Transcript Blood Disorders

Blood Disturbances
PN111
Hematologic System
Involves:
• Blood
• Blood Cells
• Lymph
• Organs involved with blood formation
• Organs involved with blood storage
Review of Blood
Blood is:
• sticky, opaque, has a salty metallic taste
• can be scarlet in colour (oxy.rich) or dull red
(oxy.poor)
• 5 times thicker than water
• healthy adults- 5-6 liters 7-10% of body wt
• called FLUID TISSUE
• solid and liquid components
• formed elements suspended in plasma
Plasma
Plasma Proteins:
• albumin
• fibrinogen
• globulins
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osmotic balance
helps maintain H2O
clotting of blood
defences (antibodies)
lipid transport
Bone Marrow
• Site of hematopoiesis
• makes up 4-5% of total body wt, one of the
largest organs of the body
• active or red marrow, and , fat or yellow
marrow
• with age red is replaced with yellow but
can reverse
• assisted in crisis by the spleen and liver
Stem Cells
• Primitive cells that can differentiate into
myeloid or lymphoid stem cells
• lymphoid develop into T or B lymphocytes
• myeloid differentiate into erythorcytes,
leukocytes or platelets
• See text for chart on stem cell
differentiation
Stem Cell differentiation
• Erythropoietin - a hormone produced
primarily by the kidney stimulates
differentiation
• when kidney detects low 02 as in anemia,
it releases erythropoietin which stimulates
bone marrow = increased RBC in about 5
days
Bone marrow function
• Erythrocyte production requires iron, vit
B12, folic acid, pyridoxine (Vit B6)
• Iron-stored in small intestine as ferritin.
When required it binds to transferring and
is transported to marrow (6 to 8 hours)
• Iron is lost in feces, bile, blood or intestinal
mucousal cells
Red blood cell destruction
• Life span 120 days
• removed from circulation by spleen or liver
• hemoglobin in recycled and some
becomes bilirubin and secreted in the bile
• most of iron is recycled, small amts lost in
feces
BM aspiration and bx
• Assess the quality and quantity of each
type of cell produced in the marrow
• aspirate usually from iliac crest looks like
slushy fluid
• biopsy from iliac crest usually looks like a
skinny worm
Blood Abnormalities
• Result from problems in the production,
function or normal destruction of any type
of blood cell.
• We will start with RBC disorders
RBC disorders
• Too few RBC = anemia
• Too many RBC = polycythemia
Anemia
• Lower than normal RBC count and Hgb
level
• Anemia is a clinical sign not a diagnosis
• results in diminished amount of O2 to body
tissues
• many different kinds of anemia but all fall
in one of three categories
3 Categories of Anemia
• Decrease in production of RBC’s
(hypoproliferative)
• Excessive destruction of RBC’s
(hemolytic)
• Loss of RBC’s(bleeding)
Etiology includes:
• genetic,
• nutrition deficit, iron deficit
• chronic disease
Complications of Anemia
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Congestive heart failure
parathesias
confusion
death
Clinical Manifestations
• Impacted by severity, speed of
development, duration, pts metabolic
requirements, co-existing disorders or
disabilities.
Clinical Manifestations
• Dyspnea, chest pain, muscle pain or
cramping
• weakness, fatigue, general malaise
• pallor of the skin, MM including sclera
• jaundice, black stool, coffee emesis
• smooth, red tongue
• beefy red tongue, sore tongue
• angular cheilosis
• brittle ridged concave nails and pica
Gerontologic Considerations
• Most common hematologic condition in
elderly due to body’s decreased ability for
bone marrow to respond to body’s need
• treat the underlying cause rather than
writing it off as an ageing problem
• elderly with concurrent cardiac or
pulmonary problem may not be able to
tolerate anemia
Diagnostic Evaluation
• Complete hematologic studies
• iron studies
• serum vitamin B12 and folate levels,
erythropoietin levels
• bone marrow aspiration and biopsy
• other studies to determine underlying
illness/cause
Medical Management
• Correct and control the cause of the
anemia
• replace lost or destroyed RBC’s
Nursing assessment
• Health hx, physical exam, review lab
values
• hx of symptoms, lifestyle, medications,
alcohol use, athletic endeavours
• ? Any loss of bld,
• family hx
• dietary habits
• ?cardiac, neuro, GI functions
Nursing Interventions
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Manage Fatigue
Monitor and Manage complications
maintain adequate nutrition
maintain adequate perfusion
monitor and manage potential
complications
Megaloblastic Anemia
• caused by lack of vit.B12, folic acid
• lack of B12 caused by a problem with the
stomach mucous, absence of intrinsic
factor, strict vegetarian diet, disease of the
ileum or pancreas, gastrectomy
• Lack of folic acid occurs when intake is
deficient or requirement increased
• risk groups include those who do not each
uncooked veggies or fruit, alcoholics,
pregnancy, malabsorptive or small bowel
disease
Signs and Symptoms
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Smooth sore tongue
mild diarrhea
weakness
paleness
confusion
paresthisis in extremities
death after several years
Diagnosis:
• shillings test, CBC, serum folate and B12
Treatment for B12 defic:
• B12 orally
• B12 IM if intrinsic factor missing
• counts usually normal in 2 months
• B12 taken for life
Treatment for folic acid defic.
• Diet = 1mg of folic acid daily
• IM folic acid for malabsorptive disease
• orally
• stopped when hgb returns to normal
except for alcoholics who continue while
alcohol is used
Nursing Care:
• Skin care especially with incontinence,
paralysis
• mouth care
• accurate urine tests
• educate re: monthly injections
(compliance)
• educate re: ongoing medical
assessment especially for increased
risk of stomach CA
Nursing Interventions
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Manage Fatigue
Monitor and Manage complications
maintain adequate nutrition
maintain adequate perfusion
monitor and manage potential
complications
Hemmorragic Anemia
• Sudden loss of blood resulting in
decreased RBC
• hemorrhage
signs and symptoms:
• you tell me
treatment:
• replace blood loss
Aplastic Anemia
• Decrease in RBC as a result of a
decrease in precursor cells (marrow
stem cells) in bone marrow - replaced
with fat
• can be congenital, acquired or
idiopathic
• can be caused by certain infections,
radiation, chemicals (benzene), antitumor agents (nitrogen mustard), toxic
material (arsenic), meds (anticonvulsants, anti-inflamatory's, gold
Signs and Symptoms
• Gradual weakness, pallor, SOBOE,
sometimes fever, adenopathy
• purpura, retinal hemorrhages
• repeated throat infections with possible
lymphadenopathy and splenomegally
Dx:
• CBC, bone marrow and biopsy
Treatment
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bone marrow (stem cell) transplant
immunosuppressants
support therapy - transfusion
frequent CBC
Nursing Care
• Assess re:hypoxia, bleeding, infection
• oral hygiene, no break in skin or
mucous membrane
• preserve energy of pt.
• if low platelets, avoid trauma of IM or
SC
• stool softeners to prevent straining
• if using potentially toxic medications,
monitor blood work
Hemolytic Anemia
• Shortened life span of RBC or destruction
of RBC
• bone marrow tries to compensate by
producing RBC’s 3 times the normal rate
• can be caused by infection or toxic
chemicals, mismatched blood trans,
disorder of the immune system, Hodgkins,
liver disease
Dx, and Tx
• Reticulocyte count (elevated)
• bilirubin increased
• haptoglobin decreased (binding protein
for free Hgb)
treatment:
• in severe cases - hospitalization
• blood transfusion
• removal of spleen
• detection and tx of cause
Nursing Care
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Bed rest to conserve energy
avoid IM’s
frequent mouth care
02 support
increased protein diet
stool softener
skin care
Sickle Cell Anemia
• Severe hemolytic anemia because of a
defective Hgb molecule
• genetic defect of a single amino acid
necessary for hemoglobin
• RBC becomes deformed in venous
circ.when exposed to low O2
• RBC takes on sickle shape and
becomes lodged in small vessels-slows
circulation to area and causes ischemia
and pain
Sickle Cell Anemia
• Sickle cell has a short life span 15-25 days
• at risk: 1 in 375 African American,
Mediterraneans, Carribeans, South and
Central American heritage, Arab and East
Indian ancestry
Signs and Symptoms
• In crisis - severe pain, fever, swelling in
arms, legs and abdomen
• anemia
• jaundice in sclera
• progressive renal damage
• In childhood - larger bones in face and
skull due to bone marrow expansion
• enlarged heart, tachy, damage to any
organ
• increased blood viscosity
Diagnosis
• Hgb and hematocrit levels, blood smear,
• electrophoresis because the sickling will
occur even if the pt has sickle cell trait or
sickle cell anemia and this test will tell the
difference
Treatment
• Diagnose early in children, genetic
counselling
• 1st crisis may be at age 1 - 2
• average life span 40 years
• blood transfusion, bone marrow transplant
• new drugs to prolong RBC life span (side
effects)
Nursing Care
• Pt.teaching re: avoiding infections
• avoiding high altitudes
• increased folic acid (where can we get
this?)
• In a crisis, ++ fluids, analgesia, monitor
blood transfusions.
• Maximize pts. Knowledge base
Potential complications
• Leg ulcers
• priaprism leading to impotence
• chronic pain and substance abuse
Iron Deficiency Anemia
• A form of anemia where the RBC contain
less Hgb.
• Usually due to an inadequate supply of
iron or due to loss of blood
• RBC may be described as microcytic or
hypochromic, what does this mean?
Signs and Symptoms
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Low energy
pallor of skin or mucous membranes
fatigue, poor appetite
smooth, sore tongue, brittle nails, angular
cheilosis, pica
• Hgb lower than hematocrit and RBC count
• low serum iron, ferritin low
Evaluation
• Bone marrow aspiration
• lab work: ferritin, blood count, serum iron
levels, total iron binding capacity (high)
Treatment
• Search for cause, treat cause
• test stools for occult bld
• iron preparations
Pharmacology
Vitamin B12:
• body can store 4 to 5 mg of B12 so it may
take 2 to 5 years for a deficiency to be
detected after intrinsic factor is no longer
released.
• Minimum daily requirement is 1 to 2
micrograms
B12
• Usual therapeutic dose is 30 - 50 ug/day
times 5 days then 100 to 200 ug monthly.
• Cyanocobalamin
• can be p.o or parenteral. Not given po.for
pernicious anemia Why?
• IM or deep sc.
• Avoid alcohol, may need intrinsic factor or
folic acid, What is the underlying cause?
Folic Acid
• For anemias related to folic acid defic.
• Not stored in great quantities in the
body
• require 1mg/day
• drug interference from oral
contraceptives, aspirin, phenytoin
• dietary sources: liver, broccoli, dried
beans, lentils, peas, spinach, beets
• Routes: po, IM, SC, IV
• may turn urine dark yellow/green
Iron (Fe)
• For anemia related to iron deficiency
• taken up by cells in duodenum/upper
jejunum
• ferritin binds with the iron and once
saturated the remaining iron is discarded
in stool
• iron is transferred as needed to plasma
proteins by binding with transferrin.
Iron
• The iron then goes to the bone marrow
where it is incorporated into Hgb.
• Side effects: GI upset
• Overdose: 4 stages, may lead to coma or
death.
• Drug interactions:
• Antidote: DESFERAL
Fe
• Take iron supplements an hour before
meals. If GI upset, take with meals and
after symptoms subside resume before
meals.
• Stool colour changes to green or black
• brush teeth and floss, take liquid with a
straw
• avoid antacids or dairy