that the father (an MD) had obtained an entire tube of EMLA from the pharmacy and was continuously re-applying it to his son’s surgical site with each diaper change to assuage a stereotypically male concern with that particular part of the anatomy. This tradition too is over 3,500 years old and also should not be messed with.
On discontinuation of the EMLA cream the baby subsequently pinked up and did fine. The father may have some issues to work through, though.
And, oh yes, as we say in the borscht belt, the mohel gets to keep the tip.