Fundraising from the heart: Local family in need after toddler diagnosed with heart condition

JJ Price, 2, was diagnosed with Brugada Syndrome as the youngest known survivor in the world

JJ Price, 2, is the youngest known survivor in the world with Brugada Syndrome, a heart condition which has brought the toddler to CHOP 14 times in the past year and a half.

Samantha Price knew the doctors were missing something when she was pregnant with her now 2-year-old son JJ. After a false positive test for trisomy, a second trimester screening to determine if a patient is at risk for chromosomal abnormalities, Price’s intuition told her something was wrong. One week after JJ’s first birthday, he was transported and admitted to the Children’s Hospital of Philadelphia with a heart rate of 303 beats per minute, more than 200 beats per minute higher than the normal resting heart rate of a baby his age, which should typically range from 80 to 130. It was determined JJ had four strands of the genetic disease Brugada Syndrome, and the toddler became the youngest known survivor in the world.

“He is the only person in the world with this condition,” Price said. “They know what Brugada is, both my husband and I each have two strands, that’s normal; for them to see all four strands, they’ve never seen that. Even the doctors who founded the gene, they’ve never seen four strands.”

According to Price, Brugada typically does not show itself until someone is in their 30s, however oftentimes the syndrome can be the cause of sudden cardiac arrest in athletes due to its ability to cause a disruption in the heart’s normal rhythm. Since JJ’s diagnosis a year and a half ago, Price said, the toddler has been admitted to CHOP 14 times due to cardiac arrest.

“Instead of his heart going lub-dub, lub-dub, his heart just speeds up for no reason,” Price said. “As horrible as it is, he makes the best of his life. At any minute, he could go into cardiac arrest for no reason, but he has saved my life, his dad’s life, his sister’s life; none of us knew we had this.”

According to Price, through JJs diagnosis, many members of her family have been tested for the syndrome, bringing attention to the disease and offering a chance for prevention. Now that she, her husband and her 5-year-old daughter Carylynn know of the strands they have, they are seen by a cardiologist every six-months and use a halter monitor, as well as electrophysiology studies to ensure there are no unexpected arrhythmias taking place.

“Him getting sick really, in a way it really stinks for us and for him, but he saved so many other people and he’s doing stem cell research to save others that could be like him in the future,” Price said.

While doctors and scientists are aware of the disease, many are unfamiliar with how to treat such a severe case such as JJ’s. Medicines such as Esmolol and Quinidine, which are often used to treat Brugada, are ineffective for JJ. Price said JJ is participating in a trial medicine as the second patient to ever take Cilostazol, however he is the first child to try the medicine.

“He’s writing his own textbook,” Price said. “He’s figuring out ways to treat himself.”

After his first admittance to CHOP, JJ underwent open-heart surgery where an implantable cardioverter defibrillator was inserted, a device that is able to shock the heart through cardioversion when his heart rate gets above 215 beats per minute, as well as pace the heart rate to remain in the low 80s. However, because JJ’s ICD is needed so often, the battery life, which is expected to last five to seven years, only has 10 to 21 months remaining. Price said doctors will monitor the battery and it will need to be replaced once it has three to four months’ expectancy.

“Every time he gets shocked, it uses a month of battery,” Price said. “He uses a lot because his heart depends on the pacer. Every time he has a run, it paces his heart and tells it to slow down, so it is constantly being used up.”

To combat the medical expenses the Price family has to face, family friend of 15 years Amber Powell of Gloucester Township has began community fundraising efforts to support JJ’s needs and his family’s struggle.

“I’ve been watching how strong Sam has stayed over the past one and a half years with JJ, and how positive she’s been. I think she’s incredible,” Powell said. “They’re struggling to keep their heads above water and they never ask for anything.”

Inspired by Price’s strength, Powell wrote a letter to Ellen DeGeneres, however she felt as though she needed to do more to help her family friends.

“What are the chances Ellen will ever call me back?” Powell said. “So, I thought to start a fundraiser to raise awareness about Brugada through JJ’s story.”

Powell created a Go Fund Me page for the Price family with the goal of raising at least $25,000. Since its creation on Nov. 3, the efforts have raised $670 from community contributions. Additionally, P.J. Whelihan’s Pub and Restaurant in Washington Township will be holding a dine and donate for JJ’s cause on Nov. 27 and Dec. 18, with 15 percent of patron’s total checks being donated when JJ is mentioned during the visit.

Powell has also designed custom T-shirts for sale for $15, designed with a heart and the words, “JJ’s heart has a different kind of rhythm — Brugada Syndrome Awareness.” Powell’s hopes are to hold a larger beef-and-beer fundraiser on March 23, 2018 at the Washington Township Senior Center from 7 p.m. to 11 p.m., with the goal of selling at least 300 tickets to raise awareness for the disease and funds for the Price family’s medical bills.

“They are a local family, they are regular people like you and me,” Powell said. “They didn’t see any of this coming; they had a healthy beautiful little boy and they were the average family. Next thing you know, JJ was almost taken from them and their entire world was flipped upside down. It could happen to anybody.”

From experience, Price encourages the community to take a CPR course if available, because “you cannot save somebody who is going into cardiac arrest without CPR.” To find local CPR courses held at Jefferson Health (formerly Kennedy Health), visit www.kennedyhealthy.org/services/cpr-life-support-education.

To learn more about JJ and the Price family, or to make a donation, visit https://www.gofundme.com/jjsmightybattle. To purchase a t-shirt, check out the “MightyJJsHeart” page on www.etsy.com. You can also follow updates about JJ’s condition on the “Mighty JJ’s Heart — Brugada Syndrome” Facebook page.