Transcript Alterations in Hematologic Function

Hematology
Jan Bazner-Chandler
CPNP, CNS, MSN, RN
Blood
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Blood is the fluid of life
Blood is composed of:
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Plasma
RBC
WBC
Platelets
Plasma
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Plasma consists of:
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90% water.
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10 % solutes: albumin, electrolytes and proteins.
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Proteins consist of clotting factors, globulins, circulating
antibodies and fibrinogen.
Red Blood Cells
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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
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Average life cycle is 120
days.
The bones are continually
producing new cells.
White Blood Cells
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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:
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Produce protective antibodies.
Surround it and devour the bacteria.
WBC’s
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WBC life span is from a few days to a few weeks.
WBC’s will increase when fighting infection.
Neutropenia – abnormal percentage of neutrophils
compared to total white blood cells; decreases a child’s
ability to fight pathologic bacteria
Platelets
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Platelets are irregularlyshaped, colorless bodies
that are present in blood.
Their sticky surface lets
them form clots to stop
bleeding.
Blood Values
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CBC with differential and platelet count.
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Hgb:
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Normal levels are 11 to 16 g / dl
Panic levels are:
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Less than 5 g / dl
More than 20 g / dl
Hematocrit
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Normal hematocrit levels are 35 to 44%.
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Panic levels:
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Hmct less than 15 %
Hmct greater than 60%
Blood Tests
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Hemoglobin and hematocrit used 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.
Coagulation Profile
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Partial thromboplastin time
Prothrombin time
Platelet count
Fibrinogen
Platelet function analysis – clotting analysis
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.
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Red blood cells – carry oxygen to tissue
Platelets – help blood to clot
White blood cells – fight infection
Bone Marrow Aspiration
Bone Marrow Transplant
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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.
Treatment Modalities
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Transfusion:
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Packed red blood cells – anemia
Platelets – platelet dysfunction
Fresh frozen plasma – coagulation factors
Blood Transfusions
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3 types of transfusion reactions
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Hemolytic
Allergic
Febrile
Hemolytic Reaction
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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
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Flank pain
Fever
Chills
Bloody urine
Rash
Low blood pressure
Dizziness / fainting
Nursing Management
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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
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Often occurs after multiple blood transfusions.
Symptoms:fever, chills, and diaphoresis.
Interventions:
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Slow transfusion and administer antipyretic.
Administer antipyretic prior to administration.
Allergic Reaction
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Symptoms: rash, urticaria,
respiratory distress, or
anaphylaxis.
Interventions:
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administer antihistamine
before transfusion
Physician may order washed
rbc’s
Hematologic Conditions
Alteration in Hematologic Status
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Disorders of hemostasis or clotting factors
Structural or quantitative abnormalities in the
hemoglobin.
Anemias
Aplastic Anemia
Genetic Implications
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The following have a genetic link: implications for genetic
screening and fetal diagnosis
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Sickle cell anemia
Thalassemia
Hemophilia
Bleeding Disorders
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Three types Hemophilia: males only
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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
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Hemophilia type A is the deficiency of clotting factor VIII.
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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
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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
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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
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Goals of care:
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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.
Assessment
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Circumcision may produce prolonged bleeding.
As child matures and becomes more active the incidence
of bleeding due to trauma increases
May be mild, moderate or severe
Bleeding into joint spaces, hemarthrosis
Most dangerous bleed would be intracranial
Diagnosis
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Presenting symptoms
Prolonged activated aPTT and decreased levels of factor
VIII or IX.
Genetic testing to identify carriers
Pharmacologic Interventions
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Products used to treat hemophilia are:
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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
Fibrin glue – mixture of fibrogen and thrombin can be applied
topically on a wound to stop minor bleeding
Multidisciplinary Interventions
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Replace the factor as ordered by physician.
Manage pain utilizing analgesics as ordered – no salicylate
products.
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.
Family Education
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Medic-Alert bracelet
Injury prevention appropriate for age
Signs and symptoms of internal bleeding or hemarthrosis
Dental checkups
Medication administration
Long Term Complications
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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
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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
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The most obvious clinical feature of DIC is bleeding.
Renal involvement = hematuria
Pulmonary involvement = hemoptysis, tachypnea, dyspnea
and chest pain.
Cutaneous involvement = petechiae, ecchymosis, jaundice,
acrocyanosis and gangrene.
Multidisciplinary Interventions
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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.
Assessment
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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
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Depends on the underlying disorder and the severity of
the DIC.
ITP
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Idiopathic thrombocytopenic purpura
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Idiopathic = cause is unknown
Thrombocytopenic = blood does not have enough platelets
Purpura = excessive bleeding / bruising
Immune Thrombocytopenic Purpura
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Antibodies destroy platelets
Antibodies see platelets as bacteria and work to eliminate
them
ITP is preceded by a viral illness
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URI
Varicella
Smallpox / measles vaccine
Mononucleosis
Flu
Two Types
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Acute: occurs in children between 1 and 6 years
Chronic (continuous or recurrent): older than 10 years
and female
Symptoms
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Random purpura
Epistaxis, hematuria, hematemesis, and menorrhagia
Petechiae and hemorrhagic bullae in mouth
Diagnostic Tests
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Low platelet count
Peripheral blood smear
Antiplatelet antibodies
Normal platelet count: 150,000 to 400,000
Pharmacologic Interventions
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For severe cases (platelet count < 20,000)
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IV gamma globulin for 2-5 days to block antibody production,
reduce autoimmune problem and increase platelet count
Corticosteroids to enhance vascular stability, increase platelet
survival
IV anti-D to stimulate platelet production
Sickle Cell Disease
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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
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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
Body Systems Affected by SS
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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
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Presents depending on location of vaso-occlusion
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Bone pain – back, knees, shoulders, elbows
Acute chest syndrome – Chest pain / pneumonia
Acute abdominal pain
Cerebrovascular accident – stoke symptoms
Priapism – nonsexual erection of penis
Splenic Sequestration
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Sudden and quickly progressing splenic enlargement
Left upper quadrant pain and vomiting
Abdominal distension, pallor, dyspnea, tachycardia
Hypovolemia and shock
Decrease hemoglobin / hematocrit and increased
reticulocyte count
Aplastic Crisis
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Diminished production and increased destruction of red
blood cells
Triggered by viral infection or depletion of folic acid
Signs include profound anemia, pallor
Diagnostic Screening
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Newborn screening is mandatory in the United States.
Screening done between 24 and 72 hours of age.
Verification of results are made by the healthcare
provider at first office visit.
Newborn Screening – Follow-up
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Contact family with screening results
Refer to specialists if positive
Evaluate infant for slenomegaly
Initiate daily penicillin VK (125 mg po bid) prophylaxis as
recommended
Educate regarding risk of sepsis, or signs of splenic
sequestration
Nursing Management - Hospital
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Increase tissue perfusion
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Oxygen / bedrest
Blood transfusion / antibiotics if ordered
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Pain management – meperidine (Demerol)
contraindicated
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Hydration
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IV fluids as ordered
Oral intake of fluids
Nursing Management - Home Care
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Adequate nutrition
Emotional Support
Discharge instructions
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Information about disease management
Daily folic acid
Control of triggers
Prophylactic antibiotics
Immunizations / Pneumococcal
Patient Education
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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
Thalassemia
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A group of inherited diseases of the blood that affect the
ability to produce hemoglobin.
Occurs most frequently in people of Italian, Greek, Middle
Eastern, Southern Asian and African Ancestry.
Two Types of Thalassemia
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Alpha or beta depending on which part of an oxygencarrying protein the red blood cell is lacking.
Child may have mild to severe form of the disease.
Cooley’s anemia is severe form of the disease.
Tests for Thalassemia
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Blood tests and family genetics study to show whether an
individual has Thalassemia or is a carrier.
Prenatal testing at 11th week of pregnancy using chorionic
villi sampling.
Amniocentesis around the 16th week of pregnancy
Assisted reproductive therapy
Clinical Manifestations
The child may be:
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pale and listless
have poor appetite
enlarged spleen, liver and heart
bones become thin and brittle
Heart failure and infection are the major cause of death.
Interdisciplinary Interventions
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Frequent blood transfusions to keep hemoglobin levels
near normal
“iron chelators” to rid body of excess iron from
numerous blood transfusions
Bone marrow transplant: only possible for a small
minority of patients who have a suitable bone marrow
donor
Iron Deficiency Anemia
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Most common nutritional deficiency
Depletion of iron stores due to
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Inadequate dietary intake
Impaired iron absorption
American Academy of Pediatrics
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Recommends screening for iron deficiency at 1 year of
age
Screening of high risk children (low socio-economic
status or poor dietary intake)
What a problem?
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IDA can have long lasting effects on motor development,
mental development and auditory and visual function.
IDA
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Occurs in children experiencing:
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Rapid physical growth
Low iron intake
Inadequate iron absorption
Loss of blood
Assessment
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Associated with low oxygenation of tissue:
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Pallor
Fatigue
Shortness or breath
Irritability
Intolerance of physical work / exercise
Diagnostic Tests
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Initial screen is hemoglobin
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Hemoglobin levels less than 8 g/dL
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Decreased levels of Serum Iron or Total Iron Binding or
Serum Ferritin
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Microcytic and hypochromic red blood cells
Pharmacologic Treatment
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Iron supplementation
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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 dark-colored stools
Management
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Infants younger than 12 months should be on formula
with iron
Solid foods – introduced about 6 months of age
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Infants 12 months or older
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Rice cereal fortified with iron
Pureed Vegetables, meats
Decrease intake of milk
Increase solid foods
Children: iron fortified cereals, meat, green leafy
vegetables, yogurt, cheeses, low-fat or non-fat milk
Teenagers: reduce junk food
Aplastic Anemia
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Acquired or inherited
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Normal production of blood cells in the bone marrow is
absent or decreased.
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A marked decrease in RBC’s, WBC’s and platelets.
Causes
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Exposure to drugs
Exposure to chemicals
Exposure to toxins
Infection
Idiopathic in nature
Inherited: Fanconi’s anemia
Blood Characteristics
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Neutophil less than 500
Platelet less than 20,000
Hemoglobin less than 7
Reticulocytes 1%
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Nursing Diagnosis?
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Bone marrow reveals hypo-cellular and fatty marrow.
Management
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Immunosuppressive therapy
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Antithymocyte globulin
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Administered IV over 4 days
Response seen within 3 months
Bone Marrow Transplant
Neonatal Hyperbilirubinemia
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Hyperbilirubinemia is defined as excessive levels of
bilirubin in the blood.
Alert: Jaundice that occurs within the first 24 hours of
life or after 2 weeks of life signifies an abnormal
physiologic process.
Kernicterus: bilirubin encephalopathy
Jaundice
Yellowing of the skin – occurs when bilirubin is deposited
into the subcutaneous tissue and it becomes visible when
the serum bilirubin levels exceed 7.0 mg/dL.
Normal Physiologic Jaundice
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Occurs in 45% to 65% of all healthy newborns in the first
3 to 5 days of life
Occurs more frequently in infants less than 38 weeks
gestation
Breast fed infants have a higher incidence of jaundice (due
to dehydration and caloric deprivation)
Assessment
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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
Multidisciplinary Interventions
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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