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?
Management of Sickle Cell Anemia Across Childhood: A Developmental Approach
Sickle cell anemia (SCA) is a genetic blood disorder characterized by the presence of abnormal hemoglobin S, leading to sickling of red blood cells, vaso-occlusion, and chronic hemolytic anemia. Early diagnosis through newborn screening allows for prompt initiation of care, which is crucial in reducing morbidity and mortality. The management of a child with SCA must be adapted to their developmental stage, with specific interventions at 2 months, 2 years, 6 years, and 13 years of age.
At 2 Months Old
At 2 months, the primary focus is on prevention and education. Prophylactic penicillin (125 mg orally twice daily) should be initiated to prevent pneumococcal infections (Yawn et al., 2014). Immunizations must be up to date, including the pneumococcal conjugate vaccine (PCV13) and Haemophilus influenzae type b (Hib) vaccine. The family should receive genetic counseling, psychosocial support, and education about recognizing signs of fever and acute splenic sequestration. A referral to a pediatric hematologist is essential for comprehensive care and ongoing monitoring.
At 2 Years Old
At this stage, the child is increasingly mobile and exposed to more infections. The focus remains on infection prevention, pain management, and monitoring for complications like dactylitis and delayed growth. Penicillin prophylaxis may continue until at least age five. Transcranial Doppler (TCD) screening should be started around this age to assess stroke risk, as early intervention with chronic transfusion therapy may be warranted for those with abnormal results (Adams et al., 1998). Continued follow-up with the hematologist and a primary care provider is crucial.
At 6 Years Old
School-aged children may begin to experience more vaso-occlusive pain episodes and psychosocial challenges. The care plan should include regular assessment of pain patterns, cognitive function, and school performance. At this stage, hydroxyurea therapy is often introduced to reduce the frequency of pain crises and hospitalizations (Ware et al., 2017). TCD screening should be continued annually if indicated. The child should have a 504 plan or Individualized Education Program (IEP) to support academic success. Coordination with school nurses and counselors helps ensure appropriate accommodations.
At 13 Years Old
Adolescents face more complex issues, including adherence to treatment, reproductive health, and transition to adult care. Puberty may be delayed, and peer relationships can be impacted by frequent absences or hospitalizations. Mental health support becomes increasingly important. Conversations around fertility, contraception, and genetic counseling are essential, especially for females considering hydroxyurea, which has teratogenic risks. Transition planning should begin, involving a multidisciplinary team including a pediatric hematologist, social worker, and adolescent medicine specialist.
Referral and Care Coordination
At every stage, referral to a sickle cell disease (SCD) comprehensive care center ensures access to multidisciplinary care. Coordination involves the pediatrician, hematologist, school personnel, mental health professionals, and social services. Use of care plans, regular communication among providers, and electronic health records facilitate information sharing. Case management or nurse care coordination can help families navigate complex healthcare systems and improve adherence to treatments and appointments.
Conclusion
The management of sickle cell anemia requires a proactive, developmentally tailored approach. By anticipating complications and addressing the physical, psychosocial, and educational needs at each stage, healthcare providers can optimize outcomes. Early and ongoing multidisciplinary collaboration is essential in supporting both the child and family throughout the disease trajectory.
References
Adams, R. J., McKie, V. C., Hsu, L., Files, B., Vichinsky, E., Pegelow, C., & Abboud, M. (1998). Prevention of a first stroke by transfusions in children with sickle cell anemia and abnormal results on transcranial Doppler ultrasonography. New England Journal of Medicine, 339(1), 5-11. https://doi.org/10.1056/NEJM199807023390102
Ware, R. E., Davis, B. R., Schultz, W. H., Brown, R. C., Aygun, B., Sarnaik, S., … & Rogers, Z. R. (2017). Hydroxyurea for the prevention of sickle cell complications. New England Journal of Medicine, 376(6), 429-439. https://doi.org/10.1056/NEJMoa1611618
Yawn, B. P., Buchanan, G. R., Afenyi-Annan, A. N., Ballas, S. K., Hassell, K. L., James, A. H., … & Tanabe, P. (2014). Management of sickle cell disease: summary of the 2014 evidence-based report by expert panel members. JAMA, 312(10), 1033-1048. https://doi.org/10.1001/jama.2014.10517
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