Hostname: page-component-586b7cd67f-gb8f7 Total loading time: 0 Render date: 2024-11-27T21:43:16.660Z Has data issue: false hasContentIssue false
  • English
  • Français

Prevention of Stroke in Sickle Cell Anemia

Published online by Cambridge University Press:  01 January 2021

Robert J. Adams
Get access Rights & Permissions [Opens in a new window]

Extract

Sickle cell anemia (SCD) is a disease characterized by abnormal hemoglobin (Hb) structure. There is a mutation in the beta-globin gene that changes the sixth amino acid from glutamic acid to valine causing the mutated hemoglobin (HbS) to polymerize reversibly when deoxygenated to form a gelatinous network of fibrous polymers that stiffen and distort the red blood cell (RBC) membrane. This leads to episodes of microvascular vasoocclusion and premature RBC destruction leading to hemolytic anemia. For reasons that are unclear, some children develop a large artery vasculopathy (gradual narrowing and ultimate occlusion causing deprivation of blood to the brain — a stroke in other words) involving the intracranial arteries supplying the brain.

The risk of stroke for a child with SCD is many times greater than that of a healthy child without SCD or heart disease. There is a technique that allows the identification of the children with SCD who have high risk even within this relatively high-risk group. And there is a highly effective preventive treatment.

Type
Symposium
Information
Journal of Law, Medicine & Ethics , Volume 42 , Issue 2 , Summer 2014 , pp. 135 - 138
Copyright
Copyright © American Society of Law, Medicine and Ethics 2014

Access options

Get access to the full version of this content by using one of the access options below. (Log in options will check for institutional or personal access. Content may require purchase if you do not have access.)

References

Benz, E. J., “Disorders of Hemoglobin,” in Fauci, A. S., Harrison's Principles of Internal Medicine, 17th ed. (New York: McGraw-Hill Medical, 2008).Google Scholar
Adams, R. J. et al., “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 (1998): 5–11.CrossRefGoogle Scholar
Adams, R. J. et al., “The Use of Transcranial Ultrasonography to Predict Stroke in Sickle Cell Disease,” New England Journal of Medicine 326 (1992): 605–610; Adams, R. J. Brambilla, D. J. Granger, S. Gallagher, D. Vichinsky, E. Abboud, M. R. Pegelow, C. H. Woods, G. Rohde, E. M. Nichols, F. T. Jones, A. Luden, J. P. Bowman, L. Hagner, S. Morales, K. H., E. S. Roach for the STOP Study Investigative Team, “Stroke and Conversion to High Risk in Children Screened with Transcranial Doppler Ultrasound during the STOP Study,” Blood 103 (2004): 3689–3694.CrossRefGoogle Scholar
See Adams, et al., supra note 2.Google Scholar
Goldstein, L. Adams, R. J. Furberg, C. D. Gorelick, P. B. Hademenos, G. Hill, M. Howard, G. Howard, V. J. Jacobs, B. Levine, S. R. Mosca, L. Sacco, R. L. Sherman, D. G. Wolf, P. A. del Zoppo, G. J., “AHA Scientific Statement;Primary Prevention of Ischemic Stroke, A Statement for Healthcare Professionals from the Stroke Council of the American Heart Association,” Circulation 113, no. 24 (2006): e873–e923.Google Scholar
See Adams, et al., supra note 2.Google Scholar
Eckrich, M. J. Wang, W. C. Yang, E. Arbogast, P. G. Morrow, A. Dudley, J. A. Ray, W. A. Cooper, W. O., “Adherence to Transcranial Doppler Screening Guidelines among Children with Sickle Cell Disease,” Pediatric Blood Cancer (2012).CrossRefGoogle Scholar
Swota, A. H. Bradfield, S. M., “‘STOP’-ping Harm: When Is State Intervention Justified?” Pediatric Blood Cancer 54, no. 1 (2010): 8–10.CrossRefGoogle Scholar
Id.Google Scholar
Catlin, A., “The Dilemma of Jehovah's Witness Children Who Need Blood to Survive,” HEC Forum 8, no. 4 (1996): 195–207.CrossRefGoogle Scholar
Adams, R. J., “Should TCD Be Used to Force Parents to Allow Transfusion?” Pediatric Blood Cancer 54, no. 1 (2010): 11–12.CrossRefGoogle Scholar