Assignment: Approaches to Disease Management: Sickle Cell Anemia
Assignment: Approaches to Disease Management: Sickle Cell Anemia
Approaches to Disease Management: Sickle Cell Anemia
I will prescribe hydroxyurea to the child at 2 months, 2 years, 6 years, and 13 years old. Hydroxyurea is the drug of choice for patients with sickle cell anemia. Hydroxyurea works by inhibiting ribonucleotide reductase. Ribonucleotide reductase is an enzyme that transforms ribonucleosides into deoxyribonucleosides, which are the building blocks needed for DNA synthesis. The inhibition of DNA synthesis leads to reduced cellular cytotoxicity. Besides the above, hydroxyurea suppresses cell stress signaling pathways and erythroid progenitors, which alters erythropoiesis kinetics and increases HbF levels through elevated production of erythroid progenitors (Ryan et al., 2020; Wang et al., 2021). Hydroxyurea’s cytotoxic effects suppress bone marrow production of reticulocytes, neutrophils, and platelets, hence, reducing inflammation risks in sickle cell disease.
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Each of the ages above has issues that should be considered in the treatment of sickle cell anemia. A 2-month-old infant has an immature immune system and organs involved in drug absorption, metabolism, and excretion as compared to a 13-year-old child. The infant has poor drug absorption, distribution, metabolism, and elimination. Often, the infant has delayed gastric emptying, which lowers drug absorption. The body of the neonate has a high percentage of water as compared to fat. This is the opposite in older children and adolescents. High water volume lowers the distribution of fat-soluble drugs and increases the absorption of water-soluble medications. The hepatic enzymes involved in hydroxyurea metabolism are immature in neonates and infants as compared to older children and adolescents. As a result, the risk of hydroxyurea toxicity is high due to reduced metabolism and prolonged half-life. Newborns and infants also have immature renal functions as compared to older children and adolescents (dos Santos Neres et al., 2023). This affects the elimination of drug metabolites, which predisposes them to drug toxicity.
Several referral options exist for this child. They include a referral to a pediatric hematologist, pediatrician, pediatric nurse, genetic counselor, and pain management specialist. Pain management specialists will help the patient in managing acute pain experienced during sickle cell crises. Genetic counselors will support the patient and her family regarding the genetic risks of the disease. The child’s family will also learn about the inheritability pattern of sickle cell anemia and the available treatment options. Pediatric nurses will provide ongoing assessment and treatment to the child. They will also link the family with social support networks for children with sickle cell anemia. Pediatricians will be actively involved in providing medical care and regular assessment for the children (Morse et al., 2022). They will ensure that the child’s immunization record is up-to-date.
Pediatric hematologists will help in the assessment, planning, implementation, monitoring, and evaluation of different treatments such as hydroxyurea and blood transfusion for sickle cell anemia. These professionals will collaborate to ensure the provision of patient-centered care. I will coordinate the care given to the child using telehealth. Telehealth will enable virtual assessment, planning, monitoring, and evaluation of the care given to the child (Morse et al., 2022). Healthcare providers will collaborate virtually with the child’s family to ensure the delivery of safe, efficient, and high-quality care that prevents and minimizes sickle cell crises.
References
dos Santos Neres, J. S., Yahouédéhou, S. C. M. A., & Goncalves, M. S. (2023). Effectiveness of Pharmacokinetic-Guided Hydroxyurea Dose Individualization in Patients with Sickle Cell Anemia: A Mini-Review. Pharmaceuticals, 16(6), Article 6. https://doi.org/10.3390/ph16060857
Morse, B. L., Carmichael, A. E., Bradford, V. A., & Pollard, A. L. (2022). Sickle Cell Disease Care Planning for School Nurses. NASN School Nurse, 37(1), 48–54. https://doi.org/10.1177/1942602X211025079
Ryan, N., Dike, L., Ojo, T., Vieira, D., Nnodu, O., Gyamfi, J., & Peprah, E. (2020). Implementation of the therapeutic use of hydroxyurea for sickle cell disease management in resource-constrained settings: A systematic review of adoption, cost and acceptability. BMJ Open, 10(11), e038685. https://doi.org/10.1136/bmjopen-2020-038685
Wang, W. C., Zou, P., Hwang, S. N., Kang, G., Ding, J., Heitzer, A. M., Schreiber, J. E., Helton, K., & Hankins, J. S. (2021). Effects of hydroxyurea on brain function in children with sickle cell anemia. Pediatric Blood & Cancer, 68(10), e29254. https://doi.org/10.1002/pbc.29254
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Approaches to Disease Management: Sickle Cell Anemia
A 2-month-old is identified during newborn screening with sickle cell anemia.
How would you manage this patient at 2 months, 2 years, 6 years, and at 13 years old?
What are the issues for each stage in development?
Where would you refer this child?
How would you coordinate the care of this child?
.Submission Instructions:
Your initial post should be at least 500 words, formatted and cited in current APA style with support from at least 2 academic sources.