Newborn Sophia Espadas has her pulse oximetry screening test at St. Luke’s Health Pavilion in Racine last year. Wisconsin has joined at least 31 other states that require testing of all newborns for critical congenital heart disease, one of the most common inherited birth defects. Credit: Kristyna Wentz-Graff

SHARE Special Report Deadly Delays: The nation's newborn screening programs depend on speed and science to save babies from rare diseases. But thousands of hospitals fall short, deadly delays are ignored and failures are hidden from public view — while babies and their families suffer. Go to section

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Wisconsin has joined at least 31 other states that require testing of all newborns for critical congenital heart disease, one of the most common inherited birth defects.

Efforts to require the test had languished in the Legislature for nearly two years, despite a 2011 federal recommendation that states require the simple $4 test.

Many hospitals had begun testing babies voluntarily for the condition, but a Milwaukee Journal Sentinel investigation in December revealed that more than a dozen were not performing the test. A 2010 study found that nine newborns in Wisconsin died at home or in the emergency room over a five-year period because their critical congenital heart disease went undetected.

Last fall, a baby born at a small hospital that was not routinely performing the test almost died, according to J. Carter Ralphe, chief of pediatric cardiology at the University of Wisconsin Hospital and Clinics.

The test, known as pulse oximetry, uses infrared light to check for oxygen saturation levels in the blood, which can be a sign of a heart defect. Critical congenital heart disease affects about 1 in 4,800 newborns nationally.

Wisconsin already requires a blood test that involves screening for 44 potentially deadly conditions, many of which emerge in the first few days of life. A Journal Sentinel investigation found many hospitals here and nationwide were not sending the blood samples to state labs in a timely manner.

In the wake of the Journal Sentinel story on the test for congenital heart disease, the Legislature amended the stalled measure to give the Department of Health Services the ability to require tests that do not involve blood or urine samples. It passed and was signed by Gov. Scott Walker in early March.

On May 29, an advisory committee to the Department of Health Services recommended adding the test, said Karen McKeown, the department's administrator of public health.

Last week, Walker signed an emergency rule requiring use of the test for all babies born in Wisconsin.

Emergency rules last for 150 days, and can be renewed, but the department also is moving to make it a permanent rule, McKeown said. That will require a final OK from the Legislature.

"I don't think there is any question the permanent rule will go into effect," she said.

In Wisconsin, mandatory testing was pushed for by the American Heart Association, the March of Dimes, the Medical College of Wisconsin and Children's Hospital of Wisconsin.

But the legislation had been opposed by the powerful Wisconsin Hospital Association, which called it a "well-intentioned but unnecessary mandate."

"Our goal all along has been that 100% of babies are screened," said Nicole Hudzinski, of the Heart Association. "We are now there."

Stuart Berger, a pediatric heart specialist at Children's Hospital of Wisconsin, said he hoped the state eventually will allow researchers to collect data from the tests to see if there are ways of improving it.

For instance, he said if rates of false positives and false negatives from babies tested in Wisconsin could be collected, doctors might know whether to use other, more extensive tests to look for congenital heart disease, such as echocardiograms.

"Perhaps we can do things better," Berger said.

Investigation leads to changes

In November, a Milwaukee Journal Sentinel investigation found that hundreds of thousands of blood samples from newborn babies arrived late at labs. In some cases, the samples were held and "batched" to save a few dollars in postage — putting babies at risk of disability and death.

The investigation also found that many state and hospital officials were unaware of the problems with newborn screening in their own health departments or hospitals. Several states said they did not keep track of how quickly hospitals were sending newborn screening samples; others refused to provide data to the Journal Sentinel.

Here is a look at some of the changes since the "Deadly Delays" series ran:

■ Both chambers of Congress have passed measures that would require experts to systematically track and improve the timeliness of newborn screening programs nationwide. The changes were made to the Newborn Screening Saves Lives Reauthorization Act, which will soon go to President Barack Obama.

■ Dozens of states have made changes to address delays, such as identifying problem hospitals and providing them with regular performance reports, adding courier services from hospitals and keeping labs open on weekends.

■ In May, Arizona — one of the poorest performing states identified by the Journal Sentinel — had 96% of babies' blood samples reach the state lab within three days of collection, compared with 67% the year before.

■ Since the investigation was published, four additional states have released hospital-by-hospital data. The District of Columbia and 20 states still refuse to provide information that would allow expectant parents and the public to see which hospitals don't properly handle the tests.