Long-Term Effects of Hydroxyurea in Children With Sickle Cell Anemia (The BABY HUG Follow-up Study)
Condition: Anemia, Sickle Cell
MeSH terms: Anemia; Anemia, Sickle Cell
Study type: Observational

Summary: 
      Sickle cell anemia (SCA) is an inherited blood disorder that can cause organ damage. The BABY
      HUG study is evaluating the use of the medication hydroxyurea at preventing organ damage in
      children with SCA. The purpose of this follow-up study is to evaluate the long-term effects
      of hydroxyurea in children who have participated in the BABY HUG study.
    

Detailed description: 
      SCA is an inherited blood disorder in which the body makes sickle-shaped red blood cells that
      contain abnormal hemoglobin. The sickled cells block blood flow in the vessels that lead to
      limbs and organs. This can cause pain, serious infections, and organ damage. The BABY HUG
      study (NCT00006400) is examining whether the medication hydroxyurea can prevent organ damage,
      especially in the spleen and kidneys, in children with SCA. This study is a follow-up study
      to the BABY HUG study and will enroll children who have participated in the BABY HUG study.
      The purpose of this study is to examine the long-term effects of using hydroxyurea as a
      treatment for SCA, including both the risks and benefits. Study researchers will also
      investigate the optimal age to begin treatment with hydroxyurea in children with SCA.

      This study will enroll children between 2 and 7 years old who participated in the BABY HUG
      study. Hydroxyurea will not be provided to participants as part of this study, but
      participants may receive the medication from their own doctors. Parents of participants can
      choose for their child to participate in this study in one of two ways-by enrolling in either
      a passive follow-up group or an active follow-up group. For participants in the passive
      follow-up group, study researchers will review participants' medical records every 6 months,
      in addition to reviewing brain ultrasound tests and computed tomography (CT) or magnetic
      resonance imaging (MRI) scans, if completed. Participants will have a blood and urine
      collection at baseline and Year 4 (or at the end of the study, whichever comes first).
      Participants in the active follow-up group will take part in the same study procedures as
      participants in the passive follow-up group. In addition, at Year 2, participants in this
      group will undergo an additional blood and urine collection, a scanning procedure to obtain
      images of the liver and spleen, a kidney test, neuropsychological testing, and an ultrasound
      imaging test to evaluate liver and spleen size.
    

Intervention name(s): Hydroxyurea
Intervention type(s): Drug
Intervention description(s): Parents and child's doctor may plan to use or not to use hydroxyurea.
Intervention MeSH terms: Hydroxyurea
Observational model: Cohort
Time perspective: Other
Sampling method: Non-Probability Sample
Study population: 
        Children from the initial BABY HUG study who agree to participate in this follow-up study.
      
Biospecimen description: 
      Stored blood and urine
    
Keywords: Sickle Cell Anemia; Hydroxyurea