An evidence-based guide

MCADD,
made clear.

Medium-chain acyl-CoA dehydrogenase deficiency — explained from the simple to the deeply scientific, so you can understand it, live safely with it, and follow the research that matters.

Incidence (US)
1 in 17,000
Most common mutation
K304E (c.985A>G)
Cornerstone of care
Avoiding fasting
In an emergency: persistent vomiting, unusual drowsiness, confusion, seizures, or being unable to eat or drink are signs to act on immediately. What to do

The 30-second version

Your body usually runs on two fuels: sugar and fat. Between meals and overnight, it switches to burning fat for energy. In MCADD, an enzyme that helps burn a certain type of fat (medium-chain fats) does not work properly.

Most of the time this causes no problem. But if a person with MCADD goes too long without food — especially during illness — the body can run out of usable energy while partly processed fats build up. That can lead to a dangerous metabolic crisis.

The good news: this is largely preventable. Avoiding long periods without food and having an emergency plan mean most people with MCADD live healthy, normal lives.

A term to know

Hypoketotic hypoglycemia

Low blood sugar with unusually low ketones — the signature of an MCADD crisis.

Browse the glossary →

Explore the guide

Five paths through the knowledge base — start anywhere, go as deep as you like.

How confident should you be?

Every claim on this site is labelled with an evidence level and its sources, so you can tell established fact from experimental work at a glance.

  • ● Level AOfficial consensus or recommendation from an authoritative guideline or institution.
  • ● Level BStrong scientific evidence — multiple consistent scientific sources.
  • ● Level CLimited evidence — small studies, observational, indirect, or significant limitations.
  • ● Level DExperimental or hypothesis — preclinical, mechanistic, animal, in-vitro.
  • ● Level XInsufficient evidence — not suitable for patient-facing recommendations.
How this content is verified

MCADD is a guide, not a doctor. All medical content here is traced to published, cited sources — verified 2026-08-21 — but your metabolic team knows your MCADD best.