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Thalassemias
1.
2. 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) .
3. 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.
7. ORMAL 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
8. ION
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
9. 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.
10. 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.
13. 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.
14. •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.
15. 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
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.
19. 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.
20. MANAGEMEN
TIt 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.
21. 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.
23. 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.
24. 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.
25. 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.
26. 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.
