Transcript Chapter 26 Hematology

Highlights from Chapter 26
Conditions of the blood and
lymphatic system
Case Study
• 6 month old infant with history and physical of
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Prematurity
Late prenatal care
16 year old mother
Has missed last two WIC clinic appts; mixing formula
with cows milk
– Underweight
– Pale in appearance
– murmur
Iron Deficiency Anemia
• A decrease in the size and amount of RBCs
directly related to the deficiency of Iron
• Microcytic, hypochromic anemia
• Risk Factors:
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Dietary
Prenatal or birth history related
Childhood risk related
Socio economic related
Iron Deficiency Anemia
Diagnosis
• Low Hgb/Hct
• Low MCV (volume of size of the RBC)
– 77-95
• Low MCHC (amount of hgb in the RBC)
– 31-37
• RDW is high (variability of size of the RBC)
• High Total Iron Binding Capacity
• Low serum ferritin level
Risk Factors
• Dietary
– Cows milk consumption
before 12 months (1605)
– Excessive cows milk after
12 months
– Lo FE formula
– BF without FE
supplementation at 6 mos
– Excessive weight gain
• Prenatal/Perinatal
– Anemia during
pregnancy
– Gestational diabetes
– Prematurity (1605)
– Low Birth Wt
– Multiple gestation
Risk Factors on
FE deficiency anemia
• Childhood risk related
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Chronic infections
Blood loss
Restricted diet
Medications that
interfere with
absorption
• Socio economic
– Low SE class
– Recent immigration
from a developing
country
Manifestations
FE deficiency anemia
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Pallor
Irritability
Anorexia
Decrease in activity
Over weight “milk babies”
Tachycardia
Flow murmur
Splenomegaly
Treatment
FE deficiency anemia
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Ferrous sulfate orally bid or tid
Give with vitamin C liquids
Between meals
NOT with milk
Take through straw
Iron rich foods
Iron IM; Z track method
AVOID IRON POISONING!!!!!!!!
IRON POISONING
• Stomach pain, fever, nausea, vomiting
– Vomiting may be brown stained or bloody
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Bluish lips, fingernails, palms
Drowsiness, weakness
Tachycardia, seizures
Metabolic acidosis
Hepatic injury
Cardiovascular collapse
Iron Poisoning Treatment
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Syrup of Ipecac
Gastric lavage with sodium bicarbonate
Deferoxamine is the antidote; IM injection
Maintain fluid and electrolyte balance
Correct metabolic acidosis
Fer in sol Drops
Sickle Cell Anemia
• RBCs through a complex chemical process and
deoxygenation become sickle shaped.
• Sickle cells cannot pass through capillaries, they adhere
to the vessel walls and each other, causing a pile-up
• Symptoms of Sickle Cell are caused by:
– Enlarging bone marrow sites that impair circulation to
the bone
– Abnormal sick cell shape that causes clumping and
obstruction in the vessel
– Ischemia to the organ that the vessel supplies
Comparison of normal and
sickle cells
• Normal
– 120 day life span
– Normal oxygen
carrying capacity
– 12-14 g of hgb/ml
– RBCs destroyed at
normal rate
• Sickled
– 30-40 day life span
– Decreased oxygen
carrying capacity
– 6-9 g of hgb/ml
– RBCs destroyed at an
accelerated rate
Sickle Cell Anemia
Sickle Cell Anemia
Manifestations of
Sickle Cell Anemia
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Chronic Anemia
Decreased stamina
Tachycardia
Delayed growth
Functional asplenia
• Sickle Cell Crisis
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Vasocclusive
Splenic Sequestration
Aplastic crises
Hyperhemolytic
Acute chest syndrome
Triggers of Crisis
Sickle Cell Anemia
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Dehydration
Cold stress
Fever
Infections
Flight travel
High altitudes
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Hypoxia
Pregnancy
Stress
Drug and alcohol
abuse
• Bleeding
Complications of
Sickle Cell Anemia
• Anemia
• Infections
• Acute Chest
Syndrome
• Pain
• Stroke
• Convulsions
• CNS, cognitive
dysfunction, learning
disabilities
• Priapism
• Iron overload
• Cholelithiasis
• Avascular necrosis
fermoral head
• Splenic sequestration
Treatments for
Sickle Cell Anemia
• Infection
treatment/prevention
• Rest
• hydration
• O2 for acute chest
syndrome
• Pain medication
• Electrolyte
management
• Blood transfusions
– Goal is to keep HbS at
<30%
– May prevent complications
such as stroke
– Usually keep hgb<10
– Exchange transfusions
• Hydroxyurea (Hydrea)
• Review 1609-10
Infection Prevention
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Prophylactic PCN
Pneumococcal vaccine
Menigococcal vaccine
Routine vaccines
Yearly influenza vaccine
About the spleen
• Functions
– Filtrates the defective and aged cells and ingests them
with macrophages
– Clears circulating bacteria
• Spleenic sequestration crisis
– A pooling of blood in liver and spleen with decreased
volume and shock
– Sudden weakness, Dyspnea, left abdomen pain
• Treatment
– Fluids
– Erythrocyte transfusions
– spleenectomy
Hydroxyurea
• Only agent shown to decrease incidence
and severity of pain. Mechanism of action is
thought to be the induction of fetal
hemoglobin production.
• Takes several months to see benefits
• Progressive dosing until leukocyte count is
>4,000 (do not go below)
• Main side effect is decrease in all blood
counts including platelets
• Renal/liver toxicity must be monitored
Priapism Care
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Increase fluid intake
Oral analgesics
Pseudoephedrine 30 mg qhs
Go to ER if event lasts >2 hours
Nursing Care
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Teaching focuses on avoiding triggers
Pain care
Oxygen maintenance in patient
Transfusion care
Hydration
Infection prevention and treatment
References for SS Anemia
• http://www.emedicine.com/ped/topic2096.
htm#section~workup
• http://www.emedicine.com/ped/topic2139.
htm
Thalassemia
• Genetic defect causing anemia
• Thalassemia Minor is associated with mild
anemia
• Thalassemia Major is a progressive severe
anemia
• The structure of the Hgb molecule is
unstable, disintegrates and damages the
RBCs in the process
Thalassemia Major
Clinical Manifestations
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Anemia
Thrombocytopenia
Increased WBC count
Hypoxic
Poor appetite; FTT
Jaundice
Hemosiderosis
Liver enlargement
spleen enlargement
• Abdomenal distention
• Cardiac failure
• Bone marrow space
enlargement
– Changes in facial
contour
– Overgrowth of upper
jaw
– Pathologic fractures
Hemosiderosis
• Iron toxicity
• Due in part to the heavy Fe turnover with
transfusions
• Also due to rapid destruction of RBCs
• Increased Fe absorption in patients with
Thalassemia due to overwhelming production of
ineffective erythrocytes.
• Chelated with subcutaneous deferoxamine
via pump 8-12 hours 5-7 days week
Management of Thalassemia
Therapeutic
• Goal to maintain sufficient Hgb levels to
prevent bone marrow expansion
(oseteoporosis and osteopenia) and to
support normal growth and physical
activity
• Hgb level above 9.5
Care Considerations in
Thalassemia Major
• Blood transfusion care
• Post surgical splenectomy care
– Proph antibiotics to prevent infection
• Chelation therapy for hemosiderosis
– Deferoxamine mesylate
Hemophilia
• Sex linked recessive trait on X
chromosome
• Two most common types:
– Christmas disease (factor IX deficiency)
– Hemophilia A (factor VIII deficiency)
• Treatment involves increasing level of
factor VIII
Manifestations of Hemophilia
• Abnormal bleeding of circumcision or umbilical
cord at birth
• Minor bruises/cut create extensive injury
• Very prolonged clotting time: up to an hour!
• Hematuria
• Hemarthrosis
• Unexpected bruising
• Mouth bleeding
Care for Hemophilia
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Factor VIII concentrate
Recombinant anti hemophilic factor
DDAVP nasal spry
Aminocaproic Acid (prevents clot destruction)
R.I.C.E.
Medical Identification
Appropriate activities that build self esteem and
promote healthy life style
Bruise on a child with
hemophilia
Cool story
• http://coalitionforhemophiliab.org/accompl
ishments/#john
Idiopathic Thrombocytopenic
Purpura
• Acquired platelet disorder
• Thought to be autoimmune reaction due to
a virus; onset two weeks later
• Platelets perceived as foreign material in
body and destroyed by the spleen
• Manifested by petechia, purpura, nose
bleeds, low platelets
• Resolves in 6 weeks to 4 months
Nursing Care for ITP
• Avoid drugs that interfere with platelet
function
• Limit activity to prevent bleeding
• Soft tooth brushes
• Occasional packed RBC transfusion
• Steroids, Intravenous immune globulin
(IVIG)
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Leukemia
• Malignant disease resulting in uncontrolled
growth of immature WBCs
• High WBC count of immature cells that don’t
function.
• Take over centers that develop RBCs and anemia
develops
• Infiltrate centers in the bone marrow that produce
platelets and bleeding tendencies develop
• Pathological fractures also develop
• Invade and drain other organs causing metabolic
starvation
• Neutropenic and thrombocytopenia are features
Three main consequences
• Anemia
• Infection
• Bleeding
Manifestations of Leukemia
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Lo grade fever
Pallor
Bruising tendency
Leg and joint pain
Listlessness
Abdominal pain
Enlargement of lymph
node
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Liver enlargement
Spleen enlargement
CNS involvement
Petechiae and purpura
Anorexia,vomiting
Weight loss
Dyspnea
Hematuria
Infections, ulcerations
Nursing Care for
Children with Leukemia
• Infection prevention a
number one priority
• Hydration to reduce
kidney damage
• Delay active
immunizations
• Immune globulin for
exposure to diseases
such as chicken pox
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Bleeding concerns
IV care: Central line
Nutritional concerns
May need TPN
I/O
Meticulous oral
hygiene
• Hair loss/wig
Hodgkin’s Disease
• Malignancy of the lymph system
• Adolescent and young adults hood
• Twice as common in boys as in girls
Manifestations of Hodgkin’s
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Low grade fever,
Anorexia
Weight loss
Night sweats
Malaise
Rash,
Itching
Nursing Care
• Relief of SE of chemo and radiation
therapy
• Pt tires easily
• Skin sensitivity; sunscreen important
• Splenectomy; increased risk of infection