PEDIA FINALS NOTES #2
Care of Client Hematologic Disorder
Thalassemia
- refers to an anemia involving problems with
hemoglobin or an anemia associated with
abnormalities of the beta chain of adult
hemoglobin(HbgA).
2 Major types of Thalassemia:
1. Thalassemia major
2. Thalassemia minor
 Thalassemia major ( homozygous Beta-Thalassemia)
is an anemia in which the child is unable to produce
normal beta hemoglobin.
- This disorder is also called Cooley’s anemia or
Mediterranean anemia because thalassemia is
a beta chain hemoglobin defect, & is most
prevalent in the Mediterranean population but
also occurs in children of African & Asian
heritage.
Effect of Thalassemia Major:
 Bone marrow-overstimulation leads to increased
facial-mandibular growth
 Skin- bronze-colored from hemosiderosis &
jaundice
 Spleen- Splenomegaly
 liver & gallbladder- cirrhosis & cholelithiasis
 Pancreas- destruction of islet cells & diabetes
mellitus
 Heart- failure from circulatory overload
Thalassemia
- Symptoms do not become apparent until the
child’s fetal hemoglobin has largely been
replaced by adult hemoglobin during the second
half of the first year.
- Resulting hypertrophy of the bone marrow,
hemosiderosis (excessive deposition of iron in
body tissues), & cardiac decompensation can
cause serious problems.
- The prognosis is improving but still grave; most
children with the disease die from cardiac failure
during adolescence or as young adults.
Assessment Findings:
1. Pallor
2. Irritability
3. anorexia
4. Increased facial-mandibular growth; slanted
eyes; broad, flattened base of nose; &
malocclusion
5. Jaundice possible
6. Bone pain
7. Hepatosplenomegaly possible
8. Epistaxis
9. Arrhythmias possible
10. Cardiac murmur possible
11. Hypochromic, microcytic red blood cells
12. Hemoglobin level less than 5 g/100 ml.
13. High serum iron level; iron saturation at 100%
Nursing Implication
- Help the child accept a permanently altered
appearance, & promote interaction with peers.
Murillo 2018-2019
- Encourage the parents to allow the child as much
activity as possible & to permit attendance at
regular school.
- Explain the benefits associated with hyper
transfusion therapy; suppresses erythropoiesis;
keeps facial alterations, osteoporosis, & cardiac
dilatation to a minimum; & reduces possibility of
splenectomy.
-Explain the risks associated with hyper-transfusion
therapy; hepatitis B &C, acquired immunodeficiency
syndrome, & hemosiderosis.
-Provide teaching about measures to help Prevent
congestive heart failure, such as digitalis
administration, diuretic therapy, & low –sodium diet.
-If splenectomy is planned, provide preoperative &
postoperative care, & stress measures to prevent
infection because the child will be more susceptible
after this surgery.
.
Thalassemia minor (heterozygous B-thalassemia)
- Is an anemia in which the child produces both
defective beta hemoglobin & normal
hemoglobin.
- This disorder is a minor form of anemia,
requiring no treatment.
- This condition represents the heterozy-gous
form of the disorder or can be compared with
children having the sickle cell trait.
Assessment Findings:
1. Pallor
2. Normal red blood cell count
3. Hemoglobin concentration level decreased 2 to
3 g/100 ml below normal levels
4. Moderately hypochromic & microcytic red blood
cells
Nursing Implication:
- Provide information about the nature of the
disorder.
- Provide reassurance that life expectancy is
normal
- Inform the parents that the child should not
receive a routine iron supplement because the
child’s inability to incorporate it well into
hemoglobin may cause him or her to
accumulate too much iron.
Therapeutic Management :
- Digitalis, diuretics, & low-sodium diet to prevent
heart failure, which could result from
decompensation that accompanies anemia, &
from myocardial fibrosis caused by invasion of
iron (hemochromatosis).
- Transfusion of Packed RBCs every 2 to 4 weeks
(hyper transfusion therapy) will Maintain
hemoglobin between 10 &12 g/ 100 ml. With
this level of hemoglobin, erythropoiesis is
suppressed & cosmetic facial alterations,
osteoporosis & cardiac dilatation are minimized.
- Hyper-transfusion therapy reduces the
possibility that splenectomy will be necessary.
- But frequent blood transfusions increase the risk
of blood-borne disease such as hepatitis B &
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PEDIA FINALS NOTES #2
deposition of iron in body
tissues(hemosiderosis).
 Hemosiderosis is focal deposition of iron that
does not cause tissue damage.
 Hemochromatosis (iron overload) is a
typically systemic process in which iron
deposition can cause tissue damage.
- Splenectomy necessary to reduce discomfort &
to reduce the rate of RBC hemolysis & the
number of necessary transfusions.
- Bone marrow stem cell transplantation- cure-
overall prognosis is improving but still grave.
- Children die of cardiac failure.
Nursing Diagnosis
- Risk for situational low self-esteem related to
changed physical appearance
Outcome Identification
- Child will demonstrate an adequate level of
self-esteem during course of illness.
Outcome Evaluation
- Child states he can accept altered appearance &
interacts with peers.
Hemophilia A ( von Willebrand’s Disease
Hemophilia A
- is the classic form of hemophilia, an inherited
interference with blood coagulation, caused by
deficiency of factor VIII, the anti-hemophilic
factor.
o This form affects 1 in 1000 white males
with severity of disease dependent on
varying levels of factor VIII.
- The female carrier has slightly lower levels of
factor VIII but sufficient enough not to manifest
a bleeding disorder.
- Factor VIII is an intrinsic factor of coagulation, &
in deficient amounts thromboplastin formation is
incomplete.
- The child’s extrinsic coagulation remains intact,
allowing eventual coagulation after an injury.
- This hemophilia may often reveal itself first with
excessive bleeding after circumcision.
- Repeated bleeding into the joints can lead to
hemarthrosis & result in severe loss of joint
mobility.
- Severe gastrointestinal, peritoneal cavity, or
central nervous system bleeding may occur.
- Even minor abrasions require administration of
factor VIII to control bleeding.
- As with all hemophilia, this disorder has no cure,
& prevention of symptoms is the goal of
therapy.
Assessment Findings:
1. Prolonged bleeding
2. Bruising, especially in lower extremities when
child begins to walk & bump into things.
3. Painful swollen, warm joints, from soft tissue
bleeding & hemorrhage into joints
4. GIT & CNS bleeding
5. Epistaxis
Murillo 2018-2019
6. Whole blood clotting times prolonged or normal
depending on level of factor VIII present
7. Abnormal thromboplastin generation test
8. Decreased partial thromboplastin time
Nursing Implication
-Obtain a thorough history from parents prenatally to
screen for possibility of this disorder.
-Observe all male infants carefully after circumcision.
-Note & evaluate any complains or signs of bruising or
painful joints.
-Evaluate the child for potential coagulation
abnormalities to assess which type of hemophilia is
present.
-Ensure that the parents & child have adequate
understanding of the pathology of factor VIII disease.
-Ensure that the child & parents are evaluated for
bleeding after any traumatic injury.
- Administer fresh whole blood, fresh frozen plasma,
or cryoprecipitate to provide repletion of factor VIII,
as prescribed.
- Administer plasma in no more than 30 minutes to
maintain the potency of factor VIII at room
temperature.
- As prescribed, administer epsilon aminocaproic acid,
a fibrinolytic enzyme that stabilizes clot formation &
promotes wound healing if antibodies to factor VIII
develop.
- Consider administration of factor IX concentrate to
halt bleeding if antibodies to factor VIII have
developed.
- Assist parents & the child in adapting to the
condition & the need for long-term monitoring.
- Teach parents & the child (over 10 years) how to
self-administer replacement factor intravenously to
prevent immediate bleeding after injury.
- Advise immobilization & ice packs to the injured
area to halt bleeding & relieve pain.
- Help parents to cope with this frightening disease, &
suggest support groups & organizations that would
help.
- Institute preventive teaching for children who are
known hemophiliacs:
- Teach parents & child prevention of injuries.
- Suggest safeguards for the house, such as removal
of sharp objects & keeping chairs away from
counters to prevent the child from climbing &
falling.
- Pad crib sides for active infants.
- Advise supervision for outside play.
- Emphasize that contact sports are prohibited.
- Teach the child to use a soft toothbrush.
- Teach an adolescent to use an electric shaver versus
a razor.
- Advise against taking any over the counter
medications that contain aspirin or ibuprofen.
- Teach parents & child how to minimize active
bleeding with firm pressure to site for 10 to 15
minutes, elevation of the injured area above the
level of the heart, & application of ice to area for 24
hours.
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- Teach parents & child to assess for hidden signs of
bleeding, including joint pain & stiffness, hematuria,
black stools, severe headache, slurred speech, &
lethargy.
- Assess the child’s self-esteem as related to living
with a chronic disease.
- Encourage the family to allow the child to lead a
normal life with toys & bicycle riding.
- Promote independence to assist the school –age
child to monitor his or her own activities.
Nursing Diagnosis
- Parental health seeking- behaviors related to
strategies for protecting the child from injury.
Outcome identification
- Parents will develop plan for preventing injury to
the child; child will not experience major
bleeding episodes during childhood.
Outcome evaluation
- Child’s skin is free of ecchymotic areas; absence
of frequent epistaxis; blood pressure within age
–appropriate parameters; absence of swelling or
warmth at joints.
Hemophilia B, Factor IX Deficiency ( Christmas Disease)
- is a clotting disorder in which factor IX is either
defective or lacking.
- Dysfunctional or deficient clotting factors
prevent normal coagulation & cause
inappropriate, prolonged bleeding.
- Christmas disease is transmitted by an X-linked
recessive inheritance pattern.
- Only 15% of hemophiliacs have this form.
- Christmas disease is treated with a concentrate
of factor IX.
Assessment Findings:
( usually asymptomatic unless injury, trauma, or surgery
occurs)
- Prolonged bleeding from any significant injury
- Epistaxis
- Abnormal partial thromboplastin time
Nursing Implications:
- Ensure that the parents & child have an adequate
understanding of the disease.
- Help the parents to cope with this frightening disease
& suggest support groups & organizations that would
help.
- Institute preventive teaching for children who are
known hemophiliacs:
- Teach parents home administration of factor IX.
- Teach parents & child about the need for preventing
injuries.
- Suggest safeguards for the house, such as removal of
sharp objects & keeping chairs away from counters
to prevent the child from climbing & falling.
- Advise supervision for outside play.
- Emphasize that contact sports are prohibited.
- Teach the child to use a soft toothbrush.
Murillo 2018-2019
- Teach the adolescent to use an electric shaver versus
a razor.
- Advise against taking over-the-counter medications
that contain aspirin or ibuprofen.
- Teach the parents & the child how to minimize active
bleeding with firm pressure to site for 10 to 15
minutes, elevation of the injured area above the level
of the heart, & application of ice to area for 24 hours.
- Teach the parents & child to assess for hidden signs
of bleeding, including joint
- Joint pain & stiffness , hematuria, black stools, severe
headache, slurred speech and lethargy.
- Assess the child’s self-esteem as related to living
with a chronic disease.
- Provide potential alternative activities that could
have a positive impact on the child’s self-esteem.
Hemophilia C, Factor XI Deficiency
- a clotting disorder is genetically transmitted as an
autosomal recessive trait & therefore occurs in
both sexes.
- Plasma thromboplastin precursor is deficient
secondary to factor XI deficiency.
- The symptoms associated with this hemophilia are
mild compared with those Children with factor VIII
or IX deficiencies.
- Bleeding episodes are treated with the transfusion
of fresh blood or plasma.
- As with all hemophilia, this disorder has no cure, &
prevention of symptoms is the goal of therapy.
Assessment Findings :
(usually asymptomatic unless injury, trauma or surgery
occurs)
- Epistaxis
- Bruising
- Minor pain & joint stiffness with hemarrhrosis-
bleeding into joints
- Spontaneous bleeding
Nursing Implications:
- Ensure that the parents & child have an adequate
understanding of the disease.
- Assist the parents & child in adapting to the
condition & the need for long-term monitoring.
- Help the parents to cope with this frightening
disease & suggest support groups & organizations
that would help. Initiate preventive teaching for
children who are known hemophiliacs:
- Teach parents & child about the need for preventing
injuries.
- Suggest safeguards for the house, such as removal
of sharp objects & keeping chairs away from
counters to prevent the child from climbing &
falling.
- Advise supervision for outside play.
- Emphasize that contact sports are prohibited.
- Teach the child to use a soft toothbrush.
- Teach the adolescent to use an electric shaver
versus a razor.
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PEDIA FINALS NOTES #2 Murillo 2018-2019
- Advise against taking over-the-counter medications
that contain aspirin like substances.

- Teach the parents & the child how to minimize

active bleeding with firm pressure to site for 10 to
15 minutes, elevation of the injured area above the
level of the heart, & application of ice to area for 24
hours.
- Teach the parents & child to assess for hidden signs
of bleeding, including joint pain & stiffness ,
hematuria, black stools, severe headache, slurred
speech and lethargy.
- Ensure that the child is evaluated for bleeding after
any traumatic injury.
- Administer fresh blood or plasma if needed to treat
spontaneous or traumatic bleeding.

- Assess for signs of transfusion reaction & treat

appropriately.
- Evaluate all body systems for signs of bleeding, &
ascertain that the child returns for a follow –up visit.
- Assess the child’s self-esteem as related to living
with a chronic disease.
- Provide potential alternative activities that could
have a positive impact on the child’s self-esteem.