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Nov 16, 2016 2:33 PM

Manchester siblings with extremely rare fatal disease head to Disney World

NH1.com

MANCHESTER - Nine-year-old Paul and 7-year-old Harlee Grace of Manchester both have an extremely rare, fatal disorder known as Canavan disease.

Paul and Heidi Gaffney married in 2007. Shortly after, their son Paul Gaffney IV (Pauly) was born. Everything looked normal, but that changed when he was about six-weeks-old. "He went in for the flu and that's when they transferred him to Children's (hospital)," Heidi said.

She says they were told they had a one in four chance of having another child with Canavan, but after Harlee Grace was born two years later, doctors reexamined their mutant genes and found out their mutation was the exact same make up, meaning any future child would have the disease.

The life expectancy for children with Canavan is 10-year-old.

Mom, Heidi, says Pauly used to be more mobile, but Canavan is a slow progression disease that is degenerative. "He used to roll over," Heidi says. "He used to walk in a walker."

Only about 450 people in the world have Canavan Disease, and Pauly and Harlee Grace are the only two in New Hampshire. Their care is constant and endless. The only break these tired parents get is when the kids go to school. Pauly and Harlee Grace are each assigned a nurse to help them during the school day. Dad, Paul is a trainer at a local boxing gym - a job he can maintain because they are flexible when he has to take time off for the kids' numerous hospitalizations. Heidi doesn't work, often running errands and trying to catch up on sleep while the kids are at school. Heidi says she and Paul sleep in 20 minute bursts overnight as they have to wake to suction the kids and make sure their oxygen tubes stay in place.

Since their world revolves around feeding tubes and suctions, Paul says they forget sometimes that not everyone lives like them. He laughs, "One of my nieces said, Uncle Paul I'm hungry. And I said, aw yeah, What do you eat?"

But despite all the challenges, Heidi says they do as much as they can with the kids.

The two are part of the Manchester Eagles Challengers Adaptive Cheerleading Team. They just won first place at Regionals and are heading to Nationals next month in Disney World. While some kids will be able to fly down, the Gaffneys will load up their RV with a hundred pounds of formula for Pauly and Harlee as well as all their hospital equipment and make the 1,300-mile trip.

The Gaffneys have set up a GoFundMe page to help pay for their Disney trip. You can also mail monetary donations to Manchester Eagles, PO Box 375, Manchester, NH 03105.

See this story Friday night at 5 and 10pm on WBINTV.

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