A. Sickle cell disease refers to a chronic hemolytic anemia associated with sickle cell hemoglobin, either homozygous or in combination with thalassemia or with another abnormal hemoglobin (such as C or F).
Appropriate hematologic evidence for sickle cell disease, such as hemoglobin electrophoresis must be included. Vaso-occlusive, hemolytic, or aplastic episodes should be documented by description of severity, frequency, and duration.
Disability due to sickle cell disease may be solely the result of a severe, persistent anemia or may be due to the combination of chronic progressive or episodic manifestations in the presence of a less severe anemia.
Major visceral episodes causing disability include meningitis, osteomyelitis, pulmonary infections or infarctions, cerebrovascular accidents, congestive heart failure, genitourinary involvement, etc.
B. Coagulation defects. Chronic inherited coagulation disorders must be documented by appropriate laboratory evidence such as abnormal thromboplastin generation, coagulation time, or factor assay.
C. Acute leukemia. Initial diagnosis of acute leukemia must be based upon definitive bone marrow pathologic evidence. Recurrent disease may be documented by peripheral blood, bone marrow, or cerebrospinal fluid examination. The pathology report must be included.
The designated duration of disability implicit in the finding of a listed impairment is contained in 107.11. Following the designated time period, a documented diagnosis itself is no longer sufficient to establish a severe impairment. The severity of any remaining impairment must be evaluated on the basis of the medical evidence.
107.01 Category of Impairments, Hemic and Lymphatic
107.03 Hemolytic anemia (due to any cause). Manifested by persistence of hematocrit of 26 percent or less despite prescribed therapy, and reticulocyte count of 4 percent or greater.
107.05 Sickle cell disease. With:
A. Recent, recurrent, severe vaso-occlusive crises (musculoskeletal, vertebral, abdominal); or
B. A major visceral complication in the 12 months prior to application; or
C. A hyperhemolytic or aplastic crisis within 12 months prior to application; or
D. Chronic, severe anemia with persistence of hematocrit of 26 percent or less; or
E. Congestive heart failure, cerebrovascular damage, or emotional disorder as described under the criteria in 104.02, 111.00ff, or 112.00ff.
107.06 Chronic idiopathic thrombocytopenic purpura of childhood with purpura and thrombocytopenia of 40,000 platelets/cu. mm. or less despite prescribed therapy or recurrent upon withdrawal of treatment.
107.08 Inherited coagulation disorder. With:
A. Repeated spontaneous or inappropriate bleeding; or
B. Hemarthrosis with joint deformity.
107.11 Acute leukemia. Consider under a disability:
A. For 2 1/2 years from the time of initial diagnosis; or
B. For 2 1/2 years from the time of recurrence of active disease.