Parents of dead, dying children blast Public Health director for newborn testing failure

Oct. 26--Speaking publicly for the first time about his department's decadelong failure to begin screening babies for a deadly disease, Illinois Public Health Director Nirav Shah told state lawmakers on Wednesday, "It's unacceptable and shameful that it's taken this long."

With parents of dead or dying children seated behind him at the state Capitol, Shah took a lashing from Rep. Mary Flowers, who called a hearing in response to the Tribune's special report "Doomed by Delay." The report exposed bureaucratic missteps that robbed those families of the chance to save their children from Krabbe disease, a devastating neurological illness that can only be treated if caught in the first days of life.

Parent after parent described for lawmakers the extreme suffering of their children, whose conditions would have been caught in time for treatment if Public Health had implemented a 2007 law that required these tests. Mothers told how their babies became paralyzed, went blind and deaf, and lost their ability to communicate anything but the feeling of pain.

Flowers offered this introduction to Shah and his department by speaking to the families of children with Krabbe: "It was important to us that the department see you, and hear you and feel your pain. I want them to listen to you because for 10 years you have been ignored, your children have been ignored. ... Nobody gave a damn. But they are here today."

She read sections of the Tribune story aloud to Shah as she admonished him for "breaking the law."

"You and your department for the last 10 years have made us all sick, and you caused the death of our children," said Flowers, a Chicago Democrat who chairs the Health Care Availability and Accessibility Committee.

In his statement to the committee, Shah said, "I can't begin to understand the anguish of the parents and your children who are affected by this disease."

Shah assured the committee that "there's an entire troop of people" at Public Health working to get the testing started before the end of the year. He said that when Gov. Bruce Rauner, a Republican, appointed him director in January 2015, he inherited a Krabbe screening project that was only "25 percent there."

Shah, a physician and attorney, said he made the screening a top priority.

His testimony, though, contrasted sharply with that of Natasha Spencer, a Chicago mother whose son, Kenan Spencer Witczak, is dying of Krabbe. Kenan was born in February 2011, after the date the Illinois legislature set as a goal for statewide Krabbe screening.

Spencer took direct aim at Shah as she addressed the committee. "Not even the disease defeats me the way Director Shah does," she said.

Spencer, who is a member of a Public Health advisory panel, wrote to Shah in September 2015, imploring him to cut through the red tape and begin screening babies so that none would experience the same suffering as Kenan.

Two babies with Krabbe were born after Spencer wrote that letter. By the time their conditions were discovered, it was too late for doctors to save them.

In the letter, Spencer walked Shah through a typical day, during which Kenan routinely stopped breathing, and implored Shah to meet with her.

Shah didn't grant her a meeting, records show, and his department did not make Krabbe the top priority. In response, Shah sent Spencer a letter that thanked her for "supporting families affected by Krabbe disease" and never mentioned her son.

"I would learn from the Chicago Tribune article ... that Director Shah, instead of addressing the questions I raised or accepting responsibility for the challenges I posted, chose to intentionally dismiss me," Spencer testified Wednesday.

"...With the cut-and-paste slap job of a form letter that Director Shah signed, (Public Health) let me know the value of my hard-earned and toiled-over insight, never once acknowledging my son Kenan is dying."

In a letter last week, Shah offered to meet with Spencer -- "two years, three Tribune articles and a legislative hearing later," she told lawmakers.

"His letter is shrouded in necessity of the wrong kind," Spencer said.

Shah told lawmakers he "was wrong" not to reach out to Spencer after she wrote to him.

Babies with Krabbe disease appear healthy at birth, but the lack of a critical enzyme leads to a deterioration of their nerves and brains. If the disease is identified through routine newborn screening, doctors can intervene with a transplant of stem cells derived from donated umbilical cord blood.

Transplants extend life and can prevent extreme suffering. Once a child shows symptoms of nerve damage, it's too late to intervene.

The Tribune's "Doomed by Delay" report identified five children whose Krabbe diagnoses could have been caught had the law been implemented. Three of those children have died, and the other two -- including Kenan, who is now 6 years old -- are dying. Spencer ended her testimony by naming each of them.

While the families waited for the hearing to begin, Shah struck a conciliatory tone with them, saying he wasn't there to "make excuses." He called the bureaucratic delays "the nonsense that led us to where we are now."

"Unfortunately this is the most maddening situation because it wasn't any one person, any one rule, any one anything," Shah told them. "Almost every step of the way somebody ran into a roadblock."

Scott Hammonds, who pushed for the 2007 law after his son, Liam, died of Krabbe in 2001, said he and his wife were so proud after the law passed and felt others would be spared Liam's suffering, only to later learn they hadn't.

"We both assumed that our job was done and were thinking those in charge would get their jobs done also," Hammonds said.

The other parents who testified all had children after the law passed.

Shermane Jenkins described through tears how she took her son, Logan Nichols, from doctor to doctor in Chicago for nearly two years. Despite repeated hospitalizations, none could help him. Jenkins, who gave birth at 18, said she lost four jobs and dropped out of school to look after Logan, who needed constant care.

"Everything was getting worse," Jenkins said. "He became a vegetable."

It wasn't until last January that she found doctors at Lurie Children's Hospital, who diagnosed Logan with Krabbe. She described for lawmakers the harrowing day in June that Logan died in her arms in a hospital elevator.

"Just to know that Illinois has the ability to change it and won't is just disgusting," Jenkins said.

Kilie Ponce, of Coal Valley, told lawmakers of the death of her daughter, Hayden, last month. "This disease robbed my daughter of her life and so did the state of Illinois," she said.

Laura Shelton, of North Riverside, said of her daughter, Lana, "I pray that she's able to take another breath."

And Robin Maubach, whose son, Dylan, died of Krabbe in 2014, implored lawmakers: "You have taken too long. Please act now."

The questioning was led by Democrats, but Tom Demmer, a Republican from Dixon, joined in and expressed frustration that lawmakers themselves hadn't kept better track of the progress of Krabbe screening until now. "There's more than enough blame to go around on everybody here," Demmer said.

Flowers told Shah she would bring him before the committee in January to see if Krabbe screening had begun as planned.

Long reported from Springfield; Callahan reported from Chicago.

pcallahan@chicagotribune.com

rlong@chicagotribune.com

Twitter: @raylong

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