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ACUTE MANAGEMENT OF MEDIUM CHAIN ACYL CO-A DEHYDROGENASE DEFICIENCY (MCAD)
- Such patients easily and frequently decompensate with minor infections, poor oral intake, vomiting, or constipation. The patient should be admitted immediately to ER and managed as triage level II.
- Immediate actions which should be accomplished within 1 hour of arrival:
- Basic life support
- Check GlucoChecks.
- Insert an IV line and take blood for blood gases, chem 1, Ammonia (NH3), lactic acid & CBC, blood C/S (if patient febrile), liver transaminase, CK level and acylcarnitine profile.
- If hypoglycemic <3.5 give 3 ml/kg of D10
- If normoglycemic but the patient cannot tolerate orally or if a patient has diarrhea, vomiting, dehydration, acidosis, or high fever, start 1 1/2 I.V.F with D10 NS +KCL. (confirm child passing urine before starting KCL)(adjust doses accordingly based on chemistry labs)
- Until the intravenous line is started, it is imperative to provide if possible / safe source of glucose like polycose solution
- Give carnitine 50mg/kg/dose IV then continue same dose divided q6.
- DO NOT administer lipids or acetylsalicylic acid.
