NUR 155: Care of Clients with Problems in

Oxygenation, Fluid and Electrolyte,

Infectious, Inflammatory and Immunologic
Response, Cellular Aberrations, Acute and
Chronic Lecture
Prepared by: Krys Raymond B. Lopez, RN, MD
 PATIENTS WITH OXYGEN-TRANSPORT
RELATED HEMATOLOGIC DISORDERS
At the end of the module, students will be able to:
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
regarding cause, incidence, clinical manifestations, management, and the
significance of preventive health care.
 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.
 Hematologic System
• 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.
 HEMATOLOGIC DISORDERS
• reflect a defect in the hematopoietic, hemostatic, or
reticuloendothelial system.
• 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.
 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.
 Pathology
• A decrease in the number of RBCs • Signs and Symptoms: Pallor,
can be traced to three different tachycardia, tachypnea,
conditions: irritability, fatigue, shortness of
1. impaired production of RBCs, breath
(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)
 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.
• Definitive diagnosis is
determined by bone marrow
aspiration. (shows
conversion of red bone
marrow to fatty bone
marrow)
 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
 Signs and Symptoms
• The manifestations of aplastic anemia are often insidious.
• Pancytopenia (deficiency of rbc, wbc and platelet)
• Typical complications are infection and the symptoms of anemia
(e.g., fatigue, pallor, dyspnea).
• Purpura (bruising) may develop later.
 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.
• Bone marrow transplant.
 Iron Deficiency Anemia
• is a common type of
anemia — a condition
in which blood lacks
adequate healthy red
blood cells.
• Red blood cells are
microcytic and
hypochromic
 Pathology

• 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
 Signs and Symptoms
• Same primary
symptoms of
anemia;
• If prolonged -
smooth, sore
tongue; brittle and
ridged nails; and
angular cheilosis
• Low hemoglobin,
hematocrit, mean
cellular volume
 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
• Do not take with milk or antacids because these items decrease
absorption.
• Instruct clients that stools will become dark in color
• To prevent staining the teeth with a liquid preparation, use a straw 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
 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.
• Macrocytic anemia
• 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)
 Pathology
• 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.
• Results from a deficiency of intrinsic factor(normally secreted by the gastric
mucosa) necessary for intestinal absorption of vitamin b12
• Gastric surgery or disease can result in a lack of intrinsic factor.
• Vitamin B12 is a nutrient found in some foods. (citrus fruits, dried fruits, green
leafy vegetables, liver, nuts, organ meats)
• The body needs this nutrient to make healthy red blood cells and to keep its
nervous system working properly.
• A lack of this protein leads to vitamin B12 deficiency.
 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.(required for rbc formation
and maturation)
• Common causes: dietary deficiency, malabsorption syndromes
• Pregnancy increases folic acid requirement
• Chronic alcoholism
 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
 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
 Medical Management
• 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.
• 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.
 Nursing Management
• 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
 Sickle Cell Anemia
• is a severe hemolytic anemia that
results from inheritance of the sickle
hemoglobin gene.
• Hemoglobin A is partly or completely
replaced by abnormal sickle
hemoglobin S
• Hemoglobin S is sensitive to changes in
the oxygen content of the RBC.
• This gene causes the hemoglobin
molecule to be defective. The sickle
hemoglobin (HbS) acquires a crystal-like
formation when exposed to low oxygen
tension.
 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.
 Pathology
• Insufficient oxygen causes the cells
to assume a sickle shape, and the
cells become rigid and clumped
together, obstructing capillary
blood flow.
• Situations that precipitate sickling:
Fever
Dehydration
Emotional or physical stress
Any condition that increases the
need for oxygen or alters the
transport of oxygen
 Pathology
• 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(hypersplenism), as
manifested by splenomegaly, and
reduced immune function.
 Sickle Crisis
Sickle cell crisis are acute
exacerbations of the disease, which
vary inconsiderably in severity and
frequency;
These include:
1. Vaso-occlusive crisis
-caused by stasis of blood with
clumping of cells in the micro-
circulation, ischemia, and infarction
Manifestations: Fever, painful
swelling of hands, feet, joints and
abdominal pain.
 Sickle Crisis
2. Splenic sequestration
-Caused by pooling and clumping of blood in the
spleen (hypersplenism)
Manifestation: profound anemia, hypovolemia
and shock

3. Hyperhemolytic Crisis
-Caused by an accelerated rate of red blood cell
destruction
Manifestations: Anemia, Jaundice and
reticulocytosis

4. Aplastic Crisis
- Caused by diminished production and
increased production of red blood cells,
triggered by viral infection or depletion of folic
acid.
Manifestations: Profound anemia and pallor
 Medical Management
• Maintain adequate hydration and blood flow through oral and IV administered
fluids. (5% in water (D5W) or dextrose 5% in 0.25 normal saline solution (3
L/m2/24 hours) is usually required for sickle crisis. )
REMEMBER: Without adequate hydration, pain will not be controlled
• Administer oxygen and blood transfusions as prescribed to increase tissue
perfusion
• Administer analgesics as prescribed (around the clock)
• Assist in assuming comfortable position
• Elevate the head of the bed
• Avoid putting strain on painful joints
• DIET: High-calorie, high-protein, folic acid supplementation
 Medical Management
• Administer antibiotics as prescribed
• Monitor for signs of complications (anemia, shock)
• Vaccination (Pneumococcal, meningococcal, annual flu)
due to susceptibility to infection secondary to functional
asplenia.
• Splenectomy may be necessary for clients who
experience recurrent splenic sequestration.
 Medical Management
• Peripheral blood stem cell transplant
• Hydroxyurea (Hydrea), a chemotherapy agent, has been shown 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
 Medical Management
• 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
 Medical Management
• 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.
 Thalassemia
• Autosomal recessive disorder characterized
by the reduced production of 1 of the globin
chains in the synthesis of hemoglobin.
• 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.
• 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
Hemoglobin Types of Beta-Thalassemia
1. Thalassemia Minor: Asymptomatic
silent carrier case
2. Thalassemia Trait:
Produces mild microcytic anemia
3. Thalassemia intermedia: Manifested
as splenomegaly and moderate to
severe anemia
4. Thalassemia Major: Results in severe
anemia requiring transfusion support
to sustain life (also known as Cooley’s
Anemia)
 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.
 Pathology
• 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.
 Signs and Symptoms

• Fatigue.
• Weakness
• Pale or yellowish skin,
• Slow growth
• Abdominal swelling,
• Dark urine Irritability
• Anorexia
• Microcytic hypochromic RBCs
 Signs and Symptoms

• Facial Bone deformities

 Signs and Symptoms

• Facial Bone deformities

• Skull X-Ray
Widening of the calvarium
“Hair-on-end” appearance
 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.
 Medical Management
• 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(deferasirox or deferoxamine), corticosteroids, antibacterial
combinations, vitamins, vaccines, antineoplastic agents, growth hormone
 Nursing Management
• If the patient had splenectomy, assess and report for any signs of
infection because of the risk of sepsis.
• Vaccination(Pneumococcal, Meningococcal, Annual Influenza)
• Provide genetic counseling to parents.
• Assess for severe anemia, splenomegaly or hepatomegaly with
abdominal enlargement, bleeding tendencies e.g., epistaxis, and
anorexia.
• Assess for anemia, jaundice, and splenomegaly, hemosiderosis caused
by increased intestinal absorption of iron.
 Glucose-6-Phosphate Dehydrogenase
Deficiency (G6PD)
• 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.
 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.
 Signs and Symptoms
• Often asymptomatic and have normal hemoglobin levels and
reticulocyte counts may develop pallor, jaundice, and hemoglobinuria
(hemoglobin in the urine).
 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.
 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.
 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).
 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.
 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.
 Medical Management
• 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
END