Volume 5, Issue 2

December 2023

Photo Credit: PSC Partners Canada "What The Cell?!" Patient Event

Photo: General session from What the Cell?! on October 14, 2023

Photo: Candle lighting to demonstrate all of those touched by PSC

Photo Credit: CZI Science and Society

Poster Presentation from CZI Science and Society

Watch the Trailer for

A Liver Love Story

Full Video Coming Soon

On October 29, PSC Awareness Day, PSC Partners Seeking a Cure released the trailer for A Liver Love Story. This is a soon-to-be-released short film that follows Jessica Travis, a Canadian PSC patient, as she searches for a liver donor. Niall McKay, who is a PSC patient and liver transplant recipient, is also a writer, editor, Emmy-winning director, and host of PSC Partners' Living with PSC Podcast, is putting the final touches on this moving film, which is set to be released in early 2024. Stay tuned for its release!  

The 19th Annual PSC Partners Patient and Caregiver Conference was held in early September in Henderson, Nevada. PSC patients, caregivers, and loved ones gathered with PSC experts from around the world to share a weekend of learning, collaboration, and support.

Several of the sessions were recorded for those who were unable to attend. Click the following link to see the full breakdown of recordings from the PSC Partners website.

In our after event survey from What The Cell?! we received feedback about people wanting more information about nutrition, diet and supplements. You asked, we listened! Did you know there is an entire webpage dedicated to this very topic on the PSC Partners Seeking a Cure website? There are also a couple of blog posts and a podcast that has been posted by PSC Partners that are linked below.

Although there is no specific diet for PSC patients to follow, there is some information about basic nutritional information for someone who might be experiencing some liver disease symptoms, grocery list and snack ideas as well as links to some previous conference presentations on nutrition and diet. Please be sure to always consult your doctor before taking any supplements or when considering changing your diet. 

Link: Nutrition Information on PSC Partners Website

Link: Dr. Jennifer Lai's talk "Nutrition in PSC" during a general session at the 2023 Annual Conference

Link: PSC Partners Guest Blog: DIET, GUT BACTERIA, AND BILE ACIDS

Link: PSC Partners Blog: NUTRITION UPDATE: 4 STEPS TO FOLLOW A LOW-SODIUM DIET LIKE A PRO

Link: Living With PSC Episode 10 - Dr. Valentina Medici: Nutrition and PSC

To learn more, please email q.rare@uhn.ca or call (416) 340.3131 ext. 6914

For patient participants:

• Receive a self help manual and complete one chapter each week for 6 weeks
• Receive weekly telephone-based support from a peer counsellor for 6 weeks
• Complete 3 questionnaires (compensation will be provided)

You Could Do This Too!

Bring us your fundraising ideas to benefit PSC Partners Canada and we’ll work with you to share the news! Lori knew a local chocolatier, Cocoa Love Chocolates, that makes delicious chocolates and she arranged to sell their handmade vegan chocolates with the proceeds supporting PSC Partners Canada. Her fundraiser was a success, raising $816 for research. Thank you Lori!

Hope for PSC Online Auction & Prize Draw

Thank you to everyone who pulled together to make this auction a huge success!

This was only possible because of each person who donated money or bought an item or donated an item. Thank you! We surpassed our goal and raised $37,938!

Congratulations to Leslie Nicholson, winner of two round trip flights to anywhere in North America that Air Canada flies.

Auction Committee members: Jessica Travis, Alexandra Polletto, Lori Roter, Tracey Berman, Maggie Huang, Virve Aljas, Sandy VanOstrand, Mary Vyas.

WALK83.01

This year the fundraising committee focused efforts on the Hope for PSC Auction. Canada WALK83.01 ran simultaneously and a number individuals took it on and raised great money for PSC research. It’s amazing what you can do when you reach out to your friends and family!

Special thanks to Andy Dench, Matt Hodgson, Team Janssens, “Pretty Serious Canadian” and The Moving Vans for taking the initiative to set up a page and raise money. Together we raised $6,551!

Summary by: Matt Hodgson

The University of Alberta Hospital (UAH) in Edmonton serves as one of the major liver transplantation centres in Western Canada. The UAH is also home to a number of leading research groups focused on gastroenterology and hepatology which include the Gastrointestinal and Liver Disease Research Group (GILDR) and The Centre of Excellence for Gastrointestinal Inflammation and Immunity Research (CEGIIR).

The first liver transplant was performed at the UAH on October 3, 1989. From then through 2022, the UAH performed 2,154 liver transplants. This total comprises both adults (1,827 transplants) and pediatrics (327 transplants).

Patients with PSC listed as their primary diagnosis for transplant represented 8.4% or 182 of the total liver transplants performed at the UAH.



The following timeline highlights some of the key milestones of liver transplant at the UAH.

Source: Liver Transplant Program, Alberta Health Services

Below are additional metrics about the liver transplant program at the UAH, including liver transplants by year, live donor statistics, province of residence for recipients, and patient survival metrics.

Source: Liver Transplant Program, Alberta Health Services

Patient Journey at UAH

Patients in need of a liver transplant are referred to the transplant program by their physician, at which point the referral is reviewed by the triaging hepatologist. Once accepted, the patient is then assigned a pre-transplant coordinator to help guide him/her through the next steps of the journey.

The transplant coordinator establishes contact with the patient or caregiver and arranges a liver transplant assessment. The first step involves the patient receiving a liver transplant package and appointment itinerary. The package includes multiple items requiring completion as well as educational information and the contact information for the transplant coordinator they have been assigned. The coordinator is the go-to resource for assisting with the completion of the liver transplant package.

Once they are listed for transplant, patients are encouraged to keep in close contact with their coordinator to keep them informed of any changes or medical issues that may arise. During this time, it’s important to keep regular follow-up appointments (at least every 3 months) with their referring gastroenterologist or hepatologist.

Patients and their families also get the chance to meet with a social worker during their assessment to learn about the additional resources that are available to them as they continue on their transplant journey.

For more information, Alberta Health Services has these informative videos that outline the process: “Welcome to the Transplant Waitlist” and “Living Liver Donation”.

Written by: Onofrio, Fernanda; Zheng, Katina; Xu, Cherry; Chen, Shiyi; Xu, Wei; Vyas, Mary; Bingham, Katie; Patel, Keyur; Lilly, Leslie; Cattral, Mark; Selzner, Nazia; Jaeckel, Elmar; Tsien, Cynthia; Gulamhusein, Aliya; Hirschfield, Gideon M.; Bhat, Mamatha

Please note this article is posted in Hepatology Communications 7(8):e0219, August 2023. at: 

https://journals.lww.com/hepcomm/fulltext/2023/08010/living_donor_liver_transplantation_can_address.13.aspx

Liver transplantation (LT) is frequently lifesaving for people living with primary sclerosing cholangitis (PSC). PSC patients are waitlisted for LT according to the model for end-stage liver disease-sodium (MELD-Na) score, which may not, however, adequately capture patient needs, in part because of underweighting of the bilirubin.  Therefore, many PSC patients elect to pursue a living donor liver transplant (LDLT).  

LDLT has been demonstrated to have similar excellent outcomes compared to deceased donor liver transplant (DDLT), despite a higher rate of surgical complications.  This study sought to analyze the clinical trajectory over time for patients with PSC once referred for LT, in a mixed deceased donor/living donor transplant program. This study occurred from November 2012 to December 2019 and included all patients with PSC referred for assessment at the University Health Network Liver Transplant Clinic in Toronto.

Of the 172 PSC patients assessed, 84% (n = 144) were listed of whom 74% were transplanted. Mean age was 47.6 years, and 66% were male. Overall mortality was 18.2% at 2 years. At listing, 82% (n = 118) had a potential living donor (pLD). Patients with pLD had significantly lower waitlist and liver-related waitlist mortality and higher rates of transplantation. Patients who receive LT for PSC have excellent outcomes in comparison to non-PSC indications, with five-year survival rates of 75 to 86.4%.  

However, the use of the MELD-Na criteria for transplant allocation has limitations and controversies. The MELD-Na score tends to under-represent the severity and extent of the disease in PSC compared to other forms of liver disease. Since the primary liver dysfunction in PSC manifests as elevation in bilirubin with comparatively lower creatinine and INR, patients will tend to present with a lower MELD-Na score despite being overtly unwell.

The MELD-Na score may also systematically disadvantage patients with PSC because other important elements that affect patients with PSC, such as poor quality of life and increased risk of disease-specific adverse outcomes (recurrent or intractable cholangitis and biliary malignancies) are not captured.  Consequently, wait times on transplant lists for patients with PSC have been typically longer than those for other indications as was also shown in this study, with an average time to transplant was 8.2 months. 

This study shows that patients with PSC benefit from having a pLD in terms of greater overall survival, lower rate of liver-related mortality, and higher rate of transplant. Although a causative factor cannot be determined definitively, these findings suggest that having a pLD is associated with better outcomes. Patients with pLD have also been shown to have greater access to social capital (less likely to be divorced, single or having immigrant status) and financial support, and were more likely to be younger. Social determinants associated with DDLT included closer distance, populous areas, and lower household income. Access to LDLT may be especially important for women with PSC since women’s typically smaller size has disadvantaged their access to DDLT. Therefore, transplant programs incorporating LDLT, as well as transplant policy organizations, must be attuned to these potential equity challenges. The addition of sex in the new MELD 3.0 score may provide greater access to transplantation for women with PSC once implemented.  Equity of access to living donor transplantation needs to be enhanced to ensure equity of opportunity for a lifesaving procedure.

This news feature from CTV Calgary caught our eye from back in the summer: Calgarian makes a life-saving plea on World Organ Donation Day.

Jonathan Ferguson says the lead up to his thirtieth birthday was spent being misdiagnosed.

He never quite felt right: and it took him years before anyone was able to correctly identify why.



It wasn't good news.

"I was diagnosed with a rare liver disease called Primary Sclerosing Cholangitis," Jonathan told CTV News. "I looked awful. I had jaundice, I was very gaunt. It wasn't fun."

Looking back Sunday, 13 years later, the Calgarian says it flipped not only his health on its head, but his life outlook.

"It became about family and travel," he says. "I visited 80 counties, a lot of them with my mom."

But eventually, PSC slowed him down. And, he was faced with a tough situation: Jonathan needed a transplant, and time was limited.

Photo Credit from CTV Calgary: Daniel, left, and Jonathan Ferguson at hospital in 2020 when Daniel donated part of his liver to Jonathan, his brother. (Supplied)

"The list is huge," he said, pointing to the more than 4,300 Canadian names on the transplant waitlist. "I thought a cadaver liver donation might happen, but it fell (through)."

So, Jonathan was left with another option: living donation.

"It was just frustrating for the whole family to see him decline over time," his brother Daniel said. "So I said 'this is something I can do.'"

Check out the full story here from CTV News Calgary. To learn more about Organ Donation in Canada, please visit the Government of Canada's information page.

Wednesday, December 6, 2023



Multiple Adults ZoomRoom Support Group, including Those with PSC, Post-Transplant Folks, Spouses, Parents, and Caregivers

Join this virtual peer-to-peer support group to chat with others who truly understand the PSC journey.

► Register Here

Tuesday, January 23, 2024



Adults with PSC & Post Transplant

Looking to connect with other PSCers? Join this virtual peer-to-peer support group to chat with others who truly understand the PSC journey.

► Register Here

PSC Partners Canada uses Facebook regularly to share news of interest to the Canadian PSC community. If you are not a Facebook user, the feed of postings is shared to a page on our website at pscpartners.ca. The Loon Duct is sent quarterly, so if you are wishing for more frequent contact, remember to follow us on Facebook, or check in periodically on the website.