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Patient presents with one or more acute life-threatening events consistent with LC- FAOD; AND 

Patient meets one of the following circumstances:
i) Triheptanoin is being used as second or subsequent line therapy in a patient who has a confirmed diagnosis of one of the types of LC-FAOD (Note 1) who is being treated with a less costly therapy with conventional even-chain MCT oil (e.g., trioctanoin) (Note 3) but is experiencing an inadequate response and requires alternative therapy to conventional even-chain medium-chain triglyceride (MCT) supplementation,
OR
ii) Triheptanoin is being initiated as first line therapy in a patient who has a confirmed diagnosis of one of the types of LC-FAOD (Note 1) who is presenting with acute life-threatening events of LC-FAOD,
OR
iii) Triheptanoin is being used as first line therapy in a patient who presents with acute life-threatening events consistent with a LC-FAOD but who does not have a confirmed diagnosis of LC-FAOD (Note 2);

AND

Patient is under the care of a clinician experienced in the management of LC-FAOD 

Provide documentation of genetic, biochemical, molecular, and clinical findings and investigations used to support the type of LC-FAOD diagnosis and the severity of the symptoms impacting the affected organ systems.

Request applications should include newborn testing results including genetic testing results as available and applicable (e.g., mutations in CPT1A, SLC25A20, CPT2, ACADVL, HADHA, HADHB genes) and may include the following to support the initiation criteria:
• plasma total and free carnitine
• plasma acylcarnitine profile
• urine organic acids
• urine acylglycines
• transaminase levels
• creatine kinase
• glucose and ketone patterns
• ammonia levels
• organ systems impacted and severity, frequency, and duration of events
• treatments used for LC-FAOD including the name, doses, and duration of use of conventional even-chain MCT oils, carnitine (as applicable), and information related to dietary measures to manage the condition.

Requests with a confirmed diagnosis should specify the type of LC-FAOD diagnosis and renewal requests initiated without a confirmed diagnosis should specify the type of LC-FAOD when the diagnosis is confirmed at the time of renewal:
i) Carnitine palmitoyltransferase 1 A deficiency (CPT1A)
ii) Carnitine palmitoyltransferase 2 deficiency (CPT 2)
iii) Carnitine-acylcarnitine translocase (CACT) deficiency
iv) Very long-chain acyl-coenzyme A dehydrogenase (VLCAD) deficiency
v) Long-chain 3-hydroxyacyl-coenzyme A dehydrogenase (LCHAD) deficiency
vi) Trifunctional protein (TFP) deficiency