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Dad goes viral with heartbroken post about son’s death from a rare, treatable disease

"Buying a 3 foot six inch coffin is at the bottom of every parent’s things to do list. Five months ago, my kindergartner, Emmett, took his last breath."
“Buying a 3 foot six inch coffin is at the bottom of every parent’s things-to-do list," Joe Monaco wrote in a post that went viral. Now he and his wife are trying to get more infants screened for the disease that killed his son.
“Buying a 3 foot six inch coffin is at the bottom of every parent’s things-to-do list," Joe Monaco wrote in a post that went viral. Now he and his wife are trying to get more infants screened for the disease that killed his son.Joe Monaco via LinkedIn

Nikki and Joe Monaco were devastated when their son Emmett died at age 5 of a genetic disease earlier this year. Then they learned that in 10 states, newborn babies are screened for the disease their son had — and it's treatable.

Born in a state with no screening, Emmett, a smiling, friendly boy, lost his ability to walk, eat and talk before he died of a disease called Krabbe. Now his parents pushing more states to screen for the disease, so other parents don't have to endure the same loss.

Emmett's family suspected something was wrong before he was diagnosed. When Emmett was 1, he stopped meeting some milestones. Over the next eight months, the family saw dozens of doctors at nearly 100 appointments to understand why he stopped developing and started deteriorating.   

I had a decision to make. I can either be swallowed by my grief or I can take that energy and use it to help other families.

Nikki Monaco

“He lost not only the ability to walk, but also the ability to sit up on his own. He lost the ability to eat and he started losing his ability to talk,” Joe Monaco, 37, of Beaverton, Oregon, told TODAY. “He just couldn’t move. He was just lying in one spot on the couch.” 

Finally, doctors ordered one more blood test and they learned what was wrong — Emmett had a rare, fatal disease called Krabbe. 

As Joe Monaco wondered how to let his colleagues know that his son Emmett passed away, he decided to write a post on Linkedin. That touching tribute to his son went viral.
As Joe Monaco wondered how to let his colleagues know that his son Emmett passed away, he decided to write a post on Linkedin. That touching tribute to his son went viral. Courtesy Apple Blossom Photography

“There’s a potential treatment option,” Joe Monaco said. “Parents can choose to treat their child if it’s diagnosed early enough, really before (they) become symptomatic.” Emmett’s diagnosis came too late and treatment couldn’t help him. That’s when the Monacos harnessed their sorrow, anger and frustration into lobbying Oregon to include Krabbe in its newborn screenings. So far, they’ve been unsuccessful, but they have no plans to stop. 

“I was so heartbroken and I had felt like I failed my son because I had been pushing and pushing and researching and still it was too late,” Nikki Monaco, 35, told TODAY Parents. “I had a decision to make. I can either be swallowed by my grief or I can take that energy and use it to help other families and future kids.” 

From infancy to illness

While Emmett was born two weeks early, he developed normally and soon became a social butterfly.  

“He was the sweetest kid. I always said that he never met a stranger, only a friend he hadn’t made yet,” said Nikki Monaco, who worked as a nurse until she became a full time caregiver to Emmett. “He, for example, would be in the nursery at church and would see a kid crying and even though he couldn’t crawl he would find a way to like roll, scooch over to them to give that kid the toy he was playing with.” 

The Monaco family: After Emmett's death, his parents are trying to turn their pain into purpose.
The Monaco family: After Emmett's death, his parents are trying to turn their pain into purpose.Courtesy Apple Blossom Photography

When he was 1, he stopped meeting milestones. After a months-long wait to see a neurologist, the doctor couldn’t feel Emmett’s deep tendon reflexes and she recommended the family visit a neuromuscular specialist.

“They told us that he’d be the first kid out in dodgeball and he’d never be an Olympic athlete but they weren’t super concerned,” Nikki Monaco said. “My mom gut, if you will, knew that something was horribly wrong.” 

Emmett was a social butterfly, always making friends even when he couldn't walk or talk. His family misses his sweet personality but turned his tragic death into advocacy for other families.
Emmett was a social butterfly, always making friends even when he couldn't walk or talk. His family misses his sweet personality but turned his tragic death into advocacy for other families.Courtesy Apple Blossom Photography

The family wanted to do genetic testing, but learned that other tests were needed before insurance would cover genetic testing. Test after test came back normal.  

“There was nothing wrong,” Nikki Monaco said. “I pushed back again. I said, ‘I understand what you’re saying that it’s normal, but where do we go from here? Because obviously there’s a reason we’re getting these tests done.’” 

When Joe and Nikki Monaco noticed Emmett regressing, doctors started taking them more seriously. Then a doctor revealed that one test had shown a small problem. 

It was fun to see his personality come through because sometimes he was a little stinker. I really enjoyed that.

Joe Monaco

“It went from a slight abnormality to the head of neurology telling us there was an obvious delay on that test we were told was normal,” Nikki Monaco said. 

Still, doctors had few answers as Emmett could no longer walk, sit, talk or eat. They decided to send him to a rare disease program in San Francisco; doctors ran one more blood test before he could be accepted there.  

“(They said), You’ve got to check one more box. It’s kind of silly. The test won’t tell us anything. It’s just a formality,’” Joe Monaco recalled. “This test should have been done months earlier … It actually gave us the answer.” 

He was diagnosed with Krabbe on January 25, 2018, when he was nearly 2 years old. 

Toward the end of his life, Emmett could not see and would often wake screaming. Nothing calmed him except when mom Nikki Monaco held him.
Toward the end of his life, Emmett could not see and would often wake screaming. Nothing calmed him except when mom Nikki Monaco held him.Courtesy Monaco Family

Krabbe Disease 

Krabbe disease is a rare genetic condition inherited when both parents carry the gene. 

“It’s a fatal disease that affects development of the brain, predominantly occurring in very young babies,” Dr. Joanne Kurtzberg, Jerome S. Harris Distinguished Professor of Pediatrics at Duke University School of Medicine, told TODAY Parents. “(It) is caused by a mutation in a gene that codes an enzyme that’s necessary for development of the brain.” 

Myelin covers nerves in the brain “like insulation on wires,” Kurtzberg explained. But in children with Krabbe, the mutated enzyme means they cannot myelinate their nerves. 

“If you can’t cover those nerves, they die,” Kurtzberg said. 

Babies born with Krabbe show no immediate symptoms. Over weeks or months, they start developing problems such as difficulty eating. Because it’s so rare, though, these babies are often mis-diagnosed as having colic or reflux. That means they don’t receive a Krabbe diagnosis until it’s too late. 

About nine months before dying, Emmett lost his vision and woke screaming in fear. Nothing calmed him other than his mom Nikki holding him.

“The babies will smile and maybe start to lift their head up at 2 months of age but then they start to lose that and they never have normal development,” Kurtzberg said. “By the time they’re 5 or 6 months of age they’re spastic, which means they’re very stiff. They have seizures. They’re often blind. They’re extraordinarily irritable because the brain is irritated. So the mom, on top of all of this, can’t really comfort the baby.” 

Babies can have late infantile Krabbe, juvenile Krabbe or adult Krabbe. Without treatment, babies die, though children and adults who develop later forms of it tend to live longer. If babies receive a stem cell transplant from cord blood within the first month of life, their lives can be extended. The problem is most babies aren’t diagnosed until after they develop symptoms, which is too late. Babies who are screened undergo chemotherapy to rid them of their bone marrow and immune system and then receive donor cells.  

Stem cell transplants have occurred for “about 20 years and appears to extend life for most babies generally at least for 20 years," Kurtzberg said.

Emmett made friends wherever he went by being kind and generous.
Emmett made friends wherever he went by being kind and generous.Courtesy Monaco Family

With treatment, Kurtzberg said, “The brain can undergo normal development. That will mean the baby will smile, will coo, will be able to feed, will be able to sit and stand and to have normal development that an infant would go through.” 

Emmett taught himself to blink to communicate — once for yes, twice for no and three times for “I love you.”

According to Krabbe Connect, only 10 states screen for Krabbe: New York, Missouri, Ohio, Kentucky, Tennessee, Illinois, New Jersey, Indiana, Georgia and Pennsylvania. That means babies like Emmett in other states are often not diagnosed in time for treatment. While the federal government mandates some newborn screening, states determine whether they screen for other less common conditions. 

“The estimates are it probably occurs in one in 100,000 births but it may even be rarer than that,” Kurtzberg said. “If a state has to spend money on health care for living children or screening for Krabbe, they’ll probably not choose to screen.” 

Life with Krabbe and life without Emmett

As Krabbe progressed, Emmett’s health worsened. Still the Monacos gave him a full life, including travel, riding horses and playing with his brothers. 

“We were not going to live in fear,” Nikki Monaco said. “We went to Disneyland with him. We took him camping. We went to Montana on a road trip. We did things because we want him to live his life to the fullest.” 

As a nurse and a mom, Nikki Monaco knew something was wrong when Emmett started regressing, so she kept pushing doctors for answers.
As a nurse and a mom, Nikki Monaco knew something was wrong when Emmett started regressing, so she kept pushing doctors for answers. Courtesy Monaco Family

The Monacos' faith helped them as they grappled with his illness and death, and believing that they’ll see him again gives them comfort. About nine months before dying, Emmett lost his vision and woke screaming in fear. Nothing calmed him other than Nikki Monaco holding him.

“It was important for us to give him as many experiences with his brothers as we could because memories are what life is made of,” Nikki Monaco said. “But my favorite memory was just holding him.” 

Emmett taught himself to blink to communicate — once for yes, twice for no and three times for “I love you.” 

“I loved to ask him questions that I potentially already knew the answer to. But it was fun to see his personality come through because sometimes he was a little stinker,” Joe Monaco said. “I really enjoyed that.” 

For Father’s Day this year, Joe Monaco shared a touching post on Linkedin about Emmett’s death, mainly as a way to notify people that Emmett had died. But it was also a call for more states, including Oregon, to screen for Krabbe. 

“Buying a 3 foot six inch coffin is at the bottom of every parent’s things-to-do list. Five months ago, my kindergartner, Emmett, took his last breath as my wife and I held him telling him how much we love him and how amazing he is,” he shared. “This will be my 9th Father’s Day as a Dad, but will be more special than ever as I remember the single greatest honor and privilege of my life — to be Emmett’s dad.”