Transcript Hematologic Alterations - NURSING FDTC Batch Spring 2011

Hematological
Alterations
NUR 264
Pediatrics
Angela Jackson, RN, MSN
Hematological Alterations:
Developmental Differences
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During fetal development, blood cells are
produced in the liver and the spleen
 In the newborn, blood cells are produced
from stem cells in the bone marrow
 Full- term newborns are able to store iron in
the bone marrow and liver tissue for up to 20
weeks
 Premature newborns may use up their iron
stores within 6-12 weeks
Iron Deficiency Anemia
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Most common
hematologic disorder of
infancy and childhood
The body does not have
enough iron to synthesize
the hemoglobin necessary
to carry oxygen to the
tissues
Affects 9% of toddlers 1-2
years of age
Affects 4% of children 3-4
years of age
Incidence rates are higher
in low income families
Iron Deficiency Anemia:
Pathophysiology
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Dietary iron is absorbed in the small intestine and is
either passed into the bloodstream or is stored as
ferritin
Iron in the blood stream binds to transferrin and is
delivered to the bone marrow, where it is combined
with other components to form hemoglobin
Iron may also be recycled from RBC’s that have been
removed from the bloodstream and catabolized
When a child does not ingest enough iron,
hemoglobin synthesis is impaired
When a child experiences blood loss, there are fewer
RBC’s to be catabolized and the iron in these cells
cannot be recycled
Iron Deficiency Anemia:
Clinical Manifestations
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Mild anemia:
– Generally asymptomatic
– May experience symptoms of moderate anemia during exertion
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Moderate anemia:
– Shortness of breath
– Rapid, pounding heart beat
– Dizziness, fainting, lethargy, irritability
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Severe anemia:
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Cardiac murmurs
Pale skin, mucous membranes, lips, nail beds and conjunctiva
Thinning and early graying of the hair
Abdominal pain, nausea, vomiting, anorexia, low-grade fever
Decreased physical growth, developmental delay
Iron Deficiency Anemia:
Diagnosis
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History and physical exam
 Laboratory studies:
– Decreased hemoglobin, hematocrit, MCV(RBC
volume), MCH(weight of Hb within a RBC),
MCHC(concentration of Hb in a RBC), serum iron,
serum ferritin
– Microcytic, hypochromic RBC’s
– Increased Total iron binding capacity
– Normal retic count
– Normal hemoglobin electrophoresis
Iron Deficiency Anemia:
Treatment
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Infants: Breast milk or iron-fortified formula
until 4-6months, then add iron-fortified cereal
 School-age children and adolescents: restrict
amount of milk, encourage iron-rich foods
and foods high in vitamin C
 Iron supplements:
– Infants and preschoolers: 3mg/kg/day
– School-age children: 60mg/day
– Adolescents: 120mg/day
Iron-Deficiency Anemia:
Nursing Considerations
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Family Teaching
– Nutrition: foods high and iron, and foods
high in Vitamin C
– Medication dosages and administration
– Side effects of medication
– Follow-up
Sickle Cell Anemia
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Autosomal recessive
disorder
Occurs in approximately
1 in every 500 AfricanAmerican births and 1
in every 1000-1400
Hispanic American
births
Sickle cell trait is
present in 1 in 12
African-Americans
Sickle Cell Anemia:
Pathophysiology
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One amino acid replaces
another, resulting in the
production of sickle hemoglobin
(Hb S)
 This form of hemoglobin
contains a semi-solid gel that
caused the RBC to stretch into
a sickle shape
 These cells are more stiff and
less able to change shape, and
are unable to pass through the
microcirculation
 Anemia results from increased
RBC destruction, worsened by
the fact that sickled cells die
after only 10-20 days
Sickle Cell Anemia:
Clinical Manifestations
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Vaso-occlusive crisis: aggregation of sickled cells within a
vessel, causing obstruction.
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Pain crisis
Hand-foot syndrome
Acute chest syndrome
Stroke
– Priapism
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Sequestration crisis: excessive pooling of blood in the liver
and spleen. Decreased blood volume may result in shock.
Aplastic crisis: decrease in red blood cell production.
Results in severe anemia
Pallor, fatigue, shortness of breath
Delayed growth
Delayed onset of puberty
Sickle Cell Anemia:
Diagnosis
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Family history and
clinical manifestations
Newborn Screening
Lab tests:
– Increased retic count
– Decreased Hgb &Hct and
TIBC
– Hemoglobin electrophoresis
reveals predominantly Hb S
– Normocytic, normochromic,
sickle shaped cells
– Normal MCV, MCH, MCHC
– Normal serum iron and
serum ferritin
Sickle Cell Anemia:
Treatment
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Primary treatment is prevention of RBC
sickling
– Avoidance of fever, infection, acidosis,
dehydration, constrictive clothing and
exposure to cold
– Immunization
– Prophylactic oral penicillin until age 5
– Routine blood transfusions for children at
high risk of CVA
Sickle Cell Anemia:
Nursing Considerations
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Promote comfort
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Administer pain medication routinely instead of PRN
Apply heat to painful areas
Allow child to determine amount of activity tolerated
Provide passive ROM exercises
Administer IV fluids as ordered to maintain hydration
Prevent infection
– Administer antibiotics as ordered
– Frequent hand washing
– Proper aseptic techniques
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Provide education
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Maintain adequate hydration
Avoid sources of infection
Promote proper nutrition
Signs and symptoms of crisis
Administration of prophylactic medications
Hemophilia
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Group of bleeding disorders in which one factor in the
first phase of coagulation is deficient
 Most common type is hemophilia A (deficiency of
factor VIII), occurs in approximately 1:5000 males
 Hemophilia B (Christmas disease, deficiency of factor
IX) second most common type, affects 10-15% of
people with hemophilia
 Least common type is hemophilia C (deficiency of
factor XI)
 Hemophilia A and B are x-linked recessive disorders,
affecting mostly males. Hemophilia C is an
autosomal recessive disorder that affects males and
females equally
Hemophilia:
Pathophysiology
One factor of the first phase of the
intrinsic pathway is deficient
 The body is unable to form clots to
repair damaged blood vessels
 Bleeding episodes occur
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Hemophilia:
Clinical Manifestations
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Clinical manifestations vary based on severity
of the disease
– Mild: excessive bleeding only after severe trauma
or surgery
– Moderate: excessive bleeding only after trauma
– Severe: excessive bleeding occurs spontaneously
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Hematomas may result from injections or firm
holding during the first year of life
Hemophilia:
Clinical Manifestations
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As the child learns to walk,
bruising occurs easily, and
hemarthrosis may develop
Persistent bleeding from
minor lacerations begin to
occur by 3-4 years of age
Minor occurrences of
hematuria, epistaxis as well
as major events such as
intracranial hemorrhage and
bleeding into the neck or
abdomen may also occur
Hemophilia:
Diagnosis
History and physical exam
 Lab tests:
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– Prolonged PTT
– Normal platelet count, standard bleeding
time, and PT
Hemophilia:
Treatment
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Hemophilia A:
– Replacement of missing coagulation factor
through infusion of recombinant factor VIII
concentrates
• May be administered three or four times/week
– Desmopressin (DDAVP) is effective for
spontaneous bleeding and in preventing
bleed prior to dental or surgical procedures
Hemophilia:
Treatment
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Hemophilia B:
– Replacement of factor IX through plasma
derived concentrates
– DDAVP is not effective
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Hemophilia C: Aggressive therapy is
usually unnecessary because of the
mild nature of the disease
Hemophilia:
Nursing Considerations
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Prevent bleeding:
– Make the environment as safe as possible
for infants learning to walk
– Encourage noncontact sports in the school
age child
– Soft-bristled toothbrushes for mouth care
and electric razors for shaving
– Prevent IM injections whenever possible
– Wear medic alert bracelet
Hemophilia:
Nursing Considerations
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Recognize and manage bleeding:
– Make family aware of signs and symptoms of
internal bleeding such as headache, slurred
speech, loss of consciousness, black tarry stools,
hematemesis, hematuria
– Factor replacement should be instituted according
to established medical protocol
– Apply pressure to the bleeding area for at least
10-15 minutes
– Immobilize and elevate the area above the level of
the heart to decrease blood flow
– Apply cold to promote vasoconstriction
Hemophilia:
Nursing Considerations
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Prevent crippling effects of joint degeneration
resulting from hemarthrosis:
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Elevate and immobilize the joint
Passive range of motion after the acute phase
Physical therapy
Nutrition counseling to maintain optimal weight
May need orthopedic intervention such as casting,
traction or aspiration of blood to preserve joint
function
Hemophilia:
Nursing considerations
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Family teaching:
– Administration of antihemophilic factor
concentrates
– Methods to prevent or stop bleeding
– Signs and symptoms that indicate an
emergent situation and the need for
immediate physician intervention
– Provide support
– Genetic counseling
Immune Thrombocytopenic
Purpura (ITP)
Acquired disease characterized by
thrombocytopenia and purpura
 Autoimmune disorder
 Peak incidence between 2 and 4
 75% of children recover completely in 3
months
 80-90% have regained normal platelet
counts within 6 months
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ITP:
Pathophysiology
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Platelets adhere to injured vessel walls, release
biochemical mediators, and form plugs, blocking
minute ruptures occurring in the microcirculation
Inadequate numbers of platelets result in purpura
under the skin and throughout the tissues
This occurs as the result of an autoimmune
process that is triggered by a viral infection
Antiplatelet antibodies bind to the platelets,
sequestering and destroying them in the spleen
ITP:
Clinical Manifestations
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Ecchymoses, general petechial rash
occurring 1-4 weeks following a viral infection
Asymmetrical bleeding, especially on legs
and trunk
Gastrointestinal or urinary tract bleeding may
occur
Nose bleeding may be present and difficult to
control
Intracranial hemorrhage occurs in only 1% of
patients
ITP:
Diagnosis
History and clinical presentation
 Peripheral blood smear with very few,
large, immature platelets
 Bone marrow aspiration will be normal
 Lab tests:
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– Decreased platelet count
– Prolonged standard bleeding time
– Normal PT and PTT
ITP:
Treatment
Self-limiting condition
 Prevent injury
 Control bleeding
 Immunosuppressive medication if sever
symptoms are present
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– Prednisone
– Gamma globulin
Questions?