• Ferlanda Pierrelus.

Sickle Cell Anemia

• Sickle cell anemia (SCA) is a blood illness caused by a lack of healthy erythrocytes responsible for transporting oxygen throughout the body. It is a genetically acquired disorder. People with SCA have crescent moon-shaped erythrocytes instead of the spherical and flexible erythrocytes found in healthy individuals. Early detection of life-threatening complications such as splenic sequestration and sepsis, according to experts, is recommended (Runkel, Klüppelholz, Rummer, Sieben, Lampert, Bollig, C….& Angelescu, 2020). This document explains SCA management approaches for a patient who is two months, two years, six years, and thirteen years old at various points in time.

Sickle cell disease is inherited in an autosomal recessive manner. Although it is most frequently found in people of African ancestry, it has also been recognized in ethnic groups from the Mediterranean, the Caribbean, Central and South America, and India ( Burns, Dunn, Brady, Starr, Blosser, & Garzon, 2017).

SCA management is complicated and should be undertaken with a pediatric hematologist ( Burns et al., 2017). Children with sickle cell disease continue to require routine primary care services and coordination of consultative and informational services. Growth is regularly monitored, vaccines must be administered on time, parents need assistance, and contact with specialty services, such as an annual ophthalmologic check by a retinal expert, should be organized. Comprehensive care ranges from routine well-child care to acute crises and hospitalization. Several critical components of caring for a child with SCA include the following:

Maintaining adequate hydration, preventing sickness, and managing pain are critical components of disease management. While NSAIDs or acetaminophen may be sufficient for mild to moderate pain, opioids should be taken when insufficient. (As is the case with everyone who uses opioids, abuse and addiction must be considered.)

The complete blood count (CBC) and reticulocyte count are performed every few months.

All routine childhood immunizations must be given on time, including the 13-valent pneumococcal conjugate vaccine (four doses at appropriate intervals) and the 23-valent pneumococcal polysaccharide vaccine (first dose at or after 24 months of age), with a second dose of PPSV23 given three years after the first dose. Infants with sickle cell disease should receive the conjugate Hib and meningococcal vaccine (HibMenCY) at 2, 4, 6, and 12 to 15 months of age, followed by booster doses MCV4 every five years after that. Annual influenza immunization is critical ( Burns et al., 2017).

The most common cause of death in young infants with SCA is an invasive bacterial infection. With penicillin V (125 mg orally, twice a day), prevention should begin at two months of age. Increase the dose to 250 mg orally twice a day at the age of three and continue for at least five years or until the kid has received two doses of PPSV23 (Rogers, 2015).

Hydroxyurea is used to lower the number of painful crises and the incidence of acute chest syndrome (a primary cause of death in adolescents with SCA) in children aged 13 years and older with severe SCA ( Burns et al.,2017). For 2- to 16-year-old children, annual stroke prevention screening of major intracranial arteries with transcranial Doppler ultrasound evaluation is planned, or as long as their bone windows enable significant evaluation. A time-averaged mean maximum velocity of more than 200 cm/sec suggests a significant risk of stroke and should prompt the start of transfusion programs to keep Hgb S levels below 30%. ( Burns et al.,2017).

SCA symptoms often appear in the second 6 months of life when Hgb S increases and the amount of Hgb F decreases. As a result, painful vaso-occlusive crises develop. Because of the multisystem nature of the difficulties, these children require immediate, thorough examination and care. After five years old, splenomegaly disappears typically because of the autoinfarction of the organ. After the age of seven, height and weight gain rates generally slow, and puberty may be delayed by three to four years ( Burns et al., 2017).

• Patients with SCA may experience both short- and long-term problems, including organ damage, cognitive impairment, stroke, "vaso-occlusive pain crises," and "acute chest syndrome," necessitating thorough care. Nurses, primary care practitioners, pediatricians, dieticians, laboratory technicians, and hematologists would comprise the expert team. Coordination of care is crucial since it can help reduce fragmentation of care and improve client interactions for a kid with SCA (Burns et al., 2017). Coordination of care would ensure a safe transition, thereby avoiding potential errors. For instance, the dietician might teach the client the recommended nutritional practices to promote growth and development.
• 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.).

Runkel, B., Klüppelholz, B., Rummer, A., Sieben, W., Lampert, U., Bollig, C., …&Angelescu, K. (2020). Screening for sickle cell disease in newborns: a systematic review. Systematic reviews, 9(1), 1-9.