2018-11-28 / Columnists

Pets, Pets, Pets

Until last summer, three-year-old Olivia the “Puppy Princess” enjoyed a carefree, charmed childhood. Six years ago, her parents, both secondary teachers, left Long Island for a small home on 11-acre Pacaberry Farm in Virginia where Olivia could frolic with alpacas and her beloved dogs, join her mom at dog shows and learn about farming and homemade crafts and jams. Then terrible news surfaced.

Olivia Schneider got the nickname “Puppy Princess” as a tiny toddler because of her love for stuffed animals and adoration of her own dogs—an Aussie, Afghan and Pyrenean Shepherds—plus the dogs she met at shows. She had a Facebook following on her Mom’s page as a “pre-school” junior handler. Who could foresee dog friends would soon come to the aid of their “Puppy Princess”?

Her family’s idyllic life changed forever in August when Olivia was diagnosed with Ataxia-telangiectasia, (or A-T) a rare and always fatal, recessive genetic disease that attacks children, causing progressive degeneration of neuro-muscle control (balance, motor skills, breathing, speech, sight, swallowing), immune system problems resulting in frequent infections, and a high rate of cancer. The condition is extremely rare with a prevalence of 1 per 40,000 to 100,000 worldwide, and currently about 7,000 cases in the U.S.

Both parents must carry this A-T recessive gene for their child to have the disease. To simplify, in A-T patients a protein known as ATM is missing or defective, which prevents a tumor suppressor called p53 from repairing mutated DNA before cells replicate; therefore increasing the risk of cancer. A-T patients are frequently wheelchair bound by their teens, on feeding tubes and breathing machines as symptoms worsen. Paralyzed ALS patients can move their eyes; A-T victims can’t, making it impossible to communicate with eye boards. A-T is generally fatal by late teens.

Unfortunately, Olivia has a rare form of this rare condition with two immunoglobulin deficiencies. At 
Olivia meets an Afghan puppy at her surprise Puppy Princess party. Olivia meets an Afghan puppy at her surprise Puppy Princess party. age three, she’s already showing symptoms of wobbliness, slurred speech and eye sensitivity to the sun. Her parents, Stephanie and Donald, had genetic testing after Olivia’s diagnosis. They are both carriers as is Olivia’s younger brother Myles.

How can this happen? Chances are so slim two people would fall in love, marry and both carry the gene for a devastating disease most people never knew existed. Both Stephanie and Donald were volunteers for Make-A-Wish before Olivia was born because Stephanie’s sister Brianne has epilepsy.

A-T Can Affect the Whole Family Physically: A-T patients are 37 times more likely to develop cancer than those in the general population. Some scientists believe ATM mutation carriers are more prone to developing certain cancers too, especially breast and stomach cancer. As a Catch-22, neither group can tolerate radiation, x-rays or strong chemo. Mammograms can be harmful. Carriers need to protect their skin and eyes from the sun also. Once A-T is discovered in a youngster, families wonder if A-T is the reason older relatives were cancer victims or suffered from motor weakness or seizures.

A-T Devastates the Family Financially & Emotionally: Each time a child is diagnosed with a deadly disease, a knife of reality plunges into the family’s hopes and dreams. When the condition is genetic, younger siblings become a worry too. Parents spend most of their time and resources traveling to specialists, getting treatment for their child and making their house handicapped accessible.

Olivia has a cough assist machine, and was getting infusions at the hospital to build her immune system. Her parents are learning how to do the infusions at home. Ironically, Olivia’s grandmother Theresa Morgan is a Northwell infusion nursing supervisor on LI.

One of four Puppy Princess logos put on fundraiser mugs. One of four Puppy Princess logos put on fundraiser mugs. Stephanie teaches middle school English, and Donald high school math in the same Virginia district. They’ve used up their family medical leave so they’re not paid for days they miss. The district is gracious about their frequent absences. However, only some of Olivia’s medical procedures are covered by insurance.

Converting their home to meet Olivia’s changing needs is expensive. Costs for a wheelchair accessible bathroom range from $15-25,000. Virginia doesn’t consider Olivia “sick enough yet” to qualify for Medicaid, whereas New York would allow a catastrophic diagnosis waiver.

Help from the Dog World: Stephanie has lots of dog friends, devotees of various breeds. Two surgeons pointed her in the right direction when Olivia’s symptoms were still mysterious. Selma Kominek is a Tennessee neurosurgeon and Afghan Hound fancier. She observed Olivia’s wobbly gait at a cook-out the Schneiders hosted, and urged Stephanie to pursue medical care at University of Virginia, a teaching hospital. Jen Cannon, an endocrine surgeon and Berger Picard fancier from N. Carolina, gave similar advice.

Take the Lead, a non-profit founded in 1993, provides support for people in the sport of purebred dogs who suffer the devastation of life-threatening or terminal illness. Take the Lead began contributing toward household expenses for the next six months.

A Briard friend designed a Zentangle t-shirt depicting Stephanie’s breed— Pyrenean Shepherds—sold online with 50% going to Olivia’s expenses. A similar Afghan shirt is in the works. A Pyr friend created four “Puppy Princess” logos that can be added to mugs ordered by dog fanciers, which generate funds for Olivia.

The Puppy Princess Party Rivaled the Royal Wedding: Our Afghan Hound community is like family. Sharon Ferraro of Montauk and Stephanie became close friends when they met at agility class. Stephanie adored Sharon’s late Afghan Manny, an advertising ace. She and Sharon co-own Honey, another Afghan champion. Stephanie’s mom Theresa owns Simon, a puppy from Sharon.

Sharon, Stephanie’s sounding board, planned the Puppy Princess Party as a surprise earlier this month. Her blonde beauty, Scarlett, whelped nine puppies in October. They are siblings to Simon. Sharon flew Stephanie and Olivia from VA to LI for a “girls’ weekend.” Olivia entered the magnificent Montauk house through balloon arches leading to Scarlett’s baby boudoir. Family and friends were waiting with gifts. Besides holding Scarlett’s puppies, the children enjoyed canine arts and crafts and ran through Sharon’s indoor agility course. It was a glorious day.

Building Awareness to A-T: This rare disease doesn’t have enough name recognition to spark widespread generosity. Kind people never hesitate to donate time or money when they hear a child has leukemia or another cancer. If Stephanie were to mention her daughter has A-T, a listener would look at her quizzically. Even Stephen King couldn’t conjure up a condition more horrifying. The A-T Children’s Project is connecting A-T families and publicizing the disease. There are research grants on the horizon, and strong, articulate parents like Stephanie and Donald spreading the word.

“Each day with our Puppy Princess Olivia is precious. Perhaps a cure will not come in time to help Olivia but I hope her story saves future A-T kids,” Stephanie said.

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