Understanding Immune Thrombocytopenia Purpura (ITP) from the Patient and Family Perspective

Principal Investigator: Mark Fluchel
Keywords: Immune Thrombocytopenia , Pediatrics Department: Pediatric Administration
IRB Number: 00063236 Co Investigator:  
Specialty: Pediatric Surgery
Sub Specialties:
Recruitment Status: Not yet recruiting

Contact Information

Mark Fluchel
mark.fluchel@imail.org
801-662-4740

Brief Summary

To develop a database of children diagnosed with or treated for Immune Thrombocytopenia Purpura (ITP) for use in current and future research.  The implementation of a survey to assess the impact the burden of care on families that is associated with the treatment of a family member with childhood ITP.  The survey will also include questions to aid in identifying any familial association ITP may have to determine the relative risk within families.  Because there is evidence of an association between the burden of care in ITP, histiocytosis and cancer, the results gathered from the survey will be used for comparison with a similar studies involving the impact of the burden of care of pediatric ITP patients and their families.

The ultimate goal of this project is to use the data obtained to identify the similar burdens families with pediatric ITP patients have with other related diseases.  The expansion of this project is to include a thorough evaluation of barriers to pediatric histiocytosis care.

For the survey aspect of the study, we ask for the home address to assess the impact of location of residence on burden.  For the UPDB aspect (i.e. attempting to assess the risk of autoimmune disease and ITP in the relatives of ITP patients), we will not be needing any geographical information.

Study Objectives:

Specific Aim 1: To create an ITP retrospective database of all children diagnosed or treated for ITP at Primary Children’s Hospital (PCH) in the last 10+ years (January 1, 2005 to May 1, 2015).

Specific Aim 2:  To create an ITP prospective database of current and new children diagnosed with or treated for ITP at PCH.

Specific Aim 3: To search for familial associations of ITP using the Utah Population Database and the UPDB kinship analysis tools.  (No geographical identifiers will be requested from the UPDB)

Specific Aim 3a: Use the Utah Population Database to search for familial associations of ITP with other autoimmune conditions.

Specific Aim 3b: Use the Utah Population Database to search for familial associations between individuals with known ITP.

Specific Aim 3c: Use the Utah Population Database to compare familial associations in ITP patients who appear to have a genetic explanation for their disease versus those who do not have any genetic explanation for their disease

Specific Aim 4:  To evaluate the burden of care and care experience associated with the treatment of ITP using a patient/family survey at PCH and nationally via the PDSA (Platelet Disorder Support Association).  (Participants will be asked to report their current address to assess the impact of location of residence on burden of care for the survey portion of the study only)

Specific Aim 5:  To compare the results with other similar studies.

Inclusion Criteria

Criteria for inclusion and the method of recruitment differ depending on the aspect of the study.

1. For the survey portion of the study:

Parents of patients at Primary Children's Hospital who were less than 18 years of age when they were diagnosed with ITP will be approached in clinic. For this portion of the study, the IHC EDW will not be used. Only parents of patients seen in clinic will be approached. The electronic record of the patients (HELP 2) will be reviewed to verify their diagnosis. Debbie K.M. Nelson and Mark Fluchel will be reviewing the charts.

Members of the PDSA support group who are parents of patients who were less than 18 years of age when they were diagnosed with ITP will be contacted via the PDSA support group and asked to participate.

2. For the UPDB aspect of the study (ie. to determine familial links with ITP)

The EDW and UUHSC will be used to identify a cohort of ITP patients that will be used in the UPDB as index cases. Our interest is in determining how those index cases are related to one another and to determine the risk of autoimmune disorders in their first degree relatives.  The initial cohort will be patients of primary children's hospital.  The 1st-3rd degrees relatives found via the EDW, UUHSC and Utah Population Database are not part of the original cohort, they charts will not be reviewed- the goal is to determine the risk of ITP and other autoimmune diseases in families with children diagnosed with ITP.

The electronic record of the patients (Help 2) will be reviewed to verify their diagnosis. Debbie K.M. Nelson and Mark Fluchel will be reviewing the charts.

3.  ICD-9 codes to be studied:

Immune Thrombocytopenic Purpura / Idiopathic Thrombocytopenic Purpura   (287.31)

Hemolytic Anemia  (283.0 is autoimmune HA)

Systemic lupus erythematosus (710.0 is SLE, 695.4 is discoid or non systemic)

Myasthenia gravis (358.0-358.01)

Membranous nephropathy

Thyroid disease (disorders of the thyroid 240-246)

            Grave’s Disease (242.0)

            Hashimoto’s Thyroiditis (245.2)

Rheumatoid Arthritis (714.0-714.2 inclusive, also V82.1 is RA)

Juvenile Rheumatoid Arthritis (714.30-714.33 inclusive)

Crohn’s Disease (555.0-555.1, also 555.9)

Ulcerative Colitis (556, including 556.0-556.9 inclusive)

Type I Diabetes Mellitus (250.0-250.9 inclusive)

Ankylosing Spondilitis (iritis, HLA-B27) (720-720.9 inclusive)

Autoimmune disease NOS   279.4

 

 

 

Exclusion Criteria

None.