alteration in hematologic function - salem state...
TRANSCRIPT
9/30/2007
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Alteration in Hematologic
function
Lorraine Murphy RN PNP
October 2007
FIGURE 28–1 Types of blood cells.
Red Blood Cells
• Most abundant cellular element of blood�4.6-5.9 million/mL
• Erythropoiesis- process of forming new RBC• STIMULATED BY HYPOXIA
• Polycythemia: condition of above-normal increase in the number of RBCs
• Functions to carry hemoglobin which is responsible for cell respiration
• Newborn has a naturally occurring elevation in red blood cells due to a ligh level of erythropoietin, which stimulates red cell production
Hemolysis
• Normal survival of RBC is 120 days
Red blood cell
IRON= FERRATINBILIRUBIN EXCRETED IN
BILE AND URING
Anemia• A reduction in the number of red blood cells, the quantity of
hemoglobin and the volume of packed red cells below normal levels
• Iron deficiency anemia– Lack of iron
– Affects production of RBCs
– RBCs appear hypochromic, decreased hemoglobin synthesis– Manifestations based on severity
• Pallor occurs when hemoglobin levels are less than 7 g perdL• Fatigue• Irritability• PICA
• Thalassemia
• Aplastic Anemia
• Pernicious Anemia
• Sickle Cell Anemia
HYPOCHROMIC
RED BLOOD
CELLS
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What causes iron deficiency
• Increased iron needs
– Rapid growth of infants and toddlers require more
– Women that are pregnant
– Adolescents with heavy menstrual periods
• Decreased iron intake or absorption
– Iron from meat is absorbed 2-3X more efficiently
than iron from plants
• Excess loss
– Hemolytic anemias
Iron deficiency anemia signs and symptomsMost common type of anemia in children
Conjunctivalpallor
Difficulty maintaining body
temperature
Inflamed tongue
glossitis
Decreased immune function
Palmar crease pallor
Irritability and anorexia
Tachycardia
Systolic murmurpica
Lethargy, increased need
for sleep
Lack of interest in toys or play
activities
Iron deficiency anemia
lab values
“How is it diagnosed?”
• Hemoglobin
• Hematocrit
• Reticulocyte count
Hemoglobin 9.5-11 g/dL Mild iron deficiency anemia
Hemoglobin 8-9.4 g/dL Moderate iron deficiency
anemia
Hemoglobin < 8 g/ dL Severe iron deficiency anemia
Reticulocytes
• Helps distinguish a hypoproductive anemia
from a destructive process
Indicates bone marrow
disorders or aplastic crisis
Indicates hemolytic process or
active blood loss
Iron deficiency Anemia
Risks
Demographic factors
•Prematurity
•Teenagers
•Female
•6 month old
•Multiple gestation
Dietary factors
•Cow’s milk before age 1yr
•> 24 oz. of cow’s milk
•Low iron formula
•Breastfeeding without iron supplementation
Social/political factors
•Low socioeconomic background
•Recent immigration from a developing country
•rate of weight gain greater than averag
Risk for Iron Deficiency anemia
Introduction of cow’s
milk in the first year
of life
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Evidence-Based careprevalence of iron deficiency anemia between the ages
of 1-3 years is greater than we think
• Third National Health
and Nutrition
Examination Survey
(NHANES III)
• Third Report on
Nutrition Monitoring in
the United States
• Reported that
prevalence of iron
deficiency anemia in
one to two-year-olds to
be 3%
• Prevalence in one-to
three-year-olds to be
12%
Why does risk increase again after 1
year?
• Cow’s milk replaces breastfeeding or iron
fortified formula
• Noniron-fortified cereals enter the diet and
• juices reduce the child’s apetite for solid food
Comparison of cow’s milk and
breastmilk r/t anemia risk
Cow’s milk breastmilk
Low in iron Low in iron
Iron is poorly absorbed 50% is bioavailable to infant
It decreases the absorption of
iron from other dietary sources.
Exclusive breastfeeding after 4-
6 months increase the risk for
iron deficiency
When are children screened for Fe
deficiency anemia
• 9- 12 months
• 24 months
Table 28-4 Infectious and Inflammatory Causes of Anemia Complications of Iron Deficiency
Anemia
• Poor muscle development
• Decreased attention span
• Decreased performance on developmental
tests
• Decreased ability to process information
obtained through hearing (auditory
processing)
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Anemia treatment
• Iron supplementation
– ORAL FERROUS SULFATE at 3-6 mg/kg/day for 4
weeks, and then repeat Hgb/hct
• Increase iron in diet
Medication therapy
Ferrous Sulfate• Liquid iron should be taken through a straw to
prevent staining of teeth. For infants administer into the back of the mouth.
• Administer on an empty stomach
• Administer with source of vitamin C
• Side effects– Black tarry stools
– Constipation
– GI discomfort
– Foul aftertaste
Dietary management of prevention of
iron deficiency anemia
newborn
breastfed
Before 4 months nothing
After 4 months
Add sources of iron
After 12 months
Add iron rich food
Formula fed
High iron
Do not give additional iron
Low iron
Not recommended
Iron rich foods
• Enriched bread and cereal
• Iron-fortified dry cereal
• Dark green vegetables
• Beans
• Meats, fish, poultryADD VITAMIN C WHICH HELPS THE
BODY ABSORB IRON
Evaluation
• With treatment, reticulocyte count increases within
3-5 days. Indicates a positive therapeutic response
• Hemoglobin should return to normal within 4-8
weeks
• If Hemoglobin has increased by more than 1 g/dL or
hematocrit has increased by more than 3%, continue
treatment for 2 more months, repeat labs,
• When labs are normal, wean from Fe supplements,
• Repeat labs in 6 months, monitor wt/ development
September is sickle
cell awareness
month. More than
70,000 people in
the US have sickle
cell disease and
more than 2
million people
carry the gene that
allows them to
pass it on to their
children
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Anemia (continued)
• Sickle cell anemia
– Most common single gene disorder in African
americans
– Genetic mutation autosomal recessive disease
– Hemoglobin S replaces normal hemoglobin
– RBC’s loose doughnut shape become sickle-shaped
– Manifestations appear in multiple body systems
– Severity based on pathologic changes
FIGURE 28–5 The clinical manifestations of sickle cell anemia result from pathologic changes to structures and systems throughout the
body.
FIGURE 28–5 (continued) The clinical manifestations of sickle cell anemia result from pathologic changes to structures and systems
throughout the body. Sickle Cell Anemia
Sickling
• Triggered by fever, emotional stress,
physical stress
• States of hypoxia
– High altitudes
– Poorly pressurized airplanes
– Hypoventilation
• Dehydration
• Cold
Sickle cell crisis
• Acute exacerbations of disease
• Vary in severity and frequency
• Three types
– Vaso-occlusive crisis
– Sequestration crisis
– Aplastic crisis
Vaso-occlusive crisis
“Painful crisis”• Happens when sickled red blood cells block small
blood vessels that carry blood to tissues or bones, causing pain that can begin suddenly and last several hours to several days.
• Thrombosis and infarction of tissue may occur if crisis is not reversed
• Fever, pain, swelling of joints in hands and feet
• Severe abdominal pain
• priapism
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Splenic sequestration
• Blood is trapped in spleen leads to vasomotor collapse and shock
• Life threatening complication, second only to infections as a cause of death in infants with sickle cell disease.
• Occurs between the ages of 4 months and 3 years
• Treated with aggressive blood transfusions
• Emergent splenectomy
Aplastic crisis
• Diminished erythropoiesis and increased
destruction of red blood cells
• Triggered by viral infection or depletion of folic
acid
• Clinical manifestations include profound
anemia, pallor and fatigue
Pain r/t sickling of RBC
• Can be reversed
• OXYGENATION
• HYDRATION
• PAIN MANAGEMENT
• rest
Risk for infection
• Daily prophylactic penicillin VK 125 mg bid
starting at 2 months of age
• Erythromycin for children with penicillin
allergies
• Vaccines to prevent pneumonia, haemophilus
influenzae type B (HIB)
Medications for SS anemia
• Hydoxyurea 15-20 mg/kg/day to start and increased
until therapeutic response (not more than 35 mg/kg)
– Decreases the production of abnormal blood cells and
leads to a lesser amount of pain.
– Side effects include bone marrow suppressin, headaches,
dizziness, nausea, and vomiting.
Anemia (continued)
• Hereditary spherocytosis
– Congenital hemolytic anemia
– No abnormality of hemoglobin
– Cells have unusual structure
– Manifestations appear in neonatal period or infancy
– Severity varies
• Thalassemias
– Groups of hereditary disorders
– Hemoglobin synthesis abnormal
– Range mild to severe
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Anemia (continued)
• Thalassemias
– Three types based on which chain in hemoglobin
molecule is defective
– Clinical manifestations based on type and severity
– Severe anemia leads to chronic hypoxia
• Autosomal recessive
• Symptoms include pallor, poor wt. gain,
hepatosplenomegaly, severe anemia
FIGURE 28–6 Red blood cell appearance in ß-thalassemia. What characteristic abnormalities can be seen on this microscopic view?
Courtesy of Dr. Ed Wong, Laboratory Medicine, Children’s National Medical Center, Washington, DC.
B-thalassemia
• To compensate for decreased HbA, production of
Hb F increases. The RBCs are fragile and are
easily destroyed, shortening their lifespan
• As hemolysis increased, hemosiderin is deposited
in the skin, causing a bronze appearance.
• Chronic anemia leads to hyerplasia of bone
marrow cavity and thinning of the bone marrow
cortex as the bone marrow attempts to
compensate for the anemia, leading to fractures
Thalassemia clinical manifestations
RBC
Hypochromic and microcyticchanges
Folic acid deficiency
Frequent epistaxis
Skeletal changes
Osteoporosis
Risk of fractures
Delayed growth
Facial deformities
Heart
Chronic congestive heart failure
Myocardial fibrosis
murmurs
Liver/gallbladder
Hepatomegaly
Hepatic insufficiency
Spleen
splenomegaly
Endocrine system
Delayed sexual development
Fibrotic pancreas
Diabetes risk
Skin
Darkening of the skin
Treatment goal
• Maintain normal hemoglobin levels
• Frequent transfusions
– Transfusion reactions
– Hemochromatosis
• Chelation to prevent iron overload
• Bone marrow transplants
– Now improved with stem cells from siblings
Problems of White Blood Cells
Leukemia
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Leukemia
• Most commonly diagnosed malignancy in
children under 14
• Cancer of the blood or bone marrow,
characterized by an abnormal proliferation of
blood cells, usually white blood cells
Childhood Leukemia
• Acute Lymphocytic Leukemia (ALL) 75% of
leukemias in children
– Peak age of onset is 2-4 years
– Rapid proliferation of immature blood cells which
makes the bone marrow unable to produce
healthy blood cells.
• Acute nonlymphocytic (ANLL) or Acute
Myelomonodcytic leukemia
Leukemia
Initial presentation• Symptoms are related to infiltration of blasts in th bone marrow, lymphoid
system, and extramedullary sites (CNS)
• Fever (60%)
• Fatigue (50%)
• Pallor (25%)
• Weight loss (26%)
• Bone pain (23%)
• Disruption of normal hematopoiesis
• Thrombocytopenia (<100,000) 75%
• Hemoglobin less than 7 g/dL (40%)
• WBC can be either elevated (>50,000) in 20% or – Decreased (< 500)
• Lymphadenopathy, splenomegaly
• CNS involvement (<5%)– Headache, vomiting, papilledema
Leukemia: Treatment
• Induction chemotherapy: kill as many
leukemic cells as quickly as possible (over a 4-
6 week period = attain remission
– vincristine
– prednisone
– L-asparaginase
– daunomycin
Leukemia: Chemotherapy phaseII
CNS Prophylaxis:
• Since leukemic cells sometimes infiltrate the CNS and
accumulate in the brain, and drugs used for
induction chemotherapy do not penetrate the
“blood-brain” barrier in sufficient quantities to
destroy leukemic cells in the brain, most ALL patients
undergo radiation and/or “intrathecal”
chemotherapy .
• ARA-C
• Methotrexate
Leukemia Chemotherapy: Phase III
Consolidation Chemotherapy
• Given to reduce the risk of relapse
• One to six courses of chemotherapy
– L-asparaginase
– Doxorubicin
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Leukemia Treatment:
Delayed Intensification
• Chemotherapy
– Vincristine
– ARA-C
– Cyclophosphamide
• Targets the leukemic cells that have survived.
Leukemia Chemotherapy: Phase IV
Mainenance Chemotherapy
• Begins after the completion of remission
induction and consolidation therapy.
• Combined drug therapies are used
• CBC’s are measured and periodic bone
marrow aspirates are performed to be sure
the child remains in remission
• Prognosis after relapse depends on timing of
relapse (after 3 yrs= good prognosis; less than
18 months < 5% chance of survival)
Follow-up therapy
• Supportive care may include
– IV nutrition
– Antibiotic prophyllaxis
Leukemia:
Bone Marrow Transplantation
• Option for patients who achieve a complete
remission following standard chemotherapy
and subsequently relapse.
• Three types of BMTs:
– Allogenic donor
– Syngeneic identical twin
– Autologous pt’s own bone marrow
Bone Marrow Transplant:
Complications• Infection (CMV)
– special air filters
– screening blood products
– staff and visitors use protective clothing
– no live plants, fruits, vegetables in pt’s room
– prophylactic antibiotics
• graft vs host disease (20-50%)
– donor’s bone marrow begins attacking organs
and tissues in pt’s body
Nursing Management
• Difficult due to multisystem effect
• Long period of treatment required
• Assessment complete and thorough
– Observe signs of bleeding
– Signs of infection
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Nursing Management (continued)
• Monitor for toxic side effects of chemotherapy or tumor cell lysis
– Renal function
• Specific gravity– I & O
– Daily weight
– Special attention for children on cyclophosphamide
• Nutrition
– Daily intake, nausea and vomiting,
– constipation
• CNS infiltration
Nursing Management (continued)
• Pain
• Bone marrow suppression
– Isolation and transmission precautions
• Education of family and child
– Careful handwashing
– Prevention of spread of infection
– Oral care
• Nurses are vital to the multidisciplinary team
Bleeding disorders
• Hemophilia A (factor VIII deficiency)
• Hemophilia B (factor IX deficiency)
• Von Willebrand Disease
• Others
Hemophilia
• Hereditary bleeding disorder
• Deficiency in clotting factors (factor VIII is most common)
• X-linked, most expressed in males, females carrier status
• Manifestations range mild to moderate to severe and is consistent between family members
• About 30% are new mutations
Degrees of severity of Hemophilia
• Normal factor VIII level= 50-150%
• Mild hemophilia
– Factor VIII or IX level is 6-50%
• Moderate hemophilia
– Factor VIII or IX level is 1-5%
• Severe hemophilia
– Factor VIII or IX is < 1%
Clinical manifestations
• Spontaneous bleeding
• Hemarthrosis: Joint bleeding
• Deep tissue hemorrhage
• Nosebleeds
• Hematuria
• Easy bruising
• Life-threatening bleeding
• Head/ intracranial
• Neck and throat
• Abdominal/ GI
• Iliopsoas muscle with decrease hip range of motion
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Complications of bleeding
• Flexion contractions
• Joint arthritis
• Chronic pain
• Muscle atrophy
• Compartment syndrome
• Neurologic impairment
Hemophilia treatment
• Goal control bleeding by replacing the missing clotting factors
– On demand
– prophyllaxis
• IV infusions consist of
– Plasma
– Fresh frozen plasma
– Cryoprecipitate
– Factor VIII
Prophylaxis
• Scheduled infusions of factor concentrates to
prevent most bleeding
• Frequency is 2-3 times per week to deep
trough factor VIII or IX levels at 2-3%
DDAVP (Desmopressin acetate)
not to be confused with DDAVP for
nocturnal enuresis• Synthetic vasopressin
• Method of action
– Release of stores from endothelial cells raising
factor VIII and vWD serum levels
• Administration
– IV
– Subcutaneously
– Nasally (stimate)
• Side effects
Amicar
(epsilon amino caproic acid)
• Antifibrinolytic
• Uses
– Mucocutaneours bleeding
– 50-100mg/kg q 6 hours
• Contraindications
– hematuria
Complications of treatment
• Inhibitors/antibody development
– IgG antibody to infused factor VIII concentrates which occurs after exposure to the extraneous VIII protein
– 20-30% of patients with severe hemophilia A
• Hepatitis A
• Hepatitis B
• Hepatitis C
• HIV
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Nursing considerations
• Factor replacement to be given on time
• Lab monitoring as ordered
• Increase metabolic states will increase factor requirements
• Factor coverage for any invasive porcedures
• Document- infusions, response to treatment
• NO NSAIDS
• NO HEAT
• NO IM INJECTIONS
• Utilize Hemophilia Center staff for questions
Psychosocial issues
• Guilt
• Challenge of hospitalizations
• Control issues
• Financial/insurance challenges
• Feeling different/unable to do certain
activities
• Counseling needs
Von Willebrand Disease
• Autosomal dominant trait
• Equal expressivity in males and females
• Manifestations
– Easy bruising
– Epistaxis
Clotting Disorders and Bleeding
Tendencies (continued)
• Idiopathic thrombocytopenic purpura (ITP)
– Autoimmune disorder
– After a viral illness
– Manifestations
• Ecchymoses
• Petechiae
• Purpura
• Bleeding from gums
• Nosebleeds
• Blood in urine
• Blood in stools
Nursing Care of a Child with a
Hematologic Disorder Is Based on
the Disorder• RBCs
– Oxygenation
– Circulation
– Fluid
– Nutrition
– Pain management
Nursing Care of a Child with a
Hematologic Disorder Is Based on
the Disorder (continued)
• WBCs
– Infection
– Oxygenation
– Nutrition
• Platelets and bleeding disorders
– Bleeding
– Oxygenation
– Circulation
– Injury prevention
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Collaborative Care Approach for a
Child with a Hematologic Disorder
• Team approach
• Family involved
– Decisions with family and child
Hematopoietic Stem Cell
Transplant (HSCT) • Treatment for disorders unresponsive to other
theapy
• Pretransplant phase
– Total body irradiation
– Strict isolation
• Transplant phase
– Intravenous transfusion of donor stem cells
– Transplant starts to grow two to four weeks
Hematopoietic Stem Cell
Transplant (HSCT) (continued)
• Posttransplant phase
– Lasts several weeks
– Major risk is infection
– Immunosuppressive agents prevent graft-versus-host disease
• Nursing care
– Disease prevention
– Prevent infections
– Injury prevention
Hematopoietic Stem Cell
Transplant (HSCT) (continued)
• Nursing care
– Growth and development
– Nutrition
– Physical activity limits
– Oral health
– Mental and spiritual health
– Family and social relationships