THE CHILD WITH

HEMATOLOGIC DISORDERS

GROUP 3
 SUBMITTED BY:

Jandoquile, Gerlie Ann

Labastida, Jaeveelyn
Lagaras, Maricar
Lagbo, Ilonah Grace
Lagunzad, Samantha
Lamerez, Jeryl
Lantajo, Ma. Francel
Lizarde,Trisha
Lopera, Frances
Lugasan, Nikki

BSN 2-A
SUBMITTED TO:
Ms. Fritzie Necitas A. Duran, RN
Clinical Instructor
HEMATOLOGIC
DISORDERS
• often called blood dyscrasias, occur when components of the
blood are formed incorrectly or either increase or decrease in
amount beyond normal ranges. Most blood dyscrasias in children
originate in the bone marrow, where blood cells are formed.
DISORDERS
OF THE
RED BLOOD
CELLS
NORMOCHROMIC,
NORMOCYTIC ANEMIAS
Acute Blood-Loss Anemia
Anemia of Acute Infection
Anemia of Renal Disease
Anemia of Neoplastic Diseases
Hyperplenism
Aplastic Anemias
Hypoplastic Anemias
ACUTE BLOOD-LOSS ANEMIA
• Blood loss that is sufficient to cause anemia can occur from
trauma such as an automobile accident with internal
bleeding; from acute nephritis in which blood is lost in the
urine; or in the newborn from disorders such as placenta
previa, premature separation of the placenta, maternal–fetal
or twin-to-twin transfusion, or trauma to the cord or placenta.

• In childhood, it can occur from the action of long-term

intestinal parasites such as a tapeworm or hookworm or, in
small infants, bedbug bites. With sudden blood loss, children
immediately appear pale. Because their heart must push the
reduced amount of blood through their body more rapidly
than usual, tachycardia will occur.
• Children will also begin to breathe rapidly because body
cells are still not able to receive adequate oxygen.

• Newborns may have gasping respirations, intercostal

retractions, and cyanosis.

• Children with rapid heart and respiratory rates due to

this do not respond well to oxygen therapy because they
lack RBCs to transport and use the oxygen. They
become listless and inactive, dizzy, and, possibly,
comatose. This type of acute blood-loss anemia
generally is transitory because the sudden reduction in
available oxygen stimulates the release of erythropoietin
from the kidney and a regeneration response in the bone
marrow.
• The reticulocyte counts rises, which is evidence that the
bone marrow is trying to increase production of
erythrocytes to meet the sudden shortage.
 Treatment involves:
• control of bleeding by addressing its underlying cause.
• The child or infant should be placed in a supine
NURSING position to provide as much circulation as possible to

MANAGEMENT • Keep the child warm with blankets; place an infant in

brain cells.

an incubator or under a radiant heat warmer.

• Blood transfusion may be necessary to provide an
immediate increase in the number of erythrocytes.
 Until blood is available for transfusion, a blood
NURSING expander such as plasma or intravenous fluid

MANAGEMENT such as normal saline or Ringer’s lactate 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.
NURSING
MANAGEMENT
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, and this causes an
accompanying decrease in erythropoietin production.

• This decreases the stimulation for

RBC production in the bone marrow, and a resultant
normocytic, normochromic anemia occurs.
 •
NURSING Administration of recombinant
human erythropoietin can increase
MANAGEMENT RBC production and correct the
anemia, but not the renal disease.
ANEMIA OF NEOPLASTIC DISEASE
• Malignant growths such as leukemia or
lymphosarcoma (common neoplasms of childhood)
result in normochromic, normocytic anemias because
invasion of bone marrow by proliferating neoplastic
cells impairs RBC production. There may be
accompanying blood loss if platelet formation also has
decreased.
 •
NURSING The treatment of such an
anemia involves measures
MANAGEMENT designed to achieve remission of
the neoplastic process and
transfusion to increase the
erythrocyte count.
HYPERSPLENISM
• Under normal conditions, blood is filtered rapidly
through the spleen. If the spleen is enlarged and
functioning abnormally, blood cells pass through more
slowly, with more cells being destroyed in the process.
This increased destruction of RBCs can cause anemia
and may lead to pancytopenia (deficiency of all cell
elements of blood). Virtually any underlying splenic
condition can cause this syndrome.
 Therapeutic management consists of:
• Treating the underlying splenic disorder,

NURSING including possible splenectomy. Although the

spleen’s role in the body’s defense mechanisms
MANAGEMENT against infection is not well documented, the
organ appears to be relatively important in early
infancy. Its function decreases as a child grows
older, and it may serve no immune function at all
in adulthood. If the spleen is removed, there is
no decrease in general immunity or in gamma
globulin or antibody formation.
 With the removal of the spleen’s filtering
function, however, there seems to be an increased

NURSING susceptibility to meningitis or pneumonia due to

pneumococci.
MANAGEMENT • For this reason, a splenectomy may be delayed
until after 2 years of age, when the risk of
meningitis decreases.
• Such children should receive immunization
against influenza, pneumococci, and H.
influenzae in addition to prophylactic penicillin
for 2 years after the splenectomy.
APLASTIC ANEMIAS
• Aplastic anemias result from depression of
hematopoietic activity in the bone marrow. The
formation and development of WBCs, platelets, and
RBCs can all be affected.
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.
• Between 4 and 12 years of age, a child begins to
manifest symptoms of pancytopenia, or reduction
of all blood cell components.
Acquired aplastic anemia
• is a decrease in bone marrow production that can occur if
a child is exposed excessively to radiation, drugs, or
chemicals known to cause bone marrow damage.
• Drugs that may cause this include chloramphenicol,
sulfonamides, arsenic (contained in rat poison, sometimes
eaten by children), hydantoin, benzene, or quinine.
Exposure to insecticides also may cause severe bone
marrow dysfunction. Chemotherapeutic drugs temporarily
reduce bone marrow production. A serious infection such
as meningococcal pneumonia might cause
autoimmunologic suppression of the bone marrow.
 • The ultimate therapy for both congenital
and acquired aplastic anemia is hemopoietic

NURSING stem cell transplantation.

MANAGEMENT • If a donor cannot be located, the disease is

managed by procedures to suppress T-
lymphocyte–dependent autoimmune responses
with antithymocyte globulin (ATG) or
cyclosporine or transfusion of new blood
elements. ATG, given intravenously, must
always be administered cautiously because of
the high risk for anaphylaxis.
 • Packed RBCs and platelet transfusions are
generally necessary to maintain adequate blood
elements. Prophylactic platelet transfusions

NURSING may be given.

• An RBC-stimulating factor (erythropoietin)
MANAGEMENT may be helpful.
• Colony-stimulating factors may also improve
bone marrow function.
• Some children show improvement with a
course of an oral corticosteroid (prednisone).
• Testosterone to stimulate RBC growth may be
tried.
 • For children who receive a hematopoietic stem

NURSING cell transplant, chances of complete recovery

are good. For others, the course is uncertain.
MANAGEMENT
• A decreased platelet count may persist for
years after other blood elements have returned
to normal. Bleeding, therefore, especially
petechiae or purpura, may be a long-term
problem.
 • Any drug or chemical suspected of causing the
bone marrow dysfunction must be

NURSING discontinued at once and the child must never

be exposed to that substance again.
MANAGEMENT • Children with aplastic anemia are apt to be
irritable because of their fatigue and recurring
symptoms. Their parents may feel responsible
for causing the illness if it originated from
exposure to a chemical such as an insecticide.
 • Many parents will have less confidence in health care
personnel if the illness followed treatment with a drug

NURSING such as chloramphenicol. When discussing with parents

the outcome of this disease, be conservatively optimistic.
MANAGEMENT
• It may be easier for parents to deal with this problem if
they face only one day or one blood test at a time rather
than trying to predict the outcomes of all the blood tests to
come. They need to feel they can discuss their frustration
and bitterness about continual abnormal results with health
care personnel.
 • Establishing good communication with
NURSING these parents does much to establish their

MANAGEMENT trust in everyone caring for their child.

HYPOPLASTIC ANEMIAS
• also result from depression of hematopoietic activity
in bone marrow; they can be either congenital or
acquired.

• Unlike aplastic anemias, in which WBCs, RBCs, and

platelets are affected, in hypoplastic anemias ONLY
RBCs are affected.
Congenital hypoplastic anemia (Blackfan-
Diamond syndrome)
• is a rare disorder that shows symptoms as early as
the first 6 to 8 months of life. It affects both sexes
and is apparently caused by an inherent defect in
RBC formation. No changes in the leukocytes or
platelets occur.

Acquired form
• is caused by infection with parvovirus, the
infectious agent of fifth disease. The onset of
hypoplastic anemia is insidious, and at first it may
be difficult to differentiate from iron-deficiency
anemia.
• In iron-deficiency anemia, blood cells
appear hypochromic and microcytic; in
hypoplastic anemia, they are normochromic
and normocytic but few in number.
 • With acquired hypoplastic anemia, the
NURSING reduction of RBCs is transient, so no therapy

MANAGEMENT is necessary.

• Children with the congenital form show

increased erythropoiesis with corticosteroid
therapy.
 • Long-term transfusions of packed RBCs are
NURSING needed to raise erythrocyte levels. As a result

MANAGEMENT of the necessary number of transfusions,

hemosiderosis (deposition of iron in body
tissue) can occur.

• Therefore, an iron chelation program such as

subcutaneous infusion (hypodermoclysis) of
deferoxamine (Desferal) may be started
concurrently with transfusions.
 • Deferoxamine binds with iron and aids its
excretion from the body in urine; it is given 5
or 6 days a week over an 8-hour period. This
NURSING is one of the few times that an infusion is

MANAGEMENT given subcutaneously. Parents can do this at

home after careful instruction, often while
their child is asleep at night. The parent must
assess that voiding is present and specific
gravity is normal (1.003 to 1.030) before drug
administration.
 • For such an infusion, an area beside the
scapula or on the thigh is cleaned with alcohol;
a short 25-gauge needle is inserted at a low

NURSING angle into only the subcutaneous tissue. The

medication is then allowed to infuse slowly.
MANAGEMENT
• Periodic slitlamp eye examinations should be
scheduled to check for cataract formation, a
possible adverse effect of deferoxamine.
 • Congenital hypoplastic anemia is a
NURSING chronic condition. However,
MANAGEMENT approximately one fourth of affected
children will undergo spontaneous
permanent remission before age 13
years.
HYPOCHROMIC
ANEMIAS
 Iron-Deficiency Anemia
 Chronic Infection Anemia

• When hemoglobin synthesis is inadequate, the

erythrocytes appear pale (hypochromia).
Hypochromia is generally accompanied by a
reduction in the diameter of cells (RBCs are also
microcytic).
IRON-DEFICIENCY ANEMIA
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 is stored in
the bone marrow to be available for haemoglobin
production.

• Inadequate dietary iron prevents proper

haemoglobin formation. With iron-deficiency
anemia, RBCs are both small in size (hypocytic)
and pale (hypochromic) due to the stunted
hemoglobin.
• Children are at high risk for iron-deficiency anemia
because they need more daily iron in proportion to
their body weight to maintain an adequate iron level
than do adults. This results in a necessary daily
intake of 6 to 15 mg of iron.

• Iron-deficiency anemia occurs most often between

ages 9 months and 3 years; its frequency rises again
in adolescence, when iron requirements increase for
girls who are menstruating. It also is found in
overweight teenagers if they ingest most of their
calories from high-carbohydrate, not iron-rich,
foods.
Causes in Infants
• Several causes lead to iron-deficiency anemia.
When an infant’s diet lacks sufficient iron, the
infant usually has enough in reserve to last for the
first 6 months. After that, if the infant’s diet
continues to be iron deficient, there will be
difficulty forming adequate RBCs. Infants of low
birth weight have fewer iron stores than those born
at term because iron stores are laid down near the
end of gestation.
Because low-birth-weight infants also
grow rapidly and their need for RBCs
expands accordingly, they tend to
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 give birth to iron-
deficient babies because the babies do
not 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 osmosis.
• The loss of transferrin, a plasma protein responsible
for binding iron to protein to facilitate its
transportation to bone marrow after absorption from
the gastrointestinal tract, further depletes this system
of iron transport.
• Infants born with structural defects of the
gastrointestinal system, such as gastroesophageal
reflux (chalasia—an immature valve between the
esophagus and stomach, resulting in regurgitation) or
pyloric stenosis (narrowing between the stomach and
duodenum, resulting in vomiting), are particularly
prone to iron-deficiency anemia.
• Although their diet is adequate, they
cannot make use of the iron because it is
never 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 becomes constipated
while ingesting iron-rich formula, but this is
the exception rather than the rule.
Causes in Older Children
• In children older than 2 years, chronic
blood loss is the most frequent cause of
iron-deficiency anemia. This is caused by
gastrointestinal tract lesions such as
polyps, ulcerative colitis, Crohn’s disease,
protein-induced enteropathies, parasitic
infestation, or frequent epistaxis.
Adolescent girls can become iron deficient
because of frequent attempts to diet and
overconsumption of snack foods low in
iron. Without sufficient iron, their body
cannot compensate for the iron lost with
menstrual flow.
 • Therapy for iron-deficiency anemia focuses on
treatment of the underlying cause. Sources of
gastrointestinal bleeding must be ruled out.
NURSING
• The diet must be rich in iron and should contain
MANAGEMENT extra vitamin 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
• Acute infection interferes with RBC production,
producing a normochromic, normocytic anemia.
When infections are chronic, anemia of a
hypochromic, microcytic type occurs. This is
probably caused by impaired iron metabolism as well
as impaired RBC production.
NURSING
MANAGEMENT
• The degree of anemia is rarely as severe
as that occurring with iron deficiency.
Administration of iron has little effect until
the infection is controlled.
MACROCYTIC
(MEGALOBLASTIC) ANEMIAS
• is one in which the RBCs are abnormally large. These
cells are actually immature erythrocytes or megaloblasts
(nucleated immature red cells).
• For this reason, these anemias are often referred to as
megaloblastic anemias. Because these anemias are
caused by nutritional deficiencies, they occur less
frequently in the United States than in developing
countries.

 Anemia of Folic Acid

 Pernicious Anemia (Vitamin B12 Deficiency)
ANEMIA OF FOLIC ACID DEFICIENCY
• A deficiency of folic acid combined with vitamin C
deficiency produces an anemia in which the
erythrocytes are abnormally large. There is often
accompanying neutropenia and thrombocytopenia.
Mean corpuscular volume and mean corpuscular
hemoglobin are increased, whereas mean corpuscular
hemoglobin concentration is normal. Bone marrow will
contain megaloblasts, indicating inhibition of the
production of erythrocytes at an early stage.
Megaloblastic arrest, or inability of RBCs to
mature past an early stage, may occur in the
first year of life from the continued use of
infant food containing too little folic acid or
from an infant drinking goat’s milk, which
tends to be deficient in folic acid.
NURSING
• Treatment is daily oral
MANAGEMENT administration of folic acid.
Response to treatment is dramatic.
PERNICIOUS ANEMIA (Vitamin B12 Deficiency)
Vitamin B12 is necessary for maturation of
RBCs. Pernicious anemia results from
deficiency or inability to use the vitamin.
• In children, the cause is more often lack of
ingestion of vitamin B12.

• The vitamin is found primarily in foods of

animal origin, including both cow’s milk
and breast milk.
• Adolescents may be deficient in vitamin
B12 if they are ingesting a long-term,
poorly formulated vegetarian diet. For
absorption of vitamin B12 from the
intestine, an intrinsic factor must be
present in the gastric mucosa.
• In adults, lack of the intrinsic factor is the most frequent
cause of the disorder. If a child has an intrinsic factor
deficiency, symptoms can occur as early as the first 2
years of life (once the intrauterine stores of vitamin B12
have been exhausted).
• The child appears pale, anorexic, and irritable, with
chronic diarrhea. The tongue appears smooth and beefy
red due to papillary atrophy. In children, neuropathologic
findings such as ataxia, hyporeflexia, paresthesia, and a
positive Babinski reflex are less noticeable than in adults.
 • Laboratory findings reveal low serum levels of
vitamin B12.

NURSING • The rate and efficiency of absorption of vitamin

B12 can be tested by the ingestion of the
MANAGEMENT radioactively tagged vitamin. The dose
absorbed in the presence and absence of a dose
of intrinsic factor can be measured.
• If the anemia is caused by a B12-deficient diet,
temporary injections of B12 will reverse the
symptoms.
 • If the anemia is caused by lack of the intrinsic
NURSING factor, lifelong monthly intramuscular
injections of B12 may be necessary. Parents
MANAGEMENT and the child need to understand that lifelong
therapy will be necessary.
HEMOLYTIC ANEMIAS
• are those in which the number of erythrocytes decreases
due to increased destruction of erythrocytes.

• This may be caused by fundamental abnormalities of

erythrocyte structure or by extracellular destruction
forces.

 Congenital Spherocytosis
 Glucose-6-Phosphate Dehydrogenase Deficiency
 Sickle-Cell Anemia
CONGENITAL SPHEROCYTOSIS
• is a hemolytic anemia that is inherited as an autosomal
dominant trait.
• It occurs most frequently in the white Northern European
population.
• RBCs are small and defective, apparently due to
abnormalities of the protein of the cell membrane that make
them unusually permeable to sodium.
• The life span of erythrocytes is diminished.
• The disease may be noticed shortly after birth, although
symptoms may appear at any age.
• The hemolysis of RBCs appears to occur in the spleen,
apparently from excessive absorption of sodium into the
cell. The abnormal cell swells, ruptures, and is destroyed.

• Chronic jaundice and splenomegaly develop.

• The mean corpuscular hemoglobin concentration is

increased because the cells are small.
• Gallstones may be present in the older
school-age child and adolescent
because of the continuous hemolysis,
bilirubin release, and incorporation of
bilirubin into gallstones.

• Infections may precipitate a crisis or

cause bone marrow failure. During such
a period, the anemia increases rapidly
as the hemolysis continues.
• Blood transfusion will be necessary to
maintain a sufficient number of
circulating erythrocytes until the crisis
passes.

• The diagnosis of the disease is based on

family history, the obvious hemolysis,
and the presence of the abnormal
spherocytes.
 • The treatment is generally
NURSING splenectomy at approximately 5 to 6

MANAGEMENT years. This measure will increase the

number of RBCs present but will not
alter their abnormal structure.
GLUCOSE-6-PHOSPHATE DEHYDROGENASE
DEFICIENCY
• The enzyme glucose-6-phosphate
dehydrogenase (G6PD) is necessary for
maintenance of RBC life.

• Lack of the enzyme results in premature

destruction of RBCs.

• The disease is transmitted as a sex-linked

recessive trait.
GLUCOSE-6-PHOSPHATE DEHYDROGENASE
DEFICIENCY
• It occurs most frequently in children of African
American, Asian, Sephardic Jewish, and
Mediterranean descent. Approximately 10% of
African American males have the disorder.

• Because the disease is sex-linked, males of high-risk

groups should be screened in infancy. G6PD occurs in
two identifiable forms.
• Children with congenital nonspherocytic
hemolytic anemia have hemolysis, jaundice,
and splenomegaly and may have aplastic
crises.
• Other children have a drug-induced form in
which the blood pattern is normal until the
child is exposed to fava beans or drugs such
as antipyretics, sulfonamides, antimalarials,
and naphthaquinolones (the most common
drug in these groups is acetylsalicylic acid
[aspirin]). Approximately 2 days after
ingestion of such an oxidant drug, the child
begins to show evidence of hemolysis.
• A blood smear will show Heinz bodies (oddly
shaped particles in RBCs). The degree of RBC
destruction depends on the drug and the
extent of exposure to it. The child may have
accompanying fever and back pain.
Occasionally a newborn is seen with marked
hemolysis because the mother ingested an
initiating drug during pregnancy.
 • G6PD deficiency may be
diagnosed by a rapid enzyme

NURSING screening test or electrophoretic

analysis of RBCs. Drug induced
MANAGEMENT hemolysis usually is self-limiting,
and blood transfusions are rarely
necessary. Both parents and
children should be told of the
abnormality in the child’s
metabolism so they can avoid
common drugs such as
acetylsalicylic acid.
SICKLE-CELL ANEMIA
• is the presence of abnormally shaped (elongated)
RBCs. It is an autosomal recessive inherited
disorder 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 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, 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
becomes ischemic, resulting in acute pain and cell
destruction.
• Because fetal hemoglobin contains a gamma, not a
beta, chain, the disease usually will not result in
clinical symptoms until a child’s hemoglobin
changes from the fetal to the adult form at
approximately 6 months.
• However, the disease can be diagnosed prenatally by
chorionic villi sampling or from cord blood during
amniocentesis. If these were not done, it can be
identified at birth by neonatal screening.

• The abnormal form of hemoglobin in this disorder is

designated hemoglobin S. A child with sickle cell
disease is said to have hemoglobin SS (homozygous
involvement).
• Both parents of the child with the disease will
have 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 produced is abnormal.
They produce enough normal hemoglobin to
compensate for the hemoglobin that is sickled
and therefore show no symptoms
• . A child with the disease (homozygous) produces no
normal hemoglobin and so shows characteristic
symptoms of sickle-cell anemia.

• A very few children have combinations of

hemoglobin S and hemoglobin C or E, leading to
mild anemia.
Sickle-Cell Crisis
• is the term used to denote a sudden, severe onset of
sickling.

• Symptoms of crisis occur from pooling of many new

sickled cells in vessels and consequent tissue hypoxia
beyond the blockage (a vaso-occlusive crisis).

• A sickle-cell crisis can occur when a child has an illness

causing dehydration or a respiratory infection that results in
lowered oxygen exchange and a lowered arterial oxygen
level, or after extremely strenuous exercise (enough to lead
to tissue hypoxia).
Sometimes no obvious cause of a crisis can be found.
Symptoms are sudden, severe, and painful.

• Laboratory reports reveal a hemoglobin level of only

6 to 8 g/100 mL.

• A peripheral blood smear demonstrates sickled cells.

• The WBC count is often elevated to 12,000 to

20,000/mm3. Bilirubin and reticulocyte levels are
increased.
• Further complications that may occur are
aseptic necrosis of the head of the femur or
humerus with increased joint pain or a
cerebrovascular accident that occurs from a
blocked artery, causing loss of motor function,
coma, seizures, or even death. If there is renal
involvement, hematuria or flank pain may be
present.
Additional types of crisis may occur.
• A sequestration crisis may occur when there is splenic
sequestration of RBCs or severe anemia occurs due to pooling
and increased destruction of sickled cells in the liver and
spleen). This leads to shock from hypovolemia. The spleen is
enlarged and tender.
• A hyperhemolytic crisis can occur when there is increased
destruction of RBCs. A megaloblastic crisis may occur if the
child has folic acid or vitamin B deficiency (new RBCs cannot
be fully formed due to lack of these ingredients).
• An aplastic crisis is manifested by severe anemia due to a
sudden decrease in RBC production. This form usually occurs
with infection.
 • The child in sickle-cell crisis has three
primary needs: pain relief and adequate
NURSING hydration and oxygenation to prevent further

MANAGEMENT sickling and halt the crisis.

• Acetaminophen (Tylenol) may be adequate pain
relief for some children; for others, a narcotic
analgesic such as intravenous morphine may be
needed. Once children are pain free, they are
able to relax, reducing the metabolic demand
for oxygen and helping to end the sickling.
 • Hydration is generally accomplished with
intensive intravenous fluid replacement therapy.
NURSING • Tissue hypoxia leads to acidosis. The acidosis

MANAGEMENT must be corrected by electrolyte replacement.

• Some kidney infarction may have occurred, so
do not administer potassium intravenously until
kidney function has been determined (the child
is voiding). Otherwise, excessive potassium
levels may occur, possibly leading to cardiac
arrhythmias.
 • If infection appears to be the precipitator for a
sickling crisis, blood and urine cultures, a chest
radiograph, and a complete blood count will be
NURSING taken and the infection will be treated by

MANAGEMENT antibiotics.

• Blood transfusion (usually packed RBCs) may

be necessary to maintain the hemoglobin above
12 g/dL (termed hypertransfusion).
 • Hydroxyurea, an antineoplastic agent that has
the potential to increase the production of
hemoglobin F (fetal hemo-globin), can be used
NURSING in children with sickle-cell disease to increase

MANAGEMENT their overall hemoglobin level.

• The drug, given orally, can cause anorexia.

Therefore, monitor the child’s nutrition intake
during this drug therapy.
 • If none of the above measures appears to be
effective, children may be given an exchange
transfusion to remove most of the sickled cells
NURSING and replace them with normal cells. Exchange

MANAGEMENT transfusion must be done with small amounts of

blood at each exchange. Otherwise, the pressure
changes can cause such irregularities in blood
volume that heart failure results. Hematopoietic
stem cell transplantation is possible for the child
who does not respond to usual therapies.
THALASSEMIAS
• are autosomal recessive anemias associated with
abnormalities of the beta chain of adult haemoglobin
(HgbA).

Thalassemia Minor (Heterozygous β-Thalassemia)

Thalassemia Major (Homozygous β-Thalassemia)
Autoimmune Acquired Hemolytic Anemia
THALASSEMIA MINOR (Heterozygous Beta-
Thalassemia)
• Children with thalassemia minor, a mild form of
this anemia, produce both defective beta
hemoglobin and normal hemoglobin. Because
there is some normal production, the RBC count
will be normal, but the hemoglobin concentration will
be decreased 2 to 3 g/100 mL below normal levels. The
blood cells are moderately hypochromic and microcytic
because of the poor hemoglobin formation.
 Children may have no symptoms other than
pallor. They require no treatment, and life
expectancy is normal. They should not receive a
NURSING routine iron supplement because their inability to

MANAGEMENT incorporate it well into hemoglobin may cause

them to accumulate too much iron. The condition
represents the heterozygous form of the disorder
or can be compared with children having the
sickle-cell trait.
THALASSEMIA MAJOR (Homozygous Beta-Thalassemia)
Thalassemia major is also called Cooley’s anemia or
Mediterranean anemia. Because
thalassemia is a beta chain hemoglobin defect,
symptoms do not become apparent until a child’s fetal
hemoglobin has largely been replaced by adult hemoglobin
during the second half of the first year of life. Unable to
produce normal beta hemoglobin, the child shows symptoms
of anemia: pallor, irritability, and anorexia.
RBCs are hypochromic (pale) and microcytic (small).
Fragmented poikilocytes and basophilic stippling (unevenness
of hemoglobin concentration) are present. The haemoglobin
level is less than 5 g/100 mL. The serum iron level is high
because iron is not being incorporated into hemoglobin; iron
saturation is 100%.
 • Digitalis, diuretics, and a low sodium diet may
be prescribed to prevent heart failure, which
could result from the decompensation that
NURSING accompanies anemia, and from myocardial

MANAGEMENT fibrosis caused by invasion of iron

(hemosiderosis).

• Transfusion of packed RBCs every 2 to 4 weeks

(hypertransfusion therapy) will maintain
haemoglobin between 10 and 12 g/100 mL. With
this level of hemoglobin, erythropoiesis is
suppressed and cosmetic facial alterations,
osteoporosis, and cardiac dilatation are
minimized.
 • Hypertransfusion therapy also reduces the
possibility that splenectomy will be necessary.
Frequent blood transfusions, unfortunately,
NURSING increase the risk of blood-borne disease, such as

MANAGEMENT hepatitis B, and hemosiderosis.

• Children may receive an oral iron-chelating

agent to remove this excessive store of iron, such
as deferasirox. Others receive deferoxamine
given subcutaneously over 6 to 8 hours as they
sleep at night.
 • Splenectomy may become necessary to reduce
discomfort and also to reduce the rate of RBC
hemolysis and the number of transfusions
NURSING needed. Bone marrow stem cell transplantation

MANAGEMENT can offer a cure. With treatment, the overall

prognosis of thalassemia is improving but still
grave. Most children with the disease die of
cardiac failure during adolescence or as young
adults if they do not receive a hematopoietic
stem cell transplant.
AUTOIMMUNE ACQUIRED HEMOLYTIC ANEMIA
Autoimmune antibodies (abnormal antibodies of the IgG
class) attach themselves to RBCs, destroying them or causing
hemolysis. This may occur at any age, and its origin is
generally idiopathic, although the disorder may be associated
with malignancy, viral infections, or collagen diseases such as
rheumatoid arthritis or systemic lupus erythematosus.
• A child may recently have had an upper respiratory
infection, measles, or varicella virus infection (chickenpox).
Such hemolysis may occur after the administration of drugs
such as quinine, phenacetin, sulfonamides, or penicillin.

• The exact cause is unknown but appears to involve a

change in the RBCs themselves, making them act as
antigens, or a change in antibody production, making
antibodies destructive to other substances.
 • In some children, the disease process runs a
limited course and no treatment is necessary.
• In others, a single blood transfusion may
NURSING correct the disturbance. For these children, it is

MANAGEMENT difficult to cross-match blood for transfusion

because the red cell antibody tends to clump or
agglutinate all blood tested. If cross-matching is
impossible, the child may be given type O Rh-
negative blood. Observe the child carefully
during any transfusion for signs of transfusion
reaction.
 • If anemia is persistent, corticosteroid therapy
(oral prednisone) to reduce the immune response is
generally effective, increasing the RBC count and
NURSING •
hemoglobin concentration in a short period.
For some children, stronger immunosuppressive
MANAGEMENT agents such as cyclophosphamide (Cytoxan) or
azathioprine (Imuran) are necessary to reduce
antibody formation. If these are ineffective,
splenectomy may be necessary. Often it is difficult
for parents to understand the process causing their
child’s condition.
NURSING • Provide the parents and child with support as they
wait for this unexplainable process to run its course
MANAGEMENT and for their child to be well again.
POLYCYTHEMIA
POLYCYTHEMIA
• is an increase in the number of RBCs. The condition results
from increased erythropoiesis, which occurs as a
compensatory response to insufficient oxygenation of the
blood in order to help supply more oxygen to body cells.
• Chronic pulmonary disease and congenital heart disease are
the usual causes of polycythemia in childhood.
• Also, it may occur from the lower oxygen level maintained
during intrauterine life in newborns or with twin transfusion
at birth (one twin receives excess blood while a second twin
is anemic).
Plethora (marked reddened appearance of the skin) occurs
because of the increase in total RBC volume. Erythrocytes are
usually macrocytic (large) and the hemoglobin content is high.
This means that the mean corpuscular hemoglobin will be
elevated; the mean corpuscular hemoglobin concentration,
however, will be normal, indicating that, although many in
number, each erythrocyte is normally saturated with
hemoglobin. The RBC count may be as high as 7
million/mm3. Hemoglobin levels may be as high as 23 g/100
mL.
 Treatment of polycythemia involves treatment
of the underlying cause. Because of the high
NURSING blood viscosity from so many crowded blood

MANAGEMENT cells, cerebrovascular accident or emboli may

occur. The risk increases particularly if the child
becomes dehydrated, such as with fever or
during surgery.
Exchange transfusion or phlebotomy to reduce
the RBC count may be necessary.
DISORDERS OF
BLOOD
COAGULATION
Platelets are necessary for blood coagulation, so disorders that
limit the number of platelets limit the effectiveness of this
process. A normal platelet level is 150,000/mm3.
Thrombocytopenia (decreased platelet count) is defined as a
platelet count of less than 40,000/mm3. Thrombocytopenia
often leads to purpura, or blood seeping from vessels into the
skin. In one rare disorder, children are born with
thrombocytopenia and are also missing the radius bone in the
forearm (TAR [thrombocytopenia/absent radius] syndrome).
Purpuras
• a hemorrhagic rash or small hemorrhages in the superficial
layer of skin. Two main types of purpura occur in children:
idiopathic thrombocytopenia purpura and Henoch-
Schönlein syndrome.

 Idiopathic Thrombocytopenic Purpura

 Henoch–Schönlein Syndrome
IDIOPATHIC THROMBOCYTOPENIC PURPURA
• is the result of a decrease in the number of circulating
platelets in the presence of adequate megakaryocytes
(precursors to platelets).

• The cause is unknown, but it is thought to result from an

increased rate of platelet destruction due to an antiplatelet
antibody that destroys platelets making this an autoimmune
illness.
• In most instances, ITP occurs approximately 2 weeks after a
viral infection such as rubella, rubeola, varicella, or an
upper respiratory tract infection.

• Congenital ITP may occur in the newborn of a woman who

has had ITP during pregnancy. An antiplatelet factor
apparently crosses the placenta and causes platelet
destruction in the newborn in the same way that Rh
incompatibility or hemolytic disease of the newborn
develops. However, in ITP, the platelets, not the RBCs, are
sensitized.
 • Oral prednisone to reduce the immune
response and intravenous immunoglobulin
(IVIG) or, in Rh-positive children, anti-D
NURSING immunoglobulin to supply anti-ITP antibodies
are used to treat ITP.
MANAGEMENT
• Platelet transfusion will temporarily increase
the platelet count, but because the life span of
platelets is relatively short, a platelet
transfusion has only limited effect. Children
with central nervous system bleeding may
require a splenectomy although this is rarely
necessary.
 • If the child experiences joint pain from
bleeding, acetaminophen (Tylenol) rather than
NURSING salicylates or ibuprofen is prescribed for pain.

MANAGEMENT Both salicylates and ibuprofen increase the

chance for bleeding as they prevent the
aggregation of platelets at wound sites.
• In most children, ITP runs a limited, 1- to 3-
month course. A few children develop chronic
ITP. A course of immunosuppressive drugs
may be attempted if the chronic state persists.
NURSING All children need to be vaccinated against the
MANAGEMENT viral diseases of childhood so that diseases such as
rubella, rubeola, and varicella are eradicated and
can no longer lead to this defective coagulation
process.
Henoch-Schönlein Syndrome
Henoch-Schönlein purpura (also called anaphylactoid
purpura) is caused by increased vessel permeability. Although
no definite allergic correlation can be identified, it is generally
considered to be a hypersensitivity reaction to an invading
allergen. It occurs most frequently in children between 2 and 8
years of age, and more frequently in boys than girls. 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.
 Treatment involves oral corticosteroid therapy
(prednisone) and mild analgesics for a short period.
NURSING Nose and throat cultures rule out continuing

MANAGEMENT bacterial involvement. Urine should be assessed

for protein and glucose to detect kidney
involvement. Typically the disease runs a course of
4 to 6 weeks. A few children develop chronic
nephritis as a complication.
Hemophilia
 Hemophilia A (Factor VIII Deficiency)
 von Willebrand Disease
 Christmas Disease (Hemophilia B, Factor IX Deficiency)
 Hemophilia C (Factor XI Deficiency)
 Disseminated Intravascular Coagulation
Hemophilia A (Factor VII Deficiency)
The classic form of hemophilia is caused by deficiency of the
coagulation component factor VIII, the antihemophilic factor.
It transmitted as a sex-linked recessive trait. In the United
States, the incidence is approximately 1 in 10,000 white
males. The female carrier may have slightly lowered but
sufficient levels of the factor VIII component so that she does
not manifest a bleeding disorder.
Males with the disease also have varying levels of factor VIII,
and their bleeding tendency varies accordingly, from mild to
severe.Factor VIII is an intrinsic factor of coagulation, so the
intrinsic system for manufacturing thromboplastin is
incomplete. The child’s coagulation ability is not absent
because the extrinsic or tissue system remains intact. Because
of this system, the child’s blood will eventually coagulate after
an injury.
 • Prevent bleeding.
• Monitor hemoglobin and hematocrit levels;
• assess for inhibitor antibody to factor VIII;
NURSING • anticipate or instruct in the need for

MANAGEMENT prophylactic treatment before high-risk

situations, such as invasive diagnostic or
surgical procedures, or dental work; and
provide replacement therapy of deficient
clotting factors.
 • With even minor abrasions, bleeding must be
controlled by the administration of factor
VIII. This may be supplied by fresh whole
NURSING blood or by fresh frozen plasma, but it is best

MANAGEMENT supplied by a concentrate of factor VIII.

 • Relieve pain. Immobilize joints and apply
NURSING elastic bandages to the affected joint if
indicated; elevate affected and apply a cold
MANAGEMENT compress to active bleeding sites, but must be
used cautiously in young children to prevent
skin breakdown.

• Prophylactic administration, although

expensive, may best reduce bleeding episodes.
von Willebrand Disease
• an inherited autosomal dominant disorder affecting both
sexes, is often referred to as a angiohemophilia.

• Along with a factor VIII defect, there is also an inability of

the platelets to aggregate. In addition, the blood vessels
cannot constrict to aid in coagulation.

• Bleeding time is prolonged, with most hemorrhages

occurring from mucous membrane sites.
Epistaxis is a major problem, because all children tend to rub
or pick at their nose as a nervous mechanism. In girls,
menstrual flow is unusually heavy and may cause
embarrassment from stained clothing. Childbirth is obviously
a risk for women with von Willebrand’s disease. Bleeding is
controlled with factor VIII replenishment as with hemophilia
or by administration of DDVAP, which stimulates factor VIII
disease.
 Treatments that can help prevent or stop bleeding
episodes depending on what type and severity your
condition is. Replacement therapies. These include:
NURSING • infusions of concentrated blood-clotting factors

MANAGEMENT • containing von Willebrand factor and factor VIII.

Use of oral contraceptives. For women, these can be
useful for controlling heavy bleeding during
menstrual periods. The estrogen hormones in birth
control pills can boost von Willebrand factor and
factor VIII activity. This effect is likely available
with birth control patches, though further study is
needed to confirm it.
Christmas Disease (Hemophilia B, Factor IX Deficiency)
• caused by factor IX deficiency, is transmitted as a sex-
linked recessive trait. Only approximately 15% of people
with hemophilia have this form.
NURSING
MANAGEMENT
Treatment is with a concentrate of factor IX,
available for home administration
Hemophilia C (Factor XI deficiency)
-or plasma thromboplastin antecedent deficiency, caused by
factor XI deficiency, is transmitted as an autosomal recessive
trait occurring in both sexes. The symptoms are generally mild
compared with those in children with factor VIII or factor IX
deficiencies.
NURSING
MANAGEMENT
Bleeding episodes are treated with administration of
DDAVP or transfusion of fresh blood or plasma.
Disseminated Intravascular Coagulation
• an acquired disorder of blood clotting that results from
excessive trauma or some similar underlying stimulus.

• Normal blood clotting is a balance between the hemostatic

(clotting) system and the fibrinolytic (dissolving) system of
the bloodstream. After a blood vessel injury, local
vasoconstriction rapidly prevents additional blood loss at the
site.
• With the tear in the vessel wall, the underlying collagen is
exposed. This causes platelets to swell and become adherent
and irregular in shape. They release adenosine diphosphate,
which attracts additional platelets and binds them together
(platelet aggregation). This phenomenon results in a platelet
plug to seal the vessel.
• The plug is strengthened by fibrin threads that form as a
result of an intrinsic and extrinsic coagulation process
into a firm, fixed structure.

• To prevent too much clotting from occurring, plasmin or

fibrinolysin, a proteolytic enzyme formed from
plasminogen, digests fibrin threads and causes lysis of the
clot along with consumption of blood clotting factors.

• As plasmin, fibrinogen, and fibrin are lysed, fibrin

degradation products are formed. These products prevent
the laying down of further fibrin and platelet aggregation.
• With DIC, an imbalance occurs between clotting activity
and fibrinolysis. Extreme clotting due to endothelial
damage begins at one point in the circulatory system,
depleting the availability of clotting factors such as
platelets and fibrin from the general circulation.

• A secondary initiation of fibrinolysis begins as well. A

paradox exists: the child has both increased coagulation
and a bleeding defect at the same time. Many of the
complications of pregnancy (abruptio placentae or death
of a fetus) initiate DIC, so this is a common complication
seen accompanying bleeding during pregnancy. DIC also
tends to occur in children with acute infections or trauma.
 • To halt the process of DIC, the underlying insult

NURSING that began the phenomenon must be halted.

MANAGEMENT interfere with the marked coagulation. Although

• Intravenous heparin administration helps to

blood transfusion may be necessary to correct

blood loss, it may be delayed until after heparin
has been administered so that the new blood
factors are not also consumed by the coagulation
process.
 • Fresh-frozen plasma, platelets, or

NURSING fibrinogen may be administered.

With adequate therapy, the results
MANAGEMENT of blood coagulation studies will
return to normal. If renal or brain
cells were damaged from occluded
capillaries, permanent injury to
these areas can result.
That in all things, God may
be glorified!