Vol. 6, No. 6
June 2014

Newborn Screening adds detection of SCID

The Iowa Newborn Screening Program, administered by the Iowa Department of Public Health (IDPH), will begin routine screening on July 1 for Severe Combined Immunodeficiency (SCID), a rare inherited disorder caused by a deficiency or absence of cells that help fight infections. Without a functional immune system, SCID patients are susceptible to recurring infections that can lead to death before the first year of life if left untreated.

This screening will be performed by the State Hygienic Laboratory’s Ankeny facility, designated by IDPH as the central laboratory for the Iowa Newborn Screening Program. The Hygienic Lab also provides laboratory support for the North Dakota and the South Dakota Newborn Screening Programs. The University of Iowa Children’s Hospital provides physician notification and recommendation services for babies born in all three states.

SCID (pronounced “skid”) is a disorder of the immune system that affects as many as one in 50,000 newborns. There are at least 13 known genetic causes for SCID, all of which lead to a deficiency or absence of T cells, affecting the baby’s immune system. This increases the risk for severe, life-threatening infections. Without early diagnosis and treatment, many babies with SCID will die by the time they reach their first birthday.

Before starting routine SCID screening in Iowa, a year-long implementation pilot was conducted. It established and optimized laboratory testing procedures and processes to identify infants at risk for the condition, and to ensure any identified at-risk baby receives timely, diagnostic clinical services with access to appropriate interventions and treatment.

"Detection of SCID in the immediate newborn period, before the onset of the first severe infection, can save a baby's life and improve the success of curing this otherwise life-threatening condition," Dr. Mary Beth Fasano, medical consultant for the Iowa SCID Newborn Screening Program, said during the early pilot phase.

Screening for SCID and other primary immune deficiencies involves a process known as T-cell Receptor Excision Circle (TREC) analysis. The addition of TREC analysis to the Iowa Newborn Screening Program is a significant advancement in diagnosis and treatment. The current sample collection practice for newborn screening won't change. All samples will simply be run through an additional testing process at the State Hygienic Lab.