Business Notes

Tuckerton Business Donates Car to Parents Facing Medical Crisis

Jon Miller Care Care Owner Founded Far-Reaching Support Website
By MARIA SCANDALE | Mar 03, 2016
Photo by: Ryan Morrill

The family-owned business Jon Miller Car Care Center, of Tuckerton, presented a used Honda to parents who needed it, a family with an ill child who had lost two other babies to the same affliction. There’s so much more to the story of goodwill because Miller knows firsthand about having a child with the same illness, the rare genetic disorder tyrosinemia.

Miller is involved nationally and beyond. He formed the nonprofit Network of Tyrosinemia Advocates Inc. (NOTA), a support group worldwide.

Donating a car is far from the usual advocacy that the repair shop owner does for other parents – he testifies before federal and state lawmakers about the need for adequate newborn screening – but this case was unique.

The recent day that the couple in need of a car, natives of Puerto Rico, came down from Connecticut to see him “was very heartwarming,” Miller recalls. The parents had relocated to be near Yale University, where their daughter is being treated.

“The couple and I had been planning and preparing for that day for months. It was very emotional for Noelia (the mother). The couple are very humble and do not ask for anything. They didn’t even ask for a car,” Miller said. “I only offered one up after I heard that their car was broken. My first instinct was to tow it in and fix it up for them. Unfortunately, the car was in dire straits, and not cost-effective to restore.”

NOTA Families Not Alone,

But Stronger Together

Too much of one amino acid in the blood, tyrosine, makes all the difference. Tyrosine is a building block of protein. Tyrosinemia is caused by the shortage of one of the enzymes needed for the process that breaks down tyrosine. If untreated, the excess of tyrosine and its byproducts build up in tissues and organs, which leads to serious problems.

And the condition sometimes is untreated until too late because it can go undiagnosed in babies and young children. Although it is the nationwide standard to test for tyrosinemia during newborn screenings, not all states meet the standard, NOTA says.

Jon and Amanda Miller’s own son, Evan, now 6 years old, was nearly lost as an infant to undiagnosed tyrosinemia. At that time it took three hospitals and rooms full of doctors to determine his condition, and he was very nearly out of time when the answers finally came. New Jersey has since come into full compliance with the necessary screening.

Meanwhile, in Mayagüez, Puerto Rico, Noelia Pena, a registered nurse, and Dioni Coronado, an electrician, were newlyweds in 2010 when their daughter Gabriela was born. Although her routine newborn screenings were all favorable, four months into her short life Gabriela was admitted to a local hospital where she succumbed to an undiagnosed disorder, believed at the time to be dengue fever.

In September 2011 their son, Adrian, was born. Once again, newborn screenings suggested a healthy baby, but his parents soon noted respiratory issues. Adrian was hospitalized several times in Mayagüez with high fever, difficulty breathing and other symptoms. Showing no improvement, he was eventually transferred to a more specialized hospital in Ponce, PR. After a full battery of genetic testing, Adrian was diagnosed with tyrosinemia type I. But with limited treatment options, his condition continued to decline.

In October 2012, he was transferred to Yale Hospital in New Haven, Conn., and placed on the emergency liver transplant list. However, at this advanced stage his liver was too damaged, and within hours of his arrival in Connecticut, Adrian suffered a cerebral oedema. He was placed on life support, but Adrian’s doctors determined that no medical intervention could save him, and he died in the arms of his parents.

Noelia and Dioni returned to their lives and careers in Puerto Rico, determined to start a family. In April 2015 their daughter Laura was born, and all standard newborn screenings came back normal. This time, Noelia and Dioni requested more-specialized metabolic testing, and Laura tested positive for tyrosinemia type I. But her parents found it hard to get medication and the special formula needed to meet Laura’s nutritional needs in Puerto Rico. Their health insurance created barriers to proper treatment as well.

Noelia was searching the Internet in her few free moments, making contacts in the tyrosinemia community, when she found the Facebook page of the newly formed Network of Tyrosinemia Advocates. NOTA immediately went to work advocating on behalf of the young family, connecting them with treatment options unavailable to them in Puerto Rico.

Inspired by their new support network, when Laura was just 2 weeks old, her parents made the difficult decision to leave their careers, friends, family and the only home they’ve ever known behind and relocated to Connecticut, entrusting the care of their daughter to the highly competent doctors of Yale.

Upon their arrival in New Haven, Noelia and Dioni established a home and bought a used car with their limited savings. However, the car proved to be unsafe, and once this news reached NOTA President Jon Miller, he was compelled to help.

When a long-time JMCCC customer traded in her well-maintained Honda for a newer car, Miller knew he had the right vehicle for Laura’s parents. He gave them the keys, compliments of the car care center and NOTA.

Noelia has since reported that Laura is now stable, and her treatment has been progressing successfully. “Thanks to the help of many people, today my family is doing much better.”

Miller reflected, “This experience demonstrates how people in the rare-disease community can come together and make a difference. I am grateful for the opportunity to make a difference. We are weak as one, but together we are strong.”

Testifying to Lawmakers

For Better Screening

Evan loves cars (especially DeLoreans, on “Back to the Future”), trains and his baby sister. He can play sports, he is a Cub Scout, and he is doing excellently as a first-grader in his Little Egg Harbor Township school.

For more information on the Network of Tyrosinemia Advocates, visit NOTAcares.org.

An interesting note is that Miller’s family has been helped by the Catastrophic Illness in Children Relief Fund, whose funding comes from taxes on New Jersey businesses.

Asked whether medical research is promising, Miller told The SandPaper on Feb. 26, “Research is being done, and is a hot topic. However, there is ultimately no cure for the disease. Treatment is available, but very expensive, and cumbersome. The child must remain on a strict low-protein diet for life. Also, they must take a medication that is upwards of $60,000 per month, and drink a special metabolic formula for life.”

The biggest obstacle with this disorder remains early and accurate diagnosis, Miller stressed, but “the National Institute of Health has mandated that proper newborn screening be performed in all of the states.”

When Evan was born, he had a false negative due to an inferior NBS test. This almost cost him his life. New Jersey has since come into full compliance, yet six states are not in compliance.

“This fault in the system is what caused the deaths of Noelia’s two other children. I recently spoke at the National Institutes of Health. I spoke to the newborn screening advisory committee. They report to the secretary of health and are responsible for NBS standards.

“Tomorrow, I will be speaking in Hartford, Conn., at the state house. I will be petitioning Connecticut to come into compliance, and then Monday I will be speaking in Trenton at the New Jersey State House.”

Jon Miller Car Care Center is a full-service auto repair facility located on Route 9 in Tuckerton. It is a licensed state inspection and emissions repair facility.

“We believe in providing quality work at competitive prices. We use the best parts available for every vehicle and deliver honest, quality services you can trust. Stop by to meet us, or visit us on the web at www.jmcarcare.com,” summarized a press release highlighting the car donation and the group’s presence.

Advocacy work takes the owner away from Tuckerton quite a bit, but he has assembled “the best crew that anybody could ask for,” he said. “I would like to thank them for believing in my cause, and holding down the fort in my absence.”

Send business-related items to mariascandale@thesandpaper.net 



 

 

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