
Sometimes when Brenda Hentosz stops to consider the circumstances of her child's extremely rare genetic illness, the feeling is overwhelming.
"We hit the lottery -- in reverse," said Mrs. Hentosz, who lives in Upper St. Clair with her husband, Dan, and four children.
Her youngest child, Angelina, 3, began experiencing prolonged seizure activity as an infant. It got to the point where her family could not leave her alone. At night, Angelina still sleeps between her parents in their bedroom.
That's going to change soon, thanks to the generosity of the family's local school and their community.
Service dogs require thousands of dollars to train and are not easily found for children, but students and staff at Baker Elementary, where two of Angelina's siblings go to school, have raised $4,837.08. In a few weeks, Angelina's family will have a new addition -- a dog that will be able to alert family members if she begins to have a seizure,.
"The outpouring ... I feel a little numb," Mrs. Hentosz said of the community's response.
A year ago, the Hentosz family took Angelina to Children's Hospital of Pittsburgh for testing. Previously, family doctors had told them Angelina had an undiagnosed form of epilepsy.
"I thought, 'Oh, she'll never drive a car, but it'll be all right,' " said Mrs. Hentosz, who had quit nursing school to spend more time with Angelina.
"We walked in there, healthy and happy. We were thinking, 'Let's get this done before the holidays.' "
For the testing, Angelina had to be taken off some of her medicines and that caused her to have multiple seizures. Because of the number of seizures, she had to be placed in a drug-induced coma.
She ended up being hospitalized in the pediatric intensive care unit for almost three weeks, her mother said. She developed pneumonia, suffered a collapsed lung and had to fight off a MRSA infection.
Angelina was released Dec. 26 and was just getting back on her feet when the family received stunning news.
"We got the call last January and they said they knew what it was, and I was so scared," Mrs. Hentosz said. "It's good NOT to have a reason, sometimes."
The diagnosis: Dravet syndrome. The extremely rare condition -- less than 650 reported cases worldwide -- is punctuated by prolonged seizures that often lead to medical and developmental regression, sometimes mental retardation, by puberty.
Also known as Severe Myoclonic Epilepsy of Infancy, the condition is caused by a genetic mutation. Protein development in the brain's sodium channels is affected, leading to seizures that don't respond well to standard medications.
Angelina, who attends preschool through the DART program at Streams Elementary School in the Upper St. Clair School District, also is on medication for attention deficit hyperactivity disorder.
She is an active little girl, running around her family's game room on tiptoes to tackle whichever sibling happens to be nearby.
Her sisters and brother -- Carissa, 12, Danny, 10 and Breanna, 8 -- don't handle her with kid gloves but tease and treat her pretty much like anyone else's kid sister.
Angelina, her mother and an aunt will travel to Kentucky next week to pick up her service dog.
Pawsabilities Unleashed is training a British Labrador retriever to respond to her specific needs, which include balance problems.
More importantly, the dog will alert family members if Angelina begins to have a seizure, especially at night. In some cases, service dogs have been able to accurately predict seizures before they occur.
After the diagnosis, Mrs. Hentosz said, she initially was at a loss. No local support groups exist for such a rare malady. But through an online forum, she discovered the e-mail address of Dina Nelson, the mother of a girl who has Dravet, and sent her a note.
"I was awake at three in the morning and I checked e-mail and said, 'Oh my God, she mailed me back.' It was like having a Christmas present."
The women have kept in touch, and the families were in Chicago in August at Children's Memorial Hospital, where they worked with Dr. Linda Laux and got to meet French physician Charlotte Dravet, who discovered the condition almost 30 years ago.
Dr. Laux said that the spectrum of patients with Dravet syndrome could be wider than originally suspected. Regression of physical and cognitive skills has been the pattern, but some older patients have had milder levels.
"While the prognosis is extremely concerning, there is some hope of the possibility of some children having less severe symptoms in the spectrum of the disorder," Dr. Laux said.
At home in Upper St. Clair, the Hentosz family room is adorned with photos of the children participating in various sports and activities.
Over the fireplace is a family portrait with Brenda and Dan, who is head of operations for CVS Caremark in Pittsburgh, and the kids gathered round.
Another photo frame features three pictures of Angelina with family and friends. "Inhale," "Exhale" and "Breath" are printed above the photos -- advice given, along with the frame, by Mrs. Nelson.
'What can we do?'
A ceremonial check was presented to the family Nov. 25 at Baker Elementary School, where Danny and Breanna are students. An anonymous donor has promised to add "a significant amount" in the coming weeks.
"When people first heard about Angelina, we received multiple calls: 'What can we do? What can we do?' " said Baker Principal Ruth Ann Matyuf.
"We had fundraisers through Denim Days on Fridays, bake sales, there was a Chick-fil-A luncheon," Dr. Matyuf said.
"In addition, we created these [plastic] wristbands and told children they could buy them for at least a dollar. It became a competition: The class that raised the most gets an extra recess."
The school PTA and local businesses kicked in. The middle and high schools had their own collections for Angelina and that money will go toward her general trust fund. Final totals are expected to be announced within the coming weeks.
One young girl in the neighborhood showed up at the family's front door with a $100 bill. She said her dentist heard about the service dog campaign and he wanted to make a contribution.
"The Hentosz's have been so much a part of our community, at sporting events, at school events. This is kind of like that movie, 'Pay It Forward,' " Dr. Matyuf said.
The fundraising continues through Angelina's Angels, a group that is selling handmade pet collars.
The Gasper family of Upper St. Clair is not particularly close with the Hentosz's, but Alyson Gasper wanted to help.
She discovered a company, Elmo's Closet, that would donate collars to be sold at the wholesale price.
"It's the right thing to do," she said. "And it's a great message to teach my sons [Colin and Alex]."
Collar styles and patterns can be viewed at the Elmo's Closet Web site, www.elmoscloset.com. Fundraising orders must be placed before Dec. 15 via e-mail to cgasper4965@msn.com.
Other donations to Angelina's Special Need Trust Fund can be made through the family's Web site at www.wishforangelina.org or by check through Washington Federal Savings Bank on Washington Road in Peters.
Because her social and language skills are delayed, Angelina has difficulty making friends. When her mother takes her to the children's play area at South Hills Village, little girls will run up to her but leave when they get little response to their overtures.
"She's actually better with boys; boys don't talk," Mrs. Hentosz said. "The little boys just say 'Chase me,' and she's all over the place with them."
That's why the British Lab "is going to be more than a piece of medical equipment," she said.
"Angelina is never going to have a real friend. This dog will be her friend."
It was clear Nov. 25, however, that Angelina has many friends. During the in-school broadcast at Baker Elementary on WBKR, the Hentosz family received the big cardboard check and countless good wishes.
"I want to thank the teachers and the students," said Mrs. Hentosz, tears welling in her eyes. Soon, just about every adult in the studio was sniffling.
"Our hope is for Angelina to be able to come to this great school in the future."
