Hydroxurea Treatment Recommendations

Recommendations
Educate all patients with SCA and their family members about hydroxyurea therapy. (See consensus treatment protocol on page 145). (Consensus-Panel Expertise)

In adults with SCA who have three or more sickle cell-associated moderate to severe pain crises in a 12-month period, treat with hydroxyurea. (Strong Recommendation, High-Quality Evidence)

In adults with SCA who have sickle cell-associated pain that interferes with daily activities and quality of life, treat with hydroxyurea. (Strong Recommendation, Moderate-Quality Evidence)

In adults with SCA who have a history of severe and/or recurrent ACS, treat with hydroxyurea.*
(Strong Recommendation, Moderate-Quality Evidence)

In adults with SCA who have severe symptomatic chronic anemia that interferes with daily activities or quality of life, treat with hydroxyurea. (Strong Recommendation, Moderate-Quality Evidence)

In infants 9 months of age and older, children,and adolescents with SCA, offer treatment with hydroxyurea regardless of clinical severity to reduce SCD-related complications (e.g., pain, dactylitis, ACS,anemia).(Strong Recommendation, High-Quality Evidence for ages 9–42 months;

Moderate Recommendation,Moderate-QualityEvidence for children >42 months and adolescents).
Note: The panel intentionally used the term “offer” realizing that patients’ values and preferences may differ particularly considering treatment burden (e.g., laboratory monitoring,office visits),availability of drug in a liquid form, and cost. Therefore, the panel strongly encourages shared decision making and discussion of hydroxyurea therapy with all patients.

In adults and children with SCD who have chronic kidney disease and are taking erythropoietin, hydroxyurea therapy can be added to improve anemia. (Weak Recommendation, Low-Quality Evidence)

In females who are pregnant or breastfeeding,discontinue hydroxyurea therapy.
(Moderate Recommendation, Very Low-Quality Evidence)

To ensure proper use of hydroxyurea and maximize benefits and safety, use an established prescribing and monitoring protocol.(Strong Recommendation, High-Quality Evidence)

In people with HbSW-thalassemia or HbSC who have recurrent sickle cell-associated pain that interferes with daily activities or quality of life,consult a sickle cell expert for consideration of hydroxyurea therapy.
(Moderate Recommendation, Low-Quality Evidence)

In people not demonstrating a clinical response to appropriate doses and duration of hydroxyurea therapy, consult a sickle cell expert. (Moderate Recommendation, Very Low-Quality Evidence)