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Thalassemia in children english
1. By – SURESH KUMAR ( Nursing Tutor )
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2. Thalassemia
• Thalassemia is an inherited blood
disorder that reduces the production of
functional hemoglobin (the protein in red
blood cells that carries oxygen). This
causes a shortage of red blood cells and
low levels of oxygen in the bloodstream,
leading to a lot of health problems
3. Hemoglobin
• Hemoglobin is a protein in the red blood
cells that carries oxygen to the body's organs
and tissues and transports carbon dioxide
from the organs and tissues back to the lungs.
Hb is composed of two pairs of dissimilar
chains, α and β, each defined by a specific
amino acid sequence and incorporating an
iron-containing heme group.
4. Causes of
thalassemia
• Thalassemia occurs when there is a defect in a gene that helps
control production of one of these proteins.
• Alpha thalassemia occurs when a gene or genes related to the
alpha globin protein are missing or changed.
• Beta thalassemia occurs when similar gene defects affect
production of the beta globin protein.
5. Classification of
thalassemia
• Thalassemia minor occurs if the child receives the faulty gene from
only one parent. People with this form of the disorder are carriers of
the disease. Most of the time, they do not have symptoms.
• Thalassemia Major occurs if you receive the faulty gene from both
the parents. People with this form of the disorder have symptoms.
Beta thalassemia major is also known as Cooley's anemia
8. Sign and symptoms
of thalassemia
• Thalassemia signs and symptoms may include:
-Fatigue -Weakness
-Pale or yellowish skin -Facial bone deformities
-Slow growth -Abdominal swelling
-Dark urine -Anorexia
-Enlargement of lymph nodes and spleen.
10. Treatment
of thalassemia
• Treatments for thalassemias depend on the type and
severity of the disorder. People who are carriers and no
symptoms. They’ll likely need little or no treatment.Some
of the treatments include:
• Blood transfusions- the child may need frequent blood
transfusions to maintain hemoglobin level.
11. Treatment
of thalassemia
• Bone marrow transplant- A stem cell transplant is the only
treatment that can cure thalassemia.
• Medications and supplements- Two medicines are used for
iron chelation therapy. Deferoxamine and Deferasirox. Folic acid
supplementation is also given.
• Surgical management-surgery is done to remove the speen or
gallbladder (only in severe cases).
12. Nursing
management
• The child may be admitted for blood transfusion, bone-marrow
transplant or surgery and need special nursing care.
• Provide psychological support to the parents to alleviate their
anxiety and answer their questions regarding management and
prognosis of thalassemia.
• If blood transfusion is scheduled than prepare the articles for BT
and use aseptic technique.
13. Nursing
management
• During blood transfusion continuous observation is needed to
identify any side effects in time.
• Prevent infections by limiting visitors in the childs unit during
blood transfusion.
• Play therapy and recreational facilities should be provided to the
child so that fear of hospital environment may be reduced.
14. Nursing
management
• Maintain personal hygiene of the child and involve family in the
care of child.
• If surgery is done provide routine postoperative care of the child.
• Other Nursing interventions – includes all nursing care of
hospitalized child that we have discussed in previous lecture
under heading Nursing care of hospitalized child in the lecture
“Child Health Nursing”.