Transcript Alterations in Hematologic Function

Hematology
Jan Bazner-Chandler
CPNP, CNS, MSN, RN
Blood


Blood is the fluid of life
Blood is composed of:




Plasma
RBC
WBC
Platelets
Plasma

Plasma consists of:

90% water.

10 % solutes: albumin, electrolytes and proteins.

Proteins consist of clotting factors, globulins,
circulating antibodies and fibrinogen.
Red Blood Cells



RBC’s travel through the body delivering
oxygen and removing waste.
RBC’s are red because they contain a protein
chemical called hemoglobin which is bright
red in color.
Hemoglobin contains iron, making it an
excellent vehicle for transporting oxygen and
carbon dioxide.
RBC’s


Average life cycle is
120 days.
The bones are
continually producing
new cells.
White Blood Cells



The battling blood cells.
The white blood cells are continually on the
look out for signs of disease.
When a germ appears the WBC will:


Produce protective antibodies.
Surround it and devour the bacteria.
WBC’s


WBC life span is from a few days to a few
weeks.
WBC’s will increase when fighting infection.
Platelets


Platelets are irregularlyshaped, colorless
bodies that are present
in blood.
Their sticky surface lets
them form clots to stop
bleeding.
Blood Values

CBC with differential and platelet count.

Hgb:


Normal levels are 11 to 16 g / dl
Panic levels are:


Less than 5 g / dl
More than 20 g / dl
Hematocrit

Normal hematocrit levels are 35 to 44%.

Panic levels:


Hmct less than 15 %
Hmct greater than 60%
Hemoglobin and Hematocrit



Can be used as a simple blood test to screen
for anemia.
The CBC with differential would be used to
help diagnose a specific disorder.
A bone marrow aspiration would be the most
conclusive in determining cause of anemia –
aplastic / leukemia.
Bone Marrow
Bone marrow is the spongy substance found in
the center of the bones.
• It manufactures bone marrow stem cells,
which in turn produce blood cells.
•
•
•
Red blood cells – carry oxygen to tissue
Platelets – help blood to clot
White blood cells – fight infection
Bone Marrow Transplant



Donor is placed under anesthesia.
Marrow is aspirated out of the iliac crest.
Marrow is filtered and treated to remove bits
of bone and other unwanted cells and debris,
transferred to a blood bag, and is infused into
the patient’s blood just like at transfusion.
Bone Marrow Aspiration
Treatment Modalities

Transfusion:



Packed red blood cells – anemia
Platelets – platelet dysfunction
Fresh frozen plasma – coagulation factors
Blood Transfusions

3 types of transfusion reactions



Hemolytic
Allergic
Febrile
Hemolytic Reaction


Refers to an immune response against
transfused blood cells.
Antigens, on the surface of red blood cells,
are recognized as “foreign proteins” and can
stimulate B lymphocytes to produce
antibodies to the red blood cell antigens.
Hemolytic reaction







Flank pain
Fever
Chills
Bloody urine
Rash
Low blood pressure
Dizziness / fainting
Nursing Management







Stop the blood transfusion.
Start normal saline infusion.
Take vital signs with blood pressure
Call the MD
Obtain blood sample and urine specimen.
Return blood to blood bank.
Document
Febrile Reaction



Often occurs after multiple blood
transfusions.
Symptoms:fever, chills, and diaphoresis.
Interventions:


Slow transfusion and administer antipyretic.
Administer antipyretic prior to administration.
Allergic Reaction


Symptoms: rash,
urticaria, respiratory
distress, or
anaphylaxis.
Interventions:


administer antihistamine
before transfusion
Physician may order
washed rbc’s
Hematologic Conditions
Alteration in Hematologic Status




Disorders of hemostasis or clotting factors
Structural or quantitative abnormalities in the
hemoglobin.
Anemias
Aplastic Anemia
Genetic Implications

The following have a genetic link: implications
for genetic screening and fetal diagnosis



Sickle cell anemia
Thalassemia
Hemophilia
Bleeding Disorders

Three types Hemophilia: males only



Type A most common – factor VIII deficiency
Type B - lack of factor IX (Christmas Disease)
Type C – lack of factor XI
Von Willebrand Disease – 1% of population – men
or women – prolonged bleeding time
Hemophilia Type A

Hemophilia type A is the deficiency of clotting
factor VIII.




A serious blood disorder
Affects 1 in 10,000 males in the US
Autoimmune disorder with lowered level of clotting
factor
All races and socio economic groups affected
equally
Hemophilia




Hemophilia is a sex-linked hereditary
bleeding disorder
Transmitted on the X chromosome
Female is the carrier
Women do not suffer from the disease itself
Historical Perspective


First recorded case in Talmud Jewish text by
an Arab physician – documentation of two
brothers with bleeding after circumcision.
Queen Victoria is carrier and spread the
disease through the male English royalty.
Goals of Care

Goals of care:



Provide factor VIII (IX) to aid blood in clotting.
To decrease transmission of infectious agents in
blood products; hepatitis & AIDS.
Future: gene therapy to increase production of
clotting factor.
Symptoms


Circumcision may produce prolonged
bleeding.
As child matures and becomes more active
the incidence of bleeding due to trauma
increases
Symptoms



May be mild, moderate or severe
Bleeding into joint spaces, hemarthrosis
Most dangerous bleed would be intracranial.
Diagnosis



Presenting symptoms
Prolonged activated aPTT and decreased
levels of factor VIII or IX.
Genetic testing to identify carriers
Treatment

Products used to treat hemophilia are:


Fresh frozen plasma and cryoprecipitate which
are from single blood donors and require special
freezing.
Second generation of factor VIII are made with
animal or human proteins.
Nursing Diagnoses




Risk for injury
Pain with bleed especially into a joint
Impaired physical mobility
Knowledge deficit regarding disease and
management of disease
Nursing interventions






No rectal temps.
Replace the factor as ordered by physician.
Manage pain utilizing analgesics as ordered.
Maintaining joint integrity during acute phase:
immobilization, elevation, ice.
Physical therapy to prevent flexion contraction and
to strengthen muscles and joints.
Provide opportunities for normal growth and
development.
Teaching



Avoid aspirin which prolongs bleeding time in people
with normal levels of factor VIII.
A fresh bleeding episode can start if the clot
becomes dislodged.
Natural reactions in the body cause the clot that is
no longer needed to “break down. This process
occurs 5 days after the initial clot is formed.
Family Education





Medic-Alert bracelet
Injury prevention appropriate for age
Signs and symptoms of internal bleeding or
hemarthrosis
Dental checkups
Medication administration
Long Term Complications


20% develop neutralizing antibodies that
make replacement products less effective.
Gene therapy providing continuous
production of the deficient clotting factor
could be the next major advance in
hemophilia treatment.
Disseminated Intravascular
Coagulation or DIC


DIC is an acquired coagulopathy that is
characterized by both thrombosis and
hemorrhage.
DIC is not a primary disorder but occurs as a
result of a variety of alterations in health.
Assessment




The most obvious clinical feature of DIC is bleeding.
Renal involvement = hematuria, oliguria, and anuria.
Pulmonary involvement = hemoptysis, tachypnea,
dyspnea and chest pain.
Cutaneous involvement = petechiae, ecchymosis,
jaundice, acrocyanosis and gangrene.
Management of DIC




Treatment of the precipitating disorder.
Supportive care with administration of platelet
concentration and fresh frozen plasma and
coagulation factors.
Administration of heparin (controversial in
children).
Heparin potentates anti-thrombin III which
inhibits thrombin and further development of
thrombosis.
Nursing Diagnoses



Altered tissue perfusion
Risk for injury
Anxiety
Nursing Interventions





Rigorous ongoing assessment of all body
systems
Monitor bleeding
No rectal temps
Avoid trauma to delicate tissue areas
All injections sites and IV sites need to be
treated like an arterial stick.
Prognosis

Depends on the underlying disorder and the
severity of the DIC.
ITP

Idiopathic thrombocytopenic purpura



Idiopathic = cause is unknown
Thrombocytopenic = blood does not have enough
platelets
Purpura = excessive bleeding / bruising
Immune Thrombocytopenic Purpura



Antibodies destroy platelets
Antibodies see platelets as bacteria and work
to eliminate them
ITP is preceded by a viral illness




URI
Varicella / measles vaccine
Mononucleosis
Flu
Symptoms



Random purpura
Epistaxis, hematuria, hematemesis, and
menorrhagia
Petechiae and hemorrhagic bullae in mouth
Diagnostic Tests



Low platelet count
Peripheral blood smear
Antiplatelet antibodies
Normal platelet count: 150,000 to 400,000
Management



IV gamma globulin to block antibody
production, reduce autoimmune problem
Corticosteroids to reduce inflammatory
process
IV anti-D to stimulate platelet production
Sickle Cell Anemia





Autosomal recessive disorder
Defect in hemoglobin molecule
Cells become sickle shaped and rigid
Lose ability to adapt shape to surroundings.
Sickling may be triggered by fever and
emotional or physical stress
Pathophysiology

When exposed to diminished levels of
oxygen, the hemoglobin in the RBC develops
a sickle or crescent shape; the cells are rigid
and obstruct capillary blood flow, leading to
congestion and tissue hypoxia; clinically, this
hypoxia causes additional sickling and
extensive infarctions.
Whaley & Wong Text
Crescent Shaped Cells
Body Systems Affected by SS






Brain: CVA – paralysis - death
Eyes: retinopathy – blindness
Lungs: pneumonia
Abdomen: pain, hepatomegaly, splenomegaly
(medical emergency due to possible rupture
Skeletal: joint pain, bone pain – osteomyelitis
Skin: chronic ulcers – poor wound healing
Vaso-occlusive Crisis
Stasis of blood with clumping of cell in the
microcirculation, ischemia, and infarction
 Most common type of crisis; painful
 Signs include fever, pain, tissue engorgement
Splenic Sequestration




Life-threatening / death within hours
Pooling of blood in the spleen
Signs include profound anemia, hypovolemia,
and shock
Abdominal distention, pallor, dyspnea,
tachycardia, and hypotension
Aplastic Crisis



Diminished production and increased
destruction of red blood cells
Triggered by viral infection or depletion of
folic acid
Signs include profound anemia, pallor
Nursing Diagnoses




Altered tissue perfusion
Pain
Risk for infection
Knowledge deficit regarding disease process
Nursing Management - Hospital

Increase tissue perfusion





Oxygen
Blood transfusion if ordered
Bed rest
Pain management
Hydration


IV fluids as ordered
Oral intake of fluids
Nursing Management
•
•
•
Adequate nutrition
Emotional Support
Discharge instructions
•
•
•
•
•
Information about disease management
Daily folic acid
Control of triggers
Prophylactic antibiotics
Immunizations / Pneumococcal
Patient Education





Necessity of following plan of care
Signs and symptoms of impending crisis.
Signs and symptoms of infection
Preventing hypoxia from physical and
emotional stress
Proving adequate rest
Beta-Thalassemia





Hereditary / autosomal defect
Genetic defect on chromosome 11
Mediterranean descent
Defect in the beta globin gene
Beta globin chains are required for synthesis
of hemoglobin A
RBC Characteristics

Microcytosis = small in
size

Hypochromia = decrease
hemoglobin

Poikilocytosis = abnormal
shape
Treatment / Prognosis



Supportive
Blood transfusions as needed
Bone marrow transplant
Poor prognosis / death within 1st year due to septicemia or
heart failure.
Iron Deficiency Anemia


Most common nutritional deficiency
Depletion of iron stores
Abnormal Laboratory Values

Hemoglobin levels less than 8 g/dL

Decreased levels of Serum Iron or Total Iron
Binding or Serum Ferritin

Microcytic and hypochromic red blood cells
IDA

Occurs in children experiencing:




Rapid physical growth
Low iron intake
Inadequate iron absorption
Loss of blood
Symptoms

Associated with low oxygenation of tissue:





Pallor
Fatigue
Shortness or breath
Irritability
Intolerance of physical work / exercise
Management

Iron supplementation



Given in a.m. on an empty stomach
To avoid staining of teeth, give using a syringe,
dropper or straw
Instruct caretaker that child may have darkcolored stools
Management



Nutritional counseling
Infants younger than 12 months should be on
formula until around 12 months of age
Infants 12 months or older




Decrease intake of milk
Introduce solid foods
Children: iron fortified cereals, foods, meat,
green leafy vegetables
Teenagers: reduce junk food
Aplastic Anemia

Acquired or inherited

Normal production of blood cells in the bone
marrow is absent or decreased.

A marked decrease in RBC’s, WBC’s and
platelets.
Causes





Exposure to drugs
Exposure to chemicals
Exposure to toxins
Infection
Idiopathic in nature
Blood Characteristics

Neutophil less than
500
Platelet less than
20,000
Hemoglobin less than
7
Reticulocytes 1%

Nursing Diagnosis?



Bone marrow reveals hypo-cellular and fatty marrow.
Management

Immunosuppressive therapy

Antithymocyte globulin



Administered IV over 4 days
Response seen within 3 months
Bone Marrow Transplant
Hyper-bilirubinemia
Hyperbilirubinemia

Many babies have some jaundice. When they are a few
days old, their skin slowly begins to turn yellow. The
yellow color comes from the color of bilirubin. When red
blood cells die, they break down and bilirubin is left. The
red blood cells break down and make bilirubin. In
newborns, the liver may not be developed enough to get
rid of so much bilirubin at once. So, if too many red blood
cells die at the same time, the baby can become very
yellow or may even look orange. The yellow color does
not hurt the baby's skin, but the bilirubin goes to the
brain as well as to the skin. That can lead to brain
damage.
Signs and symptoms





Very yellow or orange skin tones (beginning at
the head and spreading to the toes)
Increased sleepiness, so much that it is hard to
wake the baby
High-pitched cry
Poor sucking or nursing
Weakness, limpness, or floppiness
Photo Therapy
Fiberoptic Blanket
Nursing Interventions







Monitor bilirubin levels
Assess activity level – muscle tone – infant
reflexes
Encourage po intake: May need to supplement
with formula if inadequate breastfeeding
Weight daily to assess hydration status
Monitor stools – amount and number
Cover eyes while under bili-lights
Facilitate parent - infant bonding
Loss of moro or startle reflex can indicate possible brain damage due to
Kernicterus