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The Curriculum in Iron Metabolism & Related Disorders is supported by an educational grant from
Novartis Oncology

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  Learning Objectives  |   Posttest  |   Technical Requirements

Epidemiology and Disease Pathophysiology:
Sickle Cell Disease


“Sickle Cell Disease: A Short Guide to Management,” chapter 13 from the European School of Haematology - Club du Globule Rouge et du Fer Handbook on Disorders of Iron Homeostasis, Erythrocytes and Erythropoiesis (2006), is reprinted here with permission. We wish to acknowledge and thank the ESH; handbook editors Carole Beaumont, Photis Béris, Yves Beuzard, and Carlo Brugnara; chapter 13 author Frédéric Galactéros; and Novartis Oncology, Vifor International, and Amgen Inc, which have provided educational grants in support of this handbook.

Chapter 13 Table of Contents
Topic Page
1.
Introduction 277
2.
Pathophysiology and Modifying Factors 278
3.
General Health Management
282
4.
Anaemia and Principles of Transfusion
284
5.
Acute Painful Episodes
287
6.
Chronic Painful Complications
291
7.
Sickle Cell Vascular Diseases
293
8.
Organ Insufficiencies
296
9.
Other Common Problems
298
10.
Major Preventive and Curative Treatments
299
11.
Conclusions
301
References
301

Target Audience

This activity is designed for haematologists, medical oncologists, haematology-oncology specialists, and other healthcare professionals involved in the screening, evaluation, treatment, and management of adults and children with iron overload, resulting from the spectrum of chronic iron overload diseases.

Learning Objectives

After participating in this activity, participants should be better able to:
  • Based on knowledge of the pathophysiology of SCD and current treatment recommendations, institute appropriate treatment strategies to prevent/treat acute complications of SCD including vaso-occlusion, acute chest syndrome, priapism, and acute abdominal pain.
  • Based on an understanding of the factors that may provoke vaso-occlusion, counsel your patients with SCD about the things that they should avoid in order to prevent/reduce these occurrences.
  • Apply your knowledge of the risks of iron overload in patients with SCD to prevent, identify, and treat this event.
  • Utilize knowledge of the risks and signs/symptoms of sickle cell vascular diseases, including retinal impairment, acute auditory loss, cerebral vasculopathy, and pulmonary vascular disease, to identify and treat affected patients.

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