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Hydroxyurea Optimization Through Precision Study

Phase 3
Recruiting
Conditions
Sickle Cell Disease
Sickle Cell Anemia
Interventions
Registration Number
NCT03789591
Lead Sponsor
Children's Hospital Medical Center, Cincinnati
Brief Summary

Hydroxyurea Optimization through Precision Study (HOPS) is a prospective, multi-center, randomized trial that will directly compare a novel, individualized dosing strategy of hydroxyurea to standard weight-based dosing for children with SCA. The primary objective of the study is to evaluate whether a pharmacokinetics-based starting hydroxyurea dose thieves superior fetal hemoglobin response to to standard weight-based initial dosing. Patients will be recruited from the pediatric sickle cell clinic at Cincinnati Children's Hospital Medical Center and from additional pediatric sickle cell centers within the United States.

Detailed Description

The trial will recruit patients who have decided to initiate hydroxyurea therapy. All participants will have pharmacokinetics studies performed at baseline, following a 20 mg/kg oral dose of hydroxyurea. Pharmacokinetic sampling will use a sparse sampling approach, requiring collection of blood at 3 time points (15 minutes, 60 minutes, 180 minutes) following the hydroxyurea dose. Enrolled participants will be randomized to receive either hydroxyurea using a starting dose of 20 mg/kg/day (Standard Arm) or a personalized PK-guided dose (Alternative Arm) to target an area under the curve (AUC) of 115 mg\*h/L based to approximate hydroxyurea exposure seen when patients are escalated to maximum tolerated dose (MTD).

Following randomization and selection of the initial dose, participants in both arms will follow the same procedures of laboratory medication holds for hematological toxicity. The primary endpoint is fetal hemoglobin (HbF) six months following the initiation of hydroxyurea therapy with the hypothesis that participants starting with a PK-guided dose will achieve HbF at least 5% greater than those starting with a 20 mg/kg dose. Based upon the estimated number of new hydroxyurea starts at each site, it is anticipated that it will take 24 months to enroll the 116 participants required to achieve sufficient power to assess the primary endpoint. The study will conclude for each participant 12 months following hydroxyurea initiation.

Recruitment & Eligibility

Status
RECRUITING
Sex
All
Target Recruitment
116
Inclusion Criteria
  • Diagnosis of sickle cell anemia (HbSS, HbSD, HbS/ฮฒ0-thalassemia, or similarly severe SCA genotype)
  • Age 6 months to 21 years at the time of enrollment
  • Clinical decision by patient, family, and healthcare providers to initiate hydroxyurea therapy
Exclusion Criteria
  • Current treatment with chronic, monthly blood transfusions or erythrocytapheresis
  • Treatment with hydroxyurea within the past 3 months
  • Hemoglobin SC disease, HbS/ฮฒ+-thalassemia
  • Current treatment with other investigational sickle cell medications
  • Current known pregnancy or lactation

Study & Design

Study Type
INTERVENTIONAL
Study Design
PARALLEL
Arm && Interventions
GroupInterventionDescription
Standard ArmHydroxyureaParticipants randomized to the standard arm will receive a starting dose of hydroxyurea of 20 mg/kg/day.
Alternative ArmHydroxyureaParticipants randomized to the alternative arm will receive a pharmacokinetic guided starting dose of hydroxyurea based on PK labs drawn at a baseline visit to target an area under the curve (AUC) of 115 mg\*h/L in an attempt to approximate maximum tolerated dose (MTD). This dose will not exceed the maximum tolerated dose of 35 mg/kg/day.
Primary Outcome Measures
NameTimeMethod
Fetal Hemoglobin (HbF) Response Following Six Months of Hydroxyurea Therapy6 months after starting daily hydroxyurea therapy

The primary outcome will be HbF response six months after starting hydroxyurea therapy with the hypothesis that participants in the Alternative Arm (PK-guided starting dose) will have at least 5% higher HbF than the Standard Arm (20 mg/kg starting dose)

Secondary Outcome Measures
NameTimeMethod
Gene Expression Patterns of Study Participants6 Months after initial Hydroxyurea therapy

The epigenomic signature and gene expression patterns of study participants receiving hydroxyurea therapy at MTD. MTD is defined as a stable dose without any dose increases (except to account for weight gain), holds, or decreases within 8 weeks with laboratory criteria within the target range. This outcome will explain the mechanisms that yield high HbF responses.

F CellsBaseline, 6 and 12 months after initiating daily hydroxyurea therapy

In addition to traditional %HbF measurement, F cells will be measured at baseline, 6 months, and 12 months

Trial Locations

Locations (14)

Boston Children's Hospital

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Boston, Massachusetts, United States

Cleveland Clinic Children's

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Cleveland, Ohio, United States

Carle Foundation Hospital

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Urbana, Illinois, United States

Riley Hospital for Children at Indiana University Health

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Indianapolis, Indiana, United States

Children's Hospital of Illinois

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Peoria, Illinois, United States

Phoenix Children's Hospital

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Phoenix, Arizona, United States

Indiana Hemophilia & Thrombosis Center, Inc. (IHTC)

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Indianapolis, Indiana, United States

Cincinnati Children's Hospital Medical Center

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Cincinnati, Ohio, United States

Rainbow Babies / University Hospitals Cleveland Medical Center

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Cleveland, Ohio, United States

Nationwide Children's Hospital.

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Columbus, Ohio, United States

Children's Hospital of Wisconsin

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Milwaukee, Wisconsin, United States

Cohen Children's Medical Center/Northwell Health

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New Hyde Park, New York, United States

Children's Healthcare of Atlanta

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Atlanta, Georgia, United States

Children's Hospitals and Clinics of Minnesota

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Minneapolis, Minnesota, United States

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