Nursing Care of
Children with
Hematologic
Alterations
Chapter 26
By Nataliya Haliyash,
MD, BSN
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• Disorders related to the blood and blood-forming
organs in childhood encompass a wide range of
diseases and pathologic states.
• Since the blood is a multipurpose fluid involved
in the functions of so many tissues and organs,
either primary or secondary changes in the blood
are reflected in the essential functions of these
structures.
• Disorders of blood and blood-forming organs in
childhood include the anemias, defects in
hemostasis, and the immunologic deficiency
diseases. Neoplastic disorders of the
hematopoietic system are also included.
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Lecture Objectives
Upon completion of this chapter, the reader will be
able to:
• Discuss the anatomy and physiology of the
hematological system.
• Identify differences between child and adult
hematological systems.
• Explain and identify different types of anemia
and discuss the etiology, treatment, and nursing
care of those types.
• Identify several types of coagulation disorders
and discuss the etiology, treatment, and nursing
care of those disorders.
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Anatomy
The hematologic system consists of:
• the blood
• and blood-forming (hemopoietic) tissues:
– Red bone marrow (myeloid tissue)
– Lymph nodes
– Spleen.
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Anatomy
Blood is composed of two components:
• a fluid portion called plasma
• a cellular portion known as the formed
elements of the blood
The two components are approximately
equal in volume.
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Anatomy
• Plasma is about 90% water and 10%
solutes.
• The principal solutes are:
– the proteins: albumin, globulin, and
fibrinogen.
• The cellular elements are:
– red blood cells (erythrocytes),
– white blood cells (leukocytes),
– platelets (thrombocytes).
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Hematopoiesis
(blood-formation)
• All of the formed elements of the blood, except
to some extent the agranulocytes, are believed
to be formed in myeloid tissue during postnatal
life.
• During embryonic development the
mesenchyme, spleen, liver, thymus, and yolk sac
serve as additional sites of blood cell formation.
• In certain blood disorders these sites, particularly
the spleen, can be stimulated to produce blood
cells, and constitute extramedullary
hematopoiesis.
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Hematopoiesis
(blood-formation)
• Each blood cell originates from a primordial
(primitive) cell called a blast, or stem, cell in the
bone marrow. This hemocytoblast in turn gives
rise to the erythroblast, myeloblast, monoblast,
lymphoblast, and megakaryoblast.
• Reticuloendothelial system consists of widely
dispersed cells of mesodermal origin that line the
vascular and lymph channels. These cells, called
reticular cells, are capable of:
– phagocytosis (ingestion and digestion of
foreign substances),
– formation of immune bodies,
– differentiation into other cells, such as
hemocytoblasts, myeloblasts, or
lymphoblasts.
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Blood functions:
• transporting substances needed for
cellular metabolism in the tissues;
• regulating acid-base balance;
• protecting against infection and injury.
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Differences between child and
adult hematological systems
• In infants and young children all of the
bone contains red marrow (so-called
because of its color from formation of
erythrocytes).
• At the end of adolescence, only the ribs,
sternum, vertebrae, and pelvis continue
to produce blood cells. The remainder of
the bone marrow becomes yellow from
deposition of fat.
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Regulation of erythrocyte
production
• The usual life span of the mature erythrocyte is 120
days.
• They are removed from circulation, mainly by the
spleen.
• Because RBCs are not capable of replication, the
bone marrow releases new, immature erythrocytes
called reticulocytes to replace the RBCs removed
from circulation.
• The hemoglobin is broken down into the ironcontaining pigment hemosiderin and the bile
pigments biliverdin and bilirubin.
• The basic regulator of erythrocyte production is
believed to be tissue oxygenation.
• In states of tissue hypoxia, erythropoietin (also
called erythropoietic stimulating factor or
hemopoietin) is released by the kidneys into the
bloodstream.
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Age peculiarities of blood:
• Smaller than in adults general volume of blood.
• Higher than in adults relative blood volume: versus 50-80
mL/kg in adults
• RBC volume varies with age.
• Immediately after birth there is a period of inactive
erythropoiesis during which the iron obtained from
catabolized RBCs is stored as hemosiderin in the bone
marrow and the liver tissue.
• These stores are greatest at 4 to 8 weeks of age and
function to protect the infant from anemia.
• Premature infants use up these stores within 6 to 12
weeks, whereas the iron stores of full-term infants last up
to 20 weeks.
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Erythrocyte (RBC) count – age
reference
cord
5-7 × 1012 cells/L
1-3 days
6-7 × 1012 cells/L
1wk-1 yr
2.7-4.5 × 1012 cells/L
2-12 yr
3.9-5.3 × 1012 cells/L
12-18 yr: Male
4.5-5.5 × 1012 cells/L
Female
4-5 × 1012 cells/L
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Age peculiarities of blood newborn:
• Short life span of erythrocytes - 12-40 days
versus 120 days
• Erythrocytosis 6-7 × 1012 cells/L
• Anisocytosis (presence of different forms of
RBCs inside vessels)
• Hemoglobin level 180-240 g/L
• Hb F – 63-92 % versus 5 % adults
• Color index (CI) 1.1-1.3 (degree of saturation of
Hb in one erythrocyte)
• Erythrocyte sedimentation rate-0-2 mm/hour
• Leucocytosis 11-33 × 109 cells/Liter
• Presence of extramedullar sources of
hemopoiesis.
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Age peculiarities of blood –
child 1mo – 1 yr:
• Decreasing Hb level to 120-110 g/L
• Erythrocyte level decreases, as well, to 4-3.5 ×
1012 cells/L
• Reticulocyte count is up to 10 %
• Colour index is low: 0.8-0.7
• Leucocytosis 10-12 × 109 cells/L appears after:
– adding new food in menu
– during crying
– emotions
• 1st cross of WBC count – 5 mo
• Platelets have gigantic forms
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WBC differential count
during childhood
Eosinophils
Basophils
Neutrophils
“bands”
Neutrophils
“segs”
3d
5d
3 yr
5 yr
12 yr
1-4 %
1-4 %
3%
1-4 %
1-4 %
0-0.5 % 0-0.5 % 0-0.5 % 0-0.5 % 0-0.5 %
3-5 %
3-5 %
3-5 %
3-5 %
3-5 %
54-62 % 39-42 % 24-30 % 39-42 % 54-62 %
Lymphocytes
11-25 %
45 %
63 %
45 %
25-33 %
Monocytes
6-10 %
6-10 %
6-10 %
6-10 %
6-10 %
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Age peculiarities of blood –
child 1mo – 1 yr:
• Increasing of Hb level to 130-140 d/L till
15 years
• Erythrocyte level 4.5-5.0 × 1012 cells/L
• Reticulocytes are about 0.5-5 %
• Leukocyte level 7-9 × 109 cells/L
• Second WBC count cross in 5 years of
age
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Examination:
subjective data
There are some weighty clinical signs that
directly show on blood disorders. They
are:
• Hemorrage (bleeding);
• Hematoma;
• Enlargement of lymph nodes;
• Pallor;
• Ossalgia (pain in bones).
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Examination:
subjective data
Pica – character sign of irondeficient anemia.
Eating clay, ice, paste.
Other complaints:
• Often headaches,
• Dizziness, light-headednass,
• Slowed thought processes, dicreases attention
span,
• hyperthermia,
• Apathy,
• Shortness of breath,
• Easy fatigability.
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Anemias.
• A condition in which the number of RBCs
and/or the hemoglobin concentration is
reduced below normal:
– 0-14 days - Hb<145 g/L
– 15-28 days - Hb<120 g/L
– 1mo-1yr - Hb<110 g/L
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Anemias.
The basic cause of anemia is either
(1) an increased loss or destruction of red
blood cells
(2) an impaired or decreased rate of
production.
An etiologic classification is based on the
various conditions that can lead to either
of these results.
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Classification of anemias:
• Decreased Production of RBCs:
– Iron Deficiency Anemia
– Aplastic Anemia
• Increased Destruction of RBCs:
– Hemolytic Anemia
– Sickle Cell Anemia
– Thalassemia
• Blood loss:
– Iron Deficiency Anemia
– Hemorrhagic Anemia
• Severety:
– Mild: Hb 110-90 g/L
– Moderate: Hb 89-70 g/L
– Severe: Hb < 70 g/L
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Classification of anemias:
• Morphologic (describe the size of RBCs):
– (1) normocytic,
– (2) microcytic,
– (3) macrocytic.
• According to the amount of hemoglobin in
the cell (describe the color and Hb
content of the cells):
– (1) normochromic
– (2) microchromic.
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Anemia. Assessment
• Take health history:
– Careful diet history to identify any
deficiencies,
– Evidence of pica – eating clay, ice, paste.
• Observe for manifestations of anemia:
– Muscle weakness
– Easy fatigability:
•
frequent resting,
•
shortness of breath,
•
poor sucking (infants)
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Manifestations of anemia
• Pale skin, mucous membranes, lips, nail beds, and conjunctiva
– Waxy pallor seen in severe anemia
– Capillary refill – no change color
• Rapid, pounding heart beat
• CNS manifestations:
– Headache
– Dizziness
– Light-headedness
– Irritability
– Slowed thought processes, dicreases attention span
– Apathy
– Depression.
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Severe anemia
• Impaired healing and loss of skin
elasticity
• Thinning and early greying of the hair
• Abdominal pain, nausea, vomiting,
anorexia
• Low-grade fever
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What Is the Diagnosis?
While working in the community you encounter
10-month-old Wendy, who is irritable and
appears weak for her age. Her mother states
she seems more tired than usual and does not
play as much as she used to. She has tried
giving Wendy a bottle of milk like she usually
does when she appears unhappy, but that does
not seem to pacify her. You ask more about
dietary habits and find that when Wendy's
mother stopped breastfeeding, she started
her on a cow's-milk bottle. She usually uses
this bottle to calm her and when it is time for
meals, Wendy typically does not eat very
much.
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o What do you think Wendy's diagnosis is?
o
o What nursing interventions would be
appropriate?
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Aplastic anemia
• is a condition wherein injury to or
abnormal expression of the stem cells in
the bone marrow results in the production
of inadequate numbers of erythrocytes,
leukocytes, and platelets.
• Can be hereditary or acquired.
• The hereditary form of aplastic anemia,
Fanconi's anemia (FA), is a rare
autosomal recessive disorder that
develops early in life and is accompanied
by multiple congenital abnormalities
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Aplastic anemia
• The age of onset of varies.
• Typically, the first sign noted is increased
bruising caused by the decreased number of
platelets.
• Vulnerability to infection because of the
decreased number of WBCs.
• When the RBCs have decreased in number, the
signs and symptoms of anemia begin to
manifest:
– pallor, weakness, and difficulty breathing
• Impaired growth and development
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Criteria for severe aplastic anemia
Severe Aplastic Anemia
• Neutrophils < 2 × 109 cells/L
• Platelets < 50 × 109 cells/L
• Reticulocytes < 0.5 %
• Bone Marrow Cellularity < 20 %
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Nursing Diagnosis
• Ineffective tissue perfusion related to
anemia
• Imbalanced nutrition: less than body
requirements related to inadequate iron
intake
• Deficient earegiver knowledge related to
age-appropriate iron intake
• Activity intolerance related to decreased
oxygen delivery to the tissues
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Goals:
1.
2.
3.
Minimize physical exertion and emotional stress:
–
Anticipate and assist in those activities of daily
living that may be beyond child’s tolerance
–
Provide play activities that promote rest and quiet
but prevent boredom and withdrawal
–
Encourage parents to remain with child, etc.
Increase oxygen to tissues:
–
Position for optimim air exchange
–
Administer supplemental oxygen if needed
Help replace blood elements:
–
Administer blood, packed cells, platelets as
prescribed
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Goals:
4.
Promote adequate intake of iron-rich foods:
–
Encourage exclusive breastfeeding of infants for 4-6
months after birth.
–
Diet rich with food sources of iron: meat, liver, fish, egg
yolks, spinach, oysters, peas, green leafy vegetables,
nuts, whole grains, iron-fortified infant cereal and dry
cereal
–
Feed milk as supplemental food in infant’s diet after solids
are begun
–
Administer iron preparations as prescribed:

Give between meals

Administer with fruit juice or multivitamin preparation

Do not give with milk or antacids

Give fluid preparations with dropper, syringe, or straw
to avoid contact with teeth

If the child is getting enough iron, stools should be a
tarry green color.
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Iron Supplements
• Although oral iron supplementation is generally
perceived as a safe treatment alternative, an
overdose can be lethal for children.
• Initial signs and symptoms of overdose:
– vomiting,
– abdominal pain,
– bloody diarrhea.
• These manifestations are typically followed by
shock, lethargy, and dyspnea. Often the child will
appear to improve before the onset of severe
metabolic acidosis, coma, and death.
• Urgent treatment is essential and includes
flushing out unabsorbed pills, administration of
Desferal, and supportive therapy
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Blood Transfusion
Nursing responsibilities
• Positively identify donor and recipient blood
types and groups before transfusion is begun;
verify with one other nurse or physician
• Transfuse blood slowly for first 15-20 min;
remain with patient
• In event of signs or symptoms, stop transfusion
immediately, maintain patent IV Line, notify
physician
• Insert urinary catheter to monitor hourly outputs
• Monitor for evidence of shock
• Save donor blood to re-crossmatch with patient’s
blood
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BLOOD TRANSFUSION
COMPLICATIONS
• Hemolytic reactions
• Febrile reactions
• Allergic reactions
• Circulatory overload
– too rapid transfusion or excessive quantity of blood
transfused
• Air emboli
– May occur when blood is transfused under pressure
• Hypothermia:
– Allow blood to warm at room temperature (1hr)
• Hyperkalemia
– In massive transfusion or patient with renal problems
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SICKLE CELL ANEMIA
• A hereditary disorder in which normal
adult hemoglobin (HbA) is partly or
completely replaced by an abnormal
hemoglobin (HbS) causing distorsion and
rigidity of red blood cells under condition
of reduced oxygen tension.
• Sickle cell anemia is part of a group of
diseases called hemoglobinopathies.
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SICKLE CELL ANEMIA
• Sickle cell anemia is an autosomalrecessive disorder. The inheritance is
described as intermediate because the
gene is partially expressed in the
heterozygous state and completely
expressed in the homozygous state.
• Sickle cell anemia is found primarily in the
black race, although infrequently it affects
whites, especially those of Mediterranean
descent.
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SICKLE CELL ANEMIA –
Manifestations:
• Take health history, especially regarding any evidence of
sickling crisis and history of the disease in family
members.
• General:
– Growth retardation
– Chronic anemia
– Delayed sexual maturation
– Marked susceptibility to sepsis
• Other signs and symptoms.
– weakness; anorexia; joint, back, and abdominal pain;
fever; and vomiting.
– Chronic leg ulcers are common in adolescents and
adults and are thought to be the result of thrombosis
and decreased peripheral circulation.
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SICKLE CELL ANEMIA –
Vaso-occlusive (sickling) crisis
• Pain in area(s) of involvement
• Signs of ischemia:
– Extremities: painful swelling (“hand-foot” syndrome),
painful joints
– Abdomen: severe pain resembling acute surgical
condition
– Cerebrum: stroke, visual disturbances
– Chest: pseudopneumonia, propracted episodes of
pulmonary disease
– Liver: obstructive jaundice, hepatic coma
– Kidney: hematuria
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Sequestration Crisis
• Pooling of large amounts of blood:
– Hepatomegaly
– Splenomegaly
– Circulatory collapse
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Diagnostic evaluation
• For screening purposes:
– the Sickledex is commonly used. If the test
is positive, hemoglobin electrophoresis is
necessary to distinguish between those
children with the trait and those with the
disease.
• Sickling test (sickle cell slide preparation):
place a drop of blood on a slide and cover it with
a sealed cover slip to produce deox-ygenation.
Eventually sickling of the red blood cell occurs.
• Hemoglobin electrophoresis
("fingerprinting"). This test is accurate, rapid,
and specific.
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Nursing Diagnosis
• Risk for injury R/T abnormal Hb,
decreased ambient oxygen, dehydration
• Altered tissue perfusion R/T generalized
sickling
• Pain R/T tissue anoxia
• Altered family process R/T to a child with
potentially life-threatening disease
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Nursing management
Goals:
• Inc tissue oxygenation and prevent sickling:
– Family teach.: avoid strenuous physical
exertion, emotional stress, O2 envir
– Promote hydration
– Prevent infection
– ↑ tissue oxygenation: adm O2, promote
circulation through passive range of motion
exercises
– Relieve pain
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The end.
Q&A?
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