Thalassemia

Question 1

Thalassemia is what type of anemia?

Answer 1

Genetic

Question 2

What does thalassemia mean?

Answer 2

A group of diseases involved decreased/abnormal production of normal hemoglobin causing decrease rbc production

Question 3

Thalassemia is due to what?
And leads to?

Answer 3

The absent of globulin protein
Which leads to abnormal hgb synthesis

Question 4

When we are missing that globulin protein and leads to abnormal hgb synthesis, what occurs?

Answer 4

Hemolysis

Question 5

Thalassemia mainly occurs in which ethnic groups?

Answer 5

Near Mediterranean Sea
Equatorial near regions of Asia, Middle East and Africa

Question 6

Thalassemia is autosomal recessive genetic based, which means what?

Answer 6

You could get one or two thalassemic genes

Question 7

One thalassemic gene means ?
It’s also more ?
Two thalassemic gene means ?
It’s also more?

Answer 7

1 = minor ; mild form of disease
2 = major ; severe form of disease

Question 8

Thalassemia minor
Clinical manifestations (7)

Answer 8

Frequently asymptomatic
Moderate anemia
- microcytosis ( small )
- hypochromia ( pale cells )
Mild splenomegaly
Bronzed skin color
Bone marrow hyperplasia

Question 9

Do we need to treat thalassemia minor? Why?

Answer 9

No because our body adapts to the reduction of hemoglobins

Question 10

Thalassemia major
Clinical manifestations (7)
And when do symptoms develop?
At what age ^

Answer 10

Life threatening
Physical & mental growth deficits
Pale
Jaundiced
Splenomegaly ( large spleen )
Hepatmegaly ( large liver )
Cardiomyopathy ( large heart )

Age 2

Question 11

For thalassemia major
Why is splenomegaly & hepatomegaly
And cardiomyopathy’s enlarged??

Answer 11

Splenomegaly & hepatomegaly enlarged due to trying to store that iron

Cardiomyopathy for the heart overworking

Question 12

What is the pathophysiology for thalassemia Major?

What happens in a normal person?

So what happens ?

And you get what?
(4)

Answer 12

Your bone marrow over grows to try to compensate for the fact that you don’t have enough hemoglobin

Your red blood cell production is stimulated when you have decrease oxygen carrying capacity of your blood; then your body tells your bone marrow to step up and make more red blood cells; hemoglobin

So when you have thalassemia majored your bone marrow is doing this stepping up to make more hemoglobin but they make immature erythroid and they die

Which leads to chronic bone marrow hyperplasia
( overgrowth of the bones )

Question 13

Thalassemia major
clinical manifestations (4)

Answer 13

Cardiac complications due to iron overload
Pulmonary disease
Hypertension
Thrombosis

Question 14

Is thalassemia an iron deficiency anemia?

Answer 14

No

Question 15

Thalassemia is a problem of?

Answer 15

Production of hemoglobin

Question 16

So the reason why thalassemia major is not an iron problem is because have a problem with making hemoglobin. How does this relate to one another?

Answer 16

Iron is a building block to make hemoglobin, so we aren’t using that iron and we get iron overload and causes our cardiac complications ; like lung disease and hypertension

Question 17

Is there any medications or diet therapy for thalassemia? For either major or minor?

Answer 17

No

Question 18

How do you help treat thalassemia major ?
What are some nursing intervention? (5)

Answer 18

Blood transfusions with clearing agents that bind to iron to reduce iron overloading

Ascorbic acid( vitamin C ) with chelation in increase urine excretion of iron

Avoid ascorbic acid without chelation
Will increase iron absorption

Goal is Hgb of 10

Splenectomy

Question 19

What is the only cure for thalassemia?

Answer 19

Hematopoietic stem cell transplantation ( HSCT )

Question 20

We also want to be careful when doing a splenectomy on our Thalassemia major patients that?

Answer 20

They will have a higher Risk of infections because the spleen home to lymph tissues
( decrease immune function )