Management of Sickle Cell Disease

Risk Stratification and Assessment

The American Thoracic Society recommends risk stratification for guiding clinical decisions in SCD management, with mortality risk determined by tricuspid regurgitant velocity, NT-pro-BNP level, and right heart catheterization-confirmed pulmonary hypertension 1
Mortality risk can be determined by tricuspid regurgitant velocity (TRV) > 2.5 m/second via Doppler echocardiography, NT-pro-BNP level > 160 pg/ml, and right heart catheterization (RHC)-confirmed pulmonary hypertension (mean pulmonary arterial pressure > 25 mm Hg) 2
In children > 8 years old, TRV determines morbidity risk rather than mortality risk 2

The American College of Chest Physicians strongly recommends hydroxyurea as first-line therapy for patients with SCD who have increased mortality risk 1, 2

The American College of Chest Physicians recommends chronic transfusion therapy for patients who are not responsive to or not candidates for hydroxyurea 1, 2

For patients with SCD who have RHC-confirmed PH, venous thromboembolism, and no additional risk factors for hemorrhage, indefinite anticoagulant therapy is suggested 2
For select patients with RHC-confirmed PH with elevated pulmonary vascular resistance and normal pulmonary artery wedge pressure, a trial of either prostacyclin agonist or endothelin receptor antagonist may be considered 2

Patients with SCD require specialized perioperative care, including adequate hydration to prevent sickling, maintenance of normothermia to prevent peripheral stasis, thromboprophylaxis, and careful pain management 3
Elective surgery should be performed in centers with experience in caring for SCD patients, and all patients at risk should be screened for hemoglobinopathy before surgery 3