Urea cycle disorders (UCD) affect how the body processes ammonia. Below, you will learn a care plan if your child gets sick.

If you need to go to the emergency room, take this with you. Give it to the doctor so they can care for your child.

When to Call the Genetics and Metabolism Doctor

High levels of ammonia (a waste product that the liver turns into urea, a chemical in urine) in the body can be dangerous for children with UCD.

If you see the following signs in your child, please call the doctor.

  • Lethargy (extreme lack of energy)
  • Vomiting or nausea
  • Unwillingness or inability to eat, even when sick
  • Signs of low blood sugar (shaking, chills, sweating)
  • Confusion
  • Irritability
  • Rapid, shallow breathing
  • Seizures
  • Fever higher than 100.4°F (38°C)

What Should I Do If I Am Concerned?

  1. In an emergency, call 911. This includes acute or rapid illness.
  2. Page the Genetics and Metabolism doctor on call

What the ED Should Know

Below are steps to provide acute care to patients with UCD.

  1. Page the Emergency Department and the Genetics and Metabolism MD on call at Massachusetts General Hospital.
  2. Stop enteral feeds and start D10 (1/2NS or NS) at 1.5 times maintenance.
  3. Obtain labs
    • STAT ammonia (free flowing, no tourniquet, on ice)
    • CMP (glucose, chem 7, LFTs)
    • Venous blood gas

There is a Genetics and Metabolism doctor available 24/7. You can reach them by calling 617-726-2000 (pager #26396).

Ask the operator to page the genetics and metabolism doctor on call. They can talk with you if you are concerned about whether to bring your child to the emergency room. They can also help you figure out what to tell the doctors once you arrive to the emergency room.

Rev. 9/2019. Mass General for Children and Massachusetts General Hospital do not endorse any of the brands listed on this handout. This handout is intended to provide health information so that you can be better informed. It is not a substitute for medical advice and should not be used to treatment of any medical conditions.