Acyl-coa dehydrogenase, very long-chain, deficiency of
How do I know when to get medical care for my child with Very Long-Chain Acyl-Coenzyme A Dehydrogenase Deficiency (VLCADD)?
You should discuss with your metabolic doctor and metabolic dietitian about what to do when you are concerned about your child's blood sugar during your initial visit and during subsequent visits. Over time, you will get to know your child and the best way to respond when they are not eating due to illness or stress. You will also learn the signs that indicate you should take your child for emergency treatment.
If you child is sick, it is a good idea to feed them more frequently and to contact your metabolic doctor.
When you first notice that your child is not eating, you should offer him/her a favorite high sugar food, such as fruit juice. It is best to avoid Pedialyte and other rehydration drinks which do not have adequate amounts of sugar to help a child with VLCADD recover once their blood sugar starts to drop.
If a child continues to not eat, is vomiting and/or if they show signs such as low energy (lethargy) or irritability, take them to an emergency room for evaluation and treatment. Take your child's emergency protocol letter with you and present it on arrival.
A medical geneticist can be found by asking your doctor for a referral or looking on the American College of Medical Geneticists website. If you are looking for a metabolic clinic for treatment in the United States, you can search for the clinic or clinics in your state.
A metabolic dietician can be found through the Genetic Metabolic Dieticians International webpage.
A genetic professional can also be found on the Genetic Testing Registry Homepage.
SOURCE: Emory University - Department of Human Genetics in collaboration with ThinkGenetic • https://www.thinkgenetic.com/diseases/acyl-coa-dehydrogenase-very-long-chain-deficiency-of/living-with/5432 • DATE UPDATED: 2019-05-28
Fatty Oxidation Disorders Family Support Group. Retrieved 1 June 2016. Available from: https://www.fodsupport.org/diagnosis.htm
Fatty Oxidation Disorders Family Support Group. Retrieved 1 June 2016. Available from:https://www.fodsupport.org/child.htm
Arnold, GA, et al. A Delphi clinical practice protocol for the management of very long chain acyl-CoA dehydrogenase deficiency. Mol Genet Metab. 2009 Mar; 96(3): 85-90. http://www.ncbi.nlm.nih.gov/pmc/articles/PMC3219055/