C h a p ter 4 4

Nursing Care of a Family

When a Child Has a
Hematologic Disorder

KEY TERMS
• allogeneic transplantation • megakaryocytes
• autologous transplantation • pancytopenia
• blood dyscrasias • petechiae
• erythroblasts • plethora
• erythrocytes • poikilocytic
• erythropoietin
• granulocytes
• priapism
• purpura  ana is a 4-year-old girl diagnosed
• hemochromatosis • reticulocyte with thalassemia major whom you meet
• hemolysis • syngeneic transplantation
at a pediatric clinic. She has a prominent
• hemosiderosis • thrombocytes
• leukocytes • thrombocytopenia mandible and wide-spaced upper front
teeth from overgrowth of bone marrow
centers. Joey is a 7-year-old with sickle-
cell anemia who attends the same clinic.
His growth is only in the fifth percentile,
OBJECTIVES and he’s had two vaso-occlusive crises in
After mastering the contents of this chapter, you should be able to:
the past year. “Why did this happen to our
1. Describe the major hematologic disorders of childhood.
families?” Lana’s mother asks you. “What
2. Identify 2020 National Health Goals related to children with
hematologic disorders that nurses could help the nation achieve. can we do to help our children have better
3. Assess a child with a hematologic disorder such as sickle-cell anemia.
lives?”
4. Formulate nursing diagnoses related to a child with a hematologic
disorder. Previous chapters described the growth
5. Identify expected outcomes for a child with a hematologic disorder to and development of well children. This
help parents manage seamless transitions across differing health care
settings. chapter adds information about the
6. Using the nursing process, plan nursing care that includes the six dramatic changes, both physical and
competencies of Quality & Safety Education for Nurses (QSEN):
Patient-Centered Care, Teamwork & Collaboration, Evidence-Based psychosocial, that occur when children have
Practice (EBP), Quality Improvement (QI), Safety, and Informatics. a hematologic disorder.
7. Implement nursing care related to a child with a hematologic disorder,
such as reducing the possibility of infection.
8. Evaluate expected outcomes for achievement and effectiveness What additional health teaching does
of care. Lana’s mother need so she can better
9. Integrate knowledge of childhood disorders of the blood with understand hematologic diseases?
the interplay of nursing process, the six competencies of QSEN,
and Family Nursing to promote quality maternal and child health
nursing care.

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 CHAPTER 44 Nursing Care of a Family When a Child Has a Hematologic Disorder
The blood and blood-forming tissues that make up the hema-
tologic system play a vital role in body metabolism because
they transport oxygen and nutrients to body cells, remove
carbon dioxide from cells, and initiate blood coagulation
when vessels are injured. As a result of all of these functions,
any alteration in the substance or function of blood can have
immediate and life-threatening effects on the functioning of
all body systems (Zempsky, Palermo, Corsi, et al., 2013).
Hematologic disorders, often called blood dyscrasias,
occur when components of the blood are formed incorrectly
or either increase or decrease in amount beyond normal
ranges. Most blood dyscrasias in children originate in the bone
marrow, where blood cells are formed. They do not occur at
equal rates in all countries, because many of these disorders
are inherited. Sickle-cell anemia, for example, occurs mainly
in African Americans; thalassemia occurs in children of Med-
iterranean heritage. Being aware of the differences in the inci-
dence of blood dyscrasias this way can be helpful in planning
care and providing health care services for children and com-
munities. Treatment for blood disorders vary as well based on
cultural influences. Families who are Jehovah’s Witnesses, for
example may refuse blood transfusions, a common therapy
for blood disorders, on religious grounds (Kitney, Kanani,
& De Souza, 2012). Box 44.1 shows 2020 National Health
Goals related to blood disorders.
BOX 44.1 Nursing Care Planning
Based on 2020 National Health Goals
2020 National Health Goals speak to ways to improve
children’s health. Because both iron-deficiency and
sickle-cell anemia are seen worldwide, improving care
in these areas could have a dramatic effect on both
national and world health.
• Reduce the incidence of iron deficiency among
children aged 1 to 2 years from a baseline of 15.9% to
a target level of 14.3%; in children aged 3 to 4 years,
from 5.3% to 4.3%.
• Reduce the incidence of iron deficiency among ado-
lescents 12 to 18 years of age from 10.4% to 9.4%.
• Reduce the proportion of persons with hemophilia
who develop reduced joint mobility due to bleeding
into joints from 82.9% to 74.6%.
• (Developmental) Reduce hospitalization due to pre-
ventable complications of sickle-cell disease yearly
among children aged 9 years and under.
• Increase the proportion of children with special health
care needs who have access to a medical home from
47.1% to 51.8% (U.S. Department of Health and Human
Services [DHHS], 2010; see www.healthypeople.gov).
Nurses can help the nation achieve these goals by edu-
cating parents about the importance of women taking
an iron supplement during pregnancy, encouraging
iron-rich food sources for young children, and educat-
ing adolescents about healthy diets. Being certain that
parents are well informed about preventive measures for
children with all types of hematologic disorders could
help reduce hospital admissions.
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1283
Nursing P rocess Ov erv iew
For a Child With a Hematologic Disorder
Assessment
Many of the symptoms of hematologic disorders begin
insidiously, with symptoms such as pallor, lethargy, and
bruising. These are such minor symptoms that parents
may not bring their child to a health care facility for
some time. When they do, they are surprised to learn
such subtle symptoms signify the presence of a serious
illness.
Children with iron deficiency, for example, aside from
appearing pale and irritable, look plump and “healthy.”
It takes careful history taking to reveal the possibility of
an iron deficiency.
Nursing Diagnosis
When a child is diagnosed with an inherited disorder,
parents may feel guilty or blame themselves or their
partner for their child’s disease. This can make it diffi-
cult for a family to act together to support a child during
an illness when members need intensive support them-
selves. Examples of nursing diagnoses that address the
entire family include:
• Deficient knowledge related to the cause of the child’s
illness
• Imbalanced nutrition, less than body requirements,
related to family pattern of not eating iron-rich foods
• Anxiety related to frequent blood-sampling procedures
• Pain related to tissue ischemia
• Compromised family coping related to long-term care
needs of child with a chronic hematologic disorder
Outcome Identification and Planning
When helping parents plan outcomes, be certain that
the outcomes planned are realistic for both the child and
family. It may not be possible to reduce the number of
blood-sampling procedures, for example, but a child can
be helped, with distraction techniques, to deal with the
pain and anxiety that the procedures produce.
Children with hematologic disorders often are pre-
scribed a long-term medication such as a corticoste-
roid. When a child appears very ill, parents are usually
very conscientious about giving such medicine. When a
child has a disorder with few symptoms, however, like a
blood dyscrasia, it is easy for parents to forget to give the
medication. In addition, a child may refuse to take the
medication for a long time because it tastes bad or upsets
the stomach. Planning, therefore, includes helping par-
ents devise ways to disguise the taste or remember to give
medication over the long term. If a child will be restricted
in activity for long periods because the immune system is
compromised as a part of the illness, planning must in-
clude ways to keep the child engaged with friends to pro-
mote development. Parents may need help investigating
possible resources for education and support to do this.
Some organizations helpful for referral are the Aplastic
Anemia & MDS International Foundation (www.aplastic
.org), the Sickle-cell Disease Association of America (www
.sicklecelldisease.org), the American Society of Pediatric
Hematology and Oncology (www.aspho.org), and the
National Hemophilia Foundation (www.hemophilia.org).
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 1284 UNIT 7 The Nursing Role in Restoring and Maintaining the Health of Children With Physiologic Disorders
Implementation
Nursing interventions for children with hematologic
disorders range from helping to obtain blood speci-
mens for testing to assisting with blood or hemato-
poietic stem cell transfusions. Remember that a finger
puncture for blood is often as painful as a venipunc-
ture (and more painful afterward because the fingertip
hurts when the child attempts to use it). Suggesting
that blood be drawn by means of an intermittent de-
vice and applying an anesthetic cream (mixtures of
lidocaine and prilocaine) before finger punctures or
venipunctures are effective measures to help reduce
pain and improve cooperation with these procedures
and so are important to initiate. Even so, children may
need some therapeutic playtime with a syringe and a
doll to express their anger about constant invasion by
needles.
Outcome Evaluation
An evaluation focuses on whether short-term outcomes
such as moderation of pain or elimination of anxiety in
a child undergoing diagnosis or treatment were achieved
and that progress is being made toward the achievement
of long-term outcomes such as improving the ability of
the family to manage the stress of raising a child with
a chronic illness or dealing with frequently occurring
health crises.
Examples of expected outcomes that suggest goals
were achieved include:
• Parents correctly state the most frequent causes of iron-
deficiency anemia.
• Child states she feels better able to cope with blood-
sampling procedures through the use of imagery.
• Parents describe realistic plans to ensure adherence to
long-term medication administration.
• Parents voice that they understand the importance of
preventing dehydration in their school-age child with
sickle-cell anemia.
ANATOMY AND PHYSIOLOGY OF
THE HEMATOPOIETIC SYSTEM
Blood components originate in the bone marrow, circulate
through blood vessels, and ultimately are destroyed by the
spleen.
Blood Formation and Components
The total volume of blood in the human body is roughly
proportional to body weight: 85 ml/kg at birth, 75 ml/kg at
6 months of age, and 70 ml/kg after the first year. Although
the blood plasma is important in diseases that cause vomiting
and diarrhea (when this fluid may become depleted, leading
to dehydration), plasma is not a major site of hematologic
disease. The formed elements—the erythrocytes (red blood
cells [RBCs]), leukocytes (white blood cells [WBCs]), and
thrombocytes (platelets)—are the portions most affected by
hematologic disorders in children.
PILLITTERI_E7_CH44.indd 1284
Erythrocytes (Red Blood Cells)
Erythrocytes (RBCs) function chiefly to transport oxygen
to and carry carbon dioxide away from body cells. They
are formed in the bone marrow under the stimulation of
erythropoietin, a hormone formed by the kidneys that is
produced whenever a child has tissue hypoxia. Children
with kidney disease often have a low number of RBCs be-
cause erythropoietin secretion is inadequate in diseased
kidneys. Polycythemia, or an overproduction of RBCs, can
occur in children who experience prolonged systemic hy-
poxia because of erythropoietin overproduction (Rote &
McCance, 2012).
At birth, an infant has approximately 5 million RBCs per
cubic millimeter of blood. This concentration diminishes
rapidly in the first months, reaching a low of approximately
4.1 million/mm3 at 3 to 4 months of age. The number then
slowly increases until adolescence, when the adult value of
approximately 4.9 million/mm3 is reached.
RBCs form first as erythroblasts (large, nucleated cells),
then mature through normoblast and reticulocyte stages, to
mature, nonnucleated erythrocytes. An elevated reticulocyte
count (more than 1% of the total count) indicates that rapid
production of new RBCs is occurring. At the end of their life
span (about 120 days), erythrocytes are destroyed through
phagocytosis by reticuloendothelial cells, found in the highest
proportion in the spleen.
Hemoglobin. The component of RBCs that allows them to
carry out the transport of oxygen is hemoglobin, composed of
globin, a protein, and heme, an iron-containing pigment. It
is the heme portion that combines with oxygen and carbon
dioxide for transport.
The hemoglobin in erythrocytes during fetal life differs
from that formed after birth. Fetal hemoglobin is composed
of two ␣ and two ␥ polypeptide chains. At birth, 40% to
70% of the infant’s hemoglobin is this type (hemoglobin F).
During the first 6 months of life, this is gradually replaced
by adult hemoglobin (hemoglobin A), which is composed of
two ␣ and two ␤ chains. For this reason, diseases such as
sickle-cell anemia or the thalassemias, which are disorders of
the ␤ chains, do not become apparent clinically until this he-
moglobin change has occurred (at approximately 6 months of
age). Because some hemoglobin A is present, however, even
in early intrauterine life, they can be diagnosed prenatally by
hemoglobin analysis or electrophoresis in fetal or newborn
life (Kline, 2012).
Hemoglobin levels are highest at birth (13.7 to 20.1 g/
100 ml); they reach a low at approximately 3 months of age
(9.5 to 14.5 g/100 ml), and then gradually rise again until
adult values are reached at puberty (11 to 16 g/100 ml).
Bilirubin. After an RBC reaches its life span of approximately
120 days, it disintegrates and its protein component is pre-
served by the reticuloendothelial cells of the liver and spleen
for further use. Iron is reused by the bone marrow to con-
struct new RBCs. As the heme portion is degraded, it is con-
verted into protoporphyrin; protoporphyrin is then further
broken down into indirect bilirubin. Indirect bilirubin is fat
soluble and so cannot be excreted by the kidneys. It is there-
fore converted by the liver enzyme glucuronyl transferase into
direct bilirubin, which is water soluble and excreted in bile.
In the newborn, generally liver function is so imma-
ture that the conversion from indirect to direct bilirubin is
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 CHAPTER 44 Nursing Care of a Family When a Child Has a Hematologic Disorder
difficult, allowing a portion of bilirubin to remain in the in-
direct form. When the level of indirect bilirubin in the blood
rises to more than 7 mg/100 ml, it permeates outside the
circulatory system, and the infant begins to show signs of yel-
lowing or jaundice from the color of bilirubin. At any point
in life, if excessive hemolysis (destruction) of RBCs occurs
from other than usual causes, a child will also show signs of
jaundice.
Leukocytes (White Blood Cells)
Leukocytes (WBCs) are nucleated cells and few in number
compared with RBCs (there is approximately only 1 WBC to
every 500 RBCs). Their primary function is defense against
antigen invasion; their life span varies from approximately 6
hours to unknown intervals. With the exception of neutrope-
nia (a reduced number of WBCs), leukocytes are not major
hematologic concerns; because they are important in im-
mune disorders and malignancies, they are further discussed
in Chapters 42 and 53.
Thrombocytes (Platelets)
Thrombocytes are round, nonnucleated bodies formed
by the bone marrow; their function is capillary hemostasis
and primary coagulation. The usual number is 150,000 to
300,000/mm3 after the first year. Immature thrombocytes are
termed megakaryocytes. If large numbers of these are pres-
ent in serum, it indicates that a rapid production of platelets
is occurring.
Blood Coagulation
Effective blood coagulation depends on a complex series of
four events, including a combination of blood and tissue
factors released from the plasma (the intrinsic pathway)
and from injured tissue (the extrinsic pathway) (Schwartz,
Rote, & McCance, 2012). The numbers of coagulation fac-
tors refer to the order in which factors were discovered, not to
the order of action in coagulation. The plasma-released fac-
tors include factors VIII, IX, and XII. Factors released from
injured tissues are a tissue factor (an incomplete thrombo-
plastin or factor III), plus factors VII and X. Together, these
pathways unite to form factor V.
When a vessel is injured, vasoconstriction occurs in the
area proximal to the injury, thus narrowing the vessel lumen
and reducing the amount of blood that can flow to the in-
jured area. Platelets begin to adhere to the damaged vessel site
and to one another, forming a platelet plug and initiating the
first stage of clotting (Fig. 44.1).
In the second stage, factors from either the intrinsic or
the extrinsic system combine with platelet phospholipids to
form complete thromboplastin. In the third stage, throm-
boplastin converts prothrombin (factor II) to thrombin if
ionized calcium is present. The production of prothrombin
and factors VII, IX, and X all depend on the presence of
vitamin K. This stage will be incomplete, therefore, if levels
of any of factors VIII through XII, vitamin K, or calcium
are deficient.
In the fourth stage, thrombin converts fibrinogen (factor
I) to fibrin. Fibrin strands form a mesh incorporating RBCs,
WBCs, and platelets to form a permanent protective seal at
the site of injury. Factor XIII (fibrin stabilizing factor) then
acts to make the fibrin clot insoluble and permanent.
PILLITTERI_E7_CH44.indd 1285
1285
(Coagulation
Stage 1 Platelets adhere
factors
to each other
involved)
Stage 2 Platelet Complete VIII
phospholipid thromboplastin (III) IX
X
XI
XII
IV
Stage 3 Prothrombin (II) Thrombin IV
V
VII
X
Stage 4 Fibrinogen (I) Fibrin XIII
FIGURE 44.1 The steps in blood coagulation.
To prevent too much coagulation after the seal is complete,
plasminogen is then converted to plasmin (a fibrinolysin)
near the injury to halt the clotting sequence. Common tests
for blood coagulation are described in Table 44.1.
ASSESSMENT OF AND
THERAPEUTIC TECHNIQUES FOR
HEMATOLOGIC DISORDERS
An assessment of children with hematologic disorders begins
with a history to identify inherited disorders. For a specific
diagnosis, children generally require several diagnostic proce-
dures such as blood cell or bone marrow analysis.
Bone Marrow Aspiration and Biopsy
Bone marrow aspiration provides samples of bone marrow so
the type and quantity of cells being produced can be deter-
mined (Panepinto & Scott, 2011). In children, the aspiration
sites used are the iliac crests or spines (rather than the ster-
num, which is commonly used in adults) (Fig. 44.2) because
performing the test at these sites is usually less frightening for
children. These sites also have the largest marrow compart-
ments during childhood. In neonates, the anterior tibia can
be used as an additional site.
For a bone marrow aspiration, following conscious seda-
tion, a child lies prone on a hard surface such as a treatment
table because pressure is needed to insert the needle through
the surface of the bone into the marrow compartment. Topi-
cal anesthesia may be applied to help reduce pain (Hjortholm,
Jaddini, Hałaburda, et al., 2013).
The area of the aspiration is cleaned with an antiseptic
solution and draped. The overlying skin is infiltrated with
a local anesthetic and then a large-bore needle and stylus is
introduced through the overlying tissue into the bone. When
the marrow cavity is reached, the stylus is removed, a sy-
ringe is attached to the needle, and bone marrow is aspirated
(which appears as thick blood in the syringe). The syringe is
then removed, and the marrow obtained is expelled onto a
slide, sprayed with a preservative, and taken to a laboratory
for analysis. Pressure must be applied to the puncture site im-
mediately afterward to prevent bleeding. A pressure dressing
is then applied to maintain pressure and to continue to halt
bleeding.
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 1286 UNIT 7 The Nursing Role in Restoring and Maintaining the Health of Children With Physiologic Disorders

TABLE 44.1 Common Tests for Blood Coagulation

Test Definition Normal Value

Prothrombin time (PT) Measures the action of prothrombin; reveals deficiencies 11–13 s (PT) or 2.0–3.0 International
in prothrombin, factors V, VII, and X. Normalized Ratio (INR)

Partial thromboplastin time Measures activity of thromboplastin; reveals deficiencies 30–45 s

(PTT) in thromboplastin, factors VIII–XII.

Bleeding time Measures the time required for bleeding at a stab 3–10 min
wound on the earlobe to stop; reveals deficiencies in
platelet formation and vasoconstrictive ability.

Clot retraction Measures platelet function; interval from placement of Retraction at side of test tube
blood in a tube to the point clot shrinks and expels should be present by 1 hr;
serum. complete in 24 hr

Tourniquet Measures capillary fragility and platelet function; 0–2 petechiae per 2-cm area
response of tissue to application of tourniquet to
forearm for 5–10 min.

Prothrombin consumption Evaluates thromboplastin function; if clot formation used Approximately 20 s

time a great deal of prothrombin (as it should), serum
prothrombin time will be brief; prolongation denotes
defects in thromboplastin function.

Thromboplastin generation Tests basic ability to form thromboplastin; distinguishes 12 s or less

time factor VIII from factor IX disorders.

Plasma fibrinogen Measures stage 4 clotting process or level of fibrinogen 200–400 mg/100 ml plasma
in blood.

Venous clotting time (Lee- Measures factor deficits in stages 2 and 4. 9–12 min
White clotting time)

A child will feel pain from the local anesthetic injection
and hard pressure while the needle is inserted. Some report
a sharp pain when the marrow is actually aspirated. If con-
scious sedation is used, monitor vital signs until the child is
fully awake. Monitor pulse and blood pressure and observe
the dressing every 15 minutes for the first hour after the
FIGURE 44.2 A common site used for bone marrow aspira-
tion in children is the iliac crest. In neonates, the anterior tibia
may be used.
procedure to be certain no bleeding is occurring. Keep the
child fairly quiet for the first hour by playing a quiet game
or other activity. Because bone marrow aspiration is a painful
and invasive procedure, allow young children an opportunity
for therapeutic play with a doll and syringe to help them ex-
press their feelings about the procedure. If the procedure was
done as an ambulatory one, instruct parents to take the child’s
temperature 12 and 24 hours after the procedure to detect
infection.
Blood Transfusion
Transfusions of blood or its products are commonly used
in the treatment of blood disorders, and may include whole
blood, packed RBCs, washed RBCs (as much “foreign” matter
is removed as possible to reduce the possibility of an antago-
nistic reaction), plasma, plasma factors, platelets, WBCs, and
albumin. No matter what the blood product, it’s important
that it has been carefully matched with the child’s blood type
and is infused with a solution as nearly isotonic as possible
(normal saline). If blood should be given with a hypertonic
solution, this will cause fluid to be drawn out of the transfused
RBCs, causing them to shrink and be useless; if blood is in-
fused with a hypotonic solution, fluid will be drawn into the
cells, causing them to burst, and again, be destroyed.
Packed RBCs are the most common form of transfusion
used with children because they help minimize the risk of
fluid overload. The usual amount of blood transfused is typi-
cally 15 ml/kg of body weight. The commonly accepted rate

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 CHAPTER 44 Nursing Care of a Family When a Child Has a Hematologic Disorder 1287

for transfusions in a child is 10 ml/kg/hr unless the child
has hypovolemic shock and volume equilibrium needs to be
established quickly. An infusion of packed RBCs at a pro-
portion of 15 ml/kg will raise the hematocrit level 5 points.
A transfusion of platelets will elevate the platelet count by
approximately 10,000 cells. Platelets last only approximately
10 days, however, so transfusions must be repeated every
10 days to maintain a functioning platelet level (Panepinto
& Scott, 2011).
Even if given slowly, a blood transfusion is always a strain
on a child’s circulation beyond that of a regular intravenous
infusion because the circulatory system must accommodate
such a thick, difficult-to-mobilize fluid.
Before any transfusion, ensure a signed consent form is
obtained that respects sociocultural or religious beliefs. Ob-
tain vital signs to establish a baseline and monitor these about
every 15 minutes during the first hour and about every half
hour for the remainder of the transfusion. Keep the infusion
rate slow for the first 15 minutes; then, if no reaction occurs,
increase the rate to about 10 ml/kg/hr or as otherwise pre-
scribed. Common symptoms of blood transfusion reactions
that may occur are shown in Table 44.2.
To prevent children from becoming bored or attempting
to increase the infusion rate to speed up the process, think
of and provide an enjoyable activity for children during
transfusions.
Hematopoietic Stem Cell Transplantation
Stem cell transplantation is the intravenous infusion of hema-
topoietic stem cells from bone marrow obtained by marrow
aspiration or from peripheral or umbilical cord blood drawn
from a compatible donor to reestablish marrow function in a
child with deficient or nonfunctioning bone marrow.
Stem cell transplantation has become a relatively com-
mon procedure for children with blood disorders such as
acquired aplastic anemia, sickle-cell disease, thalassemia,
leukemia, and some forms of immune dysfunction diseases.

TABLE 44.2 Common Symptoms of Blood Transfusion Reactions

Symptoms Cause Time of Occurrence Nursing Interventions

Headache, chills, back Anaphylactic reaction to incom-
pain, dyspnea, hypo- patible blood; agglutination
tension, hemoglobin- of red blood cells occurs;
uria (blood in urine) kidney tubules may become
blocked, resulting in kidney
failure
Immediately after start of
transfusion.
Discontinue transfusion. Maintain nor-
mal saline infusion for accessible
intravenous (IV) line.
Administer oxygen as necessary.
Anticipate administration of a diuretic
to increase renal tubule flow and
reduce tubule plugging and/or
heparin to reduce IV coagulation.

Pruritus, urticaria (hives), Allergy to protein components Within first hour after start Discontinue transfusion temporarily.
wheezing of transfusion of transfusion Give oxygen as needed.
Anticipate administration for antihis-
tamine to reduce symptoms.

Increased temperature Possible contaminant in trans- Approximately 1 hr Discontinue transfusion. Obtain blood
fused blood after start of culture to rule out or identify
transfusion bacterial invasion.

Increased pulse, dyspnea
Circulatory overload
During course of Discontinue transfusion; give oxygen
transfusion as needed.
Provide supportive care for pulmonary
edema or congestive heart failure,
which may develop. Anticipate ad-
ministration of diuretic to increase
excretion of excess fluid.

Muscle cramping, twitch- Acid-citrate-dextrose anticoagu- During course of Discontinue transfusion.

ing of extremities, lant in transfusion combines transfusion Anticipate administration of calcium
seizure with serum calcium and gluconate intravenously to restore
causes hypocalcemia calcium level.

Fever, jaundice, lethargy, Hepatitis from contaminated Weeks or months after Obtain transfusion history of any child
tenderness over liver transfusion transfusion with hepatitis symptoms. Refer for
care of hepatitis.

Bronze-colored skin
Hemosiderosis or deposition of
iron in skin from transfusion
After repeated Support self-esteem with altered
transfusions body image.
Administer iron-chelating agent (de-
feroxamine) as prescribed to help
reduce level of accumulating iron.

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 1288 UNIT 7 The Nursing Role in Restoring and Maintaining the Health of Children With Physiologic Disorders
Although a stressful procedure to undertake, it offers
children the opportunity for a complete reversal of symp-
toms. However, there is no guarantee that the grafted cells
will be accepted by the recipient or that improvement will
occur, but with good tissue compatibility in the absence of
infection, it can be effective in most children (Thompson,
Ceja, & Yang, 2012).
Stem cell transplantation can be allogeneic, syngeneic, or
autologous. Allogeneic transplantation is the transfer of
stem cells from an immune-compatible (histocompatible)
donor, usually a sibling, or from a national cord blood bank
or national volunteer donor registry (Petrini, 2013). Synge-
neic transplantation (which is rare) involves a donor and
recipient who are genetically identical (i.e., identical twins).
Autologous transplantation involves use of the child’s own
stem cells removed from cord blood banked at the time of
the child’s birth. If this is not available, in some instances,
stem cells can be aspirated from the child’s bone marrow or
obtained from circulating blood, treated to remove abnormal
cells, and then reinfused.
Hematopoietic stem cells are recovered from a donor’s cir-
culating peripheral blood after the stimulation of stem cell
production by a cytokine or stem cell colony-stimulating
factor. Success is most likely if the recipient has not already
received multiple blood transfusions that have sensitized the
child to blood products and the donated stem cells are a close
human leukocyte antigen (HLA) match to the child’s blood.
Siblings have about a 25% chance of being HLA compatible
with the ill child.
To prevent a child’s T lymphocytes from rejecting the
newly transplanted donor stem cells, total body irradiation
to destroy the child’s marrow or an immunosuppressive drug
such as cyclophosphamide (Cytoxan) is administered intra-
venously to the child before the procedure. This is a difficult
time for the child because, even with antiemetic therapy, both
total body irradiation and the immunosuppressive drug cause
extreme nausea, vomiting, and diarrhea.
If the marrow will be taken directly from a donor rather
than from peripheral blood, on the day of the procedure, the
donor is admitted to the hospital for a 1-day stay and receives
epidural anesthesia or conscious sedation because multiple
bone marrow aspirations from the posterior iliac crests are
necessary for retrieval. The marrow is strained to remove fat
and bone particles and any other unwanted cells. An anti-
coagulant is added to prevent clotting and it is then infused
intravenously into the recipient’s bloodstream.
Because an infused hematopoietic stem cell solution is
fairly thick, the infusion takes 60 to 90 minutes. Do not use
the filter that is normally used for the infusion of blood prod-
ucts, because this would filter out marrow tissue. Monitor the
child’s cardiac rate and rhythm during the infusion to detect
circulatory overload or pulmonary emboli from unfiltered
particles.
Fever and chills are common reactions to a hematopoietic
stem cell transplant infusion. Acetaminophen (Tylenol) or di-
phenhydramine hydrochloride (Benadryl) may be prescribed
to reduce this reaction. After the infusion, take the child’s
temperature at 1 hour and then about every 4 hours to detect
an infection that could occur because the child’s WBCs are
nonfunctional from radiation or immunosuppression. Rein-
force strict hand washing and limit the child’s diet to cooked
foods to reduce exposure to bacteria.
PILLITTERI_E7_CH44.indd 1288
Almost immediately after the infusion, stem cells begin
to migrate from the child’s bloodstream into the marrow. If
engraftment occurs (the transplant is accepted), new RBCs
can be detected in the peripheral blood in approximately
3 weeks. The WBC count will be measured daily to be certain
WBCs are regenerating, although WBCs and platelet cells
may not return to normal for up to 1 year after the transplant.
Bone marrow aspirations or venous blood samples are then
scheduled at regular intervals over the next year to assess the
growth of the new marrow.
What if...44.1 Lana’s 12-year-old sister
donates hematopoietic stem cells to Lana, but
the transplant is not successful. The sister tells you she
knew it wouldn’t be successful because she and Lana
are more rivals than compatible sisters. Could sibling
rivalry this way have made a difference in the success of
the procedure?
Nursing Diagnoses and
Related Interventions
Because it takes a long time for the success or
failure of stem cell transplantation to be docu-
mented, the procedure almost always produces
anxiety not just in the child but also in the donor
and the entire family.
Nursing Diagnosis: Anxiety related to long period of
waiting to receive results of hematopoietic stem cell
transplant and necessary extended restrictions and
infection control precautions in hospital or at home.
Outcome Evaluation: Parents state they are manag-
ing their level of anxiety, are carrying out infection
restrictions as prescribed, and are expressing satisfac-
tion with child’s ongoing development.
Be certain when discussing the risks of a stem cell
transplant that both the child who received the trans-
plant and the donor understand they are not respon-
sible for the outcome of the procedure because its
success does not depend on their behavior or what
kind of person they are but on immunologic factors
over which they have no control.
Because not all hematopoietic stem cell trans-
plants are successful, some children will die of the
original disease that necessitated the transplant. And,
even if the transplant was successful, another risk is
that a child will develop an infection despite all pre-
cautions and die of sepsis in the weeks immediately
after the transplant.
To prevent the child from contracting an infection
until the WBC count returns to a safe range, children
are restricted from interacting with other children
either by remaining in the hospital or by employing
visiting restrictions at home. Be certain to visit the
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 CHAPTER 44 Nursing Care of a Family When a Child Has a Hematologic Disorder
room of an isolated child frequently and provide
sterilized play materials the child can enjoy as ap-
propriate. Because raw vegetables and fruits have
the potential to carry germs, thick-skinned fruits such
as bananas and oranges can be given soon after the
procedure, but unwashed foods are typically avoided
because these are foods most likely to carry bacteria.
Be certain children are well prepared for all pro-
cedures. Allow them to make as many choices as
they can about their care to help them preserve a
sense of control over their lives. Encourage periods of
therapeutic play into their care so they can begin to
express their anger and frustration at the number of
intravenous therapies or follow-up bone marrow as-
pirations they require. Frequent visits by their parents
and measures to help the child cope with pain, such
as imagery, can help them accept one more painful
procedure.
Ask the parents if they have made provisions for
schoolwork as soon as the child has a return of RBCs
in the peripheral blood (about 3 weeks). Help them
locate a support group in their community if they feel
this will be helpful. Be certain they feel free to call
the transplant center after discharge to discuss any
problems (Cooke, Grant, & Gemmill, 2012). Once
the danger of infection has passed and the restric-
tions can be discontinued, some parents may still be
reluctant to allow their child outside or to interact
with other children. This makes frequent follow-ups
by e-mail or texting for the next year necessary to not
only ensure that the child is free of infection but also
to assess whether the parents allow their child to pur-
sue age-appropriate activities to encourage growth
and development.
Graft-Versus-Host Disease
Graft-versus-host disease (GVHD) is a potentially lethal im-
munologic response of donor T cells to the tissue of the bone
marrow recipient (Alousi, Bolaños-Meade, & Lee, 2013).
The symptoms range from mild to severe and generally in-
clude a rash and general malaise beginning 7 to 14 days after
the transplant. Severe symptoms include high fever and diar-
rhea and liver and spleen enlargement.
Because there is no known cure for GVHD, prevention
is essential. Careful tissue typing, intravenous administra-
tion of a corticosteroid and an immunosuppressant before
transplant, and irradiation of blood products (which helps to
inactivate mature T lymphocytes) before the infusion all can
help reduce the incidence of this complication. Immunosup-
pressant drugs such as methotrexate or cyclosporine work by
killing all rapidly growing cells, including WBCs and T lym-
phocytes, so administration of these drugs after transplanta-
tion cannot be continued or they would also interfere with
the growth of the host’s new stem cells. Administration of
corticosteroids or antithymocyte globulin (ATG), an immune
serum, can be continued and may decrease the severity of
GVHD; be certain parents understand the importance of this
so, if prescribed, they give them conscientiously (Theurich,
Fischmann, Shimabukuro-Vornhagen, et al., 2012).
PILLITTERI_E7_CH44.indd 1289
1289
✔ QSEN Checkpoint Question 44.1
Patient-Centered Care
Lana, who has thalassemia major, is scheduled for a bone
marrow transplant, and her mother is highly anxious about
this upcoming procedure. Which of the following statements
is most accurate and best exemplifies patient-centered care?
a. “If you can hold her still during the procedure, the pain will
pass more quickly for her.”
b. “We will go to great lengths to make sure Lana doesn’t
develop an infection.”
c. “Lana will need to lie still while the new bone marrow
infuses into her bones.”
d. “She will not need any further bone marrow aspirations
after this.”
Look in Appendix A for the best answer and rationale.
Splenectomy
One of the purposes of the spleen is to remove damaged or
aged blood cells. This poses a problem with diseases such as
sickle-cell anemia and the thalassemias because the spleen
interprets the typical cells of these diseases as damaged and
destroys them. This causes children with these disorders to
have a continuous anemia, with hemoglobin levels as low as 5
to 9 g/ml. In some children, therefore, removal of the spleen
(splenectomy) will not cure the basic defect of the blood cells
but will limit the degree of anemia. Splenectomy formerly
required a large abdominal incision but today it can be per-
formed by laparoscopy so, although still a procedure with
risks, it does not require as long a recovery period (Deng,
Maharjan, Tang, et al., 2012).
A second function of the spleen is to strain blood particles
that might lead to blood clots as well as invading microor-
ganisms from the blood plasma so phagocytes and lympho-
cytes can destroy them. This causes children who have had
their spleen removed to be very susceptible to both throm-
bophlebitis and pneumococcal infections because these sub-
stances are no longer removed systematically from the body
(Rodeghiero & Ruggeri, 2012). After surgery, oral penicillin
is typically given as a prophylactic antibiotic for a year or two
to guard against infection. Assess to be certain the child also
receives Haemophilus influenzae type b (Hib) and pneumo-
coccal and meningococcal vaccines for further protection.
Review with parents the signs of infection (e.g., cough, fever,
general malaise), and encourage them to report any such
signs immediately to their primary care provider.
HEALTH PROMOTION AND
RISK MANAGEMENT
Hematologic disorders cover a wide range of diseases, both
inherited and as an immunosuppressive response to in-
fection, so they produce multiple symptoms in children
(Box 44.2). Health promotion and disease prevention,
therefore, begins with ensuring families have access to ge-
netic counseling so they can be aware of the incidence of a
disorder in their family and the potential for the disease to
develop in their child.
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 1290 UNIT 7 The Nursing Role in Restoring and Maintaining the Health of Children With Physiologic Disorders

a changed physical appearance and also need to avoid contact

BOX 44.2 Nursing Care Planning sports to prevent bleeding episodes, they may be the victims of
Using Assessment bullying or stigmas; be certain parents talk with their children
Assessing a Child With a Hematologic about this so children know to report bullying rather than con-
tinue to be a victim (Jenerette, Brewer, Crandell, et al., 2012).
Disorder
History
Chief concern: Fatigue, easy bruising, epistaxis. DISORDERS OF THE
Past medical history: Low birthweight; blood loss at birth; lack of vitamin K
administration at birth. RED BLOOD CELLS
Nutrition: “Picky eater” or presence of pica. Increasd milk intake.
Past illnesses: History of recent illness; history of recent medicine ingestion.
Family history: Inherited blood disorder; parents known to have sickle-cell Most RBC disorders fall into the category of the anemias, or a
trait, thalassemia minor, or hemophilia in family. reduction in the number or function of erythrocytes. Polycy-
Physical examination
themia, or an increase in the number of RBCs, can also occur
General appearance Possible significance and, because it can lead to blood clots, may be as dangerous
Obese infant Iron-deficiency anemia to a child as a reduction in RBC production.
Fatigue Anemia
Anemia occurs when the rate of RBC production falls
Eyes
Retinal hemorrhage Sickle-cell anemia
below that of cell destruction, or when there is a loss of RBCs,
Face
causing their number and the hemoglobin level to fall below
Bossing of Thalassemia that of production. Anemias are classified according to the
maxillary bone changes seen in RBC numbers or configuration, or accord-
Mouth ing to the source of the problem. Although any reduction
Pale mucous membrane Iron-deficiency anemia in the amount of circulating hemoglobin lessens the oxygen-
Ecchymotic or Decreased coagulation
bleeding gumline ability carrying capacity, clinical symptoms are not usually apparent
Heart until the hemoglobin level reaches 7 to 8 g/100 ml. Aver-
Increased rate, Anemia age values for hemoglobin and RBC number are available at
possible murmur
http://thePoint.lww.com/Pillitteri7e.
Skin
Petechiae, ecchymosis Decreased coagulation
Blood oozing from ability Normochromic, Normocytic Anemias
wound or injection point
Jaundice Hemolytic anemia Normochromic (normal color), normocytic (normal cell size)
Pallor anemias occur because of impaired production of erythrocytes
Bronze color Frequent blood
Abdomen transfusion by the bone marrow or by abnormal or uncompensated loss
Pain on palpation Sickle-cell anemia of circulating RBCs, as with acute hemorrhage. The RBCs
Increased liver or Hemolytic anemia appear normal in both color and size; however, there simply
spleen size
are too few of them for effective oxygen transport.
Genitourinary
Delayed secondary Sickle-cell anemia
sex characteristics Acute Blood-Loss Anemia
Extremities
Spoon-shaped nails Iron-deficiency anemia
Blood loss that is sufficient to cause anemia can occur from
Joint swelling, pain Hemophilia, sickle-cell trauma such as an automobile accident with internal bleeding;
Neurologic crisis from acute nephritis in which blood is lost in the urine; or in
Weak muscle tone Iron-deficiency anemia the newborn from disorders such as placenta previa, prema-
ture separation of the placenta, maternal–fetal or twin-to-twin
transfusion, or trauma to the cord or placenta. In childhood,
it can occur from the action of long-term intestinal parasites
The most frequently occurring anemia in children, iron- such as a tapeworm or hookworm or, in small infants, bedbug
deficiency anemia, could be virtually eliminated if all infants bites (Studdiford, Conniff, Trayes, et al., 2012).
were breastfed and those infants who are formula fed were fed With sudden blood loss, children immediately appear
iron-fortified formula for the full first year. The disorder oc- pale. Because their heart must push the reduced amount of
curs again with a high incidence in adolescents because ado- blood through their body more rapidly than usual, tachycar-
lescent diets tend to be low in meat and green vegetables, the dia will occur. Children will also begin to breathe rapidly be-
chief dietary sources of iron. Adolescents who begin pseudo- cause body cells are still not able to receive adequate oxygen.
vegetarian diets are another group especially prone to devel- Newborns may have gasping respirations, intercostal retrac-
oping the disorder. Counseling parents of young children to tions, and cyanosis. Children with rapid heart and respiratory
maintain well-child health care visits and urging adolescents rates due to this do not respond well to oxygen therapy be-
to ingest iron-rich foods could have a major impact on de- cause they lack RBCs to transport and use the oxygen. They
creasing the incidence of the disorder. become listless and inactive, dizzy, and possibly, comatose.
Aplastic anemia, or the inability to form blood elements, can This type of acute blood-loss anemia generally is transitory
be acquired if a child is exposed to a toxic drug or chemical. because the sudden reduction in available oxygen stimulates
Educating parents about the importance of keeping poisons out the release of erythropoietin from the kidney and a regenera-
of the reach of children and being aware of toxic substances tion response in the bone marrow. The reticulocyte count rises,
in their community could help decrease the incidence of this which is evidence that the bone marrow is trying to increase
disorder. Because children with hematologic disorders may have production of erythrocytes to meet the sudden shortage.

PILLITTERI_E7_CH44.indd 1290 7/8/13 6:39 AM

 CHAPTER 44 Nursing Care of a Family When a Child Has a Hematologic Disorder
Treatment involves control of bleeding by addressing its
underlying cause and transfusing additional RBCs. Lie the
child flat to provide as much circulation as possible to brain
cells. Keep the child warm with blankets; place the infant in
an incubator or under a radiant heat warmer. Until blood
is available for transfusion, a blood expander such as plasma
or intravenous fluid such as normal saline or Ringer’s lactate
may be given to expand blood volume and improve blood
pressure. With such emergency steps, the situation should be
transitory with no long-term consequences.
Anemia of Acute Infection
Acute infection or inflammation, especially in infants, can
cause increased destruction or decreased production of eryth-
rocytes. Common conditions that do this include osteomy-
elitis and ulcerative colitis. Management involves treatment
of the underlying condition. When the condition is reversed,
blood values will return to normal.
Anemia of Renal Disease
Either acute or chronic renal disease can cause loss of func-
tion in kidney cells, which causes an accompanying decrease
in erythropoietin production, resulting in a normocytic, nor-
mochromic anemia. Administration of recombinant human
erythropoietin can increase RBC production and correct the
anemia, but not the renal disease (Lum, 2012).
Anemia of Neoplastic Disease
Malignant growths such as leukemia or lymphoma (common
neoplasms of childhood) result in normochromic, normo-
cytic anemias because the invasion of bone marrow by prolif-
erating neoplastic cells impairs RBC production. There may
be accompanying blood loss if platelet formation also is de-
creased. The treatment of such an anemia involves measures
designed to achieve remission of the neoplastic process and
transfusion to increase the erythrocyte count.
Hypersplenism
Under usual conditions, blood filters rapidly through the
spleen. If the spleen becomes enlarged, however, blood cells
pass through more slowly, with more cells being destroyed
in the process. This increased destruction of RBCs can cause
anemia and may lead to pancytopenia (deficiency of all cell
elements of blood). Virtually any underlying splenic condi-
tion can cause this syndrome.
Therapeutic management consists of treating the underly-
ing splenic disorder, and includes a possible splenectomy.
Aplastic Anemias
Aplastic anemias result from depression of hematopoietic ac-
tivity in the bone marrow. The formation and development
of WBCs, platelets, and RBCs can all be affected (Rovó,
Tichelli, & Dufour, 2013).
Congenital aplastic anemia (Fanconi syndrome) is in-
herited as an autosomal recessive trait. A child is born with
several congenital anomalies, such as skeletal and renal
abnormalities, hypogenitalism, and short stature. Between 4
and 12 years of age, the child begins to manifest symptoms
of pancytopenia, or a reduction of all blood cell components
(Linker & Damon, 2012).
PILLITTERI_E7_CH44.indd 1291
1291
Acquired aplastic anemia is a decrease in bone marrow
production, which occurs if a child is excessively exposed
to radiation, drugs, or chemicals known to cause bone mar-
row damage. Exposure to insecticides and chemotherapeutic
drugs temporarily causes this. Other examples of drugs that
cause acquired aplastic anemia include chloramphenicol, sul-
fonamides, arsenic (contained in rat poison, sometimes eaten
by children), hydantoin, benzene, or quinine. A serious infec-
tion such as meningococcal pneumonia might cause autoim-
munologic suppression of the bone marrow, which then also
results in this condition.
Assessment. When symptoms begin, a child appears pale,
fatigues easily, and has anorexia from the lowered RBC
count and tissue hypoxia. Because of reduced platelet for-
mation (thrombocytopenia), the child bruises easily or
develops petechiae (pinpoint, macular, purplish-red spots
caused by an intradermal or submucous hemorrhage).
A child may have excessive nosebleeds or gastrointestinal
bleeding. As a result of a decrease in WBCs (neutropenia),
a child may contract an increased number of infections and
respond poorly to antibiotic therapy. Observe closely for
signs of cardiac decompensation such as tachycardia, tachy-
pnea, shortness of breath, or cyanosis from the long-term
increased workload of all these effects on the heart. Bone
marrow samples will show a reduced number of blood ele-
ments, and blood-forming spaces will be infiltrated by fatty
tissue (Kline, 2012).
The child is apt to be irritable because of the fatigue and
recurring symptoms. Parents may feel distressed if the illness
originated from exposure to a chemical they should have
kept away from their child, such as an insecticide. This can
cause parents to have less confidence in health care personnel
if the illness followed treatment with a drug such as chlor-
amphenicol. They may wonder how they can trust in a drug
to cure the illness if they believe a prescribed drug caused
the illness.
Therapeutic Management. The first step in therapy is to
immediately discontinue any drug or chemical suspected of
causing the bone marrow dysfunction and removing the sub-
stance from the child’s environment to avoid exposure. The
ultimate therapy for both congenital and acquired aplastic
anemia is hematopoietic stem cell transplantation (Korthof,
Békássy, & Hussein, 2013). If a donor cannot be located, the
disease is managed by a variety of procedures to supplement
blood or to suppress T-lymphocyte–dependent autoimmune
responses while waiting for a histocompatible donor. Packed
RBCs, platelet transfusions, cyclosporine, ATG, and an RBC-
stimulating factor such as erythropoietin are generally neces-
sary to maintain adequate blood elements. Some children
show improvement with a course of an oral corticosteroid
(prednisone) to further decrease the immune response or a
course of testosterone to stimulate RBC growth. Be certain to
observe a child well when administering ATG intravenously
because of the high risk for anaphylaxis.
For children who receive a hematopoietic stem cell trans-
plant, chances of complete recovery are good. For others, the
course will be uncertain. A decreased WBC count leaves the
child open to infection. The decreased platelet count may
persist for years after other blood elements have returned to
normal, producing long-term problems of bleeding, espe-
cially petechiae or purpura (Box 44.3).
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 1292 UNIT 7 The Nursing Role in Restoring and Maintaining the Health of Children With Physiologic Disorders

BOX 44.3 Nursing Care Planning to Empower a Family

TECHNIQUES FOR REDUCING BLEEDING WITH THROMBOCYTOPENIA

Q. Lana’s mother asks you, “What precautions do you take to limit black and blue spots on Lana when her platelet count is low?”
A. “We try, as a team, to do the following.”
• Limit the number of blood-drawing procedures; combine samples whenever possible.
• Use a blood pressure cuff instead of a tourniquet to reduce the number of petechiae.
• Apply pressure to any puncture site for a full 5 minutes before applying a bandage.
• Minimize the use of adhesive tape to the skin (pulling for removal may tear the skin and cause petechiae).
• Pad side and crib rails to prevent bruising. Assess the need for routine blood pressure determinations because tight
cuffs can lead to petechiae.
• Protect intravenous sites to avoid numerous reinsertions.
• Administer medication orally or by intravenous infusion when appropriate to minimize the number of subcutaneous or
intramuscular injection sites.
• Assess that the child is using a soft toothbrush and is offered foods that can be chewed without irritation (e.g., avoid toast crusts).
• Check toys for sharp corners, which may cause scratches. Urge the child to be careful with paper, because paper cuts
can bleed out of proportion to their size.
• Distract the child from rough play; suggest stimulating but quiet activities to minimize risk of injury.

Nursing Diagnoses and
Related Interventions
Nursing diagnoses need to speak to not only the
physical symptoms of aplastic anemia but also the
continual stress the parents feel if laboratory reports
do not reveal a dramatic change in their child’s blood
cell production. It may be helpful to urge them to
deal with this problem by facing only one day or one
blood test at a time rather than trying to dwell on the
outcomes of all the blood tests to come.
Nursing Diagnosis: Risk for disturbed body image
related to changed appearance occurring as medica-
tion side effects
Outcome Evaluation: Child views self as a worth-
while person; does not appear to be excessively shy
or reluctant to interact with peers.
Children who receive corticosteroids such as predni-
sone to suppress the immune response almost always
experience some of the drug’s side effects, such as
a cushingoid appearance, hirsutism, hypertension,
and marked weight gain. Long-term therapy with
testosterone can result in masculinizing effects, such
as growth of facial and body hair, the development
of acne, and deepening of the voice. Be certain
both children and their parents know these effects
are related to the medication and will fade when the
medication is withdrawn.
Adolescents may have an especially difficult time
accepting weight gain and increased acne and so
need a chance to express their feelings about their
changed appearance. Reinforce and emphasize
things they are doing well such as how well they are
managing this unexpected turn in their life.
Hypoplastic Anemias
Hypoplastic anemias also result from depression of hemato-
poietic activity in bone marrow and can also be either con-
genital or acquired. Unlike aplastic anemias, however, in
which WBCs, RBCs, and platelets are all affected, with hy-
poplastic anemias, only RBCs are affected.
Congenital hypoplastic anemia (Blackfan–Diamond syn-
drome) is a rare disorder apparently caused by an inherent defect
in RBC formation that affects both sexes and shows symptoms
as early as the first 6 to 8 months of life (Sakaguchi, Nakanishi,
& Kojima, 2013). An acquired form of this can be caused by
infection with parvovirus 19, the infectious agent that causes
fifth disease (Morinet, Leruez-Ville, Pillet, et al., 2011).
The onset of a hypoplastic anemia is insidious, and at first
it may be difficult to differentiate from iron-deficiency ane-
mia. With iron-deficiency anemia, blood cells appear hypo-
chromic and microcytic and are few in number; however, in
hypoplastic anemia, they are not only few in number but also
their structure is normochromic and normocytic.
With the acquired type, the reduction of RBCs is tran-
sient, so no therapy other than monitoring is necessary. Chil-
dren with the congenital form receive corticosteroid therapy
along with transfusions of packed RBCs to raise erythrocyte
levels. As a result of the necessary number of transfusions,
hemosiderosis (a deposition of iron in body tissue) can
occur. An iron chelation program such as subcutaneous infu-
sion of deferoxamine (Desferal) may be started concurrently
with transfusions to bind with iron and aid its excretion from
the body in urine. Although an oral form is available for chil-
dren over 10 years of age, this is usually given by a subcutane-
ous infusion pump over an 8-hour period for 5 or 6 nights
a week. Remind the parent to assess that the child is voiding
as usual and that his or her specific gravity is normal (1.003
to 1.030) before beginning an infusion so iron removed from
tissues can be excreted (Karch, 2013).
To begin such an infusion, an area beside the scapula or on
the thigh is cleaned with alcohol and a short 25-gauge needle
is inserted at a low angle into the subcutaneous tissue and

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 CHAPTER 44 Nursing Care of a Family When a Child Has a Hematologic Disorder
attached to an infusion pump by intravenous tubing. In addi-
tion to the assessments of voiding and specific gravity, periodic
slit-lamp eye examinations should be scheduled to check for
cataract formation, a possible adverse effect of deferoxamine.
Although congenital hypoplastic anemia has to be thought
of as a chronic condition, about one fourth of affected
children will undergo spontaneous permanent remission
before the age of 13 years. If not, they are candidates for he-
matopoietic stem cell transplantation. As with aplastic ane-
mia, both the child and the parents need support from health
care personnel to help them accept the many procedures and
tests required before full remission is finally achieved.
✔ QSEN Checkpoint Question 44.2
Informatics
Lana has received iron chelation therapy by deferoxamine in the
past. Which statement by her mother would best assure you she
understands the use and action of iron chelation therapy?
a. “I know the drug acts to remove excess iron from my child.”
b. “I have to check Lana’s pulse before I turn on the pump.”
c. “The drug is used to increase the level of iron in bone cells.”
d. “The drug has minimal side effects, so I can’t really give it
wrong.”
Look in Appendix A for the best answer and rationale.
Hypochromic Anemias
When hemoglobin production is inadequate, erythrocytes
appear pale (hypochromia) and are also usually reduced in
diameter (microcytic).
Iron-Deficiency Anemia
Although the incidence of iron-deficiency anemia is decreas-
ing in the United States due to improved infant nutrition, it
is still the most common anemia of infancy and childhood,
occurring whenever the intake of dietary iron is inadequate.
Without adequate iron, hemoglobin cannot be incorporated
into RBCs.
Children are at a higher risk for iron-deficiency anemia
than adults because they need more daily iron in proportion
to their body weight to maintain an adequate iron level than
do adults—a daily intake of 11 to 15 mg of iron. This type of
anemia occurs most often between the ages of 9 months and
3 years from infants drinking more milk than they are eating
iron-rich foods (Ziegler, 2011). Its frequency rises again in
adolescence, when iron requirements increase, especially for
girls who are menstruating. It is also found at a high incidence
in overweight teenagers if they ingest most of their calories
from high-carbohydrate, not iron-rich, foods (Moschonis,
Chrousos, Lionis, et al., 2012).
The Infant. A newborn usually has enough iron in reserve to
last for the first 6 months of life. After that, the infant needs
iron incorporated into the diet. Because iron stores are laid
down near the end of gestation, infants born preterm will have
fewer iron stores than those born at term and so tend to de-
velop iron-deficiency anemia before 5 to 6 months. Women
with iron deficiency during pregnancy tend to give birth to
iron-deficient babies because the babies do not receive iron
stores. As a preventive measure, preterm infants and those
PILLITTERI_E7_CH44.indd 1293
1293
whose mother was iron deficient during pregnancy may be
given an iron supplement beginning at about 2 months of age.
Infants born with structural defects of the gastrointesti-
nal system, such as gastroesophageal reflux or chalasia (where
an immature valve exists between the esophagus and stom-
ach resulting in regurgitation) or pyloric stenosis (narrowing
between the stomach and duodenum, resulting in vomiting),
are particularly prone to iron-deficiency anemia because iron
is not adequately digested. Infants with chronic diarrhea may
not be able to make use of iron due to inadequate absorption. If
infants are fed cow’s milk rather than breast milk, so much min-
imal gastrointestinal bleeding may occur that iron deficiency
anemia may develop.
Urging parents to breastfeed or use iron-fortified formula
as well as introduce iron-fortified cereal as a “first food” are
important health teaching measures to prevent this form of
anemia. Occasionally, infants can become constipated while
ingesting iron-rich formula, but this is the exception rather
than the rule.
Older Children. In children older than 2 years of age, chronic
blood loss is the most frequent cause of iron-deficiency ane-
mia caused by gastrointestinal tract lesions such as polyps,
ulcerative colitis, Crohn disease, protein-induced enteropa-
thies, parasitic infestation, or frequent epistaxis. Adolescent
girls with heavy menstrual periods can become iron deficient
when this is combined with frequent attempts to diet or with
overconsumption of snack foods that are low in iron.
Assessment. Common symptoms of iron-deficiency anemia
are shown in Box 44.4. The mark of iron-deficiency anemia
BOX 44.4 Nursing Care Planning
Using Assessment
Assessing a Child With Iron-Deficiency
Anemia
Pale mucous
membrane
Enlarged heart
(possible)
Enlarged spleen
(possible)
Poor
muscle tone;
decreased
activity
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 1294 UNIT 7 The Nursing Role in Restoring and Maintaining the Health of Children With Physiologic Disorders
BOX 44.5 Nursing Care Planning Based on Responsibility for Pharmacology
FERROUS SULFATE (FEOSOL)
Classification: Ferrous sulfate is an iron salt.
Action: Supplies iron for red cell production. It elevates
the serum iron concentration and then is converted to
hemoglobin or trapped in the reticuloendothelial cells for
storage and eventual conversion to a usable form of iron
(Karch, 2013).
Pregnancy Risk Category: A
Dosage: For severe iron-deficiency anemia: 4 to 6 mg/
kg/d, in three divided doses. For mild iron-deficiency
anemia: 3 mg/kg/d in two divided doses.
Possible Adverse Effects: Gastrointestinal upset,
anorexia, nausea, vomiting, constipation, dark stools,
stained teeth (liquid preparations)
Nursing Implications
• Instruct parents to administer the drug on an empty
stomach with water to enhance absorption. If this
causes gastrointestinal irritation, administer it after
is pale conjunctiva. Because this pallor develops slowly,
however, parents may describe their child as “fair skinned”
especially when their face develops the same pale appearance.
With extended iron deficiency, infants develop poor mus-
cle tone and reduced activity. Their heart may enlarge and,
on auscultation, demonstrate a soft systolic precordial mur-
mur as the heart beats faster in an attempt to supply body
cells with more oxygen. The spleen may be slightly enlarged.
Fingernails typically become spoon shaped or depressed in
contour.
As a rule, infants should not ingest more than 32 oz of
milk a day. A 24-hour dietary history of an iron-deficient in-
fant generally reveals a much higher milk intake than this,
perhaps as high as 50 oz a day. Parents also report the child
resists or parents do not offer iron-fortified cereal (1 quart of
milk provides only about 0.5 mg of iron; in contrast, 1 table-
spoon of iron-fortified baby cereal supplies 2.5 to 5.0 mg
of iron).
Laboratory studies will reveal a decreased hemoglobin level
(a level less than 11 g/100 ml of blood) and reduced hema-
tocrit level (below 33%). The RBCs are not only microcytic
and hypochromic but possibly also poikilocytic (irregular in
shape). The mean corpuscular volume, the mean corpuscular
hemoglobin, and serum iron levels are all low. Monoamine
oxidase (MAO) is an enzyme important for central nervous
system maturation. Because iron is a basic component of
MAO, without available iron, this necessary enzyme will be
absent, possibly affecting central nervous system maturation.
In school-age children, there is an association between
iron-deficiency anemia and poor school achievement,
probably related to the chronic fatigue children experience
(Falkingham, Abdelhamid, Curtis, et al., 2010). It is also as-
sociated with pica (the craving for ice cubes or the eating
of inedible substances such as dirt and paper). Until the
anemia is corrected, parents need to supervise their child’s
PILLITTERI_E7_CH44.indd 1294
meals. Avoid giving it with milk or tea because these
interfere with absorption.
• If the liquid preparation is ordered, advise parents to
mix it with water or juice to mask the taste. Have the
child drink the medication through a straw to avoid
staining the teeth.
• Remember that iron is absorbed best in the presence
of an acid. Suggest parents give the iron with a
citrus juice such as orange juice to help absorption.
Some children may be prescribed vitamin C to take
concurrently to increase absorption.
• Inform the child and parents that iron may turn stools black.
• Encourage parents to include high-fiber foods in the
child’s diet to minimize the risk of constipation.
• Reinforce the need for thorough brushing of teeth to
prevent staining.
• Remind parents about the need for follow-up blood
studies to evaluate the effectiveness of the drug.
environment to keep inedible materials out of his or her reach
(Khan & Tisman, 2010).
Therapeutic Management. Therapy for iron-deficiency anemia
focuses on treatment of the underlying cause: the lack of iron.
An iron compound such as ferrous sulfate for 4 to 6 weeks
is the drug of choice to improve RBC formation and replace
iron stores (Box 44.5) (Costa, Bracco, Gomes, et al., 2011). In
addition, the diet of the child must be changed to one rich in
iron and vitamin C, which enhances iron absorption.
Nursing Diagnoses and
Related Interventions
Nursing Diagnosis: Imbalanced nutrition, less than
body requirements, related to inadequate ingestion
of iron
Outcome Evaluation: Parents report child’s dietary
intake includes iron-rich foods; parents administer
ferrous sulfate as prescribed; serum iron levels
increase to normal by 6 months.
When planning care for an infant with iron-deficiency
anemia, it is helpful to minimize the child’s activities
to prevent fatigue, particularly at mealtime, because
a fatigued child is more reluctant to eat any food, let
alone iron-rich foods.
Counsel parents on measures to improve their
infant’s nutrition, such as adding iron-rich foods while
decreasing formula or breast milk intake. If the child is
not fond of meat, suggest parents substitute cheese,
7/8/13 6:39 AM
 CHAPTER 44 Nursing Care of a Family When a Child Has a Hematologic Disorder
eggs, green vegetables, or fortified cereal. Because
iron-rich foods are often expensive, remind parents
that these items are important and they should not
substitute less expensive, high-carbohydrate foods
for them. Help them also devise a reminder system
so they can manage to give the iron supplement over
a long period of time. Alert parents to possible side
effects, such as stomach irritation, constipation, and
that liquid iron preparations can stain teeth if not
taken through a straw. Iron is absorbed best with an
accompanying acid medium, so ascorbic acid may
also be prescribed (or the parent should be advised
to give the iron medication with orange juice) to
increase absorption. To avoid constipation, the child
may need additional fiber like that supplied by green
leafy vegetables. If oral iron is not tolerated or if there
is a doubt the child will take it, an iron–dextran injec-
tion (Imferon) can be given intramuscularly, although
this is extremely irritating and stains the skin unless it
is given by deep Z-track intramuscular injection.
Of all age groups, adolescents tend to do the least
well with taking medicine consistently. Help them
plan a daily time for taking their iron supplement with
a medication reminder chart. At first, they may reject
this as childish, but assure them that everyone needs
some sort of aid to remember such things. Review
with them the iron-rich foods they will need to eat
daily and that an iron supplement is only a supple-
ment if taken with iron-rich foods.
After 7 days of iron therapy, a reticulocyte count is
usually obtained. If elevated, this means the child is
now receiving enough iron that erythrocytes are now
proliferating and correcting the anemia. Iron medica-
tion is taken for at least 4 to 6 weeks after the RBC
count has returned to normal so iron stores are rebuilt
as well. In some children, maintenance therapy may
continue for as long as a year.
Chronic Infection Anemia
Acute infection interferes with RBC production, producing
a normochromic, normocytic anemia. When infections are
chronic, however, anemia of a hypochromic, microcytic type oc-
curs, which is probably caused by impaired iron metabolism. The
degree of anemia is rarely as severe as that occurring with iron
deficiency; administration of iron will have little effect until the
infection is controlled (Aguilar, Moraleda, Quintó, et al., 2012).
Macrocytic (Megaloblastic) Anemias
A macrocytic anemia is one in which the RBCs appear ab-
normally large (Lum, 2012). Such cells are actually immature
erythrocytes or megaloblasts (nucleated immature red cells)
so these anemias are often also referred to as megaloblastic
anemias. Because these anemias are caused by nutritional de-
ficiencies, they occur most often in developing countries.
Anemia of Folic Acid Deficiency
A deficiency of folic acid combined with vitamin C defi-
ciency produces an anemia in which the erythrocytes grow
abnormally large. There is often accompanying neutropenia
PILLITTERI_E7_CH44.indd 1295
1295
and thrombocytopenia. Although the mean corpuscular he-
moglobin concentration will be normal, the mean corpus-
cular volume and mean corpuscular hemoglobin will both
be increased. Bone marrow contains megaloblasts, indicating
inhibition of the production of erythrocytes at an early stage.
Megaloblastic arrest, or inability of RBCs to mature past this
early stage, may occur in the first year of life from the contin-
ued use of infant food containing too little folic acid or from
an infant drinking goat’s milk, which tends to be deficient in
folic acid. Treatment is daily oral administration of folic acid.
With this treatment, the response is dramatic.
Pernicious Anemia (Vitamin B12 Deficiency)
Vitamin B12 is necessary for the maturation of RBCs. Perni-
cious anemia results from a deficiency in or an inability of
the body to use the vitamin (Scott & Molloy, 2012). In chil-
dren, the cause is more often a lack of ingestion of vitamin B12
rather than poor absorption. Adolescents may be deficient in
vitamin B12 if they are ingesting a long-term, poorly formu-
lated vegetarian diet because the vitamin is found primarily in
foods of animal origin (Andres, 2012).
For vitamin B12 to be absorbed from the intestine, an
intrinsic factor must be present in the gastric mucosa. If a
child is born with an intrinsic factor deficiency, symptoms
occur as early as the first 2 years of life. The child appears pale,
anorexic, and irritable, with chronic diarrhea. The tongue ap-
pears smooth and beefy red due to papillary atrophy. If not
identified and treated at that point, neuropathologic findings
such as ataxia, hyporeflexia, paresthesia, and a positive Babin-
ski reflex will develop.
The rate and efficiency of absorption of vitamin B12 can
be tested by the ingestion of the radioactively tagged vitamin
when a dose of intrinsic factor is also measured. If the anemia
is identified as being caused by a B12-deficient diet, temporary
injections of B12 will reverse the symptoms. If the anemia is
caused by lack of the intrinsic factor, lifelong monthly intra-
muscular injections of B12 may be necessary.
Hemolytic Anemias
Hemolytic anemias are those in which the number of erythro-
cytes is low because there is increased erythrocyte destruction.
The destruction may be caused by fundamental abnormalities
in erythrocyte structure or by extracellular destruction forces.
Congenital Spherocytosis
Congenital spherocytosis is a hemolytic anemia that occurs
most frequently in the white Northern European population
and is inherited as an autosomal dominant trait. RBCs are
small and have a short life span apparently due to abnor-
malities of the protein of the cell membrane that make them
unusually permeable to sodium (Casale & Perrotta, 2011).
The anemia can be noticeable shortly after birth, al-
though symptoms may not be recognized until later in the
first year as the abnormal cells swell, rupture, and then are
destroyed by the spleen creating a severe anemia. Chronic
jaundice and splenomegaly also develop. Because the cells
are so small, the mean corpuscular hemoglobin concentra-
tion will be increased. Gallstones may be present in older
school-age children and adolescents because of the continu-
ous hemolysis, bilirubin release, and incorporation of biliru-
bin into gallstones.
7/8/13 6:39 AM
 1296 UNIT 7 The Nursing Role in Restoring and Maintaining the Health of Children With Physiologic Disorders
Infections can precipitate a crisis or cause bone marrow
failure. During such a period, the anemia increases rapidly as
the hemolysis continues. Blood transfusion will be necessary
to maintain a sufficient number of circulating erythrocytes
until the crisis passes.
The diagnosis of the disease is based on family history, the
obvious hemolysis, and the presence of the abnormal sphe-
rocytes. The treatment generally is a splenectomy at approxi-
mately 5 to 6 years. This measure will increase the number
of RBCs present but will not alter their abnormal structure.
Glucose-6-Phosphate Dehydrogenase Deficiency
The enzyme glucose-6-phosphate dehydrogenase (G6PD) is
necessary for maintenance of RBC life; lack of the enzyme
results in premature destruction of RBCs. The disease is
transmitted as a sex-linked recessive trait and occurs most
frequently in children of African American, Asian, Sephardic
Jewish, and Mediterranean descent (approximately 10% of
African American males have the disorder) (Panepinto &
Scott, 2011).
G6PD disease occurs in two identifiable forms. Children
with a congenital nonspherocytic type develop hemolysis,
jaundice, and splenomegaly and may have aplastic crises.
Other children have a drug-induced form in which the blood
pattern is normal until the child is exposed to fava beans or
drugs such as antipyretics, sulfonamides, antimalarials, and
naphthoquinones (the most common drug in these groups
is acetylsalicylic acid [aspirin]). Approximately 2 days after
ingestion of such an oxidant drug, the child begins to show
evidence of hemolysis, a low-grade fever, and perhaps back
pain. A blood smear will show Heinz bodies (oddly shaped
particles in RBCs).
The degree of RBC destruction depends on the drug
and the extent of exposure to it. Occasionally, a newborn is
seen with marked hemolysis because the mother ingested an
initiating drug during pregnancy.
G6PD deficiency may be diagnosed by a rapid enzyme
screening test or electrophoretic analysis of RBCs. The drug-
induced type usually is self-limiting, and if a child is not ex-
posed to substances that cause hemolysis, blood transfusions
are rarely necessary. Be certain that both parents and children
know about the abnormality in the child’s metabolism so they
can avoid common drugs such as acetylsalicylic acid.
Sickle-Cell Anemia
Sickle-cell anemia is an autosomal recessive inherited disor-
der carried on the ␤ chain of hemoglobin; the amino acid
valine takes the place of the normally appearing glutamic
acid. With this, the erythrocytes become characteristically
elongated and crescent shaped (sickled) when they are sub-
mitted to low oxygen tension (less than 60% to 70%), a
low blood pH (acidosis), or increased blood viscosity, such
as occurs with dehydration or hypoxia. When RBCs sickle
or change to an elongated shape, they cannot move freely
through vessels. Stasis and further sickling occurs (a sickle-
cell crisis). Blood flow halts and tissue distal to the blockage
becomes ischemic, resulting in acute pain and cell destruc-
tion (Linker & Damon, 2012).
Because fetal hemoglobin contains a ␥, not a ␤, chain,
the disease usually will not result in clinical symptoms until a
child’s hemoglobin changes from the fetal to the adult form
PILLITTERI_E7_CH44.indd 1296
at approximately 6 months. However, the disease can be
diagnosed prenatally by chorionic villi sampling or from cord
blood during amniocentesis. If these were not done, it will
be identified at birth by neonatal screening (Forman, Coye,
Levy-Fisch, et al., 2013).
Sickle-cell disease occurs in about 1 out of every 400 Af-
rican American infants in the United States. The sickle-cell
trait (a child carries a gene for the disease but does not have
active symptoms) occurs in approximately 8% of African
Americans (Hastings, Torkildson, & Agwaral, 2012).
The form of hemoglobin in this disorder is designated he-
moglobin S. A child with sickle-cell disease is said to have
hemoglobin SS (homozygous involvement). Both parents of
the child with the disease will have a combination of usual
adult and hemoglobin S types or be carriers (heterozygous)
of the sickle-cell trait (hemoglobin AS). People with the trait
produce enough normal hemoglobin to compensate for any
hemoglobin that is sickled and therefore show no symptoms.
A child with the disease (homozygous) produces no normal
hemoglobin and so will demonstrate characteristic symptoms
of sickle-cell anemia. A very few children have combinations
of hemoglobin S and hemoglobin C or E, which leads to
chronic mild anemia.
✔ QSEN Checkpoint Question 44.3
Evidence-Based Practice
The sickle-cell trait (hemoglobin AS) occurs in about 8% of
African Americans and, although typically benign, there is some
concern that intense physical exercise in such individuals could
lead to cardiac deaths from occlusive crises. To investigate if
this occurs, researchers examined the U.S. Sudden Death in
Athletes Registry. Of 271 African American football deaths in
the registry, 7% (1 in 14) were known to have the sickle-cell trait.
Each athlete experienced collapse with gradual deterioration
over several minutes during vigorous or exhaustive physical
exertion, usually during conditioning drills, typically early in the
training season, and when the outdoor temperature was at or
above 80°F (Harris, Haas, Eichner, et al., 2012).
Based on the previous study, which would be the best
exercise for Joey, who has sickle-cell disease, and his father
who has the sickle-cell trait?
a. Playing video games with each other
b. Joining a swimming program at their local YMCA
c. Watching sports together on TV each evening
d. Organizing a touch (no contact) football game each weekend
Look in Appendix A for the best answer and rationale.
Sickle-Cell Crisis. Sickle-cell crisis is the term used to denote
a sudden, severe onset of sickling. There is pooling of many
new sickled cells in blood vessels causing consequent tissue
hypoxia beyond the blockage (a vaso-occlusive crisis). Such a
crisis is most apt to occur when a child has a gastrointesti-
nal illness causing dehydration, a respiratory infection that
results in lowered oxygen exchange and a lowered arterial
oxygen level, or after extremely strenuous exercise (enough to
lead to tissue hypoxia); however, sometimes, no obvious cause
of a crisis can be found (Boxes 44.6 and 44.7). Symptoms
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 CHAPTER 44 Nursing Care of a Family When a Child Has a Hematologic Disorder 1297

are sudden, severe, and painful (Box 44.8 Nursing Care

BOX 44.6 Nursing Care Planning Planning, an interprofessional care map for the child with
Using Assessment sickle-cell anemia). A laboratory report will reveal a hemoglo-
Assessing a Child With Sickle-Cell Crisis bin level of only 6 to 8 g/100 ml. A peripheral blood smear
will demonstrate sickled cells. Bilirubin and reticulocyte lev-
els will be increased and the WBC count is often elevated to
12,000 to 20,000/mm3.
Fever Further complications that may occur are aseptic necro-
Yellow sclera sis of the head of the femur or humerus causing sharp joint
Vomiting pain or a cerebrovascular accident that occurs from a blocked
artery, resulting in loss of motor function, coma, seizures, or
even death. If there is renal involvement, hematuria or flank
pain may be present.
Other types of crisis that may occur include:
Enlarged liver
Acute back pain Enlarged spleen • A sequestration crisis occurs when there is splenic seques-
tration of RBCs or severe anemia occurs due to pooling
Possible kidney and increased destruction of sickled cells in the liver and
infarction
spleen. Shock symptoms occur from hypovolemia. The
Painful and spleen is enlarged and tender.
swollen hands • A hyperhemolytic crisis occurs when there is increased
destruction of RBCs.
Acute • A megaloblastic crisis occurs if the child has folic acid or
abdominal Joint pain, vitamin B deficiency (new RBCs cannot be fully formed
pain and swelling, and
tenderness warmth
due to lack of these ingredients).
• An aplastic crisis occurs when there is a sudden decrease in
RBC production. This form usually occurs with infection.
It creates a severe anemia.
Assessment for Sickle-Cell Anemia. Sickle-cell anemia is diag-
nosed at birth because of required blood-spot screening. At
approximately 6 months of age, children begin to show ini-
tial signs of fever and anemia. Stasis of blood and infarction
may occur in a body part, leading to local pain. Some infants

BOX 44.7 Nursing Care Planning Based on Effective Communication

Joey, who has sickle-cell disease, is seen in the emergency department with a new vaso-
occlusive crisis. His right knee is discolored by a large brush burn. He’s crying from pain.

Less Effective Communication More Effective Communication

Nurse: Hello, Mr. Harrow. I need to ask some questions
to see if anything triggered this new crisis.
Mr. Harrow: He better not have been doing something
he’s not allowed to do.
Nurse: His knee looks like he may have fallen. Were you
running, Joey? So you got dehydrated?
Mr. Harrow: He better not say he was doing that.
Nurse: Joey, how did you hurt your knee?
Joey: I don’t know.
Nurse: Okay. Let’s get you better and not worry about
what started this.
Nurse: Hello, Mr. Harrow. I need to ask some questions
to see if anything triggered this new crisis.
Mr. Harrow: He better not have been doing something
he’s not allowed to do.
Nurse: His knee looks like he may have fallen. Were you
running, Joey? So you got dehydrated?
Mr. Harrow: He better not say he was doing that.
Nurse: Mr. Harrow, why don’t we let Joey tell us how
he thinks the accident happened? Then later on,
we can talk about what are good rules for him to be
following.

Children with sickle-cell anemia have to follow what seems like a great many rules to avoid clotting or
bleeding episodes, such as not playing contact sports or playing too hard in the sun. In an emergency
room, it’s important that both children and parents recognize the priority at the moment is obtaining
an accurate history rather than assigning blame. Here, the nurse takes an active step to help the parent
move past a broken rule so therapy can be started.

PILLITTERI_E7_CH44.indd 1297 7/8/13 6:39 AM

 1298 UNIT 7 The Nursing Role in Restoring and Maintaining the Health of Children With Physiologic Disorders

BOX 44.8 Nursing Care Planning

AN INTERPROFESSIONAL CARE MAP FOR A CHILD WITH

SICKLE-CELL ANEMIA
Joey is a 7-year-old with sickle-cell anemia you see in the emergency room for a vaso-occlusive crisis.

Family Assessment Child lives with two older brothers
(10 and 12 years of age) and both parents in a three-
bedroom home. Father works as a distributor for a local
water bottling company. Mother, an X-ray technician, is
temporarily on duty with the National Guard in the Middle
East. Father rates finances as, “Not good. Medical bills are
killing us.”
Client Assessment Thin, early school-age child with
weight at fifth percentile for age. Was screened and
diagnosed with sickle-cell anemia at birth. Described as
“picky eater”; has eaten little since mother was deployed
because he doesn’t like father’s cooking. Has a history
of two former vaso-occlusive crises. Missed last regularly
scheduled health assessment 2 weeks ago because father
had difficulty taking off from work. Was playing “tag” with
older brothers this afternoon. Sclera was jaundiced and
child was crying from pain by time father returned from
work. Hemoglobin 6 g/100 ml; hematocrit 31%.
Nursing Diagnosis Altered tissue perfusion related to
vaso-occlusive crisis
Outcome Criteria Oxygen saturation level is maintained
at 95% or higher, pain decreases to tolerable level, and
symptoms of hemolytic crisis decrease.

Team Member
Responsible Assessment Intervention Rationale Expected Outcome

Activities of Daily Living, Including Safety

Nurse Assess if child under- Admit child to hospital Bed rest reduces the Child complies with
stands he will need unit; restrict to bed need for oxygen in bed rest; plays non-
to remain in bed. rest. body cells. action games with
parent or health
care personnel.

Teamwork and Collaboration

Nurse/Primary care Assess if hematology Meet with hematology Repeated vaso-occlu- Hematology service
provider service is needed service as needed sive crises suggest meets with par-
for consult. for emergency and family needs better ent and child as
long-range planning management indicated.
strategies.

Procedures/Medications for Quality Improvement

Nurse/Nurse Assess degree of child’s Administer prescribed Vaso-occlusive crises Child rates pain as
practitioner pain by use of analgesic as can cause sharp no higher than 2
FACES pain scale. needed. pain that requires following analgesia
strong analgesia. administration.

Nurse Assess oxygen Administer oxygen Oxygen saturation Child cooperates with
saturation level by by nasal prongs to decreases because pulse oximetry and
continuous pulse keep oxygen satura- sickled cells are oxygen adminis-
oximetry. tion above 95% or unable to carry a tration. Oxygen
as prescribed. full complement of saturation remains
oxygen. above 95%.

Nurse Determine whether Administer medications Folic acid and Child takes medication
child has been to initiate red blood hydroxyurea help cooperatively.
taking folic acid cell (RBC) produc- to build new RBCs
and hydroxyurea at tion as prescribed. to replace those
home. that have been
hemolyzed.

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 CHAPTER 44 Nursing Care of a Family When a Child Has a Hematologic Disorder 1299

Nutrition

Nurse Assess child’s intake Begin oral rehydra- Restoring hydration Child names best hand
and output. tion or intravenous reduces sickle-cell to start IV infusion;
(IV) therapy as clotting. drinks prescribed
prescribed. fluid.

Nurse/Nutritionist Assess child’s usual Demonstrate child’s Even “picky eaters” Parent states he will
nutrition intake by reduced weight to need to take in try harder to serve
24-hour dietary parent. Plan ways enough food daily foods the child likes;
recall history. to increase calorie to meet growth and child voices intent
intake. maintenance needs. to eat at least one
meat helping daily.

Patient-Centered Care

Nurse Assess family members’ Review with family Dehydration leads to Family members
understanding of members the impor- clumping of sickled state they are
the causes of sickle- tance of child avoid- cells, cutting off aware they must
cell vaso-occlusive ing dehydration and circulation in distant be as responsible
crises. oxygen deficiency. body parts. as the child for
avoiding sickling
circumstances.

Psychosocial/Spiritual/Emotional Needs

Nurse Assess the stress level Review with family ways When families miss Father states he will
of family in light of to maintain a tight a support per- try harder to meet
absent mother and knit family unit (e.g., son, they need to children’s needs,
child with chronic game night, com- rally together to although worrying
illness. mon activities) to devise other support about keeping his
maintain family unity methods. job and his wife’s
until mother returns. safety are major
concerns.

Informatics for Seamless Health Care Planning

Nurse Determine whether Schedule a follow- Care of a chronically ill Father states he
parent has any up visit in 3 days child can be a major understands impor-
questions about following hospital strain on a family. tance of follow-up
care of his or her discharge for evalu- Follow-up visits help visit and will keep
child. ation as prescribed. share responsibility appointment with
for care. child.

have swelling of the hands and feet (a hand–foot syndrome)
probably caused by aseptic infarction of the bones of the
hands and feet. As they grow, children tend to have a slight
build and characteristically long arms and legs. They may
have a protruding abdomen because of an enlarged spleen
and liver. In adolescence, the spleen size may decrease in size
from repeated infarction and atrophy, leaving the teenager
more susceptible to infection than usual because the spleen
can no longer filter bacteria. Pneumococcal meningitis and
salmonella-induced osteomyelitis become frequent illnesses;
the child needs prophylactic antibiotics and pneumococ-
cal and Hib vaccines to prevent these infections (Ngwube,
Jackson, Dixon, et al., 2012).
During late childhood or adolescence, an acute chest
syndrome with symptoms of fever, tachypnea, wheezing, or
cough that leads to pneumonia may begin to occur (Live-
say & Ruppert, 2012). The syndrome develops because,
when areas of the lung become inflamed and hypoxic, sickle
cells adhere to the irritated endothelium and then fail to
be reoxygenated. Blood transfusion is used to increase the
oxygen-carrying capacity of the blood, and broad-spectrum
antibiotics are given to resolve the pneumonia.
Another change that may occur is stasis of blood flow
because of cirrhosis of the liver (fibrotic degeneration),
which will eventually occur from infarcts and tissue scarring.
The kidneys may have subsequent scarring also, so kidney
function may be decreased. Eye sclera become icteric (yel-
lowed) from release of bilirubin from destruction of the
sickled cells; small retinal occlusions may lead to decreased
vision. Cell clusters in the blood vessels of the penis may
cause priapism, or a persistent, painful erection (Linker &
Damon, 2012).
Therapeutic Management. The child in a sickle-cell crisis has
three primary needs: pain relief, adequate hydration, and oxy-
genation to prevent further sickling and halt the crisis.
Acetaminophen (Tylenol) may be adequate pain relief for
some children; for others, a narcotic analgesic such as intra-
venous morphine may be necessary. Once children are pain
free and able to relax, the metabolic demand for oxygen is

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 1300 UNIT 7 The Nursing Role in Restoring and Maintaining the Health of Children With Physiologic Disorders
reduced and sickling begins to end. Hydration is generally
accomplished with intensive intravenous fluid replacement
therapy. The acidosis that develops from tissue hypoxia must
be corrected by electrolyte replacement. As a rule, because
some kidney infarction may have occurred, do not adminis-
ter potassium intravenously until kidney function has been
determined (e.g., the child voids). Otherwise, excessive po-
tassium levels can lead to cardiac arrhythmias. If infection
appears to be the precipitator for a sickling crisis, blood and
urine cultures, a chest X-ray, and a complete blood count
will be taken and the infection will be treated by antibiotics.
Blood transfusion (usually packed RBCs) may be necessary to
maintain the hemoglobin above 12 g/100 ml (termed hyper-
transfusion) (Marouf, 2011).
Hydroxyurea, an antineoplastic agent that has the potential
to increase the strength and oxygenation capacity of sickled
cells (Ponnampalam & Thalange, 2013). Given orally, a side
effect of the drug is anorexia, so children need their nutrition
intake monitored while taking the drug to be certain it is
adequate.
If none of the previous measures appears to be effective,
children may be given an exchange transfusion to remove
most of the sickled cells and replace them with normal cells.
Hematopoietic stem cell transplantation is a permanent solu-
tion to the disorder and is advocated for the child who does
not respond to usual therapies (Thompson et al., 2012).
✔ QSEN Checkpoint Question 44.4
Quality Improvement
Joey, who has sickle-cell anemia, is being treated for sickle-
cell crisis. Which statement by his father would best assure
you that Joey is receiving adequate nursing care?
a. “He never used to understand why he had these crises, but
now he can describe the reason.”
b. “He says that his pain is actually quite manageable now.”
c. “He says that the nurses he’s met so far are such nice
people.”
d. “He’s looking forward to getting home, but he really
doesn’t mind it here.”
Look in Appendix A for the best answer and rationale.
Nursing Diagnoses and
Related Interventions
Because, like most anemias, sickle-cell disease is a
chronic process, nursing diagnoses need to consider
both short- and long-term goals.
Nursing Diagnosis: Ineffective tissue perfusion
related to generalized infarcts due to sickling
Outcome Evaluation: Child’s respiratory rate is 16
to 20 breaths/min; cyanosis is absent; arterial blood
PILLITTERI_E7_CH44.indd 1300
gases within acceptable parameters, including PCO2
⫽ 40 mmHg, PO2 ⫽ 80 to 90 mmHg, oxygen satura-
tion of 95%; urine output greater than 1 ml/kg/hr.
Oxygen may be administered by either nasal cannula
or mask if arterial blood gases reveal a low PO2 level.
High concentrations of oxygen are used with caution
because hypoxia is a stimulant to erythrocyte pro-
duction, which is badly needed to replace damaged
cells. Monitor the flow rate carefully, therefore, and
use pulse oximetry to evaluate oxygen saturation
levels for changes. Encourage bed rest to reduce
oxygen expenditure.
Nursing Diagnosis: Ineffective health maintenance
related to lack of knowledge regarding long-term
needs of child with sickle-cell anemia
Outcome Evaluation: Parents accurately describe
disease process and identify special precautions nec-
essary to prevent a sickle-cell crisis.
In many children, episodes of sickling grow less se-
vere as the child reaches adolescence. Other children
experience such devastating episodes in early child-
hood that, without a stem cell transplant, the disease
becomes fatal at an early age.
Between crises, parents need to focus care on
preventing recurring crises. Although the hemo-
globin level of children may remain as low as 6 to
9 g/100 ml, children adjust well to this chronic state.
Caution parents that children who receive frequent
blood transfusions should not be given supple-
mentary iron or iron-fortified formula or vitamins
because they may receive too much iron; high
levels of excess iron are deposited in body tissues
(hemochromatosis) to a point of staining body tissue
or being incorporated into body tissue with fibrotic
scarring (hemosiderosis). Oral folic acid may be pre-
scribed to help rebuild hemolyzed RBCs. They need
to monitor urine output and may be asked to test
urine for specific gravity and hematuria to detect the
extent or presence of kidney damage occurring from
minor infarcts. Some children who have had kidney
infarcts and a lessened ability to concentrate urine
have chronic nocturnal enuresis (bed-wetting).
Be certain that children receive childhood im-
munizations so they are not vulnerable to common
childhood infections such as measles or pertussis
and receive meningococcal, pneumococcal, and
haemophilus influenzae type B vaccines to prevent
those specific infections. If children are prescribed oral
penicillin as prophylaxis against infection, help the
parents determine a method to remember or remind
conscientious administration of this.
Caution parents to bring their child to a health
care facility at the first indication of infection. Some
parents are reluctant to do this, afraid that they will
be labeled overprotective. Assure them that health
care personnel are knowledgeable about sickle-cell
anemia and that they know that a child with even a
minor infection could become very ill.
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 CHAPTER 44 Nursing Care of a Family When a Child Has a Hematologic Disorder 1301

Children should attend regular school and should Thalassemias

be allowed to participate in all school activities except
contact sports (such as football), which could result in
rupture of an enlarged spleen or liver. Long-distance
running is also inadvisable because it can lead to dehy-
dration. During the summer, parents need to offer the
child frequent drinks to prevent dehydration, especially
on long hikes and at the beach. Caution parents against
taking the child on board an unpressurized aircraft in
which the oxygen concentration may fall during flight.
Children with sickle-cell disease are at high risk if
they need surgery because the hours of being held
on nothing-by-mouth status, as well as being unable
to eat afterward, may lead to dehydration; anesthesia
may cause a transient hypoxia leading to sickling.
Caution parents that even for a simple operation
such as tooth extraction, they must alert health care
personnel about their child’s condition.
Because puberty is delayed in some children, both
parents and children may need counseling to accept
this; although they can be assured once puberty
changes do occur, they are adequate and just appear
later than usual. Children may need support and
positive reinforcement during their growth years to
enhance their self-esteem as they learn how to deal
with the results of a chronic hematologic disorder
(Box 44.9).
What if...44.2 Joey’s parent tells you he
restricts Joey, who has sickle-cell anemia, from
drinking any fluid after 4 PM to prevent bed-wetting. Is this
a good solution to bed-wetting for a child with sickle-cell
disease? How would you counsel this parent?
The thalassemias are autosomal recessive anemias associ-
ated with abnormalities of the ␤ chain of adult hemoglobin
(HgbA). Although these anemias occur most frequently in
the Mediterranean population, they also occur in children of
African and Asian heritage (Ponnampalam & Thalange, 2013).
Thalassemia Minor (Heterozygous ␤-Thalassemia)
Children with thalassemia minor, a mild form of this ane-
mia, produce a combination of both defective ␤ hemoglo-
bin and normal hemoglobin. The condition represents the
heterozygous form of the disorder and can be compared
with children having the sickle-cell trait. Because there is
some normal production, the RBC count is usually normal,
but the hemoglobin concentration will be decreased by 2 to
3 g/100 ml below usual levels. The blood cells are moderately
hypochromic and microcytic because of the poor hemoglobin
formation.
Children may have no symptoms other than pallor. They
require no treatment, and life expectancy is normal. They
should not receive a routine iron supplement because their
inability to incorporate it well into hemoglobin may cause
them to accumulate too much iron.
Thalassemia Major (Homozygous ␤-Thalassemia)
Thalassemia major is also called Cooley anemia or Mediter-
ranean anemia. It is diagnosed at birth by a blood spot test.
Because this is a ␤-chain hemoglobin defect, symptoms do
not become apparent until a child’s fetal hemoglobin has
largely been replaced by adult hemoglobin during the second
half of the first year of life. Effects of thalassemia major on
body systems are summarized in Table 44.3. Unable to pro-
duce normal ␤ hemoglobin, the child shows symptoms of
anemia, including pallor, irritability, and anorexia (Holstein
& Hohl, 2012).

BOX 44.9 Nursing Care Planning Based on Family Teaching

SCHOOL SAFETY PRECAUTIONS FOR CHILDREN WITH SICKLE-CELL DISEASE

Q. Joey’s father says to you, “What precautions should I discuss with my son’s teacher so I know he’s
safe at school?”
A. Some common suggestions include:
• Be certain your child either takes fluid with him or buys adequate fluid for lunch because he needs to maintain a high
fluid intake to prevent blood from becoming thick.
• Provide additional fluid in the summer, when dehydration is more apt to happen. Anticipate ways to provide fluid during
long hikes or school trips; time spent on a hot beach may need to be limited.
• Learn about sources high in folic acid, such as vegetables and fruit, and be certain these foods are included in your son’s
packed lunch or selected from a cafeteria every day.
• With the exception of contact sports (to avoid damage to an enlarged spleen) and long-distance running (to prevent
dehydration), encourage your son to participate in normal school activities.
• Know that bed-wetting may occur as part of the illness. Encourage your son to take baths in the morning if this occurs so
his clothes don’t smell of urine.
• Maintain routine health care such as immunizations to prevent common childhood illnesses such as measles and mumps,
which cause fever and dehydration.
• Have the school nurse call you for any sign of illness, such as an upper respiratory infection, so therapy can be started
immediately.

PILLITTERI_E7_CH44.indd 1301 7/8/13 6:39 AM

 1302 UNIT 7 The Nursing Role in Restoring and Maintaining the Health of Children With Physiologic Disorders
TABLE 44.3 Effects of Thalassemia Major
Effect of Abnormal Cell
Body Organ or System Production
Bone marrow Overstimulation of bone
marrow leads to increased
facial-mandibular growth
Skin Bronze-colored from
hemosiderosis and jaundice
Spleen Splenomegaly
Liver and gallbladder Cirrhosis and cholelithiasis
Pancreas Destruction of islet cells and
diabetes mellitus
Heart Failure from circulatory overload
RBCs are both hypochromic and microcytic. Fragmented
poikilocytes and basophilic stippling (unevenness of hemo-
globin concentration) are also usually present. The hemoglo-
bin level is less than 5 g/100 ml. The serum iron level is high
because iron is not being incorporated into hemoglobin; iron
saturation will be 100%.
Assessment. To maintain a functional level of hemoglobin,
the bone marrow hypertrophies in an attempt to produce
more RBCs, causing bone pain and the formation of tar-
get cells or large macrocytes that are short lived and non-
functional. The hyperactivity of the bone marrow results in
characteristic changes in the shape of the skull (parietal and
frontal bossing) and protrusion of the upper teeth, with
marked malocclusion. The base of the nose may be broad
and flattened; the eyes may be slanted with an epicanthal
fold, as in Down syndrome. An X-ray of the bone shows
marked osteoporotic (of lessened density) tissue, which
can lead to fractures. The child may have both an enlarged
spleen and liver due to excessive iron deposits and fibrotic
scarring in the liver and the spleen from increased attempts
to destroy defective RBCs. Abdominal pressure from the
enlarged spleen may cause anorexia and vomiting. Epistaxis
is common; diabetes mellitus may result from pancreatic
hemosiderosis (deposition of iron); and cardiac dilatation
with arrhythmias and heart failure may result in myocardial
fibrosis caused by invasion of iron.
Therapeutic Management. Stem cell transplantation is the
ultimate cure for the disorder. While waiting for a matched
donor, digitalis, diuretics, and a low-sodium diet may be
prescribed to prevent heart failure. Transfusion of packed
RBCs every 2 to 4 weeks (hypertransfusion therapy) can be
used to maintain hemoglobin between 10 and 12 g/100 ml
because, with this level of hemoglobin, erythropoiesis is
suppressed and cosmetic facial alterations, osteoporosis, and
cardiac dilatation are minimized. Hypertransfusion therapy
also reduces the possibility that a splenectomy will be nec-
essary. Frequent blood transfusions, unfortunately, increase
the risk of blood-borne diseases, such as hepatitis B, and
hemosiderosis, so children need an oral iron-chelating agent
to remove this excessive store of iron, such as deferasirox or
deferoxamine (Berdoukas, Farmaki, Carson, et al., 2012).
PILLITTERI_E7_CH44.indd 1302
A splenectomy may become necessary to reduce dis-
comfort and also to reduce the rate of RBC hemolysis and
the number of transfusions needed. Bone marrow stem cell
transplantation can offer a cure. Even without stem cell trans-
plantation, the overall prognosis of thalassemia is improving,
although it is still grave. Most children with the disease die of
cardiac failure during adolescence or as young adults if they
do not receive a hematopoietic stem cell transplant.
Nursing Diagnoses and
Related Interventions
Nursing Diagnosis: Risk for situational low
self-esteem related to changed physical appearance
Outcome Evaluation: Child states she can accept
altered appearance and interacts with peers.
Children with thalassemia major may have delayed
growth and sexual maturation. They may develop a
marked change in facial appearance because of the
overgrowth of marrow-producing centers of the facial
bones, which will be permanent. In addition, the
child who receives frequent blood transfusions may
develop such hemosiderosis that skin color appears
bronze.
Children should be allowed as much activity as
possible and should attend regular school to maintain
a nearly normal childhood. Discussions about other
children’s reactions to their changing facial appear-
ance and how people are evaluated by who they are
and not what they look like can be helpful.
What if...44.3 The last time you saw Lana
she was fair skinned, but at a clinic visit today you
notice her skin looks a lot darker. She tells you she and her
mother both spent time at a tanning salon to get ready for
a vacation in the sun. Would you admire her new tan or
worry if her darkened skin is caused by something else?
Autoimmune Acquired Hemolytic Anemia
Occasionally, autoimmune antibodies (abnormal antibod-
ies of the immunoglobulin [Ig]G class) attach themselves to
RBCs, destroying them or causing hemolysis. This can occur
at any age, and its origin is generally unknown, although
the disorder is associated with malignancy, viral infections,
or collagen diseases such as rheumatoid arthritis or systemic
lupus erythematosus. A child may recently have had an upper
respiratory infection, measles, or varicella virus infection
(chickenpox). The disorder may occur after the administra-
tion of drugs such as quinine, phenacetin, sulfonamides, or
penicillin (Garratty, 2012).
Assessment. The onset of symptoms is insidious. Children
usually develop a low-grade fever, anorexia, lethargy, pal-
lor, and icterus from release of indirect bilirubin from the
hemolyzed cells. Both urine and stools appear dark as the ex-
cess bilirubin is excreted. In a few children, the illness begins
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 CHAPTER 44 Nursing Care of a Family When a Child Has a Hematologic Disorder
abruptly with high fever, hemoglobinuria, marked jaundice,
and enlarged liver and spleen.
Laboratory findings reveal RBCs have become extremely
small and round (spherocytosis). The reticulocyte count will
be increased as the body attempts to form replacement RBCs.
A direct Coombs test result is positive, indicating the pres-
ence of antibodies attached to red cells. Hemoglobin levels
may fall as low as 6 g/100 ml.
Therapeutic Management. In some children, the disease
process runs a limited course and no treatment is necessary.
In others, a single blood transfusion may correct the distur-
bance. It is difficult to cross-match blood for transfusion for
these children, however, because the red cell antibody tends
to clump or agglutinate all blood tested. If cross-matching
is impossible, the child may be given type O, Rh-negative
blood, which doesn’t need to match. Carefully observe the
child especially during any transfusion for signs of transfu-
sion reaction.
If anemia is persistent, corticosteroid therapy (oral pred-
nisone) to reduce the immune response is generally effective,
increasing the RBC count and hemoglobin concentration in
a short period. If this is not effective, splenectomy or stron-
ger immunosuppressive agents such as cyclophosphamide
(Cytoxan) or azathioprine (Imuran) are necessary to reduce
antibody formation.
Often, it is difficult for parents to understand the process
causing their child’s condition. How could their child’s body
turn on itself this way? How long will this last? What will
stop it from happening again? Because there are no certain
answers to these questions, provide parents and children with
support as they wait for this unexplainable process to run its
course and for their child to be well again.
✔ QSEN Checkpoint Question 44.5
Teamwork & Collaboration
Autoimmune acquired hemolytic anemia can occur in any
child. You would want your team members to know the usual
cause of this disorder is:
a. Allergy to the protein found in fish or shrimp
b. A mutant gene similar to sickle-cell anemia
c. An elevated (increased) eosinophil cell count
d. Antibody production against red blood cells
Look in Appendix A for the best answer and rationale.
Polycythemia
Polycythemia is an increase in the number of RBCs (Giona,
Teofili, Moleti, et al., 2012). The condition results from
increased erythropoiesis, which occurs as a compensatory
response to insufficient oxygenation of the blood in order to
help supply more oxygen to body cells. Although this may
occur as a hereditary form, chronic pulmonary disease and
congenital heart disease are the usual causes of polycythemia
in childhood. Also, it may occur from the lower oxygen level
maintained during intrauterine life in newborns or with twin
transfusion at birth (one twin receives excess blood while a
second twin is anemic).
PILLITTERI_E7_CH44.indd 1303
1303
Plethora (marked reddened appearance of the skin)
occurs because of the increase in total RBC volume. Erythro-
cytes are usually macrocytic (large) and the hemoglobin con-
tent is high. This means the mean corpuscular hemoglobin
will be elevated; the mean corpuscular hemoglobin concen-
tration, however, will be normal, indicating that, although
many in number, each erythrocyte is normally saturated with
hemoglobin. The RBC count may be as high as 7 million/
mm3. Hemoglobin levels may be as high as 23 g/100 ml.
Treatment of polycythemia involves treatment of the
underlying cause. Because of the high blood viscosity from
so many crowded blood cells, cerebrovascular accident or
emboli may occur. The risk increases particularly if the child
becomes dehydrated, such as with fever or during surgery.
A program of low-dose aspirin to help prevent clotting, or
exchange transfusion or phlebotomy to reduce the RBC
count may be necessary.
DISORDERS OF BLOOD
COAGULATION
Platelets are necessary for blood coagulation, so disorders that
limit the number of platelets limit the effectiveness of this
process. A normal platelet level is 150,000/mm3. Thrombo-
cytopenia (decreased platelet count) is defined as a platelet
count of less than 40,000/mm3. Thrombocytopenia often
leads to purpura, or blood seeping from vessels into the skin.
In one rare disorder, children are born with thrombocyto-
penia and are also missing the radius bone in the forearm
(thrombocytopenia-absent radius [TAR] syndrome).
Purpuras
Purpura refers to a hemorrhagic rash or small hemorrhages
in the superficial layer of skin. Two main types of purpura
occur in children: idiopathic thrombocytopenia purpura and
Henoch–Schönlein syndrome.
Idiopathic Thrombocytopenic Purpura
Idiopathic thrombocytopenic purpura (ITP) is the result of
a decrease in the number of circulating platelets in the pres-
ence of adequate megakaryocytes (precursors to platelets).
The cause is unknown, but it is thought to result from an
increased rate of platelet destruction due to an antiplatelet
antibody that destroys platelets, making this an autoimmune
illness (Arnold, 2012).
In most instances, ITP occurs approximately 2 weeks after
a viral infection such as rubella, rubeola, varicella, or an upper
respiratory tract infection. Congenital ITP may occur in the
newborn of a woman who has had ITP during pregnancy.
An antiplatelet factor apparently crosses the placenta and
causes platelet destruction in the newborn in the same way
that Rh incompatibility or hemolytic disease of the newborn
develops (see Chapter 26). However, in ITP, the platelets, not
the RBCs, are sensitized.
Assessment. Manifestations often begin abruptly, first evi-
denced as miniature petechiae or as large areas of asymmetric
ecchymosis most prominent over the legs, although they may
occur anywhere on the body (Fig. 44.3). Epistaxis or bleeding
into joints may be present.
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 1304 UNIT 7 The Nursing Role in Restoring and Maintaining the Health of Children With Physiologic Disorders
FIGURE 44.3 An infant with idiopathic thrombocytopenia
purpura (ITP). Notice the tiny petechiae and larger ecchymotic
areas. (From Zitelli, B. J., & Davis, H. W. [1997]. Atlas of pediatric
physical diagnosis [3rd ed.]. St. Louis, MO: Mosby–Year Book, Inc.)
Laboratory studies reveal marked thrombocytopenia. The
platelet count may be as low as 20,000/mm3. Bone marrow
examination reveals a normal number of megakaryocytes.
Therapeutic Management. Oral prednisone to reduce the im-
mune response and intravenous immunoglobulin (IVIG) or, in
Rh-positive children, anti-D immunoglobulin to supply anti-
ITP antibodies are used to treat ITP. Platelet transfusion will
temporarily increase the platelet count, but because the life span
of platelets is relatively short, a platelet transfusion has only lim-
ited effect. Children with central nervous system bleeding may
require a splenectomy, although this is rarely necessary.
If the child experiences joint pain from bleeding,
acetaminophen (Tylenol) rather than salicylates or ibuprofen
is prescribed for pain because both salicylates and ibuprofen
increase the chance for bleeding as they prevent the aggrega-
tion of platelets at wound sites.
In most children, ITP runs a limited, 1- to 3-month
course. A few children develop chronic ITP. A course of im-
munosuppressive drugs may be attempted if the chronic state
persists. All children need to be vaccinated against the viral
diseases of childhood so that diseases such as rubella, rubeola,
and varicella are eradicated and can no longer lead to this
defective coagulation process.
Nursing Diagnoses and
Related Interventions
Nursing Diagnosis: Health-seeking behaviors related
to injury-prevention measures
Outcome Evaluation: Parents state precautions they
will take to reduce possibility of bleeding, repeat
correct dose and timing of medication therapy, child’s
skin is free of ecchymotic areas, and platelet count
rises to normal values.
The techniques for reducing bleeding described
earlier in the chapter, such as padding surfaces
PILLITTERI_E7_CH44.indd 1304
where the child plays (see Box 44.3), can be used
to reduce the possibility of bleeding for the child
with ITP. Parents cannot eliminate the possibility of
a serious bleeding injury, however, until the platelet
count returns to normal. The chief danger to the
child from ITP, aside from the psychological stress
of a perplexing illness, is intracranial hemorrhage.
Although this is rare, be alert for signs such as persis-
tent headache, nuchal rigidity, and lethargy.
Nursing Diagnosis: Risk for compromised family
coping related to diagnosis of child’s illness
Outcome Evaluation: Parents state that they under-
stand the nature of their child’s illness and have identi-
fied ways to carry out daily activities despite the illness.
Because symptoms such as the easy bruising of ITP
mimics the beginning signs of leukemia, parents may
be extremely frightened when symptoms first occur.
You can assure them after the diagnosis is made that
this bruising is not and will not lead to leukemia. A child
may have so many bruises that the parents are initially
suspected of child maltreatment. This can cause them
to become very defensive and angry at health care
personnel. Allow them time to express their anger and
regain confidence in their health care team.
It is always bewildering for parents to be told that
no one knows exactly what is causing their child’s
illness. To feel comfortable that health care person-
nel can manage their child’s care without knowing the
exact cause, parents need careful explanations of all
procedures.
Henoch–Schönlein Syndrome
Henoch–Schönlein purpura (also called anaphylactoid pur-
pura) is caused by increased vessel permeability. Although no
definite allergic correlation can be identified, it is generally
considered to be a hypersensitivity reaction to an invading
allergen. It occurs most frequently in children between 2 and
8 years of age, and more frequently in boys than girls (Liu &
Zhang, 2012). Usually, there is a history of a mild infection
before the outbreak of symptoms. The syndrome presents
(because of the purpura) as a possible platelet disorder until a
differential diagnosis is made.
Assessment. The purpural rash occurs typically on the
buttocks, posterior thighs, and extensor surface of the
arms and legs (Fig. 44.4). The tips of the ears may be
involved. The rash begins as a crop of urticarial lesions
that change to pink maculopapules. These become hemor-
rhagic (bright red) and then fade, leaving brown macular
spots that remain for several weeks. The child’s joints are
tender and swollen. The child may have gastrointestinal
symptoms such as abdominal pain, vomiting, or blood in
stools. Gross or microscopic hematuria may be present
from kidney involvement. A biopsy shows granulocytes
(white blood cells) in the walls of small arterioles.
Laboratory studies show a normal platelet count. The
child’s sedimentation rate, WBC count, and eosinophil
count are elevated.
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 CHAPTER 44 Nursing Care of a Family When a Child Has a Hematologic Disorder
FIGURE 44.4 The distinctive purpural rash of Henoch–
Schönlein syndrome appearing on the buttocks of a young
child. (From Zitelli, B. J., & Davis, H. W. [1997]. Atlas of pedi-
atric physical diagnosis [3rd ed.]. St. Louis, MO: Mosby–Year
Book, Inc.)
Therapeutic Management. Treatment involves oral cor-
ticosteroid therapy (prednisone) and mild analgesics for a
short period. Nose and throat cultures rule out continuing
bacterial involvement. Urine should be assessed for protein
and glucose to detect kidney involvement. Typically, the
disease runs a course of 4 to 6 weeks. A few children develop
chronic nephritis as a complication (Jauhola, Ronkainen, &
Koskimies, 2012).
✔ QSEN Checkpoint Question 44.6
Safety
Suppose Lana develops idiopathic thrombocytopenia purpura
(ITP) after a viral infection. Which of the following would be an
important nursing action?
a. Caution Lana and her mother that she will bruise easily.
b. Show Lana how to do a finger-stick test for glucose.
c. Tell Lana to report if she develops a sharp headache.
d. Show her mother how to test Lana’s urine for protein.
Look in Appendix A for the best answer and rationale.
Hemophilias
Hemophilias are inherited disorders of blood coagulation.
There are numerous types, each involving a deficiency of a
different blood coagulation factor.
Hemophilia A (Factor VIII Deficiency)
The classic form of hemophilia is caused by deficiency of
the coagulation component factor VIII, the antihemophilic
factor, and transmitted as a sex-linked recessive trait. In the
PILLITTERI_E7_CH44.indd 1305
1305
United States, the incidence is approximately 1 in 10,000
white males. A female carrier may have slightly lowered but
sufficient levels of the factor VIII component so that she
does not manifest a bleeding disorder. Males with the dis-
ease also have varying levels of factor VIII; their bleeding
tendency varies accordingly, from mild to severe (Chitlur &
Kulkarni, 2013).
Factor VIII is an intrinsic factor of coagulation; its absence
causes the intrinsic system for manufacturing thromboplas-
tin to be incomplete. The child’s coagulation ability is not
totally absent because the extrinsic or tissue system remains
intact. Because of this system, the child’s blood will eventu-
ally coagulate after an injury.
Assessment. Hemophilia often is recognized first in the in-
fant who bleeds excessively after circumcision. If the disease
has not shown itself for several generations in a family, the
parents may be unaware of its existence. For this reason, all
infants need careful and thoughtful observation after circum-
cision. Because infants do not receive many injuries, chil-
dren’s bleeding tendencies may not become apparent until
they become active (e.g., crawling, climbing, or walking).
Suddenly, the lower extremities (where the child bumps
things) become heavily bruised. There is soft tissue bleeding
and painful hemorrhage into joints, which become swollen
and warm. Repeated bleeding into a joint this way causes
damage to the synovial membrane (hemarthrosis), possibly
resulting in severe loss of joint mobility (Solimeno, Luck,
Fondanesche, et al., 2012).
Severe bleeding may also occur into the gastrointestinal
tract, peritoneal cavity, or central nervous system. Although
nosebleeds are common, they are not as severe as with the
platelet deficiency syndromes. The platelet count and pro-
thrombin time are both normal; the whole blood clotting
time is either markedly prolonged or normal, depending
on the level of factor VIII present. A thromboplastin gen-
eration test will be abnormal. Partial thromboplastin time
(PTT) is the test that best reveals the low levels of factor
VIII. It’s important that children with hemophilia be iden-
tified by such tests before surgery; otherwise, fatal bleeding
could occur.
Therapeutic Management. With even minor abrasions,
bleeding can be controlled by the administration of factor
VIII supplied by fresh whole blood, fresh or frozen plasma,
or a concentrate of factor VIII. The concentrate is supplied
as a powdered form that can be stored at home and recon-
stituted as needed. For most bleeding episodes, one bag of
concentrate per 5 kg of body weight is usually sufficient
to provide protection for approximately 12 hours; another
transfusion may be necessary after that time. In some chil-
dren, the administration of desmopressin (DDAVP), which
stimulates the release of factor VIII, may also help prevent
bleeding.
In a few children, antibodies (termed inhibitors) to factor
VIII develop, rendering the factor ineffective. If this happens,
⑀-aminocaproic acid, a fibrinolytic enzyme that helps to sta-
bilize clot formation and promote wound healing, can be
self-administered every 6 hours if needed. Children with
inhibitors to factor VIII can also be given a factor IX con-
centrate (Proplex or Konyne). This concentrate enters the
coagulation cascade after factor VIII and halts bleeding at
that point.
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 1306 UNIT 7 The Nursing Role in Restoring and Maintaining the Health of Children With Physiologic Disorders
Nursing Diagnoses and
Related Interventions
Nursing Diagnosis: Parental health-seeking
behaviors related to strategies for protecting child
from injury
Outcome Evaluation: Child’s skin is free of
ecchymotic areas, frequent epistaxis is absent, blood
pressure is within age-appropriate parameters, and
swelling or warmth at joints is absent.
Parents need information both about how to prevent
bleeding episodes and how to respond when one
does occur. To help prevent injuries, help parents
set appropriate limits for activity. An active infant,
for example, may need to have crib sides padded
and all toys should be inspected for sharp edges or
parts. Older children need to self-monitor activities
such as roughhousing and sports participation needs
to be evaluated as to whether it will be safe. On the
other hand, parents need to be certain their child is
not so inactive that obesity occurs (Wong, Majumdar,
Adams, et al., 2011).
Parents (and the child at about age 10 years) can
be taught to reconstruct and administer a replace-
ment factor intravenously to prevent bleeding
immediately after an injury. Although the child
needs to be assessed by a health care provider,
this action, combined with pressure applied to
the bleeding site, immobilization of the injured
extremity, and an ice pack applied locally, almost
always eliminates the need for hospital admission.
“Butterfly bandages” are used in place of sutur-
ing for lacerations whenever possible, because
sutures create additional puncture sites that could
bleed.
Nursing Diagnosis: Pain related to joint infiltration by
blood
Outcome Evaluation: Child states pain is at a
tolerable level.
The child with hemophiliac bleeding experiences
discomfort because of the bleeding into joints
and may be frightened because his parents are so
frightened. Acetaminophen (Tylenol) rather than
ibuprofen is ordered as an analgesic because ibu-
profen may prolong bleeding. As soon as effective
levels of factor VIII have been provided, the pain in
the bleeding joint is generally relieved, despite the
continued heat or swelling. Be certain that when
joints are immobilized, they are in good alignment.
As soon as the acute bleeding episode has halted
(approximately 48 hours), help the child perform
passive range of motion as prescribed to maintain
function.
Nursing Diagnosis: Risk for interrupted family pro-
cesses related to fears regarding child’s prognosis
and long-term nature of illness
PILLITTERI_E7_CH44.indd 1306
Outcome Evaluation: Family members voice their
fear regarding illness, state they are able to cope
despite stress level, and demonstrate positive coping
responses.
Parents of children with hemophilia are frightened
during a time of acute bleeding, not just because of
what is currently happening but also because they
may have seen other family members or even a previ-
ous child die of the disease. Be certain to give them
a chance to talk about how the bleeding began (e.g.,
“I should have noticed that toy had a sharp edge,”
“He fell from his bike. I should have watched him
more closely”). It is extremely important for parents
to allow the child to lead a normal life, so remind
them when they do this that it is impossible to pre-
vent all injuries (Neufeld, Recht, Sabio, et al., 2012).
von Willebrand Disease
von Willebrand disease, an inherited autosomal dominant
disorder, affects both sexes and is often referred to as angiohe-
mophilia (Blombäck, Eikenboom, Lane, et al., 2012). Along
with a factor VIII defect, there is also an inability of the plate-
lets to aggregate and the blood vessels to constrict to aid in
coagulation. Bleeding time is prolonged, with most hemor-
rhages occurring from mucous membrane sites.
Epistaxis is a major problem, because all children tend
to rub or pick at their nose as a nervous mechanism. In
girls, menstrual flow is unusually heavy and may cause em-
barrassment from stained clothing. Childbirth is obviously
a risk for women with von Willebrand disease, so women
must be monitored closely for 9 months and again during
the postpartal period (Weiss, 2012). Bleeding is controlled
with factor VIII replenishment as with hemophilia, or by
administration of DDAVP, which stimulates factor VIII
release.
Christmas Disease (Hemophilia B,
Factor IX Deficiency)
Christmas disease, caused by factor IX deficiency, is transmit-
ted as a sex-linked recessive trait. Only approximately 15% of
people with hemophilia have this form. Treatment is with a
concentrate of factor IX, which is available for home admin-
istration (Linker & Damon, 2012).
Hemophilia C (Factor XI Deficiency)
Hemophilia C, or plasma thromboplastin antecedent defi-
ciency, is caused by factor XI deficiency, is transmitted as an
autosomal recessive trait, and occurs in both sexes. The symp-
toms are generally mild compared with those in children with
factor VIII or factor IX deficiencies. Bleeding episodes are
treated with administration of DDAVP or transfusion of
fresh blood or plasma.
Disseminated Intravascular Coagulation
Disseminated intravascular coagulation (DIC) is an acquired
disorder of blood clotting that results from excessive trauma
or some similar underlying stimulus, such as an acute infec-
tion or trauma (Rote & McCance, 2012).
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