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| Investigation | Relevance | Timing | Interpretation of findings and interventions |
|
| TCD | Screening for risk of stroke | Begin at 2 years of age and continue until at least 16 years of age | Normal (all mean velocities <170 cm per sec). Continue TCD annually
Conditional (mean velocity 170 to 199 cm per sec) ¥ Initiate hydroxyurea therapy
Abnormal (mean velocity ≥200 cm per sec). Repeat TCD within 2 to 6 weeks. Start long-term transfusion therapy. If not possible, initiate HU therapy
Inadequate (no information available on one or both middle cerebral arteries) |
| Assesses blood velocity in the distal internal carotid, anterior or middle cerebral artery |
|
Ophthalmoscopy
(Dilated retinal exam) | Ischemic retinopathy | Begin at 10 years, then every 1-2 years if normal | Refer patients with suspected retinopathy to a retinal specialist for possible laser photocoagulation therapy |
|
| Echocardiography | Screening for PAH | | |
|
| Transcutaneous O2 saturation | | Begin at 12 months, continue annually or more frequently based on clinical course | |
|
| Complete blood count with WBC differential and reticulocyte counts | | Every 3 months beginning from 3 months of life, then every 6 months after 2 years of life | Frequency adjusted based on the patient’s clinical state |
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| Liver and renal function tests | Routine screening | Every 6 months | |
|
| HbF percentage | | Every 6 months for children aged 6 to 24 months, then annually | |
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| Spot urine testing | Microalbuminuria and proteinuria. Early markers of renal dysfunction | Begin by 10 years of life, and then annually if negative | If proteinuria (>300 mg per 24 hours), perform a first morning void urine albumin-creatinine ratio or 24-hour urine creatinine clearance. Consult/refer to nephrologist if abnormal
ACEIs may be indicated |
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