Rauner vows to get newborn screening for Krabbe disease ‘done’



Gov. Bruce Rauner on Tuesday said he was frustrated with how long the state is taking to begin screening Illinois newborns for a deadly disease and vowed to “get this testing done.”

He backed his Public Health Department’s handling of the matter, but neither the department nor Rauner could specify a date for when the testing will begin.


“Doomed by Delay,” a Chicago Tribune special report published online last week, showed how the department’s delays over 10 years robbed Illinois parents of a chance to save their children from Krabbe disease, a fatal neurological illness.

State legislators in 2007 mandated that all babies in the state be screened for Krabbe at birth. Babies with the disease, which is caused by a defective gene, appear healthy at birth but soon deteriorate as their nerves lose the ability to communicate with one another. Most die before their second birthday.

If the disease is caught during routine newborn screening, which Illinois babies undergo before leaving the hospital, doctors can intervene with a cord-blood stem cell transplant that can extend life and reduce suffering. To be effective, the transplants must be performed before symptoms appear, ideally within the first month of life.

Responding to that investigation, Rauner told the Tribune that testing would get implemented, despite the history of delays during his administration and previous ones.

“I’m committed, our administration is,” he said. “And we won’t give up. We’ll get this done.”

In a written response to additional Tribune questions, Rauner’s office added that Public Health anticipates screening all Illinois newborns for Krabbe before the end of 2017. An exact date was not given.

In that response, the governor’s office also praised Public Health Director Nirav Shah. “Net net, Director Shah deserves credit for finally breaking through the red tape to get this done,” the governor’s spokesman said.


But a regularly scheduled advisory committee meeting on screening Tuesday was much like many that have taken place over the years, with few specifics about when the next step in the process — which involves finalizing software for screening — would be complete and when testing would occur.

Natasha Spencer, whose son, Kenan Spencer Witczak, is dying of Krabbe after not being screened at birth, asked E. Matt Charles, a top state lab official, to be more specific about a vendor’s progress on needed software changes.

“Are we on step 6 of 10 or are we on step 7 of 10?” asked Spencer, a Chicago resident. “I would like something more concrete.”

Charles responded: “I can appreciate that, and I can also appreciate that this has taken a great deal of time. I do want to assure you that this is a very high priority of ours.”

Spencer shot back, “Is this the first priority of yours … or is it the second priority?”

The 2007 law initially set a goal for testing to begin by November 2010. But implementation has dragged on for years, beginning with a failed attempt by the department to use a cheaper but unproven alternative testing method. A Public Health spokeswoman recently acknowledged that the state is now on “Plan D.”

Since the law was passed, the Tribune found, at least five babies with Krabbe disease were born in Illinois, and their condition was not diagnosed until it was too late for a transplant to help them. Three have died.

On Tuesday, Rauner blamed the state’s byzantine procurement process for the delay in implementing Krabbe testing. The Tribune documented how bids from the Mayo Clinic, which has a lab willing to perform crucial secondary tests that the state lab cannot do, were twice rejected by Illinois procurement officials — once because a number on the bid was in the wrong column and another time because Mayo didn’t register with the Illinois Secretary of State’s office.

“Our procurement process is so broken,” Rauner said at an event at the National Museum of Mexican Art in Chicago’s Pilsen neighborhood. “And I’ve been talking about this from day one. The prior two administrations to me, as governor, have been trying to deal with this to get this testing done. It should be done. (But it’s) very difficult to get it done with our procurement process as it currently exists.”

He applauded Shah for coming up with a workaround: using the University of Illinois at Chicago Medical Center, which already has a contractual relationship with Mayo Clinic, as a go-between.

The Tribune, though, showed that the agreement with UIC sat for weeks before Shah signed it, even though Spencer had warned Shah in September 2015 that children were dying due to the lack of screening.

“Is your Director planning on signing the Agreement?” a UIC employee asked Public Health in an October 2016 email. “It’s been about 3 weeks now. Please advise us what the delay is.”

The current holdup has nothing to do with procurement. Public Health now is waiting for a vendor to add Krabbe to the computer program that takes the data from the state labs’ testing equipment, interprets the results and generates reports for pediatricians.

Public Health officials previously acknowledged that they asked that vendor to complete another unrelated project first because they didn’t want the vendor to lose six months of work on the other task.

On Tuesday, Charles told the advisory group that the other project is finished, so Public Health has made Krabbe this vendor’s top priority. He could not, however, answer Spencer’s question about how far along the vendor was in making the software change.

“I don’t have an exact project plan in front of me, so I wouldn’t be able to answer that,” Charles said.

After the meeting, Spencer said she was depressed about what she heard.

Said Spencer, “It’s the same old same old.”


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