Northern California Comprehensive Thalassemia Center
Standard of Care Guidelines 2012
04: Blood Transfusions
| 4.1 | Assessing the need for routine transfusions |
| 4.2 | Baseline laboratory tests prior to regular transfusions |
| 4.3 | Transfusion administration and monitoring |
| 4.3.1 | Transfusion facility |
| 4.3.2 | Type of blood product |
| 4.3.3 | Target hemoglobin and frequency of transfusions |
| 4.4 | Adverse reactions to transfusions |
| 4.5 | Splenectomy |
| 4.6 | Thromboembolic Disease |
05: Iron Overload and Chelation Therapy
| 5.1 | Initiation of chelation |
| 5.2 | Treatment with iron chelators |
| 5.2.1 | Treatment with deferoxamine (Desferal) |
| 5.2.2 | Treatment with deferasirox (Exjade) |
| 5.2.3 | Treatment with deferiprone (L1/Ferriprox) |
| 5.3 | Patients with significant iron overload |
| 5.3.1 | High-dose, continuous deferoxamine |
| 5.3.2 | Combination therapy: deferoxamine and deferasirox |
| 5.3.3 | Combination therapy: deferoxamine and deferiprone |
07: Assessment of Chelator Side Effects and Toxicity
| 7.1 | Audiology |
| 7.2 | Ophthalmology |
| 7.3 | Nephrology |
| 7.4 | Neutropenia |
| 7.5 | Growth |
| 7.6 | Local and allergic reactions |
| 7.7 | Over-chelation |
08: Liver and Gall Bladder Diseases
| 8.1 | Screening for hepatic dysfunction |
| 8.2 | Monitoring patients with documented hepatitis or hepatic dysfunction |
| 8.3 | Evaluation and treatment for hepatitis C |
| 8.4 | Evaluation and treatment for hepatitis B |
| 8.5 | Gall bladder disease |
09: Endocrine Dysfunction
| 9.1 | Routine endocrine screening |
| 9.2 | Specific endocrinopathies: testing and evaluation |
| 9.2.1 | Diabetes mellitus |
| 9.2.2 | Low bone mass (osteoporosis) |
| 9.2.3 | Growth hormone deficiency |
| 9.2.4 | Hypogonadism |
| 9.2.5 | Hypothyroidism |
| 9.2.6 | Hypoparathyroidism |
| 9.2.7 | Adrenal insufficiency |
10: Cardiac Dysfunction
| 10.1 | Cardiac evaluation |
| 10.2 | Echocardiography standards |
| 10.3 | Treatment of established heart failure |
| 10.4 | Pulmonary hypertension |
| 10.5 | Treatment of pulmonary hypertension |
11: Pulmonary Care
13: Hematopoietic Cell Transplantation
| 13.1 | Iron overload after HCT |
| 13.2 | Experimental HCT |
| 13.3 | Experimental drug therapy to increase fetal hemoglobin |
14: Acute Infection
16: Nutrition
17: Vaccinations
19: Thalassemia Intermedia
| 19.1 | Nontransfused thalassemia intermedia |
| 19.1.1 | Growth and development |
| 19.1.2 | Extramedullary erythropoiesis |
| 19.1.3 | Endocrinopathies |
| 19.1.4 | Cardiopulmonary assessment |
| 19.1.5 | Considerations for transfusions |
| 19.1.6 | Considerations for splenectomy |
| 19.1.7 | Assessment of iron overload |
20: Hemoglobin H Disease and Its Variants
| 20.1 | Diagnosis |
| 20.2 | Hemoglobin H deletion |
| 20.3 | Recommendations for care |
22: Psychosocial Support
| 22.1 | Child life services |
| 22.2 | Psychological services |
| 22.3 | Social services |
| 22.4 | Genetic counseling |