Pediatric Hematologic Disorder

1. NURSING CARE OF A
FAMILY WHEN A CHILD
HAS A HEMATOLOGIC
DISORDER
Dedace, Cheryll
CHAPTER 44:

2. Nursing Process: Hematologic Disorders
• Assessment
• Nursing diagnosis
• Outcome identification and planning
• Implementation
• Outcome evaluation

3. 2020 National Health Goals Related to
Hematologic Disorders in Children
•Reduce the incidence of iron deficiency among
children aged 1 to 2 years from a baselineof
15.9% to a targetlevel of 14.3%;in children aged 3
to 4 years, from 5.3% to 4.3%.
•Reduce the incidence of iron deficiency among
adolescents 12 to 18 years of age from10.4% to
9.4%.

4. •Reduce the proportion of persons
with hemophilia who develop reduced
joint mobility due to bleeding into joints
from 82.9% to 74.6%.
•(Developmental)
Reducehospitalization due to
preventable complications of sickle-
cell disease yearly among children
aged 9 years and under.
•Increase the proportion of
children with special
health care needs who
have access to a medical
home from 47.1% to
51.8%.
2020 National Health Goals Related to
Hematologic Disorders in Children— (cont.)

5. 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

6. Anatomy & Physiology of the Hematopoietic System
• Blood components originate in the bone marrow, circulate through blood vessels,
and ultimately are destroyed by the spleen.
Blood Formation & Components:
• Erythrocytes (Red Blood Cells) - transport oxygen to and carry carbon dioxide
away from body cells. They are formed in the bone marrow under the stimulation of
erythropoietin, a hormone formed by the kidneys that is produced whenever a child
has tissue hypoxia.
• Hemoglobin - composed of globin, a protein, and heme, an iron-containing
pigment. It is the heme portion that combines with oxygen and carbon dioxide for
transport. The component of RBCs that allows them to carry out the transport of
oxygen

7. • Bilirubin:
After an RBC reaches its life span of approximately 120 days, it disintegrates and its
protein component is preserved by the reticuloendothelial cells of the liver and spleen
for further use. Iron is reused by the bone marrow to construct new RBCs. 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.
• Leukocytes (WBCs) are nucleated cells and few in number compared with RBCs (thereis
approximately only 1 WBC to every 500 RBCs). Their primary function is defense against
antigen invasion; their life span varies from approximately 6 hours to unknown intervals.
With the exception of neutropenia (a reduced number of WBCs), leukocytesare not major
hematologic concerns; because they are important in immune disorders and malignancies.
• Thrombocytes are round, nonnucleated bodies formed by the bone marrow; their
function is capillary hemostasis and primary coagulation. The usual number is 150,000
to 300,000/mm3 after the first year. Immature thrombocytes are termed
megakaryocytes. If large numbers of these are present in serum, it indicates that a rapid
production of platelets is occurring.

8. Steps in Blood
Coagulation

10. • Bone marrow aspiration and biopsy
• Blood transfusion
• Hematopoietic stem cell transplantation
• Splenectomy
Therapeutic Techniques for a Child With a
Hematologic Disorder

11. Assessment and Therapeutic Techniques for Hematologic
Disorders
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 (Ambruso,
Nuss, & Wang, 2016). In children,
the aspiration sites used are the iliac crests or
spines (rather than the sternum, which is
commonly used in adults) because performing the
test at these sites is usually less
frightening for children (Fig. 44.2). These sites also
have the largest marrow
compartments during childhood. In neonates, the
anterior tibia can be used as an
additional site

12. BLOOD TRANSFUSION
Transfusions of blood or its products are
commonly used in the treatment of blood
disorders, and may include whole blood, packed
RBCs, washed RBCs (as much
“foreign” matter is removed as possible to reduce
the possibility of an antagonistic
reaction), plasma, plasma factors, platelets, WBCs,
and albumin. No matter what type of
blood product is used, 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

14. HEMATOPOIETIC STEM CELL
TRANSPLANTATION
Stem cell transplantation is 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
Stem cell transplantation can be allogeneic,
syngeneic, or autologous.
• Allogeneic transplantation is the transfer of
stem cells from an immune-compatible
(histocompatible) donor, usually a sibling, or
from a national cord blood bank or national
volunteer donor registry (Petrini, 2013).
• 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.

15. Graft-Versus-Host Disease
Graft-versus-host disease (GVHD) is a
potentially lethal immunologic response of
donor T cells to the tissue of the bone
marrow recipient (Alousi, Bolaños-Meade, &
Lee, 2013). The symptoms range from mild
to severe and generally include a rash and
general malaise beginning 7 to 14 days after
the transplant. Severe symptoms include
high fever, diarrhea, and liver and spleen
enlargement
SPLENECTOMY
One of the purposes of the spleen is to remove
damaged or aged blood cells. This poses
a problem with diseases such as sickle-cell anemia
and the thalassemias because the spleen
interprets the typical cells of these diseases as
damaged and destroys them. This causes children
with these disorders to have a continuous anemia,
with hemoglobin levels as low as 5 to 9 g/ml. In
some children, therefore, removal of the spleen
(splenectomy) will not cure the basic defect of the
blood cells but will limit the degree of anemia.
Splenectomy formerly required a large abdominal
incision, but today, it can be performed by
laparoscopy so, although still a procedure with
risks, it does not require as long a recovery period.

16. •Disorders of blood coagulation
–Idiopathic thrombocytopenia
purpura and Henoch–Schönlein
Syndrome, disseminated
vascular coagulation
Common Hematologic Disorders
• Anemia
–Can be caused by multiple
factors
–Blood loss, acute infection,
inadequate nutrition (iron, folic
acid, vitaminB12)
• Hemolytic anemias
–Sickle cell disease,
thalassemia, and other
autoimmune disorders

17. Disorders of the Red Blood Cells
NORMOCHROMIC, NORMOCYTIC 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.
• 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 underlyingcondition. When the
condition is reversed, blood values will
return to normal.

18. • 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.
Administration of recombinant human
erythropoietin can increase RBC
production and correct the anemia, but
not the renal disease.
• Anemia of Neoplastic Disease
Malignant growths such as leukemia or
lymphoma (common neoplasms of childhood)
result in normochromic, normocytic anemias
because the invasion of bone marrow by
proliferating neoplastic cells impairs RBC
production. There may be accompanying
blood loss if platelet formation also is
decreased. The treatment of such an anemia
involves measures designed to achieve
remission of the neoplastic process and
transfusion to increase the erythrocyte count.

19. • Hypersplenism
Under usual conditions, blood filters rapidly
through the spleen. If the spleen becomes
enlarged, however, blood cells pass through more
slowly, with more cells being
destroyed in the process. 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). Virtually any underlying splenic
condition can cause this syndrome.
Therapeutic management consists of treating the
underlying splenic disorder and
includes a possible 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.
• 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.
Exposure to insecticides and chemotherapeutic
drugs temporarily causes this.

20. Assessment
When symptoms begin, a child appears pale, fatigues easily,
and has anorexia from the
lowered RBC count and tissue hypoxia. Because of reduced
platelet formation (thrombocytopenia), the child bruises
easily or develops petechiae (pinpoint, macular,
purplish-red spots caused by an intradermal or
submucous hemorrhage). A child may have excessive
nosebleeds or gastrointestinal bleeding. As a result of a
decrease in WBCs (neutropenia), a child may contract an
increased number of infections and respond poorly to
antibiotic therapy. Observe closely for signs of cardiac
decompensation such as tachycardia, tachypnea, shortness
of breath, or cyanosis from the long-term increased
workload of all these effects on the heart. Bone marrow
samples will show a reduced number of blood elements,
and blood-forming spaces will be infiltrated by fatty tissue
Therapeutic Management
• The first step in therapy is to immediately
discontinue any drug or chemical suspected of
causing the bone marrow dysfunction and
removing the substance from the child’s
environment to avoid exposure. The ultimate
therapy for both congenital and acquired aplastic
anemia is hematopoietic stem cell transplantation
• For children who receive a hematopoietic stem cell
transplant, chances of complete recovery are
good. For others, the course will be uncertain. A
decreased WBC count leaves the child open to
infection. The decreased platelet count may
persist for years after other blood elements have
returned to normal, producing long-term
problems of bleeding, especially petechiae or
purpura.

21. • Hypoplastic Anemias
Hypoplastic anemias also 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
Children with the congenital form receive corticosteroid
therapy along with transfusions of packed RBCs to raise
erythrocyte levels. As a result of the necessary number
of transfusions, hemosiderosis (a deposition of iron in
body tissue) can occur. An iron chelation program such
as subcutaneous infusion of deferoxamine (Desferal)
may be started concurrently with transfusions to bind
with iron and aid its excretion from the body in urine.
Although an oral form is available for children over 10
years of age, this is usually given by a subcutaneous
infusion pump over an 8-hour period for 5 or 6 nights a
week. Remind the parent to assess that the child is
voiding as usual and that his or her specific gravity is
normal (1.003 to 1.030) before beginning an infusion so
iron removed from tissues can be excreted.

22. HYPOCHROMIC ANEMIAS
• Iron-Deficiency Anemia
Is the most common anemia of infancy and
childhood, occurring whenever the intake of dietary iron is inadequate. Without adequate iron, hemoglobin cannot be
incorporated into RBCs.
The Infants:
Infants born with structural defects of the gastrointestinal system, such as gastroesophageal reflux or chalasia (where an
immature valve exists 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 because iron
is not adequately digested. Infants with chronic diarrhea may not be able to make use of iron due to inadequate
absorption. If infants are fed cow’s milk rather than breast milk, so much minimal gastrointestinal bleeding may
occur that iron deficiency anemia may develop.
Older Children
In children older than 2 years of age, chronic blood loss is the most frequent cause of iron-deficiency anemia caused by
gastrointestinal tract lesions such as polyps, ulcerative colitis, Crohn disease, protein-induced enteropathies, parasitic
infestation, or frequent epistaxis. Adolescent girls with heavy menstrual periods can become iron deficient when this is
combined with frequent attempts to diet or with overconsumption of snack foods that are low in iron

24. Therapeutic Management
Therapy for iron-deficiency anemia
focuses on treatment of the underlying
cause: the lack of iron. An iron compound
such as ferrous sulfate for 4 to 6 weeks is
the drug of choice to improve RBC
formation and replace iron stores.In
addition, the diet of the child must be
changed to one rich in iron and vitamin
C, which enhances iron absorption.
• Chronic Infection Anemia
Acute infection interferes with RBC
production, producing a normochromic,
normocytic anemia. When infections are
chronic, however, anemia of a
hypochromic,microcytic type 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

25. • Anemia of Folic Acid Deficiency
A deficiency of folic acid combined with vitamin C
deficiency produces an anemia in which the erythrocytes
grow abnormally large. There is often accompanying
neutropenia and thrombocytopenia. Although the mean
corpuscular hemoglobin concentration will be normal,
the mean corpuscular volume and mean corpuscular
hemoglobin will both be increased. Bone marrow
contains megaloblasts, indicating inhibition of the
production of erythrocytes at an early stage.
Megaloblastic arrest, or inability of RBCs to mature past
this early stage, may occur in the first year of life from
the 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. Treatment is daily oral
administration of folic acid. With this treatment, the
response is dramatic.
• Pernicious Anemia (Vitamin B12
Deficiency)
Vitamin B12
is necessary for the maturation of RBCs. Pernicious
anemia results from a deficiency in vitamin B12
, either from inadequate intake or malabsorption
In children, the cause is more often a lack of ingestion of
vitamin B12 rather than poor absorption. Adolescents
may be deficient in vitamin B12 if they are ingesting a
long-term, poorly formulated vegetarian diet because the
vitamin is found primarily in foods of animal origin.
If a child is born with an intrinsic factor deficiency,
symptoms occur as early as the first 2 years of life. The
child appears pale, anorexic, and irritable,
with chronic diarrhea. The tongue 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.
MACROCYTIC (MEGALOBLASTIC) ANEMIAS

26. HEMOLYTIC ANEMIAS
• Congenital Spherocytosis
Congenital spherocytosis is a
hemolytic anemia that occurs most
frequently in the White Northern
European population and is
inherited as an autosomal
dominant trait. RBCs are small and
have a short life span apparently
due to abnormalities of the protein
of the cell membrane.
• 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 an
X-linked recessive trait inheritance and
is found most often in persons of
African,Mediterranean, or Asian
decent

27. • Sickle-Cell Anemia
Sickle-cell anemia is an autosomal recessive
inherited disorder carried on the β chain of
hemoglobin; the amino acid valine takes the place
of the normally appearing glutamic
acid. With this, the erythrocytes become
characteristically elongated and crescent
shaped (sickled) when they are 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 Crisis
Sickle-cell crisis is the term used to denote a
sudden, severe onset of sickling. There is
pooling of many new sickled cells in blood vessels
causing consequent tissue hypoxia
beyond the blockage (a vaso-occlusive crisis).
Such a crisis is most apt to occur when a
child has a gastrointestinal illness causing
dehydration, a respiratory infection that results in
lowered oxygen exchange and a lowered arterial
oxygen level, or after extremely strenuous
exercise (enough to lead to tissue hypoxia);
however, sometimes, no obvious cause of a crisis
can be found.

28. Other types of crisis that may occur include:
• A sequestration crisis occurs 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. Shock symptoms occur from
hypovolemia. The spleen is enlarged and tender.
• A hyperhemolytic crisis occurs when there is
increased destruction of RBCs.
• A megaloblastic crisis occurs 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 (temporary cessation of RBC
production) occurs when there is a sudden decrease
in RBC production. This form usually occurs with
infection. It creates a severe anemia.
Assessment for Sickle-Cell Anemia
- Diagnosed at birth because of required blood-spot
screening.
- At approximately 6 months of age, children begin to
show initial signs of fever and anemia.
- Stasis of blood and infarction may occur in a body part,
leading to local pain.
- Some infants have swelling of the hands and feet (a
hand–foot syndrome) probably caused by aseptic
infarction of the bones of the hands and feet.
- They may have a protruding abdomen because of an
enlarged spleen and liver.
- In adolescence, the spleen size may decrease in size from
repeated infarction and atrophy, leaving the teenager more
susceptible to infection than usual because the spleen can
no longer filter bacteria.
- Streptococcus pneumoniae and meningococcus are two
common bacterial agents that frequently cause illness; the
child with sickle-cell anemia needs prophylactic antibiotics
and age-appropriate vaccines to prevent these and other
infections

30. Therapeutic Management
The child in a sickle-cell crisis has three
primary needs: pain relief, adequate
hydration,and oxygenation to prevent
further sickling and halt the crisis.
THALASSEMIAS
The thalassemias are autosomal
recessive anemias associated with
abnormalities of the b chain of adult
hemoglobin (hemoglobin A).
Although these anemias occur most
frequently in the Mediterranean
population, they also occur in
children of African and Asian heritage

31. Thalassemia Minor (Heterozygous
β-Thalassemia)
Children with thalassemia minor, a mild form of this
anemia, produce a combination o fboth defective β
hemoglobin and normal hemoglobin. The condition
represents the heterozygous form of the disorder and
can be compared with children having the
sickle_x0002_cell trait. Because there is some normal
production, the RBC count is usually normal,but the
hemoglobin concentration will be decreased by 2 to 3
g/100 ml below usual levels. The blood cells are
moderately hypochromic and microcytic because of the
poor
hemoglobin formation.
Children may have no symptoms other than pallor. They
require no treatment, and life expectancy is normal.
They should not receive a routine iron supplement
because their inability to incorporate it well into
hemoglobin may cause them to accumulate too much
iron.
Thalassemia Major (Homozygous β-
Thalassemia)
Thalassemia major is also called Cooley anemia or
Mediterranean anemia. It is diagnosed at birth by a blood
spot test. Because this is a β-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 β hemoglobin, the child shows symptoms of
anemia, including pallor, irritability, and anorexia

33. Assessment
To maintain a functional level of hemoglobin, the bone
marrow hypertrophies in an attempt to produce more
RBCs, causing bone pain and the formation of target cells
or large macrocytes that are short lived and nonfunctional.
The hyperactivity of the bone marrow results in
characteristic changes in the shape of the skull (parietal
and frontal bossing) and protrusion of the upper teeth,
with marked malocclusion. The base of the nose may be
broad and flattened; the eyes may be slanted with an
epicanthal fold, as in Down syndrome. An X-ray of the
bone shows marked osteoporotic (of lessened density)
tissue, which can lead to fractures. The child may have
both an enlarged spleen and liver due to excessive iron
deposits and fibrotic scarring in the liver and the spleen
from increased attempts to destroy defective RBCs.
Abdominal pressure from the enlarged spleen may cause
anorexia and vomiting. Epistaxis is common, diabetes
mellitus may result from pancreatic hemosiderosis
(deposition of iron), and cardiac dilatation with
arrhythmias and heart failure may result in myocardial
fibrosis caused by invasion of iron.
Therapeutic Management
Stem cell transplantation is the ultimate cure for the disorder. While
waiting for a matched donor, digitalis, diuretics, and a low-sodium diet
may be prescribed to prevent heart failure. Transfusion of packed RBCs
every 2 to 4 weeks (hypertransfusion therapy) can be used to maintain
hemoglobin between 10 and 12 g/100 ml because with this level of
hemoglobin, erythropoiesis is suppressed and cosmetic facial alterations,
osteoporosis, and cardiac dilatation are minimized. Hypertransfusion
therapy also reduces the possibility that a splenectomy will be necessary.
Frequent blood transfusions, unfortunately, increase the risk of blood-
borne diseases, such as hepatitis B, and hemosiderosis, so children need
an oral iron-chelating agent to remove this excessive store of iron, such
as deferasirox or deferoxamine.
- A splenectomy may become necessary to reduce discomfort and also to
reduce the rate of RBC hemolysis and the number of transfusions needed.
Bone marrow stem cell transplantation can offer a cure. Even without
stem cell transplantation, the overall prognosis of thalassemia is
improving, although it is still grave. Most children with the
disease die of cardiac failure during adolescence or as late adolescents if
they do not receive a hematopoietic stem cell transplant.

34. Autoimmune Acquired Hemolytic
Anemia
Occasionally, autoimmune antibodies
(abnormal antibodies of the immunoglobulin
[Ig]G class) attach themselves to RBCs,
destroying them or causing hemolysis. This
can occur at any age, and its origin is
generally unknown, although the disorder is
associated with malignancy, viral infections,
or collagen diseases such as rheumatoid
arthritis or systemic lupus erythematosus. A
child may recently have had an upper
respiratory infection, measles, or varicella
virus infection (chickenpox). The disorder
may occur after the administration of drugs
such as quinine, phenacetin, sulfonamides,
or penicillin
Assessment
The onset of symptoms is insidious. Children usually develop a low-
grade fever, anorexia, lethargy, pallor, and icterus from release of
indirect bilirubin from the hemolyzed cells. Both urine and stools
appear dark as the excess bilirubin is excreted. In a few children, the
illness begins abruptly with high fever, hemoglobinuria, marked
jaundice, and enlarged liver and spleen. Laboratory findings reveal
RBCs have become extremely small and round
(spherocytosis). The reticulocyte count will be increased as the body
attempts to form replacement RBCs. A direct Coombs test result is
positive, indicating the presence of antibodies attached to red cells.
Hemoglobin levels may fall as low as 6 g/100 ml.
Therapeutic Management
In some children, the disease process runs a limited course and no
treatment is necessary. In others, a single blood transfusion may
correct the disturbance. It is difficult to cross-match blood for
transfusion for these children, however, because the
red cell antibody tends to clump or agglutinate all blood tested. If
cross-matching is impossible, the child may be given type O, Rh-
negative blood, which doesn’t need to match. Carefully observe the
child especially during any transfusion for signs of transfusion
reaction.

35. 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. Although
this may occur as a hereditary form, chronic
pulmonary disease and congenital heart
disease are the usual causes of polycythemia
in childhood. Also, it may occur from the
lower oxygen level maintained during
intrauterine life in newborns or with twin
transfusion at birth (one twin receives excess
blood, whereas a second twin is anemic).
Treatment of polycythemia involves treatment of the
underlying cause. Because of the high blood viscosity
from so many crowded blood cells, cerebrovascular
accident or emboli may occur. The risk increases
particularly if the child becomes dehydrated, such
as with fever or during surgery. A program of low-
dose aspirin to help prevent clotting or exchange
transfusion or phlebotomy to reduce the RBC count
may be necessary.
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.

36. Disorders of Blood Coagulation
Purpuras - 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.
• Idiopathic Thrombocytopenic Purpura
Idiopathic thrombocytopenic purpura (ITP)
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.

37. Therapeutic Management
Oral prednisone to reduce the immune response and intravenous immunoglobulin
(IVIG) or, in Rh-positive children, anti-D immunoglobulin to supply anti-ITP
antibodies are used to treat ITP. Platelet transfusion will temporarily increase the
platelet count, but because the life span of platelets is relatively short, a platelet
transfusion has only 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 salicylates or ibuprofen is prescribed for pain because 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. All children need to be vaccinated against the viral diseases of childhood
so that diseases such as rubella, rubeola, and varicella are eradicated and can no
longer lead to this defective coagulation process.

38. Henoch–Schönlein Syndrome
Henoch–Schönlein purpura (also called
anaphylactoid purpura) is caused by
increased vessel permeability, which leads to
bleeding in the small blood vessels. Although
no definite allergic correlation can be
identified, it is generally considered to be a
hypersensitivity reaction to an invading
allergen.
Assessment
The purpural rash occurs typically on the
buttocks, posterior thighs, and extensor
surface of the arms and legs.

39. Therapeutic Management
Treatment involves oral corticosteroid
therapy (prednisone) and mild analgesics
for a short period. Nose and throat
cultures rule out continuing bacterial
involvement. Urine should be assessed for
protein and glucose to detect kidney
involvement.

40. HEMOPHILIAS
• Hemophilia A (Factor VIII
Deficiency)
The classic form of hemophilia is caused by
deficiency of the coagulation component
factor VIII, the antihemophilic factor, and
transmitted as a sex-linked recessive trait. In
the United States, the incidence is
approximately 1 in 5,000 White males. A
female carrier may have slightly lowered
but sufficient levels of the factor VIII
component so that she does not manifest a
bleeding disorder. The baseline level of
factor determines the occurrence of
bleeding episodes and can range from mild
to severe
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

41. Assessment
Hemophilia often is recognized first in the
infant who bleeds excessively after
circumcision. If the disease has not shown
itself for several generations in a family, the
parents may be unaware of its existence. For
this reason, all infants need careful and
thoughtful observation after circumcision.
Because infants do not receive many injuries,
children’s bleeding tendencies may not
become apparent until they become active
(e.g.,crawling, climbing, or walking).
Therapeutic Management
With even minor abrasions, bleeding can be controlled
by the administration of factor VIII supplied by fresh
whole blood, fresh or frozen plasma, or a concentrate
of factor VIII. The concentrate is supplied as a
powdered form that can be stored at home and
reconstituted as needed. For most bleeding episodes,
one bag of concentrate per 5 kg of body weight is
usually sufficient to provide protection for
approximately 12 hours;
another transfusion may be necessary after that time.
In some children, the administration of desmopressin
(DDAVP), which stimulates the release of factor VIII,
may also help prevent bleeding.

42. • von Willebrand Disease
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. Bleeding time is
prolonged, with most hemorrhages
occurring from mucous membrane
sites.
• Christmas Disease
(Hemophilia B, Factor IX
Deficiency)
Christmas disease, first noted in 1952
in a patient by the name of Stephen
Christmas, is caused by factor IX
deficiency. It is transmitted as a sex-
linked recessive trait, and only
approximately 15% of people with
hemophilia have this form. Treatment
is with a concentrate of factor IX,
which is available for home
administration.

43. • Hemophilia C
(Factor XI
Deficiency)
Hemophilia C, or plasma
thromboplastin antecedent deficiency,
is caused by factor XI deficiency, is
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.
Bleeding episodes are treated with
administration of desmopressin
(DDAVP) or transfusion of fresh blood
or plasma.
• Disseminated
Intravascular Coagulation
Disseminated intravascular coagulation (DIC)
is an acquired disorder of blood clotting
that results from excessive trauma or some
similar underlying stimulus, such as an acute
infection or trauma

44. Quality & Safety Education for Nurses
(QSEN)
•Patient-Centered Care
• Teamwork & Collaboration
• Evidence-Based Practice
• Quality Improvement
• Safety
• Informatics

45. Thank you
for listening!
Dedace, Cheryll