Understanding sickle cell disorders

Abstract

More than 12,500 people in England have sickle cell disorders. This article explains how these disorders call be inherited and discusses the associated clinical problems. The author focuses on social, psychosocial and cultural aspects, and the implications for nursing practice are examined.

Perioperative Care of the Patient with Sickle Cell Disease

“…Sickle cell disease (SCD) is an inherited disorder that results in the development of an abnormal type of hemoglobin called hemoglobin S. Approximately 2,000 infants are born with this disorder annually, which makes SCD one of the most common genetic disorders in children and adults 1,2 . The disease is recessive, which means that both parents must carry a form of the mutated gene for it to be expressed in their child, and parents who carry the gene have a one in four chance of transmitting the gene with each pregnancy 1,3 . A person has the SCD trait when he or she has inherited one sickle cell gene and one normal hemoglobin gene, HbA.…”

“…In the United States, one in 12 African Americans carries the sickle cell gene. Sickle cell disease does not only affect African Americans, however, it also affects individuals of Mediterranean, Middle Eastern, Indian, Pakistani, Caribbean, South American, and North American descent 1 . Since 1990, all newborns in the United States, regardless of ethnicity, are screened for the sickle cell gene 4 .…”

“…Acute chest syndrome is a rapid deterioration in respiratory function caused by vaso‐occlusion in the vessels of the lungs. This is the most common cause of death among young adults with SCD 1,8 . Diagnosis is made based on chest pain, fever, hypoxia, and the presence of new infiltrates on a chest x‐ray film 5…”

Priapism Among Male Adolescents with Sickle Cell Disease

“…There are many forms of SCD and in all of them, at least one of the two abnormal genes usually causes the body to manufacture hemoglobin S. The most severe form of SCD occurs when the body forms two hemoglobin S factors. Other and more prevalent forms are hemoglobin SC and hemoglobin SBeta0 (Bennett, 2005).…”

“…Hemoglobin SS disease (sickle cell anemia) is the most common and severe type of SCD, resulting from inheritance of the hemoglobin S gene from both parents (Bennett, 2005). The second most common type, less severe than hemoglobin SS disease, is hemoglobin SC disease, resulting from inheritance of the Hb C gene and Hb S gene (Bennett, 2005). The third type of SCD is Hb SBeta 0 Thalassemia, which is similar to SS disease because there is no HbA.…”