The thalassemias are inherited disorders of hemoglobin (Hb) synthesis. Show
What is Thalassemia?The term thalassemia is applied to a variety of inherited blood disorders characterized by deficiencies in the rate of production of specific globin chains in hemoglobin.
PathophysiologyThe thalassemias are inherited disorders of Hb synthesis that result from an alteration in the rate of globin chain production.
Statistics and IncidencesBecause of immigration to the United States from all parts of the world and the intermarriages that have taken place over the years, all types of thalassemia occur in any given part of the country.
Clinical ManifestationsThe onset is usually insidious and not recognized until the latter half of infancy.
Assessment and Diagnostic FindingsLaboratory and imaging studies in thalassemia include the following:
Medical ManagementThe objective of supportive therapy is to maintain sufficient hemoglobin levels to prevent tissue hypoxia.
Pharmacologic ManagementMedications needed for the treatment of various types of thalassemias are nonspecific and only supportive.
Nursing ManagementNursing care of a child with thalassemia should also be supportive. Nursing AssessmentNursing assessment of a child with thalassemia include:
Nursing DiagnosesBased on the assessment data, the major nursing diagnoses are:
Nursing Care Planning and GoalsMajor goals for the child are:
Nursing InterventionsNursing interventions for a child with thalassemia are:
EvaluationGoals are met as evidenced by:
Documentation GuidelinesDocumentation in a child with thalassemia include:
Practice Quiz: ThalassemiaHere’s a 5-item quiz for Thalassemia study guide. Please visit our nursing test bank page for more NCLEX practice questions. 1. A child with thalassemia was given deferoxamine (Desferal); which of the following should alert the nurse to notify the physician? A. Decreased hearing. 1. Answer: A. Decreased hearing.
2. The long-term complications seen in thalassemia major are associated to which of the following? A. Anemia. 2. Answer: C. Hemochromatosis.
3. A child with β-thalassemia is undergoing a blood transfusion. To prevent organ damage from the excessive amount of iron, chelation therapy is prescribed. Which of the following medications will be added to this therapy? A. Dextromethorphan. 3. Answer: C. Deferasirox.
4. A clinic patient has a hemoglobin concentration of 10.8 g/dL and reports sticking to a strict vegetarian diet. Which of the following nutritional advice is appropriate? A. The diet is providing adequate sources of iron and requires no changes. 4. Answer: D. The patient should use iron cookware to prepare foods, such as dark green, leafy vegetables and legumes, which are high in iron.
5. Halfway through the administration of blood, the female client complains of lumbar pain. After stopping the infusion Nurse Hazel should: A. Increase the flow of normal saline. 5. Answer: A. Increase the flow of normal saline.
Which instruction would the nurse include when teaching a patient about the administration of iron supplement?Advise patient to take iron supplements an hour before meals for maximum absorption; if gastric distress occurs, suggest taking the supplement with meals — resume to between-meals schedule if symptoms subside.
Does a blood transfusion raise iron levels?On average, a person receives 2 units of blood during a red blood cell transfusion, and each unit of blood has 200 to 250 milligrams of iron. So each 2 unit blood transfusion adds an extra 400 to 500 milligrams of iron to your body.
When should I take iron after blood transfusion?Traditionally after 15 to 20 units of blood transfusions, an iron-chelating agent is initiated.
When would you need a blood transfusion for anemia?A normal hemoglobin level is about 12 to 18 g/dL.
A red blood cell transfusion may be suggested if it drops below 8 g/dL. Whether you need a transfusion for anemia depends on many factors, such as how long it took for the anemia to develop and how well your body is able to cope with it.
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