NURSING CARE OF A

FAMILY WHEN A CHILD

HAS A

HEMATOLOGIC DISORDER
 HEMATOLOGIC DISORDER
• often called blood dyscrasias
• occur when components of the blood are formed incorrectly or either
increase or decrease in amount beyond normal ranges
• most blood dyscrasias in children originate in the bone marrow
 HEMATOLOGIC SYSTEM
• Blood components originate in the bone marrow, circulate through blood
vessels, and ultimately are destroyed by the spleen.
• The total volume of blood in the human body is roughly proportional to body
weight: 85 ml/kg at birth, 75 ml/kg at 6 months of age, and 70 ml/kg after the
first year.
• formed elements—the erythrocytes (red blood cells [RBCs]), leukocytes
(white blood cells [WBCs]), and thrombocytes (platelets)—are the portions
most affected by hematologic disorders in children
 ERYTHROCYTES (RED BLOOD CELLS)
• function chiefly to transport oxygen to and carry carbon dioxide away from
body cells
• formed from the hemocytoblast in the red bone marrow under the stimulation
of erythropoietin (a hormone formed by the kidneys that is produced
whenever a child has tissue hypoxia)
• the usual life span of the mature erythrocyte is 120 days
• Polycythemia (elevated hematocrit) often occurs when a child has impaired
tissue oxygenation and increased erythropoietin
Hemoglobin
• component of RBCs that allows them to carry out the transport of oxygen
• composed of globin, a protein, and heme, an iron-containing pigment. It is
the heme portion that combines with oxygen and carbon dioxide for
transport
• Hemoglobin levels are highest at birth (13.7 to 20.1 g/100 ml); they reach a
low at approximately 3 months of age (9.5 to 14.5 g/100 ml) and then
gradually rise again until adult values are reached at puberty (11 to 16 g/100
ml)
Bilirubin
• As the heme portion is degraded, it is converted into protoporphyrin;
protoporphyrin is then further broken down into indirect bilirubin.
• Indirect bilirubin is fat-soluble and so cannot be excreted by the kidneys. It is
therefore converted by the liver enzyme glucuronyl transferase into direct
bilirubin, which is water-soluble and excreted in bile
• In the newborn, liver function is so immature that the conversion from indirect
to direct bilirubin is difficult. When the level of indirect bilirubin in the blood
rises to more than 7 mg/100 ml, it permeates outside the circulatory system,
and the infant begins to show signs of yellowing from the color of bilirubin. At
any point in life, if excessive hemolysis (destruction) of RBCs occurs from
other than usual causes, a child will also show signs of jaundice.
 LEUKOCYTES (WHITE BLOOD CELLS)
• are nucleated cells and few in number compared with RBCs
• primary function is defense against antigen invasion
• their life span varies from approximately 6 hours to unknown intervals
 THROMBOCYTES (PLATELETS)
• are round, nonnucleated bodies formed by the bone marrow
• their function is capillary hemostasis and primary coagulation
• usual number is 150,000 to 300,000/mm3 after the first year
• Immature thrombocytes are termed megakaryocytes. If large numbers of
these are present in serum, it indicates that a rapid production of platelets is
occurring.
 ASSESSMENT AND THERAPEUTIC TECHNIQUES FOR
HEMATOLOGIC DISORDERS

• An assessment of children with hematologic disorders begins with a

complete history, including family history, to identify inherited disorders.
• Complete blood count
• Peripheral Blood smear
• For a specific diagnosis, children generally require several diagnostic
procedures such as blood cell or bone marrow analysis.
 BONE MARROW ASPIRATION AND
BIOPSY
• Bone marrow aspiration provides samples of bone marrow so the type and
quantity of cells being produced can be determined
• aspiration sites used are the iliac crests or spines because is it usually less
frightening for children and have the largest marrow compartments during
childhood
• the anterior tibia can be used as an additional site for neonates
• The area of the aspiration is cleaned
with an antiseptic solution and
draped.
• The overlying skin is infiltrated with a
local anesthetic and then a large-
bore needle and stylus is introduced
through the overlying tissue into the
bone.
• When the marrow cavity is reached,
the stylus is removed, a syringe is
attached to the needle, and bone
marrow is aspirated (which appears
as thick blood in the syringe).
• The syringe is then removed, and the
marrow obtained is expelled onto a
slide, sprayed with a preservative,
and taken to a laboratory for
analysis.
• Pressure must be applied to the
puncture site for 5 to 10 minutes
immediately afterward to prevent
bleeding.
NURSING INTERVENTIONS FOR A CHILD WHO HAD BONE MARROW
ASPIRATION

• Monitor pulse and blood pressure and observe the dressing every 15 minutes
for the first hour after the procedure to be certain no bleeding is occurring.
• Keep the child fairly quiet for the first hour by playing a quiet game or other
activity.
• Because bone marrow aspiration is a painful and invasive procedure, allow
young children an opportunity for therapeutic play with a doll and syringe to
help them express their feelings about the procedure.
• If the procedure was done as an ambulatory one, instruct parents to take
the child’s temperature 12 and 24 hours after the procedure to detect
infection.
BLOOD TRANSFUSION
• commonly used in the treatment of blood disorders
• may include whole blood, packed RBCs, washed RBCs, plasma, plasma
factors, platelets, WBCs, and albumin
• it is important that it has been carefully matched with the child’s blood type
and is infused with a solution as nearly isotonic as possible (normal saline)
• If blood should be given with a hypertonic solution, this will cause fluid to be
drawn out of the transfused RBCs, causing them to shrink and be useless; if
blood is infused with a hypotonic solution, fluid will be drawn into the cells,
causing them to burst, and again, be destroyed.
 HEMATOPOIETIC STEM CELL
TRANSPLANTATION
• the intravenous infusion of hematopoietic stem cells from bone marrow
obtained by marrow aspiration or from peripheral or umbilical cord blood drawn
from a compatible donor to reestablish marrow function in a child with deficient
or nonfunctioning bone marrow
• can be allogeneic, syngeneic, or autologous
• Allogeneic transplantation is the transfer of stem cells from an immune-compatible
(histocompatible) donor
• Syngeneic transplantation (which is rare) involves a donor and recipient who are
genetically identical (i.e., identical twins).
• Autologous transplantation involves use of the child’s own stem cells removed from
cord blood banked at the time of the child’s birth. If this is not available, in some
instances, stem cells can be aspirated from the child’s bone marrow or obtained
from circulating blood, treated to remove abnormal cells and then reinfused.
• Siblings have about a 25% chance of being HLA compatible with the ill child.
• To prevent a child’s T lymphocytes from rejecting the newly transplanted donor
stem cells, total body irradiation to destroy the child’s marrow or an
immunosuppressive drug such as cyclophosphamide (Cytoxan) is administered
intravenously to the child before the procedure.
• the infusion takes 60 to 90 minutes because an infused hematopoietic stem cell
solution is fairly thick
Nursing Interventions
• Monitor the child’s cardiac rate and rhythm during the infusion to detect
circulatory overload or pulmonary emboli from unfiltered particles.
• After the infusion, take the child’s temperature at 1 hour and then about every
4 hours to detect an infection that could occur because the child’s WBCs are
nonfunctional from radiation or immunosuppression. Reinforce strict hand
washing and limit the child’s diet to cooked foods to reduce exposure to
bacteria.
 SPLENECTOMY
• removal of the spleen
• the purpose of the spleen is to remove damaged or aged blood cells
• In some children, splenectomy will not cure the basic defect of the blood
cells but will limit the degree of anemia
• Splenectomy formerly required a large abdominal incision, but today, it can
be performed by laparoscopy so, although still a procedure with risks, it does
not require as long a recovery period.
 HEALTH PROMOTION AND
MANAGEMENT
• ensuring families have access to genetic counseling so they can be aware
of the incidence of a disorder in their family and the potential for the disease
to develop in their child
• counseling parents of young children to maintain well-child healthcare visits
and urging adolescents to ingest iron-rich foods
• educating parents about the importance of keeping poisons out of the
reach of children and being aware of toxic substances in their community
• avoid contact sports to prevent bleeding episodes
 DISORDERS OF RED BLOOD CELLS
• Anemia
• reduction in the number or function of erythrocytes
• Polycythemia
• increase in the number of RBCs
 ANEMIA
Normochromic, Normocytic Anemia
• occur because of impaired production of erythrocytes by the bone marrow or by
abnormal or uncompensated loss of circulating RBCs, as with acute hemorrhage.
• RBCs appear normal in both color and size; however, there simply are too few of
them for effective oxygen transport.
Hypochromic Anemia
• When hemoglobin production is inadequate, erythrocytes appear pale
(hypochromia) and are also usually reduced in diameter (microcytic).
Macrocytic Anemia
• RBCs appear abnormally large. Such cells are actually immature erythrocytes or
megaloblasts (nucleated immature red cells)
• often also referred to as megaloblastic anemias.
Hemolytic anemias
• the number of erythrocytes is low
because there is increased erythrocyte
destruction.
• The destruction may be caused by
fundamental abnormalities in erythrocyte
structure or by extracellular destruction
forces.
Thalassemia
• autosomal recessive anemias associated
with abnormalities of the b chain of adult
hemoglobin (hemoglobin A)
• occur most frequently in the
Mediterranean population, they also
occur in children of African and Asian
heritage
 NORMOCHROMIC, NORMOCYTIC
ACUTE BLOOD- LOSS ANEMIA
• Blood loss that is sufficient to cause
anemia can occur from trauma such as
an automobile accident with internal
bleeding
Assessment
• Children immediately appear pale,
tachycardic, and breathe rapidly.
• Newborns may have gasping respirations,
intercostal retractions, and cyanosis.
• Children with rapid heart and respiratory
rates
• become listless and inactive, dizzy, and,
possibly, comatose.
ANEMIAS
Interventions:
• Treatment involves control of bleeding by
addressing its underlying cause and
transfusing additional RBCs.
• Lay the child flat to provide as much
circulation as possible to brain cells.
• Keep the child warm with blankets; place
the infant in an incubator or under a
radiant heat warmer.
• Until blood is available for transfusion, a
blood expander such as plasma or
intravenous fluid such as normal saline or
lactated Ringer’s may be given to expand
blood volume and improve blood pressure.
ANEMIA OF ACUTE INFECTION
• Acute infection or inflammation,
especially in infants, can cause
increased destruction or decreased
production of erythrocytes.
• Common conditions that do this
include osteomyelitis and ulcerative
colitis.
• Management involves treatment of
the underlying condition. When the
condition is reversed, blood values
will return to normal.
ANEMIA OF RENAL DISEASE
• Either acute or chronic renal
disease can cause loss of function in
kidney cells, which causes an
accompanying decrease in
erythropoietin production, resulting
in a normocytic, normochromic
anemia.
• Treatment: administration of
recombinant human erythropoietin
ANEMIA OF NEOPLASTIC DISEASE
• Malignant growths such as leukemia
or lymphoma result in
normochromic, normocytic
anemias because the invasion of
bone marrow by proliferating
neoplastic cells impairs RBC
production.
• The treatment of such an anemia
involves measures designed to
achieve remission of the neoplastic
process and transfusion to increase
the erythrocyte count.
HYPERSPLENISM
• Under usual conditions, blood filters
rapidly through the spleen.
• If the spleen becomes enlarged, blood
cells pass through more slowly, with
more cells being destroyed in the
process.
• The overactive spleen then leads to
increased destruction of RBCs which
can cause anemia and may lead to
pancytopenia (deficiency of all cell
elements of blood).
• Therapeutic management: treating the
underlying splenic disorder, possible
splenectomy
APLASTIC ANEMIA
Aplastic anemias result from depression of hematopoietic activity in the bone
marrow.
• Congenital aplastic anemia
(Fanconi syndrome) is inherited as
an autosomal recessive trait.
• A child is born with several
congenital anomalies, such as
skeletal and renal abnormalities,
hypogenitalism, and short stature.
• Within 10 years, most of these
children develop bone marrow
failure as well as thrombocytopenia
and neutropenia
• Acquired aplastic anemia is a
decrease in bone marrow
production, which occurs if a child is
excessively exposed to radiation,
drugs, or chemicals known to cause
bone marrow damage.
• A serious infection such as
meningococcal pneumonia might
cause autoimmunologic
suppression of the bone marrow,
which then also results in this
condition.
Assessment
• pale Diagnostic Findings
• fatigues easily • Bone marrow samples will show a
reduced number of blood elements,
• anorexia and blood-forming spaces will be
• bruises easily or develops petechiae infiltrated by fatty tissue
(pinpoint, macular, purplish-red spots
caused by an intradermal or
submucous hemorrhage)
• excessive nosebleeds or gastrointestinal
bleeding
• increased number of infections
• cardiac decompensation such as
tachycardia, tachypnea, shortness of
breath, or cyanosis
• irritable
Treatment
• The first step in therapy is to immediately discontinue any drug or chemical
suspected of causing the bone marrow dysfunction and removing the
substance from the child’s environment to avoid exposure.
• The ultimate therapy for both congenital and acquired aplastic anemia is
hematopoietic stem cell transplantation.
• Packed RBCs, platelet transfusions,
• cyclosporine
• antithymocyte globulin (ATG)
• RBC-stimulating factor such as erythropoietin
• course of an oral corticosteroid (prednisone)
• a course of testosterone
Nursing Diagnosis and Related Interventions
• Risk for disturbed body image related to changed appearance occurring as
medication side effects
• Be certain both children and their parents know these effects are related to the
medication and will fade when the medication is withdrawn.
• Reinforce and emphasize things they are doing well such as how well they are
managing this unexpected turn in their life.
Nursing Management

• Parents may feel distressed if the illness originated from exposure to a

chemical they should have kept away from their child, such as an
insecticide
• Parents have less confidence in healthcare personnel if the illness followed
treatment with a drug such as chloramphenicol. They may wonder how they
can trust in a drug to cure the illness if they believe a prescribed drug
caused the illness
HYPOPLASTIC ANEMIA

• result from depression of hematopoietic activity in bone marrow and can

also be either congenital or acquired.
• Unlike aplastic anemias, however, in which WBCs, RBCs, and platelets are all
affected, with hypoplastic anemias, only RBCs are affected
• Congenital hypoplastic anemia (Blackfan–Diamond syndrome) is a rare
disorder apparently caused by an inherent defect in RBC formation that
affects both sexes and shows symptoms as early as the first 6 to 8 months of
life.
• An acquired form of this can be caused by infection with parvovirus B19, the
infectious agent that causes fifth disease.
Treatment
• With the acquired type, the reduction of RBCs is transient, so no therapy
other than monitoring is necessary.
• Children with the congenital form receive corticosteroid therapy along with
transfusions of packed RBCs.
• Iron chelation program
• hematopoietic stem cell transplantation
 HYPOCHROMIC ANEMIAS

IRON DEFICIENCY ANEMIA

• most common anemia of infancy and childhood
• occurs whenever the intake of dietary iron is inadequate
• without adequate iron, hemoglobin cannot be incorporated into RBCs
• A newborn usually has enough iron in reserve to last for the first 6 months of
life. After that, the infant needs iron incorporated into the diet.
• Urging parents to breastfeed or use iron-fortified formula as well as introduce
iron fortified cereal as a “first food” are important health teaching measures
Risk Factors
Infants
• Infants born with structural defects of
the gastrointestinal system
• gastroesophageal reflux or chalasia
(where an immature valve exists
between the esophagus and stomach
resulting in regurgitation)
• pyloric stenosis (narrowing between the
stomach and duodenum, resulting in
vomiting)
• Chronic diarrhea
• infants that are fed with cow’s milk
Children older than 2 years of age
• chronic blood loss
• gastrointestinal tract lesions such as
polyps, ulcerative colitis,
• Crohn disease
• protein-induced enteropathies
• parasitic infestation
• frequent epistaxis
• adolescent girls with heavy menstrual
periods
• frequent attempts to diet or with
overconsumption of snack foods that
are low in iron
 Assessment
• pale conjunctiva
• pale appearance
• poor muscle tone
• reduced activity
• heart may enlarge
• soft systolic precordial
murmur
• spleen may be slightly
enlarged
• fingernails typically become
spoon shaped or depressed
in contour
• chronic fatigue
• pica
• decreased hemoglobin
level (a level less than 11
g/100 ml of blood)
• reduced hematocrit level
(below 33%)
• RBCs are microcytic and
hypochromic and possibly
poikilocytic (irregular in
shape)
• mean corpuscular volume,
the mean corpuscular
hemoglobin, and serum iron
levels are all low
Therapeutic Management

• Therapy for iron-deficiency anemia focuses on treatment of the underlying

cause: the lack of iron.
• Iron compound such as ferrous sulfate for 4 to 6 weeks
• diet must be changed to one rich in iron and vitamin C
Nursing Diagnosis and Related Interventions

• Imbalanced nutrition, less than body requirements, related to inadequate

ingestion of iron
• It is helpful to minimize the child’s activities to prevent fatigue, particularly at
mealtime because a fatigued child is more reluctant to eat any food, let alone
iron-rich foods.
• Counsel parents on measures to improve their infant’s nutrition, such as adding
iron-rich foods.
• Help parents devise a reminder system so they can manage to give the iron
supplement over a long period of time.
CHRONIC INFECTION ANEMIA

• When infections are chronic, anemia of a hypochromic, microcytic type

occurs, which is probably caused by impaired iron metabolism.
• The degree of anemia is directly related to the severity of the underlying
disease
• Treatment of the underlying cause will usually result in increased hemoglobin
levels.
 MACROCYTIC (MEGALOBLASTIC)
ANEMIAS
ANEMIA OF FOLIC ACID DEFICIENCY
• deficiency of folic acid combined with vitamin C deficiency
• erythrocytes grow abnormally large
• often accompanied by neutropenia and thrombocytopenia.
• Although the mean corpuscular hemoglobin concentration will be normal, the
mean corpuscular volume and mean corpuscular hemoglobin will both be
increased.
• Treatment: daily oral administration of folic acid
PERNICIOUS ANEMIA (VITAMIN B12 DEFICIENCY)

• results from a deficiency in vitamin B12, either from inadequate intake or malabsorption
• For vitamin B12 to be absorbed from the intestine, an intrinsic factor must be present in the
gastric mucosa. If a child is born with an intrinsic factor deficiency, symptoms occur as
early as the first 2 years of life.
• Assessment
• The child appears pale, anorexic, and irritable, with chronic diarrhea. The tongue appears
smooth and beefy red due to papillary atrophy.
• If not identified and treated at that point, neuropathologic findings such as ataxia,
hyporeflexia, paresthesia, and a positive Babinski reflex will develop.
• The rate and efficiency of absorption of vitamin B12 can be tested by the ingestion of the
radioactively tagged vitamin when a dose of intrinsic factor is also measured.
• Treatment:
• If the anemia is identified as being caused by a vitamin B12–deficient diet, temporary
injections of vitamin B12 will reverse the symptoms.
• If the anemia is caused by lack of the intrinsic factor, lifelong monthly intramuscular
injections of vitamin B12 may be necessary.
 HEMOLYTIC ANEMIAS
CONGENITAL SPHEROCYTOSIS

• a hemolytic anemia that occurs most frequently in the White Northern European
population and is inherited as an autosomal dominant trait
• RBCs are small and have a short life span apparently due to abnormalities of the
protein of the cell membrane
• The abnormal cells swell, rupture, and then are destroyed by the spleen creating
a severe anemia.
• Chronic jaundice and splenomegaly also develop.
• Because the cells are so small, the mean corpuscular hemoglobin concentration
will be increased.
• Diagnostic evaluation: family history, the obvious hemolysis, and the presence of
the abnormal spherocytes
• Treatment: splenectomy at approximately 5 to 6 years
GLUCOSE-6-PHOSPHATE DEHYDROGENASE DEFICIENCY (G6PD)
• The enzyme glucose-6-phosphate dehydrogenase (G6PD) is necessary for maintenance of
RBC life
• lack of the enzyme results in premature destruction of RBCs
• an X-linked recessive trait inheritance and is found most often in persons of African,
Mediterranean, or Asian decent
• G6PD disease occurs in two identifiable forms.
• a congenital nonspherocytic type develop hemolysis, jaundice, and splenomegaly and
may have aplastic crises.
• a drug-induced form in which the blood pattern is normal until the child is exposed to fava
beans or drugs such as antipyretics, sulfonamides, antimalarials, and naphthoquinones (the
most common drug in these groups is acetylsalicylic acid [aspirin]).
• Diagnostic evaluation: rapid enzyme screening test or electrophoretic analysis of RBCs
• Be certain that both parents and children know about the abnormality in the child’s
metabolism so they can avoid common drugs such as acetylsalicylic acid.
 SICKLE-CELL ANEMIA

• an autosomal recessive inherited disorder carried on the β chain of hemoglobin;.

• occurs in about 1 out of every 400 Black infants in the United States
• sickle-cell trait (a child carries a gene for the disease but does not have active
symptoms) occurs in approximately 1 in 12 Blacks in the United States
• usually will not result in clinical symptoms until a child’s hemoglobin changes from
the fetal to the adult form at approximately 6 months
• Diagnostic evaluation:
• prenatally by chorionic villi sampling or from cord blood during amniocentesis;
• at birth by neonatal screening
• the amino acid valine takes the place of the
normally appearing glutamic acid,
• the erythrocytes become characteristically
elongated and crescent shaped (sickled) when
they are submitted to low oxygen tension (less than
60% to 70%), a low blood pH (acidosis), or
increased blood viscosity, such as occurs with
dehydration or hypoxia.
• When RBCs sickle or change to an elongated
shape, they cannot move freely through vessels.
• Stasis and further sickling occurs (a sickle-cell crisis).
• Blood flow halts and tissue distal to the blockage
becomes ischemic, resulting in acute pain and cell
destruction
Sickle Cell Crises
• term used to denote a sudden, severe onset
of sickling
• there is pooling of many new sickled cells in
blood vessels causing consequent tissue
hypoxia beyond the blockage (a vaso-
occlusive crisis)
• Symptoms are sudden, severe, and painful
• A laboratory report will reveal a hemoglobin
level of only 6 to 8 g/100 ml.
• A peripheral blood smear will demonstrate
sickled cells.
• Bilirubin and reticulocyte levels will be
increased and the WBC count is often
elevated to 12,000 to 20,000/mm3
Assessment for Sickle Cell Anemia
• at approximately 6 months of age, children begin to show initial signs of fever and anemia
• local pain
• infants have swelling of the hands and feet (a hand–foot syndrome)
• children tend to have a slight, thin build and characteristically long arms and legs
• may have a protruding abdomen because of an enlarged spleen and liver.
• in adolescence, the spleen size may decrease in size
• during late childhood or adolescence, an acute chest syndrome with symptoms of fever,
tachypnea, wheezing, or cough that leads to pneumonia may begin to occur
• kidney function may be decreased
• eye sclera become icteric (yellowed)
• small retinal occlusions may lead to decreased vision
• Priapism, a persistent painful erection, is a clinical manifestation of sickle cell caused by
vaso-occlusion in the penis
Therapeutic Management
• pain relief, adequate hydration, and oxygenation to prevent further sickling
and halt the crisis
• Analgesics (Acetaminophen, narcotic analgesiscs)
• intensive intravenous fluid replacement therapy
• electrolyte replacement to correct acidosis that develops from tissue hypoxia
• If infection appears, infection will be treated with antibiotics.
• Blood transfusion (usually packed RBCs) may be necessary
• Hydroxyurea, an antineoplastic agent, has the potential to increase the
strength and oxygenation capacity of sickled cells
• exchange transfusion
• Hematopoietic stem cell transplantation
Nursing Diagnosis and Intervention
 THALASSEMIA

THALASSEMIA MINOR (Heterozygous β-Thalassemia)

• a mild form of this anemia
• produce a combination of both defective β hemoglobin and normal
hemoglobin
• The condition represents the heterozygous form of the disorder and can be
compared with children having the sickle cell trait.
• the RBC count is usually normal, but the hemoglobin concentration will be
decreased by 2 to 3 g/100 ml below usual levels.
• blood cells are moderately hypochromic and microcytic because of the
poor hemoglobin formation
• Children may have no symptoms other than pallor.
• They require no treatment, and life expectancy is normal.
THALASSEMIA MAJOR (Homozygous β-Thalassemia)
• also called Cooley anemia or
Mediterranean anemia
• RBCs are both hypochromic and
microcytic
• Fragmented poikilocytes (abnormally
shaped RBCs) and basophilic stippling
(unevenness of hemoglobin
concentration) are also usually present.
• The hemoglobin level is less than 5
g/100 ml.
• The serum iron level is high because iron
is not being incorporated into
hemoglobin; iron saturation will be
100%.
Assessment
• changes in the shape of the skull (parietal and frontal
bossing) and protrusion of the upper teeth with marked
malocclusion (due to hyperactivity of the bone marrow)
• base of the nose may be broad and flattened
• the eyes may be slanted with an epicanthal fold
• an enlarged spleen and liver (due to excessive iron deposits and
fibrotic scarring)
• anorexia and vomiting (Abdominal pressure from the enlarged spleen)
• epistaxis
• cardiac dilatation with arrhythmias
• Diagnostic Evaluation: X-ray of the bone shows marked
osteoporotic (of lessened density) tissue
Therapeutic Management

• Stem cell transplantation is the ultimate cure

• digitalis, diuretics, and a low-sodium diet
• Transfusion of packed RBCs every 2 to 4 weeks
• oral iron-chelating agent to remove this excessive store of iron
• splenectomy
Nursing Diagnosis and Intervention

• Risk for situational low self-esteem related to changed physical appearance

• Children should be allowed as much activity as possible and should attend
regular school to maintain a nearly normal childhood.
• Discussions about other children’s reactions to their changing facial appearance
and how people are evaluated by who they are and not what they look like
can be helpful.
AUTOIMMUNE ACQUIRED HEMOLYTIC ANEMIA
• autoimmune antibodies (abnormal antibodies of the immunoglobulin IgG
class) attach themselves to RBCs, destroying them or causing hemolysis
• can occur at any age, and its origin is generally unknown
• may occur after the administration of drugs such as quinine, phenacetin,
sulfonamides, or penicillin
Assessment
• onset of symptoms is insidious.
• low-grade fever, anorexia, lethargy, pallor, and icterus
• urine and stools appear dark
• In a few children, the illness begins abruptly with high fever, hemoglobinuria,
marked jaundice, and enlarged liver and spleen.
• Laboratory findings reveals:
• RBCs have become extremely small and round (spherocytosis).
• reticulocyte count is increased
• A direct Coombs test result is positive
• Hemoglobin levels may fall as low as 6 g/100 ml.
Therapeutic Management

• disease process runs a limited course and no treatment is necessary

• single blood transfusion
• If anemia is persistent, corticosteroid therapy (oral prednisone) to reduce the
immune response is generally effective,
• If not effective, splenectomy or stronger immunosuppressive agents
 POLYCYTHEMIA

• an increase in the number of RBCs

• results from increased erythropoiesis, which occurs as a compensatory
response to insufficient oxygenation of the blood in order to help supply
more oxygen to body cells
• this may occur as a hereditary form, chronic pulmonary disease and
congenital heart disease
• it may also occur from the lower oxygen level maintained during intrauterine
life in newborns or with twin transfusion at birth (one twin receives excess
blood, whereas a second twin is anemic)
• Assessment:
• Plethora (marked reddened appearance of the skin)
• the means the mean corpuscular hemoglobin will be elevated, however, the
mean corpuscular hemoglobin concentration will be normal
• RBC count may be as high as 7 million/mm3
• Hemoglobin levels may be as high as 23 g/100 ml

• Treatment of polycythemia:
• involves treatment of the underlying cause.
• A program of low-dose aspirin to help prevent clotting or
• exchange transfusion
• phlebotomy
 DISORDERS OF BLOOD COAGULATION
• Purpura
• refers to a hemorrhagic rash or small hemorrhages in the superficial layer of skin.
• Two main types of purpura occur in children: idiopathic thrombocytopenic
purpura and Henoch–Schönlein syndrome.
• Hemophilia
• are inherited disorders of blood coagulation.
• involves a deficiency of a different blood coagulation factor
 PURPURA

IDIOPATHIC THROMBOCYTOPENIC PURPURA

• result of a decrease in the number of circulating platelets in the presence of

adequate megakaryocytes (precursors to platelets)
• cause is unknown
• it is thought to result from an increased rate of platelet destruction due to an
antiplatelet antibody that destroys platelets, making this an autoimmune
illness
• In most instances, ITP occurs approximately 2 weeks after a viral infection.
• Congenital ITP may occur in the newborn of a woman who has had ITP
during pregnancy.
Assessment
• first evidenced as miniature petechiae or
as large areas of asymmetric ecchymosis
most prominent over the legs, although
they may occur anywhere on the body
• Epistaxis
• bleeding into joints
• marked thrombocytopenia- platelet count
may be as low as 20,000/mm3.
• Bone marrow examination reveals a
normal number of megakaryocytes
Therapeutic Management

• Oral prednisone to reduce the immune response

• intravenous immunoglobulin (IVIG) or, in Rh-positive children, anti-D
immunoglobulin to supply anti-ITP antibodies are used to treat ITP
• Platelet transfusion
• Splenectomy
• If the child experiences joint pain from bleeding, acetaminophen (Tylenol) is
prescribed for pain
Nursing Diagnosis and Intervention

• Health-seeking behaviors related to injury-prevention measures

• The techniques for reducing bleeding such as padding surfaces where the child
plays can be used to reduce the possibility of bleeding.
• Be alert for signs such as persistent headache with vomiting, lethargy, slurred
speech, weakness on one side of the body, and new onset of seizure.
• Risk for compromised family coping related to diagnosis of child’s illness
• Assure parents after the diagnosis is made that this bruising is not and will not
lead to leukemia.
• Allow parents to have time to express their anger and regain confidence in their
healthcare team. To feel comfortable that healthcare personnel can manage
their child’s care without knowing the exact cause, parents need careful
explanations of all procedures.
HENOCH–SCHÖNLEIN PURPURA
• also called anaphylactoid purpura
• caused by increased vessel permeability, which leads to bleeding in the
small blood vessels.
• generally considered to be a hypersensitivity reaction to an invading
allergen
• occurs most frequently in children between 2 and 8 years of age and more
frequently in boys than girls. Usually, there is a history of a mild infection
before the outbreak of symptoms.
• The syndrome presents (because of the purpura) as a possible platelet
disorder until a differential diagnosis is made.
Assessment
• purpural rash occurs typically on the buttocks, posterior
thighs, and extensor surface of the arms and legs, the tips
of the ears may be involved
• rash begins as a crop of urticarial lesions that change to
pink maculopapules. These become hemorrhagic (bright
red) and then fade, leaving brown macular spots that
remain for several weeks.
• The child’s joints are tender and swollen
• gastrointestinal symptoms such as abdominal pain,
vomiting, or blood in stools.
• Gross or microscopic hematuria
• A biopsy shows granulocytes (WBCs) in the walls of small
arterioles.
• normal platelet count sedimentation rate, WBC count,
and eosinophil count are elevated.
Therapeutic Management

• oral corticosteroid therapy (prednisone)

• mild analgesics
 HEMOPHILIA

HEMOPHILIA A (FACTOR VIII DEFICIENCY)

• caused by deficiency of the coagulation component factor VIII,

the antihemophilic factor
• transmitted as a sex-linked recessive trait
• the incidence is approximately 1 in 5,000 White males (US)
• A female carrier may have slightly lowered but sufficient levels of
the factor VIII component so that she does not manifest a
bleeding disorder.
• factor VIII is an intrinsic factor of coagulation; its absence causes
the intrinsic system for manufacturing thromboplastin to be
incomplete.
• The child’s coagulation ability is not totally absent because the
extrinsic or tissue system remains intact. Because of this system,
the child’s blood will eventually coagulate after an injury.
Assessment
• Hemophilia often is recognized first in the infant who bleeds excessively
• The lower extremities (where the child bumps things) become heavily bruised. There
is soft tissue bleeding and painful hemorrhage into joints, which become swollen
and warm.
• Repeated bleeding into a joint this way causes damage to the synovial membrane
(hemarthrosis), possibly resulting in severe loss of joint mobility.
• Severe bleeding may also occur into the gastrointestinal tract, peritoneal cavity, or
central nervous system.
• Nosebleeds are common, they are not as severe as with the platelet deficiency
syndromes.
• The platelet count and prothrombin time are both normal; the whole blood clotting
time is either markedly prolonged or normal, depending on the level of factor VIII
present. A thromboplastin generation test will be abnormal.
• Partial thromboplastin time (PTT) is the test that best reveals the low levels of factor
VIII.
Therapeutic Management

• administration of factor VIII supplied by fresh whole blood, fresh or frozen

plasma, or a concentrate of factor VIII
• administration of desmopressin (DDAVP), which stimulates the release of
factor VIII, may also help prevent bleeding
• children with inhibitors to factor VIII can also be given a factor IX
concentrate (Proplex or Konyne). This concentrate enters the coagulation
cascade after factor VIII and halts bleeding at that point.
Nursing Diagnosis and Intervention
• Parental health-seeking behaviors related to strategies for protecting child from injury
• Educate parents about how to prevent bleeding episodes and how to respond when one
does occur.
• To help prevent injuries, help parents set appropriate limits for activity. An active infant, for
example, may need to have crib sides padded and all toys should be inspected for sharp
edges or parts. Older children need to self-monitor activities such as roughhousing, and
sports participation needs to be evaluated as to whether it will be safe. On the other hand,
parents need to be certain their child is not so inactive that obesity occurs.
• Parents (and the child at about age 10 years) can be taught to reconstruct and administer
a replacement factor intravenously to prevent bleeding immediately after an injury.
• Pain related to joint infiltration by blood
• The child with hemophiliac bleeding experiences discomfort because of the bleeding into
joints and may be frightened because his parents are so frightened. Acetaminophen
(Tylenol) rather than ibuprofen is ordered as an analgesic because ibuprofen may prolong
bleeding.
• Be certain that when joints are immobilized, they are in good alignment. As soon as the
acute bleeding episode has halted (approximately 48 hours), help the child perform passive
range of motion as prescribed to maintain function.
Nursing Diagnosis and Intervention

• Risk for interrupted family processes related to fears regarding child’s prognosis and
long-term nature of illness
• Be certain to give parents a chance to talk about how the bleeding began (e.g., “I
should have noticed that toy had a sharp edge,” “He fell from his bike. I should have
watched him more closely”). Parents often blame themselves, so it is important to be
sensitive and nonjudgmental. It is extremely important for parents to allow the child to
lead a normal life, so remind them when they do this that it is impossible to prevent all
injuries.
 VON WILLEBRAND DISEASE
• an inherited autosomal dominant disorder
• affects both sexes and is most common among Whites
• Along with a factor VIII defect, there is also an inability of the platelets to
aggregate and the blood vessels to constrict to aid in coagulation.
• Assessment:
• prolonged Bleeding time
• Epistaxis
• In girls, menstrual flow is unusually heavy
• Childbirth is obviously a risk for women with von Willebrand disease, so women
must be monitored closely for 9 months and again during the postpartal period
• Treatment:
• factor VIII replenishment
• administration of desmopressin (DDAVP)
 CHRISTMAS DISEASE
(HEMOPHILIA B, FACTOR IX DEFICIENCY)

• first noted in 1952 in a patient by the name of Stephen Christmas

• caused by factor IX deficiency
• transmitted as a sex-linked recessive trait, and only approximately 15% of
people with hemophilia have this form.
• Treatment: concentrate of factor IX, which is available for home
administration
 HEMOPHILIA C (FACTOR XI
DEFICIENCY) HEMOPHILIA C
• plasma thromboplastin antecedent deficiency
• caused by factor XI deficiency
• transmitted as an autosomal recessive trait, and occurs in both sexes.
• The symptoms are generally mild compared with those in children with factor
VIII or factor IX deficiencies.
• Treatment:
• administration of desmopressin (DDAVP)
• transfusion of fresh blood or plasma
 DISSEMINATED INTRAVASCULAR
COAGULATION (DIC)
• an acquired disorder of blood clotting that results from excessive trauma or
some similar underlying stimulus
• A child begins to develop petechiae or have uncontrolled bleeding from
puncture sites from injections or intravenous therapy. Ecchymoses and
petechiae form on the skin.
• Observe all children with a serious illness carefully for signs such as these of
increased bleeding to help identify that this is happening.