Different Treatment Modalities in the Management of the Painful Crisis in Pediatric Sickle- Cell Anemia
Condition: Vaso-occlusive Crisis; Sickle Cell Disease; Sickle Cell Anemia in Children
MeSH terms: Anemia; Anemia, Sickle Cell
Study type: Interventional
Phase: Phase 2/Phase 3

Summary: 
      The aim of the present study is comparing the effectiveness of different treatment regimens
      for investigating the therapeutic potential for each one in management of Vaso-occlusive pain
      in pediatric sickle cell disease. In addition, investigators apply the Cost-effectiveness
      analysis (CEA) as a form of economic analysis that compares the relative costs and outcomes
      (effects) for different treatment regimens on vaso-occlusive painful crisis.
    

Detailed description: 
      "Sickle cell disease is an inherited blood disorder characterized by defective hemoglobin (a
      protein in red blood cells that carries oxygen to the tissues of the body).

      Sickle cell disease involves the red blood cells, or hemoglobin, and their ability to carry
      oxygen. Normal hemoglobin cells are smooth, round, and flexible, like the letter "O," so they
      can move through the vessels in our bodies easily. Sickle cell hemoglobin cells are stiff and
      sticky and form into the shape of a sickle, or the letter "C," when they lose their oxygen.
      These sickle cells tend to cluster together and cannot easily move through the blood vessels.
      The cluster causes a blockage in small arteries or capillaries and stops the movement of
      healthy, normal oxygen-carrying blood. This blockage is what causes the painful and damaging
      complications of sickle cell disease".

      "Acute vaso-occlusive crisis (VOC) is a hallmark of sickle cell disease (SCD). Multiple
      complex pathophysiological processes can result in pain during a VOC. Despite significant
      improvements in the understanding and management of SCD, little progress has been made in the
      management of pain in SCD, although new treatments are being explored".

      The Painful Episodes:

      "The day-to-day management of sickle cell disease often equates with the management of acute
      and chronic pain. Patients manage many painful events at home so that hospital visits
      underestimate the frequency of pain

      Acute painful episodes are the most commonly encountered vaso-occlusive events in patients of
      all ages. Presumed to be caused by sickle vaso-occlusion, pain often starts in young children
      as the hand-foot syndrome or dactylitis, a painful swelling of hands and feet due to
      inflammation of the metacarpal and metatarsal periosteum. Painful episodes, which last from
      hours to many days, usually occur with little warning and a clear precipitating event is not
      often found.
    

Intervention name(s): Omega 3; Vit D; Zinc sulfate; Statins (Cardiovascular Agents); Hydroxy Urea; Folic Acid Supplementation; Morphine Sulfate; blood transfusion session
Intervention type(s): Drug; Drug; Drug; Drug; Drug; Drug; Drug; Procedure
Intervention description(s): Omega-3 supplementation (300-400mg EPA & 200-300mg DHA) per day for 8 consecutive months up to 10 months; 50 patients from each participating hospital that will receive Vit-D medication (1500 IU to 3500 IU ) per day for 8 consecutive months up to 10 months.
in addition to the experimental treatment, this group will receive the traditional treatment of hydroxyurea, Folic acid, pain killer plus regular blood transfusion with a dose de-escalation methods till efficacy of experimental treatment proved.; 50 patients from each participating hospital that will receive Zinc supplements (15 mg to 50 mg ) per day for 8 consecutive months up to 10 months.
in addition to the experimental treatment, this group will receive the traditional treatment of hydroxyurea, Folic acid, pain killer plus regular blood transfusion with a dose de-escalation methods till efficacy of experimental treatment proved.; 50 patients from each participating hospital that will receive Simvastatin orally (20 mg to 40 mg ) per day for 8 consecutive months up to 10 months.
in addition to the experimental treatment, this group will receive the traditional treatment of hydroxyurea, Folic acid, pain killer plus regular blood transfusion with a dose de-escalation methods till efficacy of experimental treatment proved.; 50 patients from each participating hospital that will receive the ordinary treatment of Hydroxyurea (20 mg/kg/day) with monitoring blood count every 2 weeks maximum daily dose: (40 mg/kg/day) for 8 consecutive months up to 10 months.; Folic Acid dose of 0.5 to 1 mg daily for 3 to 4 weeks until definite hematologic response; Morphine medication as a pain killer is administered, if Patient weight <50 kg: Opioid naïve: Initial: 0.05 mg/kg/dose; usual maximum initial dose: 1 to 2 mg/dose.; Regular blood transfusion session based on patient hematological profile starts from one session every 2 weeks.
Other intervention name(s): omega-3 supplementation capsules; Vit-D medication oral drops; Zinc tablet medication; Simvastatin 20mg; Hydroxy Urea tablet medication 20mg/kg/day; Folic Acid tablet medication 1mg/day; Morphine Sulfate intra venous medication
Intervention MeSH terms: Zinc; Folic Acid; Vitamin B Complex; Hydroxyurea; Morphine; Zinc Sulfate; Simvastatin
Allocation: Randomized
Intervention model: Parallel Assignment
Intervention model description: Four experimental groups, one control group
Masking: Double (Participant, Care Provider)
Primary purpose: Treatment
Keywords: omega3; Vaso-occlusive painful crisis; Pediatric sickle cell anemia; comparative effective analysis in VOC; Anti-inflammatory effect of Vit-D; Anti-hemolytic effect of Vit-D; Anti-hyperlipidemia of Vit-D; effect of omega-3 on blood rheology; effect of omega-3 on blood viscosity; Antiaggregation effect of omega-3; Anti-inflammatory effect of statins; Anti-inflammatory effect of zinc supplements; effect of zinc supplements on blood viscosity; effect of zinc supplements on blood rheology; Antiaggregation effect of zinc supplements; pediatric sickle cell disease