New Management of Sickle Cell Disease in Children and Teens

New Management of Sickle Cell Disease in Children and Teens

(HealthDay News) — Management of children and adolescents with sickle cell disease (SCD) requires comprehensive care from a pediatric primary care provider and a multidisciplinary team, according to a clinical report published online July 22 in Pediatrics.

Amber M. Yates, M.D., from the Baylor College of Medicine and Texas Children’s Hospital in Houston, and colleagues present an overview focusing on the practical management of children and adolescents with SCD.

The authors note that SCD is a complex disorder with multisystem manifestations requiring comprehensive care from a pediatric primary care provider and a multidisciplinary SCD team. All aspects of general pediatric care should be managed by the pediatric primary care provider, and they should co-manage SCD-specific manifestations with the SCD team. The patient and family should be involved in all decisions. At least annual consultation with a multidisciplinary SCD team is strongly advised, although the extent to which the comprehensive care is delivered by a pediatric primary care provider versus the multidisciplinary specialist team will vary by community as well as family preference and the frequency and severity of SCD manifestations. Age-specific screening for disease complications is important at all SCD comprehensive visits. Education about and planning for urgent medical evaluation should be developed early and reviewed at each visit. The patient and family should be made aware that SCD is treatable but requires attention and active management to achieve optimum life outcomes.

“Improvements in care have resulted in almost all children with SCD surviving into adulthood and transitioning into adult care, which has allowed shifting the goal of care to improved quality of life,” the authors write.

Several authors disclosed ties to the pharmaceutical industry.

What is sickle cell disease?

Sickle cell disease (SCD) is a painful and potentially life-threatening inherited blood disorder that primarily affects people of African descent. This genetic condition causes red blood cells to become misshapen, resembling a crescent moon or sickle. These abnormal cells can block blood flow, leading to a cascade of health problems. While SCD can affect people of any race, its prevalence is disproportionately high among Black American children.

The genetic roots of SCD lie in Africa, where the sickle cell trait offered a survival advantage against malaria. As populations migrated, the gene spread to other regions, including the Americas. Consequently, SCD is more common in populations with African ancestry. In the United States, approximately 1 in 365 Black or African American babies is born with SCD, a rate significantly higher than other racial groups.

How does sickle cell disease affect Black children?

The impact of SCD on Black American children is profound and multifaceted. The disease often manifests early in life, with symptoms including severe pain, fatigue, swelling, and frequent infections. These painful episodes, known as sickle cell crises, can be debilitating and disrupt a child’s normal development. The chronic nature of the condition can lead to missed school days, social isolation, and emotional distress.

Beyond the physical toll, SCD also imposes a significant financial burden on affected families. Medical expenses for treatments, hospitalizations, and specialized care can be overwhelming. This economic hardship can further exacerbate the challenges faced by Black families, who already experience disproportionate rates of poverty and limited access to quality healthcare.

The psychological impact of SCD on children and their families cannot be overstated. Living with a chronic and potentially life-threatening condition can lead to anxiety, depression, and fear of the future. Children with SCD may experience discrimination and stigma, further affecting their mental health and well-being.

Can sickle cell be treated?

While significant advancements have been made in the treatment of SCD, including blood transfusions, pain management, and new medications, there is still no cure. Ongoing research is focused on developing more effective therapies and ultimately finding a cure. Additionally, addressing the social determinants of health, such as poverty, inequality, and access to care, is essential for improving the lives of children with SCD.

In conclusion, sickle cell disease is a devastating condition that disproportionately affects Black American children. The physical, emotional, and financial burdens associated with SCD have far-reaching consequences for individuals, families, and communities. Continued research, improved access to care, and comprehensive support systems are crucial for mitigating the impact of this disease and ensuring a brighter future for children with SCD.

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