Medical Surgical Nursing 1 - Lecture
STUDENT ACTIVITY SHEET
BS NURSING / THIRD YEAR
Session # 7
LESSON TITLE: PATIENTS WITH OXYGEN-TRANSPORT
RELATED HEMATOLOGIC DISORDERS
LEARNING OUTCOMES:
Upon completion of this lesson, the nursing student can:
1. Discuss the pathophysiologic responses to
alterations/problems in oxygenation specifically with
patients with oxygen-transport related hematologic
disorders;
2. Assess the at-risk and sick adult client’s one’s health
status/competence;
3. Formulate with the client a plan of care to address needs
/problems and based on priority;
4. Implement safe and quality nursing interventions with client
to address needs/ problems;
5. Institute appropriate corrective actions to prevent or
minimize harm arising from adverse effects; and,
6. Compare patients with oxygen-transport related
hematologic disorders with regard to cause, incidence,
clinical manifestations, management, and the significance
of preventive health care.
Materials:
Pen, paper, index card, book, and class List
Reference:
Hinkle, J. L., Cheever, K. H., & Hinkle, J. L.
(2018). Brunner & Suddarth's Textbook of
Medical-Surgical Nursing. Wolters Kluwer.
Grinslade, S., Singel, L. J., & Keys, P. L.
(2005). Study Guide for Black & Amp; Hawks
Medical Surgical Nursing: Clinical Management
for Positive Outcomes. Elsevier Saunders.
Anef, C. D. R. D., & Faan, R. P. L. W. M. (2012).
Medical-Surgical Nursing: Patient-Centered
Collaborative Care, Single Volume (Ignatavicius,
Medical-Surgical Nursing, Single Vol) (7th ed.).
Saunders.
LESSON PREVIEW/REVIEW (5 minutes)
Instruction: Differentiate ARF and ARDS. Explain briefly
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MAIN LESSON (50 minutes)
The hematologic system consists of the blood and the sites where blood is produced, including the bone marrow and the
reticuloendothelial system (RES). Blood is a specialized organ that differs from other organs in that it exists in a fluid
state. Blood is composed of plasma and various types of cells. Plasma is the fluid portion of blood; it contains various
proteins, such as albumin, globulin, fibrinogen, and other factors necessary for clotting, as well as electrolytes, waste
products, and nutrients. About 55% of blood volume is plasma.
Common Nursing Diagnosis for Patients with Oxygen-Transport Related Hematologic Disorders
1. Risk for ineffective tissue perfusion related to sickled cells and infarction
2. Acute pain related to tissue infarction
3. Risk for bleeding related to factor deficiencies
4. Risk for ineffective management of therapeutic regimen related to knowledge deficit
5. Risk for injury from infection or bleeding
6. Fatigue related to decreased red cell count and oxygenation and effects of treatment
HEMATOLOGIC DISORDERS
Most hematologic diseases reflect a defect in the hematopoietic, hemostatic, or reticuloendothelial system. The defect can
be quantitative (e.g., increased or decreased production of cells), qualitative (e.g., the cells that are produced are
defective in their normal functional capacity), or both. Initially, many hematologic conditions cause few symptoms.
Therefore, extensive laboratory tests are often required to diagnose a hematologic disorder.
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1. Anemia is an abnormally low hemoglobin concentration in the blood. There are several methods of classification, but
the prevailing systems are based on red cell size and shape.
Signs and Symptoms: Pallor, tachycardia, tachypnea, irritability, fatigue, shortness of breath
Pathology
A decrease in the number of RBCs can be traced to three different conditions:
1. impaired production of RBCs, (aplastic anemia, nutritional deficiencies)
2. increased destruction of RBC’s (hemolytic, sickle cell anemia)
3. massive or chronic blood loss
Medical Management
 Directed toward correcting or controlling the cause of the anemia
 If the anemia is severe, the erythrocytes that are lost or destroyed may be replaced with a transfusion of packed red
blood cells (PRBCs)
2. Aplastic Anemia is rare disease caused by a decrease in or damage to marrow stem cells, damage to the
microenvironment within the marrow, and replacement of the marrow with fat.
Signs and Symptoms
 The manifestations of aplastic anemia are often insidious.
 Typical complications are infection and the symptoms of anemia (e.g., fatigue, pallor, dyspnea).
 Purpura (bruising) may develop later.
Pathology
 (AA) is characterized by bone marrow hypocellularity, resulting in peripheral cytopenia.
 An antigen-driven and likely auto-immune dysregulated T-cell homeostasis results in hematopoietic stem cell injury,
which ultimately leads to the pathogenesis of the acquired form of this disease.
 The cells produced from the BM are normal in size and shape but is limited in number, the resulting peripheral
pancytopenia and marrow hypoplasia deters the function of the bone marrow and the capacity to carry oxygen and
nutrients to the body
Medical Management
 Those who are younger than 60 years, who are otherwise healthy, and who have a compatible donor can be cured of
the disease by a bone marrow transplant (BMT) or peripheral blood stem cell transplant (PBSCT).
 the disease can be managed with immunosuppressive therapy, commonly using a combination of anti -thymocyte
globulin (ATG) and cyclosporine or androgens.
 Supportive therapy plays a major role in the management of aplastic anemia. Any offending agent is discontinued.
 The patient is supported with transfusions of PRBCs and platelets as necessary. Death usually is caused by
hemorrhage or infection.
Nursing Management
 They should be assessed carefully for signs of infection and bleeding.
 If patients require long-term cyclosporine therapy, they should be monitored for long-term effects, including renal or
liver dysfunction, hypertension, pruritus, visual impairment, tremor, and skin cancer.
 Nurses must also monitor for side effects of therapy, particularly for hypersensitivity reaction while administering ATG,
due to this each new prescription needs careful assessment for drug–drug interactions
 Patients also need to understand the importance of not abruptly stopping their immunosuppressive therapy.
3. Iron Deficiency Anemia is a common type of anemia — a condition in which blood lacks adequate healthy red blood
cells.
Signs and Symptoms
 Same primary symptoms of anemia;
 If prolonged - smooth, sore tongue; brittle and ridged nails; and angular cheilosis
Pathology
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 Iron deficiency anemia develops when body stores of iron drop too low to support normal red blood cell (RBC)
production.
 Inadequate dietary iron, impaired iron absorption, bleeding, or loss of body iron in the urine may be the cause.
 Iron equilibrium in the body normally is regulated carefully to ensure that sufficient iron is absorbed in order to
compensate for body losses of iron
Medical Management
 People 50 years of age or older should have periodic colonoscopy, endoscopy, or x-ray examination of the GI tract to
detect ulcerations, gastritis, polyps, or cancer
 Oral iron preparations—ferrous sulfate, ferrous gluconate, and ferrous fumarate—are available for treating iron
deficiency anemia. it is important that the patient continue taking the iron for as long as 6 to 12 months.
 In cases of poorly absorbed oral iron IV or, infrequently, intramuscular (IM) administration of iron may be needed. A
test dose must be administered to check for hypersensitivity, and epinephrine should be close at hand.
Nursing Management
 Preventive education is important, because iron deficiency anemia is common in menstruating and pregnant women,
instruct clients on a healthy balanced diet with food sources high in iron and taking these iron rich foods with a vitamin
c source to improve absorption and eat foods high in fiber to minimize problems with constipation.
 Instruct clients that stools will become dark in color
 To prevent staining the teeth with a liquid preparation, use a straw or place a spoon at the back of the mouth to take
the supplement. Rinse the mouth thoroughly afterward
 To prevent skin staining when administering an IM iron supplementation use the z track method. The nurse avoids
vigorously rubbing the injection site after the injection
4. Megaloblastic Anemias are anemias caused by deficiencies of vitamin B12 or folic acid, identical bone marrow and
peripheral blood changes occur because both vitamins are essential for normal DNA synthesis Those cells that are
released into the circulation are often abnormally shaped. The neutrophils are hyper-segmented. The platelets may be
abnormally large. The erythrocytes are abnormally shaped, and the shapes may vary widely (poikilocytosis)
Signs and Symptoms
 Folate Deficiency Anemia – Fatigue, shortness of breath, dizziness
 Pernicious Anemia develops a smooth, sore, red tongue and mild diarrhea, they are extremely pale, particularly in the
mucus membranes. May have paresthesia’s in the legs
Pathology
 Folate Deficiency Anemia is the lack of folic acid in the blood. Folic acid is a B vitamin that helps your body make
red blood cells.
 Pernicious Anemia is a condition in which the body can't make enough healthy red blood cells because it doesn't
have enough vitamin B12.
 Vitamin B12 is a nutrient found in some foods.
 The body needs this nutrient to make healthy red blood cells and to keep its nervous system working properly.
 People who have pernicious anemia can't absorb enough vitamin B12 from food.
 This is because they lack intrinsic factor, a protein made in the stomach.
 A lack of this protein leads to vitamin B12 deficiency.
Medical Management
 Folate deficiency is treated by increasing the amount of folic acid in the diet and administering 1 mg of folic acid daily.
Folic acid is administered intramuscularly only to people with malabsorption problems.
 Patients with alcoholism should receive folic acid as long as they continue to consume alcohol
 Vitamin B12 deficiency is treated by vitamin B12 replacement. Vegetarians can prevent or treat deficiency with oral
supplements with vitamins or fortified soy milk.
 When the deficiency is due to the more common defect in absorption or the absence of intrinsic factor, replacement is
by monthly IM injections of vitamin B12, to prevent recurrence of pernicious anemia, vitamin B12 therapy must be
continued for life.
Nursing Management
 Assessment of patients who have or are at risk for megaloblastic anemia includes inspection of the skin, mucous
membranes, and tongue.
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 The nurse needs to pay particular attention to ambulation and should assess the patient’s gait and stability as well as
the need for assistive devices (e.g., canes, walkers) and for assistance in managing daily activities. Of particular
concern is ensuring safety when position sense, coordination, and gait are affected.
 Because mouth and tongue soreness may limit nutritional intake, the nurse advises the patient to eat small amounts
of bland, soft foods frequently. The nurse also may
 explain that other nutritional deficiencies, such as alcohol induced anemia, can induce neurologic problems
5. Sickle Cell Anemia is a severe hemolytic anemia that results from inheritance of the sickle hemoglobin gene. This
gene causes the hemoglobin molecule to be defective. The sickle hemoglobin (HbS) acquires a crystal-like formation
when exposed to low oxygen tension.
Signs and Symptoms
 Symptoms of sickle cell anemia vary and are only somewhat based on the amount of HbS.
 Anemia (if chronic, tachycardia, cardiac murmurs, cardiomegaly) jaundice, enlargement of the bones of the face and
skull
Pathology
 Due to the deformed shape, HbS induces RBC membrane damage leading to calcium influx into the cell.
 Calcium influx leads to crosslinking of the membrane proteins and activating channels that allow for the efflux of
potassium and water from the cell.
 This leads to RBC dehydration exacerbating the sickling.
 The extravascular hemolysis that occurs when inflexible cells are trapped in the spleen and phagocytosed by the
reticuloendothelial system.
 Bone marrow tries to compensate by increasing RBC production but it cannot match the rate of destruction.
 Splenic sequestration of sickle cells leads to splenic congestion, as manifested by splenomegaly, and reduced
immune function.
Sickle Crisis
1. Sickle Cell Crisis results from tissue hypoxia and necrosis due to inadequate blood flow to a specific region of tissue
or organ
2. Aplastic Crisis results from infection with the human parvovirus. The hemoglobin level falls rapidly and the marrow
cannot compensate, as evidenced by an absence of reticulocytes.
3. Sequestration Crisis results when other organs pool the sickled cells. Although the spleen is the most common
organ responsible for sequestration in children, most children with sickle cell anemia have had a splenic infarction by
10 years of age, and the spleen is then no longer functional (auto splenectomy). In adults, the common organs
involved in sequestration are the liver and, more seriously, the lungs.
Medical Management
 Peripheral blood stem cell transplant
 Hydroxyurea (Hydrea), a chemotherapy agent, has been hown to be effective in increasing fetal hemoglobin (ie,
hemoglobin F) levels in patients with sickle cell anemia, thereby decreasing the formation of sickled cells.
 Arginine has anti-sickling properties and enhances the availability of nitric oxide, the most potent vasodilator, resulting
in decreased pulmonary artery pressure. Arginine may be useful in managing pulmonary hypertension and acute
chest syndrome
 Transfusion therapy - RBC transfusions have been shown to be highly effective in several situations: in an acute
exacerbation of anemia (e.g., aplastic crisis), in the prevention of severe complications from anesthesia and surgery,
in improving the response to infection (when it results in exacerbated anemia), and in severe cases of acute chest
syndrome
 Patients with sickle cell anemia require daily folic acid replacements to maintain the supply required for increased
erythropoiesis from hemolysis. Infections must be treated promptly with appropriate antibiotics; infection, particularly
pneumococcal infection, remains a major cause of death. These patients should receive pneumococcal and annual
influenza vaccinations
 Acute chest syndrome is managed by prompt initiation of antibiotic therapy. Incentive spirometry has been shown to
decrease the incidence of pulmonary complications significantly
 Pain management is a significant issue. The use of medication to relieve pain is important.
 Adequate hydration is important during a painful sickling episode. Oral hydration is acceptable if the patient can
maintain adequate fluid intake; IV hydration with dextrose
 5% in water (D5W) or dextrose 5% in 0.25 normal saline solution (3 L/m2/24 hours) is usually required for sickle crisis.
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 Supplemental oxygen may also be needed.
 The patient is assessed for signs of dehydration. History of fluid intake and careful examination of mucous
membranes, skin turgor, urine output, and serum creatinine and blood urea nitrogen (BUN) values.
 Because patients with sickle cell anemia are susceptible to infections, they are assessed for the presence of any
infectious process.
6. Thalassemia is a group of hereditary anemias characterized by hypochromia (an abnormal decrease in the
hemoglobin content of erythrocytes), extreme microcytosis (smaller-than-normal erythrocytes), destruction of blood
elements (hemolysis), and variable degrees of anemia.
Signs and Symptoms
 Fatigue.
 Weakness
 Pale or yellowish skin,
 Facial bone deformities
 Slow growth
 Abdominal swelling,
 Dark urine Irritability
 Anorexia
Pathology
 Thalassemia is associated with defective synthesis of hemoglobin; the production of one or more globulin chains
within the hemoglobin molecule is reduced. When this occurs, the imbalance in the configuration of the hemoglobin
causes it to precipitate in the erythroid precursors or the erythrocytes themselves. This increases the rigidity of the
erythrocytes and thus the premature destruction of these cells.
 Thalassemia is classified into two major groups according to which hemoglobin chain is diminished: alpha or beta.
 The alpha-thalassemia’s occur mainly in people from Asia and the Middle East, and the beta-thalassemia’s are most
prevalent in people from Mediterranean regions but also occur in those from the Middle East or Asia.
 Thalassemia Major is characterized by severe anemia, marked hemolysis, and ineffective erythropoiesis. With early
regular transfusion therapy, growth and development through childhood are facilitated. Organ dysfunction due to iron
overload results from the excessive amounts of iron in multiple PRBC transfusions.
Medical Management
 The objective of supportive therapy is to maintain sufficient hemoglobin levels to prevent tissue hypoxia.
 Splenectomy is the principal surgical procedure used for many patients with thalassemia.
 Transfusions are the foundation of medical management; recent studies have evaluated the benefits of maintaining
the child’s hemoglobin level above 10g/dl, a goal that may require transfusions as often as every 2 -4 weeks.
 Bone marrow transplantation offers the possibility of a cure for some children with thalassemia, either using marrow
from an unaffected sibling, or a matched, unrelated donor.
 Diet. A normal diet is recommended, with emphasis on the following supplements: folic acid, small doses of ascorbic
acid (vitamin C), and alpha-tocopherol (vitamin E); iron should not be given, and foods rich in iron should be avoided.
 Medications needed for the treatment of various types of thalassemia is nonspecific and only supportive, such as
antipyretics, antihistamines, chelating agents, corticosteroids, antibacterial combinations, vitamins, vaccines,
antineoplastic agents, growth hormone
Nursing Management
 Assess for severe anemia, splenomegaly or hepatomegaly with abdominal enlargement, frequent infections, bleeding
tendencies e.g., epistaxis, and anorexia.
 Assess for anemia, jaundice, and splenomegaly, hemosiderosis caused by increased intestinal absorption of iron.
7. Glucose-6-Phosphate Dehydrogenase Deficiency (G6PD) is a genetic disorder that most often affects males. It
happens when the body doesn't have enough of an enzyme called glucose-6-phosphate dehydrogenase (G6PD).
G6PD helps red blood cells work.
Signs and Symptoms
 Often asymptomatic and have normal hemoglobin levels and reticulocyte counts may develop pallor, jaundice, and
hemoglobinuria (hemoglobin in the urine).
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Pathology
 G6PD deficiency renders the RBC susceptible to oxidative stress, which shortens RBC survival.
 Hemolysis occurs following an oxidative challenge, commonly after fever, acute viral or bacterial infections, and
diabetic acidosis.
 Hemolysis is episodic and self-limited, although rare patients have chronic, ongoing hemolysis in the absence of
oxidative challenge.
Medical Management
 The treatment is to arrest the source and stop the offending medication.
 Transfusion is necessary only in the severe hemolytic state, which is more commonly seen in the Mediterranean
variety of G-6-PD deficiency.
Nursing Management
 Patients are educated about the disease and given a list of medications to avoid.
 Patients should be instructed to wear Medic Alert bracelets that identify that they have G-6-PD deficiency.
 Genetic counseling may be indicated.
8. Polycythemia Vera Polycythemia refers to an increased volume of erythrocytes. The term is used when the
hematocrit is elevated (more than 55% in males, more than 50% in females. Polycythemia Vera (“P vera”), or primary
polycythemia, is a proliferative disorder of the myeloid stem cells.
Signs and Symptoms
 Patients typically have a ruddy complexion and splenomegaly.
 Symptoms result from increased blood volumes and may include headache, dizziness, tinnitus, fatigue, paresthesia,
and
 blurred vision, or from increased blood viscosity and may include angina, claudication, dyspnea, and thrombophlebitis,
particularly if the patient has atherosclerotic blood vessels.
Pathology
 Polycythemia vera involves increased production of all cell lines, including red blood cells (RBCs), white blood cells
(WBCs), and platelets. Thus, polycythemia vera is a panmyelosis because of elevations of all 3 peripheral blood
components.
 Increased production confined to the RBC line is termed erythrocytosis; isolated erythrocytosis may occur with
polycythemia vera but is more commonly due to other causes (see secondary erythrocytosis). In polycythemia vera,
RBC production proceeds independently of erythropoietin level.
 Secondary Polycythemia is caused by excessive production of erythropoietin. This may occur in response to a
reduced amount of oxygen, which acts as a hypoxic stimulus, as in heavy cigarette smoking, chronic obstructive
pulmonary disease, or cyanotic heart disease, or in non-pathologic conditions such as living at a high altitude.
Medical Management
 The objective of management is to reduce the high blood cell mass.
 Phlebotomy is an important part of therapy. It involves removing enough blood (initially 500 mL once or twice weekly)
to reduce blood viscosity and to deplete the patient’s iron stores, thereby rendering the patient iron deficient and
consequently unable to continue to manufacture erythrocytes excessively.
 Chemotherapeutic agents (e.g., hydroxyurea) can be used to suppress marrow function, but this may increase the risk
of leukemia
 Interferon alfa-2b (Intron-A) is the most effective treatment for managing the pruritus associated with polycythemia
vera but may be difficult for patients to tolerate because of its frequent side effects (e.g., flulike syndrome,
depression).
Nursing Management
 The nurse’s role is primarily that of educator. Risk factors for thrombotic complications, particularly smoking, obesity,
and poorly controlled hypertension, should be assessed, and the patient should be instructed about the signs and
symptoms of thrombosis.
 The patient needs to be instructed to avoid iron supplements, including those within multivitamin supplements,
because the iron can further stimulate RBC production.
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CHECK FOR UNDERSTANDING (60 minutes)
You will answer and rationalize this by yourself. This will be recorded as your quiz. One (1) point will be given to correct
answer and another one (1) point for the correct ratio. Superimpositions or erasures in you answer/ratio is not allowed.
You are given 60 minutes for this activity:
Multiple Choice
1. In a severely anemic patient, you expect to find
A. dyspnea and tachycardia.
B. cyanosis and pulmonary edema.
C. cardiomegaly and pulmonary fibrosis.
D. ventricular dysrhythmias and wheezing.
ANSWER: ________
RATIO:___________________________________________________________________________________________
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2. You are caring for a patient with a diagnosis of iron-deficiency anemia. Which clinical manifestations are you most likely
to observe when assessing this patient?
A. Convex nails, bright red gums, and alopecia
B. Brittle nails; smooth, shiny tongue; and cheilosis
C. Tenting of the skin, sunken eyes, and complaints of diarrhea
D. Pale pink tongue; dull, brittle hair; and blue mucous membranes
ANSWER: ________
RATIO:___________________________________________________________________________________________
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3.When providing teaching for the patient with iron-deficiency anemia who has been prescribed iron supplements, you
should include taking the iron with which beverage?
A. Milk
B. Ginger ale
C. Orange juice
D. Water
ANSWER: ________
RATIO:___________________________________________________________________________________________
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4.The primary pathophysiology underlying thalassemia is
A. erythropoietin deficiency.
B. abnormal hemoglobin synthesis.
C. autoimmunity.
D. S-shaped hemoglobin.
ANSWER: ________
RATIO:___________________________________________________________________________________________
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5. Which individual is at high risk for a cobalamin (vitamin B12) deficiency anemia?
A. A 47-year-old man who had a gastrectomy (removal of the stomach)
B. A 54-year-old man with a history of irritable bowel disease and ulcerative colitis
C. A 26-year-old woman who complains of heavy menstrual periods
D. A 15-year-old girl who is a vegetarian
ANSWER: ________
RATIO:___________________________________________________________________________________________
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6. You encourage the patient with cobalamin deficiency to seek treatment becaus e untreated pernicious anemia may
result in
A. death.
B. liver failure.
C. heart failure.
D. gastrectomy.
ANSWER: ________
RATIO:___________________________________________________________________________________________
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7. The Schilling test for pernicious anemia involves
A. administration of radioactive cobalamin and measuring its excretion in the urine.
B. blood cultures for organism identification.
C. the measurement of serum iron.
D. the administration of iron and blood assessment of total iron binding in 24 hours.
ANSWER: ________
RATIO:___________________________________________________________________________________________
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8. Which finding allows you to identify the patient's anemia as folic acid deficiency rather than cobalamin deficiency?
A. Loss of appetite
B. Lack of neuromuscular symptoms
C. Red tongue
D. Change in nail shape
ANSWER: ________
RATIO:___________________________________________________________________________________________
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9. Which foods should you encourage patients with folic acid deficiency to include in their daily food intake (select all tha t
apply)?
A. Ready-to-eat cereal
B. Wheat tortillas
C. Lentils
D. Strawberries
E. Potatoes
ANSWER: ________
RATIO:___________________________________________________________________________________________
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10. You are evaluating the laboratory data of the patient with suspected aplastic anemia. Which findings support this
diagnosis?
A. Reduced RBCs, reduced white blood cells (WBCs), and reduced platelets
B. Reduced RBCs, normal WBCs, and normal platelets
C. Normal RBCs, reduced WBCs, and reduced platelets
D. Elevated RBCs, increased WBCs, and increased platelets
ANSWER: ________
RATIO:___________________________________________________________________________________________
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RATIONALIZATION ACTIVITY (THIS WILL BE DONE DURING THE FACE-TO-FACE INTERACTION)
The instructor will now rationalize the answers to the students. You can now ask questions and debate among yourselves.
Write the correct answer and correct/additional ratio in the space provided.
1. ANSWER: ________
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RATIO:_______________________________________________________________________________________
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10. ANSWER: ________
RATIO:_______________________________________________________________________________________
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LESSON WRAP-UP (5 minutes)
You will now mark (encircle) the session you have finished today in the tracker below. This is simply a visual to help you
track how much work you have accomplished and how much work there is left to do.
You are done with the session! Let’s track your progress.
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AL Activity: Muddiest Point:
In today’s session, what was least clear to you?
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