Rea of Hope Fundraiser for NPC: October 14th at Bellingham Town Common

By Jennifer Russo

“What I’m trying to cultivate is not blind optimism, but radical hope.”- Junot Diaz

Niemann-Pick Type C, also known as NPC, is an extremely rare disease affecting only about 100 patients in the United States currently. It is an inherited neurodegenerative disease which causes damage to the nervous system over time due to abnormal processing of lipids, namely cholesterol, in the body’s tissues. These lipids build up in parts of the body like the brain, liver and spleen, affecting physical and cognitive function.  

Sadly, NPC is ultimately fatal, with patients who show symptoms in childhood often only living to their late teens or early twenties. Patients with less severe cases can live into adulthood, but the disease will continue to progress. 

Though there is no cure for NPC, therapy and some new medications can delay its progress and extend life expectancy of patients. Some of these medications are experimental and are at risk of non-approval  or discontinuation even though they are shown to work. Without any medication at all, patients will slowly begin losing their ability to move properly, speak, and eat. It will impact their memory function, and often lead to early dementia, hearing loss, eye control, seizures, and liver failure.

Bellingham’s Meredith Piotti is one of the greatest advocates to finding a cure and keeping these medications available to NPC patients. Her daughter, Reagen, was diagnosed with NPC in 2021, after several visits to doctors to try and understandwhy she was falling more and had a decline in motor function. 

“It was a gut punch harder than anything in this world,” shares Meredith. “Not only did we just welcome her youngest sister to the family, but Reagan had previously been tested for NPC, and we were wrongly told she did not have it. It was unthinkable and heartbreaking, and we had to live with the fear that either of her sisters could also have it. At the same time, we knew something was going on and that her symptoms were progressing. To have an answer, even a horrible one, gave us something to fight.”

And fighting they are.

Reagan takes an off-label medication three times a day and every two weeks they travel to Connecticut to have another medication administered through lumbar puncture. Before June of last year, they were flying to Chicago to get this treatment because it was not available in New England.

“We are blessed that the NPC community, although tiny, is filled with many fighters and legacy families have made incredible strides so that hope is now on the horizon…we are fighting to move hope into the hands of those living with NPC and those to come. We can’t reverse the damage that NPC has done to Reagan already, but her combination of medicine appears to be making her stable. We need approval and further advances, but today we are ok... and that helps. Most days we are focused on enjoying our family and pushing forward this initiative however we can,” Meredith says.

According to Meredith, Reagan is a sweet, creative and outgoing girl. She loves singing, arts and crafts, and collecting whichever toys are her current obsession.

“She is tenacious and, although she tires easily, always gives everything her best efforts.  She is always thinking of others,” Meredith tells us.

Meredith has been busy spearheading the second annual fundraiser called Rea of Hope. The fundraiser will be raising money for an organization called Hope for Marian, which began when another set of parents learned their daughter had NPC. After an experimental medication was noticeably helping their daughter, they began the foundation to support patient advocacy, provide a community for families impacted by NPC, educate about the disease and fight to gain and maintain access to these medications.

“There are so many organizations and people within this community that have supported us. People have donated raffle items and we also have a sign-up list for different activities and it’s just unbelievable how many people have stepped in to help me,” shares Meredith.

The Rea of Hope Fundraiser will take place at Bellingham Common on Saturday, October 14th from 10 a.m. – 2 p.m. It will include all kinds of fun family attractions like local favorite, Trevor the “Games Man”, along with live music, face painting, food trucks, raffles, and more. We encourage everyone to attend and help families impacted by NPC.

You can learn more about the event or purchase a ticket for only $10 per person or $30 per family on www.facebook.com/ReaofHopeNPC

Other ways you can help:

Learn more about NPC and how to help advocate for patient treatment at: https://www.hopeformarian.org/ .