The Dallas Hereditary Spherocytosis Cohort Study

• Primary indications for splenectomy [ Time Frame: Approximately at 5 years ] [ Designated as safety issue: No ]
  Primary indication for splenectomy determined at time of splenectomy, if performed.
• Complications of HS [ Time Frame: Approximately at 5 years and at 10 years ] [ Designated as safety issue: No ]
• Complications of splenectomy [ Time Frame: Approximately at 5 years ] [ Designated as safety issue: No ]
• Diagnosis of HS [ Time Frame: At enrollment ] [ Designated as safety issue: No ]

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.

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).

Center for Cancer and Blood Disorders outpatient clinics

Inclusion Criteria:

• Diagnosis of HS with or without prior splenectomy
• Age 0 - 21 years
• Spanish-speaking subjects are eligible to participate

Exclusion Criteria:

• Unable to provide contact information for follow-up