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NPSA issues MCADD guidance

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Deaths caused by medium chain acyl-CoA dehydrogenase deficiency (MCADD) could be reduced after guidance was issued to ensure nurses and other medical staff working with pregnant women take action on any mention of a family history of the condition.

The National Patient Safety Agency (NPSA) has acted to ensure that medical staff are fully aware of the significance of the disorder, which affects one in 10,000 babies.

If both parents are MCADD carriers, their child has a one-in-four chance of inheriting the disease. Newborn babies are particularly dependent on fat metabolism for energy in the first few days of their life as they have not established a regular feeding pattern. Subsequently, newborn babies with MCADD are especially vulnerable to early neonatal death.

The National Reporting and Learning System (NRLS) recorded two cases of MCADD babies who died after being born into families with a history of the disease over a five-year period.

It is thought that healthcare staff were not educated on the significance of MCADD and did not refer these patients to specialists or take any other precautions, despite the family history being mentioned during pregnancy.

The NRLS also recorded six additional cases in which no harm was caused to MCADD babies who were not referred for special treatment.

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