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Thalassemias
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Peer Group Presentation
Subject – Medical Surgical Nursing
Topic – Thalassemia
Presented by
Mr. Hari Singh Nagar
M.Sc Nursing 1st year
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Thalassemia
Introduction - It is a group of hereditary anaemia
associated with defective synthesis of Hb, the
production of one or more globin chain within the
Hb molecules is reduced leads to -
• Hypochromia (hypo – less, and chromia means –
color that means an abnormal decrease in the Hb
content of RBCs) &
• Extreme Microcytosis (small then normal
RBCs),
• Haemolysis (destruction of blood elements) .
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Thalassemia
Incidence - Thalassemia affects
approximately 4.4 of every 10,000 live
births throughout the world. it was found
that 50% of the patients had died before age
35. At that age, 50% of the patients from an
Italian long-term study were still alive.
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Normal level
RBC - Male – 4.5-6.2 million cells/mcl
Female = 4-5.5million cells/mcl
Haemoglobin - Male = 14-16.5mg/dl
Female = 12-15mg/dl
Bilirubin - Direct (conjugated) – 0-0.3mg/dl
Indirect (unconjugated) – 0.1-1gm/dl
Total – less then 1.5gm/dl
Serum iron – Male = 65-175mcg/dl
Female = 50-170mcg/dl
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Definition
It is the form of inherited blood disorder
characterized by abnormal formation of Hb. It
results in improper oxygen transport and
destruction of RBCs which leads to anaemia.
Causes
• Mutation in gene or defect in gene
• Genetic
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Types
Haemoglobin made up of two parts.
• Alpha globins
• Beta globins
Thalassemia occurs when there is a defect in genes
that helps control production of one of these
proteins. There are 2 main types of thalassemia.
1. Alpha – it occurs when a genes related to the alpha
globins are missing or changed (mutated).
2. Beta – it occurs when similar gene defect affect
production of the B-globin protein.
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Both A & B thalassemia including the following
two forms –
1. Thalassemia major – you must inherit the gene
defect from both parents to develop thalassemia
major. It is also called as Cooley's anemia.
2. Thalassemia minor – it occurs if you receive the
faulty gene from only one parents.
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Clinical manifestation
1. Thalassemia minor - Those who have
inherited the defective gene for alpha chain of
Hb usually don’t show any symptoms that’s
because the alpha gene defect doesn’t cause
any major problem that interfere with the Hb
function.
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Clinical manifestation
But people who have a beta chain of Hb have
mild anaemia. The Hb can range from
completely normal to 1-2 gm/DL less then
normal. If it is occurs you must have a
greater chances to develop a more serious
form of the disease.
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2. Thalassemia major
People who have inherited the defect for beta
chain of Hb from both the parents suffer from
thalassemia major, also called Hb H disease or
Cooley's anaemia. The symptoms are severe &
usually seen within the first two years of life.
• Severe anaemia
• A pale appearance
• Anorexia, jaundice
• Delay growth & puberty due to anaemia.
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• Weak bone – abnormal facial bone
• Infection
• iron overload
• Enlargement of spleen, liver and heart.
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Diagnostic evaluation
• History
• Physical examination
• Blood test – CBC, microscopic analysis –
abnormal RBCs.
• Hb electrophoresis – show abnormal from of
Hb.
• Mutational analysis – it can be done when
results of Hb electrophoresis are inconclusive.
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Management
It is treated with regular blood transfusion and
chelation therapy.
1. Blood transfusion – it is given to the patient with
major thalassemia. It is required in every 2-3 weeks
to supplement RBCs and maintain a Hb level of
around 9gmldL.
2. Iron chelation therapy – with repeated blood
transfusion, iron load of the body in patient
increase, the condition is called iron overload.
Which can create complication and damage other
healthy organs include heart & liver. To prevent
iron overload medicines are prescribed.
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Deferoxamine & deferasirox are common iron
chelators that free iron in the blood and cause it’s
elimination through the urine.
3. Supplements – folic acid supplements must be
given to enhance the production of healthy RBCs.
4. Surgery
• Bone marrow transplantation
• Spleenectomy – it is performed who required
repeated blood transfusion (assist in relieving the
abdominal pressure & increase the life span of
supplemental RBCs). Instruct the patient to report
sign of infection because of the risk of sepsis.
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Nursing management
1. Nursing diagnosis – ineffective tissue perfusion related
to reduce cellular components as evidence by cyanosis.
2. Nursing diagnosis – activity intolerance related to
imbalance of oxygen supply and consumption need as
evidence by fatigue.
3. Nursing diagnosis – imbalance nutrition less the body
requirements related to lack of appetite as evidence by
weight and skin integrity.
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Nursing management
1. Nursing diagnosis – ineffective tissue perfusion related
to reduce cellular components as evidence by cyanosis.
Nursing goal – To improve tissue perfusion.
Intervention – Monitor sign of hypoxia such as
cyanosis, hyperventilation, increase pulse, breath
frequency and B.P.
• Provide frequent rest periods to reduce oxygen
consumption.
• Provide oxygen therapy to patients.
• Monitor vital sign.
• Observe any restlessness and confusion.
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2. Nursing diagnosis – activity intolerance related to
imbalance of oxygen supply and consumption need as
evidence by fatigue.
Nursing goal – To improve the activity tolerance
Intervention - Assess the capability of doing activity.
• Monitor vital sign before & after the activity.
• Create a schedule to perform the activities.
• To stop activities if pulse, BP, respiration, fatigue or
dizziness will increase.
• Provide frequent rest periods to reduce oxygen
consumption.
• To administer blood products.
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3. Nursing diagnosis – imbalance nutrition less the body
requirements related to lack of appetite as evidence by
weight and skin integrity.
Nursing goal – To maintain the nutritional level
Intervention
• Allow to eat food that can be tolerated to improve the
nutritional quality at the appetite increase.
• Provide nutritious food which include high caloric, high
protein, vitamin and minerals.
• Check the weight daily.
• Provide IV fluid if not able to take by mouth.
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Recent study regarding the thalassemia
The life expectancy of patients with
thalassemia major has significantly
increased in recent years, as reported by
several groups in different countries.
However, complications are still frequent
and affect the patients' quality of life. In a
recent study from the United Kingdom, it
was found that 50% of the patients had
died before age 35.
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Recent study regarding the Thalassemia
At that age, 65% of the patients from an
Italian long-term study were still alive.
Heart disease is responsible for more than
half of the deaths. rates of survival and
complication-free survival continue to
improve, due to better treatment strategies.
New complications are appearing in long-
term survivors. Iron overload of the heart
remains the main cause of morbidity and
mortality.
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Bibliography
• “Suddarth’s & burnner” text book of medical
surgical nursing, twelth edition,Wolters publication,
Page no. 925-926
• “Saunders” comprehensive review for the NCLEX
RN examination, fifth edition, elsevier publication,
page no. 522-523
• www.authorstream.com
• www.slideshare.com
• www.scribed.com
• www.webmd.com
