Simvastatin (Zocor) Therapy in Sickle Cell Disease

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This research study focuses on individuals with sickle cell disease (SCD). There is scientific evidence suggesting that treatment with the statin drug, simvastatin (Zocor), may be helpful for people with vascular diseases like SCD. This study looks at the effect this drug may have in preventing injury to the blood vessels. It will check for a change in the levels of certain substances in the...

Brief Summary

Official Title: “Phase I/II Study of Simvastatin (Zocor) Therapy in Sickle Cell Disease”

This research study focuses on individuals with sickle cell disease (SCD). There is scientific evidence suggesting that treatment with the statin drug, simvastatin (Zocor), may be helpful for people with vascular diseases like SCD. This study looks at the effect this drug may have in preventing injury to the blood vessels. It will check for a change in the levels of certain substances in the blood that can damage blood vessels. The study will also help us find out whether, and at what dose, simvastatin is safe and useful for people with SCD.

  • Study Type: Interventional
  • Study Design: Endpoint Classification: Safety/Efficacy Study, Intervention Model: Single Group Assignment, Masking: Open Label, Primary Purpose: Treatment
  • Study Primary Completion Date: June 2012

Detailed Clinical Trial Description

Although statins have been used extensively for their cholesterol-lowering effects, recent clinical and experimental data indicate that statins regulate yet other processes, many of which play a major role in sickle cell disease (SCD). Independent of their cholesterol-lowering effects, statins have been shown to prevent damage to blood vessels in several ways, through upregulation of endothelial nitric oxide (NO)and decreased inflammation. Numerous studies documenting the protective effects of statins, together with data showing the therapeutic role of NO in SCD, provide the basis for investigating the potential clinical benefit of simvastatin in SCD.

Data supporting the safety and tolerability of simvastatin in patients with SCD are now needed. For this phase I/II dose-escalation study of oral simvastatin in SCD, we propose the following specific aims:

1. To determine specific dose-response effects of oral simvastatin on patients with SCD, and

2. To assess the safety and tolerability of oral simvastatin in patients with SCD.

Intervention(s) in this Clinical Trial

  • Drug: Simvastatin
    • Comparison of 3 dosages of simvastatin given in a dose-escalating fashion. 20 mg, 40 mg, or 80 mg PO QD x 21 days followed by a drug taper x 4 days.

Arms, Groups and Cohorts in this Clinical Trial

  • Other: Simvastatin, Dose Escalation
    • There are no arms in this study. Simvastatin will be given in a dose-escalating fashion to 3 sequential dosage groups (20 mg/day, 40 mg/day, 80 mg/day).

Outcome Measures for this Clinical Trial

Primary Measures

  • Evaluate the effect of simvastatin on plasma biomarkers of endothelial activation in three escalating treatment dosage (20mg/day,40mg/day,80mg/day) groups.
    • Time Frame: 25 days
      Safety Issue?: No

Secondary Measures

  • Safety and tolerability of simvastatin in patients with sickle cell disease, as measured by changes in clinical adverse affects and laboratory (hematologic, renal, hepatic and lipid) profiles.
    • Time Frame: 25 days
      Safety Issue?: Yes

Criteria for Participation in this Clinical Trial

Inclusion Criteria:

  • Established diagnosis of sickle cell disease (HbSS, SC or Sβ-thalassemia)
  • Age greater than or equal to thirteen years
  • Weight greater than or equal to 35 kg

Exclusion Criteria:

  • Renal dysfunction (Serum Creatinine > 1.5 UNL)
  • Hepatic dysfunction (ALT > 2X UNL)
  • Pretreatment total cholesterol < 100 mg/dL or triglycerides < 30 mg/dL
  • Pretreatment baseline creatine kinase >1X UNL (215 U/L)
  • Pregnancy/lactation
  • RBC transfusion in the last 30 days
  • Vaso-Occlusive Event needing hospitalization in the past 30 days
  • Treatment with any statin drugs within the past 30 days
  • Treatment with drugs having known metabolic interactions with statin drugs (e.g.
  • cytochrome P450 3A4 metabolism), including ketoconazole, itraconazole, fluconazole, erythromycin, clarithromycin, azithromycin, niacin (nicotinic acid), digoxin, coumadin, sildenafil or amiodarone within the past 30 days
  • Treatment (past or present) with amiodarone
  • Musculoskeletal disorder associated with an elevated creatine kinase level
  • Past or present history of substance abuse (alcohol, cocaine, amphetamines, heroin, PCP)
  • Allergy to statins

Gender Eligibility for this Clinical Trial: Both

Minimum Age for this Clinical Trial: 13 Years

Maximum Age for this Clinical Trial: N/A

Are Healthy Volunteers Accepted for this Clinical Trial?: No

Clinical Trial Investigator Information

Lead Investigator: Children's Hospital & Research Center Oakland Other

Overall Clinical Trial Officials and Contacts

Carolyn C Hoppe, M.D. Principal Investigator Children's Hospital and Research Center Oakland  

Overall Contact: Carolyn C Hoppe, M.D. (510) 428-3193 choppe@mail.cho.org

Related Publications

References

Hebbel RP. Extracorpuscular factors in the pathogenesis of sickle cell disease. Am J Pediatr Hematol Oncol. 1982 Fall;4(3):316-9.

Hebbel RP. Perspectives series: cell adhesion in vascular biology. Adhesive interactions of sickle erythrocytes with endothelium. J Clin Invest. 1997 Jun 1;99(11):2561-4. Review. No abstract available.

Hebbel RP. Special issue of microcirculation: examination of the vascular pathobiology of sickle cell anemia. Microcirculation. 2004 Apr;11(2):99-100. No abstract available.

Kaul DK, Liu XD, Choong S, Belcher JD, Vercellotti GM, Hebbel RP. Anti-inflammatory therapy ameliorates leukocyte adhesion and microvascular flow abnormalities in transgenic sickle mice. Am J Physiol Heart Circ Physiol. 2004 Jul;287(1):H293-301. Epub 2004 Mar 4.

Wood KC, Hebbel RP, Granger DN. Endothelial cell P-selectin mediates a proinflammatory and prothrombogenic phenotype in cerebral venules of sickle cell transgenic mice. Am J Physiol Heart Circ Physiol. 2004 May;286(5):H1608-14. Epub 2004 Jan 2.

Belcher JD, Marker PH, Weber JP, Hebbel RP, Vercellotti GM. Activated monocytes in sickle cell disease: potential role in the activation of vascular endothelium and vaso-occlusion. Blood. 2000 Oct 1;96(7):2451-9.

Haffner SM, Alexander CM, Cook TJ, Boccuzzi SJ, Musliner TA, Pedersen TR, Kjekshus J, Pyorala K. Reduced coronary events in simvastatin-treated patients with coronary heart disease and diabetes or impaired fasting glucose levels: subgroup analyses in the Scandinavian Simvastatin Survival Study. Arch Intern Med. 1999 Dec 13-27;159(22):2661-7.

Laufs U, Wassmann S, Hilgers S, Ribaudo N, Bohm M, Nickenig G. Rapid effects on vascular function after initiation and withdrawal of atorvastatin in healthy, normocholesterolemic men. Am J Cardiol. 2001 Dec 1;88(11):1306-7. No abstract available.

Hebbel RP, Vercellotti GM. The endothelial biology of sickle cell disease. J Lab Clin Med. 1997 Mar;129(3):288-93. Review. No abstract available.

Solovey A, Lin Y, Browne P, Choong S, Wayner E, Hebbel RP. Circulating activated endothelial cells in sickle cell anemia. N Engl J Med. 1997 Nov 27;337(22):1584-90.

Solovey A, Gui L, Key NS, Hebbel RP. Tissue factor expression by endothelial cells in sickle cell anemia. J Clin Invest. 1998 May 1;101(9):1899-904.

Reiter CD, Gladwin MT. An emerging role for nitric oxide in sickle cell disease vascular homeostasis and therapy. Curr Opin Hematol. 2003 Mar;10(2):99-107. Review.

Gladwin MT, Crawford JH, Patel RP. The biochemistry of nitric oxide, nitrite, and hemoglobin: role in blood flow regulation. Free Radic Biol Med. 2004 Mar 15;36(6):707-17. Review.

Platt OS. Sickle cell anemia as an inflammatory disease. J Clin Invest. 2000 Aug;106(3):337-8. No abstract available.

Brown MD, Wick TM, Eckman JR. Activation of vascular endothelial cell adhesion molecule expression by sickle blood cells. Pediatr Pathol Mol Med. 2001 Jan-Feb;20(1):47-72.

Takemoto M, Liao JK. Pleiotropic effects of 3-hydroxy-3-methylglutaryl coenzyme a reductase inhibitors. Arterioscler Thromb Vasc Biol. 2001 Nov;21(11):1712-9. Review.

Corsini A, Bellosta S, Baetta R, Fumagalli R, Paoletti R, Bernini F. New insights into the pharmacodynamic and pharmacokinetic properties of statins. Pharmacol Ther. 1999 Dec;84(3):413-28. Review. Erratum in: Pharmacol Ther 2000 May;86(2):199.

Citations Reporting Results

Hoppe C, Kuypers F, Larkin S, Hagar W, Vichinsky E, Styles L. A pilot study of the short-term use of simvastatin in sickle cell disease: effects on markers of vascular dysfunction. Br J Haematol. 2011 Jun;153(5):655-63. Epub 2011 Apr 8.

Additional Information

Information obtained from ClinicalTrials.gov on February 09, 2012

Link to the current ClinicalTrials.gov record. http://clinicaltrials.gov/show/NCT00508027

Study ID Number: 1R01FD003080-01A1

ClinicalTrials.gov Identifier: NCT00508027

Health Authority: United States: Food and Drug Administration

Children's Hospital and Research Center Oakland Official Website

NHLBI Website - Sickle Cell Disease

Medline Plus: Sickle Cell Anemia

CHO Library: Sickle Cell Disease Information

CHO Sickle Cell Program: National Center for Sickle Cell Disease

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