REPLY POSTS:Reply separately to two of your classmates article critique's posts (See attached classmates posts, post#1 and post#2). - Use at least two scholarly references per peer post. The expectati

RUNNING HEAD: SICKLE CELL ANEMIA

Discussion Post – Week 4

Sickle Cell Anemia

Chapter 10

Regis College

Jennifer Pike

The purpose of this discussion post is to critique a research article focused on the treatment of Sickle Cell Anemia (SCA) with Hydroxyurea. SCA is attributed to an inherited characteristic that leads to the formation of abnormal hemoglobin, referred to as hemoglobin S (HbS) (Hubert & VanMeter, 2018). There are over 300,000 infants born each year worldwide with sickle cell anemia, it is an autosomal recessive disease. The clinical manifestations of SCA can include hemolytic anemia, vaso-occlusive crisis, and bacterial susceptibility that can have a lasting impact on many of the organs (Becker et al., 2019). Many of the signs and symptoms of SCA do not appear until a child is around 12 months of age when their fetal hemoglobin has been replaced by the HbS, the amount of hemoglobin that is replaced by HbS determines how severe the illness will be (Hubert & VanMeter 2018). One of the medications used for the treatment of SCA is Hydroxyurea which when effective for an individual is able to reduce the frequency of vaso-occlusive episodes and prolong the lifespan.

The article titled, “Optimizing Hydroxyurea Treatment for Sickle Cell Disease Patients: The Pharmacokinetic Approach,” the article was published in the Journal of Clinical Medicine in October 2019. The authors include Charlotte Nazon, Amelia-Naomi Sabo, Guillaume Becker, Jean-Marc Lessinger, Veronique Kemmel, and Catherine Paillard. The purpose of the article presented is to optimize and encourage the use of Hydroxyurea in SCA patients by showing the efficacy in reducing the frequency of vaso-occlusive episodes, hospitalizations, need for blood transfusions, and overall improved quality of life. The authors used a method of dose increasing in order to reach optimal level of treatment.

The participants of the study were patients with SCA that were under 20 years of age and being treated with Hydroxyurea currently by the authors hospital of affiliation between February and May 2018. The sample size consisted of 9 patients that were on Hydroxyurea for multiple vaso-occlusive events or acute chest syndrome. According to the researchers most of the participants in the study had already been on Hydroxyurea for more than four years with a daily dose of 12.9 to 24.6 mg/kg/day. Plasma samples of the participant was collected at pre-determined times including, pre-dose, at 10 minutes, 20 minutes, 1 hour, 2 hour, 4 hour, and the 6 hour mark after dosing of the patients usual dose. The levels of neutrophil count, reticulocyte count and platelet count were monitored in order to ensure maximum dose tolerance could be reached without the risk of toxicity.

None of the patients in the study were able to reach maximum dose tolerance with myelosuppression. The inability of the participants to reach maximum dose tolerance was attributed to two factors. The dose used on the patients in this study was smaller than the doses used in previous studies with better results. Another factor that was brought up was patients’ compliance to taking the medication. Four out of the 9 patients were suspected as being non-compliant due their low MCV levels. The authors described another gap and limitation of this research study is using the patients as reporting compliance as this can often be unreliable method of knowing whether or not they were truly compliant. Another large limitation of this study is the sample size of only 9 patients, this doesn’t allow for the most accurate or widely accepted number of evidence having such a small sample size.

What is made apparent in this research study is the importance of proper hydroxyurea dosing to improve the lives of patients with SCA. This is important for any APRN who is treating a patient with SCA at the level of preventive care to optimize and pro-long their lives. The use of Hydroxyurea is widely known to help reduce vaso-occlusive episodes is not as widely used as it could or should be. SCA being prominent starting in early childhood can bring some fears in use of such a strong medication. Parents are often fearful of the medication and the side effects that can happen without having significant knowledge of the benefits of the medication (Bogen et al., 2015). As APRNs taking care of children or anyone with SCD it is important to be up to date of the proper dosing and usage of Hydroxyurea because it is one of the only medications that is approved to help. Bogen et al., 2015, says that providers should offer hydroxyurea to all pediatric patients with severe SCD to encourage that the parents have shared decision making when it comes to the treatment of their child. This article is important to highlight the usage of the medication and I would recommend this to any provider or colleague who was treating children with SCD who needed guidance or any education on the dosage and use of the medication.

References:

Becker, G., Kemmel, V., Lessinger, J.-M., Paillard, C., Nazon, C., & Sabo, A.-N. (2019). Optimizing Hydroxyurea Treatment for Sickle Cell Disease Patients: The Pharmacokinetic Approach. Journal of Clinical Medicine, 8(10), 1701–1701. https://doi.org/10.3390/jcm8101701

Bogen, D. L., Creary, S., Krishnamurti, L., Ross, D., & Zickmund, S. (2015). Hydroxyurea therapy for children with sickle cell disease: Describing how caregivers make this decision. BMC Research Notes, 1. https://doi.org/10.1186/s13104-015-1344-0

Hubert, R., & VanMeter, K. (2018). Gould’s Pathophysiology for the Health Professions (Sixth). Elsevier.