Management of Sickle Cell Disease with Elevated Alkaline Phosphatase

Disease Management Principles

The American Thoracic Society recommends initiating or continuing hydroxyurea as the first-line intervention for patients with increased mortality risk markers, representing a strong recommendation with moderate-quality evidence 1
For patients unresponsive to or not candidates for hydroxyurea, the American Thoracic Society suggests considering chronic transfusion therapy 2, 3
The American Thoracic Society advises against using phosphodiesterase-5 inhibitors (sildenafil, tadalafil, vardenafil) as they increase hospitalization risk for vaso-occlusive crisis 6, 7, 8, 3

Evaluate for avascular necrosis (particularly femoral/humeral heads) and bone infarction clinically and with imaging as indicated, according to Circulation 5

The American Thoracic Society recommends evaluating proteinuria/microalbuminuria, which is common in SCD and may improve with ACE inhibitors 6, 7
The American Thoracic Society suggests evaluating obstructive sleep apnea via polysomnography if suspected, as treatment can reduce mortality risk 6, 7
If TRV >2.5 m/second or NT-pro-BNP >160 pg/ml, the patient has increased mortality risk requiring intensified disease-modifying therapy, according to the American Journal of Respiratory and Critical Care Medicine 1, 2

Avoid volume depletion as this precipitates erythrocyte sickling, and maintain adequate hydration but use diuretics cautiously for volume overload to minimize sickling risk, according to the American Journal of Respiratory and Critical Care Medicine 6, 7, 4
Maintain arterial oxygen saturation ≥90% at rest, with exertion, and during sleep with supplemental oxygen, as recommended by the American Journal of Respiratory and Critical Care Medicine 6, 7, 4