Volume 3, Issue 1
September 2021
Canada Auction a Success!Clinical Trial Shows a Third COVID-19 Vaccine Shot Protects Transplant Patients Call for Stories from the PSC Community Living Donor Week  Living Donor Circle of Excellence Living Organ Donation-Why it Matters for PSC Crafted by Community: Inspirational Athletes Update on Research : Organ-whats? Upcoming PSC Related Events 2022 Annual Conference
Our first ever Online Auction for PSC Partners Canada was a huge success!

The idea grew as our committee worked hard to put the auction together. We reached out to our PSC Community and they came through! 
Together we raised a total of $30,000!!!!
An unexpected benefit of the auction was the feeling of connection that developed within our PSC community. People with PSC often feel quite alone, yet with the auction it was obvious that there was an active community wanting to offer their support. Several PSCers commented that they felt less alone as they scrolled through the auction items and bid on a few of them, often finding they were outbid by people bidding above value for items. Thank you to all of the generous bidders! A total of 126 items were sold.

A HUGE thank you to our fundraising team and to all those who donated, volunteered their time, provided their input, shared their story and spread the word to their networks. This is just a small testament to what an amazing community we are privileged to be a part of. When we put our voices and resources together, we can exceed our expectations and create a real impact!

Submitted on behalf of committee: Sandy VanOstrand, Jessica Travis, Maggie Huang, Mary Vyas
Thank you for your continued support to PSC Partners Seeking a Cure Canada over the years!
Clinical Trial Shows a
Third COVID-19 Vaccine Shot
Protects Transplant Patients
Researchers at the Ajmera Transplant Centre at UHN have conducted a first-in-the-world trial testing whether third-dose COVID-19 booster vaccines can increase transplant patients’ immune response to the virus. The results of this study, published in the New England Journal of Medicine shows a third booster shot substantially improved protection for these patients. This is particularly important because transplant recipients are at a greater risk of COVID-19 due to the immunosuppressive medications they must take.

“We knew from previous studies that two doses were not enough to produce a good immune response against COVID-19 in transplant patients,” says Dr. Deepali Kumar, Director of Transplant Infectious Diseases, Ajmera Transplant Centre and joint-senior author of the study. “Based on our study, a third dose of COVID vaccine is definitely the best way to increase protection.”

Half of the participants received a third shot of the vaccine two months after their second dose, while the other half received a placebo, allowing researchers to compare the impacts of a third dose against a control group.

Study researchers hoped that this third booster shot would cause recipients’ immune systems to generate higher antibody levels against the spike protein of the virus. In the placebo group, the response rate was only 18 per cent whereas in the Moderna three-dose group, the response rate was 55 per cent.

Dr. Atul Humar, Medical Director of the Ajmera Transplant Centre and the joint-senior author of the clinical trial stated “The third dose was safe and well tolerated and should lead to a change in practice of giving third doses to this vulnerable population.”

Improving the body’s ability to fight back against COVID-19

In addition to the study’s goal of increasing overall antibody levels, the researchers also looked at the effectiveness of neutralizing antibodies, which neutralize the virus. 60 per cent of the patients who received a third dose of the Moderna vaccine developed neutralizing antibodies compared to 25 per cent in the placebo group.

Additionally, the third booster vaccine was very well tolerated with only mild side effects and did not cause acute organ rejections. 

Source: UHN Article
As of September 5, Ontario, Quebec, and Alberta are offering a third shot of the COVID-19 vaccine to those with comprised immune systems, whereas British Columbia is preparing for this possibility. 

Please contact your local health unit or doctor for more information about the third COVID-19 vaccine dose if you are immune compromised.  
Calling all Canadian PSCers! 
We are looking for people to share a few sentences, a short one minute clip, or some photos about your PSC story.
No matter if you are a caregiver, or a PSCer, we would like to get to know our fellow Canadian PSCers and share your story to our social media pages or featured in the Loon Duct's "Stories from the PSC Community"

Here are a few ideas of what you could tell us about:
  • How has PSC affected your life?
  • What is something that helps you through your tough days?
  • What would be the one thing you would want people to know most about PSC?
  • If you could share one picture/image or say one word to describe what it’s like living with/seeing something live with PSC what would it be?
  • What is something good that has come with your experience living with/caring for someone who has PSC?
Share your story by emailing us at ContactUs@PSCPartners.ca
Learn More About Living Organ Donation 
Living Donation Week 
Every year, living donor transplants save the lives of hundreds of Canadians awaiting a liver or kidney transplant. Living donation can offer a shorter wait time than a deceased donor transplant and could provide patients with a quicker route to better health.
Living Donation Week is a national awareness week that will take place from September 12 - 18th. This week is an opportunity to spread the word and educate Canadians about living donation and will provide free information sessions and participation events.

The Celebration event for living donors will kick off the week on Monday, September 13 at 12:00pm EST to recognize those who donated between January 1, 2019 and December 31, 2020.

Events that will occur during this week are on:
  • Tuesday, September 14, 12:00pm EST, a professional education webinar will cover indications for liver transplantation, listing criteria and referral process, living donor assessment and more.
  • Wednesday, September 15, 12:00 pm – 2:00 pm EST, a peer-led online workshop will discuss how to find a living donor. This interactive workshop will provide an overview and tips of how to facilitate a campaign to search for a living donor.

A Canada-wide live pitch competition will take place on Friday, September 17 at 12pm EST. #LDW2021

Register for this free event:
Living Donor Circle of Excellence

It is a corporate recognition program to promote and celebrate companies that implement internal policies to help support employees who volunteer to be living organ donors. The goal of the program is to eliminate the financial costs of living organ donation for donors. A major disincentive for many donors is the financial burden of being a living kidney or liver donor. A leave for a kidney donor is typically 4 weeks, and is longer for a liver donor. Not many companies have policies that cover the cost of lost wages. Living organ donors will often use their vacation time or take an unpaid leave of absence in order to be able to take the time off work necessary to donate. Other out of pocket expenses may be incurred such as travel and lodging expenses.
The aim of this program is to provide recognition with the Circle of Excellence Seal to companies who provide lost wages to living donors. This could be done by implementing internal policies to provide salary support for an employee who chooses to be a living donor and/or the living donor of an employee who lacks salary support during their recovery period. By eliminating the financial barriers during the recovery period, this could increase living donor transplants.

The benefit for companies who implement these types of policies will not only be recognized with the Circle of Excellence Seal, but it may help them attract and retain employees, and this may help their employees return to work sooner.
Crafted by Community
Content created by members of the Canadian PSC community
Living Organ Donation
Why it Matters for PSC
By: Virve Aljas
On July 29, I attended a Pathways to Liver Donation online webinar. This interactive webinar was specifically about living organ donation, facilitated by Transplant Manitoba and the Centre for Living Organ Donation, at the University Health Network in Toronto. You can view the recorded video of the July 29 session here, and for archived and future videos you can subscribe to their YouTube channel. It’s a fantastic resource for reputable information and for inspirational stories about living donations.
Living organ donation is an important topic for PSC patients to learn about. In short, there are a variety of reasons why it is beneficial for PSCers to receive an organ from a living donor as opposed to one from a deceased donor. The session packed a large amount of information into an hour, discussing trends in liver disease and treatments, deceased vs. living organ donation, life after transplant, the process for assessing living donors as well as experiences from liver recipients and living donors.

Living Organ Donation and MELD Scores
A key takeaway that applies strongly to PSC patients is the reMarylationship between living donation and the MELD Score. As you may know, MELD (Model for End-Stage Liver Disease) is a way of evaluating which patients are most urgently in need of a transplant. The score is intended to act as an objective method to establish how “sick” someone is, and is a mathematical formula based on blood test results.

This becomes complicated for PSC patients because we face unique risks due to complications that are specific to our disease, like repeated cholangitis or cholangiocarcinoma. These may not be reflected within the MELD score alone. Additionally, it’s been shown that women score differently on the MELD scale than men due to factors like average body mass. So while a female patient might actually be more ill, the comparative score may not reflect their actual experience.
To receive a transplant from a deceased donor, patients have to walk a fine line between being the sickest among others on the waitlist, yet also healthy enough for their body to accept the transplant. This is where one of the great benefits of living donation comes in. While you still have to register at a certain level on the MELD score, you don’t have to wait until the very end stage. The search for a donor can begin earlier, increasing your chances of finding a match. Aside from the obvious physical benefits of a possible earlier transplant, patients have a sense of control over their situation—a sometimes rare opportunity when you live with this rare disease.

The recorded video goes into further details about other benefits and how the process of finding a liver donor works. I strongly recommend taking a look. See the video here.
Update on 2020 PSC Partners Canada Research Grant on Liver Organoids

Organ-whats?

Creation of liver organoids from pluripotent stem cells derived from donors with PSC
A simplified version of this year's researcher update
First degree relatives of patients with PSC have an approximately 100-fold increased risk of developing PSC compared to the general population. And specific genes are linked to an increased risk of PSC. This strongly suggests that there is an inherited or genetic risk for PSC. A study funded by PSC Partners Canada in 2020 by Drs Mullen and Pratt of Mass General Hospital in Boston is focused on advancing the understanding of how genetics, or the sequence of DNA in patients with PSC, affects the development of the liver, and the interaction between different cell types in the liver.
 
The grant application proposed to develop pluripotent cell lines from patients with PSC, and from healthy controls, and then direct these cells to differentiate into small clusters of cells that contain the cell types that make up the liver. These miniature livers in culture are called liver organoids
By analyzing the genes expressed in the different liver cell types and how these different cell types interact with each other in organoids, the researchers hope to learn how the sequence of DNA in patients with PSC puts them at risk of developing liver failure and cancer. 
 
This project is the first step in developing pluripotent stem cell lines and differentiating them into liver organoids. It will form the foundation for future work to expand the understanding of how genetics affects development of PSC and the risk of liver cancer.
During year one of this two-year project, Drs Mullen and Pratt have reported the following accomplishments: 
 
  • Generated organoids and studied them to determine whether the cell types and interactions are representative of what is seen in a liver. 
  • Performed single cell analysis and confirmed the differentiation of hepatocyte-, cholangiocyte- and stellate cell-like populations.
  • Studied how liver organoids respond in a model of fibrosis and found that cell types and gene expression shift when the liver organoids are treated with a fibrotic signal, called Transforming Growth Factor Beta 1 (TGF-beta). These findings were supported by gene expression, single cell analysis, and collagen protein expression. We also identified new cell populations that are induced with treatment of TGF-beta and the induction of fibrosis. 
  • Evaluated how cells interact with one another and how those interactions change with TGF-beta treatment and the development of fibrosis. As expected, increased interaction was found between stellate cells and cholangiocytes under these conditions.
  • Currently analyzing the effects of biliary injury in this model as well.
In the coming year, Drs Mullen and Pratt plan to perform these analyses in liver organoids produced from induced pluripotent stem cells from blood from donors with PSC and matched controls and will evaluate how these organoids respond to fibrotic stress and bile acid injury. 

These studies will serve as proof of concept to demonstrate that liver organoids can be produced efficiently from induced pluripotent cells from PSC patients. Their analysis may also generate further hypotheses as to how genetic changes in PSC affect cholangiocyte-cholangiocyte interactions and the interface between cholangiocytes and neighboring cell types in the liver.

For more information on this and other PSC Partners- and PSC Partners Canada- funded research grants, visit https://pscpartners.org/research/.

Definitions:
  • Cholangiocyte - epithelial cells lining the intrahepatic and extrahepatic bile ducts; they are heterogeneous in size and function and contribute to bile composition and flow. 1
  • Stellate - a type of cell in the liver that play an important role in the mechanisms of liver injury, regeneration and fibrosis.2
  • Hepatocytes - the major functional cells in the liver that play pivotal roles in metabolism, detoxification, and protein synthesis. Hepatocytes also activate innate immunity against invading microorganisms by secreting innate immunity proteins.3

2 Definition from Cholangiocyte pathobiology
Inspirational Athletes
Profile: Jackson Blizzard

Inspired by the 2021 Olympics, we wanted to take a closer look at the experiences of athletes who have faced the life-changing diagnosis of liver disease. This month we meet Jackson Blizzard—a 25-year-old active in weightlifting and motocross, currently living in Raleigh, North Carolina.

Jackson was initially diagnosed with Vanishing Bile Duct syndrome at 19 years old. Because of how his PSC presented itself, he wasn't officially diagnosed until four years later. He originally only had fatigue and elevated lab results, and then later experienced symptoms of Ulcerative Colitis (UC). After being diagnosed with UC, doctors then concluded he had PSC.
Has sport always been a part of your life?
Yes, I originally started racing motocross at the age of 8 until I was 16. Motorcycles are a part of my family. My brother races and my father raced when he was younger too. My path to weightlifting started in the 7th grade when I wrestled to stay in shape for motocross. I wanted to pursue it more seriously, and continued wrestling through high school. After high school I dabbled in CrossFit, but I mainly enjoyed the Olympic Lifting aspect. Now I train roughly five days a week and compete when I have sufficient time away from my studies. 

What does sport/competition mean to you?
Competition means a tremendous amount to me. Specifically, competing as an individual opposed to as a team. I love the idea that my success is reliant on my own work ethic and not a teammate. Recently, weightlifting has been a way of validating my health and proving to myself that I'm not "that sick". It is almost an added bonus being able to best people that are healthy, knowing all the trials people with PSC go through.
How did you feel when you received your diagnosis?
When I received my initial diagnosis in 2015 I pushed it to the back of my mind and was naïve. It wasn't a tremendous problem in my day-to-day life yet. I also had no gauge for how tired I really was, because losing weight for wrestling leaves you extremely fatigued. After graduation, this fatigue never left and I almost had learned to live with it. Recently I have started to consider the gravity of my diagnosis and have had times where it is overwhelming. But I'm surrounded by incredible people that care for, and support me.
Fatigue is one of the main symptoms that liver disease patients live with. How did you overcome that?
I think the best way of dealing with fatigue is planning your day accordingly and being regimented and strict with yourself. Another thing I often tell people is "you have to lie to yourself". I try to be aware of my internal dialogue and redirect it in a positive direction if I notice it going south. If I start to feel tired, I will repeat positive affirmations in my head or out loud. "You're fine, you can do this." Another quote I love, from my favorite motivational speaker Eric Thomas is, "I can, I will, I must." Eric Thomas, Inky Johnson and Les Brown have helped me tremendously and I listen to them daily.
What would you want to tell other PSC patients?
It's okay and normal to be upset for a little while but don't linger there for too long. Every day you spend down in the dumps is a day you could have spent enjoying the health you have. PSC is a progressive disease and each day you have is a gift. Sometime in the future you might be wishing you feel the way you do now.

Is there anything else you'd like people to know about you?
You have been given the gift of being aware of your own mortality. Many people’s lives end abruptly without truly enjoying their days, and that should be scarier than any PSC diagnosis.
Do you know an inspiring athlete? Tell us about them! Email us at: ContactUs@PSCPartners.ca
Upcoming PSC Related Events!

We have details on Webinars, Peer Group Meetups, and our 2022 Conference! See below!
Join us for
Upcoming Webinars
as part of the
PSC Partners
ROADMAP Initiative
In-Depth... Engaging... Thought-Provoking

The ROADMAP Initiative is an ongoing virtual event conducted by PSC Partners.
Whether you're a PSC Patient, Caregiver, Clinician, or Researcher,
WE NEED YOUR VOICE!
Please mark your calendars for these next two ROADMAP Initiative webinars!
Wednesday, September 29
ROADMAP: Initiative Webinar Research 201.
The PSC Research Landscape: Now and Into the Future Identifying Research Questions

Tuesday, October 26
ROADMAP: Initiative Webinar Research 301.
The PSC Research Landscape: Now and Into the Future Charting a Path Forward

What is the PSC Partners ROADMAP Initiative?
ROADMAP is the acronym for Research Objectives and Development Meetings to Accelerate Progress.

This initiative is the outcome of hard work from the past few years from the PSC Partners community which has included the PSC Partners patient registry, and the Patient Focused Drug Development Forum.
ROADMAP’s goals are to accelerate progress to find therapies and a cure for PSC by:
  • educating the patient community on the research front developments.
  • expanding the researcher base and strengthening multi-stakeholder communication.
  • creating opportunities for researchers and clinicians to engage in meaningful discussions with an informed patient community.

This initiative is about expanding the role of the patients in PSC research and drug development. This is about everyone within the PSC community coming together in a collaborative approach.

Why involve PSC patients in this initiative?
Because PSC patients:
  • bring their trustworthy relationship with the medical community;
  • can best describe their symptoms and quality of life; and
  • bring the urgency of finding effective treatments and a cure.

Logistically these ROADMAP discussions will occur over a series of webinars that will occur from April 2021 to April 2022. They will focus on six major topics which will represent the current gaps in PSC research. Two webinars for each topic will occur. The “101” sessions will be community focused while the “201” sessions will be multi-stakeholder focused.  The topics for discussion will be based upon input from you, PSC Partners leadership, the scientific and medical advisory committee, and from past PSC Partners grant recipients.  All stakeholders are welcome to attend these webinars.

For more information, please visit the ROADMAP initiative page on the PSC Partners website here.
PSC Partners’ ZoomRoom meetings are a great way for Peer-to-Peer Connections!
You're Not Alone Zone!
Tuesday, September 21 at 6 p.m. MT (8pm EST): ZoomRoom: PSC Patients, ages 18 & up

Wednesday, October 13 at 6 p.m. MT (8pm EST): ZoomRoom: Caregivers, Spouses & Parents 

Tuesday, October 19 at 6 p.m. MT (8pm EST): ZoomRoom: PSC Teens, ages 13 - 20 

Wednesday, November 10 at 6 p.m. MT (8pm EST): ZoomRoom: PSC Patients, ages 18 & up

Wednesday, December 1 at 6 p.m. MT (8pm EST): ZoomRoom: Caregivers, Spouses & Parents 

Tuesday, December 14 at 6 p.m. MT (8pm EST): ZoomRoom: PSC Teens, ages 13 - 20 
PSC ZoomRooms are a chance to connect with your peer groups and ask questions. Join and laugh, cry, and connect with old and new friends. ZoomRooms are open to the intended audience only to allow for open communication and relationship building. Advance registration is required.
Please visit the events page on the PSC Partners website for all scheduled ZoomRooms and other events of interest to our community. 
Conference Dates and Location Set for
2022 PSC Partners Conference
June 2 - 5, 2022 in Boston, MA USA
It's with great pleasure that we announce the dates of the 2022 PSC Partners Conference, in collaboration with Dr. Josh Korzenik, Brigham and Women's Hospital, and Dr. Dan Pratt, Massachusetts General Hospital. 

We hope you'll join us in
Boston, Massachusetts,
Thursday, June 2 - Sunday, June 5, 2022.

We are still formulating plans for the conference kick-off, which may start early in the day on Thursday, June 2, so we recommend holding off on making plane reservations until the agenda is finalized.

A link to make reservations at the conference hotel, the Burlington Marriott, will be available ten months prior to the conference. A special rate has been negotiated. Please don't reserve a room until our special conference link is live.

Watch the PSC Partners website, newsletter, and social media for conference updates.

See you soon in Boston in 2022!

FOLLOW PSC PARTNERS CANADA ONLINE!
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.
Our mission is to provide education and support to PSC patients, families and caregivers and to raise funds to research causes, treatments and potential cures for primary sclerosing cholangitis.

PSC Partners Seeking a Cure Canada
A Canadian registered charity affiliated with
PSC Partners Seeking a Cure
2021