Group Members: Amala Nisanthi Kavitha Afiqah Shuhaila

GROUP MEMBERS
 Amala
 Nisanthi
 Kavitha
 Afiqah
 Shuhaila
Hemolytic
anemia
GROUP 4
DEFINITION
 Is a disorder in which the red blood cells are destroyed faster
than the bone marrow can produce them
 The term for destruction of red blood cells is hemolysis
 Iron and other by –products remain in plasma.Lysis within
circulatory system or due to phargocytosis by cells of
retiloendothetial system
 Hematopoietic activities of bone marrow increase
ETIOLOGY
 There are 2 types of hemolytic anemia including the
following:
 *Intrinsic-the destruction of the RBC due to a defect
within the RBC themselves
 Intrinsic hemolytic anemia's are often inherited, such

as sickle cell anemia and thalassemia
 These conditions produce RBC that do not live as long

as normal RBC
Con't
 Extrintic- RBC are produced healthy but are later
destroyed by becoming trapped in the
spleen,destroyed by infection
CLINICAL MANIFESTATION
 Abnormal paleness or lack of color of the spleen
 Jaundice
 Dark color of urine
 Fever
 Weakness
 Dizziness
 Confusion
 Enlargement of the spleen and liver
 tachycardia
Diagnosis test
 Blood test
 Medical history
 Physical examination
TREATMENT
o Change in diet
o Medication
o Splenectomy-surgery to remove the spleen

Sickle Cell
Anemia
Definition
 Sickle cell anemia is also known as inherited
hemolytic anemia or sickle cell disease
 This genetic disorder has abnormal hemoglobin S

rather than hemoglobin A in the RBCs
Etiology
 Hemoglobin is a protein inside red blood cell that
carries oxygen
 Abnormal hemoglobin call hemoglobin S
 Distorts the shape of red blood cells especially
when exposed to low oxygen levels
 Sickle cell deliver less oxygen to the body tissue
 Clog more easily in small blood vessel, break into
pieces that disrupt healthy blood flow
 Inherited from both parents
CLINICAL MANIFESTATION
 Bone pain
 Breathlessness
 Fatigue
 Rapid heart rate
 Ulcer on the lower leg(adult)
 Yellowing of the eyes and skin(jaundice)
 Excessive thirst
 Poor eye sight/blindness
 Stroke
COMPLICATION
 Infection and gangrene

 Hypovolemic shock and death due to massive
entrapment of cells
 Necrosis
 Cerebral vessel occlusion due to organ infraction
DIAGNOSIS TEST
 CBC –Complete Blood Count
 Sickle Cell Test

TREATMENT
 Folic acid (essential for producing RBC),RBC are
turned over so quickly
 Narcotic – To treat the pain(but some people will
large doses of narcotics)
 Kidney transplant for kidney disease(Dialysis)
 Antibiotic and vaccine-prevent bacterial
infection(children with sickle cell)
 Large amount of oral@I.V. fluids to correct
hypovolemia and prevent dehydration vessel
occlusion
NURSING DIAGNOSIS
 Acute pain related occlusion of small vessel by

sickle cells
Nursing intervention
• Assess pain types, location and intensity

 R:Other vessel to the brain ,heart , lungs, spleen and
penis become occluded , causing pain
• Support joints and lower extremities with pillows
 R:Relieve the joints pain
• Administer analgesic as ordered by doctor
 R: Relief from pain
• Document patient progress in nursing report
 R: Document pain levels
NURSING DIAGNOSIS
 Ineffectiveness Tissue Perfusion related to a
decreased number of RBCs
NURSING INTERVENTION
 Monitor vital sign and mental alertness
 R:As a baseline and monitor abnormality
• Encourage to drink 8 to 10 glasses of water daily
 R: Dehydration causes RBCs to sickle cells
• Monitor for symptom of obstructed vessels such as leg
ulcerations, and dyspnea
 R: RBCs are sickling, vessels supplying blood to other organ
can become obstucted
• Document patient progress in nursing report
 R: Document the abnormal of condition such as breathing
pattern
THALASSEMIA
THALASSEMIA
Thalassemia is an inherited
autosomal recessive blood disease,
genetic defect
PATHOPHYSIOLOGY
 the molecular abnormalities that led to the

thalassemic syndromes, it still is not known how
accumulation of excess unmatched alpha-globin
in beta thalassemia.
 Oxidant injury may cause hemolysis, but there is
no evidence that it causes ineffective
erythropoiesis
CONTS…
 Hemoglobin E/beta thalassemia is now a
worldwide clinical problem. The reasons
underlying the heterogeneity and occasional
severity of the syndrome remain obscure.
 Ineffective erythropoiesis now appears to be
caused by accelerated apoptosis, in turn caused
primarily by deposition of alpha-globin chains in
erythroid precursors.
ETIOLOGY…
 Heredity disorders of
hemoglobin-chain synthesis.
2 TYPES…
1.ALPHA THALASSEMIA
2. BETA THALASSEMIA
1. ALPHA THALASSEMIA
 occurs when a gene or genes related to the alpha globin
protein are missing or changed(mutated).
 occur most commonly in persons from southeast Asia, the
Middle East, China, and in those of African descent.
2. BETA THALASSEMIA
 occurs when similar gene defects affect production of the
beta globin protein.
 occur in persons of Mediterranean origin, and to a
lesser extent, Chinese, other Asians, and African
Americans.
 There are many forms of thalassemia. Each type
has many different subtypes. Both alpha and
beta thalassemia include the following two
forms:
1. THALASSEMIA MINOR
2. THALASSEMIA MAJOR
1. THALASSEMIA MINOR
• Less severe
• Receive the defective gene from only one parent.
• Blood smear are small(microcytic),
pale(hypochromic), variously shape(poikilocytosis)
RBC.
• Carry less oxygen.
• Persons with this form of the disorder are carriers of
the disease and usually do not have symptoms.
1. THALASSEMIA MAJOR
• Severe
• Inherit the defective gene from both parents
• Oxygen depletion in the body becomes apparent
within the first 6 months of life.
• Left untreated, death usually results when a few
years.
CLINICAL
MANIFESTATIONS…
 Still birth(death of the unborn baby during birth or the
late stages of pregnancy).
 Children born with thalassemia major (Cooley's
anemia) are normal at birth, but develop severe
anemia during the first year of life.
 Bone deformities in the face
 Fatigue
 Growth failure
 Shortness of breath
 Yellow skin (jaundice)
INVESTIGATIONS…
 CBC
 Iron level and total iron-binding capacity
 Serum ferritin
 Sickle cell test
 Bone marrow examination
 Quantitive essay of g6pd
∆Risk for injury: falls related to
weakness and dizziness
 Assess the patient at risk for falls using a fall risk
assessment tool
 Assist the patient to change positions
 Assist the patient with ambulation
 Always raise the cord side bed to prevent
patient falling down.

∆Impaired oral mucous membrane
related to nutritional deficiencies
 Assess patients general condition
 Refer dietitian
 Serve nutritious food according during meal
times
 Assess oral condition
 Perform oral care for patients
G6PD deficiency (glucose-
6-phosphate
dehydrogenase
deficiency)
DEFINITION
 Glucose-6-phosphate dehydrogenase
deficiency is an X-linked recessive hereditary
disease characterised by abnormally low levels of
glucose-6-phosphate dehydrogenase.
 Glucose-6-phosphate dehydrogenase deficiency
is a genetic disorder that occurs most often in
males.
PATHOPHYSIOLOGY
 G6PD deficiency reduces energy available to

maintain the integrity of the red cell membrane,
which shortens RBC survival.
 Hemolysis selectively affects older RBCs among
affected blacks and among most affected whites.
CONTS…
 Hemolysis occurs commonly after fever, acute
viral and bacterial infections, and diabetic
acidosis. Less commonly, hemolysis occurs after
exposure to drugs or to other substances that
produce peroxide and cause oxidation of Hb and
RBC membranes.
ETIOLOGY…
 Inherited disorders, sex-

linked, RBC metabolic
disorder
G6PD Deficiency inheritance
chart
 Father is UNAFFECTED, mother is UNAFFECTED.
ALL of their children will be UNAFFECTED.
 Father is G6PD DEFICIENT, mother is UNAFFECTED.
All sons will be UNAFFECTED.
All daughters will be PARTIALLY DEFICIENT.
 Father is G6PD DEFICIENT, mother is PARTIALLY
DEFICIENT.
Out of two sons, one will be G6PD DEFICIENT and the other
will be UNAFFECTED.
Out of two daughters, one will be PARTIALLY DEFICIENT
and the other will be G6PD DEFICIENT.
 Father and mother are G6PD DEFICIENT.
All children will be G6PD DEFICIENT.
 Father is UNAFFECTED, mother is PARTIALLY
DEFICIENT.
Out of two sons, one will be G6PD DEFICIENT and the other
will be UNAFFECTED.
Out of two daughters, one will be PARTIALLY DEFICIENT,
the other UNAFFECTED.
 Father is UNAFFECTED, mother is G6PD DEFICIENT.
All sons will be G6PD DEFICIENT.
All daughters will be PARTIALLY DEFICIENT.
CLINICAL
MANIFESTATIONS…
 Paleness (in darker-skinned children paleness is
sometimes best seen in the mouth, especially on
the lips or tongue)
 Extreme tiredness
 Rapid heartbeat
 Rapid breathing or shortness breath
 Jaundice or yellowing of the skin and eyes
particularly in newborns and enlarged spleen
 Dark, tea-colored urine
INVESTIGATIONS…
 CBC
 Iron level and total iron-binding capacity
 Quantitive essay of G6PD
∆Activity intolerance related to poor oxygen supply.
 Rest in bed
 Assess the patients general condition
 Adminiser oxygen as prescribed
 Refer abnormal investigation results
 Give medication as ordered by doctor
∆Imbalanced nutrition :less than body requirements
related to disease,treatment,or lack of knowledge
of adequate nutrition
 Consult a dietition
 Administer supplements as ordered
 Administer liquid supplements with a drinking straw to
avoid staining the teeth
 Administer intramuscular iron injection by the Z-track
method to avoid staining the injection site
THANK YOU

Group Members: Amala Nisanthi Kavitha Afiqah Shuhaila

Uploaded by

Copyright:

Available Formats

Group Members: Amala Nisanthi Kavitha Afiqah Shuhaila

Uploaded by

Document Information

Original Title

Copyright

Available Formats

Share this document

Share or Embed Document

Sharing Options

Did you find this document useful?

Is this content inappropriate?

Copyright:

Available Formats

Group Members: Amala Nisanthi Kavitha Afiqah Shuhaila

Uploaded by

Copyright:

Available Formats

GROUP MEMBERS

 Intrinsic hemolytic anemia's are often inherited, such

 These conditions produce RBC that do not live as long

o Splenectomy-surgery to remove the spleen

 This genetic disorder has abnormal hemoglobin S

 Infection and gangrene

 CBC –Complete Blood Count

 Sickle Cell Test

 Acute pain related occlusion of small vessel by

• Assess pain types, location and intensity

 the molecular abnormalities that led to the

patient falling down.

 G6PD deficiency reduces energy available to

 Inherited disorders, sex-

You might also like