Alterations in Coagulation Factor Levels in Patients With End Stage Liver Disease

Study Overview
=================
Brief Summary
-----------------
The goal of this observational study is to learn about the changes in coagulation factor VIII and IX levels in patients undergoing liver transplantation to help guide future management of coagulation factor replacement in patients with hemophilia and liver disease. The question we aim to answer is: should the recommendations for factor replacement in patients with hereditary bleeding disorders be altered in the setting of end stage liver cirrhosis? Participants will be asked to provide two blood samples, one at the beginning of their liver transplant, and one after their liver transplant.

Detailed Description
-----------------
Current guidelines for management of hemophilia B suggest replacement of factor IX to 100% prior to major abdominal surgery. However, in patients with concurrent liver cirrhosis where the liver does not produce Factor IX, is it worth considering adjusting the factor replacement strategy? We recently had a case of a patient with Hemophilia B and end-stage liver disease (ESLD) who underwent orthotopic liver transplantation and received the standard pre-operative recombinant factor IX replacement. His case was complicated by intra-cardiac thrombus and hypotension. We conduct a small study to assess the pre-operative thromboelastography (TEG) and factor levels in ESLD patient which we hope will help guide clinical decision making in future hemophilia B patients with cirrhosis.

Official Title
-----------------
Understanding the Alterations in Coagulation Factor Levels in Patients With End Stage Liver Disease

Conditions
-----------------
Liver Cirrhosis, Hemophilia, Hemophilia A, Hemophilia B, Liver Transplant Disorder, Liver Failure, Coagulation Factor Deficiency

Participation Criteria
=================
Eligibility Criteria
-----------------
Inclusion Criteria: participants who have end stage liver disease who are listed for liver transplantation and have an accepted organ offer age > 18+ MELD > 25 Exclusion Criteria: undergoing multi-organ transplant tumor MELD exception points has hereditary coagulation disease currently on therapeutic blood thinner or anti-platelet medication (ie. aspirin, plavix, warfarin, heparin)

Ages Eligible for Study
-----------------
Minimum Age: 18 Years

Sexes Eligible for Study
-----------------
All

Accepts Healthy Volunteers
-----------------
No

Study Plan
=================
How is the study designed?
-----------------

Arms and Interventions

| Participant Group/Arm | Intervention/Treatment |
| --- | --- |
| ESLD<br>All participants recruited will belong to this group. These participants will all have end stage liver disease and be listed for liver transplant with an accepted organ offer. | |

What is the study measuring?
-----------------
Primary Outcome Measures

| Outcome Measure | Measure Description | Time Frame |
| --- | --- | --- |
| Factor VIII level | We will collect Factor VIII level pre- and post- transplant | 12 hours | 
| Factor IX level | We will collect Factor IX level pre- and post- transplant | 12 hours | 
| Thromboelastography (TEG) values | We will collect thromboelastography values pre- and post- transplant | 12 hours | 

Secondary Outcome Measures

| Outcome Measure | Measure Description | Time Frame |
| --- | --- | --- |
| Complications | We will collect data on bleeding or clotting events during the liver transplant surgery and 24 hours post-operatively | 24 hours | 

 Terms related to the study
=================
Keywords Provided by Centre Hospitalier Valida
-----------------
liver transplant, hemophilia, coagulation, thrombosis, bleeding, cirrhosis