ATHN 11: Liver Transplantation Outcomes Study

NCT ID: NCT04055051

Last Updated: 2023-05-10

Study Results

Results pending

The study team has not published outcome measurements, participant flow, or safety data for this trial yet. Check back later for updates.

Basic Information

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Recruitment Status

COMPLETED

Total Enrollment

86 participants

Study Classification

OBSERVATIONAL

Study Start Date

2020-06-18

Study Completion Date

2022-12-01

Brief Summary

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This study is designed as a multi-center, observational cohort study of participants with hemophilia A and B who have and have not undergone liver transplantation. Participants will be asked to complete health related quality of life questionnaires and provide medical history.

Detailed Description

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At a time when gene therapy is becoming a reality for individuals with hemophilia A and B, little is known about long-term cures. Yet, there are few data to provide real life testing of the impact of long-term cures. Specifically, little is known about the relationship between factor level achieved with gene therapy and clinical outcomes or quality of life. The analogy to liver transplant is clear. There has been no systematic method to collect and analyze the long-term outcomes of liver transplantation in individuals with hemophilia. Previous efforts to access data through the United Network for Organ Sharing national database have been complicated by lack of identification of those with hemophilia. As most of the individuals with hemophilia who undergo liver transplantation receive care at a hemophilia treatment center (HTC), ATHN proposes to leverage its existing relationship with the HTCs to support HTC execution of this study.

ATHN, in collaboration with US Hemophilia Treatment Centers (HTCs), can provide the infrastructure and organization to support a longitudinal cohort study of subjects with congenital hemophilia who have and have not undergone liver transplantation. This cohort study will compare quality of life outcomes between cases that have undergone liver transplantation and controls that have not undergone liver transplantation. The study will determine if factor levels attained post-transplantation correlate with or predict improvement in quality of life measures.

The existence of ATHN and the ATHN System for clinical care and research provides an opportunity to observe a cohort of subjects after liver transplantation with facility. During the study period, it is predicted that sustained normalization in factor VIII or IX levels following liver transplantation improves clinical functioning and quality of life in adults with hemophilia A and B.

All study related contact will be timed to coincide with routine, scheduled care whenever possible. The study schedule will include:

Study enrollment - Study activities will begin after participant consent. Activities include the documentation of relevant medical history and the administration of quality of life (QoL) questionnaires.

Participants will be asked to complete health related (QoL) questionnaires and provide medical history. These questionnaires include:

1. Hemophilia Quality of Life Questionnaire for Adults (Haem-A-QoL): A quality of life questionnaire designed to asses health-related quality of life in adult patients with hemophilia. Domains include physical health, feelings, view of self, sports and leisure, work and school, dealing with hemophilia, treatment, future, family planning, and partnership and sexuality.
2. Patient Reported Outcomes Measurement Information System (PROMIS-29): A quality of life questionnaire designed to assess physical function, anxiety, depression, fatigue, sleep disturbance, ability to participate in social roles and activities, pain interference, and pain intensity.
3. Hemophilia Orthotopic Liver Transplantation QoL Questionnaire (HOT): A quality of life questionnaire, adapted from a cancer survivorship tool, designed to assess adults with hemophilia who have received a liver transplant. Domains include physical well-being, psychological well-being, stress, anxiety, fear, work, family, relationships, and spiritual well-being.

Conditions

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Hemophilia A and B Liver Transplantation

Study Design

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Observational Model Type

COHORT

Study Time Perspective

PROSPECTIVE

Study Groups

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Hemophilia A and B Cases

No intervention. Only patients that have undergone a liver transplant per study eligibility are in this cohort.

No interventions assigned to this group

Hemophilia A and B Controls

No intervention. Comparable patients to those in Case cohort will be put in this cohort.

No interventions assigned to this group

Eligibility Criteria

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Inclusion Criteria

1. Congenital hemophilia A or B of any severity, who have and have not undergone a liver transplant;
2. Age \> 18; and
3. Sex assigned at birth was male

Exclusion Criteria

1\. Age \<18.
Minimum Eligible Age

18 Years

Eligible Sex

ALL

Accepts Healthy Volunteers

No

Sponsors

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BioMarin Pharmaceutical

INDUSTRY

Sponsor Role collaborator

American Thrombosis and Hemostasis Network

NETWORK

Sponsor Role lead

Responsible Party

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Responsibility Role SPONSOR

Principal Investigators

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Margaret Ragni, MD, MPH

Role: PRINCIPAL_INVESTIGATOR

University of Pittsburgh Medical Center

Locations

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Orthopaedic Institute for Children Hemophilia Program

Los Angeles, California, United States

Site Status

University of California, San Francisco

San Francisco, California, United States

Site Status

Bleeding and Clotting Disorders Institute

Peoria, Illinois, United States

Site Status

Indiana Hemophilia and Thrombosis Center (IHTC)

Indianapolis, Indiana, United States

Site Status

Maine Hemophilia and Thrombosis Center

Scarborough, Maine, United States

Site Status

Henry Ford Health System

Detroit, Michigan, United States

Site Status

Mayo Comprehensive Hemophilia Center

Rochester, Minnesota, United States

Site Status

Mary M. Gooley Hemophilia Center, Inc.

Rochester, New York, United States

Site Status

Comprehensive Hemophilia Treatment Center, University of North Carolina at Chapel Hill

Chapel Hill, North Carolina, United States

Site Status

Ohio State University Medical Center Hemophilia Treatment Center

Columbus, Ohio, United States

Site Status

University of Pittsburgh

Pittsburgh, Pennsylvania, United States

Site Status

Gulf States Hemophilia and Thrombophilia Center

Houston, Texas, United States

Site Status

Utah Center for Bleeding and Clotting Disorders

Salt Lake City, Utah, United States

Site Status

Hemophilia Outreach Center Green Bay

Green Bay, Wisconsin, United States

Site Status

Comprehensive Center for Bleeding Disorders, Milwaukee

Milwaukee, Wisconsin, United States

Site Status

Countries

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United States

References

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Madeira CL, Layman ME, de Vera RE, Fontes PA, Ragni MV. Extrahepatic factor VIII production in transplant recipient of hemophilia donor liver. Blood. 2009 May 21;113(21):5364-5. doi: 10.1182/blood-2009-02-206979. No abstract available.

Reference Type BACKGROUND
PMID: 19470440 (View on PubMed)

Subramanian A, Sulkowski M, Barin B, Stablein D, Curry M, Nissen N, Dove L, Roland M, Florman S, Blumberg E, Stosor V, Jayaweera DT, Huprikar S, Fung J, Pruett T, Stock P, Ragni M. MELD score is an important predictor of pretransplantation mortality in HIV-infected liver transplant candidates. Gastroenterology. 2010 Jan;138(1):159-64. doi: 10.1053/j.gastro.2009.09.053. Epub 2009 Sep 30.

Reference Type BACKGROUND
PMID: 19800334 (View on PubMed)

Ragni MV, Moore CG, Soadwa K, Nalesnik MA, Zajko AB, Cortese-Hassett A, Whiteside TL, Hart S, Zeevi A, Li J, Shaikh OS; HHH Study Group. Impact of HIV on liver fibrosis in men with hepatitis C infection and haemophilia. Haemophilia. 2011 Jan;17(1):103-11. doi: 10.1111/j.1365-2516.2010.02366.x. Epub 2010 Aug 16.

Reference Type BACKGROUND
PMID: 20722744 (View on PubMed)

Ragni MV, Devera ME, Roland ME, Wong M, Stosor V, Sherman KE, Hardy D, Blumberg E, Fung J, Barin B, Stablein D, Stock PG. Liver transplant outcomes in HIV+ haemophilic men. Haemophilia. 2013 Jan;19(1):134-40. doi: 10.1111/j.1365-2516.2012.02905.x. Epub 2012 Jul 5.

Reference Type BACKGROUND
PMID: 22762561 (View on PubMed)

Terrault NA, Roland ME, Schiano T, Dove L, Wong MT, Poordad F, Ragni MV, Barin B, Simon D, Olthoff KM, Johnson L, Stosor V, Jayaweera D, Fung J, Sherman KE, Subramanian A, Millis JM, Slakey D, Berg CL, Carlson L, Ferrell L, Stablein DM, Odim J, Fox L, Stock PG; Solid Organ Transplantation in HIV: Multi-Site Study Investigators. Outcomes of liver transplant recipients with hepatitis C and human immunodeficiency virus coinfection. Liver Transpl. 2012 Jun;18(6):716-26. doi: 10.1002/lt.23411.

Reference Type BACKGROUND
PMID: 22328294 (View on PubMed)

Aguero F, Rimola A, Stock P, Grossi P, Rockstroh JK, Agarwal K, Garzoni C, Barcan LA, Maltez F, Manzardo C, Mari M, Ragni MV, Anadol E, Di Benedetto F, Nishida S, Gastaca M, Miro JM; FIPSE/NIH HIVTR/NEAT023 Investigators. Liver Retransplantation in Patients With HIV-1 Infection: An International Multicenter Cohort Study. Am J Transplant. 2016 Feb;16(2):679-87. doi: 10.1111/ajt.13461. Epub 2015 Sep 28.

Reference Type BACKGROUND
PMID: 26415077 (View on PubMed)

Mehta KD, Ragni MV. Bleeding and liver transplantation outcomes in haemophilia. Haemophilia. 2017 Mar;23(2):230-237. doi: 10.1111/hae.13104. Epub 2016 Nov 4.

Reference Type BACKGROUND
PMID: 27813318 (View on PubMed)

Ragni MV, Humar A, Stock PG, Blumberg EA, Eghtesad B, Fung JJ, Stosor V, Nissen N, Wong MT, Sherman KE, Stablein DM, Barin B. Hemophilia Liver Transplantation Observational Study. Liver Transpl. 2017 Jun;23(6):762-768. doi: 10.1002/lt.24688.

Reference Type BACKGROUND
PMID: 27935212 (View on PubMed)

Ragni MV, Nalesnik MA, Schillo R, Dang Q. Highly active antiretroviral therapy improves ESLD-free survival in HIV-HCV co-infection. Haemophilia. 2009 Mar;15(2):552-8. doi: 10.1111/j.1365-2516.2008.01935.x.

Reference Type BACKGROUND
PMID: 19347994 (View on PubMed)

Other Identifiers

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ATHN 11: Liver Transplantation

Identifier Type: -

Identifier Source: org_study_id

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