Imagine this — your baby’s diaper smells like maple syrup rather than rancid poo. Great, right? Unfortunately, no.
If your newborn baby’s diapers are giving off a sweet scent similar to that of the stuff you put on pancakes, it’s not because of something they ate. In fact, he or she might be suffering from a bizarre disease known as Branched-Chain Ketoaciduria.
More commonly known as Maple Syrup Urine Disease (MSUD), this condition is often diagnosed by a sweet, maple syrup-like smell coming from a child’s urine.
The sweet smell may also make an appearance in the child’s earwax.
MSUD is most frequently diagnosed in newborns, but it can also rear its bizarre head during early childhood.
MSUD is a homozygous-recessive disorder, meaning that both parents passed on their recessive genes.
The disease most commonly is caused by a deficiency of branched-chain alpha-keto acid dehydrogenase complex (BCKDC). (Try saying that five times fast.) This deficiency causes a buildup of the amino acids leucine, isoleucine, and valine that can lead to serious issues like seizures, coma, or even death if not carefully watched.
In addition to the obvious smell, symptoms of MSUD include vomiting, dehydration, seizures, hypoglycemia, pancreatitis, and rapid neurological decline.
Controlling MSUD requires blood chemistry monitoring.
It’s also recommended that the child’s diet be watched closely. Children with it should steer clear of foods containing the three amino acids that tend to build up. Sadly, the only way to stave off negative symptoms is to monitor diet as strictly as possible.
Well, I’m never eating pancakes again, so there’s that.