baby with VLCADD

VLCAD Deficiency Mortality Rates

Very long-chain acyl-CoA dehydrogenase deficiency or VLCAD deficiency is a rare disease where someone struggles to break down fats for energy. It can range from mild to severe, and because of this, the prognosis of VLCAD deficiency can also vary drastically.

Like most fatty acid oxidation disorders (FAODs), VLCAD deficiency is typically diagnosed at birth from newborn screening. This early diagnosis can help doctors create a treatment plan, but even with this plan in place, this disorder could be life-threatening for some.

Life Expectancy for Infants with VLCADD

Severe cases of this FAOD can be fatal. Infants with a severe form of this disorder will start to show symptoms immediately that can be life-threatening. One of the most threatening issues involves the baby’s heart. Early-onset cardiomyopathy, a disease that makes it harder to pump blood, is common in infants with VLCAD deficiency and leads to high mortality rates.1` Along with heart problems, these infants can struggle with life-threatening low blood sugar called hypoglycemia that can lead to coma and death.

Life Expectancy for Children with VLCADD

Thankfully, the VLCAD deficiency life expectancy rate increases with age. Those who make it through infancy to later childhood can still experience the same life-threatening problems they did in infancy, but the frequency of these issues typically begins to decrease with time. Part of the reason for this change is that as children age, they are able to go longer periods of time in between eating without the threat of hypoglycemia. These people will still experience some uncomfortable symptoms, but so long as they follow their doctor’s treatment plan, their lives are in less danger.

Life Expectancy for Adults with VLCAD

Along with those symptomatic infants who make it to adulthood, some adults with VLCAD deficiency will have a milder form of the disorder that is only diagnosed later in life. When this occurs, the adults are typically less likely to experience these life-threatening issues and are able to live mostly normal lives.

Unfortunately, heart problems and low blood sugar issues related to this disorder can occur at any age and can still be life-threatening. It is important to follow the doctor’s treatment plan to avoid increasing the risk of any major issues if you or someone you love has this disorder. Living with a fatty acid oxidation disorder is possible, but the person still needs to take specific precautions.

At International Network for Fatty Acid Oxidation Research and Management (INFORM), we want to be a valuable source of information for both individuals and their families struggling with FAODs. Check back with our fatty acid oxidation blog frequently for more helpful information.

Sources:

  1. NCBI – Cardiac failure in very long-chain acyl-CoA dehydrogenase deficiency requiring extracorporeal membrane oxygenation (ECMO) treatment: A case report and review of the literature