Press-Republican

May 20, 2013

Rare disease doesn't defeat Moriah native

Genetic ailment doesn't defeat Moriah native

BY LOHR McKINSTRY Press-Republican
Press-Republican

---- — MORIAH — Sitting in a car across from an insurance claimant’s home, Moriah native Johnathan Harrington brings a digital video camera with telephoto lens up to one eye.

As the allegedly disabled claimant exits the home, Harrington presses the shutter release and records her while she gets into her car and drives away.

“She said in her disability claim she was in agonizing pain and could not drive,” he said in an interview with the Press-Republican recently. “But she’s doing fine today.”

FAMILY CONDITION

Harrington, 31, is a private investigator in the Capital District; the fact that he can work at all is a tribute to modern medicine — along with his self-reliance and determination.

He has hereditary angioedema (HAE), a rare and potentially life-threatening genetic condition that can cause episodes of swelling in various body parts, including the hands, feet, face and airways.

“All the males in my family have it: my father, my grandfather, my two brothers and their two sons,” he said. 

Harrington grew up in Moriah and attended Plattsburgh State. 

He worked at Mountain Lake Services in Ticonderoga then relocated to Guilderland, where he enjoys his investigator’s job a lot — even though much of it is surveillance work that means long hours sitting in a car and eating fast food for meals.

“Being a PI doesn’t sound like much, but the excitement gets your blood pumping.”

FIVE-DAY COMA

When the HAE symptoms first hit him as a youth, doctors weren’t sure what it was.

“I had facial swelling. When I went to the ER, they attributed it to a spider bite. The drugs they gave me never did anything.”

Then an episode in 2008 put him in a coma, he said, even though by that time he knew what it was.

“I had a facial swelling on my bottom lip, but it started to go back into my cheeks and down into my throat. I was in a coma for five days.”

Harrington said he told emergency-room staff that he had HAE, but they didn’t know how to treat it.

“Now when we go to the emergency room, we call ahead, and they get the drugs ready.”

He is treated with a drug called Cinryze, an esterase inhibitor designed to prevent hereditary angioedema attacks.

“It’s an injection. I had a port put into my chest, and I now I self-administer the medication. 

“We‘ve been lucky. The insurance companies have been great. It’s $50,000 a month for the medication, and we don’t pay a dime.”

DAUGHTER AT RISK

The Cinryze, taken twice weekly, keeps the disease under control, he said, and must be taken twice a week.

“If I forget to take it, I get excruciating stomach pain. People go through agony because they can’t afford the medication.”

Another drug he can take is Firazyr, a peptidomimetic medication consisting of 10 amino acids, which is used to treat severe attacks of HAE.

Harrington and his wife, Nicole, wed in 2008, and they have a daughter, Kati.

There’s a 50 percent chance she will develop HAE, he said.

“One thing I pray for every day is that my daughter doesn’t have it. She’ll be 3 in July, and we’ll take her to Boston for genetic testing.”

WIFE IS ADVOCATE

He said Nicole has been very supportive of him.

“She keeps me on top of things. She does research, looks up articles online. She keeps me on my toes. She’s a really good resource.”

Nicole said she sees herself as an advocate for people like her husband who have HAE.

“It’s more fighting with doctors and nurses to get them to listen to me,” she said. 

“It’s taught me a lot about standing up to people, to see that he gets the proper treatment.”

On their 2008 ER visit, they told the staff Johnathan had HAE, she said.

“They had never seen a patient with it. They didn’t know what to do with him. The ER doctor admitted to me he didn’t know what to do. 

“That’s what started me researching what to do.”

RAISING AWARENESS

The second-annual HAE Awareness Day was May 16.

“People don’t know about the disease or that they have it,” Harrington said. “We ordered 200 awareness bracelets. We’ll send them out.”

He has two stepsons, Chris and Michael Ardito, and Michael recently wrote a paper on HAE that he delivered at school.

“My stepfather has HAE and had an attack in 2008 that landed him in a coma for 5 days,” Michael wrote. 

“Although it is extremely rare amongst most people, my 2 year old sister has a 50 percent chance of having this disease as well, so to me, it feels pretty common.”

‘SPREAD THE WORD’

Michael asked that people spread the word and suggested they look up HAE at haea.org.

“Not many others know about HAE, including doctors and hospital workers. You can help raise awareness just by getting everyone you know to tell at least two people each.  

“Hopefully, if my sister does have HAE, by the time she is my stepdad’s age, there will be a cure.”

Johnathan’s mother, Donna Jerdo of Moriah, said she’s proud of her son for working to beat the disorder.

“I can’t believe how far he’s come. He’s shown a lot of courage.”

Email Lohr McKinstry:lmckinstry@pressrepublican.com