What You Need to Know About Acute Chest Syndrome.ppt

What You Need to Know About Acute Chest Syndrome By Susan Hernandez, RN, CNN, BSN, and G. Elaine Patterson, RN-C, EdD, MA, Med, FPN-C Nursing2009, June 2009 Online: ? 2009 by Lippincott Williams Wilkins. All world rights reserved. Acute chest syndrome (ACS) Potentially life-threatening complication of sickle-cell disease Can lead to respiratory failure ACS is the leading cause of death among patients with sickle-cell disease Acute chest syndrome (ACS) Sickle-cell disease affects 80,000 Americans Inherited disorder Seen in equatorial African descent, Mediterranean, East Indian, Middle Eastern lineage Background of sickle-cell disease Hemoglobin is oxygen-carrying protein in RBCs Normal adult hemoglobin is designated hemoglobin A. A patient with sickle-cell disease has abnormal hemoglobin (designated hemoglobin S), alone or in combination with other abnormal hemoglobin (typically hemoglobin C) Background of sickle-cell disease Homozygous form of sickle-cell disease (hemoglobin SS, or sickle-cell anemia) is most severe, accounts for about 65% of cases Other types are sickle-cell thalassemia and sickle-cell disease with hemoglobin SC Background of sickle-cell disease Signs and symptoms of sickle-cell disease are caused by elongated and rigid hemoglobin S Abnormal RBCs cause vascular occlusions, creating a cycle of more deoxygenation, sickling, and sluggish blood flow. This ultimately leads to ischemia and infarction in distal organs Background of sickle-cell disease Abnormal hemoglobin also means RBC has shorter life (16 days vs. 120 for normal); leads to chronic intravascular and extravascular hemolysis 30 years ago, life expectancy was 14 years; now patients are living into 40s and 50s. Acute complications experienced by adult patients include vaso-occlusive crisis, ACS, renal failure Vaso-occlusive crisis Low oxygen tension causes red blood cells (RBCs) to lose their round shape RBCs adhere to each other and the endothelium Causes pain, edem