ADVANCED

MEDICAL – SURGICAL
NURSING
MIN223_A
Care of clients with alterations in Oxygen
carrying Capacity
(HEMATOLOGIC DISORDER)
THALASSEMIA AJO R
E MI A M
HAL A SS
BETA T

COOLEY’s ANEMIA
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DESIRED LEARNING OUTCOMES:
AFTER THE LECTURE ABOUT THALASSEMIA, NURSES WILL BE ABLE TO:

1. Discuss what thalassemia is.

2. Identiy different types of
thalassemia.
3. Identify the cause of
thalassemia.
4. Identify and apply the latest trend
in caring of patient with
thalassemia.
5. Identify and discuss different
home management or care of
patient with thalassemia.

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PATIENT’S
PROFILE
CASE STUDY
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PATIENT
M.A. an 11 year old male from
Bulacan.
He is the youngest among
3 siblings and currently
continuing his 5th grade under
“new normal” mode of education.
According to M.A’s mother, his
illness does not hinder him from
performing well at school.

M.A. is the only child that was

diagnosed with BETA
THALASSEMIA MAJOR.
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History
of the
patient
• In his 14th month, M.A started to receive blood transfusion.
• M.A was diagnosed
Thereafter, M.A receives
with BETA blood transfusion
THALASSEMIA MAJORevery 2 weeks
before his first
birthday.
because his hemoglobin level remains below normal yet no
confirmatory test was
• In his first 6 months, M.Adone,
started until hesome
to show reaches 2 years
symptoms old. His
of the beta
pediatrician
thalassemiarequested for hemoglobin
major like lethargic, electrophoresis
feeding problems, and
jaundice and irritability.
was diagnosed with beta thalassemia major. He was also
• Series of pedia-consultations were done and laboratory requests were
advised to have CBC.
asked especially iron chelation therapywith
Despite the vitamins because of his
iron that M.A’s regular
pediatrician
blood
has transfusion therapy.level was still below normal (11.3–14.1g/dL).
prescribed, hemoglobin
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Recent Hospitalization
October 14, 2020 : Date of admission at Bulacan
Medical Center
1 week PTA: M.A experienced
loss of appetite,
body weakness,
difficulty of breathing
and paleness of the skin

Remarkable decrease in the level of hemoglobin

(8.2 g/dL) was seen in his latest CBC result.

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Family History
• M.A’s parents were undiagnosed of beta thalassemia and no signs
and symptoms of the illness are noted to both parents.
• Both M.A’s siblings does not show any signs and symptoms of beta
thalassemia.

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INTRODUCTION
WHAT is THALASSEMIA
• is a group of inherited blood disorders
Etiology involving inadequate production of
normal hemoglobin, and therefore
decreased erythrocyte production.
• Thalassemia is due to an absent or
reduced globulin protein.
α-thalassemia β-thalassemia
α-Globin chains β-globin chains

• insufficient production of normal

hemoglobin is the predominant problem.

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Who is at risk for thalassemia?

You are at risk for thalassemia if you have a family history of it. Thalassemia
can affect both men and women. Certain ethnic groups are at greater risk:
α-thalassemia β-thalassemia
Southeast Asian, Indian, Mediterranean (Greek,
Chinese, or Filipino Italian and Middle
Eastern), Asian, or African

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GENETIC LINK β-thalassemia

An individual
α-thalassemia with thalassemia may• have Involvesa 2 genes (you
• Involves 4 genes (you get 2 of these genes from your
heterozygous or homozygous
mother and 2 of these genes from your father).
form ofgetthe
1 gene from your
mother and 1 gene
disease.
• If you only get 1 mutated gene, you will have no
from your father).
signs or symptoms of Athalassemia.
person who is heterozygous
However, you has one
• If you get only 1
can still pass on the gene mutation to your mutated gene, you’ll
thalassemic gene and
children (this is called being a “carrier”). one normal gene and is signs or
have mild
said to have thalassemia
• If you get 2 mutated genes, you will have mild minor (or thalassemic
symptoms of
thalassemia.
trait), which is a mild form of the disease. A
symptoms.
• If you get 2 mutated
• If you get 3,homozygous
you’ll have moderateperson has two thalassemicgenes,
to severe genes,you’ll have
symptoms.
causing a severe condition knownsymptoms as
moderate to severe
that usually
thalassemia
• A baby who inherits all 4 mutated genes will be
very sick, and probably won’t live long after birth.
major develop in the first 2
years of life.

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PATHOPHYSIOLOGY OF THALASSEMIA

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What tests are used to determine if a
patient has thalassemia?
Hemoglobin
Complete Blood Electrophoresis
Count (CBC)
measures the different types of hemoglobin in the bloodstream
• measures the size, number, and maturity of the different
• The blood cells
most especially
common types ofred blood
normal cells, white
hemoglobin are: blood cells,
and platelets
Hemoglobin in anormal
F, the specific
typevolume
found inof blood.
fetuses andThis is one of
newborns.
the most
It’s replaced by common
hemoglobin tests
A soonperformed
after birth.
Hemoglobin A, the normal type most commonly found
• inhelps
healthy kids
the and adults.team
healthcare Somecheck
diseases,
anysuch as severesuch
symptoms, formsas
of thalassemia, may cause hemoglobin A levels to be low and
weakness, fatigue, or bruising, you may have. A CBC
hemoglobin F levels to be high.
also helps him or her diagnose conditions, such as
anemia,
• The infection,
most common typesand many other
of abnormal disorders
hemoglobin are: Hemoglobin
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Clinical Manifestations of BETA THALASSEMIA

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Large image slide

O F R
T
N MA J O
M E
G E M I A
N A S E
A
M ALA S
T H
TA
BE

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MANAGEMENT OF THALASSEMIA
• In general, children with eta thalassemia major
will begin transfusions somewhere between six
months and two years of age.

• The best time to begin transfusion based on a

number of factors, usually including the child’s
hemoglobin level and level of energy.

• Once transfusions begin in a child with

thalassemia major, it is generally desirable to
maintain pre-transfusion hemoglobin levels
of between 8.5 and 10g/dL.

• It may be higher than this for some time but

shouldn't usually exceed 14g/dL.

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MANAGEMENT OF THALASSEMIA

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MANAGEMENT OF THALASSEMIA

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MANAGEMENT OF THALASSEMIA

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Large image slide

LATEST TREND
ABOUT
BETA THALASSEMIA
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Video slide

T h er a py i n
Gene
l as sa e m i a
β-tha Jens
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Gene
Video Therapy
slide in Thalassemia

T h er a py i n
Gene
l as sa e m i a
β-tha Jens
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Jens
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Large image slide

HOME CARE CHECKLIST

BETA THALASSEMIA
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 Take daily maintenance medication and
supplement : Chelation medicine, supplements to boost
immune system multivitamin and mineral supplement
(that contains no iron) 
R E
 Prevent infection
CA
 Wash your hands frequently
E i s t
 Cover your mouth when sneezing or coughing
OM kl
 Tend to cuts, scratches and other injuries by washing, H he c
disinfecting and bandaging C
 Try to stay away from sick people.
 Wipe down surfaces with antibacterial wipes in any shared work
spaces.
 Don’t share personal items (toothbrushes, towels, nail clippers, etc.)
 If traveling to other countries, drink bottled water.
 Immunizations can help protect against many infection
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 Good nutrition
 Include healthy foods in their diet, such as dairy products, fruits and
vegetables.
 Spinach is an excellent source of many nutrients (fiber, vitamin A, folate,
calcium) and is also high in anti-oxidants.
 Avoid eating food high in vitamin C together with non-heme iron food

lis E
ck AR
 Avoid “empty calories” like sodas and highly refined carbohydrates

he C

t
like cookies and alcohol

E
M
HO
C
 Exercise and sports
 Getting an appropriate amount of exercise. Remember: physical activity
can lengthen your life and add to the quality of life as well.
 Balance study and playing time.

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 Managing Stress
 Sharing your feelings with others is very important
 Engage in activities that you enjoy. Reach out to others with a similar
experience. When you are in a more positive frame of mind, you
areal so more open to seeing possible solutions to problems.
 Have a healthy spiritual life.
 Family support especially in psychosocial development.

R E
CA st
E l i
M ck
HO he
C
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 ES
KY TUN
FUN
Launch

Thank BOFF
IN

You