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ICU Nurse Co-Founds First Charitable Organization For Progressive Familial Intrahepatic Cholestasis, The Rare Disease That Affects Her Own Daughter | NEWS-Line for Nurses

ICU Nurse Co-Founds First Charitable Organization For Progressive Familial Intrahepatic Cholestasis, The Rare Disease That Affects Her Own Daughter


Louisville, Kentucky, ICU nurse Emily Ventura is mother to Cedar, a seven-year-old with a rare and life-threatening disease called progressive familial intrahepatic cholestasis (PFIC). When Cedar was diagnosed as an infant, Emily searched for information about PFIC and for a chance to connect with other parents, but there was little available. So, she along with two other moms she met online, Tara Kerns and Melanie Karakaidos, set out to create a supportive community by co-founding the charitable organization, PFIC Advocacy and Resource Network, LLC (PFIC Network).

As the only advocacy organization for families affected by PFIC in the U.S., the nonprofit has come a long way since its inception. In June, PFIC Network teamed up with Alagille Syndrome Alliance, another rare liver disease advocacy group, to arrange the first-ever PFIC Family Conference in Cincinnati, OH.

A total of 22 families affected by PFIC gathered to share experiences and support during this historic event. Clinicians, industry partners, researchers and advocates also attended and presented updates on research, nutrition, and patient management. In a particularly poignant moment, Emily was able to finally meet in person parents she’d previously counseled online or over the phone.

Emily noted that meeting others who understand the challenges of PFIC was invaluable, and families shared many insights on how to access help and handle different situations effectively. “There was an instant connection,” said Emily. “Every family’s journey with PFIC is different, but at some point, all of our stories overlapped. All the kids use multiple medications and live with severe itching. When we were together, they were able to just play and be kids.”

Children born with PFIC (about one in every 50,000 to 100,000 live births worldwide) have a genetic mutation that affects liver bile excretion. Bile acids accumulate to high levels in the liver and bloodstream, and cause symptoms, including jaundice (yellowing) and pruritus (severe itching). PFIC may increase risk of cirrhosis and liver failure, and in many cases, patients experience end-stage liver disease by the time they are ten years old. There are no approved pharmacologic treatments for PFIC, and there is a need for more research and treatment options, as most patients resort to surgical interventions, including liver transplant.

But there may be hope on the horizon. One life sciences company, Albireo Pharma, Inc., is working to evaluate an investigational drug candidate called odevixibat in patients with PFIC (subtypes 1 or 2) in a global Phase 3 clinical trial that is actively recruiting. Topline results from clinical research are expected in mid-2020.

At five months old, Cedar developed unexplained swelling and bruising on her back and shoulder. A trip to the hospital confirmed that she was in liver failure, but no one could understand why. After nearly a month of testing, Cedar was diagnosed PFIC type 2 (PFIC 2) and she experienced insatiable all-over itching, one of the most severe manifestations of liver disease. "You're trying to take in this heavy, heavy diagnosis, but you also have a baby that's in agony because of itching," Emily said.

"I was a first-time mom who was just trying to get through daily life, as well."

Because she was experiencing rapid liver decompensation, Cedar underwent liver transplant at five years old. Now, she is closely monitored due to the risk of complications and suppressed immunity associated with transplant surgery. She will likely be on immunosuppressive medications for the rest of her life. A common cold could cause her serious harm, so to reduce her risk of infections, she has never traveled on an airplane and she is home schooled.

Emily continues her work in helping other families affected by PFIC across the U.S. Her experience as a nurse has provided her with many important insights about supporting others. One thing Emily is particularly interested in is progress in research for PFIC. “I chose to share our experience in hopes that others will also share their experience and help increase PFIC awareness,” said Emily. “I am passionate about science and research and about understanding what is possible for PFIC in the future.”

To learn more about PFIC, please visit PFIC Network or PFIC Voices.

Source:PFIC Network

Photo Credit:Emily Ventura

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