is further manifested with abdominal pain, distension and a systolic murmur (Ravikanth, Abraham & Alapati, 2017). Three differentials in this case would be psoriatic arthritis as it also presents with dactylitis, juvenile rheumatoid arthritis as it also presents with joint pain and septic arthritis as it also presents with joint pain and swelling. In SCD, the spleen doesn’t work properly or doesn’t work at all. This problem makes people with SCD more likely to get severe infections. What is the treatment for Miah and education for the family?The leading cause of death of young children with sickle cell disease is an invasive bacterial infection (Burns et al., 2016). Severe infections can lead to death due to a compromisedimmune system from spleen dysfunction, so prophylactic antibiotics are given to combat this risk. Penicillin should be given to all patients from 2 months to 5 years, after which evidence-based research shows no clinical benefit from continuing the antibiotic regimen (National Heart, Lung and Blood Institute, 2014). According to clinical practice guidelines in the management of sickle cell disease, from 2 months to 3 years, the child is given 125mg of penicillin, twice daily, then from 3-5 years, the child is given 250mg of penicillin, twice daily (National Heart, Lung and Blood Institute, 2014).Family education includes emphasis on the severity of the risk associated with a bacterial infection, the high risk and fatality associated with septicemia and meningitis, the importance of maintaining hydration and avoiding hypoxic states associated with exertion, ensuring patients are vaccinated or on the road to becoming vaccinated with all recommended childhood vaccines especially Streptococcus pneumoniae, and the need to seek medical attention for fevers of 101.3°F or 38.5°C due to the risk of severe infection (Burns et al.,2016; National Heart, Lung and Blood Institute, 2014). Additionally, the importance of simple hand hygiene to control infection should always be emphasized.
Burns, C., Dunn, A., Brady, M., Starr, N., Blosser, C. (2016). Pediatric primary care: A handbook for nurse practitioners, (6th ed.). Philadelphia: Saunders. ISBN:9780323243384National Heart, Lung and Blood Institute. (2014). Evidence-based management of sickle cell disease: Expert panel report, 2014. Retrieved from -topics/all-publications-and-resources/evidence-based-management-sickle-cell-disease-expert-0Ravikanth, R., Abraham, M. J., & Alapati, A. (2017). Musculoskeletal manifestations in sickle cell anemia. Medical Journal of Dr. D.Y. Patil University, 10 (5), 453–457.
Heather, Great job on your discussion post! Education is a crucial part in effectively managing patients and families with sickle cell crisis. Parents are scared for their children and may feel a loss of control when such a serious diagnosis is given to their child. It is important as practitioners that
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- Summer '17
- Sickle-cell disease, National Heart, Blood Institute