Dad Loses Son to a Rare but Treatable Disease — His Painful Tribute Urges People to Test Their Kids

• Nikki Monaco and Joe Monaco welcomed a son, Emmett, who changed their lives forever.
• Emmett died at age five, and his father opened up about the little boy’s short life, including what he went through.
• Emmett’s mother took up a mission to ensure no other child goes through what her son endured.

Nikki Monaco and Joe Monaco, from Beaverton, Oregon, welcomed a son, Emmett, a smiling and friendly child. The couple, who have two other kids, were excited about their parenting journey.

Sadly, they later lost their child, Emmett. Joe said being a father to his son was the single greatest honor and privilege in his life.

Joe remembered how excited he was to have Emmett in their lives, saying, “The sun rose without him and my life forever changed.”

He also opened up about Emmett’s life before his death and the avoidable devastating experience his little boy had to go through.

Joe and Nikki said their son had a kind spirit. They noted that even at 16 months old, Emmett would go over to a kid who didn’t have a toy and give that kid a toy. “Emmett has this altruistic attitude,” said Joe.

The couple felt that their child would grow to become a kind soul in a world that needed such people. Nikki noted:

“He was the sweetest kid. I always said that he never met a stranger, only a friend he hadn’t made yet.”

However, their hopes of having their sweet little boy grow normally began to fade away when Emmett turned 18 months and started to lose his skills.

At first, the symptoms were not outright, as they included severe constipation, difficulty walking, and missed milestones. Then the symptoms got worse; Joe recalled:

“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. He just couldn’t move. He was just lying in one spot on the couch.”

The young boy also experienced blindness, muscle spasms, and painful seizures. Emmett’s parents took him to dozens of doctors and close to 100 appointments to understand why their son had stopped developing and started deteriorating.

When Emmett turned one, his parents took him to a neurologist due to the symptoms he had developed. The neurologist couldn’t feel his deep tendon reflexes and told the parents to take him to a neuromuscular specialist. Nikki narrated:

“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. My mom’s gut, if you will, knew that something was horribly wrong.”

Nikki and Joe wanted to do genetic testing, but their insurance wouldn’t cover it until other tests were done. Still, all the other tests they did came back normal. Emmett’s parents didn’t give up, as they knew something was wrong. Eventually, due to their persistence, doctors took them seriously.

Joe also revealed that Emmett could have lived a long healthy life if he had been diagnosed with Krabbe at birth.

Doctors finally decided to send Emmett to a rare disease program in San Francisco. Before he could be accepted to the program, doctors ran one more test on him. Joe remembered what the doctors 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. This test should have been done months earlier … It actually gave us the answer.”

After several misdiagnoses, the one more blood test the doctors ordered informed everyone about what was affecting Emmett. Doctors diagnosed the young boy with Krabbe, a rare and fatal disease. Most children with Krabbe don’t make it past their second birthday.

Krabbe is a neurological condition caused by an enzyme deficiency. The condition causes toxins to build up in a child’s nerves until their nerves die.

Doctors said they didn’t expect the young boy to live past the age of 10. Emmett lived up to 6 years old. During this period, his only way of communicating was through blinking, and his parents had to care for him 24/7.

Emmett would blink once for yes, twice for no, and three times to say I love you. After his parents learned that he had Krabbe, they didn’t let fear rule his life. They ensured their boy lived fully in the short time he had.

Nikki said they took Emmett to Disneyland, went camping with him, and took him on a road trip. They did all these things because they wanted him to experience life. Emmett rode horses and played with his brothers. Joe also recalled:

“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. I really enjoyed that.”

Nine months before Emmett died, he woke up screaming in fear because he lost his sight. Nothing could calm him down except his mom holding him.

After his death, Joe wrote an emotional post on LinkedIn announcing that his son, who was in kindergarten, had taken his last breath:

“My wife and I held him telling him how much we love him and how amazing he is. It was 5AM in Portland, Oregon when he passed.”

After Emmett’s death, Joe shared a picture of his three kids on LinkedIn. In the picture, Emmett was in a coffin as his two siblings stood saying one last goodbye to him. Joe wrote, “These are my three kids. Buying a 3 foot 6 inch coffin is at the bottom of every parent’s things to do list.”

Joe also revealed that Emmett could have lived a long and healthy life if he had been diagnosed with Krabbe at birth. He noted that Krabbe was treatable if diagnosed at birth through standard newborn screening at 24 hours old.

However, Emmett wasn’t diagnosed because the state of Oregon made a decision not to include Krabbe in the panel of tests. Joe said his wife was now on a mission:

“My wife has taken it upon herself to create a bill at our state Capitol to add Krabbe so no other Oregon child with Krabbe has to live and die with this wretched condition.”

Joe and Nikki testified in front of Oregon lawmakers to advocate for the creation of a bill that would ensure every newborn is screened for Krabbe.

House Bill 2987 directs Oregon Health Authority to expand newborn screening so that babies delivered in Oregon must be tested for multiple diseases, such as Krabbe.

As per Krabbe Connect, only ten states, including New York, Missouri, Ohio, Kentucky, Tennessee, Illinois, New Jersey, Indiana, Georgia, and Pennsylvania had made Krabbe testing part of their newborn screenings.

Joe and Nikki want to ensure children like Emmett are diagnosed in time for treatment because, with treatment, these kids undergo normal brain development and normal growth just like other healthy kids.

The parents revealed that when Emmett’s younger brother, Michael, was born, he was tested for Krabbe, and the results were positive. Michael was immediately treated with a stem cell transplant, and at ten years old, he was symptom-free.

Joe and Nikki’s youngest son Leo tested negative for Krabbe. The parents now want every family in Oregon to have a similar chance of testing their kids for Krabbe to get a sense of relief or give their babies a fighting chance.

Nikki said this mission would also ensure Emmett’s life had meaning and that he left a legacy. The couple believes Emmett will make the world a better place and hopes lawmakers will recognize this and support it.

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