SPA Newsletter

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Effect of hydroxyurea on the frequency of painful crises in sickle cell anemia

Samuel Charache, MD, et al

This paper is a follow-up of a previous open label study of hydroxyurea therapy in patients with sickle cell anemia which found increased production of fetal hemoglobin in treated patients with evidence of only mild myelotoxicity. This trial, which was blinded and double-blinded 299 adults were assigned to receive either hydroxyurea or placebo. Those in the treatment group had lower annual rates of painful crises. This difference did not begin to emerge until about two months into the study. Fewer patients in the treatment group had acute chest syndrome and received fewer transfusions. Overall there was a 445 reduction in the number of painful crises in the treatment group compared to the placebo group.

There was a four-week period at the start of the study prior to randomization when all patients received folic acid only. Hydroxyurea was begun as 15 mg/kg/day and increased every 12 weeks by 5 mg/kg/day unless evidence of myelosupression was seen the dose was adjusted downward by 2.5 mg/kg/day. During the study 12 patients did not require transfusions. Eleven on these patients were in the treatment group.

In their discussion, the authors point out the possible therapeutic and also deleterious effects of treatment with hydroxyurea. They speculate that the mechanism by which hydroxyurea exerts its therapeutic effect are increased fetal hemoglobin production, increased rbc water, and/or decreased rbc adhesion. The authors point out reports possibly linking long-term use of hydroxyurea to the development of leukemia and the unproved safety of this agent's use in pregnancy. Finally they state that hydroxyurea is useful as a preventative measure for painful crises but it has no role in treatment of crises in progress.

Table of Contents

• Editor's Corner
• President's Message
• Society for Pediatric Anesthesia Policy Statment on Provision of Pediatric Anesthesia Care
• Williams Syndrome, Supravalvar Aortic Stenosis and Cardiac Arrest During Anesthesia
• Book Corner
• Out of Africa
• Peds Passport
• MHAUS Research Opportunities
• For Patients: Frequently Asked Questions


• Reviews & Commentary
  • Bariatric surgery for severely overweight adolescents: concerns and recommendations.
    Inge T, et al. Pediatrics 2004;114;217-223
  • Conscious sedation of children with propofol is anything but conscious
    Reeves ST, Havidich JE, and Tobin DP. Pediatrics 2004;114:e74-e76. URL http://www.pediatrics.org/cgi/content/ full/114/1/e74
  • A factorial trial of six interventions for the prevention of postoperative nausea and vomiting.
    Apfel CC, et al. N Engl J Med 2004; 50:2441-2451
  • The effect of dexrazoxane of myocardial injury in dozorubicin-treated children with acute lymphoblastic leukemia
    Lipshultz SE, et al. N Engl J Med 2004; 351:145-153
  • Unilateral negative-pressure pulmonary edema in an infant during bronchoscopy
    Shai Hannania, MD, et al. Pediatrics 2004;113:e501-e503. URL: http://www.pediatrics.org/cgi/content/full/113/5/e501
  • Does pediatric surgical specialty training affect outcome after Ramstedt Pyloromyotomy? A population-based study.
    Langer J, To T. Pediatrics 2004;113:1342-1347
  • Overweight children and adolescents; A risk group for iron deficiency
    Nead KG, et al. Pediatrics 2004;114:104-108
  • A comparison of conservative and aggressive transfusion regimens in the perioperative management of sickle cell disease
    Elliot P. Vichinsky, MD, et al and the Preoperative Transfusion in Sickle Cell Disease Study Group
  • Cholecystectomy in sickle cell anemia patients: Perioperative outcome of 364 cases from the National Preoperative Transfusion Study
    Charles M. Haberkern, et al, and the Preoperative Transfusion in Sickle Cell Disease Study Group
  • Effect of hydroxyurea on the frequency of painful crises in sickle cell anemia
    Samuel Charache, MD, et al
  • Epidural Analgesia in the Management of Severe Vaso-Occlusive Sickle-Cell Crisis.
    Yaster M, et al. Pediatrics 1994;93:310-315


• Literature Reviews
  • A case of propofol toxicity: further evidence for a causal mechanism
    Davinia E Withington, Mary K. Decell, Tareq Al Ayed. Pediatric Anesthesia 2004;14:505
    Death after re-exposure to propofol in a 3-year-old child: a case report
    Josef Holzki, Christoph Aring, Alex Gillor. Paediatric Anaesthesia 2004;14:265
  • Does anaesthesia harm the developing brain - evidence or speculation?
    Andrew Davidson and Sulpicio Soriano. Paediatric Anaesthesia 2004; 14: 199-20.
  • An evaluation of pediatric in-hospital advanced life support interventions using the pediatric Utstein guidelines: a review of 203 cardiorespiratory arrests.
    J. Guay, L. Lortie.Can J Anesth 2004:51:4:373-378
  • A factorial trial of six interventions for the prevention of postoperative nausea and vomiting
    Christian C. Apfel, MD, et al. IMPACT Investigators. N Engl J Med 2004;350:2441-51
  • Interactive Music Therapy as a Treatment for Preoperative Anxiety in Children: A Randomized Controlled Trial
    Kain Z, Caldwell-Andrews A, Krivutza D, Weinberg M, Gaal D, Wang SM, Mayes L. Anesthesia & Analgesia 2004;98:1260-1266.
  • Trends in the practice of parental presence during induction of anesthesia and the use of preoperative sedative premedication in the United States, 1995-2002: results of a follow-up national survey.
    Kain, Z.N. et al. Anesth Analg 2004; 98:1252-9.
  • Anesthetic Complications of Tympanostomy Tube Placement in Children.
    Hoffmann KK, Thompson GK, Burke BL et al. Arch Otolaryngol Head Neck Surg. 128:1040-1043, 2002
  • Neurobehavioral Implications of Habitual Snoring in Children.
    O'Brien CM, et al. Pediatrics 2004; 116:44-50

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