Nursing Care During Iron Chelating Therapy in Children with Sickle Cell Disease REVISED
1. Nursing Care During Iron Chelating
Therapy in Children with Sickle Cell
Disease
Ashley Wagner, BSN, RN
University of Alabama Hospital
Birmingham Black Nurses Association
2. Objectives
At the conclusion of this presentation
participants will be able to:
• Discuss the use of blood transfusions as a treatment.
• Recognize complications of excessive build-up of iron in
the body.
• Identify iron-chelating therapy’s mechanism of action and
appropriate nursing care.
• Discuss key areas of teaching for families of a child
receiving chronic transfusion therapy.
3. Sickle Cell Disease
• An autosomal recessive genetic disorder
• Affects hemoglobin
• Results in distortion, increased rigidity, and
destruction of RBCs
• Tissue hypoxia results in acute and chronic
tissue damage
4. Pathophysiology
Mutation of the β-
globin chain in the
hemoglobin
Glutamic acid is
replaced with with
valine at the 6th
position
• Absence of a polar amino
acid promotes
aggregation
Distortion of red
blood cells into a
sickle shape with
decreased elasticity
8. Iron Chelating Therapy:
Mechanism of Action
• 2 molecules of the iron chelator bind to 1
molecule of iron
• Highly protein bound and the chelator is
metabolized by the liver
• Excess iron is excreted in the feces or urine
9. Iron Chelating Drugs
• deferasirox (Exjade, Asunra, Desirox)
• Drug Administration
• Recommended therapeutic ranges
• Advantages
• Disadvantages
10. Exjade: Drug Administration
• Oral Drug
• DO NOT break, crush, or allow patient to
swallow whole
– Dosages < 1 gram should be dissolved in 100 mL
of liquid
– Dosages > 1 gram should be dissolved in 200 mL
of liquid
• Drop tablet into water or juice
• Stir until completely dissolved
17. Desferal: Recommended Ranges
• IV Administration:
– 40-50 mg/kg/day over 8-12 hours over 5-7 days
per week
• Subcutaneous Administration:
– 1-2 gram over 8-24 hours
• Intramuscular Administration:
– 0.5- 1 gram every day
Do not exceed 1 gram daily
19. Desferal: Disadvantages
• A minimum of 5 days per week is needed to
attain adequate excretion
• Adversely affects skeletal maturation and
growth development
• Orange to red urine
• Patient Compliance
– Desferal infusion must be administer over 8- 24
hours
20. Desferal: Adverse Effects
• Hypotension with to rapid IV infusion
• Tachycardia
• Shock
• Acute Renal Failure
22. Continuing Care
• Audiology evaluation
• Ophthalmology evaluation
• Cardiac Evaluation
• Observe administration site for irritation and
infection
• Explain common side effects
• Instruct patient and family on drug administration
and equipment
23. Nursing Consideration
• Aseptic Technique
• Rotate Sites
• Encourage patient to increase fluid intake
• Provide support to encourage compliance with
treatment and reporting adverse occurrences
• Observe administration sites for irritation or
infection
25. Home Management
• Discuss avoidance of excess iron in the patient’s
diet
• Discuss drug compliance with patient in family
• Allow patients and parents to practice and observe
medication administration, pump training, and
appropriate infusion technique
27. Bibliography
Latsko, J., & Martens, A. (2009). Promoting patient
adherence to iron chelation therapy: findings from
EPASS (Exjade Patient Assistance and Support
Services). Oncology Nursing Forum, 36(3), 26-27.
NIH Publication No. 02-2117. Revised May28, 2002
(Fourth Edition) National Institutes of Health, National
Heart, Lung, and Blood Institute.
The toxicity of iron is believed to be due to free radical damage to tissues induced by iron that is circulating unbound to plasma proteins such as transferrin. The human body does not excrete a significant amount of iron
Prior to the availability of DF therapy, chronically transfused thalassemia patients usually died from cardiac failure by their twenties. These individuals also developed hepatic cirrhosis, insulin dependent diabetes mellitus due to pancreatic insufficiency, growth and pubertal delays due to hypopituitarism, and possibly other hormone deficiencies. Since the advent of subcutaneous DF therapy using a portable pump, the longevity of chronically transfused individuals has improved considerably. Among patients with beta-thalassemia major who had at least 67% of ferritin levels £ 2500 ng/ml , there was a 91% chance of surviving for 15 years after initiation of DF therapy. Freedom from cardiac complications correlates well with the degree of patient compliance to DF therapy. However, growth and pubertal delays may not be completely preventable even in well-chelated patients who have been transfused since infancy.
