Liver Transplant For Patients with PSC Jennifer Guy, MD, MAS Director, Liver Cancer Program Transplant Hepatologist California Pacific Medical Center

Liver Transplant Questions Who needs a liver transplant for PSC? What is a liver transplant? How do you get a liver transplant? What is life like after liver transplant? Healing is an art form; there is the science and the art.

Why do patients with PSC need a liver transplant? Recurrent and Severe Episodes of Cholangitis = Infections in Damaged Bile Ducts Progressive Scarring Causing Cirrhosis That is Symptomatic Any liver disease can cause inflammation and injury – we’ll go through what are some of the most common If the liver disease or injury is chronic or persistent it can cause scarring and eventually can cause scarring throughout the entire liver - cirrhosis Cholangiocarcinoma Abnormal growth of cancerous cells in the bile duct

How to determine severity of cirrhosis? Symptom Lab Swelling Creatinine, sodium (albumin) Bleeding INR (platelets) Jaundice Bilirubin Confusion (ammonia)

MELDNa Score Predicts Death in Patients with Cirrhosis MELD = Model for End Stage Liver Disease MELDNa = MELD Sodium Score (NA) Equation that Includes INR, Bilirubin, Sodium and Creatinine Low Score Good High Score Bad Data from Chronic Liver Disease Surveillance Network from 1999-2001 to get a population based assessment of the burden of chronic liver diseaes. Wiesner, et al

UNOS MELDNa Score Calculator Data from Chronic Liver Disease Surveillance Network from 1999-2001 to get a population based assessment of the burden of chronic liver diseaes. . https://optn.transplant.hrsa.gov/resources/allocation-calculators/meld-calculator/

Who Needs a Transplant? Not everyone with PSC needs a transplant 5% of all transplants in the US are for PSC 50-75% of patients with PSC on the wait list have cirrhosis 40% of patients with PSC on wait list have had a prior episode of cholangitis 30% of patients on the wait list will have another episode of cholangitis Khungar, V et al. 2016 Pena Polanco, et al 2017

Liver Transplant Questions Who needs a liver transplant for PSC? What is a liver transplant? How do you get a liver transplant? What is life like after liver transplant? Healing is an art form; there is the science and the art.

Deceased Donor Liver Transplant Data from Chronic Liver Disease Surveillance Network from 1999-2001 to get a population based assessment of the burden of chronic liver diseaes. Bell B et al AJG 2008; 103:2727-2736.

Living Donor Liver Transplant Data from Chronic Liver Disease Surveillance Network from 1999-2001 to get a population based assessment of the burden of chronic liver diseaes. PSC Patients are more likely than patients with other causes of liver disease to undergo LDLT Bell B et al 2008 Goldberg, D et al 2011

Liver Transplant Questions Who needs a liver transplant for PSC? What is a liver transplant? How do you get a liver transplant? What is life like after liver transplant? Healing is an art form; there is the science and the art.

Evaluation: Meeting the Team Liver Doctor (Hepatologist) staff scheduler Liver Surgeon NPs/PA The Patient and caregivers! Nurse Social worker cardiology, psychiatry Phlebotomists/ Radiologiy techs Financial Coordinator Support Group Members You LEARN about liver transplant , MELD allocation, logistics, the surgery, the medications We LEARN about your liver, medical conditions, who you and your caregivers are

Liver Transplant Evaluation Your goals: Do I understand why I need a transplant? What are the benefits and risks of transplant? How can I expect to feel physically before and after transplant? What should I expect mentally before and after transplant? What resources are available to me? Who do I call if I don’t feel well or have a question?

Liver Transplant Evaluation Transplant committee goals: Do you meet medical criteria for a transplant? Is there anything medically or socially that makes transplant too high risk for you? Justice Utility

How do I get a liver transplant? Step 1: Referred and Evaluated for Transplant Step 2: Approved for Being Placed on the Wait List at the Transplant Center (s) Step 3: Priority on the Wait List is determined by how sick each person is, as measured by the MELDNa Score Step 4: You are matched with an organ offer when your MELDNa Score is at the top of the wait list

MELDNa Score Predicts Death in Patients with Cirrhosis MELD = Model for End Stage Liver Disease MELDNa = MELD Sodium Score (NA) Equation that Includes INR, Bilirubin, Sodium and Creatinine Low Score Good High Score Bad Data from Chronic Liver Disease Surveillance Network from 1999-2001 to get a population based assessment of the burden of chronic liver diseaes. Wiesner, et al

How to account for recurrent cholangitis ? Your transplant team can petition to the United Network of Organ Sharing (UNOS) regional review board for extra points, called an Exception Score Guidelines for awarding an Exception Score for recurrent cholangitis include evidence of multiple, severe infections If meets criteria, the MELDNa score that is requested is granted and requires re-certification every three months Exception criteria also apply to certain cases of cholangiocarcinoma, where transplant is done at specific centers

MELD at Transplant Varies by Region MELD Scores at Transplant Also Vary by Blood Type High MELD Score Data from Chronic Liver Disease Surveillance Network from 1999-2001 to get a population based assessment of the burden of chronic liver diseaes. Low MELD Score Source: Politico 6/21/15

Liver Transplant Questions Who needs a liver transplant for PSC? What is a liver transplant? How do you get a liver transplant? What is life like after transplant? Healing is an art form; there is the science and the art.

Survival after Transplant for PSC is Excellent PSC/PBC Data from Chronic Liver Disease Surveillance Network from 1999-2001 to get a population based assessment of the burden of chronic liver diseaes. Data Source: UNOS thru 2004 . Liver Transplantation 2004

Survival after Transplant for PSC is Excellent Summary of small letters and numbers: 85% 5 year survival Data from Chronic Liver Disease Surveillance Network from 1999-2001 to get a population based assessment of the burden of chronic liver diseaes. . Pena Polanco, et al. 2017

Considerations after liver transplant Liver transplant is a “chronic cure” Medications to prevent rejection lifelong Medications to prevent infection in the early months post transplant Potential for side effects from medications The risk of recurrent PSC is up to 22% by 5 years Surgical risks are low but surgical strictures can occur which require ERCP and stenting Clinical course of IBD is variable post transplant Adjustment to new phase -- living without PSC but living with a transplant

Working as a Team Patients and caregivers should feel empowered to advocate Communicate goals and set expectations Be open to hearing the good and the bad Take notes Make a list of questions to review at your appointments Let your team know if your needs are not being met Patients should be aware that doctors/teams do not always have records/latest labs/necessary information at all times Get copies of results and information if you can Back up plans should be discussed If I get sick, who should I call? If plan A doesn’t work, what is plan B? If you want straight talk, ask for it Know what resources are available and use them - Nutrition, social work, PSC Partners, support groups, mindfulness classes

Thank you!