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Therapeutic Response Evaluation and Adherence Trial (TREAT)

Not Applicable
Recruiting
Conditions
Anemia, Sickle Cell
Interventions
Registration Number
NCT02286154
Lead Sponsor
Children's Hospital Medical Center, Cincinnati
Brief Summary

The primary objectives of this prospective study of hydroxyurea for children with sickle cell anemia are 1) Develop and prospectively evaluate a population pharmacokinetic/pharmacodynamics model to predict the maximum tolerated dose (MTD); 2) Identify urine biomarkers of hydroxyurea adherence using a novel metabolomics approach; 3) Identify pharmacogenomics modifiers of hydroxyurea MTD; and 4) Longitudinal monitoring of the effect of hydroxyurea upon organ function and quality of life.

Detailed Description

There is now ample clinical evidence that hydroxyurea is a safe and effective medication for adults and children with sickle cell anemia (SCA), and most hematologists agree the short-term safety and efficacy of hydroxyurea has been proven. The National Heart, Lung, and Blood Institute have recently released evidence-based guidelines for SCA, recommending that hydroxyurea be offered to all affected children as young as nine months of age, regardless of clinical severity. Despite the overwhelming evidence demonstrating safety and efficacy, hydroxyurea remains underutilized for a variety of reasons. In this prospective study, the investigators will utilize innovative strategies designed to address and overcome some of the barriers that currently limit the use of hydroxyurea for children with SCA. The investigators will utilize novel laboratory techniques and pharmacometric modeling in order to accurately predict the most effective hydroxyurea dose referred to as the maximum tolerated dose. The investigators aim to develop a screening urine test to objectively and accurately determine adherence to hydroxyurea therapy. In addition, the study will document critical laboratory and clinical characteristics of this unique population of patients with SCA who begin hydroxyurea at a young age.

This study will follow two groups of patients. The first group, referred to as the New Cohort, will include mostly young infants who are not receiving hydroxyurea therapy upon entering the study. The starting dose of hydroxyurea for each of the participants in the New Cohort will be individually determined using the novel population PK/PD dose-prediction model. The second group of study participants, referred to as the Old Cohort, will include patients who are already receiving hydroxyurea therapy upon study entry. Both the Old and New Cohort (New Cohort) will be included in the development of a urine biomarker of adherence and will be followed throughout the study to document the effect hydroxyurea has upon organ function and quality of life. It is important to note that this is not a therapeutic drug trial. Prior to enrollment in the study, participants, along with their families and clinical providers, have decided to initiate hydroxyurea therapy for clinical indications. Except for the dose prediction model for the New Cohort, participants will be treated and monitored according to the routine clinical practice guidelines of the Cincinnati Children's Hospital Comprehensive Sickle Cell Center.

Recruitment & Eligibility

Status
RECRUITING
Sex
All
Target Recruitment
150
Inclusion Criteria
  1. Diagnosis of sickle cell anemia (HbSS or Hbβ0-thalassemia)
  2. Age 6 months to 21 years at the time of enrollment
  3. Clinical decision by patient, family, and healthcare provider to initiate hydroxyurea therapy, including patients who are transitioning from chronic transfusions to hydroxyurea therapy
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Exclusion Criteria
  1. Family unwillingness to sign informed consent or comply with study treatments
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Study & Design

Study Type
INTERVENTIONAL
Study Design
SINGLE_GROUP
Arm && Interventions
GroupInterventionDescription
HydroxyureaHydroxyureaAll enrolled participants will receive hydroxyurea, but upon enrollment, participants will be identified as part of the "New Cohort" or "Old Cohort" "New Cohort" participants include those who are not receiving hydroxyurea therapy upon study entry. "Old Cohort" participants include those who are already receiving hydroxyurea therapy upon study entry. New Cohort participants will have starting dose predicted using PK/PD data and Old Cohort participants will continue dosing per clinical guidelines.
Primary Outcome Measures
NameTimeMethod
Time to Reach Maximum Tolerated Dose (months)Twelve months

Time it takes to reach maximum tolerated dose (MTD) of hydroxyurea quantified in months.

Secondary Outcome Measures
NameTimeMethod
Hydroxyurea adherenceMonthly until MTD then yearly up to ten years

Hydroxyurea adherence as measured by analysis of urine metabolites

Splenic functionAnnually up to ten years

Splenic function as measured by pocked red blood cell counts ("pit counts")

Neurological functionYearly

Neurological function as measured by transcranial Doppler study (yearly), brain MRI (every 5 years beginning at age 5).

Kidney functionAnnually, up to ten years

Kidney function as measured by BUN/creatinine, urinalysis, and cystatin-C

Cardiac function (assessment and growth)Every Five Years, up to 21 years of age

Cardiac function as measured by echocardiogram and ECG

Non-invasive Transcranial Cerebral OximetryMonthly until MTD then every six months, up to ten years

Non-invasive transcranial cerebral oximetry

Assessment of GrowthEvery six months, up to ten years

Assessment of growth as defined by height and weight

Trial Locations

Locations (1)

Cincinnati Children's Hospital Medical Center

🇺🇸

Cincinnati, Ohio, United States

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