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

• 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

• 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

• 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

• 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

• 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), anti-tumor agents (nitrogen mustard), toxic material (arsenic), meds (anti-convulsants, anti-inflamatory's, gold compounds)

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

• 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

• 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

• 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