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Warning-sign checks

Urea cycle disorders: 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

    If you or someone you care for has a urea cycle disorder, follow the personal emergency plan during illness.

    Seek emergency medical care for:

    • Sudden confusion, extreme agitation, or unusual behaviour
    • Increasing sleepiness or difficulty waking
    • Repeated vomiting with weakness or reduced alertness
    • New problems with balance, speech, or coordination
    • A seizure, collapse, or loss of consciousness
    • Abnormal breathing or rapidly worsening symptoms

    Tell emergency staff about the urea cycle disorder and show them the emergency letter.

    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

    Contact the metabolic team promptly for fever, repeated vomiting, diarrhoea, poor food intake, or difficulty taking prescribed treatment.

    Early advice may prevent a crisis from becoming severe.

    Do not wait for symptoms to resolve on their own or try to manage severe symptoms by making major dietary changes at home.

  3. 3

    Does any of this apply?

    Book an appointment

    Arrange routine follow-up for concerns such as poor growth, recurring headaches, medicine side effects, increasing muscle stiffness, school difficulties, or trouble following the nutrition plan.

    Pregnancy and planned surgery should also be discussed with the metabolic team in advance.

Every sign above is quoted from the sofidex article on Urea cycle disorders. If you are not sure, err upward — it is always reasonable to be checked.