Table 10.

Prevention of Secondary Manifestations in Individuals with VLCAD Deficiency

Manifestation/
Situation		PreventionConsiderations/Other
Acute decompensation /
Rhabdomyolysis
  • Intense & ongoing education of affected persons & caregivers re natural history, maintenance & emergency treatment, prognosis, & risks of acute decompensation / rhabdomyolysis
  • Treatment protocols & provision of emergency letters or cards to incl guidance for care in event of illness while away from home
  • Medical alert bracelets/pendants, or car seat stickers
  • Written protocols for maintenance & emergency treatment should be provided to parents & primary care providers/pediatricians, & to teachers & school staff. 1, 2
  • Emergency letters/cards should be provided summarizing key info & principles of emergency treatment for VLCAD deficiency & containing contact info for primary treating metabolic center.
  • For any planned travel or vacations, consider contacting a center of expertise near destination prior to travel dates.
Surgery or procedure
(incl dental procedures)
  • Notify designated metabolic center in advance of procedure to discuss perioperative mgmt w/surgeons & anesthesiologists. 3
  • Emergency surgeries/procedures require planning input from physicians w/expertise in inherited metabolic diseases (w/respect to perioperative fluid & nutritional mgmt).
  • Some anesthetics may be contraindicated (see Agents/Circumstances to Avoid).
Consider placing a "flag" in affected person's medical record so that all care providers are aware of diagnosis & need to solicit opinions & guidance from designated metabolic specialists in setting of certain procedures.
1.

Essential information including written treatment protocols should be provided before inpatient emergency treatment may be necessary.

2.

Parents or local hospitals should immediately inform the designated metabolic center if (1) temperature rises above 38.5 °C, (2) vomiting/diarrhea or other symptoms of intercurrent illness develop, or (3) new neurologic symptoms occur.

3.

For affected individuals deemed to be high risk for perioperative complications, perioperative/perianesthetic management precautions may include an evaluation at a specialty anesthetic clinic.

From: Very Long-Chain Acyl-Coenzyme A Dehydrogenase Deficiency

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