The Child with Hematologic or
Immunologic Dysfunction
BSN310 Pediatric Nursing – Theory
Week 9
Semester 1- 2021 - 2022
Reviewed and Updated by,
Dr. Briliya Devadas, Oct. 2021
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Learning Outcomes
By the end of the lecture, the student will be able to :
 Identify major hematologic disorders that affect children in the UAE
 Determine assessment, pathophysiology and management for children
with anemias
 Discuss the nursing care for children with sickle cell anemia and
thalassemia
 Explain briefly about immunological conditions in children
 Describe nursing care guidelines for a child receiving blood transfusion
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Introduction
• History and physical examination are essential to identify
hematologic dysfunction, and the nurse is often the first person
to suspect a problem based on information from these
sources.
• A careful physical appraisal, especially of the skin, can reveal
findings (e.g., pallor, petechiae, bruising) that may indicate
minor or serious hematologic conditions.
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Red Blood Cell Disorders
Anemia
• The term anemia describes a condition in which the number of
red blood cells (RBCs) or the hemoglobin (Hgb ) concentration is
reduced below normal values for age.
• The anemias are the most common hematologic disorder of
infancy and childhood and could be an indication or manifestation
of an underlying pathologic process.
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Red Blood Cell Disorders
Anemia
Classification:
Anemias can be classified using two basic approaches:
Etiology, manifested by erythrocyte or Hgb depletion,
Morphology, the characteristic changes in RBC size, shape, or
color.
Although the morphologic classification is useful in terms of laboratory
evaluation of anemia, the etiology provides direction for planning
nursing care.
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Red Blood Cell Disorders
Anemia
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Red Blood Cell Disorders
Anemia
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Red Blood Cell Disorders
Anemia
Consequences of Anemia:
• Decrease in the oxygen-carrying capacity of blood and consequently
a reduction in the amount of oxygen available to the cells.
• The increased circulation and turbulence within the heart may
produce a murmur.
• Cyanosis, which results from an increased quantity of deoxygenated
Hgb in arterial blood
• Growth retardation, resulting from decreased cellular metabolism
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Red Blood Cell Disorders
Anemia
Therapeutic Management
• The objective of medical management is to reverse the anemia by
treating the underlying cause.
• In nutritional anemias, the specific deficiency is replaced.
• In blood loss from acute hemorrhage, RBC transfusion is given.
• In patients with severe anemia, supportive medical care may
include oxygen therapy, bed rest, and replacement of intravascular
volume with intravenous (IV) fluids.
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Red Blood Cell Disorders
Anemia
Nursing Care Management
• Decrease Tissue Oxygen Needs by assessing the child's level of
tolerance for activities of daily living and play, and make adjustments
to allow as much self-care as possible without undue exertion.
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Red Blood Cell Disorders
Iron Deficiency Anemia
• Anemia caused by an inadequate supply of dietary iron is the
most prevalent and preventable nutritional disorder.
• Adolescents are also at risk because of their rapid growth rate
combined with poor eating habits, menses, obesity, or strenuous
activities.
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Red Blood Cell Disorders
Iron Deficiency Anemia
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Red Blood Cell Disorders
Iron Deficiency Anemia
Pathophysiology
Iron-deficiency anemia can be caused by any number of factors that
• Decrease the supply of iron,
• Impair its absorption,
• Increase the body's need for iron, or
• Affect the synthesis of Hgb.
• Although the clinical manifestations and diagnostic evaluation are
similar regardless of the cause, the therapeutic and nursing care
management depends on the specific reason for the iron deficiency.
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Red Blood Cell Disorders
Iron Deficiency Anemia
During the last trimester of pregnancy :
• Iron is transferred from the mother to the fetus. The iron is stored in
the fetal liver, spleen, and bone marrow.
• These iron stores are usually adequate for the first 5 to 6 months in
a full-term infant but for only 2 to 3 months in preterm infants and
multiple births.
• If dietary iron is not supplied to meet the infant's growth demands
after the fetal iron stores are depleted, iron deficiency anemia
results.
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Red Blood Cell Disorders
Iron Deficiency Anemia
• Physiologic anemia should not be confused with iron-deficiency
anemia resulting from nutritional causes. Although infants with irondeficiency anemia are underweight, many are overweight because of
excessive milk ingestion (known as milk babies).
• These children become anemic for two reasons:
• (1) milk, a poor source of iron, is given almost to the exclusion of solid
foods, and
• (2) increased fecal loss of blood occurs in 50% of iron-deficient
infants fed cow's milk.
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Red Blood Cell Disorders
Iron Deficiency Anemia
Therapeutic Management
• In formula-fed infants, the most convenient and best sources of
supplemental iron are iron fortified commercial formula and ironfortified infant cereal. Oral iron supplements are prescribed If dietary
sources of iron cannot replenish the body stores. Ascorbic acid
(vitamin C) appears to facilitate absorption of iron and may be given
as vitamin C–enriched foods and juices with the iron preparation.
• Parenteral (IV or IM) iron administration reserved for children who
have iron malabsorption
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Red Blood Cell Disorders
Iron Deficiency Anemia
Nursing Care Management
• Oral iron should be given as prescribed in two divided doses between
meals.
• Citrus fruit or juice taken with the medication aids in absorption.
• An adequate dosage of oral iron turns the stools a tarry green or black
color. The nurse needs to advise parents of this normally expected
change.
• Absence of the greenish black stool may be a clue to poor compliance.
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Red Blood Cell Disorders
Sickle Cell Anemia
Although the defect is
inherited, the sickling
phenomenon
is
usually not apparent
until later in infancy
because
of
the
presence of fetal Hgb
(HbF).
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Types of Crisis in
Sickle Cell Anemia
• Vaso-occlusive crisis (VOC), preferably called a “painful episode,” is
characterized by ischemia causing mild to severe pain that may last
from minutes to days or longer.
• Sequestration crisis is a pooling of a large amount of blood usually
in the spleen that causes a decreased blood volume and ultimately
shock.
• Aplastic crisis is diminished RBC production,
• Hyper hemolytic crisis is an accelerated rate of RBC destruction
characterized by anemia, jaundice, and reticulocytosis.
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Red Blood Cell Disorders
Sickle Cell Anemia
Precipitating factors:
• Anything that increases body’s need for oxygen or alters
transport of oxygen
• Trauma/ Infection, fever
• Physical and emotional stress
• Increased blood viscosity due to dehydration
• Hypoxia: From high altitude, poorly pressurized airplanes,
hypoventilation, vasoconstriction due to hypothermia
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Mosby items and derived items © 2009, 2005 by Mosby, Inc., an affiliate of Elsevier Inc.
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Red Blood Cell Disorders
Sickle Cell Anemia
Therapeutic Management:
Medical management of a crisis is usually directed toward
supportive, symptomatic and specific treatments.
The main objectives are to provide
1. rest to minimize energy expenditure and to improve oxygen
utilization
2. hydration through oral and IV therapy
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Red Blood Cell Disorders
Sickle Cell Anemia
3. electrolyte replacement because hypoxia results in metabolic
acidosis, which also promotes sickling
4. analgesia for the severe pain from vaso-occlusion
5. blood replacement to treat anemia and to reduce the viscosity of
the sickled blood
6. antibiotics to treat any existing infection
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Red Blood Cell Disorders
Sickle Cell Anemia
Prognosis
• No cure (except with bone marrow transplants)
• Supportive care/prevent sickling episodes
• Frequent bacterial infections may occur due to immunocompromise
• Bacterial infection is leading cause of death in young children with
sickle cell disease
• Strokes in 5%-10% of children with disease
Result in neurodevelopment delay, mental retardation
10/17/2021
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Extended Reading
Hamad’s Story (May 2018, CryoSave Arabia)
Hamad is an eight-year-old Emirati boy who
was cured of Sickle Cell Disease thanks to
the cord blood stem cells from his younger
brother, Abdullah, that were stored by
CryoSave Arabia.
To learn more about cord blood banking,
visit Parent's Guide to Cord Blood
Foundation at
https://parentsguidecordblood.org/en/news/
hamads-story
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Red Blood Cell Disorders
Thalassemia
• Inherited blood disorders
of hemoglobin synthesis
• Autosomal recessive with
varying expressivity
– Both parents must be
carriers to have
children with disease
10/17/2021
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Red Blood Cell Disorders
Thalassemia
• Classified by the type of Hgb chain affected and by amount of effect :
• -Thalassemia
• Alpha chains affected
• Occurs in Chinese, Thai, African, and Mediterranean people
Incidence of -Thalassemia high in UAE
• -Thalassemia
• Occurs in Greeks, Italians, and Syrians
•  is most common and has four forms
10/17/2021
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Red Blood Cell Disorders
Thalassemia
Four types
• Thalassemia minor—asymptomatic silent carrier
• Thalassemia trait—mild microcytic anemia
• Thalassemia intermediate—moderate to severe anemia +
splenomegaly
• Thalassemia major (“Cooley’s anemia”) —severe anemia
requiring transfusions to survive
10/17/2021
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Red Blood Cell Disorders
Thalassemia
Pathophysiology; Clinical manifestations of thalassemia
• Anemia results from defective synthesis of Hgb, structurally
impaired RBCs, and shortened life of RBCs → hemolysis → severe
anemia
• Chronic hypoxia
Headache,
 irritability,
10/17/2021
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Red Blood Cell Disorders
Thalassemia
precordial and bone pain,
exercise intolerance, anorexia, epistaxis
• Detected in infancy or toddlerhood
Pallor, FTT, hepatosplenomegaly, severe anemia (Hgb <6)
 Bone changes in older children if untreated
10/17/2021
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Red Blood Cell Disorders
Thalassemia
Medical Management of Thalassemia
• Blood transfusion to maintain normal Hgb levels
• Side effect—hemosiderosis (Iron overload)
• Treat with iron chelating drugs such as deferoxamine (Desferal)
that binds excess iron for excretion by kidney
10/17/2021
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Red Blood Cell Disorders
Thalassemia
• Nursing Management
• Observe for complications of transfusion
• Emotional support to family
• Encourage genetic counseling
• Parent and patient teaching for self-care
10/17/2021
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Red Blood Cell Disorders
Thalassemia
Prognosis
 Retarded growth
 Delayed or absent secondary sex characteristics
 Expected to live well into adulthood with proper clinical
management
 Long term complication of multiple organ failure secondary to
hemochromatosis ( tissue damage from iron overload)
 Hematopoietic stem cell transplantation is a potential cure
10/17/2021
MS Patricia Matizha
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Extended Reading
Thalassemia carrier population continues to remain high in
UAE (2018)
https://www.khaleejtimes.com/nation/dubai/thalassemia-carrier-population-continuesto-remain-high-in-uae
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Immunological Disorders
A number of disorders can cause profound, often life-threatening
alterations within the body's immune system. In autoimmune
disorders, antibodies, macrophages, and lymphocytes attack healthy
cells.
Several classifications of immune dysfunction exist. AIDS, and SCID
(Severe Combined Immuno-deficiency disease) are syndromes
where in the body is unable to mount an immune response.
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Common Clinical Manifestations of Human
Immunodeficiency Virus Infection in Children
• Lymphadenopathy
• Hepatosplenomegaly
• Oral candidiasis
• Chronic or recurrent diarrhea
• Failure to thrive
• Developmental delay
• Parotitis
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Nursing Care Management
includes – patient outcomes
• Early recognition of infection,
• Health education about standard precautions
• Rate of infection slowed or maintained
• Growth and development promoted
• No infectious complications or cancer development
• Adherence to antiretroviral therapy
• Prolonged survival
• Quality of life supported
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Blood Transfusion Therapy
• Technologic advances in blood banking and transfusion
medicine enable the administration of only the blood
component needed by the child, such as packed RBCs in
anemia or platelets for bleeding disorders.
• Regardless of the blood component administered, the nurse
must be aware of the possible transfusion reactions
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Transfusion Reactions
• Hemolytic: the most severe, but rare
• Febrile reactions: fever, chills
• Allergic reaction: urticaria, pruritus, laryngeal edema
• Air emboli: may occur when blood is transfused under pressure
• Hypothermia
• Electrolyte disturbances: hyperkalemia from massive
transfusions or patient with renal problems
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Blood Transfusion Therapy
General guidelines :
• Take vital signs, including blood pressure, before administering blood
to establish baseline data for pre transfusion and post transfusion
comparison; 15 minutes after initiation; hourly while blood is infusing;
and on completion of transfusion.
• Administer the first 50 ml of blood or initial 20% of the volume
(whichever is smaller) slowly and stay with the child.
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Blood Transfusion Therapy
General guidelines that apply to all transfusions include:
• Administer blood through an appropriate filter to eliminate particles in
the blood and prevent the precipitation of formed elements;
• Use blood within 30 minutes of its arrival from the blood bank;
• If a reaction of any type is suspected, stop the transfusion, take vital
signs, maintain a patent IV line with normal saline and new tubing,
notify the practitioner, and do not restart the transfusion until the
child's condition has been medically evaluated
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Let’s review
A child with sickle cell anemia (SCA) is admitted in a vaso-occlusive
crisis (VOC). Which of the following interventions should the nurse
expect to see ordered? (Select all that apply).
a. Cold compresses to painful joints
b. IV fluids started, and oral fluids encouraged
c. Meperidine ordered every 4 hours for pain
d. High-calorie, high-protein diet
e. Antibiotics ordered for any existing infection
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References
• Hockenberry, Marylin,J., Wilson, David, 10 th Edition, 2017, Wong's
essentials of pediatric nursing, Elsevier,Mosby
• Marilyn J. Hockenberry & Wilson Winkelstein (2013) Wong’s Essentials of
Pediatric Nursing, 9th Edition, Elsevier Mosby
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