1 |
Diagnosis of Sickle Cell Disease (SCD) and Related Conditions |
A. Tests for hemoglobin (Hb) type using Hb separation or specific immunologic methods, and DNA-based mutation analyses for confirmation |
B. Tests to diagnose heterozygous beta thalassemia (trait) in those with “No Hb variant” visible by the Hb separation methods (Hb phenotypes FA, AF, AFA2, AA2F, AA2, A) |
C. Rapid Screening and Point of Care tests for Hb S, C, A |
D. DNA- based tests |
2 |
Health Maintenance and Preventive Therapy |
A. Organizing Clinical Care (Outpatient routine; outpatient acute; and inpatient) |
B. Infection Prevention: General |
C. Immunization |
D. Prevention of invasive pneumococcal disease (IPD) |
E. Prevention of Malaria |
F. Prevention of enteric Gram-negative organisms (salmonella, E. coli, klebsiella, etc) |
G. Genetic and Reproductive counselling |
H. Female reproductive health (Pregnancy, Contraception and Fertility) |
I. Male reproductive health |
J. Nutrition |
K. Growth and development monitoring in children |
L. Education and psychosocial counselling |
M. Organizing support groups |
N. Transitioning of adolescents to adult care |
O. Travel management |
3 |
Screening for Specific Complications of Sickle Cell Disease |
A. Screening for stroke risk |
B. Screening for renal disease |
C. Screening for retinopathy |
D. Screening for pulmonary disease |
E. Screening for cardiovascular disease |
F. Screening for hypertension |
G. Screening for red cell antibodies related to pregnancy |
4 |
Management of Acute Complications of SCD |
A. Acute anaemia |
B. Acute chest syndrome |
C. Acute hepatobiliary complications |
D. Acute ocular complications |
E. Acute renal failure |
F. Acute SCD pain (vaso-occulsive pain episode, VOPE, or “pain crisis) |
G. Acute splenic sequestration |
H. Acute stroke |
I. Fever and other signs of infection |
J. Osteomyelitis and septic arthritis |
K. Multisystem Organ Failure |
L. Priapism |
5 |
Management of Chronic Complications of Sickle Cell Disease |
A. Avascular necrosis |
B. Cardiac complications |
C. Chronic hypersplenism |
D. Chronic pain |
E. Endocrine complications |
F. Gastrointestinal complications |
G. Leg ulcers |
H. Nocturnal enuresis |
I. Ophthalmologic complications |
J. Psychological complications |
K. Renal complications |
L. Pulmonary complications |
M. Seizures under neurological complications |
N. Stuttering/Recurrent Priapism |
6 |
Special Management Protocols |
A. Analgesic Medications and their Use in Sickle Cell Disease |
B. Haematopoietic stem cell transplantation |
C. Hydration guide |
D. Hydroxyurea Therapy in Sickle Cell Disease |
E. Peri-operative care and surgery |
F. Transfusion Therapy in Sickle Cell Disease/Iron chelation |
G. Organizing SCD clinical service (outpatient routine and acute care, in patient) |