Clinical Trial: The Dallas Hereditary Spherocytosis Cohort Study

Study Status: Terminated
Recruit Status: Terminated
Study Type: Observational

Official Title: The Dallas Hereditary Spherocytosis Cohort Study

Brief Summary:

The purpose of this study is to

  1. better characterize the short term and long term natural history of hereditary spherocytosis (HS) including diagnosis, complications, and indications for and response to splenectomy
  2. evaluate and describe the health-related quality of life in children with HS.

Detailed Summary:

Patients with a new or established diagnosis of HS seen at Children's Medical Center will be asked to enroll in the study. Previous and current medical records will be reviewed to systematically catalogue their history of HS, including diagnosis, complications, hospitalizations, medications and laboratory data. Health-related quality of life questionnaires will be given to the patients and their parents at enrollment and periodically during the follow-up. Those who agree will have up to three small samples of blood collected and frozen for future laboratory studies of complications associated with HS and/or splenectomy.

We anticipate enrolling approximately 200 children and young adults with HS in this study and following them until adulthood (age 18-21 years).


Sponsor: University of Texas Southwestern Medical Center

Current Primary Outcome: Health related quality of life [ Time Frame: Approximately at 5 years ]

PedsQL measurement


Original Primary Outcome: Same as current

Current Secondary Outcome:

  • Primary indications for splenectomy [ Time Frame: Approximately at 5 years ]
    Primary indication for splenectomy determined at time of splenectomy, if performed.
  • Complications of HS [ Time Frame: Approximately at 5 years and at 10 years ]
  • Complications of splenectomy [ Time Frame: Approximately at 5 years ]
  • Diagnosis of HS [ Time Frame: At enrollment ]


Original Secondary Outcome: Same as current

Information By: University of Texas Southwestern Medical Center

Dates:
Date Received: June 4, 2010
Date Started: May 2010
Date Completion:
Last Updated: May 13, 2013
Last Verified: May 2013