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  1. 1. Nursing Care during Iron Chelating Therapy in Children with Sickle Cell Disease Nursing care of children receiving chronic transfusion therapy should include attention to the complications of iron chelating. As a result of long-term blood transfusions, children with sickle cell disease receive various chelating agents that could cause impairments in growth, development, and physical health. Without iron chelation therapy, impending death is certain since there is no physiological mechanism for excreting accumulating iron. Iron overload results in cardiac toxicity, hepatic failure and cancer. Thus, chelating agents are used to counteract iron overload resulting from blood transfusions. Some of the more common complications are liver fibrosis, renal insufficiency, growth retardation, sensorineural ototoxicity, ocular toxicity, and agranulocytosis. Consequently, a thorough nursing assessment is paramount in recognizing symptoms that suggest the occurrence of these complications. This presentation discusses nursing management of the child with sickle cell disease who is receiving chelating agents. Priorities for care and key areas of assessment are offered. Objectives: At the conclusion of this presentation participants will be able to: 1. Explain the use of blood transfusions as a treatment alternative among individuals with sickle cell disease 2. Recognize symptoms that lead to complications of excessive build-up of iron in the body. 3. Identify specific iron-chelating drugs and its corresponding drug administration, advantages, and disadvantages 4. Identify iron-chelating therapy’s mechanism of action 5. Discuss appropriate nursing care during the administration of iron chelation therapy 6. Discuss key areas of teaching for families of a child receiving chronic transfusion therapy. Content outline I. Sickle cell disease A. Pathophysiology B. Indications for chronic transfusion therapy a. Stroke prevention b. Severe or reoccurring acute chest syndrome c. Vaso-occlusive crisis d. Incapacitating pain C. Indications of Iron Overload a. Gray or bronzed colored skin b. Shortness of breath c. Arthritis d. Liver disease (cirrhosis, liver chancer)
  2. 2. e. Enlarged spleen f. Abdominal pain g. Weight loss h. Lethargy i. Cardiac toxicity (cardiac failure, cardiac arrhythmias) j. Increased serum transferrin saturation k. Increased serum ferritin levels II. Iron chelating therapy i. Mechanism of action ii. Iron chelating drugs 1. Deferasirox (Exjade, Asunra, Desirox) a. Drug Administration b. Recommended therapeutic ranges c. Advantages d. Disadvantages 2. Deferoxamine (Desferal) a. Drug Administration b. Recommended therapeutic ranges c. Advantages d. Disadvantages A. Complications a. Nausea b. Diarrhea c. Rash d. Renal insufficiency (increased creatinine or tubular dysfunction, excessive excretion of calcium) e. Liver fibrosis f. Growth retardation g. Ocular toxicity h. Agranulocytosis B. Nursing care a. Audiology evaluation (Prior to the initiation of therapy, during therapy, and after therapy has concluded) b. Ophthalmology evaluation (Prior to the initiation of therapy, during therapy, and after therapy has concluded) c. Cardiac Evaluation (Prior to the initiation of therapy, during therapy, and after therapy has concluded) d. Observe administration site for irritation and infection e. Explain common side effects f. Instruct patient and family on drug administration and equipment g. Nursing Considerations: i. Aseptic technique ii. Rotate sites iii. Encourage increasing fluid intake
  3. 3. III. Patient Education A. Home management a. Discuss avoidance of excess iron in the patient’s diet b. Discuss drug compliance with patient in family c. Allow patients and parents to practice and observe medication administration, pump training, and appropriate infusion technique B. Consistent healthcare a. Yearly Monitoring i. Liver Function Test (ALT, AST) ii. CNS Evaluation (MRI, and other neurocognitive testing for patients with strokes) iii. Liver biopsy iv. 24 hour urinary iron excretion v. EKG, Echocardiogram vi. Audiology evaluation vii. Ophthalmology evaluation viii. Endocrine evaluation