Author: Kara Martina, Biopsychology B. Sc.
The blood Journal describes Hydroxyurea as both a feared and an overused drug. It's praised one moment, shot down the next. What's the truth about hydroxyurea and its potential treatment for those with Sickle Cell disease? The answers aren't so simple. Given its both lauded and loathed reputation, hydroxyurea is worth knowing more about......
Did you know hydroxyurea reduces the amount of sickle cell crises?
According to WebMD, Hydroxyurea increases fetal hemoglobin production (HbF). Fetal hemoglobin is the main oxygen transport for unborn babies. HbF continues to develop until the babies are roughly six months old. But small amounts also persist throughout adult hood, especially in those with the sickle cell trait, who have a genetic predisposition to produce more of it. Fetal hemoglobin reduces the chance that red blood cells will sickle by interfering with the polymerization of the sickle cell hemoglobin and then reducing the occurrence of sickling-related complications (1). Which is why hydroxyurea is prescribed widely to adults and children alike with Sickle Cell Anemia.
Did you know hydroxyurea also kills your healthy cells?
Hydroxyurea is classified as a chemotherapy drug. Once absorbed into the cells, hydroxyurea prevents those cells from dividing, resulting in their death. According to the American Cancer Society, chemotherapy drugs kill cells that are rapidly dividing. But unfortunately, chemotherapy does not know the difference between the cancerous cells and the normal cells. Hydroxyurea isn’t different; it sees Sickle blood cells and normal body cells equally. It also attacks the cells in the mouth, stomach and bowel, and the hair follicles. The result can be low blood counts, mouth sores, nausea, diarrhea, and even hair loss. The side effects of Hydroxyurea and their degree depend on how much of the drug is taken, varying from person to person.
Did you know Leukemia has been linked as a potential side effect of taking hydroxurea?
Although not considered leukemogenic, according to the US National Library of Medicine, several studies have questioned the link between prior use of hydroxyurea and later leukemia on-set (2). According to WebMD, more research is needed on the long term effects of hydroxyurea (3).
Did you know alternatives are being developed?
Despite its use for over 30 years, hydroxyurea’s method of action is uncertain. However, recent studies have demonstrated that hydroxyurea generates the nitric oxide (NO) radical, a chemical involved in vasodilatation. It’s been hypothesized that NO-donor properties might determine the hemoglobin phenotype expressed in the body (4). Strong evidence for a NO-derived mechanism of HbF induction by hydroxyurea suggest possibilities for therapies based on NO-releasing or NO-inducing agents. Perhaps in the future, alternatives to chemotherapy will be available to Sickle Cell patients.
References
1. Alkinsheye I, Alsultan A, Solovieff N, DuyenNgo, Baldwin C, Sebastiani P, Chui D, Steinberg M (2011). Fetal hemoglobin in sickle cell anemia. BloodJournal, 118(1):19-27.
2. Baz W, Forte F, Najfeld V, Yotsuya M, Talwar J, Terjanian T (2012). Development of Myelodysplastic Syndrome and Acute Myeloid Leukemia 15 Years after Hydroxyurea Use in a Patient with Sickle Cell Anemia. Clin Med Insights Oncol, 2012; 6: 149–152.
3. Strouse JJ, et al. (2008). Hydroxyurea for sickle cell disease: A systematic review for efficacy and toxicity in children. Pediatrics, 122(6): 1332–1342.
4. Beleslin-Cokic B, Cokic V, Gladwin M, Miller J, Njoroge J, Schechter A, Smith R (2003). Hydroxyurea induces fetal hemoglobin by the nitric oxide–dependent activation of soluble guanylyl cyclase. Journal of Clinical Investigation. 111(2): 231–239
The blood Journal describes Hydroxyurea as both a feared and an overused drug. It's praised one moment, shot down the next. What's the truth about hydroxyurea and its potential treatment for those with Sickle Cell disease? The answers aren't so simple. Given its both lauded and loathed reputation, hydroxyurea is worth knowing more about......
Did you know hydroxyurea reduces the amount of sickle cell crises?
According to WebMD, Hydroxyurea increases fetal hemoglobin production (HbF). Fetal hemoglobin is the main oxygen transport for unborn babies. HbF continues to develop until the babies are roughly six months old. But small amounts also persist throughout adult hood, especially in those with the sickle cell trait, who have a genetic predisposition to produce more of it. Fetal hemoglobin reduces the chance that red blood cells will sickle by interfering with the polymerization of the sickle cell hemoglobin and then reducing the occurrence of sickling-related complications (1). Which is why hydroxyurea is prescribed widely to adults and children alike with Sickle Cell Anemia.
Did you know hydroxyurea also kills your healthy cells?
Hydroxyurea is classified as a chemotherapy drug. Once absorbed into the cells, hydroxyurea prevents those cells from dividing, resulting in their death. According to the American Cancer Society, chemotherapy drugs kill cells that are rapidly dividing. But unfortunately, chemotherapy does not know the difference between the cancerous cells and the normal cells. Hydroxyurea isn’t different; it sees Sickle blood cells and normal body cells equally. It also attacks the cells in the mouth, stomach and bowel, and the hair follicles. The result can be low blood counts, mouth sores, nausea, diarrhea, and even hair loss. The side effects of Hydroxyurea and their degree depend on how much of the drug is taken, varying from person to person.
Did you know Leukemia has been linked as a potential side effect of taking hydroxurea?
Although not considered leukemogenic, according to the US National Library of Medicine, several studies have questioned the link between prior use of hydroxyurea and later leukemia on-set (2). According to WebMD, more research is needed on the long term effects of hydroxyurea (3).
Did you know alternatives are being developed?
Despite its use for over 30 years, hydroxyurea’s method of action is uncertain. However, recent studies have demonstrated that hydroxyurea generates the nitric oxide (NO) radical, a chemical involved in vasodilatation. It’s been hypothesized that NO-donor properties might determine the hemoglobin phenotype expressed in the body (4). Strong evidence for a NO-derived mechanism of HbF induction by hydroxyurea suggest possibilities for therapies based on NO-releasing or NO-inducing agents. Perhaps in the future, alternatives to chemotherapy will be available to Sickle Cell patients.
References
1. Alkinsheye I, Alsultan A, Solovieff N, DuyenNgo, Baldwin C, Sebastiani P, Chui D, Steinberg M (2011). Fetal hemoglobin in sickle cell anemia. BloodJournal, 118(1):19-27.
2. Baz W, Forte F, Najfeld V, Yotsuya M, Talwar J, Terjanian T (2012). Development of Myelodysplastic Syndrome and Acute Myeloid Leukemia 15 Years after Hydroxyurea Use in a Patient with Sickle Cell Anemia. Clin Med Insights Oncol, 2012; 6: 149–152.
3. Strouse JJ, et al. (2008). Hydroxyurea for sickle cell disease: A systematic review for efficacy and toxicity in children. Pediatrics, 122(6): 1332–1342.
4. Beleslin-Cokic B, Cokic V, Gladwin M, Miller J, Njoroge J, Schechter A, Smith R (2003). Hydroxyurea induces fetal hemoglobin by the nitric oxide–dependent activation of soluble guanylyl cyclase. Journal of Clinical Investigation. 111(2): 231–239
