CPT I

Baby is 4 hours old. I witnessed his birth (by cesarean), suctioned his airway, dried him with a towel, examined him, and saw him make the critical transition from life inside the womb to life outside. Strong cry, good tone, he’s good, I said.
Pager goes off: “Come to OB floor now”.

Problem #1: Hypoglycemia; baby cannot maintain his blood sugars after feeding

Problem #2: Temperature instability; his body cannot maintain his core temperature and it is dropping

Mom is homozygous for CPT 1 deficiency; her body cannot make a liver enzyme that converts fat to energy. Dad only has one good copy of the gene that makes the enzyme. Mom didn’t have any problems with her 1^st baby but here we are.  The problem is that these symptoms could also be signs of a serious bacterial infection—sepsis. Time to multitask.

#1: Place baby under warmer.

#2: Baby needs to get glucose. I tell the nurse he is regurgitating his formula. His small stomach has no more room. We need to get a peripheral line—nurses are good at this, we’ll be fine.

Right arm: fail

Left arm: fail

Left foot: fail

Right foot: fail

Plan B, get an umbilical catheter. My colleague asks for backup. I have never placed one or seen one placed. Medical director to the rescue—masterfully succeeds in placing it. He leaves. It clots.

Plan C, get a scalp catheter. Senior nurse succeeds. I want to give her a hug.

#3: Call Anchorage, this baby may need to be medevac’d to a place that has a neonatal intensive care unit. Hope it doesn’t come to that—process including flight would take 4 hours.

#4: Chest xray, blood work, start thinking about antibiotics if baby doesn’t improve with intravenous sugar solution.  

#5: Breathe.  Note baby’s scalp catheter makes him look like a little unicorn.

Happy ending: baby recovered, he maintained his temperature and blood sugars, he didn't end up being septic. We stopped his IV sugar solution and took out his catheter after 36 hours, then discharged him home 2 days later. Few things are as amazing, beautiful, and stressful as birth. 

Lessons learned:

CPT-1 is a rare metabolic condition that is more common in Native Alaskan Inuit people.

How to place an umbilical catheter.