Cardiovascular and Hematologic Disorders Flashcards
(98 cards)
1
Q
Cardiac Output (CO) Formula
A
CO = heart rate x stroke volume
2
Q
Cardiac Output (CO)
A
The volume of blood ejected from the left ventricle (or the right ventricle) into the aorta (or pulmonary trunk) each minute.
3
Q
Stroke Volume
A
volume of blood ejected by the ventricle during each contraction.
4
Q
Fetal Circulation
A
Designed to ensure a high oxygen (O2) blood supply to the brain and myocardium (heart muscle)
5
Q
Fetal Lungs
A
Receive less than 10% of the blood volume; no gas exchange occurs in the fetal lungs.
6
Q
Right Atrium of Fetal Heart
A
chamber with the highest O2 concentration.
7
Q
Structural Changes of Fetal Heart
A
- Ductus Venosus: Constricts within 3-7 days after birth and becomes the ligamentum venosum.
- Foramen Ovale: Closes when pressure in the left atrium exceeds that of the right atrium; closes within the first few weeks after birth.
- Ductus Arteriosus: Connects the pulmonary artery to the aorta.
8
Q
What Causes Congenital Heart Disorders
A
- Failure of heart to progress beyond embryonic development
- Maternal rubella
- Heredity
9
Q
Classifications of Congenital Heart Disease: Traditional Classification
A
- Acyanotic Heart Disease: Heart defects that do not cause cyanosis (bluish discoloration of the skin due to low blood oxygen).
- Cyanotic Heart Disease: Heart defects that cause cyanosis due to mixing of oxygenated and deoxygenated blood.
10
Q
Classifications of Congenital Heart Disease: Hemodynamic Characteristics Classification
A
- Disorders with increased pulmonary blood flow: Conditions where there’s abnormally high blood flow to the lungs.
- Disorders with obstruction of blood flow: Conditions that block or restrict blood flow within the heart or its major vessels.
- Disorders with decreased pulmonary blood flow: Conditions where there is abnormally low blood flow to the lungs.
- Disorders with mixed blood flow: Conditions where oxygenated and deoxygenated blood mix, leading to cyanosis.
11
Q
Disorders with Increased Pulmonary Blood Flow Facts
A
disorders involve a left-to-right shunt, meaning blood flows from the left side of the heart (higher pressure) to the right side (lower pressure) through an abnormal opening or connection between the two circulatory systems or arteries. This results in increased blood flow to the lungs.
12
Q
Disorders with Increased Pulmonary Blood Flow
A
- Ventricular Septal Defect
- Atrial Septal Defect
- Patent Ductus Arteriosus
13
Q
Ventricular Septal Defect
A
- An opening in the septum (wall) separating the two ventricles of the heart.
- Leads to right ventricular hypertrophy (enlargement of the right ventricle) and increased pressure in the pulmonary artery (pulmonary hypertension).
14
Q
Ventricular Septal Defect S/Sx
A
Easy fatigability, a low, harsh, pansystolic murmur, and a palpable thrill/vibration.
15
Q
Ventricular Septal Defect MGT.
A
- Cardiac Catheterization or Transcatheter Closure (TCC) with occlusive device
- Open Heart Surgery reserved for VSDs in children younger than 2 years old to prevent the development of pulmonary artery hypertension and involves placing a Silastic or Dacron patch to close the defect. Post-operatively, vigilance is crucial for detecting and managing any arrhythmias (irregular heartbeats).
16
Q
Atrial Septal Defect
A
- abnormal communication (opening) between the two atria (upper chambers) of the heart.
- Results in right ventricular hypertrophy (enlargement of the right ventricle) and increased pressure in the pulmonary artery.
17
Q
Types of Atrial Septal Defect
A
- Ostium primum defect (ASD 1): Opening at the lower end of the atrial septum.
- Ostium secundum defect (ASD 2): Opening near the center of the atrial septum. This is the most common type of ASD.
- Sinus venosus defect: The superior portion of the atrial septum fails to form near the junction of the atrial wall with the superior vena cava.
18
Q
Atrial Septal Defect S/Sx
A
- Harsh systolic murmur
- Fixed splitting (of the second heart sound)
19
Q
Arial Septal Defect Management
A
Surgery
- between 1-3 years of age.
- involves placing a Silastic or Dacron patch to close the defect. Post-operative monitoring is crucial to watch for arrhythmias (irregular heartbeats).
- Cardiac Catheterization or Transcatheter Closure (TCC) with occlusive device
20
Q
Arial Septal Defect MGT.
A
- IV Indomethacin
- can be used to close the PDA
- careful monitoring is needed due to potential side effects, including reduced glomerular filtration rate, impaired platelet aggregation, and diminished gastrointestinal and cerebral blood flow - Ibuprofen
- Another medication option for PDA closure; it generally has fewer side effects than indomethacin - Cardiac Catheterization
- less invasive procedure
- insertion of Dacron coated stainless steel coils to occlude (close) the PDA
- done in infants between 6 months and 1 year of age - Ductal Ligation
- Surgical closure of the PDA
21
Q
Patent Ductus Arteriosus S/Sx
A
- Wide pulse pressure, low diastolic pressure
- a continuous, machine-like murmur
- heartbeat heard in the left 2nd or 3rd intercostal space.
22
Q
A
23
Q
Disorders with Obstruction of Blood Flow Facts
A
blood leaving the heart meets a narrowed or stenosed area, the pressure increases both within the ventricle (the heart chamber pumping the blood) and in the large artery located just before the obstruction.
24
Q
Disorders with Obstruction of Blood Flow
A
- Pulmonary Stenosis
- Aortic Stenosis
- Coarctation of the Aorta
25
Pulmonary Stenosis
- narrowing of the pulmonary valve or pulmonary artery, leading to decreased blood flow to the lungs causing increased pressure in the right ventricle, resulting in right ventricular hypertrophy (enlargement)
26
Pulmonary Stenosis S/Sx
- Cyanosis (bluish discoloration of the skin due to low oxygen)
- systolic ejection murmur
- thrill (vibration felt on the chest wall)
- widely split second heart sound ("lub b' dub").
27
Pulmonary Stenosis MGT.
Balloon angioplasty or valvotomy (surgical widening of the pulmonary valve).
28
Aortic Stenosis
- Narrowing (stricture) of the aortic valve leads to increased pressure and hypertrophy (enlargement) of the left ventricle, and can cause pulmonary edema (fluid buildup in the lungs)
- reduced blood flow through the aorta also decreases coronary artery blood flow
29
Aortic Stenosis S/Sx
- Rough systolic murmur
- thrill (vibration felt on the chest)
- chest pain
- In severe cases, faint pulses, hypotension (low blood pressure), tachycardia (rapid heart rate), and inability to suckle (in infants) may be present.
30
Aortic Stenosis MGT.
1. Beta-blocker or Calcium Channel Blocker
- used for stabilization, helping to manage symptoms and improve heart function.
2. Balloon Valvuloplasty
- widen the narrowed aortic valve.
3. Artificial Valve Replacement
- Surgical replacement of the diseased aortic valve with a prosthetic valve.
31
Coarctation of the Aorta
Narrowing of the aorta near the insertion of the ductus arteriosus.
32
Coarctation of the Aorta S/Sx
- Increased systemic circulation above the narrowed area
- Decreased systemic circulation below Stricture
- Left Ventricular Hypertrophy
33
Corarctation of the Aorta: S/Sx_Increased systemic circulation above the stricture
- Bounding radial and carotid pulse
- Moderately loud systolic murmur
- Increased blood pressure in the upper body
- Headache
- Dizziness
- Epistaxis (nosebleed)
34
Corarctation of the Aorta: S/Sx_Decreased Systemic Circulation Below the Stricture
- Absent femoral pulses
- Cool lower extremities
- Leg pain
35
Coarctation of the Aorta MGT.
1. Angioplasty
2. Surgery
- Resection (surgical removal) of the narrowed segment of the aorta with anastomosis (reconnection) of the remaining ends.
3. Digoxin and diuretics before surgery to reduce CHF if patient is 2 y.o.
36
Disorders with Decreased Pulmonary Blood Flow Facts
- Obstruction of Pulmonary Blood Flow preventing blood from exiting right side of the heart
- Increased Pressure on the right side of heart
- exceeds pressure on left side of the heart
- Desaturated and oxygen poor blood flows from right to left
37
Disorder with Decreased Pulmonary Blood Flow
Tetratology of Fallot
38
Tetratology of Fallot has 4 Associated Defects
1. Pulmonary Valve Stenosis
2. VSD (Ventricular Septal Defect)): A hole in the wall separating the ventricles (lower heart chambers).
3. Overriding of the Aorta: aorta receiving blood from both ventricles instead of just the left ventricle
4. Hypertrophy of the Right Ventricle
39
Tetratology Fallout S/Sx
- Polycythemia (increased red blood cell count)
- Severe dyspnea (shortness of breath)
- Growth restriction
- Clubbing of fingers
- Squatting position when resting
- Tet spells (episodes of cyanosis and hypoxia)
40
Tetratology MGT.
1. Blalock-Taussig Procedure
- palliative (temporary) surgical procedure to increase pulmonary blood flow.
- can even be performed prenatally
- No blood pressure (BP) measurements or venipunctures should be taken on the right arm after this procedure.
2. Management of Hypoxic Episodes
- Minimize hypoxic (low-oxygen) episodes by providing supplemental oxygen (O2)
- encouraging the squatting position, and using morphine or propranolol as needed.
3. Brock Procedure
- Complete repair of all the anomalies associated with Tetralogy of Fallot
- performed soon after birth.
41
Disorders With Mixed Blood Flow
- Fully Saturated Systemic Blood Flow Mixed with the desaturated pulmonary blood flow causing relative desaturation of the systemic blood flow
42
Disorders With Mixed Blood Flow: Transposition of the Great Arteries
- serious congenital heart defect where the aorta arises from the right ventricle instead of the left, and the pulmonary artery arises from the left ventricle instead of the right
- incompatible with life unless there's a communication (shunt) between both sides of the heart
- Such communications can be an atrial septal defect (ASD), a ventricular septal defect (VSD), or a patent ductus arteriosus (PDA).
43
Transposition of the Great Arteries S/Sx
Cyanotic from birth
44
Transposition of the Great Arteries MGT.
1. Open Heart Surgery
2. Pediatric Prostaglandins
- used to maintain the patency (openness) of the ductus arteriosus, ensuring some mixing of oxygenated and deoxygenated blood before surgery.
3. Balloon Atrial Septal Pull-Through
- less invasive procedure to create a larger opening between the atria (upper heart chambers), improving mixing of the blood
- used as a temporary measure before definitive surgical repair
45
Nursing Intervention For Child Undergoing Cardiac Catheterization: Preparation
- Complete Nursing History
- NPO (Nothing by Mouth) for 4 to 6 hrs
- Complete Assessment including calculating the body surface area
- Check for Allergies to iodine, contrast dyes, or shellfish
- Document Baseline Assessment of pedal pulses and pulse oximetry.
- Offer support to the child and family to alleviate anxiety.
- arrange a tour of the cardiac catheterization lab for the child
- Explain specific aspects of the procedure
- Demonstrate how the skin will be washed with brown soap and how the numbing medication will be administered
- Explain how the contrast dye will affect the patient and how the sedation will make the child feel
46
Nursing Intervention For Child Undergoing Cardiac Catheterization: After Procedure
- Monitor the patient using a cardiac monitor and pulse oximeter
- Monitor temperature and color distal (away from the heart) to the catheter insertion site, and check the pulse of the extremity distal to the insertion site
- Take vital signs every 15 minutes for the first hour, then hourly there after
- Monitor for trends and assess for possible hypotension (low blood pressure), tachycardia (rapid heart rate), and bradycardia (slow heart rate)
- Check the pressure dressing for bleeding
- Observe the insertion site for bleeding or hematoma
- Monitor fluid intake and output
- Educate the patient on observing for signs of inflammation and infection, monitoring for fever, avoiding strenuous activities for a few days, avoiding tub baths for 48–72 hours, and using acetaminophen or ibuprofen for discomfort
47
Cardiac Surgery: Preoperative Care
- Address Questions regarding the procedure
- Orient the child and parents/caregivers to the unfamiliar surroundings of the surgical area
- Check the chart to ensure that a signed informed consent form is present
- Verify the identity of the patient.
- Ensure that the side rails of the bed are securely fastened.
- Use appropriate restraints during transport.
- Check laboratory values for any systemic alterations
- Bathe and groom the child.
- Provide oral care
- Cleanse the operative site using the prescribed method.
- Administer antibiotics as ordered.
- Remove jewelry, makeup, and prosthetics as needed
- Check for any loose teeth.
- Provide preoperative teaching to reduce anxiety
- Prepare the child and family for postoperative procedures, such as nasogastric tube (NGT) placement, wound care, and the use of monitoring apparatus
- Administer preoperative sedation as prescribed.
48
Cardiac Surgery Postoperative Care
- Ensure the child is in a safe and comfortable position according to the physician's orders
- Perform all standing orders.
- Maintain proper handwashing technique.
- Assess the wound for bleeding and signs of infection
- Provide appropriate wound care
- Assess breath sounds.
- Perform neurologic checks.
- Monitor vital signs frequently.
- Administer fluids as needed.
- Monitor fluid loss through the chest tube (if present)
- Provide pharmacologic support as ordered, including sedatives and analgesics for comfort.
- Allow caregivers to visit as soon as possible
- Monitor electrolytes and supplement with infusions as ordered.
49
Rheumatic Fever
- inflammatory disease that occurs after an infection with Group A beta-hemolytic streptococcus (GABHS) pharyngitis (strep throat).
- involves the joints, skin, brain, central nervous system, heart, and subcutaneous tissue.
- most significant complication is rheumatic heart disease, which can cause damage to the cardiac valves.
50
Rheumatic Fever Risk Factors
- Inadequate health care
- Limited access to antibiotics
51
Rheumatic Fever S/Sx
1. Heart
- Endocarditis (inflammation of the inner lining of the heart)
- mitral and aortic valve stenosis (narrowing of the valves).
2. Joints
- Edema (swelling), inflammation
- effusion (fluid buildup) in the knees, elbows, hips, shoulders, and wrists (polyarthritis)
3. Skin
- Erythema marginatum (a distinctive rash)
- erythematous macules (red spots) with a clear center and wavy, demarcated borders on the trunk and proximal extremities
4. Neurologic
- Chorea (involuntary movements)
5. Other Manifestations
- Aschoff bodies (characteristic lesions in the heart)
- subcutaneous nodules (lumps under the skin)
- low-grade fever
- epistaxis (nosebleeds)
- abdominal pain
- arthralgia (joint pain)
- weakness
- fatigue
- pallor (pale skin)
- anorexia (loss of appetite)
- weight loss.
52
Aschoff bodies
small nodules in the heart muscle that are typical of rheumatic heart disease. They consist of swollen collagen, cells, and fibrils.
53
Criteria for Rheumatic Fever: Major Criteria
J.O.N.E.S C.A.F.E. P.A.L
J: Joint involvement (polyarthritis)
O: Carditis (inflammation of the heart; "O looks like a heart")
N: Subcutaneous nodules
E: Erythema marginatum
S: Sydenham chorea (involuntary movements)
54
Criteria for Rheumatic Fever: Minor Criteria C.A.F.E. P.A.L
C: Increased C-reactive protein (CRP)
A: Arthralgia (joint pain)
F: Fever
E: Elevated erythrocyte sedimentation rate (ESR)
P: Prolonged PR interval on electrocardiogram (ECG)
A: Anamnesis of rheumatic fever (previous history)
L: Leukocytosis (increased white blood cell count)
55
Criteria for Rheumatic Fever diagnosis
patient needs at least two major criteria, or one major and two minor criteria, to meet the diagnostic criteria for rheumatic fever.
56
Rheumatic Fever Nursing Intervention for Drug Therapy
- Drug levels must be maintained for 10–14 days.
- Prophylactic antibiotic therapy should continue for at least 5 years after the initial attack and is also recommended before any dental surgery.
57
Rheumatic Fever Pharmacological MGT.
1. Penicillin or Erythromycin
- antibiotics are used for the treatment of streptococcal infections, which are the underlying cause of rheumatic fever
2. Benzathine Penicillin IM
- long-acting penicillin injection is used for the treatment of streptococcal infections.
3. Corticosteroids
- used to reduce inflammation
- potential side effects include hirsutism (excessive hair growth), a "round moon face," and increased susceptibility to infections
4. Phenobarbital & Diazepam
- used to manage Sydenham's chorea (involuntary movements), a neurological manifestation of rheumatic fever
58
Nursing Interventions for Rheumatic Fever
- Encourage bed rest to reduce the workload on the heart during the acute phase of the illness.
- Gradually increase activity levels during recovery
- Handle painful joints gently and maintain functional alignment to prevent deformities.
- Use a pain rating scale to assess pain levels and medicate appropriately.
- Provide small, frequent feedings and encourage the intake of nutritious foods and fluids.
- Promote the development of quiet hobbies and collections to aid in relaxation and recovery.
- Provide emotional support to the patient and family
59
Nursing Intervention Outcome Evaluation for Rheumatic Fever
- Maintains cardiac output within acceptable limits.
- Consumes adequate calories for growth.
- Reports minimal pain.
- Maintains mobility of joints.
60
Kawasaki Disease
- Mucocutaneous Lymph Node Syndrome; A febrile (fever associated), multisystem disorder.
- Vasculitis: Vasculitis (inflammation of blood vessels) is the principal and life-threatening finding because it can lead to myocardial infarction (MI, heart attack) or aneurysm (weakening and bulging of a blood vessel).
- The cause of Kawasaki disease is currently unknown.
61
Criteria For Diagnosis of Kawasaki Disease
- Fever lasting 5 days or longer.
- Bilateral congestion of the ocular conjunctiva (white part of the eye).
- Changes in the mucous membranes of the upper respiratory tract (URT), including redness of the pharynx (throat) and lips, and a "strawberry tongue" (red tongue with prominent papillae).
- Changes in the peripheral extremities, including peripheral erythema (redness) and edema (swelling), and desquamation (peeling skin) of the palms and soles.
- A truncal (torso), polymorphous (varied appearance) rash
- Swelling of the cervical lymph nodes (neck glands).
- Extreme irritability.
- Joint stiffness and pain (arthralgia).
62
Nursing Intervention with Nursing Diagnosis for Kawasaki Disease
1. Risk for Ineffective Peripheral Tissue Perfusion
- Observe for signs of heart failure, including tachycardia (rapid heart rate), dyspnea (shortness of breath), rales (crackling sounds in the lungs), and edema (swelling).
- Inspect extremities for color and warmth.
- Check Capillary Refill
2. Pain r/t Swelling of Lymph Nodes & Inflammation of Joints
- Provide rocking and holding to comfort the child.
- Protect edematous (swollen) areas from pressure.
- Keep the child free from heavy blankets or clothing.
- Use a soft, padded toothbrush for brushing.
63
Kawasaki Disease Therapeutic Interventions
1. Aspirin
- administered in large doses initially, followed by a lower dose
- Ibuprofen can be used as an alternative
2. Abciximab
- platelet receptor inhibitor, may be used in some cases to reduce the risk of blood clots.
3. IVIG (Intravenous Gamma Globulin)
- common treatment to reduce inflammation and the risk of coronary artery aneurysms
4. Avoid Steroids as they may increase the risk of aneurysm formation.
64
Kawasaki Disease: Nursing Care
- Administer aspirin and closely monitor for signs of toxicity (e.g., bleeding, tinnitus).
- Provide supportive care, including measures to prevent dehydration and minimize cardiac complications.
- Closely monitor cardiac function.
- Administer intravenous gamma globulin (IVIG) and observe for allergic reactions and side effects.
- Minimize discomfort with cool baths, unscented lotions, and soft, loose clothing.
- Maintain a quiet environment and provide emotional support to the patient and family.
65
Salicylate Poisoning
- is a toxicity reaction when giving aspirin to children
- Severe Toxicity indicated by metabolic acidosis and seizures.
- Toxic Level for a 30lb Child is a 12 adult aspirin or 48 baby aspirin
66
S/Sx Salicylate Poisoning
- Tinnitus (ringing in the ears)
- Nausea/Vomiting
- Lethargy/Excitability
- Hyperventilation leading to respiratory alkalosis
67
Formula for Estimated Blood Volume
Child's Weight x Age Group Average Value
68
Blood Components
- 55% Plasma
- 1% White Blood Cells and Platelets
- 44% Red Blood Cells
69
Coagulation Process
1. Injury or rupture to a blood vessel.
2. Blood vessels around the wound constrict to reduce blood flow to the damaged area.
3. Activated platelets stick to the injury site.
4. Platelets become sticky and clump together to form a platelet plug.
5. Platelets and damaged tissue release clotting factors (e.g., Factor VIII).
6. A blood clotting mechanism forms fibrin, which acts like a mesh to stop the bleeding.
70
Anemias
reduction in the concentration of RBCs or hemoglobin
71
Anemia Classification
1. Iron Deficiency Anemia
2. Megaloblastic Anemia
3. Aplastic Anemia
4. Hemolytic Anemia
72
Iron Deficiency Anemia
- Commonly caused by gastrointestinal (GI) bleeding, menstruation, and malignancy
- Other causes include inadequate dietary intake, malabsorption, and increased demand.
73
Megaloblastic Anemia
- Folate Deficiency: Associated with alcoholism, malabsorption, pregnancy, and lactation.
- Pernicious Anemia: Lack of intrinsic factor in the stomach, which prevents the absorption of vitamin B12, reducing the formation of an adequate number of erythrocytes (red blood cells).
74
Aplastic Anemia
- Also known as hypoplastic anemia
- caused by bone marrow depression or destruction
- leads to leukopenia (low white blood cell count), thrombocytopenia (low platelet count), decreased erythrocytes (red blood cells), and decreased leukocytes (agranulocytosis).
75
Hemolytic Anemia
- Excessive or premature destruction of red blood cells (RBCs).
- Causes include sickle cell disease, thalassemia, glucose-6-phosphate dehydrogenase deficiency, antibody reactions, infection, and toxins.
76
Clinical Findings for Anemia: Subjective
- Patients may report fatigue, headache, paresthesias (numbness or tingling), and dyspnea (shortness of breath).
- A sore mouth is common, and bleeding gums and epistaxis (nosebleeds) are particularly associated with pernicious anemia and thrombocytopenic purpura (low platelet count).
77
Clinical Findings for Anemia: Objective
- Physical examination may reveal ankle edema (swelling), dry and pale mucous membranes, and pallor (pale skin).
- Exceptions include polycythemia vera and hemolytic anemia.
- Koilonychias (spoon-shaped fingernails) are seen in iron deficiency anemia (IDA), and a beefy red tongue (glossitis) is characteristic of megaloblastic anemia.
78
Therapeutic Interventions for Anemia
- Improve diet by including vitamin C, which enhances iron uptake.
- Administer supplements as needed, including iron, vitamin B12, and folic acid.
- Blood transfusion therapy may be necessary in severe cases.
- Provide oxygen as needed to address hypoxia (low oxygen levels).
- Use epoetin (erythropoietin) to stimulate bone marrow function and increase red blood cell production.
- Bone marrow transplant/ replacement for Aplastic Anemia
79
Sickle Cell Anemia
- Prescence of abnormally shaped RBCs
- Autosomal recessive inherited disorder
80
Clinical Findings for Sickle Cell Anemia
1. Sickle Turbidity Test
- finger-stick blood determines the presence of HbS (sickle hemoglobin).
2. Hemoglobin Electrophoresis:
- Confirms the diagnosis
- test measures different types of hemoglobin in red blood cells.
3. Vaso-occlusive Crisis
- painful episode
- most common type of crisis, but generally not life-threatening.
4. Sequestration Crisis
- large quantity of blood pools in the spleen, causing a drop in blood pressure (BP) and shock.
- an acute episode that typically occurs between 8 months and 5 years of age; it can be life-threatening.
5. Aplastic Crisis
- Diminished red blood cell (RBC) production due to infection.
- Profound anemia results from rapid destruction of RBCs combined with decreased production.
6. Hyperhemolytic Crisis
- Increased rate of destruction of RBCs
- rare complication associated with G-6-PD deficiency
7. Acute Chest Syndrome
- Pneumonia-like manifestations, chest pain, fever, cough, tachypnea (rapid breathing), wheezing, and hypoxia (low blood oxygen).
8. Brain Attack
- Cerebrovascular accident (stroke).
81
Sickle Cell Anemia S/Sx
- Fever
- Swelling of hands & feet
- Yellowed sclera (jaundice)
- Vomiting
- Enlarged spleen
- Protruding abdomen
- Acute back pain
- Enlarged liver
82
Sickle Cell Nursing Intervention
- O2 Therapy
- Bed Rest
- No Supplemental Iron
83
Sickle Cell MGT.
- Pain Relief: acetaminophen
- Adequate hydration
- Oxygenation
- Hydroxyurea to increase hgb
- Exchange transfusion
84
Thalassemia
Autosomal recessive disorder most common in individuals of Mediterranean descent
85
Thalassemia Classification
- Minor: Asymptomatic carrier state.
- Trait: Heterozygous; mild microcytic anemia.
- Intermedia: Splenomegaly (enlarged spleen); moderate to severe anemia.
- Major: Severe anemia; requires transfusions to sustain life.
86
Effects of Thalassemia to the body or system
Bone Marrow
- Increased facial mandibular growth
Skin - Bronze colored
Spleen - Splenomegaly (enlarged spleen)
Liver & gallbladder - Cirrhosis & cholelithiasis
Pancreas - Destruction of islet cells & DM (diabetes mellitus)
Heart - Failure from circulatory overload
87
Hemochromatosis
Excessive iron storage with resultant cellular damage.
88
Hemochromatosis and Management
1. Iron-chelating agents (e.g., deferoxamine)
- medications help remove excess iron from the body.
2. Splenectomy
- Surgical removal of the spleen to reduce the need for blood transfusions.
3. Prophylactic antibiotics
- Prevent infections by reducing the risk of overwhelming infection.
4. Bone marrow transplant
- potential curative treatment option.
89
Sickle Cell MGT.
- Use digitalis, diuretics, and a low-sodium diet to manage cardiovascular complications.
- Transfusion of packed red blood cells (RBCs) to address anemia.
- Administer deferoxamine to remove excess iron (Fe).
90
Nursing Interventions for Sickle Cell Anemia
- Teach the patient to avoid contact with individuals who have infections
- Ensure immunizations are up-to-date.
- Administer prophylactic antibiotics as ordered
- Monitor blood transfusions
- Administer chelating agents and folic acid as prescribed.
- avoid activities that increase the risk of fractures.
- Prepare the patient for a potential bone marrow transplant.
91
Hemophilia A
- Factor VIII Deficiency
- Sex linked recessive trait
- Partial thromboplastin time (PTT) is used to test for Hemophilia A.
92
Hemophilia A: S/Sx
Individuals with Hemophilia A may experience extremely bruised lower extremities even from minor bumps, and swollen and warm joints
93
Hemophilia A: MGT.
treatment may involve fresh whole blood, frozen plasma, or desmopressin.
94
Von Willebrand's Disease
- Inherited as an autosomal dominant trait.
- Angiohemophilia is characterized by a Factor VII deficiency, resulting in platelets being unable to aggregate properly.
95
Von Willebrand's Disease Clinical Manifestation
Epistaxis (nosebleeds) is a major problem, often accompanied by heavy menstrual flow.
96
Von Willebrand's Disease Management
blood transfusions and arginine desmopressin.
97
Christmas Disease
FACTS
- Also known as Hemophilia B or Factor IX deficiency
- It is a sex-linked recessive trait
TREATMENT
- replacing the defective clotting factor (Factor IX concentrates).
INTERVENTION
- Genetic counseling
98
Hemophilia C
FACTS
- characterized by Factor XI deficiency or thromboplastin antecedent deficiency.
- inherited as an autosomal recessive trait.
TREATMENT
- involves plasma infusions.
INTERVENTIONS
- Genetic Counseling
