PE & CF

# Nursing: A Concept-Based Approach to Learning Volume One, Fourth Edition – Module 16 Perfusion

Exemplar 16.K – Pulmonary Embolism

Copyright

- Copyright © 2023, 2019, 2015 Pearson Education, Inc. All Rights Reserved

Learning Outcomes

  • 2.2 Promote education that supports client values and beliefs.

  • 3.1 Promote client engagement in achieving holistic well-being.

  • 4.2 Exercise nursing judgment to anticipate a status change in the complex client.

  • 5.2 Analyze assessment data.

  • 5.3 Implement plan of care according to assessment data.

  • 6.2 Anticipate needs of the complex client.

  • 6.3 Recognize client cues indicating a change in health status.

  • 11.2 Apply evidence-based knowledge when delivering education to clients, families, and communities.

- 11.3 Integrate Evidence-Based Practice (EBP) involving nursing care decisions.

Overview of Pulmonary Embolism

  • Definition: Pulmonary embolism (PE) is a blockage of pulmonary vascular blood flow due to the presence of an embolus.

    • Thromboembolism: When the embolus is a clot.

    • Embolus can be composed of:

    • Fat

    • Amniotic fluid during childbirth

    • Erroneous intravenous (IV) air injection

    • Other materials, such as a broken piece of IV catheter

    • Unrecognized and/or untreated Deep Vein Thrombosis (DVT) can lead to PE.

- Life-Threatening Nature: Approximately 50% of PE cases will result in loss of life within 2 hours if not treated.

Pathophysiology

  • Normal Physiology:

    • Venous blood typically enters the lungs from the right side of the heart.

    • Blood travels through pulmonary capillary beds located in the alveolar walls.

    • Gas exchange occurs in alveoli where O2 and CO2 swap; this process must be matched with ventilation for effective gas exchange.

  • V/Q Mismatch:

    • When ventilation (V) and perfusion (Q) are not synchronized, it is termed V/Q mismatch.

    • This triggers additional physiological responses:

      • Vasoconstriction: Occurs to increase pulmonary pressure.

      • If the condition remains uncorrected, it may develop into

      • Pulmonary hypertension leading to right-sided heart failure.

      • Systemic hypotension and reduced cardiac output can occur.

      • Dead Space: Refers to alveoli that are ventilated but not perfused with blood, leading to:

      • Decreased surfactant levels.

      • Increased risk for atelectasis (collapse of part or all of a lung).

  • PE Specifics:

    • A low alveolar oxygen level (partial pressure of oxygen, PO2) leads to constriction of alveolar capillaries.

    • This condition causes shunting of blood to functional regions of the lungs.

    • The impact on pulmonary arterial blood flow depends on the extent of occlusion:

    • A large pulmonary artery occlusion can cause sudden death.

    • A pulmonary infarct results from blocked blood flow, potentially causing lung scarring or fibrosis.

- A small segment occlusion may cause parenchymal injury but is not permanent.

Etiology of Pulmonary Embolism

  • Primary Cause: Thrombus from lower extremity veins is the leading cause of PE.

    • Example: If thrombi in the popliteal and iliofemoral veins break free, they may flow into progressively larger venous systems but become lodged in the narrow pulmonary arterial system, causing occlusion.

  • Less Common Causes:

    • Fat emboli, occurring less frequently.

- Importance of Etiology: Understanding the cause of a PE is essential for identifying risk factors that contribute to the condition.

Risk Factors for Pulmonary Embolism

  • General Risk Factors:

    • Venous stasis

    • Damage to blood vessel walls

    • Altered blood coagulation:

    • Includes inherited thrombophilias and cancers producing coagulation factors.

    • Prolonged immobility

    • Trauma, including hip or femur fractures.

    • Recent surgeries, particularly orthopedic, pelvic, or gynecologic.

    • Smoking cigarettes

    • Presence of coronary artery disease

    • History of myocardial infarction (MI) or heart failure

    • Obesity

    • Advanced age

    • Family history of DVT or PE

    • Certain racial/ethnic considerations: African Americans face a higher risk than other races; genetic disorders such as Factor V Leiden are more common among Caucasians.

    • Many of these risk factors align with those seen in DVT cases.

  • Female-Specific Risk Factors:

    • Use of estrogen, including in:

    • Oral contraceptives

    • Hormonal therapy.

    • The risk increases with concurrent smoking.

    • Pregnancy & childbirth can increase risk for amniotic embolism.

- Gynecological and pelvic surgeries can elevate exposure.

Prevention of Pulmonary Embolism

  • Primary Prevention: Focuses on DVT prevention.

    • In Hospital:

    • Administer anticoagulants before or after surgery for at-risk patients.

    • Use of compression stockings and/or pneumatic compression as dictated by facility policies.

    • Early patient ambulation is critical to reduce venous stasis.

    • When necessary, incorporate passive range of motion exercises.

    • Elevating legs during bed rest is advised; avoiding placing pillows behind knees.

    • While Traveling:

    • Take breaks from prolonged sitting to mobilize.

    • Flex ankles while seated.

    • Stay hydrated with plenty of fluids.

- Utilize support stockings.

Clinical Manifestations of Pulmonary Embolism

  • Dependence on Size/Location: Clinical symptoms of PE vary based on the emboli's size and location.

    • Smaller emboli may be asymptomatic, while larger cases can develop symptoms abruptly.

  • Common Symptoms:

    • Dyspnea (difficulty breathing)

    • Pleuritic chest pain

    • Syncope (loss of consciousness) and cyanosis in massive PE

    • Tachycardia (increased heart rate) and tachypnea (increased breathing rate)

    • Crackles and cardiac gallop on auscultation

    • Anxiety and a sense of impending doom

    • Low-grade fever

    • Coughing, sometimes associated with hemoptysis (coughing up blood)

  • Differential Diagnosis Difficulties:

    • Differentiating PE from myocardial infarction (MI) or pneumonia can be challenging.

  • Manifestations of Fat Emboli:

    • Sudden onset of cardiopulmonary and neurologic symptoms:

    • Dyspnea

    • Tachypnea

    • Tachycardia

    • Confusion or delirium and decreased level of consciousness

- Petechiae (small red or purple spots) on chest and arms.

Diagnostic Tests for Pulmonary Embolism

  • Initial Assessment Tools:

    • PERC (Pulmonary Embolism Rule-out Criteria), Modified Wells Criteria, or Geneva Criteria can help estimate the risk of PE.

    • No further testing is required if results indicate low probability; monitoring should continue.

  • Criteria Indicating Risk for PE:

    • Pulse greater than 99 beats per minute

    • SpO2 (peripheral capillary oxygen saturation) less than 95% on room air

    • History of DVT or PE

    • Presence of hemoptysis

    • Recent surgery or trauma

    • Cancer history

    • Unilateral lower extremity swelling and/or pain

  • Further Testing: If risk factors and symptoms are present, additional imaging tests are required:

    • CT chest with contrast

    • Chest X-ray

    • CT angiography

    • Lung scans (V/Q scans)

  • Laboratory Tests:

    • D-dimer levels

    • PTT for heparin therapy

    • PT/INR for warfarin treatment

    • Arterial Blood Gas analysis

- Other diagnostics that may include Electrocardiogram (EKG) and End-Tidal CO2 monitoring (ETCO2).

Collaborative Treatment Approaches

  • Supportive Measures:

    • Oxygen therapy to provide supplementary oxygen.

    • Analgesics for relieving severe pleuritic pain and anxiety.

    • Promoting pulmonary arterial dilation where necessary.

    • Monitoring pulmonary artery and wedge pressures through a Swan-Ganz catheter.

- Monitoring for dysrhythmias due to impaired gas exchange.

Pharmacologic Therapy

  • Preventative Anticoagulant Therapy: Offered to high-risk patients to prevent the occurrence of PE.

  • Acute PE Treatment: For treatment of acute PE, often includes:

    • IV Heparin Drip and Oral Warfarin administered concurrently.

    • Protamine sulfate is the antidote for heparin.

    • Vitamin K is the antidote for warfarin sodium.

    • A significant risk associated with anticoagulant therapy is bleeding.

    • Fibrinolytics: Used for treating massive PE and hypotension; these agents work to restore pulmonary blood flow by breaking down emboli, which results in:

    • Reduced pulmonary pressure.

    • Improvement in right-sided heart failure, provided no permanent damage has occurred.

- Contraindications include: intracranial disease, recent stroke, active bleeding, bleeding disorders, pregnancy, severe hypertension, recent surgery, or trauma.

Surgical Intervention

  • Indications for Surgery: Surgical interventions occur when pharmacologic treatments are ineffective or contraindicated.

    • Open embolectomy: A procedure to physically remove the embolus or clot.

- Venous screens/filters: Utilized prophylactically to prevent recurrent PE.

Lifespan Considerations

Children
  • Incidence: Pulmonary embolism is rare in children.

    • Any deaths related to PE often stem from underlying conditions causing the embolism.

    • Symptoms often go unrecognized or misdiagnosed; symptoms can be ambiguous.

    • Traditional risk prediction tools may not be valid for children, and standard D-dimer tests are not useful.

  • Risk Factors:

    • Obesity

    • Prolonged immobilization

    • Indwelling central lines

    • Cancer

    • Genetic disorders

    • History of prior PE or DVT.

  • Manifestations: Includes tachypnea, tachycardia, chest pain, shortness of breath, and cough.

  • Diagnostics: V/Q scans, CT, and CT Angiography may be utilized.

- Treatment: Unfractionated or low molecular weight heparin are typical therapeutic options.

Pregnant Women
  • Statistics: PE is the sixth leading cause of death among pregnant women.

    • Diagnosis is complicated due to overlapping symptoms with normal pregnancy effects.

  • Diagnosis:

    • DVT and PE are evaluated with bilateral venous compression ultrasound of the lower extremities.

    • For PE without DVT, initial chest X-ray is performed; if normal, then a V/Q scan is used, followed by a CT pulmonary angiography if the V/Q scan is inconclusive.

    • If abnormal, CT pulmonary angiography is performed directly.

  • Treatment:

    • Unfractionated or low molecular weight heparin is administered, while warfarin is contraindicated due to potential harm to the fetus.

    • If heparin is contraindicated, may consider the insertion of a vena cava filter.

- Administration of IV heparin may be warranted close to delivery, with a recommendation to continue anticoagulants for at least six weeks postpartum.

Nursing Process in Care of Patients with PE

Primary Role of Nursing
  • Focus on Prevention: Includes preventing DVTs by identifying modifiable risk factors.

    • Ambulate patients following acute illness or surgery.

    • Utilize compression stockings and/or sequential compression devices (SCDs).

    • Encourage adherence to preventative anticoagulation therapy.

    • Recommend leg exercises: calf raises, muscle pumps, and both active and passive range of motion.

  • Patient Education:

    • Explain the risks associated with immobility.

    • Teach proper use of compression stockings and adherence to protocols.

    • Discuss smoking cessation programs.

- Explore alternatives to oral contraceptives, and discourage leg crossing.

Assessment
  • Observation and Interview: Collect data regarding chest pain (onset, severity, and precipitating factors), shortness of breath, recent hospitalization or surgical history, current medications, and use criteria tools such as PERC and Wells Criteria.

  • Physical Examination: Assess:

    • Level of consciousness (LOC), vital signs, and respiratory rate.

    • Skin color, temperature, and moisture levels.

    • Presence of jaundice or peripheral edema.

- Heart and breath sounds, as well as jugular vein distention (JVD).

Nursing Diagnoses
  • Potential Nursing Diagnoses:

    • Compromised gas exchange

    • Decreased cardiac output

    • Inadequate protection

    • Acute pain

    • Anxiety

  • Planning Goals/Outcomes: Nursing goals may encompass that the patient will:

    • Maintain oxygen saturation levels greater than 94%.

    • Verbally express fears.

    • Achieve pain relief.

    • Exhibit adequate tissue perfusion.

- Keep vital signs within normal ranges.

Implementation – Nursing Interventions
  • Compensating for Gas Exchange Issues:

    • Position patients in Fowler's or high-Fowler's position.

    • Ensure bed rest is maintained.

    • Repeatedly assess the respiratory status.

    • Monitor arterial blood gas results.

  • Preserving Cardiac Output:

    • Educate patients on symptom reporting.

    • Regularly assess skin color and temperature.

    • Auscultate heart sounds every 2 to 4 hours.

    • Monitor cardiac rhythm.

    • Administer vasopressors and relevant medications.

    • Monitor for changes in pulmonary arterial pressures, neck vein distention, and peripheral edema.

- Ensure maintenance of IV and arterial access sites.

Implementation – Safety Measures
  • Promoting Safety:

    • Identify possible drug interactions and contraindications.

    • Maintain adequate fluid intake and assess for overt or covert signs of bleeding.

    • Timely report coagulation study results that are outside desired parameters.

    • Keep antidotes (protamine sulfate for heparin and vitamin K for warfarin) readily available.

  • Relieving Anxiety:

    • Assess patients' anxiety levels.

    • Clearly explain all procedures and treatments to patients.

    • Reduce environmental stimuli where possible.

    • Encourage family support and remain with the patient as much as possible.

- Administer analgesics to help manage pain and anxiety.

Evaluation
  • Expected Outcomes: Successful outcomes may include:

    • Patient maintains adequate tissue perfusion.

    • Pain is controlled adequately.

    • Patient maintains effective airway clearance.

- Healthcare providers possess necessary tools and treatments readily available for patients at high risk of further PE.

Cystic Fibrosis

Overview
  • Cystic Fibrosis (CF): An inherited disorder that affects secretory glands, particularly those responsible for secreting:

    • Mucus

    • Digestive enzymes

    • Sweat

    • Influences multiple organs: lungs, sinuses, digestive organs, reproductive organs.

  • Prevalence: More common among Caucasians (occurring in approximately 30,000 individuals in the U.S. and around 70,000 globally).

- Annually, ~1,000 new cases diagnosed; 75% diagnosed before the age of 2.

Pathophysiology
  • Mutations: CF is a result of mutations in the CFTR gene, causing dysfunction/absence of the CFTR protein, which governs chloride ion movement into and out of body cells.

    • This mutation impacts water and salt movement, leading to the production of thick, sticky mucus which obstructs ducts and passageways throughout the organism.

    • Thick mucus creates an environment conducive to bacterial growth.

- Immune Response: The body sends white blood cells (WBCs) to localized infections, which release sticky substances into the mucus, worsening obstruction, inflammation, and infection.

Etiology
  • Genetics: CF is an autosomal recessive disorder—patients inherit an abnormal gene from both parents.

    • Carrier Status: Carriers of the CF gene typically do not show symptoms.

    • Inheritance Probabilities:

    • 25% chance that a child receives both abnormal genes and expresses CF.

    • 50% chance that a child receives one abnormal gene, becoming a carrier.

    • 25% chance of not inheriting the abnormal gene, resulting in neither carrying nor exhibiting traits of CF.

  • Prevention: Genetic screening to determine carrier status is advised under the following conditions:

    • Individual has personal or family history of CF.

    • Partner has history of CF.

- About 90% of faulty CFTR genes can be detected through carrier screening.

Clinical Manifestations of Cystic Fibrosis
  • Key Characteristic: Elevated chloride levels in sweat serve as a hallmark indicator of CF.

- Chronic Effects: Numerous chronic manifestations lead to long-term consequences affecting various organ systems.

Collaboration in Treatment
  • Collaborative efforts may include a multidisciplinary team:

    • Primary healthcare provider

    • Respiratory specialists

    • Gastrointestinal (GI) and nutritional specialists

    • Reproductive specialists

- Obstetricians

Diagnostic Tests for Cystic Fibrosis
  • Prenatal Screening: If a mother has an abnormal CFTR gene, the father is also tested; if both are carriers, fetal testing may be conducted via:

    • Amniocentesis

    • Chorionic villus sampling (CVS).

  • Newborn Screening: Blood tests are performed at birth in the U.S. to detect immunoreactive trypsinogen. If positive, a sweat test is conducted to confirm CF.

- Older Children/Adults: Screening may be warranted for individuals exhibiting symptoms such as bronchiectasis, chronic lung or sinus infections, nasal polyps, pancreatitis, or male infertility.

Surgical Interventions
  • Surgical needs will vary per the patient's requirements:

    • Common Procedures:

    • Removal of nasal polyps to enhance breathing.

    • Insertion of feeding tubes for additional nutrient administration.

    • Bowel surgery might be necessary for obstructions.

- Lung transplantation could be indicated for patients with life-threatening pulmonary complications or persistent infections resistant to antibiotics.

Pharmacologic Therapy
  • Therapeutic Options:

    • Bronchodilators: Aimed at opening airways for patients with a milder form of CF.

    • Mucolytics: Help break up viscous mucus.

    • Antibiotics: Such as aminoglycosides and monobactams can be administered intravenously or via inhalation.

    • CFTR Modulators: A relatively new class of drugs which can be expensive and targeted towards the defective CFTR protein to reduce mucus viscosity.

    • Vaccination: It's essential to stay current with vaccinations like flu, measles, and pertussis (inactivated versions preferred).

    • Anti-inflammatory Drugs: Use of drugs aimed at reducing lung inflammation.

- Digestive Support: Pancreatic enzyme replacements taken orally are crucial and dissolve in the small intestine.

Nonpharmacologic Therapy
  • Focus: Aimed at alleviating complications and symptoms associated with CF.

    • Airway Clearance Techniques:

    • Techniques including coughing and huffing.

    • Chest Physiotherapy: Involving percussion, vibration, and deep breathing, typically used alongside postural drainage to enhance effectiveness.

    • Pulmonary Rehabilitation: Strategies focused on improving the quality of life and maximizing lung function through education and exercise.

    • Lifestyle Interventions:

- Adopting a healthy, well-balanced diet, increasing fluid intake, avoiding smoking, and practicing good hygiene are recommended.

Lifespan Considerations for Cystic Fibrosis

Infants and Children

  • Indicators:

    • Prenatal Indicators include hyper-echoic bowel on ultrasound.

    • Neonates may show symptoms like mecconium ileus indicating intestinal blockage.

- Ongoing Assessments: Essential for monitoring respiratory status, weight, and growth in children.

Adolescents

  • Continued Care: Adolescents still require airway clearance techniques, enzymes, and vaccinations, along with nutritional supplementation.

- Developing Autonomy: Teens should engage in self-care and are encouraged to discuss future plans regarding education and careers.

Adults

  • Concerns: Long-term damage to lungs, pancreas, and liver may necessitate organ transplantation.

    • Women may require pelvic exercises and may face fertility challenges due to illness.

- Men have lower fertility rates, with only 2–3% being fertile.

Pregnant Women

  • Pregnancy Risks: CF may complicate pregnancy; good lung functioning correlates to improved outcomes.

    • Nutritional supplementation is often necessary, and risk of gestational diabetes increases.

- Therapeutic regimens may be altered during pregnancy, with caution needed for drug discontinuations pre-delivery.

Nursing Process for Cystic Fibrosis Patients
  • Role in Continuity of Care: The nurse's role is critical due to the chronic nature of CF. Thorough assessments should occur at each visit focusing on maintaining oxygenation and nutrition.

  • Assessment:

    • Observe respiratory status, noting cough characteristics and adherence to medication regimens.

- Conduct physical exams including vital signs and reference sputum samples for infection checks (via cultures when symptomatic).

Diagnosis and Nursing Problems

- Identifying Potential Issues: Patient problems may include ineffective breathing patterns, inadequate airway clearance, fluid volume deficiencies, undernutrition, inability to tolerate activity, and caregiver burdens.

Planning and Goals

- Patient Goals: Positive outcomes might involve the patient being able to report decreased effort in maintaining adequate oxygenation, proper techniques for airway clearance, and maintaining nutritional status while also demonstrating knowledge of required dietary restrictions.

Implementation – Nursing Interventions
  • Promoting Effective Breathing: Teach calling the primary care provider during pulmonary exacerbations, administer bronchodilators prior to implementing airway clearance techniques, and use incentive spirometers as prescribed.

- Promoting Airway Clearance: Educate on airway clearance techniques, assist with chest physiotherapy, and increase frequency of treatments during exacerbations (up to 4 times/day whenever necessary).

Infection Control/Prevention Measures
  • Infection Control: Teach self-administration of inhaled antibiotics and effective hand hygiene practices.

- Teach proper cough etiquette and encourage social distancing from others with CF to avoid transmission of resistant pathogens.

Nutrition Monitoring

- Monitoring Nutrition: Advocate for the use of gastronomy tubes if needed, collaborate with dietitians for optimal nutrition plans, monitor for electrolyte imbalances, and assure caloric intake is elevated during pulmonary exacerbations.

Physical Activity Support

- Encouragement: Instruct on hydration strategies during exercise and advocate for safe participation in physical activities by children with CF in school settings.

Patient and Caregiver Support

- Emotional Support: Provide educational resources about CF, support groups, and counseling referrals to help patients and caregivers navigate their disease journey.

Evaluation of Outcomes
  • Expected Outcomes: Positive assessments may include successful mucus expectoration, adherence to prescribed medications, and understanding of nutritional needs.

    • Given the chronic nature of CF, adjustments to treatments may be necessary, requiring patient advocacy for needed changes.