Sickle cell disease: Recommended screening and interventions
| Nephropathy Screen annually for albuminuria: spot urine test to estimate protein-to-creatinine ratio If micro- or macroalbuminuria is present: 24-hour urine test If protein excretion rate > 300 mg/24 hours, refer to a nephrologist Consider angiotensin-converting enzyme inhibitor therapy |
| Pulmonary Assess for respiratory problems Pulmonary function testing If findings suggest pulmonary hypertension, refer for cardiology evaluation |
| Hypertension Screen; treat to ≤ 130/80 mm Hg a |
| Retinopathy Refer to an ophthalmologist for a dilated eye examinationb; rescreen in 1–2 years if normal Refer to a retinal specialist for suspected retinopathy |
| Stroke Screening limited to children Blood transfusion: simple or exchange Hydroxyureac |
| Leg ulcers Inspect lower extremities for active and healed ulcers Treat with debridement, wet-to-dry dressings, topical agents Chronic recalcitrant deep leg ulcers: evaluate for osteomyelitis, consult wound care specialist |
| Reproductive counseling Reproductive life plan Refer partners for hemoglobinopathy status testing if status is unknown Test women anticipating pregnancy for red blood cell alloantibodies Discuss contraception choices with no restrictions for use in sickle cell disease: progestin-only contraceptives, barrier methods; reinforce the need for barrier methods for patients on hydroxyurea |
| Avascular necrosis Elicit from history and physical examination Confirm with radiography and magnetic resonance imaging Refer for physical therapy, orthopedic clinic |
↵a Systolic value based on updated American Society of Hematology guidelines on sickle cell disease management: https://ashpublications.org/bloodadvances/article/3/23/3867/429210/American-Society-of-Hematology-2019-guidelines-for.
↵b Sickle cell retinopathy is more common in the SC variant, but other genotypes carry a risk.
↵c While hydroxyurea has been shown to be comparable to transfusion therapy in the prevention of stroke, chronic transfusions have remained an efficient method of reducing the occurrence of secondary stroke.
From National Heart, Lung, and Blood Institute. Evidence-based management of sickle cell disease: Expert panel report, 2014. www.nhlbi.nih.gov/guidelines.