While waiting at the State House to testify on a bill to screen newborns, Tess Laweryson sat by Peggy Anderson-Smith in the hall waiting for the hearing.
Laweryson had just met Anderson-Smith that day. The two were waiting to listen to a public hearing on whether or not to add screening for Krabbe disease and four other similar diseases to the list of those screened for at birth in Maine under L.D. 84, An Act Concerning Screening of Newborns for Lysosomal Storage Disorders.
Krabbe Disease
What it is: A disease that destroys myelin, the protective coating of nerve cells in the brain and nervous system
Cause: A genetic mutation inherited from both parents
What it does: Deterioration of nerve cells’ protective coating eventually leads to a loss of the ability of the cells to receive and send messages
It’s impact: Symptoms begin gradually and progressively get worse, resulting in infection, respiratory difficulty and failure, and ultimately death
Treatment: For infants with symptoms there is no cure. Early genetic screening provides an opportunity for stem cell transplants and other therapies that can slow progress of the disease.
Source: mayoclinic.org
Anderson-Smith was there to support her three-year-old granddaughter Addilyn Davis, who had been diagnosed with Krabbe. Laweryson, like dozens of other supporters who lined the halls and filled the overflow room Monday, was there to support the bill because she had been following Addilyn’s life through Facebook. Reading about Addilyn’s journey, said Laweryson, left her feeling a need to rally around the cause.
“These babies that were born with Krabbe don’t have a choice,” she said.
Addilyn’s mother, Jamie Davis, has posted online for years about the progression of the disease and the family’s struggle through it on a Facebook page called Addilyn’s Journey of Hope. The page has garnered about 107,000 followers who read Davis’ frequent updates on her daughter’s health and who comment back en masse to tell Davis they are praying for her.
The rare disease, which affects 1 in 100,000 babies, comes with severe symptoms like nerve pain, muscle spasticity and inflammation. Those afflicted with it usually don’t make it past age two.
Kyle Davis, Addilyn’s father, recalled in his testimony when he and Jamie were told their daughter’s diagnosis.
“So we asked one more question: How do we treat it? We got one more answer: You can’t, it’s fatal,” said Davis, taking a moment to compose himself before continuing his testimony.
About 75 supporters of the bill packed the public hearing at the Joint Standing Committee on Health and Human Services Monday afternoon.
A few of the supporters joined with the family and bill sponsors to testify, but most stood by emotionally listening to the testimony. The supporters wore t-shirts and sweatshirts printed over the years with different versions of phrases supporting Addilyn, and most wore blue ribbons for awareness pinned to their shirts.
Former state representative Lance Harvell, who used to represent Farmington and Industry, said in his testimony that one of the struggles that parents of children with the disease go through is that while waiting for doctors to diagnose the rare disease, the symptoms look like those of a baby born of a drug-addicted mother.
“My sense was that what most upset her is that a doctor is assuming what he is looking at is a cocaine baby,” said Harvell. “Jamie’s a young woman, and this is in all likelihood what a doctor has far more seen. She’s trying to tell that doctor that ‘I’m not a drug addict’ and that doctor is thinking, ‘People lie about drugs. That’s what I’m looking at.'”
Harvell described Jamie Davis’ passion for the cause, which drew him in, as inspiring and that she took “what might debilitate some people” and instead turned the situation into a positive cause. Harvell emotionally recalled when Jamie Davis told him about how she can’t plan for a future with her daughter.
“She said normally mothers, when they have daughters, they are planning for their weddings and their children. And she said, ‘I’ve been planning for a funeral her whole life,'” said Harvell.
State Rep. Andy Buckland, R-Farmington, said the screenings would cost $1 to $6 per infant and could be done at an existing out-of-state facility. The cost for caring for a child with Krabbe is about $700,000 a year, said Buckland, but if there was early screening, then the babies could be treated one time with a $300,000 to $850,000 stem cell treatment.
State Rep. Patricia Hymanson, D-York, cautioned Buckland about referring to the chance for stem cell transplants as a cure, since the Mayo Clinic advised that the treatments would slow the disease’s progress but were not curative.
One of those who testified, Betty Scott of Waterville, said she learned about Krabbe disease after her daughter started keeping up with Addilyn’s Facebook page. Scott asked the members of the legislative committee to approve the bill in response to the pleas of the 107,000 followers of Addilyn’s journey.
“We plead with you, speak for us and pass this bill,” she said.
Kaitlin Schroeder — 861-9252
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