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Continuous Manual Exchange Transfusion for Patients with Sickle Cell Disease: An Efficient Method to Avoid Iron Overload
Published on: March 14, 2017
Transition and sickle cell disease
Michael R DeBaun1, Joseph Telfair
1Department of Pediatrics, Vanderbilt University School of Medicine and Monroe Carell Jr. Children’s Hospital at Vanderbilt, Nashville, Tennessee 37232-9000, USA. m.debaun@vanderbilt.edu
Insights
Transitioning adolescents with sickle cell disease (SCD) to adult care requires specialized guidelines. Addressing unique challenges like cognitive impairment and comorbid conditions is crucial for successful long-term health management.
Area of Science:
- Genetics and Hematology
- Pediatric and Adult Medicine
- Public Health
Background:
- Sickle cell disease (SCD) is a prevalent genetic disorder, affecting approximately 1 in 2400 newborns in the US, with higher incidence in individuals of African descent.
- Despite a low pediatric mortality rate, evidence-based guidelines for transitioning SCD patients from pediatric to adult care are lacking.
- Adolescents with SCD face unique challenges, including higher rates of public insurance, cognitive impairment from strokes, lower high school graduation rates, and comorbidities like asthma.
Purpose of the Study:
- To highlight the critical need for improved transition interventions for adolescents and young adults with SCD.
- To identify key challenges unique to SCD patients during the transition to adult healthcare.
- To emphasize the collaborative role of primary care physicians and pediatric specialists in enhancing transition care.
Main Methods:
- Review of existing literature and clinical practices regarding SCD transition care.
- Analysis of unique health, social, and educational factors impacting adolescents with SCD.
- Identification of potential strategies for primary care physicians and specialists to improve transition outcomes.
Main Results:
- Young adults with SCD are living longer, making the transition to adult primary care a critical health management step.
- SCD patients experience specific obstacles during transition, including cognitive deficits, educational risks, and higher comorbidity burdens.
- Current transition programs often lack specific evidence-based guidelines tailored to the needs of SCD patients.
Conclusions:
- Enhanced transition interventions are essential for improving long-term health outcomes in young adults with SCD.
- Collaboration between pediatric specialists and primary care physicians is key to developing effective, individualized transition plans.
- Addressing the multifaceted challenges faced by SCD patients is vital for successful healthcare management beyond pediatric care.
Abstract:
Sickle cell disease (SCD), the most common genetic disease screened for in the newborn period, occurs in ~1 in 2400 newborns in the general population and 1 in 400 individuals of African descent in the United States. Despite the relative high prevalence and low pediatric mortality rate of SCD when compared with other genetic diseases or chronic diseases in pediatrics, few evidence-based guidelines have been developed to facilitate the transition from pediatrics to an internal medicine or family practice environment. As with any pediatric transition program, common educational, social, and health systems themes exist to prepare for the next phase of health care; however, unique features characterizing the experience of adolescents with SCD must also be addressed. These challenges include, but are not limited to, a higher proportion of SCD adolescents receiving public health insurance when compared with any other pediatric genetic or chronic diseases; the high proportion of overt strokes or silent cerebral infarcts (~30%) affecting cognition; risk of low high school graduation; and a high rate of comorbid disease, including asthma. Young adults with SCD are living longer; consequently, the importance of transitioning from a pediatric primary care provider to adult primary care physician has become a critical step in the health care management plan. We identify how the primary care physicians in tandem with the pediatric specialist can enhance transition interventions for children and adolescents with SCD.
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