Sickle Cell Anemia: Discontinuing Blood Exchange After Transition to Adulthood Linked with Increased Mortality

MedicalResearch.com Interview with:

Samir K. Ballas MD FACP Emeritus Professor of Medicine and Pediatrics Department of Medicine/Cardeza Foundation for Hematologic Research Thomas Jefferson University Philadelphia, PA

Dr. Samir Ballas

Samir K. Ballas MD FACP
Emeritus Professor of Medicine and Pediatrics
Department of Medicine/Cardeza Foundation for Hematologic Research
Thomas Jefferson University
Philadelphia, PA

Medicalresearch.com: What is the background for this study?

Dr. Ballas: Previous studies have shown that the incidence of overt stroke is about 11% in children with sickle cell anemia by ages 2 – 20 years. Untreated stokes recur periodically with increasing severity and mortality. Transfusion therapy has been documented to decrease the frequency and morbidity of stroke in children by 90%. Accordingly, children who develop overt stroke are treated with chronic blood exchange transfusion to prevent the recurrence of additional strokes. When children reach the age of 18-20 years their medical care is transitioned to adult programs. This transition process is associated with several issues one of which is the discontinuation of chronic blood exchange transfusion in patients with history of overt stokes partly due to logistic considerations and partly due to lack of research in strokes in adult patients and the complications of chronic blood transfusion .

Medicalresearch.com: What are the main findings?

Dr. Ballas: The major finding of the study is that the discontinuation of chronic blood exchange after transition to adult programs is associated with increased mortality. All the patients who discontinued blood transfusion died within 3-5 years after transition whereas patients who continued having blood exchange transfusion survived to a mean age of 36 years at the time of writing this study.

Medicalresearch.com: What should readers take away from your report?

Dr. Ballas: Stroke, also referred to as cerebrovascular accident, is a serious complications of sickle cell disease in children, It used to be a major cause of death in children with sickle cell anemia. Research on this subject confirmed that chronic blood exchange transfusion in children with overt stroke prevents the recurrence of new strokes and decreases mortality significantly.

Medicalresearch.com: What recommendations do you have for future research as a result of this study?

Dr. Ballas: The transition of medical care of patients with sickle cell disease from pediatrics to adult programs is the weakest point in their life. At this stage, children are vulnerable and their medical care loses the empathy, interest, dedication and the quality they had as children. Efforts to improve the process of transition are greatly needed.

Medicalresearch.com: Is there anything else you would like to add?

Dr. Ballas: Overt stroke in patients with sickle cell anemia (SS) is an acute neurologic event due to either ischemic infarction or hemorrhage causing focal neurologic motor and/or sensory signs and symptoms lasting more than 24 hours in the presence of positive imaging studies. Trials in the 1990’s used transcranial Doppler (TCD) ultrasonography to prevent the occurrence of overt stroke. Specifically, patients who had abnormal TCD and received blood exchange transfusion did not develop strokes whereas those with abnormal TCD who did not receive transfusion had strokes. Accordingly TCD is done routinely on children starting at the age of 2 years and repeated annually. Patients with abnormal TCD are started on chronic blood exchange transfusion to prevent primary strokes. These children soon will become 18-20 years old. Whether these patients who never had overt stroke should continue to receive chronic blood exchange transfusion after transition to adult programs is unknown and further research needs to be done to answer this question.

Citation:

Transfusion. 2016 May;56(5):1014-21. doi: 10.1111/trf.13418. Epub 2015 Nov 23.

High mortality among children with sickle cell anemia and overt stroke who discontinue blood transfusion after transition to an adult program.

McLaughlin JF1,2, Ballas SK1.

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