Hydroxyurea to Prevent Organ Damage in Children With Sickle Cell Anemia

NCT ID: NCT00006400

Last Updated: 2020-08-19

Study Results

Results available

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Basic Information

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Recruitment Status

COMPLETED

Clinical Phase

PHASE3

Total Enrollment

193 participants

Study Classification

INTERVENTIONAL

Study Start Date

2000-08-31

Study Completion Date

2009-09-30

Brief Summary

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The purpose of this study is to determine if hydroxyurea therapy is effective in the prevention of chronic end organ damage in pediatric patients with sickle cell anemia.

Detailed Description

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BACKGROUND:

In 1995, the Multicenter Study of Hydroxyurea (MSH) demonstrated that hydroxyurea is effective in decreasing the frequency of painful crises, hospitalizations for crises, acute chest syndrome, and blood transfusions by 50%. The recently completed phase II study of hydroxyurea in children (PED HUG) demonstrated that children have a response to hydroxyurea similar to that seen in adults in terms of increasing fetal hemoglobin levels and total hemoglobin, and decreasing complications associated with sickle cell anemia. In addition, this study demonstrated that the drug does not adversely affect growth and development between the ages of 5 and 15. A recently completed pilot study of hydroxyurea given to children between the ages of 6 months and 24 months demonstrated that the drug is tolerated well by small infant, and that the fetal hemoglobin switch can be forced to remain in the "on position" by hydroxyurea administration.

A Special Emphasis Panel (SEP) met on April 12, 1996 to review the results of the MSH trial and the progress to date of the PED HUG study. The SEP recommended that NHLBI undertake the BABY HUG trial.

DESIGN NARRATIVE:

BABY HUG is a randomized, double-blind, placebo-controlled study to determine if hydroxyurea can prevent the onset of chronic end organ damage in young children with sickle cell anemia. Approximately 200 children with sickle cell disease will be recruited to receive either hydroxyurea or placebo. The children will be screened at study entry for signs of abnormal brain, kidney, pulmonary, and splenic function, and developmental milestones. They will then be randomly assigned to receive either hydroxyurea or placebo and followed yearly to assess chronic end organ damage of the major organ systems. The primary endpoint will be a 50% reduction in rates of damage to the major organs with surrogate markers of organ function during follow-up in Phase II of the trial.

Conditions

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Hematologic Diseases Anemia, Sickle Cell

Study Design

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Allocation Method

RANDOMIZED

Intervention Model

PARALLEL

Primary Study Purpose

PREVENTION

Blinding Strategy

DOUBLE

Participants Investigators

Study Groups

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Hydroxyurea

Participants will receive hydroxyurea.

Group Type ACTIVE_COMPARATOR

Hydroxyurea

Intervention Type DRUG

Participants will receive hydroxyurea.

Placebo

Participants will receive placebo.

Group Type PLACEBO_COMPARATOR

Placebo

Intervention Type DRUG

Participants will receive placebo.

Interventions

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Hydroxyurea

Participants will receive hydroxyurea.

Intervention Type DRUG

Placebo

Participants will receive placebo.

Intervention Type DRUG

Eligibility Criteria

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Inclusion Criteria

* Majority fetal and sickle (FS or SF) hemoglobin pattern confirmed centrally by electrophoresis (screening may begin at 7 months of age)

Exclusion Criteria

* Chronic transfusion therapy
* Cancer
* Less than 5th percentile (10th percentile for the pilot study) height, weight, or head circumference for age
* Severe developmental delay (e.g., cerebral palsy or other mental retardation, Grade III/IV intraventricular hemorrhage)
* Stroke with neurological deficit
* Surgical splenectomy
* Participating in other clinical intervention trials
* Probable or known diagnosis of Hemoglobin S-Hereditary Persistence of Fetal Hemoglobin
* Known hemoglobin S-beta plus thalassemia (hemoglobin A present)
* Any condition or chronic illness, which in the opinion of the principal investigator, makes participation unadvised or unsafe
* Inability or unwillingness to complete baseline (pre-enrollment) studies, including blood or urine specimen collection, liver-spleen scan, abdominal sonogram, neurological examination, neuropsychological testing, or transcranial Doppler ultrasound (interpretable study not required, but confirmed velocity greater than 200 cm/sec results in ineligibility)
* Previous or current treatment with hydroxyurea (HU) or another anti-sickling drug

1. Hemoglobin less than 6.0 gm/dL
2. Reticulocyte count less than 80,000/cu mm if hemoglobin is less than 9 gm/dL
3. Neutrophil count less than 2,000/cu mm
4. Platelet count less than 130,000/cu mm
5. Blood transfusion in the 2 months prior to study entry unless HbA is less than 10%
6. ALT greater than twice the upper limit of normal
7. Ferritin less than 10 ng/ml
8. Serum creatinine greater than twice the upper limit of normal for age
9. Bayley standardized mental score below 70
Minimum Eligible Age

9 Months

Maximum Eligible Age

18 Months

Eligible Sex

ALL

Accepts Healthy Volunteers

No

Sponsors

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National Heart, Lung, and Blood Institute (NHLBI)

NIH

Sponsor Role lead

Responsible Party

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Responsibility Role SPONSOR

Principal Investigators

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Sherron Jackson, MD

Role: PRINCIPAL_INVESTIGATOR

Medical University of South Carolina

James F. Casella, MD

Role: PRINCIPAL_INVESTIGATOR

Johns Hopkins University

Lori Luchtman-Jones, MD

Role: PRINCIPAL_INVESTIGATOR

Children's National Research Institute

Rathi V. Iyer, MD

Role: PRINCIPAL_INVESTIGATOR

University of Mississippi Medical Center

Scott T. Miller, MD

Role: PRINCIPAL_INVESTIGATOR

SUNY Health Science Center, Brooklyn

Sohail R. Rana, MD

Role: PRINCIPAL_INVESTIGATOR

Howard University

Zora R. Rogers, MD

Role: PRINCIPAL_INVESTIGATOR

University of Texas SW Medical Center

Bruce W Thompson, Ph.D.

Role: PRINCIPAL_INVESTIGATOR

Clinical Trials and Surveys Corp

Julio Barredo, MD

Role: PRINCIPAL_INVESTIGATOR

University of Miami Medical Center

Winfred C. Wang, MD

Role: STUDY_CHAIR

St. Jude Children's Research Hospital

Courtney Thornburg, MD

Role: PRINCIPAL_INVESTIGATOR

Duke University

Thomas Howard, MD

Role: PRINCIPAL_INVESTIGATOR

University of Alabama at Birmingham

Lori Luck, MD

Role: PRINCIPAL_INVESTIGATOR

Drexel University

R. Clark Brown, MD, PhD

Role: PRINCIPAL_INVESTIGATOR

Emory University

Sharada Sarnaik, MD

Role: PRINCIPAL_INVESTIGATOR

Wayne State University

Locations

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University of Alabama at Birmingham

Birmingham, Alabama, United States

Site Status

Children's National Medical Center

Washington D.C., District of Columbia, United States

Site Status

Howard University

Washington D.C., District of Columbia, United States

Site Status

University of Miami

Miami, Florida, United States

Site Status

Emory University School of Medicine

Atlanta, Georgia, United States

Site Status

Johns Hopkins University

Baltimore, Maryland, United States

Site Status

Children's Hospital of Michigan/Wayne State Univ.

Detroit, Michigan, United States

Site Status

University of Mississippi Medical Center

Jackson, Mississippi, United States

Site Status

SUNY Health Science Center, Brooklyn

Brooklyn, New York, United States

Site Status

Duke University Medical Center

Durham, North Carolina, United States

Site Status

Drexel University

Philadelphia, Pennsylvania, United States

Site Status

Medical University of South Carolina

Charleston, South Carolina, United States

Site Status

St. Jude Children's Research Hospital

Memphis, Tennessee, United States

Site Status

University of Texas SW Medical Center

Dallas, Texas, United States

Site Status

Countries

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United States

References

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Heeney MM, Whorton MR, Howard TA, Johnson CA, Ware RE. Chemical and functional analysis of hydroxyurea oral solutions. J Pediatr Hematol Oncol. 2004 Mar;26(3):179-84. doi: 10.1097/00043426-200403000-00007.

Reference Type BACKGROUND
PMID: 15125610 (View on PubMed)

Thompson BW, Miller ST, Rogers ZR, Rees RC, Ware RE, Waclawiw MA, Iyer RV, Casella JF, Luchtman-Jones L, Rana S, Thornburg CD, Kalpatthi RV, Barredo JC, Brown RC, Sarnaik S, Howard TH, Luck L, Wang WC. The pediatric hydroxyurea phase III clinical trial (BABY HUG): challenges of study design. Pediatr Blood Cancer. 2010 Feb;54(2):250-5. doi: 10.1002/pbc.22269.

Reference Type BACKGROUND
PMID: 19731330 (View on PubMed)

Wang WC, Ware RE, Miller ST, Iyer RV, Casella JF, Minniti CP, Rana S, Thornburg CD, Rogers ZR, Kalpatthi RV, Barredo JC, Brown RC, Sarnaik SA, Howard TH, Wynn LW, Kutlar A, Armstrong FD, Files BA, Goldsmith JC, Waclawiw MA, Huang X, Thompson BW; BABY HUG investigators. Hydroxycarbamide in very young children with sickle-cell anaemia: a multicentre, randomised, controlled trial (BABY HUG). Lancet. 2011 May 14;377(9778):1663-72. doi: 10.1016/S0140-6736(11)60355-3.

Reference Type RESULT
PMID: 21571150 (View on PubMed)

Wang WC, Pavlakis SG, Helton KJ, McKinstry RC, Casella JF, Adams RJ, Rees RC; BABY HUG Investigators. MRI abnormalities of the brain in one-year-old children with sickle cell anemia. Pediatr Blood Cancer. 2008 Nov;51(5):643-6. doi: 10.1002/pbc.21612.

Reference Type RESULT
PMID: 18478575 (View on PubMed)

Miller ST, Wang WC, Iyer R, Rana S, Lane P, Ware RE, Li D, Rees RC; BABY-HUG Investigators. Urine concentrating ability in infants with sickle cell disease: baseline data from the phase III trial of hydroxyurea (BABY HUG). Pediatr Blood Cancer. 2010 Feb;54(2):265-8. doi: 10.1002/pbc.22189.

Reference Type RESULT
PMID: 19621454 (View on PubMed)

Pavlakis SG, Rees RC, Huang X, Brown RC, Casella JF, Iyer RV, Kalpatthi R, Luden J, Miller ST, Rogers ZR, Thornburg CD, Wang WC, Adams RJ; BABY HUG Investigators. Transcranial doppler ultrasonography (TCD) in infants with sickle cell anemia: baseline data from the BABY HUG trial. Pediatr Blood Cancer. 2010 Feb;54(2):256-9. doi: 10.1002/pbc.22282.

Reference Type RESULT
PMID: 19813252 (View on PubMed)

Thornburg CD, Rogers ZR, Jeng MR, Rana SR, Iyer RV, Faughnan L, Hassen L, Marshall J, McDonald RP, Wang WC, Huang X, Rees RC; BABY HUG Investigators. Adherence to study medication and visits: data from the BABY HUG trial. Pediatr Blood Cancer. 2010 Feb;54(2):260-4. doi: 10.1002/pbc.22324.

Reference Type RESULT
PMID: 19856395 (View on PubMed)

Ware RE, Rees RC, Sarnaik SA, Iyer RV, Alvarez OA, Casella JF, Shulkin BL, Shalaby-Rana E, Strife CF, Miller JH, Lane PA, Wang WC, Miller ST; BABY HUG Investigators. Renal function in infants with sickle cell anemia: baseline data from the BABY HUG trial. J Pediatr. 2010 Jan;156(1):66-70.e1. doi: 10.1016/j.jpeds.2009.06.060.

Reference Type RESULT
PMID: 19880138 (View on PubMed)

Wynn L, Miller S, Faughnan L, Luo Z, Debenham E, Adix L, Fish B, Hustace T, Kelly T, Macdermott M, Marasciulo J, Martin B, McDuffie J, Murphy M, Rackoff B, Reed C, Seaman P, Thomas G, Wang W. Recruitment of infants with sickle cell anemia to a Phase III trial: data from the BABY HUG study. Contemp Clin Trials. 2010 Nov;31(6):558-63. doi: 10.1016/j.cct.2010.08.007. Epub 2010 Aug 24.

Reference Type RESULT
PMID: 20797449 (View on PubMed)

Rogers ZR, Wang WC, Luo Z, Iyer RV, Shalaby-Rana E, Dertinger SD, Shulkin BL, Miller JH, Files B, Lane PA, Thompson BW, Miller ST, Ware RE; BABY HUG. Biomarkers of splenic function in infants with sickle cell anemia: baseline data from the BABY HUG Trial. Blood. 2011 Mar 3;117(9):2614-7. doi: 10.1182/blood-2010-04-278747. Epub 2011 Jan 7.

Reference Type RESULT
PMID: 21217080 (View on PubMed)

Wang WC, Oyeku SO, Luo Z, Boulet SL, Miller ST, Casella JF, Fish B, Thompson BW, Grosse SD; BABY HUG Investigators. Hydroxyurea is associated with lower costs of care of young children with sickle cell anemia. Pediatrics. 2013 Oct;132(4):677-83. doi: 10.1542/peds.2013-0333. Epub 2013 Sep 2.

Reference Type DERIVED
PMID: 23999955 (View on PubMed)

Thornburg CD, Files BA, Luo Z, Miller ST, Kalpatthi R, Iyer R, Seaman P, Lebensburger J, Alvarez O, Thompson B, Ware RE, Wang WC; BABY HUG Investigators. Impact of hydroxyurea on clinical events in the BABY HUG trial. Blood. 2012 Nov 22;120(22):4304-10; quiz 4448. doi: 10.1182/blood-2012-03-419879. Epub 2012 Aug 22.

Reference Type DERIVED
PMID: 22915643 (View on PubMed)

Alvarez O, Miller ST, Wang WC, Luo Z, McCarville MB, Schwartz GJ, Thompson B, Howard T, Iyer RV, Rana SR, Rogers ZR, Sarnaik SA, Thornburg CD, Ware RE; BABY HUG Investigators. Effect of hydroxyurea treatment on renal function parameters: results from the multi-center placebo-controlled BABY HUG clinical trial for infants with sickle cell anemia. Pediatr Blood Cancer. 2012 Oct;59(4):668-74. doi: 10.1002/pbc.24100. Epub 2012 Jan 31.

Reference Type DERIVED
PMID: 22294512 (View on PubMed)

Lebensburger JD, Miller ST, Howard TH, Casella JF, Brown RC, Lu M, Iyer RV, Sarnaik S, Rogers ZR, Wang WC; BABY HUG Investigators. Influence of severity of anemia on clinical findings in infants with sickle cell anemia: analyses from the BABY HUG study. Pediatr Blood Cancer. 2012 Oct;59(4):675-8. doi: 10.1002/pbc.24037. Epub 2011 Dec 20.

Reference Type DERIVED
PMID: 22190441 (View on PubMed)

Miller ST, Rey K, He J, Flanagan J, Fish BJ, Rogers ZR, Wang WC, Ware RE; BABY HUG Investigators. Massive accidental overdose of hydroxyurea in a young child with sickle cell anemia. Pediatr Blood Cancer. 2012 Jul 15;59(1):170-2. doi: 10.1002/pbc.23244. Epub 2011 Jul 8.

Reference Type DERIVED
PMID: 21744485 (View on PubMed)

McCarville MB, Luo Z, Huang X, Rees RC, Rogers ZR, Miller ST, Thompson B, Kalpatthi R, Wang WC; BABY HUG Investigators. Abdominal ultrasound with scintigraphic and clinical correlates in infants with sickle cell anemia: baseline data from the BABY HUG trial. AJR Am J Roentgenol. 2011 Jun;196(6):1399-404. doi: 10.2214/AJR.10.4664.

Reference Type DERIVED
PMID: 21606305 (View on PubMed)

Provided Documents

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Document Type: Study Protocol and Statistical Analysis Plan

View Document

Document Type: Informed Consent Form

View Document

Other Identifiers

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N01HB07150

Identifier Type: NIH

Identifier Source: secondary_id

View Link

N01HB07151

Identifier Type: NIH

Identifier Source: secondary_id

View Link

N01HB07152

Identifier Type: NIH

Identifier Source: secondary_id

View Link

N01HB07153

Identifier Type: NIH

Identifier Source: secondary_id

View Link

N01HB07154

Identifier Type: NIH

Identifier Source: secondary_id

View Link

N01HB07155

Identifier Type: NIH

Identifier Source: secondary_id

View Link

N01HB07156

Identifier Type: NIH

Identifier Source: secondary_id

View Link

N01HB07157

Identifier Type: NIH

Identifier Source: secondary_id

View Link

N01HB07158

Identifier Type: NIH

Identifier Source: secondary_id

View Link

N01HB07159

Identifier Type: NIH

Identifier Source: secondary_id

View Link

N01HB07160

Identifier Type: NIH

Identifier Source: secondary_id

View Link

89

Identifier Type: -

Identifier Source: org_study_id

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