Chapter 27

The Child with a Condition of

the Blood, Blood-Forming
Organs, or Lymphatic System
 Lesson 27.1
Objectives (1 of 2)
1. Define each key word listed.
2. Summarize the components of blood.
3. List two laboratory procedures commonly
performed on children with blood disorders.
4. List the symptoms, prevention, and
treatment of iron deficiency anemia.
5. Review the effects of severe anemia on the
heart.
 Lesson 27.1
Objectives (2 of 2)
6. Recommend four food sources of iron for a
child with iron deficiency anemia.
7. Examine the pathology and signs and
symptoms of sickle cell disease.
8. Describe four types of sickle cell crises.
9. Devise a nursing care plan for a child with
sickle cell disease.
 Lymphatic System (1 of 2)

 Drains regions of the body to lymph node

 Where infectious organisms are destroyed
 Antibody production is stimulated.
 Lymphadenopathy is an enlargement of
lymph nodes.
 Indicative of infection or disease
 Lymphatic System (2 of 2)

 Spleen is largest organ of the lymphatic

system
 One of the main functions is to bring blood
into contact with lymphocytes
 Most common pathological condition is
enlargement (splenomegaly)
 Enlarges during infections, congenital and
acquired hemolytic anemias, and liver
malfunction
 Circulating Blood (1 of 2)

 Consists of two portions

 Plasma
 Formed elements
 Erythrocytes
 Leukocytes (white blood cells [WBCs])
 Thrombocytes (platelets)
 Circulating Blood (2 of 2)

 Erythrocytes
 Transport oxygen and carbon dioxide to and from
the lungs and tissues
 Leukocytes act as the body’s defense against
infection.
 Lymphocytes are produced in the lymphoid
tissues of the body.
 Anemias

 Can result from many different underlying

causes
 A reduction in the amount of circulating
hemoglobin (Hgb) reduces the oxygen-carrying
ability of the blood.
• An Hgb below 8 g/dL results in an increased cardiac
output and a shunting of blood from the periphery to the
vital organs.
• Can result in pallor, weakness, tachypnea, SOB, CHF
 Iron Deficiency Anemia (1 of 4)

 Most common nutritional deficiency of

children
 Incidence is highest during infancy (from 9th to
24th month) and adolescence
 May be caused by severe hemorrhage,
inability to absorb iron received, excessive
growth requirements, or an inadequate diet
 Giving whole cow’s milk to infants can lead to GI
bleeding, leading to anemia
 Iron Deficiency Anemia (2 of 4)

 Manifestations
 Pallor
 Irritability
 Anorexia
 Decrease in activity
• Infants may be overweight because of excessive milk
consumption.
 Iron Deficiency Anemia (3 of 4)

 Blood tests
 RBC count
 Hgb and hematocrit
 Morphological cell changes
 Iron concentrations
 Stool may be tested for occult blood.
 Iron Deficiency Anemia (4 of 4)

 Untreated, iron deficiency anemia will

progress slowly
 In severe cases, heart muscle becomes too weak
to function.
 Children with long-standing anemia may also
show growth retardation and cognitive changes.
 Treatment
 Iron, usually ferrous sulfate, orally 2 to 3 times a
day
 Vitamin C aids in absorption.
 Two Types of Sickle Cell Disease
(1 of 2)
 Sickle cell trait (asymptomatic)
 Blood of the patient contains a mixture of Hgb A
and sickle (Hgb S).
 Proportions of Hgb S are low because the disease
is inherited from only one parent.
 Hgb and RBC counts are normal.
 Two Types of Sickle Cell Disease
(2 of 2)
 Sickle cell anemia (more severe)
 Clinical symptoms do not appear until the last part
of the first year of life.
• May be an unusual swelling of the fingers and toes
• Symptoms caused by enlarging bone marrow sites that
impair circulation to the bone and the abnormal sickle
cell shape that causes clumping, obstruction in the
vessel, and ischemia to the organ the vessel supplies.
 Manifestations

 Hgb level ranges 6 to 9 g/dL or lower.

 Child is pale, tires easily, and has little appetite.
 Sickle cell crises are painful and can be fatal.
 Symptoms: severe abdominal pain, muscle
spasms, leg pain, or painful swollen joints may be
seen
• Fever, vomiting, hematuria, convulsions, stiff neck, coma,
or paralysis can result.
• Risk for stroke as a complication of a vaso-occlusive
sickle cell crisis
 Types of Sickle Cell Crises

 Vaso-occlusive (painful crises)

 Splenic sequestration
 Aplastic crises
 Hyperhemolytic
 Lesson 27.2
Objectives
10. Recognize the effects on the bone marrow of
increased red blood cell production caused by
thalassemia.
11. Recall the pathophysiology and signs and symptoms
of hemophilia A and B.
12. Identify the nursing interventions necessary to
prevent hemarthrosis in a child with hemophilia.
13. Compare and contrast four manifestations of
bleeding into the skin.
14. Recognize normal blood values of infants and
children.
 Thalassemia (1 of 2)

 Group of hereditary blood disorders in which

the patient’s body cannot produce sufficient
adult Hgb
 RBCs are abnormal in size and shape and
are rapidly destroyed; results in chronic
anemia
 Body attempts to compensate by producing
large amounts of fetal Hgb
 Thalassemia (2 of 2)

 Categorized according to the polypeptide

chain affected
 Beta-thalassemia is the most common variety;
involves impaired production of beta chains
 Two forms
• Thalassemia minor
• Thalassemia major, also known as Cooley anemia
 Can also occur from spontaneous mutations
 Hemophilia

 Inherited sex-linked recessive trait

 Defective gene is located on the X, or female,
chromosome.
 Fetal blood samples detect hemophilia.
 Two most common types
 Hemophilia B (Christmas disease [a factor IX
deficiency])
 Hemophilia A (a deficiency in factor VIII)
 A deficiency in any one of the factors will
interfere with normal blood clotting.
 Hemophilia A
 Caused by a deficiency of coagulation factor VIII, or
antihemophilic globulin (AHG)
 Severity depends on level of factor VIII in the plasma.
 Some patients’ lives can be endangered by a minor
scratch, but others may simply bruise more easily
than the average person.
 Aim of therapy is to increase level of factor VIII to
ensure clotting.
 This is checked by a blood test call partial
thromboplastin time (PTT).
Manifestations of Hemophilia (1 of 2)

 Can be diagnosed at birth because factor VIII

cannot cross the placenta and be transferred
to the fetus
 Usually not apparent in the newborn unless
abnormal bleeding occurs at the umbilical cord or
after circumcision
 Normal blood clots in 3 to 6 minutes.
 In severe hemophilia, it can take up to 1 hour or
longer.
Manifestations of Hemophilia (2 of 2)

 Anemia, leukocytosis, moderate increase in

platelets may be seen in hemorrhaging; may
also be signs of shock
 Spontaneous hematuria is seen.
 Death can result from excessive bleeding,
especially if it occurs in the brain or neck.
 Severe headache, vomiting, and
disorientation may be symptoms.
 Idiopathic (Immunological)
Thrombocytopenic Purpura (ITP)
 Acquired platelet disorder that occurs in
childhood
 Most common of the purpuras
 Cause is unknown but is thought to be an
autoimmune reaction to a virus
 Platelets become coated with antiplatelet
antibody, seen as “foreign,” and are
eventually destroyed by the spleen.
 ITP occurs in all age groups, with main
incidence between 2 and 4 years of age.
 Manifestations of ITP

 Classic symptom is easy bruising.

 Results in petechiae and purpura
 May have recent history of rubella, rubeola,
or viral respiratory infection
 Interval between exposure and onset is about 2
weeks.
 Platelet count below 20,000/mm3 (normal
range is between 150,000 and 400,000/mm3)
 Diagnosis confirmed by bone marrow aspiration
 Treatment of ITP (1 of 2)

 Neurologic assessments are a priority of

care.
 Treatment is not indicated in most cases.
 If indicated, prednisone, IV gamma globulin,
and anti-D antibody are some of the
treatment options.
 In cases of chronic ITP, a splenectomy may
be required.
 Treatment of ITP (2 of 2)

 Drugs to avoid
 Aspirin
 Phenylbutazone
 Phenacetin
 Caffeine
 Activity is limited during acute states to avoid
bruising.
 Platelets are usually not given because they
are destroyed by the disease process.
 Complications of ITP

 Bleeding from the GI tract

 Hemarthrosis
 Intracranial hemorrhage
 Prevention may be helped by immunizing all
children against the viral diseases of
childhood.
 Lesson 27.3
Objectives
[Link] the nursing care of a child with
leukemia.
[Link] the nursing care of a child receiving
a blood transfusion.
[Link] the effects of chronic illness on the
growth and development of children.
 Disorders of White Blood Cells
Leukemia
 Most common form of cancer in childhood
 Refers to a group of malignant diseases of the bone
marrow and lymphatic system
 Classified according to what type of WBC affected
 Two most common
 Acute lymphoid leukemia (ALL)
 Acute non-lymphoid (myelogenous) leukemia (AMLL or
AML)
 Cytochemical markers, chromosome studies, and
immunologic markers differentiate the two types.
 Leukemia
 A malignant disease of the blood-forming organs that
results in an uncontrolled growth of immature WBCs
 Involves a disruption of bone marrow function caused
by the overproduction of immature WBCs in the
marrow
 These immature WBCs take over the centers that are
designed to form RBCs, and anemia results.
 Platelet counts are also reduced.
 Invasion of the bone marrow causes weakening of
the bone, and pathological fractures can occur.
 Manifestations of Leukemia (1 of 2)

 Most common symptoms

 Initial phase
• Low-grade fever
• Pallor
• Bruising tendency
• Leg and joint pain
 Listlessness
 Abdominal pain
 Enlargement of lymph nodes
 Anemia severe despite transfusions
 Manifestations of Leukemia (2 of 2)

 Gradual or sudden onset

 As it progresses, the liver and spleen become
enlarged.
 Skin may have a lemon-yellow color.
 Petechiae and purpura may be early
objective symptoms.
 Anorexia, vomiting, weight loss, and dyspnea
are also common.
 Diagnosis
 Based on history and symptoms
 Results of extensive blood tests
 Demonstrate presence of leukemic blast cells in the blood,
bone marrow, or their tissues
 Radiographs of the long bones show changes.
 Spinal tap may be done to check for CNS
involvement.
 Kidney and liver function studies are done.
 The adequacy of their function is essential to the outcomes
of chemotherapy.
 Treatment of Leukemia

 Radiation and chemotherapy target specific

cells.
 Components of chemotherapy include
 Induction period
 CNS prophylaxis for high-risk patients
 Maintenance
 Reinduction therapy (if relapse occurs)
 Extramedullary disease therapy
 Bone marrow transplant
 Side Effects of Chemotherapy

 Steroids can mask signs of infection, cause fluid

retention, induce personality changes, and
cause the child’s face to appear moon shaped.
 Certain chemotherapy agents can cause
nausea, diarrhea, rash, hair loss, fever, anuria,
anemia, and bone marrow depression.
 Peripheral neuropathy may be signaled by
severe constipation caused by decreased nerve
sensations to the bowel.
 Nursing Care of the Child with
Leukemia
 Encourage the child to verbalize feelings.
 Giving permission to discuss their concerns will
help clear up misconceptions and to decrease
feelings of isolation.
 Frequently observe child for infection.
 Monitor vital signs and for symptoms of
thrombocytopenic bleeding (a common
complication of leukemia).
 Meticulous mouth and skin care
 Hodgkin Disease (aka Hodgkin
Lymphoma)
 A malignancy of the lymph system that primarily
involves the lymph nodes
 May metastasize to the spleen, liver, bone marrow, lungs, or
other parts of the body
 Presence of giant multinucleated cells called Reed-
Sternberg cells is diagnostic of the disease
 Rarely seen before 5 years of age; incidence
increases during adolescence and early adulthood
 Twice as common in boys as in girls
 Treatment of Hodgkin Lymphoma

 Both radiation and chemotherapy are used in

accordance with the clinical stage of the
disease.
 Cure is primarily related to the stage of
disease at diagnosis.
 Long-term prognosis is excellent.
 Nursing Care of Patients with
Hodgkin Lymphoma
 Mainly directed toward symptomatic relief of the side
effects of radiation and chemotherapy
 Education of patient and family
 Malaise is common after radiation therapy; child tires
easily and may be irritable and anorexic
 Skin in treated area may be sensitive and must be
protected against exposure to sunlight and irritation.
 The patient does not become radioactive during or
after therapy.
Emotional Support of the Patient with
Hodgkin Lymphoma
 Support provided should be age appropriate.
 Activity is generally regulated by the patient.
 Appearance of secondary sexual
characteristics and menstruation may be
delayed in pubescent patients.
 Sterility is often a side effect of treatment.
 Nursing Care of the Chronically Ill Child
Chronic Illness
 Behavior problems are lessened when patients can
verbalize specific concerns with persons sensitive to
their problems.
 If they feel rejected by and different from their peers,
they may be prone to depression.
 Nurses must develop an awareness of the
adolescent’s particular fears of forced dependence,
body invasion, mutilation, rejection, and loss of face,
especially within peer groups.
 It is important to recognize the adolescent’s need for
self-determination.
 Developmental Disabilities

 Children with developmental disabilities may

often be overprotected, unable to break away
from supervision, and deprived of necessary
peer relationships.
 The pubertal process with its emerging
sexuality concerns parents and may
precipitate a family crisis.
 Care of the Chronically Ill Child

 Focusing on what the child can do and

providing successful experiences are more
effective than focusing on the disability.
 Involvement of the entire family with the care of
the chronically ill child aids in normal family
interaction.
 Child should be integrated into rather than
isolated from the community and society.
 The wellness of the child, rather than the
disability, should be the center of the child’s life.
 Lesson 27.4
Objectives
[Link] the nurse’s role in helping families
deal with the death of a child.
[Link] techniques the nurse can use to
facilitate the grieving process.
[Link] age-appropriate responses to a
sibling’s death and the nursing interventions
required.
[Link] the stages of dying.
 Nursing Care of the Dying Child
Facing Death
 The nurse must understand
 The grieving process
 Personal and cultural views concerning that
process
 The views of a parent losing a child
 Perceptions of the child facing death
 The response to a child’s death is influenced
by whether there was a long period of
uncertainty before the death or whether it was
a sudden, unexpected event.
 Facing Death
 The nurse must
 Show compassion.
 Demonstrate a nonjudgmental approach.
 Be sensitive and effective in the provision of care.
 Facilitate the grief process by anticipating psychological and
somatic responses while maintaining open lines of
communication.
 Support the family’s efforts to cope, adapt, and grieve.
 Know that hostility is a normal response and may drive away
those who do not understand its normalcy in the acute
grieving process.
 The Child’s Reaction to Death
 Cognitive development, rather than chronological
age, affects the response to death.
 Children younger than 5 years of age are mainly
concerned with separation from their parents and
abandonment.
 Preschool children respond to questions about death
by relying on their experience and by turning to
fantasy.
 Children do not develop a realistic concept of death
as a permanent biological process until 9 or 10 years
of age.
 Physical Changes of Impending
Death
 Cool, mottled, cyanotic skin and the slowing
of all body processes
 Loss of consciousness, but hearing may still
be intact
 Rales in the chest may be heard, which are
caused by increased pooling of secretions in
the lungs.
 Movement and neurologic signs lessen.
 Stages of Dying and the Nurse’s
Role
 Stages
 Denial
 Anger
 Bargaining
 Depression
 Acceptance
 Nurse’s role
 Listen.
 Provide privacy.
 Provide therapeutic intervention.
 Provide information.
 Use appropriate phrases and open-ended statements.