Introduction
Treatment for thalassemia has dramatically improved. Patients should live full lives with
careers and children of their own. Unfortunately, many patients die prematurely or develop morbid
preventable complications. Outcomes are far better for patients whose care is coordinated by
thalassemia centers (Modell, B., Khan, M., and Darlison, M. Survival in beta thalassaemia major in the
UK: Data from the UK Thalassaemia Register. Lancet 355 [2000]: 2051–2052. Porter, J.B., and Davis, B.A.
Monitoring chelation therapy to achieve optimal outcome in the treatment of thalassaemia. Best
Practice & Research: Clinical Haematology 15 [2002]: 329–368). The majority of patients are managed in
small programs which may not have access to recommended monitoring and treatments. Therefore, an
established network of care between thalassemia centers, local providers, and patients is required for
optimal treatment of thalassemia patients in North America.
Each component of this network should follow the Standards of Care Guidelines and communicate
frequently. A group of providers first developed and finalized the first set of guidelines in 2000, with the
goal of standardizing the management of care for thalassemia patients throughout the State of
California. Since then, significant changes in technology and treatment have required updating of the
guidelines. The current version, including the PDF, can be found here on the Clinical Practice Guidelines
page.
Assessments
All patients should undergo at least an annual comprehensive assessment at a thalassemia center.
During such an assessment, recommendations are summarized after consultation with multiple
specialists and communicated directly to the primary provider and family. Verbal and written
communication between the center and the primary provider should occur at least every six months
following the formal annual visit and when there are changes in the patient’s clinical and treatment
plan.
Specialty Care Centers
A specialty center manages the regular care of at least 20 patients. A specialty program includes a team
of thalassemia experts working closely together. This team includes a hematologist, a nurse specialist, a
hepatologist, a cardiologist, an endocrinologist, a psychologist, a genetics counselor, a social worker,
and a dietitian. A center includes linkage to a thalassemia-oriented bone marrow transplant and fertility
service. Within the center, specialty laboratory support includes diagnostic imaging, a
hemoglobinopathy reference laboratory, and a clinical research center.
Standard-of-care clinical practice guidelines
Please click on standards of care under the Treatment section, or follow this link for more information on the Thalassemia Treatment Guidelines of 2012.