Alterations in Hematologic Function

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ALTERATIONS IN HEMATOLOGIC FUNCTION

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HEMATOLOGY A&P

Hematologic Process:

• Involves continuous blood circulation through arteries, veins, and capillaries.

• The circulatory (vascular) system includes arteries, veins, and capillaries.

• Arteries carry oxygenated blood from the heart to the body.

• Veins return deoxygenated blood from the body to the heart.

• Capillaries are small blood vessels that exchange nutrients, oxygen, and waste between arteries and veins.

Key Components of Blood:

• Red Blood Cells (RBCs): Also known as erythrocytes.

  • Responsible for transporting oxygen to body tissues and collecting carbon dioxide for exhalation.

  • Have a lifespan of approximately 120 days.

• White Blood Cells (WBCs): Also known as granulocytes.

  • Crucial for the immune response, specifically in fighting infections.

• Platelets:

  • Play a key role in blood clotting.

  • Help form blood clots to stop bleeding after an injury.

• Plasma Cells:

  • Responsible for humoral immunity.

  • Secrete immunoglobulins (antibodies) to help fight infections.

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BLOOD CLOTS

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Anatomy & Physiology of Blood Clotting:

• Blood clotting is a normal physiological process that prevents excessive blood loss after injury.

• Platelets and fibrin form the clot, which has a gel-like consistency.

• Thrombus: A blood clot that forms in veins or arteries, potentially blocking blood flow.

• Arterial thrombosis occurs when a clot forms in an artery, affecting blood flow to organs like the heart or brain.

• Thromboembolism refers to a clot that travels (embolus), potentially causing life-threatening issues if it blocks essential blood flow.

• Clots in the lungs (pulmonary embolism, PE), heart (myocardial infarction, MI), or brain (ischemic stroke) can lead to severe health complications.

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METRIC

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Pathophysiology & Etiology:

• Clots form normally to respond to injury but can form abnormally in veins or arteries due to factors like ischemic stroke, deep vein thrombosis (DVT), and PE.

• Clots can travel to distant parts of the body, blocking blood flow and leading to complications.

Risk Factors/Incidence:

• Injury, especially trauma to veins, increases the risk of clotting.

• Immobility (e.g., bed rest post-surgery) slows blood flow, increasing clot risk.

• Oral contraceptives, pregnancy, and hormone replacement therapy elevate clotting risk, especially with estrogen.

• Smoking, a sedentary lifestyle, and certain medications can also increase the risk of blood clots.

Comorbidities:

• Cancer (lung, brain, pancreas, etc.) increases clotting risk, especially with treatment like chemotherapy.

• Atrial fibrillation (A-fib) is a significant comorbidity, increasing the risk of clot formation.

• Heart disease, lung disease, previous blood clots, and trauma also increase the likelihood of clot formation.

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Epidemiology:

• Venous thromboembolism affects about 900,000 people in the U.S. annually.

• Up to 100,000 deaths occur each year from blood clots, often without warning signs.

• Cancer-related clots are a leading cause of death in cancer patients.

Impact on Health:

• A PE can damage the lungs and other organs due to reduced oxygen.

• A clot causing an MI can severely damage the heart or even result in death.

• Stroke (ischemic) can lead to brain damage, paralysis, or death.

• The location and extent of the clot can significantly impact the patient’s overall health.

Considerations for Aging Adults:

• Risk of blood clots doubles every decade after age 40.

• Older adults may have slower mobility, making them more sedentary and prone to clot formation.

• Nursing homes can increase clot risk due to decreased movement and reduced circulation.

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Clinical Presentation of Blood Clogs:

• Pulmonary Embolism (PE):

  • Chest pain, tachycardia, shortness of breath, dizziness, hemoptysis.

• Myocardial Infarction (MI):

  • Chest pain, pain radiating down the left arm, shortness of breath, diaphoresis.

• Stroke:

  • Facial weakness, speech difficulty, visual issues, side weakness, seizures.

Diagnostic Testing:

• D-dimer test: Elevated levels suggest a clot, but can also be influenced by pregnancy or surgery.

• Ultrasound: Can evaluate blood flow in veins.

• CT Scan: Detailed imaging, useful for diagnosing PE or stroke.

• MRA: Non-invasive imaging for blood vessels, often with contrast for clearer images.

• V/Q Scan: Checks lung perfusion to detect PE.

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Role of the Nurse:

• Educate patients about medication (e.g., anticoagulants) and dietary changes.

• Advocate for early ambulation post-surgery to reduce clot risk.

• Monitor patients on anticoagulants closely for signs of bleeding and drug interactions.

• Provide support for older adults managing blood clot prevention and treatment.

Safety Considerations:

• Anticoagulants like warfarin (Coumadin) carry a risk of bleeding. Close monitoring (e.g., INR levels) is required.

• High-alert medications: Ensure proper dosing and avoid interactions.

• Vitamin K can reverse warfarin’s effects in cases of excessive bleeding or elevated INR.

Treatment & Therapies:

• Prevention: Anticoagulants, mobility, and early ambulation.

• Thrombolytics: Medications that dissolve clots that have already formed.

• Antiplatelet drugs (e.g., aspirin, Plavix) prevent clot formation by inhibiting platelet aggregation.

• Thrombectomy: Surgical removal of a clot when other therapies fail.

Patient Education:

• Warfarin: Consistent medication timing, diet monitoring (vitamin K intake), and regular INR checks.

• In cases of mechanical heart valves, a higher INR range is necessary to prevent clotting around the valve.

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BLOOD CLOTS

MEDS:

• Warfarin

• Enoxaparin/Heparin

• Clopidogrel

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DEEP VEIN THROMBOSIS (DVT)

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Definition:

• DVT is a blood clot that forms in one of the deep veins, commonly in the lower extremities, but it can also occur in the upper extremities or pelvis.

Common Symptoms:

• Pain, swelling, redness, and warmth in the affected extremity.

• Often occurs in the lower leg or thigh.

Anatomy and Physiology:

• DVT occurs in the deep veins, particularly in the legs.

• It is a type of venous thromboembolism (VTE) and is the third most common cause of death after stroke and heart attack.

• Can be asymptomatic but may dislodge, leading to a pulmonary embolism (PE), which is life-threatening.

Pathophysiology:

• Can be caused by damage to the blood vessel wall, blood flow turbulence (e.g., atrial fibrillation), or hypercoagulability (increased clotting).

• DVT may form when blood flow is reduced or when there is damage to the vein's wall.

Etiology:

• Caused by immobility, injury to a vein (e.g., after surgery or IV catheter insertion), dehydration, or genetic conditions like Factor V Leiden (FVL).

• Hospitalized patients, especially those with malignancy or heart failure, are at higher risk.

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Deep Veins of the Leg

• Normal

• Blood Flow

• Deep Vein Thrombosis

• Embolus

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Risk Factors/Incidence:

• Reduced mobility (e.g., bed rest, long flights, surgery).

• Cancer, sepsis, obesity, smoking, pregnancy, and increased age.

• Genetic factors (e.g., Factor V Leiden mutation) increase risk.

• Obesity places extra pressure on veins and reduces peripheral circulation, contributing to DVT risk.

Severity Stages of DVT:

• Provoked: Triggered by external factors like obesity or cancer.

• Unprovoked: Occurs without an obvious cause.

• Proximal: Above the knee; higher risk for complications like PE.

• Distal: Below the knee; lower risk but still dangerous.

Considerations for the Aging Adult:

• Aging increases the risk of DVT due to sedentary lifestyle, decreased circulation, and comorbidities.

• Elderly clients may have difficulty affording or obtaining medications, highlighting the need for care coordination.

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Clinical Presentation:

• Common in the lower extremities, with symptoms including pain, swelling, and redness.

• Physical exam findings: tenderness, warmth, dilated veins.

Laboratory Testing and Diagnostic Studies:

• Venous Doppler Ultrasound: Detects blood clots using ultrasound waves.

• Contrast Venography: Invasive but highly accurate for diagnosing DVT.

• D-dimer Test: Elevated levels can suggest a clot but are not specific for DVT.

• MRI: Can also detect DVT.

Role of the Nurse:

• Monitor for symptoms of worsening DVT or potential PE (e.g., chest pain, shortness of breath).

• Educate the patient on lifestyle changes, medication adherence, and anticoagulant safety.

• Ensure early mobilization post-surgery to prevent DVT.

• Provide emotional support, especially for older adults who may struggle with medication access.

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Prevention and Safety Considerations:

• Prevention: Early ambulation, use of sequential compression devices (SCDs), and compression stockings.

• Medications: Anticoagulants (e.g., enoxaparin, heparin) to prevent further clotting. Monitor for bleeding complications.

• PE Risk: A DVT can lead to PE, requiring early detection and intervention.

Environmental Factors:

• Half of all DVTs occur in hospitalized patients or those post-surgery. Preventive measures, including low molecular weight heparin (LMWH) injections, are common.

Treatment and Therapies:

• Anticoagulants: Medications like heparin, warfarin, and newer drugs (e.g., rivaroxaban, apixaban) to prevent clot formation.

• Thrombolytics: Medications to dissolve existing clots.

• Thrombectomy: Surgery to remove large clots when thrombolytics are not effective.

• Inferior Vena Cava (IVC) Filters: Rarely used for acute DVT, except in high-risk bleeding patients.

• Prophylaxis: SCDs, compression stockings, and early ambulation post-surgery.

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Client Education:

• Teach the patient to avoid prolonged immobility and to ambulate as soon as possible after surgery.

• Encourage leg exercises, wearing compression stockings, and smoking cessation.

• Educate about the effects of estrogen therapy, including its impact on DVT risk.

Monitoring and Follow-up:

• Regular lab checks for clients on anticoagulation therapy (e.g., INR for warfarin).

• Educate clients on recognizing signs of bleeding and the importance of consistent medication use.

• Follow up for recurrence of DVT or complications like PE.

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HEMORRAGE

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Definition:

• Hemorrhage refers to blood loss due to damaged blood vessels, occurring inside or outside the body. Its severity determines treatment protocols and impact on prognosis.

Pathophysiology:

• The average adult body circulates 4.5 to 5.5 liters of blood. Losing up to 14% of total blood volume can be tolerated without significant physical symptoms.

• As blood volume decreases, the body compensates by redirecting blood to vital organs (brain, heart, lungs).

• Major hemorrhages can occur from injuries to the brain, chest, abdominal cavity, and long bones.

Severity of Hemorrhage:

• Class I: Up to 15% blood loss.

• Class II: 15-30% blood loss, with symptoms like tachycardia, tachypnea, and pallor.

• Class III: 30-40% blood loss, with delayed capillary refill, hypotension, and confusion.

• Class IV: Greater than 40% blood loss, leading to shock, decreased urine output, absent peripheral pulses.

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Types of Hemorrhages:

• Intracranial Hemorrhages: Includes subdural, subarachnoid, epidural, intracerebral, and intraventricular bleeds.

• Pulmonary Hemorrhages: Hemothorax due to trauma, cancer, or clotting disorders.

Strokes:

• Ischemic stroke: Caused by blood clots or narrowed vessels.

• Hemorrhagic stroke: Caused by blood vessel rupture and blood leakage into the brain.

Etiology:

• Common causes include trauma, cancer, clotting disorders, alcohol use disorder, childbirth complications, and violence (e.g., gunshot wounds).

• High blood pressure, aneurysms, or trauma can also cause hemorrhages.

Risk Factors:

• Trauma, accidents, violence (e.g., gunshot wounds, stabbings), and certain medical conditions (postpartum hemorrhage, blood clotting disorders).

• Postpartum hemorrhage is common and occurs in about two-thirds of clients with no prior risk factors.

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Safety Considerations:

• Orthostatic hypotension: A drop in systolic BP greater than 20 mmHg when standing from a lying position, which can cause dizziness and lead to falls.

• Aging Adults:

  • Older adults are at higher risk for hemorrhage due to aging-related changes and comorbidities (e.g., diabetes, hypertension, stroke).

  • Medications like anticoagulants and NSAIDs increase the risk of bleeding. A bleeding risk assessment is crucial for older adults undergoing surgery.

Clinical Presentation:

• Symptoms of hemorrhage depend on the bleeding source and severity:

  • Signs of external bleeding: Nausea, dizziness, weakness, cool skin, tachycardia, confusion, chest pain, and hypotension.

  • Signs of internal bleeding: Hemoptysis (coughing up blood), hematemesis (vomiting blood), abdominal rigidity, or confusion.

  • Intracranial hemorrhage: Headaches, slurred speech, weakness/numbness, decreased consciousness, seizures, or difficulty walking.

  • Traumatic injury: Symptoms include diaphoresis, hypotension, tachycardia, and tachypnea.

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Diagnostic Studies:

• CBC: To evaluate red blood cells, hemoglobin, hematocrit, and platelets.

• Coagulation Studies: Prothrombin time (PT) and partial thromboplastin time (PTT) to assess clotting.

• Imaging:

  • CT scan for brain hemorrhage.

  • Chest X-ray, CT, and ultrasound for chest/abdominal bleeding.

  • Endoscopy for gastrointestinal bleeding.

Client Education:

• Set realistic rehabilitation goals post-hemorrhage, with the help of physical and occupational therapy.

• Advise gradual increases in activity to prevent fatigue, dizziness, and fainting.

• Monitor for signs of recurrence and educate on preventive measures, including lifestyle changes.

Treatments and Therapies:

• Fluid Resuscitation: IV fluids (normal saline or lactated Ringer’s) for volume replacement and blood pressure stabilization.

• Blood Products: Packed red blood cells, fresh frozen plasma, or platelets for hemodynamic stability.

• Controlling Bleeding: Depending on the site, may require surgical intervention or medications.

• Provide emotional support and ensure the client understands their treatment plan and prevention of further complications (e.g., lifestyle modifications, monitoring for recurrence).

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ANEMIA

Types:

• Iron Deficiency

• Vitamin Deficiency

• Sickle Cell

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Anatomy & Physiology:

• Most common form of anemia worldwide.

• Diagnosed when hemoglobin levels are significantly below normal (2 g/dL lower than average).

• Hemoglobin relies on iron to carry oxygen to tissues, and insufficient iron impairs oxygen delivery to organs.

Etiology:

• Caused by poor nutrition, gastrointestinal disorders, chronic diseases, blood loss, and parasitic infections.

Risk Factors:

• Poor absorption (e.g., celiac disease, gastric bypass surgery), blood loss (e.g., GI bleed), and excessive cow's milk consumption in children.

Risk Factors/Incidence:

• Pregnancy: Increased iron requirements during pregnancy can lead to iron deficiency.

• Age: Adults over 50 are at higher risk due to blood loss, especially gastrointestinal.

• Socioeconomic factors: Low-income populations, particularly children and females, are at greater risk.

Comorbidities:

• Autoimmune disorders (e.g., rheumatoid arthritis), inflammatory bowel diseases (e.g., Crohn’s, ulcerative colitis), and gastrointestinal cancer increase risk.

• Kidney and thyroid diseases also contribute to higher risk.

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IRON DEFICIENCY ANEMIA

Epidemiology:

• Affects about 25% of the global population; prevalence is lower in developed countries like the U.S.

• 10% of individuals of childbearing age (especially females) in the U.S. are affected, largely due to blood loss from menstruation.

• 9% of children aged 12-36 months in the U.S. have iron deficiency.

Safety Considerations:

• Prioritize airway, breathing, and circulation in clients with significant blood loss.

• Fatigue: Ensure clients can ambulate safely with support if needed (assistive devices like canes or walkers).

Considerations for Older Adults:

• Increased risk due to decreased mobility, pre-existing health conditions, and nutritional deficiencies.

Clinical Presentation:

• Symptoms: Shortness of breath, fatigue, dizziness, palpitations, headaches, chest pain, pallor.

• Observation: Nurses may notice pale skin due to reduced RBCs.

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IRON DEFICIENCY ANEMIA

Laboratory Testing and Diagnostic Studies:

• Tests:

  • Serum Ferritin: Low ferritin indicates low iron stores.

  • Total Iron Binding Capacity (TIBC): Measures transferrin's ability to bind to iron; increases when iron is low.

  • Reticulocyte Count: Assesses RBC production.

  • Bone Marrow Examination: Determines bone marrow function.

  • Stool Test: Checks for occult blood (GI bleeding).

  • Bidirectional Endoscopy: Assesses upper and lower GI tract for potential bleeding sources.

  • Colonoscopy and Ultrasound: Evaluates for GI bleeding, polyps, or uterine fibroids in cases of heavy menstrual bleeding.

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IRON DEFICIENCY ANEMIA

Role of the Nurse:

Client Education:

• Iron-Rich Foods:

  • Heme Iron: From animal sources (red meats, turkey, chicken, fatty fish like salmon).

  • Non-Heme Iron: From plant-based sources (fortified cereals, dark leafy greens, beans, lentils, tofu, raisins).

  • Vitamin C: Enhances absorption of non-heme iron (e.g., citrus fruits, tomatoes, berries).

Safety Considerations:

• Monitor for fatigue, dizziness, and signs of gastrointestinal bleeding (e.g., dark stools).

• Advise rest periods and limit strenuous activity if needed to avoid falls/injuries.

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IRON DEFICIENCY ANEMIA

Treatment and Therapies:

Primary Treatment:

• Oral Iron Supplements (e.g., ferrous sulfate) are commonly prescribed.

• Taken on an empty stomach for optimal absorption.

• Side effects: Nausea, constipation, dark stools.

• Response: Typically visible within 14 days, but treatment often continues for at least 3 months.

• Intravenous Iron: For clients who cannot tolerate oral supplements or need rapid absorption.

• Blood Transfusions: May be necessary for severe iron deficiency anemia.

Supplementation Guidelines:

• Avoid taking iron with antacids (antacids interfere with absorption).

• Iron may be taken with vitamin C (e.g., orange juice) to improve absorption.

• If nausea occurs with oral iron, it can be taken with food.

Special Considerations:

• Clients with cancer: Monitor for fatigue and advise appropriate rest.

• Monitor stools for blood and contact the provider if noticed.

• Encourage clients to follow up with providers for labs to ensure proper response to treatment.

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Definition:

• Vitamin deficiency anemia occurs when the body lacks sufficient vitamin B12 (cobalamin) and folate, leading to fewer healthy RBCs. This can result from either inadequate absorption or insufficient dietary intake.

Pathophysiology:

• Vitamin B12:

  • Water-soluble and primarily obtained from animal-based foods (e.g., dairy, eggs, red meat).

  • Requires intrinsic factor (produced in the stomach) for absorption in the terminal ileum.

  • Essential for synthesizing DNA, myelin, and fatty acids. Deficiency affects neurological and hematologic function.

  • Excess vitamin B12 is stored in the liver.

Etiology:

• Pernicious Anemia: Autoimmune condition where the body cannot produce intrinsic factor, preventing vitamin B12 absorption.

• Malabsorption: Conditions like gastric bypass surgery or diseases (e.g., celiac disease, Crohn's disease) can hinder vitamin B12 absorption.

• Nutritional Deficit: Strict vegan diets or inadequate food intake can lead to vitamin B12 deficiency.

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Etiology for Vitamin Deficiency

Autoimmune Etiology:

• Pernicious Anemia: The terminal ileum cannot absorb Vitamin B12.

Malabsorption:

• Lack of intrinsic factor needed for the absorption of Vitamin B12.

• History of gastric surgery.

• Excessive alcohol consumption.

• Medications (Antiseizure medications, metformin, proton pump inhibitors, & oral contraceptives).

Disease Processes:

• Any disease process (such as celiac disease, or Crohn's disease) that interferes with the absorption of Vitamin B12 can cause deficiency.

Nutritional Deficits:

• Clients who follow a strict vegan diet for a prolonged period of time.

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VITAMIN DEFICIENCY

Risk Factors/Incidence:

• Intrinsic Factor Deficiency: Common in pernicious anemia or after gastric surgery.

• Lifestyle Habits: Excessive alcohol consumption impairs B12 absorption.

• Medications:

  • Anti-seizure drugs, metformin (for diabetes), proton pump inhibitors (for acid reflux), and oral contraceptives can interfere with B12 absorption.

• Age: Individuals over 75 years are at higher risk due to slower metabolism and decreased absorption capacity.

Impact on Overall Health:

• Neurological Effects: Vitamin B12 deficiency can lead to progressive neurological symptoms such as neuropathy, ataxia, and subacute combined degeneration of the spinal cord, affecting mobility and activities.

• Psychological Effects: Cognitive impairments like memory loss, confusion, and irritability may develop.

• Physiological Effects: Untreated deficiency can result in long-term damage, including visual deficits.

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VITAMIN DEFICIENCY

Safety Considerations:

• Neurological Deficits: Watch for neuropathy (tingling, pain in extremities) and ataxia (coordination problems), which can increase fall risk.

• Visual Deficits: May cause difficulty with mobility and activities, also increasing fall risk.

• Mobility: Ensure safe ambulation, considering clients may need assistance due to visual and neurological deficits.

Considerations for Aging Adults:

• Age 75+: Aging adults have a higher risk of B12 deficiency due to slower metabolism and decreased ability to absorb nutrients.

Clinical Presentation:

• Common Symptoms: Similar to iron deficiency anemia, including fatigue, shortness of breath, headaches, pallor, and dizziness.

• Neurological Progression: If untreated, clients may experience difficulty walking, tingling or pain in extremities, confusion, forgetfulness, irritability, visual deficits, diarrhea, and weight loss.

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VITAMIN DEFICIENCY

Laboratory Testing and Diagnostic Studies:

• Blood Test:

  • CBC with peripheral smear: Can show macrocytic anemia (larger RBCs).

  • Serum B12 Level:

  • Normal B12 > 300 pg/mL

  • Deficient B12 < 200 pg/mL

  • Borderline deficiency: 200-300 pg/mL.

  • Folate Levels: A test for folate deficiency may be done to rule out other causes.

  • History & Physical: Comprehensive to identify potential causes like diet, medication, or medical history.

Role of the Nurse:

• Education: Teach the client about the causes, effects, and treatment of vitamin B12 deficiency.

• Dietary Recommendations: Discuss the importance of consuming vitamin B12-rich foods like red meats, chicken, fish, eggs, milk, and fortified cereals.

• Follow-Up: Emphasize the need for regular follow-up with the provider to monitor B12 levels and assess for complications.

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Safety Considerations:

• Neuropathy: Warn clients about peripheral neuropathy and its impact on daily activities and fall risk.

• Visual Problems: Address any visual deficits that might make mobility more difficult, increasing fall risk.

• Support: Suggest a backup plan for assistance (e.g., family, friends, neighbors) to help with mobility and ensure safety.

Client Education:

• Medications: Explain the importance of adhering to prescribed medications (e.g., B12 injections, nasal sprays, or oral supplements).

• Follow-Up: Educate about the need for follow-up B12 testing and lab work.

• Diet: Educate clients on fortified foods (e.g., cereals, plant-based milk) and encourage the inclusion of vitamin B12-rich foods in their diet.

Treatments and Therapies:

• Vitamin B12 Supplements:

  • Oral: Most common, may be given daily.

  • Intramuscular Injections: May be necessary for severe deficiencies or for life if the client cannot absorb oral B12.

  • Nasal Spray/Gel: Alternative for clients who prefer non-injection methods.

  • Dietary Changes: Include more lean meats, chicken, fish, dairy, eggs, and fortified cereals to prevent deficiency.

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Pathophysiology:

• Inherited Disorder: Results from a single gene mutation causing abnormal hemoglobin (HbS), which makes red blood cells (RBCs) sticky and rigid.

• Sickle Shape: The abnormal hemoglobin causes RBCs to form a sickle shape, leading to slow blood flow and blockage of blood vessels.

• Hypoxia and Pain: Blocked blood flow leads to hypoxia in tissues, causing severe pain (can last for hours to days), particularly in the abdomen, chest, and joints.

Etiology:

• Recessive Inheritance: Both parents must be carriers of the sickle cell trait for the child to be affected.

• Sickle Cell Trait: Individuals with the trait produce both normal and sickle-shaped hemoglobin but do not have disease symptoms; they can pass the gene to offspring.

Risk Factors/Incidence:

• Ethnicity: More common in individuals of African, Mediterranean, or Middle Eastern ancestry.

• Genetic Inheritance: Newborns with parents carrying the sickle cell gene are at risk.

• 1 in 13 Black newborns in the U.S. have the sickle cell trait.

Comorbidities:

• Stroke Risk: Due to sickle-shaped RBCs, there is a risk of blockage of blood flow to the brain, leading to stroke.

• Pregnancy Complications: Increased risk of blood clots, hypertension, miscarriage, premature birth, and low birth weight.

• Acute Chest Syndrome: A serious complication where sickle RBCs block blood flow to the lungs, causing difficulty breathing, fever, and chest pain.

• Organ Damage: Increased risk of kidney disease, pulmonary hypertension, leg ulcers, blindness, priapism, DVT, and gallstones.

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Sickle Cell Anemia

• Normal red blood cell

• Sickle cell

• Stiff and sticky

• Sickle cells get stuck in blood vessel

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Epidemiology:

• U.S. Prevalence: Affects over 100,000 individuals in the U.S.

• Global Impact: Approximately 75% of newborns with sickle cell disease are born in sub-Saharan Africa. By 2050, an estimated 400,000 newborns will be born with sickle cell anemia annually.

Impact on Overall Health:

• Early Onset: Symptoms often begin around 6 months of age.

• Vaso-Occlusive Crisis (VOC): A common painful episode caused by the blockage of blood flow, which can occur anywhere in the body.

• Chronic Pain: Frequent pain episodes interfere with daily activities and can lead to hospitalization for pain management.

• Fatigue: Due to rapid RBC breakdown (10-20 days vs. 120 days for normal RBCs), leading to anemia and reduced oxygen delivery.

• Psychosocial Impact: Anxiety, depression, and stress are common due to chronic pain and disease complications.

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Safety Considerations:

• Blindness: Due to damage to the blood vessels in the eyes, clients should see an ophthalmologist regularly.

• Iron Overload: Frequent blood transfusions can cause iron overload, which can damage organs like the heart and liver. Iron chelation therapy may be necessary.

• Infection Risk: Sickle cell anemia can damage the spleen, increasing susceptibility to infections. Vaccination and good hygiene are essential.

Considerations of the Aging Adult:

• Physical Challenges: Older adults with sickle cell anemia may experience multiorgan disease, frequent pain episodes, and difficulty with daily activities.

• Social Isolation: Chronic pain and physical limitations can lead to social withdrawal and isolation.

Clinical Presentation:

• Anemia: Due to the rapid breakdown of sickle RBCs, leading to decreased oxygen delivery.

• Pain Episodes: Common in various body parts due to the blockage of blood vessels.

• Swelling: Hands and feet may become swollen due to circulation blockage.

• Visual Deficits: Caused by blocked blood vessels in the eyes.

• Infections: Increased susceptibility due to spleen damage.

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Possible Complications:

• Brain: Thrombosis or hemorrhage causing paralysis, sensory deficits, or death.

• Eye: Hemorrhage, retinal detachment, blindness, retinopathy.

• Lung: Acute chest syndrome, pulmonary hypertension, pneumonia.

• Heart: Heart failure.

• Kidney: Hematuria, renal failure.

• Spleen: Splenic atrophy (autosplenectomy).

• Bones and joints: Hand-foot syndrome, osteonecrosis.

• Liver-gallbladder: Hepatomegaly, gallstones.

• Penis: Priapism.

• Skin: Stasis ulcers of hands, ankles, and feet.

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Laboratory Testing and Diagnostic Studies:

• Newborn Screening: A blood test for sickle cell is routinely performed on newborns in the U.S.

• Genetic Testing: Can identify carriers of the sickle cell trait and diagnose sickle cell disease.

• Amniotic Fluid Testing: Used to detect sickle cell disease in unborn children.

Risk of Infection:

• Infection Susceptibility: Due to spleen damage, clients are more prone to infections.

• Vaccination: Encourage clients to receive recommended vaccinations, including the flu vaccine and routine childhood vaccines.

Role of the Nurse:

• Hydration: Encourage clients to drink at least eight glasses of water daily to reduce the risk of dehydration, which can trigger a crisis.

• Pain Management: Assist in developing an individualized pain management plan.

• Mental Health Support: Refer clients to support groups or mental health professionals to cope with chronic disease and stress.

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Client Education:

• Hydration and Pain Control: Stress the importance of staying hydrated and understanding pain management strategies.

• Preventing Complications: Educate about the risk of stroke, organ failure, infection, and acute chest syndrome.

• Vaccinations: Ensure clients receive appropriate vaccines to prevent infections.

Treatments and Therapies:

Pain Management:

• NSAIDs: For mild pain.

• Opioids: Administered intravenously or subcutaneously for severe pain.

• Pain Reassessment: Should occur every 15-30 minutes.

• Hydration: IV fluids are essential to reduce the risk of dehydration and improve circulation.

• Oxygen Therapy: Administer if oxygen saturation falls below 95%.

• Blood Transfusions: Used to reduce complications and increase normal RBCs but carry risks of infection and iron overload.

• Hydroxyurea:

  • A medication used to decrease pain episodes and complications associated with sickle cell disease.

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Hydroxyurea

Classification:

• Ribonucleotide reductase inhibitor.

• Given for treatment of sickle cell anemia.

Action:

• An oral medication that reactivates the production of fetal hemoglobin, thereby helping to reduce sickle cell disease events such as pain crisis and acute chest syndrome.

Therapeutic Use:

• For those experiencing sickle cell pain and have three or more pain crises that are moderate to severe in a one-year period or that interfere with their daily life.

Adverse Effects:

• Can cause some nail discoloration, skin hyperpigmentation, and myelosuppression. There is also a risk of teratogenicity.

Client Teaching:

• Medications should not be crushed.

• Blood counts should be monitored every four weeks.

• Must take every day and can take three to six months to notice improvements.

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BLOOD CANCERS

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Anatomy and Physiology:

• Bone Marrow: Blood cells are produced in the bone marrow, where stem cells mature into red blood cells, white blood cells, or platelets.

• Lymphatic System: Lymphoma affects the lymphatic system, a key part of the immune system.

• Multiple Myeloma: Originates in plasma cells of the bone marrow, where abnormal cells multiply and crowd out healthy cells.

Lymphoma and Myeloma Overview:

• Lymphoma: A cancer that grows in lymphatic cells. Two main types:

  • Hodgkin Lymphoma (rarer)

  • Non-Hodgkin Lymphoma (more common)

• Multiple Myeloma: A cancer that originates in plasma cells of the bone marrow, often with an unknown causative factor.

Epidemiological and Etiological Risk Factors:

• Non-Hodgkin Lymphoma:

  • Causative factors: Exposure to workplace chemicals, tobacco smoke, UV rays, viruses like Hepatitis C, HIV, and Epstein-Barr.

  • Accounts for 3% of all cancers worldwide.

• Multiple Myeloma:

  • Etiology: Connection to chromosome 14; associated with obesity, alcohol abuse, and environmental factors (radiation, Agent Orange).

  • 1.8% of cancer diagnoses in the U.S. annually.

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HODGKIN vs NON-HODGKIN LYMPHOMA

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What is Multiple Myeloma

Facts:

• Multiple Myeloma is a Cancer of the Plasma Cells

• Inhibits the Growth of Red and White Blood Cells

• Healthy Bone Marrow: Normal amount of plasma cells and antibodies.

• 4 Year Overall Survival of 75%

• Multiple Myeloma: Abnormal plasma cells multiply and make abnormal antibodies.

• Second Most Common Blood Cancer

• 65: Sixty-Five is the Median Age of Diagnosis

• Affected Area: Bone, Kidney, Blood.

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Risk Factors/Incidence:

• Non-Hodgkin Lymphoma:

  • Survival rates have improved, with stage 1 diagnosis survival at 83.5% (2010–2016).

  • Modifiable risk factors: Obesity, vitamin deficiency, tobacco, alcohol, and radiation exposure.

  • Non-modifiable: Autoimmune diseases, age, gender, and family history.

• Hodgkin Lymphoma: Typically affects younger adults (20-39 years) and older adults (65+).

• Multiple Myeloma:

  • Predominantly affects males over the age of 70.

Epidemiology:

• Non-Hodgkin Lymphoma: Affects 509,600 new cases globally (2018).

• Estimated 77,200 new U.S. cases in 2020.

• Multiple Myeloma: Affects 1.8% of new cancer diagnoses annually in the U.S., predominantly in males aged 70+.

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Impact on Overall Health:

Psychosocial Impact:

• Cancer diagnosis can cause emotional distress, fatigue from treatments, and stress over financial concerns.

• May lead to challenges in continuing work or daily activities.

Safety Considerations:

• Fatigue may require home assistance or transportation support for treatment.

• Elderly individuals may need help with activities of daily living due to weakness.

Considerations of Aging Adults:

• Hodgkin Lymphoma affects adults over 65, while Multiple Myeloma predominantly affects men over 70.

• Both cancers can have severe impacts on older adults, requiring extra attention to mobility and care needs.

Clinical Presentation:

• Lymphoma:

• Swollen lymph nodes in neck, groin, or underarms.

• Other symptoms: Itchy skin, weight loss, fever, night sweats, shortness of breath, fatigue.

Multiple Myeloma:

• Anemia, bone pain, fatigue, elevated creatinine, weight loss, and hypercalcemia.

• Hypercalcemia symptoms: Increased thirst/urination, bone/abdominal pain, nausea/vomiting, and altered mental status.

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Laboratory Testing and Diagnostic Studies:

• Lymphoma:

• Biopsy of swollen lymph nodes.

• Imaging tests: MRI or CT scan.

• Bone marrow biopsy may be performed.

• Multiple Myeloma:

• Bone marrow biopsy or aspiration.

• CBC, plasma cell FISH, 24-hour urine for protein, BUN, and creatinine.

• CT scan for further evaluation.

Role of the Nurse:

• Physical and Emotional Support:

• Provide care to address both physical symptoms (fatigue, anemia) and emotional needs.

• Assist with managing pain and coping strategies for cancer treatment.

• Fatigue Management: Encourage rest and provide resources to manage fatigue caused by treatments like chemotherapy.

Client Education:

• Emotional Support: Guide the client to build a strong support system at home for emotional and practical help.

• Treatment Understanding: Explain the side effects of chemotherapy, radiation, and targeted therapies.

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Treatments and Therapies:

• Lymphoma:

• Chemotherapy: Kills fast-growing abnormal cells (IV or oral).

• Radiation Therapy: High-energy x-rays to target and kill cancer cells.

• Targeted Therapy: Drugs that specifically target cancer cells with minimal damage to healthy cells.

• Multiple Myeloma:

• Similar treatments as lymphoma, but chemotherapy often involves drugs like thalidomide, lenalidomide, or bortezomib.

• Stem cell transplant may be an option for some clients.

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ACUTE LYMPHOBLASTIC LEUKEMIA (ALL)

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Anatomy and Physiology:

• Bone Marrow: Produces immature blood stem cells, which mature into red blood cells (RBCs), white blood cells (WBCs), and platelets.

• Blood stem cells differentiate into lymphoid or myeloid stem cells.

• Lymphoid stem cells produce B lymphocytes (fight infection and make antibodies), T lymphocytes (help B lymphocytes), and Natural Killer (NK) cells (attack viruses and cancer cells).

• Excessive production of lymphocytes: The bone marrow makes too many B or T lymphocytes, outnumbering healthy WBCs, RBCs, and platelets.

• Results in impaired ability to fight infection and causes anemia and bleeding disorders.

Etiology:

• Cause is unknown, but possible contributing factors include:

  • Ionizing radiation

  • Benzene exposure

  • Previous chemotherapy or radiotherapy

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Normal Blood vs. Leukemia

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Risk Factors/Incidence:

• Past radiation or chemotherapy exposure.

• High environmental radiation exposure (e.g., nuclear accidents).

• Age: Most common in children (2-10 years old).

• Genetic disorders: Certain syndromes increase risk.

• Incidence: Approximately 4,000 new cases in the U.S. annually.

• Affects 3.3 cases per 100,000 children.

• 85% survival rate since the 1980s, with better prognosis in children than adults.

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Impact on Overall Health:

• Adults: Prognosis is less favorable compared to children, with more complicated treatment and recovery.

• Considerations of the Aging Adult: May require additional support for medical appointments and therapies.

• Likely to need more emotional support due to the disease’s severity and treatment side effects.

Clinical Presentation:

• Clinical Manifestations:

  • Fatigue, easy bruising.

  • Fever, weight loss.

  • Bone pain, stomach pain.

  • Frequent infections, petechiae.

  • Night sweats, shortness of breath.

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Diagnosis:

• Health history and physical examination to assess symptoms and exposures.

• CBC with differential and peripheral smear.

• Bone marrow aspiration and biopsy for confirmation.

• Blood chemistry tests may also be performed to evaluate organ function.

Role of the Nurse:

Infection Control:

• Emphasize the importance of good hand hygiene to prevent infections (clients are immunocompromised).

Fatigue Management:

• Encourage rest when fatigued.

Nutrition:

• Advise small, frequent meals if appetite is poor, focusing on protein-rich foods.

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Safety Considerations:

• Metastasis: ALL can spread to the spinal cord and brain, so be vigilant for symptoms of metastasis.

• Bleeding Risk: Monitor for bleeding: Use a soft toothbrush, electric razor, and assess for any signs of bruising or bleeding.

• Take precautions to reduce injury risk.

Client Education:

Managing Symptoms:

• Educate about common manifestations (e.g., bleeding, sore gums).

• Recommend a soft and bland food diet if the client experiences oral bleeding.

Rest and Fatigue:

• Remind clients to rest and take breaks as fatigue is a common symptom.

When to Seek Medical Help:

• Advise the client to contact their provider if symptoms worsen or if they experience new bleeding, fever, or infections.

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Treatments and Therapies:

• Chemotherapy: Primary treatment option for ALL, may be combined with stem cell transplant.

• Stem Cell Transplant: Involves collecting immature blood cells from the client or donor, followed by chemotherapy or radiation, and reinfusing the cells after treatment.

• Radiation Therapy: High-energy x-rays used to target and destroy cancer cells.

• Targeted Therapy: Uses drugs or substances that specifically target cancer cells, minimizing damage to healthy cells.

• Immunotherapy: Emerging treatment in clinical trials that helps the immune system fight cancer.

• ALL does not have a standard staging system but is classified based on treatment response:

  • Untreated: Manifestations are treated, and more than 5% blast cells are present in the bone marrow.

  • Remission: Less than 5% blast cells in the bone marrow, CBC is normal, and no signs of leukemia.

  • Recurrent: The cancer returns after being in remission.

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BLOOD TRANSFUSIONS

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Indications for Blood Transfusion:

• Administered when a client has insufficient amounts or components of blood.

Common Conditions Requiring Transfusions:

• Anemia

• Various cancers

• Sickle cell anemia

• Hypovolemic shock

Components of Blood:

• Plasma

• Platelets

• Red blood cells

Determination:

• The transfusion is ordered based on the client’s symptoms and hemoglobin levels.

Blood Compatibility:

• Blood Type Matching: The client’s blood type must be compatible with the donor’s blood type.

Blood Donation Process:

• Blood donors are thoroughly screened (medical history, medications, and travel history).

• The blood is labeled, dated, and stored in the blood bank until needed.

• Expired or unsafe blood is discarded.

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Risks of Blood Transfusion:

• Transfusion Reaction: The client’s immune system attacks foreign proteins in the donated blood cells.

• Infection Risks: Exposure to hepatitis B or hepatitis C (though screening minimizes risk).

• Transfusion-Related Circulatory Overload (TACO): Can occur if blood is transfused too rapidly and the heart cannot manage the load.

• Iron Overload: Frequent transfusions can lead to an iron overload in the body.

Refusal of Blood Transfusion:

• Client's Rights: Clients have the right to refuse a transfusion.

• They must be informed of the risks and benefits of declining a transfusion.

Religious Beliefs:

• Jehovah’s Witnesses typically refuse transfusions of whole blood and blood components (red blood cells, white blood cells, platelets).

• They may accept derivatives such as albumin, clotting factor concentrates, and immunoglobulins.

Cultural and Religious Sensitivity:

• Health providers should respect and verify religious and cultural beliefs before administering a transfusion.

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Nursing Process for Blood Transfusion:

• Informed Consent: Obtain signed consent from the client, indicating they understand the risks and consent to the transfusion.

• Pre-Transfusion Assessment:

  • Vital signs: Check temperature, heart rate, blood pressure.

  • Verification: Ensure the client’s name, the medical provider’s order, and blood type match.

• Administration: Blood transfusion is administered intravenously (IV).

• Monitor the client’s vital signs after 15 minutes of transfusion start and at intervals throughout the transfusion.

• Transfusion Time: Typically takes up to 3 hours, depending on the volume of blood being transfused.

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Blood Transfusion Reaction Manifestations:

Immediate Reactions:

• Fever, chills, breathing difficulties, and rash.

Hemolytic Transfusion Reaction:

• Occurs when the immune system destroys the transfused red blood cells.

• Action: Stop the transfusion immediately and notify the healthcare provider.

Signs of Reaction:

• Bruising or pain at the IV site.

• Fever and chills, dark or reddish urine, back pain.

Delayed Reactions:

• Rapid heartbeat, chest pain, wheezing, difficulty breathing.

• Headache, dizziness, rash, hives, itching, and vomiting.

Action in Cases of Reactions:

• In any of the above cases, the client should immediately notify the healthcare provider.

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Types of Reaction | Causes | Manifestations | Interventions

|-------------------|---------|----------------|-------------|
| Acute Reactions | | |
| Transfusion-associated circulatory overload (TACO) | Happens when a large volume is transfused over a short period of time. Those at greatest risk include infants and clients older than 70 years of age. | Tachycardia, jugular venous distention, increased blood pressure, dyspnea, orthopnea, cyanosis | Stop the transfusion; place the client in high Fowler position; notify the provider; administer oxygen |
| Acute hemolytic reaction | Occurs when a client receives blood components that are not compatible with their own. | Fever; chills; tachycardia; hypotension; dyspnea; pain in the chest, abdomen, back, or flank; hypotension; red-colored urine; shock | Stop the transfusion; call for rapid response; change the administration set and infuse 0.9% sodium chloride; treat for shock |
| Non-hemolytic febrile reaction | Antibodies attack the client’s leukocytes or platelets | Fever, chills, headache, nausea and vomiting | Stop the transfusion; notify the provider; change the administration set and infuse 0.9% sodium chloride; monitor vital signs; if prescribed, restart transfusion slowly. |
| Allergic reaction (mild) | Occurs when the client is allergic to something in the infusion | Pruritis, urticaria, wheezing, facial flushing | Stop the transfusion; notify the provider; infuse 0.9% sodium chloride to keep the vein open; monitor vital signs; if prescribed, restart transfusion slowly. |
| Anaphylaxis | Occurs when the client has antibodies to components in the donor blood | Hypotension, tachycardia, bronchospasm, urticaria, anxiety, nausea, vomiting, shock | Stop the transfusion; call for rapid response; notify the provider; infuse 0.9% sodium chloride; administer cardiopulmonary resuscitation if needed. |
| Transfusion-related acute lung injury (TRALI) | Occurs when the client has antibodies to WBCs in the donor blood | Pulmonary edema | Stop the transfusion; notify the provider; administer oxygen; provide respiratory support, such as mechanical ventilation. |
| Delayed Reactions | | |
| Delayed hemolytic transfusion reaction (delayed HTR) | Occurs days to weeks after the transfusion. | Occurs when there is an incompatibility between the RBC components (other than ABO type) between the client and the donor blood | Fever, malaise, jaundice, decreased hemoglobin levels | Monitor laboratory results, including hemoglobin, coagulation, and renal function. |
| Transfusion-associated graft-versus-host disease (TA-GVHD) | Rare but often fatal. | T-lymphocytes within the donor blood react with the client’s blood. | Fever; rash; watery diarrhea; low RBC, WBC, and platelet levels; increases in liver enzyme levels. | Monitor laboratory results, including liver function and CBC; manage symptoms; no effective therapy available. |