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Warning-sign checks

Hyperammonemia due to ornithine transcarbamylase deficiency: when to get help

Go through these in order and stop at the first one that applies. If you are reading this for someone else, answer for them.

  1. 1

    Is any of this happening now?

    Call 911

    Call emergency services or go to an emergency department if you or someone with possible or confirmed OTC deficiency has:

    • New confusion, severe agitation, or unusual behaviour
    • Marked sleepiness or difficulty being awakened
    • A seizure, collapse, or loss of consciousness
    • Repeated vomiting together with weakness or drowsiness
    • Poor coordination, slurred speech, or rapidly worsening symptoms

    If an emergency letter or illness plan is available, take it with you.

    Tell the emergency team about OTC deficiency or the possibility of hyperammonemia.

    Other emergency signs, for anyone
    • Someone who is struggling to breathe, gasping, choking, or cannot get words out
    • Someone who has chest pain, tightness or pressure lasting more than a few minutes
    • Someone who has sudden weakness or drooping on one side of the face or body, slurred speech, or sudden confusion
    • Someone who is unconscious, or cannot be woken
    • Someone who is bleeding heavily and it will not stop with firm pressure
    • Someone who has had a seizure that will not stop, or does not wake up afterwards
    • Someone who has lips or skin that look pale, blue, grey or blotchy
    • Someone who you believe has taken an overdose or swallowed something poisonous
  2. 2

    Is any of this happening?

    Be seen today

    Do not wait for symptoms to improve on their own.

    Contact the metabolic team promptly for fever, vomiting, reduced eating, or difficulty taking prescribed treatment.

    This is especially important for infants, during pregnancy, after childbirth, or before surgery.

  3. 3

    Does any of this apply?

    Book an appointment

    Routine follow-up is used to review growth, nutrition, medicines, blood results, learning, and emotional wellbeing.

    New mild symptoms or problems following the management plan can usually be discussed at these visits or through the specialist team’s usual contact route.

Every sign above is quoted from the sofidex article on Hyperammonemia due to ornithine transcarbamylase deficiency. If you are not sure, err upward — it is always reasonable to be checked.