Chapter

Nursing Care of a Family When a

44 Child Has a Hematologic Disorder

K E Y T E R M S Lana is a 4-year-old

• agranulocytes • leukopenia girl diagnosed with

• allogeneic transplantation • megakaryocytes thalassemia major.
• autologous transplantation • normoblasts
• blood dyscrasias She has a prominent
• pancytopenia
• erythroblasts • petechiae mandible and wide-spaced upper front
• erythrocytes • plethora
teeth from overgrowth of bone marrow
• erythropoietin • poikilocytic
• granulocytes • priapism centers. Her skin is bronze from the
• hemochromatosis • purpura number of transfusions (64) she has
• hemolysis • reticulocytes
• hemosiderosis • synergeneic transplantation received in her short lifetime. Joey, a
• hypodermoclysis • thrombocytes 7-year-old with sickle-cell anemia, is
• leukocytes • thrombocytopenia
seen at the same clinic. His growth is
O B J E C T I V E S only in the 5th percentile, and he’s had
After mastering the contents of this chapter, you should be able to: two vaso-occlusive crises in the past
1. Describe the major hematologic disorders of childhood. year. “Why did this happen?” Lana’s
2. Identify National Health Goals related to children with hematologic mother asks you. “Why were our two
disorders that nurses could help the nation achieve.
3. Use critical thinking to analyze ways that nursing care for a child families so unlucky? What could we do
with a hematologic disorder could be more family centered. to help our children have better lives?”
4. Assess a child with a hematologic disorder.
5. Formulate nursing diagnoses for a child with a hematologic disor- Previous chapters described the
der such as sickle-cell anemia. growth and development of well chil-
6. Identify expected outcomes for a child with a hematologic disorder.
7. Plan nursing care for a child with a hematologic disorder. dren. This chapter adds information
8. Implement nursing care related to a child with a hematologic disor- about the dramatic changes, both
der, such as reducing the possibility of infection.
9. Evaluate expected outcomes for achievement and effectiveness
physical and psychosocial, that occur
of care. when children have a hematologic
10. Identify areas related to care of children with hematologic disorders
disorder.
that could benefit from additional nursing research or application of
evidence-based practice.
11. Integrate knowledge of hematologic disorders in children with What additional health teaching does
nursing process to achieve quality maternal and child health
nursing care. Lana’s mother need to help her better
understand these hematologic diseases?

1289
1290 UNIT 7 The Nursing Role in Restoring and Maintaining the Health of Children and Families With Physiologic Disorders

The blood and blood-forming tissues that make up the well. For example, families who are Jehovah’s Witnesses may
hematologic system play a vital role in body metabolism: refuse blood transfusions, a common therapy for blood disor-
transporting oxygen and nutrients to body cells, removing ders, on religious grounds.
carbon dioxide from cells, and initiating blood coagulation
when vessels are injured. As a result, any alteration in the
substance or function of blood or its components can have Nursing Process Overview
immediate and life-threatening effects on the functioning of
all body systems. For instance, an alteration in the process of For a Child With a Hematologic Disorder
coagulation can result in death from acute and uncontrol-
lable blood loss. Inadequate red cell formation results in de- Assessment
creased oxygenation in tissues (Linker, 2009). Many of the symptoms of hematologic disorders begin
Hematologic disorders, often called blood dyscrasias, insidiously, with symptoms such as pallor, lethargy, and
occur when components of the blood are formed incor- bruising. These are such minor symptoms that parents
rectly or either increase or decrease in amount beyond nor- may not bring their child to a health care facility for
mal ranges. Most blood dyscrasias in children originate some time. They are surprised to learn when they do that
in the bone marrow, where blood cells are formed. National such subtle symptoms can signify the presence of a serious
Health Goals related to blood disorders are shown in illness.
Box 44.1. Many hematologic disorders are inherited. When a
Blood dyscrasias do not occur at equal rates in all countries, child is diagnosed with one, parents may feel guilty or
because many of these disorders are inherited. Sickle-cell ane- blame themselves or their partner for their child’s disease.
mia, for example, occurs mainly in African Americans; tha- It may be difficult for parents to support a child during an
lassemia occurs in children from Mediterranean countries. illness when they need intensive support themselves be-
Iron-deficiency anemia, an example of a noninherited disor- cause they feel the illness is their fault. Be certain both
der, tends to occur in children from lower socioeconomic areas parents and children receive the support and comfort that
of many countries, because iron-rich foods often are expensive. they need.
Being aware of the differences in the incidence of blood Asking at routine checkups about a child’s dietary intake
dyscrasias this way can be helpful in planning care and provid- often reveals iron-deficiency anemia. Many infants with
ing health care services for children and communities. this problem have been drinking too much milk and not
Treatment for blood disorders can be culturally influenced as eating enough iron-containing foods. This makes them
iron-deficient, but aside from paleness and irritability, they
appear plump and “healthy.” Their parents do not suspect
their baby’s appearance masks a nutritional deficiency.
BOX 44.1 ✽ Focus on Nursing Diagnosis
National Health Goals Nursing diagnoses commonly used with children who
have hematologic disorders are:
National Health Goals have been set to address both
iron-deficiency anemia and sickle-cell anemia, two com-
• Deficient knowledge related to the cause of child’s
mon blood disorders in children:
illness
• Imbalanced nutrition, less than body requirements,
• Reduce the incidence of iron deficiency among chil- related to parental lack of knowledge of need for iron-
dren aged 1 to 2 years to less than 5% and among rich foods
women of childbearing age to less than 7% from • Anxiety related to frequent blood-sampling procedures
baselines of 9% and 11%. • Pain related to tissue ischemia
• Reduce hospitalization for sickle-cell disease yearly • Compromised family coping related to long-term care
among children aged 9 years and under from a base- needs of child with a chronic hematologic disorder
line of 41.3 hospitalizations per 100,000 children to 33
per 100,000 (http://www.nih.gov). Outcome Identification and Planning
When helping parents plan outcomes, be certain that the
Nurses can help the nation achieve these goals by ed- outcomes are realistic for both the child and family. It
ucating parents about the importance of women taking may not be possible to reduce the number of blood-sam-
an iron supplement during pregnancy and adding iron- pling procedures, for example, but a child can be helped,
rich cereal to their infants’ diets. They could help reduce with distraction techniques, to deal with the pain and
hospital admissions by being certain that parents are anxiety the procedures produce.
well informed about their child’s sickle-cell disease and Children with hematologic disorders often are pre-
precautions they can take to help avoid disease crises. scribed long-term medication such as a corticosteroid.
Nursing research questions that could add important in- When a child appears very ill, parents are usually very
formation for prevention for iron-deficiency anemia in- conscientious about giving such medicine. When a child
clude: what are ways of increasing adherence among has a disorder with few symptoms, however, like a blood
pregnant women that would help ensure that all women dyscrasia, it is easy for parents to forget to give the med-
take an iron supplement during pregnancy and do in- ication. In addition, a child may refuse to take the med-
fants maintain higher iron levels when cereal is eaten ication for a long time because it tastes bad or upsets the
with milk or orange juice? stomach. Planning includes helping parents devise ways to
 CHAPTER 44 Nursing Care of a Family When a Child Has a Hematologic Disorder 1291

disguise the taste or remember to give the medication over ANATOMY AND PHYSIOLOGY OF THE
the long term.
Nutritional planning is another area that needs consid- HEMATOPOIETIC SYSTEM
eration. Parents of children with iron-deficiency anemia, for Blood components originate in the bone marrow, circulate
example, may need to modify meal plans not only for the through blood vessels, and ultimately are destroyed by the
child but also for the entire family; this is not necessarily spleen.
easy to do, because iron-rich foods are expensive. If children
are “fussy eaters,” parents may need a great deal of support Blood Formation and Components
to insist that children eat foods containing iron rather than
giving them what they want to eat. If a child will be re- The formation of blood cells begins in the fetal yolk sac as
stricted in activity for long periods because the immune sys- early as week 2 of intrauterine life. By month 2 of intrauter-
tem is compromised as a part of the illness, planning must ine life, the liver and spleen begin forming blood components.
include ways to keep the child engaged with friends to pro- At approximately month 4, the bone marrow becomes and re-
mote development. Parents may need help investigating mains the active center for the origination of blood cells. As
possible resources for education and support to do this. in extrauterine life, the spleen serves as the organ for the de-
Because of the chronicity of some of the hematologic struction of blood cells once their normal life span has passed.
disorders, parents and children often need the support of The total volume of blood in the body is roughly propor-
outside agencies. Some organizations helpful for referral are tional to body weight: 85 mL/kg at birth, 75 mL/kg at 6
the Aplastic Anemia Foundation of America (http:// months of age, and 70 mL/kg after the first year. The blood
www.aplastic.org), Sickle-cell Disease Association of plasma (the liquid portion containing proteins, hormones,
America (http://www.sicklecelldisease.org), American enzymes, and electrolytes) is in equilibrium with the fluid of
Society of Pediatric Hematology and Oncology (http:// the interstitial tissue spaces. Although it is important in dis-
www.aspho.org), and National Hemophilia Foundation eases causing vomiting and diarrhea (when this fluid may be-
(http://www.hemophilia.org). come depleted, leading to dehydration), plasma is not a
major site of hematologic disease. The formed elements—the
Implementation erythrocytes (red blood cells), leukocytes (white blood cells),
Nursing interventions for children with hematologic dis- and thrombocytes (platelets)—are the portions most affected
orders include helping to obtain specimens for testing and by hematologic disorders in children.
assisting with blood or hematopoietic stem cell transfu-
sions. Remember that a finger puncture for blood is often Erythrocytes (Red Blood Cells)
as painful as a venipuncture (and more painful afterward Erythrocytes (red blood cells [RBCs]) function chiefly to
because the fingertip is irritated every time the child at- transport oxygen to and carry carbon dioxide away from
tempts to use it). Suggesting that blood be drawn by body cells. RBCs are formed under the stimulation of ery-
means of an intermittent device may reduce the number thropoietin, a hormone produced by the kidneys that is
of times a child is subjected to venipuncture. Applying an stimulated whenever a child has tissue hypoxia. Children
anesthetic cream (mixtures of lidocaine and prilocaine) with kidney disease often have a low number of RBCs be-
before finger punctures or venipunctures also helps to cause erythropoietin secretion is inadequate in diseased kid-
reduce pain and improve cooperation with these proce- neys. Polycythemia, or an overproduction of RBCs, can occur
dures. Even so, children may need some therapeutic play- in children who experience prolonged systemic hypoxia be-
time with a syringe and a doll to express their anger about cause of erythropoietin overproduction.
constant invasion by needles. RBCs form first as erythroblasts (large, nucleated cells),
then mature through normoblast and reticulocyte stages to
Outcome Evaluation mature, nonnucleated erythrocytes. Approximately 1% of
Evaluation focuses on whether short-term outcomes such RBCs are in the reticulocyte stage at all times. An elevated
as moderation of pain or elimination of anxiety in a child reticulocyte count indicates that rapid production of new
undergoing diagnosis or treatment were achieved and RBCs is occurring. This is seen in children with iron-deficiency
progress is being made toward the achievement of long- anemia once iron therapy is begun and the body is again able
term outcomes such as improving the ability of the family to produce RBCs. The absence of a nucleus in the mature red
to manage the stress of raising a child with a chronic ill- blood cell allows for increased space for oxygen transport, but
ness or deal with frequently occurring health crises. it also limits the life of cells because metabolic processes are
Examples of expected outcomes that suggest goals were limited. At the end of their life span (about 120 days), ery-
achieved are: throcytes are destroyed through phagocytosis by reticuloen-
dothelial cells, found in the highest proportion in the spleen.
• Parents correctly state the most frequent causes of In infants, the long bones of the body are filled with red
iron-deficiency anemia. marrow actively producing RBCs. In early childhood, yellow
• Child states she feels better able to cope with blood- marrow begins to replace this in long bones, so blood ele-
sampling procedures through the use of imagery. ment production is then carried out mainly in the ribs,
• Parents describe realistic plans to ensure adherence to scapulae, vertebrae, and skull bones. The yellow marrow re-
long-term medication administration. maining in the extremities can be activated if necessary to
• Parents voice they understand importance of prevent- produce additional blood products.
ing dehydration in school-age child with sickle-cell
anemia. ❧ At birth, an infant has approximately 5 million RBCs per
cubic millimeter of blood. This concentration diminishes
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1292 UNIT 7 The Nursing Role in Restoring and Maintaining the Health of Children and Families With Physiologic Disorders

rapidly in the first months, reaching a low of approximately cell cytoplasm). Granulocytes (often referred to as polymor-
4.1 million per cubic millimeter at 3 to 4 months of age. The phonuclear forms) are further differentiated as neutrophils,
number then slowly increases until adolescence, when the basophils, and eosinophils. The agranulocytic leukocytes are
adult value of approximately 4.9 million per cubic millime- further differentiated as lymphocytes and monocytes.
ter is reached. A typical total white cell count is 5000 to 10,000 cells
Hemoglobin. The component of RBCs that allows them to per cubic millimeter of blood. The WBC count in newborns
carry out the transport of oxygen is hemoglobin, a complex is approximately 20,000 per cubic millimeter, a high level
protein. Hemoglobin is composed of globin, a protein (like caused by the trauma of birth. In the newborn, granulocytes
all proteins) dependent on nitrogen metabolism for its for- are the most common WBCs. By 14 to 30 days of life, the
mation, and heme, an iron-containing pigment. Deficiency total WBC count falls to approximately 12,000 per cubic mil-
of either iron stores or nitrogen, therefore, will interfere with limeter, and lymphocytes become the dominant type. By
the synthesis of hemoglobin. It is the heme portion that com- 4 years of age, the WBC count reaches an adult level (5000 to
bines with oxygen and carbon dioxide for transport. 10,000 cells/mm3), and granulocytes are again the dominant
The hemoglobin in erythrocytes during fetal life is different type. Leukocytes are produced in response to need. Their life
from that formed after birth. Fetal hemoglobin has a special span varies from approximately 6 hours to unknown intervals.
affinity for oxygen, so it can absorb oxygen at the low oxygen Thrombocytes (Platelets)
tension that exists in utero. It is composed of two alpha and
two gamma polypeptide chains. At birth, 40% to 70% of the When blood is centrifuged in a test tube, plasma rises to the
infant’s hemoglobin is fetal hemoglobin (hemoglobin F). This top as a clear yellow fluid; red cells sink to the bottom as a
is gradually replaced by adult hemoglobin (hemoglobin A) dark-red paste. Between these two layers a thin white strip
during the first 6 months of life. Hemoglobin A is composed (often termed a buffy coat) forms that consists of the WBCs
of two alpha and two beta chains. For this reason, diseases such and thrombocytes. Thrombocytes are round, nonnucleated
as sickle-cell anemia or the thalassemias, which are disorders of bodies formed by the bone marrow. Their function is capil-
the beta chains, do not become apparent clinically until this lary hemostasis and primary coagulation. The normal range
hemoglobin change has occurred (at approximately 6 months is 150,000 to 300,000 per cubic millimeter after the first
of age). However, because some hemoglobin A is present even year. Immature thrombocytes are termed megakaryocytes.
in early intrauterine life, they can be diagnosed prenatally by If large numbers of these are present in serum, it indicates
hemoglobin analysis or electrophoresis. that rapid production of platelets is occurring.
The hemoglobin amount in blood varies according to the
number of RBCs present and the average amount of hemo- Blood Coagulation
globin each cell contains. Hemoglobin levels are highest at
birth (13.7 to 20.1 g/100 mL); they reach a low at approxi- Effective blood coagulation depends on a complex series of
mately 3 months of age (9.5 to 14.5 g/100 mL), and then events including a combination of blood and tissue factors
gradually rise again until adult values are reached at puberty released from the plasma (the intrinsic pathway) and from
(11 to 16 g/100 mL). injured tissue (the extrinsic pathway). The plasma-released
factors are factors VIII, IX, and XII. Factors released from in-
Bilirubin. After a RBC reaches its life span of approximately jured tissues are a tissue factor (an incomplete thromboplas-
120 days, it disintegrates and its protein component is pre- tin or factor III), plus factors VII and X. Together, these
served by specialized cells in the liver and spleen (reticulo- pathways form factor V. The names for coagulation factors
endothelial cells) for further use. Iron is released for reuse by are given in Box 44.2.
the bone marrow to construct new RBCs. As the heme por-
tion is degraded, it is converted into protoporphyrin.
Protoporphyrin is then further broken down into indirect
bilirubin. Indirect bilirubin is fat soluble and cannot be ex- BOX 44.2 ✽ Blood Coagulation Factors
creted by the kidneys in this state. It is therefore converted by
the liver enzyme glucuronyl transferase into direct bilirubin, I Fibrinogen
which is water soluble. This is then excreted in bile. II Prothrombin
In the newborn, generally liver function is so immature that III Thromboplastin
the conversion from indirect to direct bilirubin cannot be IV Calcium
made. Because of this, bilirubin remains in the indirect form. V Labile factor (platelet phospholipids)
When the level of indirect bilirubin in the blood rises to more VII Stable factor
than 7 mg/100 mL, it permeates outside the circulatory sys- VIII Antihemophilic factor
tem, and the infant shows signs of yellowing or jaundice. If ex- IX Christmas factor; antihemophilic factor B;
cessive hemolysis (destruction) of RBCs occurs from other plasma thromboplastin component
than natural causes, a child will also show signs of jaundice. X Stuart factor
XI Plasma thromboplastin antecedent
Leukocytes (White Blood Cells)
(antihemophilic factor C)
Leukocytes (white blood cells [WBCs]) are nucleated cells. XII Hageman factor
They are few in number compared with RBCs, with approx- XIII Fibrin stabilizing factor
imately 1 WBC to every 500 RBCs. Their primary function Numbers refer to the order in which factors were
is defense against antigen invasion. There are two main forms discovered, not to the order of action in coagulation.
of WBCs: granulocytes (those with granules in the cell cy-
toplasm) and agranulocytes (those without granules in the
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CHAPTER 44 Nursing Care of a Family When a Child Has a Hematologic Disorder 1293

Stage 1 Platelets adhere

(Coagulation other, forming a platelet plug. This is the first stage of clot-
to each other
factors ting (Fig. 44.1).
involved) In the second stage, factors from either the intrinsic or the
Stage 2 Platelet Complete VIII extrinsic system combine with platelet phospholipid to form
phospholipid thromboplastin (III) IX complete thromboplastin.
X In the third stage, thromboplastin converts prothrombin
XI (factor II) to thrombin if ionized calcium is present. The pro-
XII duction of prothrombin and factors VII, IX, and X depends
IV on the presence of vitamin K. This stage will be incomplete
Stage 3 Prothrombin (II) Thrombin IV if levels of any of factors VIII through XII, vitamin K, or cal-
V
cium are deficient.
VII
X
In the fourth stage, thrombin converts fibrinogen (factor
Stage 4 Fibrinogen (I) Fibrin XIII I) to fibrin. Fibrin strands form a mesh incorporating RBCs,
WBCs, and platelets to form a permanent protective seal at
FIGURE 44.1 Steps in blood coagulation. the site of injury. Factor XIII (fibrin stabilizing factor) acts to
make the fibrin clot insoluble and permanent.
To prevent too much coagulation after the seal is com-
plete, plasminogen is then converted to plasmin (a fibri-
When a vessel is injured, vasoconstriction occurs in the nolysin) near the injury to halt the clotting sequence. Blood
area proximal to the injury, narrowing the vessel lumen and coagulation problems will result if any step or factor in the
reducing the amount of blood to the injured area. Platelets process is inadequate. Common tests for blood coagulation
begin to adhere to the damaged vessel site and to one an- are described in Table 44.1.

TABLE 44.1 ✽ Tests for Blood Coagulation

Test Definition Normal Value

Prothrombin time (PT) Measures action of prothrombin after complete 11–13 seconds (PT)
thromboplastin is added to the blood in a 2.0–3.0 (INR)
test tube; reveals deficiencies in
prothrombin, factors V, VII, and X.
International Normalized Ratio (INR) is a
comparative rating of PT ratios that allows
for more sensitive analysis.
Partial thromboplastin time (PTT) Measures activity of thromboplastin after 30–45 seconds
incomplete thromboplastin is added to
blood in a test tube; reveals deficiencies in
thromboplastin, factors VIII–XII
Bleeding time Measures the time required for bleeding at a 3–10 minutes
stab wound on the earlobe to cease;
reveals deficiencies in platelet formation
and vasoconstrictive ability
Clot retraction Measures platelet function; interval from Retraction at side of test tube
placement of blood in a tube to the point in 1 hour; complete in
clot shrinks and expels serum 24 hours
Tourniquet Measures capillary fragility and platelet 0–2 petechiae per 2-cm area
function; response of tissue to application
of tourniquet to forearm for 5–10 minutes
Prothrombin consumption time Evaluates thromboplastin function; child’s Approximately 20 seconds
blood is allowed to clot and PT is then
done on the serum; if clot formation used a
great deal of prothrombin (as it should),
serum prothrombin time will be brief;
prolongation denotes defects in
thromboplastin function
Thromboplastin generation time Tests basic ability to form thromboplastin; 12 seconds or less
difficult test to do; ordered rarely to
distinguish factor VIII from factor IX defects
Plasma fibrinogen Measures stage 4 clotting process; level of 200–400 mg/100 mL plasma
fibrinogen in blood
Venous clotting time (Lee-White) Measures factor defects in stages 2 and 4; 9–12 minutes
time it takes venous blood to clot in a
test tube
1294 UNIT 7 The Nursing Role in Restoring and Maintaining the Health of Children and Families With Physiologic Disorders

ASSESSMENT OF AND THERAPEUTIC A child feels pain from the local anesthetic injection and
hard pressure while the needle is inserted. Some report a
TECHNIQUES FOR HEMATOLOGIC sharp pain when the marrow is actually aspirated. If con-
DISORDERS scious sedation is used, monitor vital signs until the child is
fully awake. Monitor pulse and blood pressure and observe
Assessment of children with hematologic disorders begins
the dressing every 15 minutes for the first hour after the pro-
with a history to identify inherited disorders. For specific di-
cedure to be certain that no bleeding is occurring. Keep the
agnosis, children generally require several diagnostic proce-
child fairly quiet for the first hour by playing a quiet game or
dures such as blood cell or bone marrow analysis.
other activity. Allow young children an opportunity for ther-
apeutic play with a doll and syringe to help them express
Bone Marrow Aspiration and Biopsy their feelings about such a painful, invasive procedure. If the
Bone marrow aspiration provides samples of bone marrow so procedure was done as an ambulatory procedure, instruct
that the type and quantity of cells can be determined (Beattie, parents to take the child’s temperature 12 and 24 hours after
2007). In children, the aspiration sites used are the iliac crests the procedure to detect infection.
or spines (rather than the sternum, which is commonly used in
adults; Fig. 44.2) because these sites have larger marrow com- Blood Transfusion
partments during childhood. Also, performing the test at these
sites is usually less frightening for children. In neonates, the an- Transfusions of blood or its products are used in the treat-
terior tibia can be used. ment of many disorders, including the anemias and primary
For a bone marrow aspiration, a child lies prone on a immunodeficiency disorders (see Chapter 42). A variety of
treatment table. Use of a hard table rather than a bed is ad- forms of blood are available, including whole blood, packed
vantageous because pressure is needed to insert the needle RBCs, washed RBCs (as much “foreign” matter is removed
through the surface of the bone into the marrow compart- as possible to reduce the possibility of blood reaction),
ment. Conscious sedation may be used to help reduce the plasma, plasma factors, platelets, WBCs, and albumin. No
child’s fear. Topical anesthesia helps reduce pain. matter what is the blood product, it is important that it has
The area of the aspiration is cleaned with an antiseptic been carefully matched with the child’s blood type. Blood
solution and draped. The overlying skin is infiltrated with must be infused accompanied with a solution as nearly iso-
a local anesthetic. After a few minutes, a large-bore needle tonic as possible (normal saline). If blood is given with a
and stylus is introduced through the overlying tissue into hypertonic solution, fluid will be drawn out of the RBCs,
the bone. This involves considerable pressure. When the causing them to shrink; if infused with a hypotonic solution,
marrow cavity is reached, the stylus is removed, a syringe is fluid will be drawn into the cells, and they will burst. In both
attached to the needle, and bone marrow is aspirated (ap- instances, they will be destroyed.
pears as thick blood in the syringe). The syringe is then re- Packed RBCs is the most common form of transfusion
moved, and marrow is expelled onto a slide and allowed to used with children because they help minimize the risk of
dry. After being sprayed with a preservative, it is taken to fluid overload. The usual amount of blood transfused to chil-
the laboratory for analysis. The aspiration needle is re- dren is 15 mL/kg body weight. The commonly accepted rate
moved, and pressure is applied to the puncture site to pre- for transfusions in a child is 10 mL/kg/hr unless the child
vent bleeding. After another few minutes, a pressure dress- has hypovolemic shock and volume equilibrium needs to be
ing is applied. established. An infusion of packed RBCs at a proportion of
15 mL/kg can be expected to raise the hematocrit level 5
points. A transfusion of platelets will elevate the platelet
count by approximately 10,000 cells. Platelets last only ap-
proximately 10 days, however, so transfusions of these must
be repeated every 10 days.
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
a thick, difficult-to-mobilize fluid.
Before any transfusion, ensure that a signed consent form
is obtained to respect sociocultural or religious beliefs. Also
obtain vital signs to establish a baseline. Monitor vital signs
about every 15 minutes during the first hour and approxi-
mately every half hour for the remainder of the transfusion.
Give the infusion slowly for the first 15 minutes; then
increase the rate to 10 mL/kg/hr if no reaction occurs.
Common symptoms of blood transfusion reactions are
shown in Table 44.2.
Provide an enjoyable activity for children during trans-
FIGURE 44.2 A common site used for bone marrow aspira- fusions. Without this, a child can become bored and could
tion in children is the iliac crest. In neonates, the anterior tibia attempt to increase the infusion rate to speed up the
may be used. process.
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CHAPTER 44 Nursing Care of a Family When a Child Has a Hematologic Disorder 1295

TABLE 44.2 ✽ Common Symptoms of Blood Transfusion Reactions

Symptoms Cause Time of Occurrence Nursing Interventions

Headache, chills, back Anaphylactic reaction to Immediately after start Discontinue transfusion.
pain, dyspnea, hy- incompatible blood; of transfusion Maintain normal saline
potension, hemoglo- agglutination of red infusion for accessible
binuria (blood in urine) blood cells occurs; intravenous line.
kidney tubules may Administer oxygen as
become blocked, re- necessary.
sulting in kidney Anticipate physician order
failure for diuretic to increase
renal tubule flow and
reduce tubule plugging
and/or heparin to
reduce intravascular
coagulation.
Pruritus, urticaria (hives), Allergy to protein compo- Within first hour after start Discontinue transfusion
wheezing nents of transfusion of transfusion temporarily.
Give oxygen as needed.
Anticipate physician
order for antihistamine
to reduce symptoms.
Increased temperature Possible contaminant in Approximately 1 hour Discontinue transfusion.
transfused blood after start of transfusion Obtain blood culture to
rule out bacterial inva-
sion as ordered.
Increased pulse, Circulatory overload During course of Discontinue transfusion.
dyspnea transfusion Give oxygen as needed.
Provide supportive care
for pulmonary edema
and congestive heart
failure.
Anticipate physician order
for diuretic to increase
excretion of fluid.
Muscle cramping, twitch- Acid-citrate-dextrose During course of Discontinue transfusion.
ing of extremities, anticoagulant in trans- transfusion Anticipate physician order
convulsion fusion is combining for calcium gluconate
with serum calcium and intravenously to restore
causing hypocalcemia calcium level.
Fever, jaundice, lethargy, Hepatitis from contami- Weeks or months after Obtain transfusion history
tenderness over liver nated transfusion transfusion of any child with
hepatitis symptoms.
Refer for care of hepatitis.
Bronze-colored skin Hemosiderosis or deposi- After repeated Support self-esteem with
tion of iron from transfu- transfusions altered body image.
sion in skin Administer iron-chelating
agent (deferoxamine)
as ordered to help
reduce level of
accumulating iron.

Hematopoietic Stem Cell Transplantation Hematopoietic stem cell transplantation has become a
relatively common procedure for children with blood dis-
Stem cell transplantation is the intravenous infusion of orders such as acquired aplastic anemia, sickle-cell anemia,
hematopoietic stem cells from bone marrow obtained by mar- thalassemia, and leukemia and some forms of immune dys-
row aspiration or from peripheral or umbilical cord blood function diseases. Although a stressful procedure to un-
drawn from a donor to reestablish marrow function in a child dertake, it offers children the opportunity for a complete
with defective or nonfunctioning bone marrow. Donors are reversal of symptoms (Nuss & Wilson, 2007). There is no
compatible when their human leukocyte antigen (HLA) sys- guarantee that the graft will be accepted by the recipient, or
tem matches that of the recipient. that improvement will occur. However, with good tissue
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1296 UNIT 7 The Nursing Role in Restoring and Maintaining the Health of Children and Families With Physiologic Disorders

compatibility in the absence of infection, this can be effec- the child’s cardiac rate and rhythm during the infusion to de-
tive in most children. tect circulatory overload or pulmonary emboli from unfil-
Hematopoietic stem cells can be recovered from circulat- tered particles.
ing peripheral blood after the stimulation of stem cell pro- Fever and chills are common reactions to a hematopoietic
duction by a cytokine or stem cell colony-stimulating factor. stem cell transplant infusion. Acetaminophen (Tylenol),
Stem cell transplants are most successful when recipients diazepam (Valium), or diphenhydramine hydrochloride
have not already received multiple blood transfusions that (Benadryl) may be prescribed to reduce this reaction.
have sensitized them to blood products. Success also de- After the infusion, take the child’s temperature at 1 hour
pends on the HLA compatibility of donated stem cells to the and then every 4 hours to detect infection that could occur
child’s blood. An identical twin is the ideal donor; a parent because the child’s WBCs are nonfunctional from radiation
or sibling may be next best, although compatibility is not or immunosuppression. Reinforce strict handwashing and
guaranteed. Siblings have about a 25% chance of being limit the child’s diet to cooked foods to reduce exposure to
HLA compatible. bacteria.
Stem cell transplants are allogeneic, synergeneic, or autol- Almost immediately after the infusion, stem cells begin to
ogous. Allogeneic transplantation involves the transfer of migrate from the child’s bloodstream into the marrow. If en-
stem cells from an immune-compatible (histocompatible) graftment occurs (the transplant is accepted), new RBCs can
donor, usually a sibling, although a national registry allows be detected in the peripheral blood in approximately
compatible volunteer donors to be located. Synergeneic 3 weeks. WBCs and platelet cells may not return to normal
transplantation involves a donor and recipient who are ge- for up to 1 year after a transplant; weight gain may also be
netically identical (are identical twins). Autologous trans- delayed (Box 44.3).
plantation involves use of the child’s own stem cells. The At first, the WBC count must be measured daily; bone
source of most autologous transfusions is cord blood that was marrow aspirations or venous blood samples are scheduled at
banked at the child’s birth (Tse, Bunting, & Laughlin, regular intervals to assess the growth of the new marrow.
2008). If this is not available, stem cells are aspirated from
the bone marrow or obtained from circulating blood, treated What if... Lana’s 12-year-old sister donates hematopoi-
to remove abnormal cells, and then reinfused. etic stem cells to Lana, but the transplant does not
Parents who are found to be incompatible donors may feel “take”? How would you explain to the donor child that
guilty and frustrated they can not do more for their child. If she did not fail?
the most compatible person for transplant is a young sibling,
health care personnel and parents alike may have some reser-
vations about submitting the young child to bone marrow as-
piration (Weiner et al., 2007).
To prevent a child from rejecting newly transplanted
donor stem cells by the T lymphocytes, a drug such as cy- BOX 44.3 ✽ Focus on Evidence-
clophosphamide (Cytoxan) will be administered intra- Based Practice
venously to the child before the procedure to suppress
marrow and T-lymphocyte production. This may cause nau- Do children continue to grow following hematopoietic
sea and vomiting. Total body irradiation to destroy the stem cell transplantation?
child’s marrow also may be done. This is a difficult time for To analyze the growth patterns of children following
the child because even with antiemetic therapy, total body ir- hematopoietic stem cell transplantation, researchers
radiation causes extreme nausea, vomiting, and diarrhea. secured anthropometric measurements on 35 children
To obtain hematopoietic stem cells from peripheral who received a transplant in a tertiary children’s hos-
blood, donors receive 5 days of a colony-stimulating factor to pital prior to their transplant and again at 2 and
promote the release of stem cells into the peripheral blood. 4 months afterward. The results of the study showed
Blood is then collected by a plasmapheresis technique. If um- that although height showed an increase, weight, skin-
bilical cord blood is used, it is drained from the placenta im- fold triceps, and mid-arm circumference showed a
mediately after birth. It is then cryopreserved in a cord blood decrease over the 4-month measurement period.
bank and infused into the recipient by usual blood infusion Almost all children experienced gastrointestinal symp-
technique. toms such as constipation, diarrhea, nausea, vomiting,
If the marrow will be taken directly from a donor, on the change in taste, dry mouth, and loss of appetite that
day of the procedure, the donor is admitted to the hospital could have interfered with nutrition and be a cause of
for a 1-day stay and receives epidural anesthesia or conscious the delayed growth.
sedation, as multiple bone marrow aspirations from the pos-
terior iliac crests are necessary for retrieval. The marrow is Based on the above study, would you be surprised if a
strained to remove fat and bone particles and any other child gained no weight in the 3 months since a hematopoi-
unwanted cells. An anticoagulant is added to prevent clot- etic stem cell transplant? Would it be important to assess
ting. It is then infused intravenously into the recipient’s gastrointestinal symptoms in all posttransplant children?
bloodstream. Source: Rodgers, C., et al. (2008). Growth patterns and gas-
Because an infused hematopoietic stem cell solution is trointestinal symptoms in pediatric patients after hematopoi-
fairly thick, the infusion takes 60 to 90 minutes. Do not use etic stem cell transplantation. Oncology Nursing Forum, 35(3),
a filter that is normally used for the infusion of blood prod- 443–448.
ucts, because this would filter out marrow tissue. Monitor
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CHAPTER 44 Nursing Care of a Family When a Child Has a Hematologic Disorder 1297

peripheral blood (approximately 3 weeks). On the day

Nursing Diagnoses and Related of discharge, parents may be surprised that the
Interventions child’s blood replacement is not complete and that
✽ they will need to continue infection control measures
and restrictions at home. Help them locate a support
Nursing Diagnosis: Anxiety related to lack of knowl-
edge about procedure and expected outcome of group in their community if possible. Be certain they
hematopoietic stem cell transplant feel free to call the transplant center after discharge if
they have any problems. Once the danger of infection
Outcome Evaluation: Parents state the reason for the has passed and the restrictions can be discontinued,
transplant; state they are agreeable to the procedure some parents may still be reluctant to allow their child
even though they know the transplant may not be suc- outside, fearing that the child may still be susceptible
cessful, depending on immunologic factors that are to infection. Frequent follow-up for the next year not
not totally known to science. only is necessary to ensure that a child is free of in-
Hematopoietic stem cell transplantation is an emo- fection but assesses whether the parents allow their
tional experience not only for the child but also for the child to pursue age-appropriate activities.
parents and the marrow donor. Be certain that both If children are prepared adequately for these painful
the child who receives the transplant and the donor or restrictive procedures and supported throughout,
understand they are not responsible for the outcome they should have no long-term consequences. Not all
of the transplant. Its success does not depend on hematopoietic stem cell transplants are successful,
their behavior or what kind of person they are but on however, and some children will die of the original dis-
immunologic factors over which they have no control. ease that necessitated the transplant. A risk is that
Be certain donors know that if bone marrow aspiration some children develop an infection despite all precau-
was done, the donor sites will feel tender afterward. tions and die of sepsis in the weeks immediately after
Conscious sedation will leave them feeling exhausted the transplant.
for several days. Donors who have undergone bone
marrow aspiration generally are asked to return to Graft-Versus-Host Disease
their primary care provider in 24 to 48 hours to be cer-
tain the aspiration sites are not infected (no local Graft-versus-host disease (GVHD) is a potentially lethal im-
swelling, redness, intense pain, or fever). munologic response of donor T cells against the tissue of the
bone marrow recipient (Saria & Gosselin-Acomb, 2007).
Nursing Diagnosis: Risk for delayed growth and devel-
The symptoms range from mild to severe and include a rash
opment related to extended restrictions and infection
and general malaise beginning 7 to 14 days after the trans-
control precautions in hospital or at home
plant. Latent virus infections may become active. Severe
Outcome Evaluation: Parents express satisfaction with symptoms include high fever and diarrhea and liver and
child’s ongoing development. Objective tests of de- spleen enlargement as cells are destroyed.
velopmental stage show child within age-appropriate Because there is no known cure for GVHD, prevention is
ranges. essential. Careful tissue typing; intravenous administration of
Children may be restricted from interacting with other an immunosuppressant such as methotrexate, a cortico-
children to prevent them from contracting an infection steroid, or cyclosporine before transplant; and irradiation of
following stem cell transplantation until the WBC count blood products (which helps to inactivate mature T lympho-
returns to a safe range. Because of this restriction, be cytes) before stem cell infusion all can help reduce the inci-
certain they are not isolated from health care providers. dence of this complication. Drugs such as methotrexate and
Visit the room frequently; provide sterilized play materi- cyclosporine kill all rapidly growing cells, including WBCs
als as appropriate. Most children grow tired of a re- and T lymphocytes, so administration of these drugs after
stricted diet and may crave fresh fruits and vegetables. transplantation cannot be continued because they would also
Thick-skinned fruits such as bananas and oranges can slow the growth of the host’s new stem cells. Depletion of
be given soon after the procedure, but unwashed foods mature T lymphocytes from donor bone marrow before it is
are avoided as they could carry bacteria. infused into the child offers good results, as does the admin-
Be certain children are well prepared for all proce- istration of corticosteroids or antithymocyte globulin (ATG),
dures. Allow them to make as many choices as they can an immune serum, to children after the transplant
about their care to help them preserve a sense of con- (Grosskreutz et al., 2007).
trol over their life. Children who receive a transplant
need periods of therapeutic play incorporated into their ✔Checkpoint Question 44.1
care so they can begin to express their anger and frus- Lana, who has thalassemia major, is scheduled for a bone
tration at the number of intravenous therapies or follow- marrow transplant. Which is the best instruction for her re-
up bone marrow aspirations they require. Measures to garding this?
help children cope with pain, such as imagery, can help
a child accept one more painful procedure. Encourage a. She must not move while the bone marrow is infused into her.
parents to spend time with their child during long peri- b. She will not be allowed to eat raw fruit following the transplant.
ods of hospitalization for additional support. c. Her hip bones will feel tender from the marrow transplantation.
Provisions for completing schoolwork need to be d. She will not need any further bone marrow aspirations
made as soon as a child has a return of RBCs in the after this.
1298 UNIT 7 The Nursing Role in Restoring and Maintaining the Health of Children and Families With Physiologic Disorders

Splenectomy Box 44.4 Assessment

One of the purposes of the spleen is to remove damaged or Assessing a Child With a
aged blood cells. This poses a problem with diseases such as Hematologic Disorder
sickle-cell anemia and the thalassemias because the spleen rec-
ognizes the typical cells of these diseases as damaged and de-
History
stroys them. This causes children with these disorders to have Chief concern: Fatigue, easy bruising, epistaxis.
a continuous anemia, with hemoglobin levels as low as 5 to Past medical history: Low birthweight; blood loss at birth; lack of vitamin K
administration at birth.
9 g/mL. In some children, therefore, removal of the spleen Nutrition: “Picky eater” or presence of pica. Increasd milk intake.
(splenectomy) will not cure the basic defect of the blood cells Past illnesses: History of recent illness; history of recent medicine ingestion
but will limit the degree of anemia (Owusu-Ofori & Hirst, Family history: Inherited blood disorder; parents known to have sickle-cell
trait, thalassemia minor, or hemophilia in family.
2009). Splenectomy formerly required a large abdominal in-
cision but today it can be performed by laparoscopy. Physical examination
A second function of the spleen is to strain the plasma for General appearance Possible significance
Obese infant Iron-deficiency anemia
invading microorganisms so that phagocytes and lympho- Fatigue Anemia
cytes can destroy them. This causes children who have had Eyes
their spleen removed to be very susceptible to pneumococcal Retinal hemorrhage Sickle-cell anemia
infections because this bacteria is no longer removed system- Face
atically from the body (Riddington & Owusu-Ofori, 2009). Bossing of Thalassemia
maxillary bone
After surgery, oral penicillin is given as a prophylactic an-
Mouth
tibiotic for a year or two to guard against infection. The Pale mucous membrane Iron-deficiency anemia
child should receive pneumococcal and meningococcal vac- Ecchymotic or Decreased coagulation
cines as well as routine immunizations, including influenza bleeding gumline ability

vaccine. Teach parents about signs of infection (cough, fever, Heart

Increased rate, Anemia
general malaise), and encourage them to report any such possible murmur
signs immediately. Skin
Petechiae, ecchymosis Decreased coagulation
Blood oozing from ability
wound or injection point
Jaundice Hemolytic anemia
HEALTH PROMOTION AND Pallor Anemia
Bronze color Frequent blood
RISK MANAGEMENT Abdomen transfusion
Pain on palpation Sickle-cell anemia
Hematologic disorders cover a wide range of diseases and Increased liver or Hemolytic anemia
produce multiple symptoms in children (Box 44.4). Many spleen size
disorders such as sickle-cell anemia and hemophilia are in- Genitourinary
Delayed secondary Sickle-cell anemia
herited. Health promotion and disease prevention, therefore, sex characteristics
begins with ensuring that families have access to genetic Extremities
counseling so they can be aware of the incidence of a disor- Spoon nails Iron-deficiency anemia
der in their family and the potential for the disease to develop Joint swelling, pain Hemophilia, sickle-cell
crisis
in their child. Neurologic
Weak muscle tone Iron-deficiency anemia
The most frequently occurring anemia in children, iron-
deficiency anemia, is preventable. This condition could be vir-
tually eliminated if all infants were breastfed and those infants
who are formula-fed were fed iron-fortified formula for the full
first year. Also, when cereal is introduced, iron-fortified types cream or topical lidocaine can greatly reduce the pain of
should be used. The disorder occurs again with a high inci- venipuncture. Helping a child to use a distraction technique
dence in adolescents because adolescent diets tend to be low in such as imagery can reduce apprehension or fear associated
meat and green vegetables, the chief dietary sources of iron. with the procedures or treatments.
Adolescents who begin vegetarian diets become especially
prone to developing the disorder. Counseling parents of young
children to maintain well-child health care visits and urging DISORDERS OF THE RED BLOOD CELLS
adolescents to ingest iron-rich foods could have a major impact
on decreasing the incidence of the disorder. Most RBC disorders fall into the category of the anemias, or
Aplastic anemia, or the inability to form blood elements, a reduction in the number or function of erythrocytes.
can be acquired if a child is exposed to a toxic drug or chem- Polycythemia, or an increase in the number of RBCs, can
ical. Educating parents about the importance of keeping also occur and may be as dangerous to a child as a reduction
poisons out of the reach of children could help decrease the in RBC production.
incidence of this disorder. Anemia occurs when the rate of RBC production falls
Nurses can also help make the therapy for these disorders below that of cell destruction, or when there is a loss of
much less painful and distressing than it was in the past. All RBCs, causing their number and the hemoglobin level to fall
hematologic disorders require obtaining blood specimens for below the normal value for a child’s age. Anemias are classi-
diagnosis and continued testing for follow-up. Many thera- fied according to the changes seen in RBC numbers or con-
pies include blood product transfusion. The use of EMLA figuration, or according to the source of the problem.
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CHAPTER 44 Nursing Care of a Family When a Child Has a Hematologic Disorder 1299

Although any reduction in the amount of circulating hemo- erythropoietin production. This decreases the stimulation for
globin lessens the oxygen-carrying capacity, clinical symp- RBC production in the bone marrow, and a resultant nor-
toms are not apparent until the hemoglobin level reaches 7 to mocytic, normochromic anemia occurs. Administration of
8 g/100 mL. Average values for hemoglobin and RBC num- recombinant human erythropoietin can increase RBC produc-
ber are shown in Appendix F. tion and correct the anemia, but not the renal disease (Tse,
Bunting, & Laughlin, 2008).
Normochromic, Normocytic Anemias
Anemia of Neoplastic Disease
Normochromic, normocytic anemias are marked by im-
paired production of erythrocytes by the bone marrow, or by Malignant growths such as leukemia or lymphosarcoma
abnormal or uncompensated loss of circulating RBCs, as (common neoplasms of childhood) result in normochromic,
with acute hemorrhage. The RBCs are normal in both color normocytic anemias because invasion of bone marrow by
and size, but there are simply too few of them. proliferating neoplastic cells impairs RBC production. There
may be accompanying blood loss if platelet formation also
Acute Blood-Loss Anemia has decreased. The treatment of such an anemia involves
measures designed to achieve remission of the neoplastic
Blood loss sufficient to cause anemia might occur from process and transfusion to increase the erythrocyte count.
trauma such as an automobile accident with internal bleed-
ing; from acute nephritis in which blood is lost in the urine; Hypersplenism
or in the newborn from disorders such as placenta previa,
premature separation of the placenta, maternal–fetal or twin- Under normal conditions, blood is filtered rapidly through
to-twin transfusion, or trauma to the cord or placenta, as the spleen. If the spleen is enlarged and functioning abnor-
might occur with cesarean birth. It can also occur from in- mally, blood cells pass through more slowly, with more cells
testinal parasites such as tapeworm (Barnett et al., 2007). being destroyed in the process. This increased destruction of
Children are in shock from acute blood loss and appear RBCs can cause anemia and may lead to pancytopenia (defi-
pale. As the heart attempts to push the reduced amount of ciency of all cell elements of blood). Virtually any underlying
blood through the body more rapidly, tachycardia will occur. splenic condition can cause this syndrome.
Loss of RBCs needed for oxygen transport causes body cells Therapeutic management consists of treating the underly-
to register an oxygen deficit, and children begin to breathe ing splenic disorder, including possible splenectomy.
rapidly. Newborns may have gasping respirations, sternal re- Although the spleen’s role in the body’s defense mechanisms
tractions, and cyanosis. They will not respond to oxygen against infection is not well documented, the organ appears
therapy because they lack RBCs to transport and use the oxy- to be relatively important in early infancy. Its function de-
gen. Such infants become listless and inactive. creases as a child grows older, and it may serve no immune
This type of acute blood-loss anemia generally is transi- function at all in adulthood. If the spleen is removed, there
tory because the sudden reduction in available oxygen stim- is no decrease in general immunity or in gamma globulin or
ulates the release of erythropoietin from the kidney and a antibody formation. With the removal of the spleen’s filter-
regeneration response in the bone marrow. The reticulocyte ing function, however, there seems to be an increased sus-
count becomes elevated, evidence the bone marrow is trying ceptibility to meningitis or pneumonia due to pneumococci.
to increase production of erythrocytes to meet the sudden For this reason, a splenectomy may be delayed until after
shortage. 2 years of age, when the risk of meningitis decreases. Such
Treatment involves control of bleeding by addressing its children should receive immunization against influenza,
underlying cause. The child or infant should be placed in a pneumococci, and H. influenzae in addition to prophylactic
supine position to provide as much circulation as possible to penicillin for 2 years after the splenectomy.
brain cells. Keep the child warm with blankets; place an in-
fant in an incubator or under a radiant heat warmer. Blood Aplastic Anemias
transfusion may be necessary to provide an immediate in- Aplastic anemias result from depression of hematopoietic ac-
crease in the number of erythrocytes. Until blood is available tivity in the bone marrow. The formation and development
for transfusion, a blood expander such as plasma or intra- of WBCs, platelets, and RBCs can all be affected.
venous fluid such as normal saline or Ringer’s lactate may be Congenital aplastic anemia (Fanconi syndrome) is inherited
given to expand blood volume and improve blood pressure. as an autosomal recessive trait. A child is born with several con-
genital anomalies, such as skeletal and renal abnormalities, hy-
Anemia of Acute Infection pogenitalism, and short stature. Between 4 and 12 years of age,
Acute infection or inflammation, especially in infants, may a child begins to manifest symptoms of pancytopenia, or re-
lead to increased destruction or decreased production of ery- duction of all blood cell components (Linker, 2009).
throcytes. Common conditions include osteomyelitis, ulcer- Acquired aplastic anemia is a decrease in bone marrow
ative colitis, and kidney infection. Management involves production that can occur if a child is exposed excessively to
treatment of the underlying condition. When this is reversed, radiation, drugs, or chemicals known to cause bone marrow
the blood values will return to normal. damage. Drugs that may cause this include chloramphenicol,
sulfonamides, arsenic (contained in rat poison, sometimes
Anemia of Renal Disease eaten by children), hydantoin, benzene, or quinine. Exposure
to insecticides also may cause severe bone marrow dysfunc-
Either acute or chronic renal disease can cause loss of function tion. Chemotherapeutic drugs temporarily reduce bone mar-
in kidney cells, and this causes an accompanying decrease in row production. A serious infection such as meningococcal
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1300 UNIT 7 The Nursing Role in Restoring and Maintaining the Health of Children and Families With Physiologic Disorders

pneumonia might cause autoimmunologic suppression of the

bone marrow. Nursing Diagnoses and Related
Assessment. When symptoms begin, a child appears pale, fa-
Interventions
tigues easily, and has anorexia. These symptoms reflect the ✽
lower RBC count (anemia) and tissue hypoxia. Because of re- Nursing Diagnosis: Risk for infection related to dra-
duced platelet formation (thrombocytopenia), the child matic decrease in number of WBCs
bruises easily or has petechiae (pinpoint, macular, purplish- Outcome Evaluation: Child’s temperature remains
red spots caused by intradermal or submucous hemorrhage). below 100° F (38.0° C) axillary; symptoms of infection
A child may have excessive nosebleeds or gastrointestinal such as cough, vomiting, or diarrhea are absent.
bleeding. As a result of a decrease in WBCs (leukopenia), a Exposure to other children must be limited as long as
child may contract an increased number of infections and re- WBC production is inadequate to prevent infection.
spond poorly to antibiotic therapy. Observe closely for signs Remind parents of the signs and symptoms of infec-
of cardiac decompensation such as tachycardia, tachypnea, tion and advise them to come for treatment promptly if
shortness of breath, or cyanosis from the long-term increased their child shows any of these signs. In the absence of
workload on the heart. Ask about any exposure to drugs or granulocytes, however, antibiotic therapy may be in-
chemicals or recent infection. Bone marrow samples will effective, and severe septicemia can result. WBCs
show a reduced number of blood elements; blood-forming (granulocytes) may be transfused to counteract a se-
spaces become infiltrated by fatty tissue. vere infection.
Therapeutic Management. The ultimate therapy for both con- Nursing Diagnosis: Risk for disturbed body image re-
genital and acquired aplastic anemia is hemopoietic stem cell lated to changed appearance occurring as medication
transplantation (Gluckman et al., 2007). If a donor cannot side effect
be located, the disease is managed by procedures to suppress Outcome Evaluation: Child views self as a worthwhile
T-lymphocyte–dependent autoimmune responses with an- person; does not appear to be excessively shy or re-
tithymocyte globulin (ATG) or cyclosporine or transfusion of luctant to interact with peers.
new blood elements (Ambruso, Hays, & Goldenberg, 2008).
ATG, given intravenously, must always be administered cau- Children who receive corticosteroids such as pred-
tiously because of the high risk for anaphylaxis. Packed RBCs nisone to suppress the immune response almost
and platelet transfusions are generally necessary to maintain ad- always experience some of the drug’s side effects,
equate blood elements. Prophylactic platelet transfusions may such as a cushingoid appearance, hirsutism, hyper-
be given (Ferrara et al., 2007). An RBC-stimulating factor (ery- tension, and marked weight gain. Long-term therapy
thropoietin) may be helpful. Colony-stimulating factors may with testosterone can result in masculinizing effects,
also improve bone marrow function. Some children show im- such as growth of facial and body hair, the develop-
provement with a course of an oral corticosteroid (prednisone). ment of acne, and deepening of the voice. Be sure
Testosterone to stimulate RBC growth may be tried. both children and their parents know that these effects
For children who receive a hematopoietic stem cell trans- are related to the medication and that they will remain
plant, chances of complete recovery are good. For others, for an extended period but will fade when the medica-
the course is uncertain. A decreased platelet count may per- tion is withdrawn.
sist for years after other blood elements have returned to Adolescents may have an especially difficult time
normal. Bleeding, therefore, especially petechiae or purpura, accepting weight gain and increased acne. They
may be a long-term problem. Any drug or chemical sus- need a chance to express their feelings about their
pected of causing the bone marrow dysfunction must be dis- changed appearance. Reinforce and emphasize pos-
continued at once and the child must never be exposed to itive attributes.
that substance again. Children with aplastic anemia are apt Nursing Diagnosis: Risk for injury related to ineffective
to be irritable because of their fatigue and recurring symp- blood clotting mechanisms secondary to inadequate
toms. Their parents may feel responsible for causing the ill- platelet formation
ness if it originated from exposure to a chemical such as an
Outcome Evaluation: Child exhibits no ecchymotic skin
insecticide. Many parents will have less confidence in health
areas, gingival bleeding, or epistaxis; stools are neg-
care personnel if the illness followed treatment with a drug
ative for occult blood.
such as chloramphenicol. They wonder how they can trust
in a drug to cure the illness if they believe that one drug Inadequate platelet formation interferes with blood co-
caused the illness. How can they trust that their child will agulation, placing a child at risk for bleeding (Box 44.5).
not be harmed further?
When discussing with parents the outcome of this disease, Hypoplastic Anemias
be conservatively optimistic. It may be easier for parents to
deal with this problem if they face only one day or one blood Hypoplastic anemias also result from depression of
test at a time rather than trying to predict the outcomes of all hematopoietic activity in bone marrow; they can be either
the blood tests to come. They need to feel they can discuss congenital or acquired. Unlike aplastic anemias, in which
their frustration and bitterness about continual abnormal re- WBCs, RBCs, and platelets are affected, in hypoplastic ane-
sults with health care personnel. Establishing good commu- mias only RBCs are affected.
nication with these parents does much to establish their trust Congenital hypoplastic anemia (Blackfan-Diamond syn-
in everyone caring for their child. drome) is a rare disorder that shows symptoms as early as
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CHAPTER 44 Nursing Care of a Family When a Child Has a Hematologic Disorder 1301

period. This is one of the few times that an infusion is given

BOX 44.5 ✽ Techniques for Reducing Bleeding subcutaneously. Parents can do this at home after careful in-
With Thrombocytopenia struction, often while their child is asleep at night. The parent
must assess that voiding is present and specific gravity is nor-
Some techniques for reducing bleeding due to in- mal (1.003 to 1.030) before drug administration.
adequate platelet formation include: For such an infusion, an area beside the scapula or on the
• Limit the number of blood-drawing procedures; thigh is cleaned with alcohol; a short 25-gauge needle is in-
combine samples whenever possible. serted at a low angle into only the subcutaneous tissue. The
• Use a blood pressure cuff instead of a tourni- medication is then allowed to infuse slowly. Periodic slit-
quet to reduce the number of petechiae. lamp eye examinations should be scheduled to check for
• Apply pressure to any puncture site for a full 5 cataract formation, a possible adverse effect of deferoxamine.
minutes before applying a bandage. Congenital hypoplastic anemia is a chronic condition.
• Minimize use of adhesive tape to the skin However, approximately one fourth of affected children will
(pulling for removal may tear the skin and undergo spontaneous permanent remission before age
cause petechiae). 13 years. If not, they are candidates for hematopoietic stem
• Pad side and crib rails to prevent bruising. cell transplantation. Both the child and the parents need sup-
• Protect intravenous sites to avoid numerous port from health care personnel to help them accept the
reinsertions. many procedures and tests required.
• Administer medication orally or by intravenous
infusion when appropriate to minimize the
number of injection sites.
✔Checkpoint Question 44.2
• Assess that the child is offered foods that can Lana has received iron chelation therapy in the past. Iron chela-
be chewed without irritation (avoid toast tion therapy is:
crusts, for example). a. A procedure to remove excess iron from the child’s body.
• Urge the child to use a soft toothbrush. b. A procedure to help iron move effectively into hemoglobin.
• Check toys for sharp corners, which may c. A therapy to increase the iron level in bone and muscle cells.
cause scratches. Urge the child to be careful d. A therapy to convert iron into calcium to increase heart action.
with paper, because paper cuts can bleed out
of proportion to their size.
• Assess the need for routine blood pressure Hypochromic Anemias
determinations. Tight cuffs could lead to
When hemoglobin synthesis is inadequate, the erythrocytes
petechiae.
appear pale (hypochromia). Hypochromia is generally ac-
• Distract the child from rough play; suggest
companied by a reduction in the diameter of cells (RBCs are
stimulating but quiet activities to minimize risk
also microcytic).
of injury.
• Keep a record of blood drawn; do not draw
Iron-Deficiency Anemia
extra amounts “just in case” so children do not
become more anemic. Although the incidence of iron-deficiency anemia is decreasing
in the United States due to improved infant nutrition, it is still
the most common anemia of infancy and childhood, occurring
when the intake of dietary iron is inadequate. Most iron in the
body is incorporated in hemoglobin, but an additional amount
the first 6 to 8 months of life. It affects both sexes and is ap- is stored in the bone marrow to be available for hemoglobin
parently caused by an inherent defect in RBC formation. production. Inadequate dietary iron prevents proper hemoglo-
No changes in the leukocytes or platelets occur. An acquired bin formation. With iron-deficiency anemia, RBCs are both
form is caused by infection with parvovirus, the infectious small in size (hypocytic) and pale (hypochromic) due to the
agent of fifth disease (Servey, Reamy, & Hodge, 2007). stunted hemoglobin.
The onset of hypoplastic anemia is insidious, and at first Children are at high risk for iron-deficiency anemia be-
it may be difficult to differentiate from iron-deficiency ane- cause they need more daily iron in proportion to their body
mia. In iron-deficiency anemia, blood cells appear weight to maintain an adequate iron level than do adults.
hypochromic and microcytic; in hypoplastic anemia, they are This results in a necessary daily intake of 6 to 15 mg of iron.
normochromic and normocytic but few in number. Iron-deficiency anemia occurs most often between ages
With acquired hypoplastic anemia, the reduction of RBCs 9 months and 3 years; its frequency rises again in adoles-
is transient, so no therapy is necessary. Children with the con- cence, when iron requirements increase for girls who are
genital form show increased erythropoiesis with corticosteroid menstruating. It also is found in overweight teenagers if they
therapy. Long-term transfusions of packed RBCs are needed ingest most of their calories from high-carbohydrate, not
to raise erythrocyte levels. As a result of the necessary number iron-rich, foods (Brotanek et al., 2007).
of transfusions, hemosiderosis (deposition of iron in body
tissue) can occur. Therefore, an iron chelation program such Causes in Infants. Several causes lead to iron-deficiency ane-
as subcutaneous infusion (hypodermoclysis) of deferoxam- mia. When an infant’s diet lacks sufficient iron, the infant
ine (Desferal) may be started concurrently with transfusions. usually has enough in reserve to last for the first 6 months.
Deferoxamine binds with iron and aids its excretion from the After that, if the infant’s diet continues to be iron deficient,
body in urine; it is given 5 or 6 days a week over an 8-hour there will be difficulty forming adequate RBCs. Infants of
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1302 UNIT 7 The Nursing Role in Restoring and Maintaining the Health of Children and Families With Physiologic Disorders

low birth weight have fewer iron stores than those born at
term because iron stores are laid down near the end of ges-
Box 44.6 Assessment
tation. Because low-birth-weight infants also grow rapidly Assessing a Child With
and their need for RBCs expands accordingly, they tend to Iron-Deficiency Anemia
develop iron-deficiency anemia before 5 to 6 months. As a
preventive measure, they may be given an iron supplement
beginning at about 2 months of age.
Women with iron deficiency during pregnancy tend to Pale mucous
give birth to iron-deficient babies because the babies do not membrane
receive iron stores. Low hemoglobin levels from iron-
deficiency anemia lead to diffusion of plasma proteins such as
albumin and gamma globulin out of the bloodstream by os-
mosis. The loss of transferrin, a plasma protein responsible Enlarged heart
for binding iron to protein to facilitate its transportation to (possible)
bone marrow after absorption from the gastrointestinal tract, Enlarged spleen
further depletes this system of iron transport. (possible)
Infants born with structural defects of the gastrointestinal
system, such as gastroesophageal reflux (chalasia—an imma-
ture valve between the esophagus and stomach, resulting in
regurgitation) or pyloric stenosis (narrowing between the
Poor
stomach and duodenum, resulting in vomiting), are particu- muscle tone;
larly prone to iron-deficiency anemia. Although their diet is decreased
adequate, they cannot make use of the iron because it is never activity
adequately digested. Infants with chronic diarrhea are also
prone to this form of anemia due to inadequate absorption.
Some infants develop minimal gastrointestinal bleeding if fed
cow’s milk; this is why breast milk or commercial formula is
recommended for the full first year.
Iron-deficiency anemia can be prevented in formula-fed
infants by giving them iron-fortified formula. If an infant is
breastfed, iron-fortified cereal should be introduced when
solid foods are introduced in the first year. Fortunately, these
cost no more than nonfortified foods. Occasionally, an infant than 32 oz of milk a day. Infants with iron-deficiency anemia
becomes constipated while ingesting iron-rich formula, but may be drinking up to 50 oz a day. One quart of milk pro-
this is the exception rather than the rule. vides only approximately 0.5 mg of iron. In contrast, 1 ta-
blespoon of iron-fortified baby cereal supplies 2.5 to 5.0 mg
Causes in Older Children. In children older than 2 years, of iron.
chronic blood loss is the most frequent cause of iron-deficiency As iron-deficiency anemia develops, laboratory studies will
anemia. This is caused by gastrointestinal tract lesions such as reveal a decreased hemoglobin level (a hemoglobin level less
polyps, ulcerative colitis, Crohn’s disease, protein-induced en- than 11 g/100 mL of blood) and reduced hematocrit level
teropathies, parasitic infestation, or frequent epistaxis. (below 33%). The RBCs are microcytic and hypochromic and
Adolescent girls can become iron deficient because of fre- possibly poikilocytic (irregular in shape). The mean corpus-
quent attempts to diet and overconsumption of snack foods cular volume is low. The mean corpuscular hemoglobin may
low in iron. Without sufficient iron, their body cannot com- be reduced. Serum iron levels are normally 70 ␮g/100 mL;
pensate for the iron lost with menstrual flow. with iron-deficiency anemia the level is often as low as
Assessment. Common symptoms of iron-deficiency ane- 30 ␮g/100 mL, with an increased iron-binding capacity (more
mia are shown in Box 44.6. Children with iron-deficiency than 350 ␮g/100 mL). The level of serum ferritin reflects the
anemia appear pale. Because the pallor develops slowly, how- extent of iron stores so is less than 10 ␮g/100 mL (normal
ever, parents may not realize how extensive it is. They may is 35 ␮g/mL). Without iron, heme precursors cannot be used,
describe their child as “fair-skinned” even though the child’s so free erythrocyte protoporphyrins increase to more than
pallor is so extreme the skin is transparent. In dark-skinned 10 ␮g/g from a normal of 1.9 ␮g/g.
infants, pale mucous membranes may be the most significant Monoamine oxidase (MAO) is an enzyme important for
finding. central nervous system maturation. Iron is incorporated into
Infants may show poor muscle tone and reduced activity. MAO, so without iron this necessary enzyme is absent and
They are generally irritable from fatigue. Their heart may be central nervous system maturation may be affected.
enlarged, and there may be a soft systolic precordial murmur There may be an association in school-age children between
as the heart increases its action, attempting to supply body iron-deficiency anemia and poor school achievement, probably
cells with more oxygen. The spleen may be slightly enlarged. related to chronic fatigue. Iron-deficiency anemia is also asso-
Fingernails become typically spoon-shaped or depressed in ciated with pica (the eating of inedible substances such as dirt
contour. and paper). Eating ice cubes is common in adolescents. Until
A 24-hour dietary history generally reveals an abnormally the anemia is corrected, parents need to supervise the child’s
high milk intake. As a rule, infants should not ingest more environment to keep inedible materials out of his or her reach.
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CHAPTER 44 Nursing Care of a Family When a Child Has a Hematologic Disorder 1303

Outcome Evaluation: Parents report child’s dietary in-

BOX 44.7 ✽ Focus on take includes iron-rich foods; parents administer fer-
Pharmacology rous sulfate as prescribed; serum iron levels increase
to normal by 6 months.
Ferrous Sulfate (Feosol)
When planning care for an infant with iron-deficiency
Classification: Ferrous sulfate is an iron salt. anemia, it is helpful to minimize the child’s activities to
Action: Supplies iron for red cell production. It elevates prevent fatigue, particularly at mealtime, as a fatigued
the serum iron concentration and then is converted child is more reluctant to eat any food, let alone eat
to hemoglobin or trapped in the reticuloendothelial iron-rich foods.
cells for storage and eventual conversion to a usable Counsel parents on measures to improve their in-
form of iron (Karch, 2009). fant’s nutrition, such as adding iron-rich foods while
Pregnancy risk category: A decreasing formula or breast milk intake. If the child is
Dosage: For severe iron-deficiency anemia: 4 to 6 mg/kg/ not fond of meat, suggest parents substitute cheese,
day, in three divided doses. For mild iron-deficiency eggs, green vegetables, or fortified cereal. Because
anemia: 3 mg/kg/day in two divided doses. iron-rich foods are often expensive, remind parents
Possible adverse effects: Gastrointestinal upset, that these items are important and that they should
anorexia, nausea, vomiting, constipation, dark stools, not substitute less expensive, high-carbohydrate
stained teeth (liquid preparations) foods. Before iron therapy is started, help parents cre-
Nursing Implications ate reminder sheets so they can manage to give the
supplement over a long period of time. Alert parents
• Instruct parents to administer the drug on an empty to possible side effects, such as stomach irritation,
stomach with water to enhance absorption. If this constipation, and that liquid iron preparations can
causes gastrointestinal irritation, administer it after stain teeth if not taken through a straw. Iron is
meals. Avoid giving it with milk, eggs, coffee, or tea. absorbed best with an accompanying acid medium
• If the liquid preparation is ordered, advise parents to so ascorbic acid may also be prescribed (or the par-
mix it with water or juice to mask the taste and pre- ent be advised to give the iron medication with orange
vent staining of teeth. Encourage the parents to have juice) to increase absorption. To avoid constipation,
the child drink the medication through a straw to the child may need additional fiber like that supplied
avoid staining of the teeth. by green leafy vegetables. If oral iron is not tolerated
• Keep in mind that iron is absorbed best in the pres- or if there is a doubt the child will take it, an iron-
ence of vitamin C. Suggest parents give the iron with dextran injection (Imferon) can be given intramus-
a citrus juice such as orange juice to help absorption. cularly. Imferon stains the skin and is extremely
Some children may be prescribed vitamin C to take irritating unless it is given by deep Z-track intramus-
concurrently to increase absorption. cular injection.
• Inform child and parents that iron may turn stools Of all age groups, adolescents tend to do the least
black. well with taking medicine consistently. Help them plan
• Encourage parents to include high-fiber foods in the a daily time for taking their iron supplement with a
child’s diet to minimize the risk of constipation. medication reminder chart. At first, they may reject
• Reinforce the need for thorough brushing of teeth to this as childish, but assure them that everyone needs
prevent staining. these charts. Review with them the iron-rich foods
• Remind parents about the need for follow-up blood they will need to eat daily. An iron supplement is ef-
studies to evaluate the effectiveness of the drug. fective only 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 adequate iron and the rapid prolifera-
tion of new erythrocytes is correcting the anemia. Iron
Therapeutic Management. Therapy for iron-deficiency medication must be taken for at least 4 to 6 weeks
anemia focuses on treatment of the underlying cause. after the RBC count is normal to rebuild iron levels in
Sources of gastrointestinal bleeding must be ruled out. The the blood. In some children, maintenance therapy
diet must be rich in iron and should contain extra vitamin may continue for as long as a year.
C, as this enhances iron absorption. 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 Chronic Infection Anemia
(Domellof, 2007) (Box 44.7).
Acute infection interferes with RBC production, producing
a normochromic, normocytic anemia. When infections are
chronic, anemia of a hypochromic, microcytic type occurs.
Nursing Diagnoses and Related This is probably caused by impaired iron metabolism as well
Interventions as impaired RBC production.
✽ The degree of anemia is rarely as severe as that occurring
Nursing Diagnosis: Imbalanced nutrition, less than body with iron deficiency. Administration of iron has little effect
requirements, related to inadequate ingestion of iron until the infection is controlled (Adamson & Longo, 2008).
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1304 UNIT 7 The Nursing Role in Restoring and Maintaining the Health of Children and Families With Physiologic Disorders

Macrocytic (Megaloblastic) Anemias Congenital Spherocytosis

A macrocytic anemia is one in which the RBCs are abnor- Congenital spherocytosis is a hemolytic anemia that is inher-
mally large (Primack & Mahaniah, 2008). These cells are ited as an autosomal dominant trait. It occurs most frequently
actually immature erythrocytes or megaloblasts (nucleated in the white Northern European population (Adamson &
immature red cells). For this reason, these anemias are often Longo, 2008). RBCs are small and defective, apparently due
referred to as megaloblastic anemias. Because these anemias to abnormalities of the protein of the cell membrane that
are caused by nutritional deficiencies, they occur less fre- make them unusually permeable to sodium. The life span of
quently in the United States than in developing countries. erythrocytes is diminished.
The disease may be noticed shortly after birth, although
Anemia of Folic Acid Deficiency symptoms may appear at any age. The hemolysis of RBCs
appears to occur in the spleen, apparently from excessive
A deficiency of folic acid combined with vitamin C defi- absorption of sodium into the cell. The abnormal cell
ciency produces an anemia in which the erythrocytes are ab- swells, ruptures, and is destroyed. Chronic jaundice and
normally large. There is often accompanying neutropenia splenomegaly develop. The mean corpuscular hemoglobin
and thrombocytopenia. Mean corpuscular volume and mean concentration is increased because the cells are small.
corpuscular hemoglobin are increased, whereas mean corpus- Gallstones may be present in the older school-age child and
cular hemoglobin concentration is normal. Bone marrow will adolescent because of the continuous hemolysis, bilirubin
contain megaloblasts, indicating inhibition of the production release, and incorporation of bilirubin into gallstones.
of erythrocytes at an early stage. Megaloblastic arrest, or in- Infections may precipitate a crisis or cause bone marrow
ability of RBCs to mature past an early stage, may occur in failure. During such a period, the anemia increases rapidly as
the first year of life from the continued use of infant food the hemolysis continues. Blood transfusion will be necessary
containing too little folic acid or from an infant drinking to maintain a sufficient number of circulating erythrocytes
goat’s milk, which tends to be deficient in folic acid. until the crisis passes.
Treatment is daily oral administration of folic acid. Response The diagnosis of the disease is based on family history, the
to treatment is dramatic. obvious hemolysis, and the presence of the abnormal sphero-
cytes. The treatment is generally splenectomy at approxi-
Pernicious Anemia (Vitamin B12 Deficiency) mately 5 to 6 years. This measure will increase the number of
Vitamin B12 is necessary for maturation of RBCs. Pernicious RBCs present but will not alter their abnormal structure.
anemia results from deficiency or inability to use the vitamin
(Waterbury, 2007). In children, the cause is more often lack Glucose-6-Phosphate Dehydrogenase Deficiency
of ingestion of vitamin B12. The vitamin is found primarily in
foods of animal origin, including both cow’s milk and breast The enzyme glucose-6-phosphate dehydrogenase (G6PD) is
milk. Adolescents may be deficient in vitamin B12 if they are necessary for maintenance of RBC life. Lack of the enzyme re-
ingesting a long-term, poorly formulated vegetarian diet. sults in premature destruction of RBCs. The disease is trans-
For absorption of vitamin B12 from the intestine, an in- mitted as a sex-linked recessive trait. It occurs most frequently
trinsic factor must be present in the gastric mucosa. In adults, in children of African American, Asian, Sephardic Jewish, and
lack of the intrinsic factor is the most frequent cause of the Mediterranean descent. Approximately 10% of African
disorder. If a child has an intrinsic factor deficiency, symp- American males have the disorder (Waterbury, 2007). Because
toms can occur as early as the first 2 years of life (once the in- the disease is sex-linked, males of high-risk groups should be
trauterine stores of vitamin B12 have been exhausted). The screened in infancy.
child appears pale, anorexic, and irritable, with chronic diar- G6PD occurs in two identifiable forms. Children with
rhea. The tongue appears smooth and beefy red due to pap- congenital nonspherocytic hemolytic anemia have hemolysis,
illary atrophy. In children, neuropathologic findings such as jaundice, and splenomegaly and may have aplastic crises.
ataxia, hyporeflexia, paresthesia, and a positive Babinski re- Other children have a drug-induced form in which the blood
flex are less noticeable than in adults. pattern is normal until the child is exposed to fava beans or
Laboratory findings reveal low serum levels of vitamin drugs such as antipyretics, sulfonamides, antimalarials, and
B12. The rate and efficiency of absorption of vitamin B12 can naphthaquinolones (the most common drug in these groups
be tested by the ingestion of the radioactively tagged vitamin. is acetylsalicylic acid [aspirin]). Approximately 2 days after
The dose absorbed in the presence and absence of a dose of ingestion of such an oxidant drug, the child begins to show
intrinsic factor can be measured. evidence of hemolysis.
If the anemia is caused by a B12-deficient diet, temporary in- A blood smear will show Heinz bodies (oddly shaped par-
jections of B12 will reverse the symptoms. If the anemia is ticles in RBCs). The degree of RBC destruction depends on
caused by lack of the intrinsic factor, lifelong monthly intra- the drug and the extent of exposure to it. The child may have
muscular injections of B12 may be necessary. Parents and the accompanying fever and back pain. Occasionally a newborn
child need to understand that lifelong therapy will be necessary. is seen with marked hemolysis because the mother ingested
an initiating drug during pregnancy.
Hemolytic Anemias G6PD deficiency may be diagnosed by a rapid enzyme
screening test or electrophoretic analysis of RBCs. Drug-
Hemolytic anemias are those in which the number of ery- induced hemolysis usually is self-limiting, and blood transfu-
throcytes decreases due to increased destruction of erythro- sions are rarely necessary. Both parents and children should be
cytes. This may be caused by fundamental abnormalities of told of the abnormality in the child’s metabolism so they can
erythrocyte structure or by extracellular destruction forces. avoid common drugs such as acetylsalicylic acid.
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CHAPTER 44 Nursing Care of a Family When a Child Has a Hematologic Disorder 1305

Sickle-Cell Anemia Box 44.8 Assessment

Sickle-cell anemia is the presence of abnormally shaped Assessing a Child
(elongated) RBCs. It is an autosomal recessive inherited dis- With Sickle-Cell Crisis
order on the beta chain of hemoglobin; the amino acid valine
takes the place of the normally appearing glutamic acid. The
erythrocytes become characteristically elongated and cres-
cent-shaped (sickled) when they are submitted to low oxygen Fever
tension (less than 60% to 70%), a low blood pH (acidosis), Yellow sclera
or increased blood viscosity, such as occurs with dehydration Vomiting
or hypoxia. When RBCs sickle, they can not move freely
through vessels. Stasis and further sickling occur (a sickle-cell
crisis). Blood flow halts and tissue distal to the blockage be-
comes ischemic, resulting in acute pain and cell destruction Enlarged liver
(Linker, 2009).
Acute back pain Enlarged spleen
Because fetal hemoglobin contains a gamma, not a beta,
chain, the disease usually will not result in clinical symptoms Possible kidney
until a child’s hemoglobin changes from the fetal to the adult infarction
form at approximately 6 months. However, the disease can Painful and
be diagnosed prenatally by chorionic villi sampling or from swollen hands
cord blood during amniocentesis. If these were not done, it
can be identified at birth by neonatal screening (Lees, Davies, Acute
& Dezateux, 2009). The abnormal form of hemoglobin in abdominal Joint pain,
this disorder is designated hemoglobin S. A child with sickle- pain and swelling, and
cell disease is said to have hemoglobin SS (homozygous tenderness warmth
involvement).
Sickle-cell disease occurs in about 1 of every 400 African
American infants in the United States. Sickle-cell trait occurs
in approximately 1 in 12 (Ambruso, Hayes, & Goldenberg,
2008).
Both parents of the child with the disease will have both
normal adult and hemoglobin S or be carriers (heterozygous)
of the sickle-cell trait (have hemoglobin AS). In people with
the trait, approximately 25% to 50% of hemoglobin pro- there is renal involvement, hematuria or flank pain may be
duced is abnormal. They produce enough normal hemoglo- present.
bin to compensate for the hemoglobin that is sickled and Additional types of crisis may occur.
therefore show no symptoms. A child with the disease (ho- • A sequestration crisis may occur when there is splenic se-
mozygous) produces no normal hemoglobin and so shows questration of RBCs or severe anemia occurs due to pool-
characteristic symptoms of sickle-cell anemia. A very few ing and increased destruction of sickled cells in the liver
children have combinations of hemoglobin S and hemoglo- and spleen). This leads to shock from hypovolemia. The
bin C or E, leading to mild anemia (Creary, Williamson, & spleen is enlarged and tender.
Kulkarni, 2007). • A hyperhemolytic crisis can occur when there is increased
Sickle-Cell Crisis. Sickle-cell crisis is the term used to denote destruction of RBCs. A megaloblastic crisis may occur if
a sudden, severe onset of sickling. Symptoms of crisis occur the child has folic acid or vitamin B deficiency (new
from pooling of many new sickled cells in vessels and conse- RBCs cannot be fully formed due to lack of these ingre-
quent tissue hypoxia beyond the blockage (a vaso-occlusive dients).
crisis). A sickle-cell crisis can occur when a child has an illness • An aplastic crisis is manifested by severe anemia due to a
causing dehydration or a respiratory infection that results in sudden decrease in RBC production. This form usually
lowered oxygen exchange and a lowered arterial oxygen level, occurs with infection.
or after extremely strenuous exercise (enough to lead to tissue Assessment for Sickle-Cell Anemia. Hemoglobin elec-
hypoxia). Sometimes no obvious cause of a crisis can be trophoresis is used to diagnose sickle-cell anemia at birth
found (Box 44.8). Symptoms are sudden, severe, and painful from the few red cells that have already converted to their
(see Focus on Nursing Care Planning Box 44.9). Laboratory adult form. At approximately 6 months of age, children
reports reveal a hemoglobin level of only 6 to 8 g/100 mL. A with sickle-cell disease begin to show initial signs of fever
peripheral blood smear demonstrates sickled cells. The WBC and anemia. Stasis of blood and infarction may occur in any
count is often elevated to 12,000 to 20,000/mm3. Bilirubin body part, leading to local pain. Some infants have swelling
and reticulocyte levels are increased. of the hands and feet (a hand–foot syndrome) probably
Further complications that may occur are aseptic necro- caused by aseptic infarction of the bones of the hands and
sis of the head of the femur or humerus with increased joint feet. Children with sickle-cell anemia tend to have a slight
pain or a cerebrovascular accident that occurs from a build and characteristically long arms and legs. They may
blocked artery, causing loss of motor function, coma, have a protruding abdomen because of an enlarged spleen
seizures, or even death (Owusu-Ofori & Hirst, 2009). If and liver. In adolescence, the spleen size may decrease from
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1306 UNIT 7 The Nursing Role in Restoring and Maintaining the Health of Children and Families With Physiologic Disorders

BOX 44.9 ✽ Focus on Nursing Care Planning

A Multidisciplinary Care Map for a Child With Sickle-Cell Anemia

Joey is a 7-year-old with sickle-cell anemia. He is seen in the emergency room for a vaso-occlusive crisis.

Family Assessment ✽ Child lives with two older over meals. Has a history of two former vaso-occlusive
brothers (10 and 12) and both parents in a 3-bedroom crises. Missed last regularly scheduled health assess-
home. Father works as a distributor for a local water ment 2 weeks ago, as father had difficulty taking off
bottling company. Mother, an x-ray technician, is from work for visit. Was playing “tag” with older brothers
temporarily on duty with the National Guard in the on local beach this afternoon. Sclera was jaundiced and
Middle East. Father rates finances as, “Not good. child was crying from pain by time father returned from
Medical bills kill us.” work. Hemoglobin 6 g/100 mL; hematocrit 31%.
Nursing Diagnosis ✽ Altered tissue perfusion related to
Client Assessment ✽ Thin, black boy whose weight is
vaso-occlusive crisis
at only 5th percentile for age. Was screened and
diagnosed with sickle-cell anemia at birth. Described as Outcome Criteria ✽ Oxygen saturation level is main-
“picky eater”; has eaten almost no meat since mother is tained at 95% or higher; pain decreases to tolerable
not at home. Father states “I don’t have time to fuss” level; symptoms of hemolytic crisis decrease.

Team Member
Responsible Assessment Intervention Rationale Expected Outcome

Activities of Daily Living

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

Consultations

Nurse Assess if hematology Meet with hematology Repeated vaso- Hematology service
practitioner/ service is needed service as needed occlusive crises meets with
physician for consult. for emergency and suggest family physician/nurse
long-range consult. needs better practitioner and
management parent and child
strategies. as indicated.

Procedures/Medications

Nurse/nurse Assess degree of Administer prescribed Vaso-occlusive crises Child rates pain as no
practitioner child’s pain by narcotic as required. can cause sharp higher than 2
use of FACES pain that requires following analgesia
pain scale. strong analgesia. administration.
Nurse Assess O2 saturation Administer oxygen by O2 saturation decreases Child cooperates with
level by pulse face mask to keep as sickled cells are pulse oximetry and
oximetry. O2 saturation unable to carry a full oxygen administra-
above 95% or as complement of tion. SO2 remains
prescribed. oxygen. above 95%.
Nurse Determine whether child Administer folic acid as Folic acid is necessary Child takes folic acid
has been taking folic prescribed. to build new red cooperatively.
acid at home. cells to replace those
that have been
hemolyzed.

Nutrition

Nurse Assess child’s Begin IV therapy as IV therapy helps restore Child names best hand
intake and prescribed. hydration and reduce to start infusion; co-
output. sickle-cell clotting. operates with arm
board restriction.
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CHAPTER 44 Nursing Care of a Family When a Child Has a Hematologic Disorder 1307

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

Patient/Family Education

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

Psychosocial/Spiritual/Emotional Needs

Nurse Assess the stress Review with family When families miss a Father states he will try
level of the family ways to maintain a support person, harder to meet chil-
in light of absent tight family unit they need to rally dren’s needs al-
mother and child (game night, com- together to devise though worrying
with chronic mon activities) to other support about wife’s safety
illness. maintain family until methods. is a major concern.
mother returns.

Discharge Planning

Nurse Determine whether Schedule a follow-up Care of a chronically ill Father states he under-
parent has any visit in 3 days for child can be a major stands importance
questions about evaluation. strain on a family. of follow-up visit and
care of a child Follow-up visits help will keep
with sickle-cell share responsibility appointment with
anemia. for care. child.

repeated infarction and atrophy. An atropic spleen leaves a apism, or persistent, painful erection (Chinegwundoh &
child more susceptible to infection than normal because the Anie, 2009).
spleen can no longer filter bacteria. Pneumococcal meningi- Therapeutic Management. The child in sickle-cell crisis
tis and salmonella-induced osteomyelitis become frequent has three primary needs: pain relief and adequate hydration
illnesses; prophylactic antibiotics and pneumococcal vaccine and oxygenation to prevent further sickling and halt the
may be prescribed to prevent these infections (Davies et al., crisis.
2009). Acetaminophen (Tylenol) may be adequate pain relief for
An acute chest syndrome with symptoms of pulmonary some children; for others, a narcotic analgesic such as intra-
infiltrates with chest pain, fever, tachypnea, wheezing, or venous morphine may be needed. Once children are pain
cough that leads to pneumonia may also occur. Acute chest free, they are able to relax, reducing the metabolic demand
syndrome develops because, when areas of the lung become for oxygen and helping to end the sickling. Hydration is gen-
inflamed and hypoxic, sickle cells adhere to the activated erally accomplished with intensive intravenous fluid replace-
endothelium and then fail to be reoxygenated (Adamson & ment therapy. Tissue hypoxia leads to acidosis. The acidosis
Longo, 2008). Blood transfusion is used to increase the must be corrected by electrolyte replacement. Some kidney
oxygen-carrying capacity of blood, and broad-spectrum an- infarction may have occurred, so do not administer potas-
tibiotics are given to resolve the pneumonia. sium intravenously until kidney function has been deter-
Another common complication occurs when the liver mined (the child is voiding). Otherwise, excessive potassium
becomes enlarged from stasis of blood flow. Eventually, cir- levels may occur, possibly leading to cardiac arrhythmias. If
rhosis (fibrotic degeneration) will occur from infarcts and infection appears to be the precipitator for a sickling crisis,
tissue scarring. The kidneys may have subsequent scarring blood and urine cultures, a chest radiograph, and a complete
also, so kidney function may be decreased. The sclerae are blood count will be taken and the infection will be treated by
generally icteric (yellowed) from release of bilirubin from antibiotics. Blood transfusion (usually packed RBCs) may be
destruction of the sickled cells; small retinal occlusions may necessary to maintain the hemoglobin above 12 g/dL
lead to decreased vision. Regular eye examinations are nec- (termed hypertransfusion).
essary in children with sickle-cell disease to detect this. Cell Hydroxyurea, an antineoplastic agent that has the poten-
clusters in the blood vessels of the penis may cause pri- tial to increase the production of hemoglobin F (fetal hemo-
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1308 UNIT 7 The Nursing Role in Restoring and Maintaining the Health of Children and Families With Physiologic Disorders

globin), can be used in children with sickle-cell disease to

increase their overall hemoglobin level (Jones, Davies, & BOX 44.10 ✽ Focus on Communication
Olujohungbe, 2009). The drug, given orally, can cause
anorexia. Therefore, monitor the child’s nutrition intake Joey, who has sickle-cell disease, is seen in the emer-
during this drug therapy. If none of the above measures ap- gency department with a new vaso-occlusive crisis. His
pears to be effective, children may be given an exchange right knee is discolored by a large brush burn. He’s cry-
transfusion to remove most of the sickled cells and replace ing from pain.
them with normal cells. Exchange transfusion (see Chapter
26) must be done with small amounts of blood at each ex- Less Effective Communication
change. Otherwise, the pressure changes can cause such ir- Nurse: Hello, Mr. Harrow. I need to ask some questions
regularities in blood volume that heart failure results. to see if anything triggered this new crisis.
Hematopoietic stem cell transplantation is possible for the Mr. Harrow: He better not have been doing something
child who does not respond to usual therapies. he’s not allowed to do.
Nurse: His knee looks like he fell. Were you running,
✔Checkpoint Question 44.3 Joey? So you got dehydrated?
Mr. Harrow: He better not say he was doing that!
Joey, who has sickle-cell anemia, has had two vaso-occlusive Nurse: Joey, how did you hurt your knee?
crises in the past year. A vaso-occlusive crisis occurs because: Joey: I don’t know.
a. An enlarged spleen causes blood to pool there. Nurse: Okay. Let’s get you better and not worry about
b. Dehydration leads to thrombosed sickle cells. what started this.
c. Hemorrhage reduces a child’s total blood volume.
More Effective Communication
d. Decreased platelet number leads to poor coagulation.
Nurse: Hello, Mr. Harrow. I need to ask some ques-
tions to see if anything triggered this new crisis.
Mr. Harrow: He better not have been doing something
Nursing Diagnoses and Related he’s not allowed to do.
Interventions Nurse: His knee looks like he fell. Were you running,
✽ Joey? So you got dehydrated?
Nursing Diagnosis: Ineffective tissue perfusion related Mr. Harrow: He better not say he was doing that!
to generalized infarcts due to sickling Nurse: Mr. Harrow, I need to get an accurate history of
what happened. I’d like Joey to tell us how he thinks
Outcome Evaluation: Child’s respiratory rate is 16 to the accident happened. Then later on, we can talk
20/min; cyanosis is absent; arterial blood gases within about what are good rules for him to be following.
acceptable parameters including PCO2 ⫽ 40 mm Hg;
PO2 ⫽ 80 to 90 mm Hg; oxygen saturation of 95%; Children with blood disorders have to follow a great many
urine output greater than 1 mL/kg/hr. rules to avoid clotting or bleeding episodes, such as not
playing contact sports or playing too hard in the sun.
Oxygen may be administered by nasal cannula or
Because these forbidden activities are appealing, chil-
mask if arterial blood gases reveal a low PO2 level.
dren occasionally break the rules. In an emergency room,
Oxygen may not reach every distal body part effec-
it is important that children and parents both recognize
tively, however, if blood flowing to the part is ob-
that the priority at the moment is obtaining an accurate
structed by the sickled cells. When hemoglobin S is
history. Until they realize this, they may be so concerned
below 40%, there generally is adequate blood flow to
with the broken rule that they are unable to move beyond
body cells. High concentrations of oxygen are not
that to secure adequate therapy.
used because hypoxia is a stimulant to erythrocyte
production—production badly needed to replace
damaged cells. Monitor the flow rate carefully and use
pulse oximetry to evaluate oxygen saturation levels for
changes. Encourage bed rest to relieve the pain and the stresses of chronic illness. Other children experi-
reduce oxygen expenditure (Box 44.10). ence such devastating episodes in early childhood
It is important to maintain accurate intake and out- that the disease becomes fatal at an early age. Parents
put records and to test urine for specific gravity and need support to supervise children carefully day by
hematuria to detect the extent or presence of kidney day when they are aware that, due to the intense
damage from infarcts. episodes, the child may die despite the precautions.
Nursing Diagnosis: Ineffective health maintenance re- Between crises, care focuses on preventing recur-
lated to lack of knowledge regarding long-term needs ring crises. Although the hemoglobin level of children
of child with sickle-cell anemia may remain as low as 6 to 9 g/100 mL, children adjust
well to this chronic state. Children who receive frequent
Outcome Evaluation: Parent accurately describes dis- blood transfusions should not be given supplementary
ease process and identifies special precautions nec- iron or iron-fortified formula or vitamins or they may re-
essary to prevent sickle-cell crisis. ceive too much iron; high levels of excess iron are de-
In many children, episodes of sickling grow less se- posited in body tissues (hemochromatosis) to a point
vere as a child reaches adolescence. Such children of staining body tissue or being incorporated into body
will have a normal life expectancy but still experience tissue with fibrotic scarring (hemosiderosis). Oral folic
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CHAPTER 44 Nursing Care of a Family When a Child Has a Hematologic Disorder 1309

acid may be prescribed to help rebuild hemolyzed mia, and they know that a child with even a minor in-
RBCs. fection could become very ill.
Children with sickle-cell anemia need to be fol- Parents must make decisions regarding children’s
lowed at regular health care visits. They must receive activity levels. Children should attend regular school
childhood immunizations so they are not vulnerable to and should be allowed to participate in all school ac-
common childhood infections such as measles or per- tivities except contact sports (such as football), which
tussis. They are also candidates for meningococcal, could result in rupture of an enlarged spleen or liver.
pneumococcal, and influenza vaccines to prevent in- Long-distance running is also inadvisable because it
fection. They may be prescribed oral penicillin as pro- can lead to dehydration. During the summer, parents
phylaxis for the first 5 years. Puberty may be delayed, need to offer the child frequent drinks to prevent de-
and both parents and children may need counseling hydration, especially on long hikes and at the beach.
to accept this. Once puberty changes do occur, they Caution parents against taking the child on board an
are adequate, just later than usual. Parents and chil- unpressurized aircraft in which the oxygen concentra-
dren with sickle-cell disease also need support and tion may fall during flight.
positive reinforcement to enhance the child’s self- Some children who have had kidney infarcts and
esteem and to learn how to deal with problems that lessened ability to concentrate urine have chronic
occur as a result of this chronic hematologic disorder nocturnal enuresis (bedwetting).
(Box 44.11). Children with sickle-cell disease are at high risk if
Caution parents to bring their child to a health care they need surgery. The hours of being held on noth-
facility at the first indication of infection. Some par- ing-by-mouth status, as well as being unable to eat af-
ents are reluctant to do this, afraid that they will be la- terward, may lead to dehydration. Anesthesia may
beled overprotective. Assure them that health care cause a transient hypoxia leading to sickling. Parents
personnel are knowledgeable about sickle-cell ane- must be cautioned that even for such a simple opera-
tion as tooth extraction, therefore, they must alert
health care personnel about their child’s condition.

BOX 44.11 ✽ Focus on

Family Teaching What if... Joey’s parent tells you he restricts Joey, who
has sickle-cell anemia, from drinking any fluid after
School Safety Precautions for Children with 4 PM to prevent bedwetting? Is this a good solution to
Sickle-Cell Disease bedwetting for this child? How would you counsel this
Q. Joey’s father says to you, “My son has sickle-cell dis- parent?
ease. What can I do to help keep him safe at school?”
A. Use these guidelines to help ensure your son’s safety
at school:
Thalassemias
• Be certain your child either takes fluid with him or buys
The thalassemias are autosomal recessive anemias associated
adequate fluid for lunch. Children with sickle-cell ane-
with abnormalities of the beta chain of adult hemoglobin
mia need to maintain a high fluid intake to prevent their
(HgbA). Although these anemias occur most frequently in
blood from becoming thick.
the Mediterranean population, they also occur in children of
• Provide additional fluid in the summer, when dehy-
African and Asian heritage (Waterbury, 2007).
dration 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. Thalassemia Minor (Heterozygous Beta-Thalassemia)
• Learn about sources high in folic acid, such as veg- Children with thalassemia minor, a mild form of this ane-
etables and fruit, and be certain these foods are in- mia, produce both defective beta hemoglobin and normal
cluded in your son’s diet every day. hemoglobin. Because there is some normal production, the
• Encourage the boy to get adequate sleep at night as RBC count will be normal, but the hemoglobin concentra-
a general measure to prevent illness. tion will be decreased 2 to 3 g/100 mL below normal levels.
• With the exception of contact sports (to avoid dam- The blood cells are moderately hypochromic and microcytic
age to an enlarged spleen) and long-distance run- because of the poor hemoglobin formation.
ning (to prevent dehydration), encourage your son to Children may have no symptoms other than pallor. They
participate in normal school activities. require no treatment, and life expectancy is normal. They
• Know that bedwetting may occur as part of the ill- should not receive a routine iron supplement because their
ness. Encourage your son to take baths in the morn- inability to incorporate it well into hemoglobin may cause
ing if this occurs so his clothes don’t smell of urine. them to accumulate too much iron. The condition represents
• Maintain routine health care such as immunizations to the heterozygous form of the disorder or can be compared
prevent common childhood illnesses such as measles with children having the sickle-cell trait.
and mumps, which cause fever and dehydration.
• Call your primary health care provider at the first sign Thalassemia Major (Homozygous Beta-Thalassemia)
of illness, such as an upper respiratory infection, so
therapy can be begun immediately. Thalassemia major is also called Cooley’s anemia or
Mediterranean anemia. Because thalassemia is a beta chain
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1310 UNIT 7 The Nursing Role in Restoring and Maintaining the Health of Children and Families With Physiologic Disorders

erythropoiesis is suppressed and cosmetic facial alterations, os-

TABLE 44.3 ✽ Effects of Thalassemia Major teoporosis, and cardiac dilatation are minimized.
Hypertransfusion therapy also reduces the possibility that
Body Organ or Effect of Abnormal splenectomy will be necessary. Frequent blood transfusions,
System Cell Production unfortunately, increase the risk of blood-borne disease, such
Bone marrow Overstimulation of bone
as hepatitis B, and hemosiderosis. Children may receive an
marrow leads to oral iron-chelating agent to remove this excessive store of iron,
increased facial- such as deferasirox (Stumpf, 2007). Others receive deferox-
mandibular growth amine given subcutaneously over 6 to 8 hours as they sleep at
Skin Bronze-colored from night (Karch, 2009).
hemosiderosis and Splenectomy may become necessary to reduce discomfort
jaundice and also to reduce the rate of RBC hemolysis and the number
Spleen Splenomegaly of transfusions needed. Bone marrow stem cell transplanta-
Liver and gallbladder Cirrhosis and cholelithiasis tion can offer a cure. With treatment, the overall prognosis of
Pancreas Destruction of islet cells
thalassemia is improving but still grave. Most children with
and diabetes mellitus
Heart Failure from circulatory
the disease die of cardiac failure during adolescence or as
overload young adults if they do not receive a hematopoietic stem cell
transplant (Muncie, 2008).

Nursing Diagnoses and Related

hemoglobin defect, symptoms do not become apparent until
a child’s fetal hemoglobin has largely been replaced by adult
Interventions
hemoglobin during the second half of the first year of life. ✽
Effects of thalassemia major on body systems are summarized Nursing Diagnosis: Risk for situational low self-esteem
in Table 44.3. Unable to produce normal beta hemoglobin, related to changed physical appearance
the child shows symptoms of anemia: pallor, irritability, and Outcome Evaluation: Child states he can accept al-
anorexia (Muncie, 2008). tered appearance and interacts with peers.
RBCs are hypochromic (pale) and microcytic (small).
Children with thalassemia major may have delayed
Fragmented poikilocytes and basophilic stippling (uneven-
growth and sexual maturation. They usually develop a
ness of hemoglobin concentration) are present. The hemo-
marked change in facial appearance because of the
globin level is less than 5 g/100 mL. The serum iron level is
overgrowth of marrow-producing centers of the facial
high because iron is not being incorporated into hemoglobin;
bones. This can be demoralizing because these
iron saturation is 100%.
changes will be permanent. In addition, the child who
Assessment. To maintain a functional level of hemoglobin, receives frequent blood transfusions may develop
the bone marrow hypertrophies in an attempt to produce such hemosiderosis that skin color appears bronze.
more RBCs. This may cause bone pain; the ineffective at- Children should be allowed as much activity as pos-
tempt often leads to the formation of target cells or large sible and should attend regular school, if possible, to
macrocytes that are short-lived and nonfunctional. As bone maintain a nearly normal childhood. Discussions about
marrow becomes hyperactive, this results in a characteristic other children’s reactions to their changing facial ap-
change in the shape of the skull (parietal and frontal bossing) pearance can be helpful.
and protrusion of the upper teeth, with marked malocclusion.
The base of the nose may be broad and flattened; the eyes may Autoimmune Acquired Hemolytic Anemia
be slanted with an epicanthal fold, as in Down syndrome. A
radiograph of bone shows marked osteoporotic (of lessened Occasionally, autoimmune antibodies (abnormal antibodies
density) tissue, possibly resulting in fractures. The child may of the IgG class) attach themselves to RBCs, destroying them
have hepatosplenomegaly due to excessive iron deposits and or causing hemolysis. This may occur at any age, and its ori-
fibrotic scarring in the liver and the spleen’s increased at- gin is generally idiopathic, although the disorder may be asso-
tempts to destroy defective RBCs. Abdominal pressure from ciated with malignancy, viral infections, or collagen diseases
the enlarged spleen may cause anorexia and vomiting. such as rheumatoid arthritis or systemic lupus erythematosus.
Epistaxis is common, as is diabetes mellitus due to pancreatic A child may recently have had an upper respiratory infection,
hemosiderosis (deposition of iron) and cardiac dilatation with measles, or varicella virus infection (chickenpox). Such he-
an accompanying murmur. Arrhythmias and heart failure are molysis may occur after the administration of drugs such as
frequent causes of death. quinine, phenacetin, sulfonamides, or penicillin.
The exact cause is unknown but appears to involve a
Therapeutic Management. Digitalis, diuretics, and a low- change in the RBCs themselves, making them act as antigens,
sodium diet may be prescribed to prevent heart failure, which or a change in antibody production, making antibodies de-
could result from the decompensation that accompanies ane- structive to other substances.
mia, and from myocardial fibrosis caused by invasion of iron
(hemosiderosis). Transfusion of packed RBCs every 2 to Assessment. The onset is insidious. Children have a low-
4 weeks (hypertransfusion therapy) will maintain hemoglobin grade fever, anorexia, lethargy, pallor, and icterus from re-
between 10 and 12 g/100 mL. With this level of hemoglobin, lease of indirect bilirubin from the hemolyzed cells. Both
 CHAPTER 44 Nursing Care of a Family When a Child Has a Hematologic Disorder 1311

urine and stools appear dark because the excess bilirubin is number, each erythrocyte is normally saturated with hemo-
being excreted. In some children, the illness begins abruptly globin. The RBC count may be as high as 7 million/mm3.
with high fever, hemoglobinuria, marked jaundice, and pal- Hemoglobin levels may be as high as 23 g/100 mL.
lor. The liver and spleen may be enlarged. Treatment of polycythemia involves treatment of the un-
Laboratory findings reveal that the RBCs are extremely derlying cause. Because of the high blood viscosity from so
small and round (spherocytosis), resembling hereditary sphero- many crowded blood cells, cerebrovascular accident or em-
cytosis. The reticulocyte count is increased as the body attempts boli may occur. The risk increases particularly if the child be-
to form replacement RBCs. A direct Coombs’ test result is pos- comes dehydrated, such as with fever or during surgery.
itive, indicating the presence of antibodies attached to red cells. Exchange transfusion or phlebotomy to reduce the RBC
Hemoglobin levels may fall as low as 6 g/100 mL. count may be necessary.
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- DISORDERS OF THE WHITE BLOOD CELLS
bance. For these children, it is difficult to cross-match blood Most disorders characterized by a decrease or increase in the
for transfusion because the red cell antibody tends to clump number of WBCs or specific WBC components occur in
or agglutinate all blood tested. If cross-matching is impos- response to another disease (often infection or an allergic
sible, the child may be given type O Rh-negative blood. reaction) in the body (Table 44.4). Laboratory values of
Observe the child carefully during any transfusion for signs WBCs, therefore, provide one of the first objective indicators
of transfusion reaction. of infectious disease, often aiding in specific diagnoses. These
If anemia is persistent, corticosteroid therapy (oral pred- diseases are discussed in Chapter 43. Leukemia, overproduc-
nisone) to reduce the immune response is generally effective, tion of WBCs, is discussed with other malignant conditions
increasing the RBC count and hemoglobin concentration in in Chapter 53.
a short period. For some children, stronger immunosuppres-
sive agents such as cyclophosphamide (Cytoxan) or azathio-
prine (Imuran) are necessary to reduce antibody formation.
If these are ineffective, splenectomy may be necessary. DISORDERS OF BLOOD COAGULATION
Often it is difficult for parents to understand the process Platelets are necessary for blood coagulation, so disorders
causing their child’s condition. How could a child’s body that limit the number of platelets limit the effectiveness
turn on itself? How long will this last? What will stop it from of this process. A normal platelet level is 150,000/mm3.
happening again? There are no answers to these questions. Thrombocytopenia (decreased platelet count) is defined as a
Provide the parents and child with support as they wait for platelet count of less than 40,000/mm3. Thrombocytopenia
this unexplainable process to run its course and for their often leads to purpura, or blood seeping from vessels
child to be well again. into the skin. In one rare disorder, children are born with
thrombocytopenia and are also missing the radius bone
✔Checkpoint Question 44.4 in the forearm (TAR [thrombocytopenia/absent radius]
Autoimmune acquired hemolytic anemia can occur in any syndrome).
child. The usual cause of this disorder is:
a. Allergy to the protein found in fish. Purpuras
b. A mutant gene similar to sickle cell. Purpura refers to a hemorrhagic rash or small hemorrhages
c. An elevated eosinophil cell count. in the superficial layer of skin. Two main types of purpura
d. Antibody production against red cells. occur in children: idiopathic thrombocytopenia purpura and
Henoch-Schönlein syndrome.

Polycythemia Idiopathic Thrombocytopenic Purpura

Polycythemia is an increase in the number of RBCs (Zull, Idiopathic thrombocytopenic purpura (ITP) is the result of
2007). The condition results from increased erythropoiesis, a decrease in the number of circulating platelets in the pres-
which occurs as a compensatory response to insufficient oxy- ence of adequate megakaryocytes (precursors to platelets).
genation of the blood in order to help supply more oxygen to The cause is unknown, but it is thought to result from an in-
body cells. Chronic pulmonary disease and congenital heart creased rate of platelet destruction due to an antiplatelet an-
disease are the usual causes of polycythemia in childhood. tibody that destroys platelets making this an autoimmune
Also, it may occur from the lower oxygen level maintained illness (Schmidt & Aldeen, 2007).
during intrauterine life in newborns or with twin transfusion In most instances, ITP occurs approximately 2 weeks
at birth (one twin receives excess blood while a second twin after a viral infection such as rubella, rubeola, varicella, or an
is anemic). upper respiratory tract infection. Congenital ITP may occur
Plethora (marked reddened appearance of the skin) occurs in the newborn of a woman who has had ITP during preg-
because of the increase in total RBC volume. Erythrocytes are nancy. An antiplatelet factor apparently crosses the placenta
usually macrocytic (large) and the hemoglobin content is and causes platelet destruction in the newborn in the same
high. This means that the mean corpuscular hemoglobin will way that Rh incompatibility or hemolytic disease of the
be elevated; the mean corpuscular hemoglobin concentration, newborn develops (see Chapter 26). However, in ITP, the
however, will be normal, indicating that, although many in platelets, not the RBCs, are sensitized.