THALASSEMIA IN CHILDREN - ENGLISH
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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
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.
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.
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
SIGN
AND SYMPTOMS-
•
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.
DIAGNOSTIC
INVESTIGATIONS-
•
Diagnostic
investigations includes-
•
Blood
examination
•
Hb
electrophoresis
•
Bone
marrow examination
•
X-ray
examination
TREATMENT-
•
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.
•
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).
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.
•
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.
•
Maintain
personal hygiene of the child and involve family in the care of child.
•
If
surgery is done provide routine postoperative care to 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”.
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