Approaches to Disease Management
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?
• Your initial post should be at least 500 words, formatted and cited in current APA style with support from at least at least 3 academic sources no older than 5 years (2017-2022).
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Approaches to Disease Management
Sickle cell disease is a genetic autosomal recessive disorder that occurs due to the mutation of the normal hemoglobin (Hb) to sickle Hb, HbS (Runkel et al., 2020). Various management strategies are used to avoid complications and symptoms and vaso-occlusive crises. Sickle cell disease management requires a multidisciplinary approach. This discussion post focuses on the management of a 2-month-old infant diagnosed with sickle cell management at different ages, specifically at 2 months, 2 years, 6 years, and 13 years.
On diagnosis of sickle cell disease, the child should be referred to a hematologist who is an expert in blood disorders. Infants with sickle cells disease start manifesting symptoms of sickle cell anemia around 5 months because fetal hemoglobin prevents sickling of the red blood cells (Runkel et al., 2020). At around five months old, the sickle hemoglobin replaces the fetal hemoglobin and thus the symptoms start manifesting. Therefore, at 2 months old, the infant requires preventative interventions such as ensuring that the infant receives the pneumonia vaccination to reduce and prevent the risk of pneumonia. At 2 months, the infant needs to be administered with prophylactic penicillin in children. The child also needs a Meningococcus vaccine at 2, 4, 6, 12, and 15 months of age. The infant also needs to receive all the routine immunizations. The parents need education on the care, complications, management, and warning signs in sickle cell.
At 2 years, it is important to ensure the child is adhering to the pneumococcal vaccinations and receiving prophylactic oral penicillin (125 mg twice daily) until the age of 5 years (Ogu et al., 2021). The child should also receive all routine immunizations. Additionally, the child needs folic acid supplementation because vitamin B helps to make healthy red blood cells (Sanyaolu et al., 2020). The child should get a flu shot annually after 6 months of age. For pain management, the child can be administered ibuprofen as required.
At five years, the prophylactic penicillin should be discontinued and this means that unless the child has invasive pneumococcal disease or splenectomy, the prophylactic penicillin should not be administered at six years (Ogu et al., 2021). At six years, the child should be administered a dose of 13-valent pneumococcal conjugate vaccine. Hydroxyurea therapy is also recommended to increase fetal hemoglobin and improve health outcomes such as reducing the frequency of crises and the need for RBC transfusions (Salinas & Thein, 2020). At six years, the child and the mother need to be educated on various measures in sickle cell disease management such as drinking plenty of water, trying not to get too cold or too hot, avoiding situations associated with high attuites such as flying or climbing, athletic competition or exhausting exercises (Ogu et al., 2021). Booster meningitis immunization needs to continue as recommended. Additionally, at 6 years the child should continue receiving a flu shot and folic acid. A high intake of diet rich in iron, vegetables, and fruits are recommended. To help decrease the number of pain crises, hydroxyurea is recommended (Sanyaolu et al., 2020). However, close monitoring by a doctor is important to prevent complications and infections.
At 13 years, close monitoring needs to continue, including receiving the flu and pneumonia shots accordingly. The child should also be referred to an ophthalmologist for dilated retinal examination. The child should also be closely monitored by a nephrologist to examine the kidney function, including screening for renal disease (Ogu et al., 2021). This should actually start at 10 years of age. It is also important to screen the child for vascular disease and stroke risk. Apart from management, at 13 years the child needs mental health help from a mental health practitioner to provide counseling that may be necessary.
The multidisciplinary team members involved in the care of people with sickle cell disease include a hematologist, primary care provider, cardiologist, neurologist, pulmonologists, nurses, mental health practitioner, and a social worker (Al Zahrani et al., 2020). Each team member has a role to play, for example, the cardiologist manages heart issues, the pulmonologist manages respiratory issues, the hematologist manages sickle cell condition including RBC levels while a mental health practitioner provides the necessary counseling services, etc.
Al Zahrani, O., Hanafy, E., Mukhtar, O., Sanad, A., & Yassin, W. (2020). Outcomes of multidisciplinary team interventions in the management of sickle cell disease patients with opioid use disorders. A retrospective cohort study. Saudi medical journal, 41(10), 1104–1110. https://doi.org/10.15537/smj.2020.10.25386
Ogu, U. O., Badamosi, N. U., Camacho, P. E., Freire, A. X., & Adams-Graves, P. (2021). Management of Sickle Cell Disease Complications Beyond Acute Chest Syndrome. Journal of blood medicine, 12, 101–114. https://doi.org/10.2147/JBM.S291394
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.
Salinas G., & Thein, S. L. (2020). Recent Advances in the Treatment of Sickle Cell Disease. Frontiers in physiology, 11, 435. https://doi.org/10.3389/fphys.2020.00435
Sanyaolu, A., Agiri, E., Bertram, C., Brookes, L., Choudhury, J., Datt, D., … & Williams, M. (2020). Current modalities of sickle cell disease management. Blood Science, 2(04), 109-116.