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CHAPTER 26: ASSESSMENT AND vasospastic disorders, and

MANAGEMENT OF PATIENTS WITH congenital malformations


VASCULAR DISORDERS AND PROBLEMS ● Circulatory insufficiency of the
OF PERIPHERAL CIRCULATION extremities

SYSTEMIC AND PULMONARY


VASCULAR SYSTEM CIRCULATION

● Consist of two interdependent systems


○ Right side of the heart pumps
blood through the lungs to the
pulmonary circulation
○ Left side of the heart pumps
blood to all other body tissues
through the systemic
circulation
● Arteries and arterioles
● Capillaries
● Veins and venues
● Lymphatic vessels

FUNCTION OF THE VASCULAR SYSTEM

● Circulatory needs of tissues


● Blood flow
● Blood pressure
● Capillary filtration and reabsorption
● Hemodynamic resistance
● Peripheral vascular regulating GERONTOLOGIC CONSIDERATION
mechanisms
● Aging produces changes in the walls of
PATHOPHYSIOLOGY OF THE VASCULAR the blood vessels that affect the
SYSTEM transport of oxygen and nutrients to
the tissues
● Pump failure ● Changes causes vessels to stiffen and
○ Inadequate peripheral blood results
flow occurs when the heart’s ○ Increased peripheral resistance
pumping action becomes ○ Impaired blood flow
inefficient ○ Increased left ventricular
● Alterations in blood and lymphatic workload
vessels
○ Arteries can become damaged ASSESSMENT OF THE VASCULAR SYSTEM
or obstructed as a result of
atherosclerotic plaque or a ● Health history
thromboembolus (a blood clot ○ Intermittent claudication
that may become dislodged ■ Pain in the calf
from the vessel where it ■ Less commonly in the
originally formed), chemical or thigh or buttocks
mechanical trauma, infections ■ caused by narrowing or
or inflammatory processes, blockage in the main
artery taking blood to the ankle to the systolic BP in
your leg (femoral the arm; it is an objective
artery). indicator of arterial disease
■ This is due to that allows the examiner to
hardening of the quantify the degree of stenosis
arteries ○ ABI is determined by having
(atherosclerosis). the patient in supine position
■ The blockage means for 5 minutes. Then, an
that blood flow in the appropriate sized bp cuff
leg is reduced. (about 10 cm in adults) is
○ “Rest pain” applied to the patients ankle
○ Location of the pain above the malleolus. After
● Physical assessment identifying arterial signals of
○ Skin (cool, pale, pallor, rubor, the posterior tibial and dorsalis
loss of hair, brittle nails, dry or pedis, systolic pressure are
scaling skin, atrophy or obtained in both ankles while
ulceration) listening to the doppler signal
○ Pulses of each artery
○ Used when pulse cannot be
Buerger’s Disease reliably palpated
○ Handheld UTZ device that
detects blood flow, combined
with computation of ankle or
arm pressures
○ Signals are reflected by the
moving blood cells
○ Diagnostic technique helps
characterize the nature of
peripheral vascular disease
● Exercise testing
○ Used to determine how long a
● Vasoconstriction due to smoking patient can walk and to
● Common in males measure the ankle systolic BP
in response to walking
ASSESSING PERIPHERAL PULSE ● Duplex ultrasonography
○ Noninvasive
● Peripheral pulse locations ○ Used to determine the level or
○ Carotid extent venous disease as well
○ Brachial as the chronicity of the disease
○ Radial ○ Patients who undergo
○ Femoral abdominal vascular duplex utz
○ Popliteal should be advised to not eat or
○ Dorsalis pedis drink for at least 6 hours prior
○ Posterior tibial artery to the examination to decrease
bowel gas that can interfere
DIAGNOSTIC EVALUATION with the exam
● Computed tomography scanning
● Doppler ultrasound flow studies (CT scan)
○ Used to determine ankle ○ Provides cross-sectional images
-brachial index (ABI); ABI is of soft tissue and visualizes the
the ratio of the systolic BP in area of volume changes to an
extremity and the ➢ attainment/maintenance of
compartment where change tissue integrity
takes place ➢ Adherence to the self-care
● Angiography program
○ May be used to confirm the
diagnosis of occlusive arterial IMPROVING PERIPHERAL ARTERIAL
disease when surgery or other CIRCULATION
interventions are considered
● Magnetic resonance angiography ● Positioning strategies- body part below
○ Performed with MRI the level of the heart; do not elevate
○ Contraindicated in patients with legs
metal implants ● Exercise program and activities:
● Contrast phlebography walking, graded isometric exercises
(venography) ○ Consult primary provider
○ Involves injecting a radiopaque before engaging in an exercise
contrast agent to the venous routine
system ● Temperature, effects of heat and cold
○ If thrombus is present, x-ray ● Discourage use of nicotine; can cause
image reveals unfilled segment vasoconstriction
of vein ● Stress reduction
● Lymphoscintigraphy
○ Involves injection of a GOALS IN VASCULAR PROBLEMS
radioactively labeled colloid
subcutaneously in the second ● Vasodilation
interdigital space ● Maintain neutral position
○ The extremity is then exercised ● Do not elevate legs
to facilitate the uptake of the
colloid by the lymphatic system ARTERIAL DISORDERS

ASSESSMENT OF THE PATIENT ● Arteriosclerosis and atherosclerosis


WITH PERIPHERAL VASCULAR ● Peripheral artery disease
PROBLEMS ● Upper extremity arterial disease
● Aortoiliac disease
● Health history ● Aneurysms (thoracic, abdominal,
● Medications other)
● Risk factors ● Aortic dissection
● Signs and symptoms of arterial ● Arterial embolism and arterial
insufficiency thrombosis
● Claudication and rest pain ● Raynaud’s phenomenon and other
● Color changes acrosyndrome
● Weak or absent pulses
● Skin changes and skin breakdown PROGRESSION OF ATHEROSCLEROSIS

❖ Major goals include


➢ Increased arterial blood supply
➢ Decrease in venous congestion
➢ Promotion of vasodilation and
prevention of vascular
compression
➢ Relief of pain
RISK FACTORS FOR ATHEROSCLEROSIS ● Ischemic rest pain is usually worse at
AND PAD night and often wakes the patient

● Modifiable
○ Nicotine use
○ Diabetes
○ Hypertension
○ Hyperlipidemia
○ Diet
○ Stress
○ Sedentary lifestyle
○ C-reactive protein
○ Hyperhomocysteinemia
● Nonmodifiable
○ Increasing age
○ Genetics
● Prevention
○ Diet modification and exercise PHARMACOLOGIC THERAPY FOR PAD
to reduce blood lipid levels
○ HMG-CoA reductase inhibitor ● Phosphodiesterase III inhibitor (direct
(statins) as first line therapy in vasodilator that can inhibit platelet
patients with PAD aggregation)
● Endovascular Therapy ○ Cilostazol
○ Includes various procedures ● Antiplatelet agents (prevent
that use a puncture or small thromboembolic formation)
incision to place catheters ○ Aspirin
inside a blood vessel to repair ○ Clopidogrel
it ● Statins

PERIPHERAL ARTERIAL DISEASE (PAD) QUESTION #1


The nurse is teaching a patient
● Most common in men diagnosed with PAD. What should be included
● Most common cause of disability in the teaching plan?
● Legs are commonly affected A. Elevate the lower extremities
● Hallmark symptom is intermittent B. B. exercise is discouraged
claudication; described as aching, C. Keep the lower extremities in a
cramping, or inducing fatigue or neutral or dependent position
weakness; sensation of coldness or D. PAD should not cause pain
numbness in the extremities
● Skin and nail changes, ulceration,
gangrene, and muscle atrophy may be ANEURYSMS
evident
● Examination of peripheral pulses is an ● Localizee sac or dilation formed at a
important part of assessing PAD weak point in the wall of the artery
● Occurs with some degree of exercise or ● Classified by ts shape or form
activity ● Most common forms of aneurysm are
● Relieved with rest saccular and fusiform
● Pain is associated with critical ischemia ○ Saccular aneurysm projects
of the distal extremity and is described from only one side of the
as persistent aching, or boring (rest vessel
pain)
○ When an entire arterial ● The systolic is maintained at
segment becomes dilated, a approximately 90to 120 mmHg in
fusiform aneurysm develops order to maintain a mean arterial
pressure of 65 to 75 mmHg;
hydralazine is used for this purpose

RAYNAUD’S PHENOMENON

❖ Intermittent arterial vasoocclusion,


usually of the fingertips or toes.
● Raynaud's disease: primary or
idiopathic
● Raynaud's syndrome:
associated with other
underlying diseases such as
scleroderma.
THORACIC AORTIC ANEURYSM ❖ Manifestation: sudden vasoconstriction
results in color
● 70% cases are caused by changes,numbness,tingling and
atherosclerosis burning pain.
● Occur most frequently in ages 50 and ❖ Episodes brought on by a trigger such
70 years old as cold or stress
● Thoracic area is the most common site ❖ Occurs most frequently in young
of dissecting aneurysm women.
❖ Protect from cold and other triggers.
CLINICAL MANIFESTATION ❖ Avoid injury to hands or fingers.
● Depends how rapidly the aneurysm
dilates and how the pulsating mass
affects surrounding intra-thoracic
structures
● Some are asymptomatic
● Pain is the most prominent symptom;
usually constant and boring; may occur
when the patient is supine
● Other symptoms: dyspnea, dysphagia,
aphonia, cough
VENOUS DISORDERS
ASSESSMENT AND DIAGNOSTIC
FINDINGS ❖ Venous thromboembolism (VTE)
● Superficial veins of the neck, chest, or condition
arms become dilated ● DVT and PE
● Diagnosis includes x-ray, CT scan, ❖ Chronic venous insufficiency
MRA, or TEE postthrombotic syndrome
❖ Leg ulcers
MEDICAL MANAGEMENT ❖ Varicose Veins
● Control BP
● Correct risk factors Venous Thromboembolism
● Beta blockers have been the mainstay ❖ Pathophysiology
treatment ❖ Risk factors
❖ Endothelial damage
● Venous stasis ❖ Assess nutrition
● altered coagulation
❖ Manifestations
● Deep veins
● Superficial veins
❖ Preventive measures MEDICAL MANAGEMENT OF PATIENTS
● Early ambulation and leg WITH LEG ULCER
exercises
● Graduated compression ❖ Anti-Infective therapy depends on the
stockings infecting agent
● Intermittent pneumatic ❖ Oral antibiotics are usually prescribed
compression devices ❖ Compression therapy
● Subcutaneous heparin or ❖ Débridement of wound (debridement:
LMWH removal of dead tissue)
● Lifestyle changes ❖ Dressings
- Weight loss ❖ Other
- Smoking cessation
- Regular exercise NURSING INTERVENTIONS FOR THE
PATIENT WITH LEG ULCER
QUESTION #2
Which patient is at highest risk for venous ❖ Restoring skin integrity
thromboembolism? ● Cleansing wound; positioning;
A. A 50-year-old postoperative patient avoiding trauma; avoid heat
B. A 25-year-old patient with a central venous sources
catheter in place to treat septicemia ❖ Improving physical mobility
CA 71-year-old otherwise healthy older adult ● Physical activity initially
D. A pregnant 30-year-old woman due in 2 restricted to promote
weeks healing:gradual progression of
activity
B. A 25-year-old patient with a central venous ● Activity to promote blood flow;
catheter in place to treat septicemia encourage patient to move
Rationale: Some risk factors for venous about in bed and exercise
thromboembolism Include but are not limited upper extremities
to age older than 65 years, patients ● Diversional activities
undergoing surgery, central venous catheter ● Analgesic agents before
placement, septicemia, and pregnancy, The scheduled activities
client in this question with two risk factors is ❖ Promoting adequate nutrition
the 25-year-old with a central venous catheter ● Protein; Vitamins C and A;
in place to treat septicemia. All other patients Iron, Zinc
only have one risk factor.
VARICOSE VEINS

❖ Prevention
● Avoid activities that cause venous
ASSESSMENT FOR THE PATIENT WITH LEG stasis (wearing socks that are too
ULCER tight at the top or that leave marks
on the skin, crossing the legs at
History of the condition the thighs, and sitting or standing
❖ Assess pain, peripheral pulses, edema for long periods)
❖ Treatment depends on the type of ulcer ● Elevate the legs 3 to 6 inches
❖ Assess for presence of infection higher than heart level
● Encourage to walk 30 minutes ● Elevate affected area 3 to 6
each day if there are no inches above heart level
contraindications ● Warm, moist packs to site
● Wear graduated compression every 2 to 4 hours
stockings ● Educate regarding prevention
● Overweight patients should be of recurrence
encouraged to begin weight ● Reinforce education about skin
reduction plans and foot care

LYMPHATIC DISORDERS

❖ Lymphangitis: inflammation or
Infection of lymphatic channels the CHAPTER 28: ASSESSMENT OF
❖ Lymphadenitis: inflammation or HEMATOLOGIC FUNCTION AND
infection of the lymph nodes TREATMENT MODALITIES
❖ Lymphedema: tissue swelling related
to obstruction of lymphatic flow HEMATOLOGIC SYSTEM
● Primary: congenital
● Secondary: acquired ❖ The blood and the blood-forming sites,
obstruction including the c bone marrow and the
reticuloendothelial system (RES)
Question #3 ❖ Blood
Which of the following is an effective ● Plasma: fluid portion of blood
strategy used to promote lymphatic ● Blood cells: erythrocytes,
drainage and prevent edema In clients leukocytes, thrombocytes
with lymphedema? ❖ Hematopoiesis: complex process of the
A. Antibiotic therapy for 14 days formation and maturation of the blood
B. Constant elevation of the affected cells (RBCs)
extremity
C. Application of heat therapy twice per Question #1
day Is the following statement true or false?
D. Daily exposure to the sun Hematopoiesis is the complex process of the
formation and maturation of blood cells.
B. Constant elevation of the affected
extremity Red Blood Cells: Erythrocytes
Rationale: Constant elevation of the ❖ Types-Figure 28-2
affected extremity and observation for ● Hemoglobin- iron containing
complications are essential. After protein of RBCs;delivers
surgery, antibiotics may be prescribed oxygen to tissue
for 3 to 7 days. The patient is ● Reticulocytes- slightly
Instructed to avoid the application of immature RBCs, usually only
heating pads or exposure to sun to 1% of total circulating RBCs
prevent burns or trauma to the area. ❖ Erythropoiesis - process of formation of
RBCs
CELLULITIS ❖ Iron stores and metabolism Vitamin B
and folic acid
❖ S&S: localized swelling or redness, ❖ Destruction
fever, chills, sweating
❖ Treat with oral or IV antibiotics based White Blood Cells: Leukocytes
on severity ❖ Granulocytes (granulated WBC)
❖ Nursing Interventions
● Eosinophils:Involved in allergic Monocytes
reactions (neutralizes Eosinophils
histamine);digests foreign
proteins.
● Basophils:Involved in allergic RETICULOENDOTHELIAL SYSTEM
reactions (neutralizes
histamine); digests foreign
proteins. ❖ Histiocytes
● Neutrophils:Essential in ● Kupffer cells: a phagocytic cell
preventing or limiting bacterial which forms the lining of the
infection via phagocytosis. sinusoids of the liver and is
Bands: left shift
involved in the breakdown of
red blood cells
❖ Agranulocytes
● Peritoneal macrophages: major
● Monocytes:Enters tissue as
cell type of peritoneal cells that
macrophage, highly phagocytic
participate in multiple aspects
,especially against fungus;
of innate and acquired
immune surveillance
immunity in the peritoneal
● Lymphocytes: Integral
cavity.
component of immune system.
● Alveolar macrophages:also
- T cells and B cells
known as dust cells are a type
of white blood cells.
Platelets: Thrombocytes
❖ Spleen
❖ Thrombopoietin:
❖ Fibrin:filamentous protein; basis of
➔ NOTE: Macrophages give rise to tissue
thrombus and clot
histiocytes phagocytic cells that are
present in loose connective
Plasma and Plasma Proteins
tissue.These include Kupffer cells of the
❖ Albumin
liver, peritoneal tissue macrophages,
❖ Globulins
alveolar macrophages, and other
● Alpha
components of the RES.
● Beta
➔ NOTE: The spleen is the site of activity
● Gamma
for most macrophages. Most of the
❖ Impact on fluid balance
spleen (75%) is made of red pulp.

Which type of cells increase in number when


the patient is exposed to a bacterial Infection?
HEMOSTASIS
A. Erythrocyte
B. Eosinophil
❖ Assessment of Hematologic Health
C. Neutrophil
❖ Health history (refer to Chart 28-1)
D. Thrombocyte
❖ Physical assessment (refer to Table
E. Monocyte
28-2)
❖ Diagnostic evaluation
C.Neutrophil
❖ Hematologic studies
Rationale: The neutrophils are the mature,
❖ Bone marrow aspiration and biopsy
circulating white blood cells. When a bacterial
infection occurs, the neutrophils will increase in
Bone Marrow Aspiration
order to phagocytize the bacteria.
REMEMBER: Never Let Monkeys Eat Bananas
Neutrophils Basophils
Lymphocytes
➢ Correct administration
techniques per agency’s
policies and procedures
➢ Refer to Charts 28-3 and 28-4
Type Universal Universal
Recipient Donor

A B AB O

Anti-B Anti-A Neither Both A, B

A B A,B,O O

Transfusion Complications
❖ Febrile nonhemolytic reaction
Therapeutic Approaches ❖ Acute hemolytic reaction
❖ Splenectomy ❖ Allergic reaction
❖ Apheresis (refer to Table 28-3) ❖ transfusion-associated circulatory
❖ Hematopoietic stem cell overload
transplantation (HSCT) ❖ Bacterial contamination
❖ Phlebotomy ❖ Transfusion-related acute lung injury
❖ Blood component therapy ❖ Delayed hemolytic reaction
❖ Special preparations ❖ Disease acquisition — Chart 28-6
Question #3 ❖ Long-term transfusion therapy
Is the following statement true or false? Nursing Management Of Transfusion
Reactions
Petechiae are pinpoint hemorrhagic lesions ❖ Stop
often seen on a patient with thrombocytopenia. ❖ Assess
Answer: True ❖ Notify primary provider and Implement
Rationale: A thrombocyte is a cellular prescribed treatments. Continue to
component of blood involved in blood monitor.
coagulation. Thrombocytopenia can often ❖ Return blood
include petechiae, pinpoint hemorrhagic lesions ❖ Obtain any samples needed
often more prominent on the trunk or anterior ❖ document
aspects of the lower extremities. Transfusion Alternatives
❖ Refer to Chart 28-7
Blood and Blood Products ❖ Growth factors
❖ Donor requirements ❖ Erythropoietin
❖ Donation types-Refer to Table 28-4 ❖ Granulocyte colony-stimulating factor
➢ Directed ❖ Thrombopoietin
➢ Standard
➢ Autologous CHAPTER 29: MANAGEMENT OF PATIENTS
➢ Intraoperative blood salvages WITH NONMALIGNANT HEMATOLOGIC
➢ hemodilution DISORDERS
❖ Complications of donation
❖ Blood processing ANEMIA
Transfusion
❖ Common settings ❖ Lower than normal hemoglobin and
❖ Pretransfusion assessment fewer than normal circulating
❖ Patient education erythrocytes; a sign of an underlying
❖ Transfusion process disorder
❖ Hypoproliferative: defect in production ❖ Iron studies
of erythrocytes (RBCs) ❖ Vitamin B12
➢ Caused by iron, vitamin B12, ❖ Folate
or folate deficiency, decreased ❖ Haptoglobin and erythropoietin levels
erythropoietin production, ❖ Bone marrow aspiration
cancer, bone marrow damage Medical Management Of Anemias
❖ Hemolytic: excess destruction of ❖ Correct or control the cause
erythrocytes (RBCs) ❖ Transfusion of packed RBCs
➢ Caused by altered ❖ Treatment specific to type of anemia
erythropoiesis, or directed ➢ Dietary therapy
injury to the erythrocyte ➢ Iron or vitamin
➢ Refer to Chart 29-1 and Table supplementation: iron, folate,
29-1 B12
Manifestations of Anemias ➢ Transfusions
❖ Depends on the rapidity of the ➢ Immunosuppresive therapy
development of the anemia, duration ➢ other
of the anemia, metabolic requirements Hemolytic Anemias
of the patient, concurrent problems, ❖ Sickle cell disease
and contaminant features ❖ Thalassemia
❖ Fatigue, weakness, malaise ❖ Glucose-6-phosphate
❖ Pallor or jaundice dehydrogenasedeficiency
❖ Cardiac, GI, neurologic and respiratory ❖ Immune hemolytic anemia
symptoms ❖ Hereditary hemochromatosis
❖ Tongue changes ❖ Others (refer to Chart 29-1)
❖ Nail changes Hypoproliferative Anemias
❖ Angular cheilitis ❖ Iron deficiency anemia
❖ Pica (eating of things that are not ❖ Anemia in renal disease
edible) ❖ Anemia of inflammation
Question #1 ❖ Aplastic anemia
What type of anemia results from red blood ❖ Megaloblastic anemia
cell destruction? ➢ Folic acid deficiency
➢ Vitamin B12 deficiency
A. Iron deficiency Neutropenia
B. Hemolytic ❖ Decreased production or increaed
C. Hypoproliferative destruction of neutrophils
D. None of the above (<2000/mm3)
Answer: B. Hemolytic ❖ Incrased risk for infection: monitor
Rationale: Hemolytic anemia results from red closely
blood cell destruction. In hemolytic anemias, ❖ Absolute neutrophil count (ANC)
premature destruction of erythrocytes results ❖ Medical management: tretament
in the liberation of hemoglobin from the depends on the cause
erythrocytes into the plasma. The bilirubin ❖ Nursing management: patient
concentration rises, and increased erythrocyte education, preventing and managing
destruction leads to tissue hypoxia, which in complications
turn stimulates erythropietin production, ❖ Refer to Charts 29-5 and 29-7
reflected in an increased reticulocyte count. Lymphopenia
❖ Lymphocyte count less than 1500/mm3
Diagnostic Testing for Anemia ❖ Causes
❖ Hemoglobin and hematocrit ➢ Exposure to radiation
❖ Reticulocyte count ➢ Long-term use of
❖ RBC indices corticosteroids
➢ Infections ❖ Immune thrombocytopenic purpura
➢ Neoplasms (ITP)
➢ Alcohol abuse ❖ Platelet defects- refer to chart 29-9
Polycythemia ❖ Hemophilia
❖ Increased volume of RBCs ❖ Von willebrand disease
❖ Secondary polycythemia ❖ Refer to chart 29-6 and 29-8 table
➢ Excessive production of Pl29-3
erythropoietin from reduced
amounts of oxygen, cyanotic Assessment of the Patient with Anemia
heart disease, nonpathologic ❖ Health history and physical exam
conditions or neoplasms ❖ Laboratory data
❖ Presence of symptoms and impact of
❖ Medical management those symptoms on patients life;
➢ Treatment not needed if fatigue, weakness, malaise and pain
condition is mild ❖ Nutritional assessment
➢ Treat underlying cause ❖ Medications
➢ Therapeutic phlebotomy ❖ Cardiac and GI assessment
Question #2 ❖ Blood loss: menses, potential GI loss
Is the following statement true or false? ❖ Neurologic assessment

Polycythemia is a form of anemia. Collaborative Problems and Potential


Answer: False Complications of the Patient with Anemia
Rationale: Polycythemia is caused by ❖ Heart failure
excessive production of erythropoietin. ❖ Angina
Examples of conditions causing polycythemia ❖ Paresthesias
include smoking, obstructive sleep apnea, ❖ Confusion
chronic obstructive pulmonary disease, severe ❖ Injury related to falls
heart disease, living at high altitudes or ❖ Depressed mood
exposure to low levels of carbon monoxide.
Secondary polycythemia can result from Planning and Goals for the Patients with
neoplasms that stimulate erythropoietin Anemia
production. ❖ Major goals include decreased fatigue,
attainment or maintenance of
Bleeding Disorders #1 adequate tissue perfusion, compliance
with prescribed therapy, and absence
❖ Failure of hemostatic mechanisms of complications
❖ Causes
➢ Trauma Interventions for the Patient with Anemia
➢ Platelet abnormality ❖ Balance physical activities, exercise,
➢ Coagulation factor abnormality and rest
❖ Medical management: ❖ Maintain adequate nutrition
➢ Specific blood products ❖ Maintain adequate perfusion
❖ Nursing management: ❖ Patient education to promote
➢ Limit injury compliance with medications and
➢ Assess for bleeding nutrition
➢ Bleeding precautions ❖ Monitor VS and pulse oximetry
(95-100); provide supplemental
Bleeding Disorders #2 oxygen (1-2 L) as needed
❖ Monitor for potential complications
❖ Secondary thrombocytosis
❖ Thrombocytopenia
Assessment of the Patient with Sickle Cell ❖ 2 major forms of hemophilia-
Disease (DEPRANOCYTOSIS) hemophilia a or classic
❖ Health history and physical exam ❖ Hemophilia a: deficiency of factor
❖ Pain assessment vili, most common common type,
❖ Laboratory data: S-shaped incidence is 1 in 10,000 males
hemoglobin ❖ Hemophilia b or Christmas disease:
❖ Presence of symptoms and impact of deficiency of factor ix, incidence is 1
those symptoms on patients life; in 100,000 males
swelling, fever, pain
❖ Sickle cell crisis assessment Hemophilia
❖ Blood loss: menses, potential GI loss ❖ Early in the twentieth century, survival
❖ Cardiovascular and neurologic was normally to 11 years, by the
assessment 1970s it rose to 68 years median
● Adequate tissue perfusion survival. Infection with HIV from factor
- O2 stat gathered from pools of donors
Chronic Skin Ulcers of Sickle Cell decreased the life expectancy to 49
years in the 1980s, life expectancy is
improving because of tests now
available for HIV and hepatitis, better
screening of donors, and heat
treatment of some products.

Hemophilia (Medical Management)


❖ Before 1986, HIV infections from factor
transfusion gathered from many
Collaborative Problems and Potential donors was common. Factor safety has
Complications of the Patients with Sickle been increased with heat and chemical
Cell Disease treatment of factor concentrates, and
❖ Hypoxia, ischemia, infection recombinant DNA technologies for
❖ Dehydration making factor that does not use
❖ Cerebral vascular accident (CVA) donors.
❖ Anemia ❖ The body can produce inhibitors to
❖ Acute and chronic kidney disease factors vili and ix, making these
❖ Heart failure treatments less effective. One strategy
❖ Impotence is to suppress the immune system to
❖ Poor compliance show this response.
❖ Substance abuse

Interventions for the Patient with Sickle Hemophilia ( Assessment)


Cell Disease ❖ Skin bruising or ecchymoses
❖ Pain management ❖ Neurologic manifestations such as
❖ Manage fatigue pain, lack of sensation in a limb,
❖ Infection prevention paralysis
❖ Promote coping ❖ Musculoskeletal manifestation-
❖ Education of disease process such as joint swelling from bleeding
❖ Monitor of complications internally (hemarthrosis); may have
❖ Refer to chart 29-4 deformities of joints, causing mobility
problems
Hemophilia
❖ Hereditary bleeding disorder caused by Hemophilia (Diagnostic Tests)
a lack of coagulation factors
❖ Diagnostic tests- blood tests for ❖ Diagnostic tests- Bleeding time;
intrinsic factor vili, ix, xi, or xii prolonged due to dysfuntional
deficiency platelets; reduction in factor will
❖ Coagulation tests: partial ❖ Medical management
thromboplastin time may be prolonged, Desmopressin acetate(DDAVP)-given
others may be normal because the Intravenously or as a nasal spray
extrinsic system is not involved. Increases factor will and VWF
concentration; decreases bleeding time
Hemophilia (Nursing Intervention) But the effect is short-live;useful to
❖ Assess the client history and physical, control bleeding from a dental
family, or personal history excessive or procedure or surgery
delayed bleeding from minor skin ❖ Nursing interventions- same as
trauma, dental extractors, or hemophilia
hematuria. Acquired Coagulation Disorders
❖ Acute intervention focus is to stop ❖ Liver disease
bleeding. ❖ Vitamin K deficiency
❖ Administer coagulation factor; ❖ Complications of anticoagulant therapy
concentrate on time and for long ❖ Disseminated intravascular
enough time. coagulation(DID)
❖ Encourage bed rest for joint or major ❖ Thrombotic disorders
muscle bleed. ❖ Hyperhomocysteinemia
❖ Administer prescribed drugs for pain. ❖ Antithrombin deficiency
❖ Prevent airway compromise ❖ Protein C & S deficiency
❖ Assess for intracranial bleeding provide ❖ Activated protein C resistance and
referral to genetic counseling, factor V Leiden mutation
psychosocial counseling, and support. ❖ Acquired thrombophilia
❖ Instruct the client and family regarding ❖ Malignancy
prevention and early signs of bleeding,
home administration of factors, pain Disseminated Intravascular Coagulation
management and rehabilitation ❖ Not a disease but a sign of underlying
considerations. disorder
❖ Instruct the client to wear a medic ❖ Severity is variable; may be life
alert bracelet, participate in noncontact threatening
sports, and avoid injury from tools in ❖ Triggers may include sepsis, trauma,
the home. shock, cancer, abruptio placentae,
toxins and allergic reactions
Von Willebrand’s Disease ❖ Altered hemostasis mechanism causes
❖ Hereditary bleeding disorder caused by massive clotting in microcirculation. As
the deficiency of the Von Willebrand clotting factors are consumed, bleeding
coagulation protein (VWF) and platelet occurs. Symptoms are related to tissue
dysfunction; most common type of ischemia and bleeding
bleeding disorder (1 in 100), can range ❖ Laboratory tests
from mild to serve ❖ Treatment; treat underlying
❖ Autosomal dominant; occurs in both cause,correct tissue ischemia, replace
sexes fluids and electrolytes, maintain blood
❖ Assessment same as hemophilia pressure, replace coagulation factors ,
use heparin or LMWH

Von Willebrand’s Disease Assessment of the Patient with DIC


● Be aware of patients who at risk for ❖ infection, trauma , etc)
DIC and assess for signs and ❖ Heparin and antithrombin III (AT III)
symptoms of the condition to prevent
● Asses for signs and symptoms and ❖ Treat thrombosis 3)
progressions of thrombia and bleeding Epsilon-aminocaproic acid (AMICAR) to
inhibit fibrinolysis
Collaborative Problems and Potential Question number 3
Complications of the Patient with DIC Is the following statement true or false?
❖ Kidney injury
❖ Gangrene Disseminated intravascular coagulation is
❖ Pulmonary embolism or hemorrhage caused by alteration of normal hemostatic
❖ Acute respiratory distress syndrome mechanisms.
❖ Stroke Answer to Question number 3

Planning and Goals for the Patient with True


DIC
❖ Major goals may include maintenance Rationale; Normal hemostatic mechanisms are
of hemodynamic status, maintenance altered in DIC. The inflammatory response
of intact skin and oral mucosa, generated by the underlying disease initiates
maintenance of fluid balance, the process of inflammation and coagulation
maintenance of tissue perfusion, within the vasculature. Normal anticoagulation
enhanced coping, and absence of pathways are impaired and fibrinolysis is
complications suppressed allowing small clots to form. As
platelets and clotting factors are consumed by
Interventions for the Patient with DIC the microthrombi, coagulation falls, leading to
❖ Assessment and interventions should excessive clotting and bleeding.
target potential sites of organ damage
❖ Monitor and assess carefully
❖ Avoid trauma and procedures that
increase the risk of bleeding, including
activities that would increase

Interventions for the Patient with DIC


❖ Acute DIC is a medical emergency and
the nurse should do everything
possible to facilitate procedures for
appropriate testing
❖ Ongoing assessment for external and
internal bleeding
❖ Minimize damage to tissues to prevent
new sites of bleeding
❖ Administer blood products,
cryoprecipitate, and fresh frozen
plasma promptly and safely
❖ Instruct the client about the entity of
DIC and the rationale for seemingly
paradoxical therapies.

Medical Management for the Patient with


DIC
❖ Treat underlying cause- (malignancy,
medication
● You can donate blood after 90 days
or 3 months
● There are 13 clotting factors
● Neutrophil increases when there is a
bacterial infection
● Liver has the capability to regenerate
● You can only donate 450mL of blood
● If BT is prohibited to patient d/t
religion, provide options such as
plasma expanders
● There are 2 types of anemia:
hypoproliferative and hemolytic
● You should take iron with vitamin c
on an empty stomach to increase
absorption. You may take it with
orange juice
● In a child, use straw when giving
iron because it can cause teeth
staining
● Other name of sickle cell disease is
OTHER INFORMATION MENTIONED IN drepanocytosis; there is a decrease
THE LECTURE in globin which is responsible for a
cells shape;painful
● Venous Assessment ● Goal in sickle cell disease is
○ Pulse adequate tissue perfusion by oxygen
○ Capillary blood refill (should supplementation
be 1-3 secs) ● Normal lifespan of a cell is 120 days.
● Smoking promotes vasoconstriction But in sickle cell disease, cell last for
● Left ventricle supply oxygenated only 7-10 days
blood
● There is hardening and narrowing
happening in intermittent
claudication CLOTTING FACTORS
● Raynaud’s Phenomenon
○ Blood insufficiency in the
psalm and hands
FACTOR I: Fibrinogen
○ Common in females
FACTOR II: Prothrombin
○ Remember Elsa from Frozen.
Where does her ice power FACTOR III: Tissue thromboplastin
come from? In the hands. FACTOR IV: Calcium ions
● Stroke has no first aid. Once you see FACTOR V: Labile factor
early signs of stroke, immediately FACTOR VII: Stable factor
bring the patient to the nearest FACTOR VIII: Antihemophilic factor
hospital
FACTOR IX: Christmas factor
● Gangrene means tissue death
● The hallmark sign of PAD is FACTOR X: Stuart-Prower factor
intermittent claudication FACTOR XI: Plasma thromboplastin antecedent
● Debridement means removal of FACTOR XII: Hageman factor (contact factor)
tissue FACTOR XIII: Fibrin stabilizing factor
● Pressure ulcer medications
○ Calmoseptine for rashes or
redness; it is a
lotion/ointment
○ Mupirocin
(Bactroban/Bactifree- for
open wounds
○ Bazot powder- powerful

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