hcp.siklosusa.comHydroxyurea-based treatment for pediatric patients with sickle cell anemia – Siklos® (hydroxyurea)

- Siklos® HCP The first and only hydroxyurea-based treatment for BOTH adult and pediatric patients with sickle cell anemia Unique hydroxyurea pediatric indication Body-weight adjusted dosing Dissolvable in water for oral administration All individuals depicted throughout website are not actual patients. Important Safety Information Prescribing Information Medication Guide Instructions for Use Patient Site Clinical Experience Prescribing Siklos ® FAQ News and Resources Savings Important Safety Information Prescribing Information Medication Guide Instructions for Use Patient Site What is Siklos ® ? Siklos ® is the first and only FDA-approved hydroxyurea-based treatment indicated to reduce the frequency of painful crises and to reduce the need for blood transfusions in adult and pediatric patients 2 years of age and older with sickle cell anemia with recurrent moderate to severe painful crises. 1 Impact of Sickle Cell Disease Children with sickle cell disease (SCD) are at increased risk of infection and recurrent painful episodes during childhood. These may lead to multi-organ damage, associated with poor prognosis and early mortality. 2 Therefore, as recommended by the National Institutes of Health (NIH), it is important to start disease-modifying therapy with hydroxyurea as early as possible. 3 Additionally, it is important to continue Hydroxyurea treatment into adulthood in order to reduce the frequency and severity of complications of Sickle Cell Disease. 3 Consider Siklos for your appropriate patients! Why Siklos ® ? Siklos ® is the first and only hydroxyurea-based sickle cell disease treatment indicated for both adult and pediatric patients.. Siklos ® has two strengths to help optimize dosing: 100 mg scored tablets and 1,000 mg triple-scored tablets. Siklos ® tablets are small* and breakable allowing for dosing adjustments in 50 mg increments. Siklos ® has been found to be safe and effective in adults and children aged 2 and older with sickle cell disease with recurrent moderate to severe painful crisis. See Clinical Data . Siklos ® tablets may be dissolved in water for those who have trouble swallowing them whole. Please see Instructions for Use . Siklos ® can accompany growing patients along their treatment journeys into adulthood. * can be broken into smaller parts Prescribing Siklos ® As the patient grows, Siklos ® allows for flexible dosing based on body weight, blood count, and clinical response. Therefore, patient growth and blood counts must be closely monitored throughout treatment and dosage adjusted accordingly. Learn more about prescribing Siklos ® Siklos ® is available in 100 mg scored (2 x 50 mg) tablets and in 1,000 mg triple-scored (4 x 250 mg) tablets to allow dose adjustments in increments as small as 50 mg, based on patient body weight, blood count, and clinical response. When both strengths of Siklos ® (100 mg and 1,000 mg) are prescribed simultaneously, make sure that the patient and/or the parents or caregivers understand the dosing in order to avoid confusion. Please see Full Prescribing Information , including Boxed Warning regarding myelosuppression and malignancies. Mechanism of Action Hydroxyurea has many characteristics of an ideal drug for sickle cell anemia (SCA) and provides therapeutic benefit through multiple mechanisms of action”. 4 Fetal hemoglobin (HbF) appears to minimize clinical severity of sickle cell disease and low levels of HbF are associated with a higher risk of vaso-occlusive complications, organ damage and early death. 5 That is where hydroxyurea comes in. As explained by Dr. Barbara P. Yawn and colleagues 6 , Increasing the concentration of fetal hemoglobin is the primary effect of hydroxyurea and provides the greatest benefit to persons with SCD, but other mechanisms of action and benefits exist. For example, hydroxyurea lowers the number of circulating leukocytes and reticulocytes and decreases their expression of adhesion molecules, thus reducing vascular occlusion. Hydroxyurea also increases red blood cell size and improves cellular deformability, which increases blood flow and reduces vaso-occlusion. In addition, nitric oxide released directly from hydroxyurea metabolism may contribute to local vasodilation. 7 Hydroxyurea therapy substantially reduces the frequency of painful episodes and ACS events and the need for erythrocyte transfusions and hospitalizations. 8 Long-term hydroxyurea administration results in a reduction in mortality." 9-10 The precise mechanism by which hydroxyurea produces its cytotoxic and cytoreductive effects is not known. However, various studies support the hypothesis that hydroxyurea causes an immediate inhibition of DNA synthesis by acting as a ribonucleotide reductase inhibitor, without interfering with the synthesis of ribonucleic acid or of protein. 1 The mechanisms by which Siklos ® produces its beneficial effects in patients with sickle cell anemia are uncertain. Known pharmacologic effects of Siklos ® that may contribute to its beneficial effects include increasing hemoglobin F levels in red blood cells, decreasing neutrophils, increasing the water content of red blood cells, increasing deformability of sickled cells, and altering the adhesion of red blood cells to endothelium. Siklos ® News Stay up to date on Siklos ® Sign up to receive the latest news about Siklos ® . Subscribe now Siklos ® Savings Want to help patients pay less for their Siklos ® prescription? Download and print savings options for your patients Patients having difficulty affording their Siklos ® prescription? Learn more about our Patient Assistance Program References Siklos ® (hydroxyurea) tablets, for oral use [Prescribing Information]. Addmedica, December 2021. Ferster, A. et al. Hydroxyurea for treatment of severe sickle cell anemia: a pediatric clinical trial. Blood 88, 1960-1964 (1996). Yawn BP, John-Sowah, J. Management of Sickle Cell Disease: Recommendations from the 2014 Expert Panel Report. Am Fam Physician. 2015 Dec 15;92(12):1069-76. Ware, R.E. How I use hydroxyurea to treat young patients with sickle cell anemia. Blood. 2010;115](26):5300-5311. Leikin SL, Gallagher D, Kinney TR, et al. Mortality in children and adolescents with sickle cell disease: Cooperative Study of Sickle Cell Disease. Pediatrics. 1989;84(3):500-508; Platt OS, Brambilla DJ, Rosse WF, et al. Mortality in sickle cell disease: life expectancy and risk factors for early death. N Engl J Med. 1994;330(23): 1639-1644. Management of Sickle Cell Disease, Summary of the 2014 Evidence-Based Report by Expert Panel Members. Barbara P. Yawn, MD, MSc, MSPH1; George R. Buchanan, MD2; Araba N. Afenyi-Annan, MD, MPH3; et al. JAMA. 2014;312(10):1033-1048. doi:10.1001/jama.2014.10517. King SB. Nitric oxide production from hydroxyurea. Free Radic Biol Med. 2004;37(6):737-744. Charache S, Terrin ML, Moore RD, et al; Investigators of the Multicenter Study of Hydroxyurea in Sickle Cell Anemia. Effect of hydroxyurea on the frequency of painful crises in sickle cell anemia. N Engl J Med. 1995;332(20):1317-1322. Steinberg MH, Barton F, Castro O, et al. Effect of hydroxyurea on mortality and morbidity in adult sickle cell anemia: risks and benefits up to 9 years of treatment [published correction appears in JAMA. 2003;290(6):756]. JAMA. 2003;289(13):1645-1651. Voskaridou E, Christoulas D, Bilalis A, et al. The effect of prolonged administration of hydroxyurea on morbidity and mortality in adult patients with sickle cell syndromes: results of a 17-year, single-center trial (LaSHS). Blood. 2010;115(12): 2354-2363. Indication and important safety information INDICATION SIKLOS is an antimetabolite indicated to reduce the frequency of painful crises and to reduce the need for blood transfusions in adult and pediatric patients 2 years of age and older, with sickle cell anemia with recurrent moderate to severe painful crises. IMPORTANT SAFETY INFORMATION WARNING: MYELOSUPPRESSION and MALIGNANCIES See Full Prescribing Information...