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                                                                                                                            Approaches to sickle cell anemia management

           Sickle cell anemia is an inherited red blood cell disorder in which there are not enough healthy red blood cells to carry oxygen throughout the body. Sickle cell diseases are due to a mutation in the gene responsible for producing HbA, which results in the formation of a different Hb, called S, which has recessive inheritance (Vieira Figueiredo et al., 2018). In individuals presenting with this disease, the erythrocytes assume the shape of a scythe, which hinders adequate blood flow, generating processes of Vaso-occlusion and infarcts in the affected areas. Therefore, ischemia, pain, damage to various organs and tissues, necrosis, and chronic hemolysis. In addition, the regional hypoxia process itself may intensify the sickling of new erythrocytes, increasing ischemic damage (McCance Burns et al., 2017.

All babies have a newborn screening test for SCD. Newborn screening checks for serious but rare and mostly treatable conditions at birth. It includes blood, hearing, and heart screening. With newborn screening, SCD can be found and treated early. Management of a child with sickle cell anemia (SCA) is complicated and should be done in consultation with a pediatric hematologist (McCance Burns et al., 2017).  Children with sickle cell disease still need regular primary care services, consultative services, and information coordination. Some of the critical aspects of care for the child are hydration, illness prevention, and pain management, which are fundamental aspects of disease management: NSAIDS or acetaminophen may be adequate for mild to moderate pain; CBC and reticulocyte count are monitored every few months; All the usual childhood immunizations are to be administered on time (McCance Burns et al., 2017). 

At two-month-old, penicillin V prophylaxis, 125mg orally twice daily, should be initiated. At three years old, the dose should be increased to 250 mg orally twice a day and continue at least until the fifth birthday or until the child received two doses of PPSV23. Aggressive treatment of infections and maintenance of hydration and body temperature are used to prevent hypoxia and acidosis. Furthermore, treatment of coexisting medical problem associated with lower oxygen saturations such as asthma and obstructive sleep apnea should be done (Phillips, 2021).

In addition, in children with severe SCA, hydroxyurea is used to reduce the number of painful crises and incidences of acute chest syndrome. Last, annual stroke prevention screening of major intracranial vessels transcranial doppler ultrasound evaluation is planned for 2- to 16-year-old children if their bone windows allow meaningful evaluation (McCance Burns et al., 2017).

           In resume, children with sickle cell anemia should be treated as follow: Pain medicines for a crisis, drinking plenty of water daily (8 to 10 glasses in some cases, IV fluids may be needed, Blood transfusions to treat anemia, chronic pain, acute chest syndrome, and splenic sequestration, and to prevent stroke; Vaccines and antibiotics to prevent infections; Folic acid to help prevent severe anemia; regular eye exams yearly to screen for an eye condition called retinopathy; Stem cell transplant can cure some children with SCD; Hydroxyurea to reduce the number of sickle cells in the blood. It reduces complications, painful episodes (crises), and hospital stays (Phillips, 2021).

Best care practices for SCD include preventive measures to minimize morbidity and mortality, manage symptoms, and recognize severe life-threatening complications (Phillips, 2021). Family members’ knowledge about sickle cell disease varied. Therefore, they should receive sickle cell disease health guidelines to help them cope with the illness in their child’s life (Vieira Figueiredo et al., 2018). In addition, the APRN should assess for psychosocial complications, make appropriate referrals, provide patient and family education and promote patient and family self-management.

References 

McCance Burns, C. E., Dunn, M. A., Brady, A. M., Starr, B. N., Blosser, G. C., & Garzon, L. D. (2017). Pediatric primary care (6th ed.). Elsevier.

Phillips, S. (2021). Caring for individuals with sickle cell disease. American Nurse Today, 16(6), 6–11.

Vieira Figueiredo, S., Alexandre Lima, L., Batista e Silva, D. P., Carvalho Oliveira, R. de M., Pinto dos Santos, M., & Verde Gomes, I. L. (2018). Importance of health guidance for family members of children with sickle cell disease. Revista Brasileira de               Enfermagem, 71(6), 2974–2982. https://doi.org/10.1590/0034-7167-2017-0806