Very long chain Acyl-CoA dehydrogenase deficiency (VLCADD) is a genetic condition in which the body cannot break down certain fats into energy. In this handout, you will learn a care plan if your child with VLCADD gets sick.

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

When to Call the Genetics and Metabolism Doctor

Fasting or long periods of time without food can lead to metabolic decompensation. Seizures or coma can develop 1-2 hours after symptoms start.

When a child with VLCADD gets sick there are specific signs to look for that require further evaluation by a doctor, such as:

  • Lethargy (extreme lack of energy)
  • Vomiting or nausea
  • Unwillingness or inability to eat, even when sick
  • Signs of low blood sugar (shaking, chills, sweating)
  • Chest pain
  • Seizures or coma
  • Fever higher than 100.4°F (38°C)
  • Muscle pain
  • Red or brown urine

What Should I Do If I Am Concerned?

  1. Call Mass General Hospital at 617-726-2000. Ask the operator to page the Genetics and Metabolism doctor on call.
  2. Bring your child to the emergency room. Have the doctors follow the steps below.

What the Doctor Should Know

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

  1. Triage this patient IMMEDIATELY
  2. Page the Genetics and Metabolism MD on call at Massachusetts General Hospital.
  3. Place patient on cardiac monitor.
  4. Immediately start IVF with D10 (1/2NS or NS) and run at 1.5x maintenance. Obtain labs
    • STAT blood glucose (finger stick, if needed)
    • Comprehensive metabolic panel
    • Ammonia
    • Serum CK
    • BNP (NT-proBNP)
    • Urinalysis
  5. Consider EKG.

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. MassGeneral Hospital 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.