Cystic Fibrosis Nursing Diagnosis Care Plan: A Comprehensive Guide for Nurses

Cystic fibrosis (CF) is a complex, inherited disorder that significantly impacts the respiratory and digestive systems, and can affect other organs throughout the body. As a progressive and lifelong condition, CF necessitates specialized and comprehensive nursing care. Primarily diagnosed in newborns, cystic fibrosis currently has no cure, and individuals with CF require continuous management to improve their quality of life and extend lifespan.

The root cause of CF lies in mutations of the CFTR (cystic fibrosis transmembrane conductance regulator) gene. This genetic defect disrupts the movement of chloride, a crucial component of salt, across cell membranes. Consequently, water is not adequately drawn to the cell surface, leading to the production of abnormally thick and sticky mucus. This tenacious mucus accumulates in vital organs, particularly the lungs and pancreas, causing severe respiratory complications, digestive issues, and increasing susceptibility to infections.

Nurses play a pivotal role in the multidisciplinary care of patients with cystic fibrosis. While CF is considered a rare disease, nurses in specialized areas such as pulmonology, pediatrics, and transplant units are likely to encounter and care for these individuals. Whether managing respiratory infections in children or providing post-transplant care for adults, a thorough understanding of the multifaceted nature of CF is essential for nurses to deliver effective and compassionate care. This guide aims to provide a comprehensive overview of cystic fibrosis nursing diagnosis and care plans, focusing on delivering optimal patient outcomes.

Nursing Assessment for Cystic Fibrosis

The cornerstone of effective nursing care is a meticulous nursing assessment. This process involves gathering comprehensive data – physical, psychosocial, emotional, and diagnostic – to understand the patient’s unique needs and challenges related to cystic fibrosis. This section will delve into both subjective and objective data collection crucial for formulating accurate cystic fibrosis nursing diagnoses.

Gathering Subjective Data: Health History Review

A detailed health history is paramount in assessing a patient with suspected or diagnosed cystic fibrosis. Nurses should explore various aspects of the patient’s history to gain a holistic understanding of their condition.

1. General Symptom Assessment: Inquire about common clinical manifestations of cystic fibrosis. These may include:

• Salty-tasting skin: Often noted by parents when kissing their child.
• Persistent cough with thick mucus: Characterized by its viscosity and difficulty in expectoration.
• Wheezing: An audible whistling sound during breathing, indicating airway narrowing.
• Dyspnea (Shortness of breath): Subjective sensation of breathing difficulty.
• Recurrent lung or sinus infections: Increased frequency and severity of respiratory infections.
• Nasal polyps: Noncancerous growths in the nasal passages, contributing to airway obstruction.
• Delayed growth and development: Failure to meet expected growth milestones.
• Difficulty gaining weight: Despite adequate caloric intake.
• Steatorrhea (Greasy, foul-smelling stools): Indicative of fat malabsorption due to pancreatic insufficiency.
• Constipation: Infrequent or difficult bowel movements, sometimes leading to intestinal obstruction.
• Male infertility: Resulting from congenital bilateral absence of vas deferens (CBAVD).

2. Age of Onset and Diagnosis: Note the patient’s age at symptom onset and diagnosis. Two-thirds of CF patients are diagnosed within the first year of life, with an average diagnosis age between 6 to 8 months. Symptom presentation can vary significantly across different age groups.

3. Medical History: Explore the patient’s past medical history, particularly focusing on:

• Meconium ileus in neonates: One of the earliest signs of CF, indicating intestinal obstruction at birth.
• Other Gastrointestinal Manifestations: History of intestinal obstruction, volvulus (twisting of the intestine), perforation, or other GI complications.

4. Parental Health History (for Pediatric Patients): For children, obtain a detailed history from parents regarding:

• Early Respiratory Symptoms: Wheezing, coughing, recurrent respiratory infections, and pneumonia, especially in infants under one year old.
• Early Gastrointestinal Symptoms: Steatorrhea, failure to thrive (inadequate weight gain for age), or both in early infancy.

5. Family and Genetic History: Cystic fibrosis is an autosomal recessive genetic disorder. Assess family history to identify:

• Family history of CF: Presence of CF in siblings or other family members.
• Carrier status: Determine if parents are known carriers of the CF gene. Understanding inheritance patterns is crucial for genetic counseling and risk assessment.

6. Racial and Ethnic Background: While CF affects individuals of all races, it is most prevalent among white populations of Northern European descent. However, CF is increasingly recognized in other ethnic groups, and diagnosis should not be excluded based on race.

Gathering Objective Data: Physical Assessment

A thorough physical examination provides crucial objective data to complement the subjective information gathered. The physical manifestations of CF vary depending on the disease progression and the organ systems involved.

1. Comprehensive Physical Examination: Perform a systematic physical examination, paying close attention to the following systems:

• Head, Eyes, Ears, Nose, and Throat (HEENT):
  • Rhinitis (inflammation of the nasal mucous membrane)
  • Nasal polyps
  • Inflammation of the submandibular or parotid glands (salivary glands)
• Respiratory System:
  • Rapid breathing (tachypnea)
  • Respiratory distress: Signs like nasal flaring, retractions (drawing in of chest muscles during breathing)
  • Chest retractions
  • Dry or productive cough
  • Increased anterior-posterior chest diameter (barrel chest)
• Integumentary System (Skin):
  • Finger clubbing: Enlargement of the fingertips and changes in nail angle due to chronic hypoxia.
  • Cyanosis: Bluish discoloration of the skin and mucous membranes, indicating low oxygen levels.
  • Aquagenic wrinkling of the palms (AWP): Wrinkling of palms after brief water immersion, a less common but specific sign.
• Gastrointestinal System:
  • Abdominal distention (swelling)
  • Hepatomegaly (liver enlargement) and splenomegaly (spleen enlargement) – hepatosplenomegaly
  • Rectal prolapse: Protrusion of the rectum through the anus.
  • Cheilosis: Swelling and fissuring at the corners of the lips.
  • Steatorrhea (observe stool characteristics if possible)
  • Chronic constipation
  • Poor weight gain (assess growth charts)
• Musculoskeletal System:
  • Scoliosis (lateral curvature of the spine)
  • Kyphosis (excessive outward curvature of the spine, hunchback)

2. Auscultation of Lung Sounds: Listen to lung sounds using a stethoscope. Patients with pulmonary involvement may exhibit:

• Wheezing: High-pitched whistling sounds, indicating airway narrowing.
• Crackles (rales): Fine, crackling sounds, suggesting fluid in the small airways.
• Decreased breath sounds: Indicates reduced airflow to certain lung areas.

3. Chest Percussion: Percuss (tap) the chest to assess resonance. Hyperresonance (an abnormally loud, low-pitched sound) may indicate hyperinflation of the lungs, a common finding in CF.

4. Assessment of Airway Inflammation: While not directly auscultable or percussible, recognize that airway inflammation is a hallmark of CF lung disease, even in the absence of active infection.

5. Digestive Symptom Assessment: Further investigate digestive issues by:

• Inquiring about stool frequency, consistency, odor, and appearance.
• Assessing for abdominal pain, bloating, and discomfort.
• Reviewing dietary intake and nutritional status.

6. Assessment of Male Genitalia and Fertility: In male patients, consider:

• Congenital Bilateral Absence of Vas Deferens (CBAVD): A significant indicator of CF in males, present in 75-80% of males with CF gene mutations.
• Obstructive Azoospermia (absence of sperm in semen): In adult males, this can be a sign of CF-related infertility.

Diagnostic Procedures for Cystic Fibrosis

Definitive diagnosis of cystic fibrosis relies on a combination of clinical symptoms and laboratory evidence of CFTR dysfunction.

1. Diagnostic Criteria for CF: Diagnosis is typically based on:

• Clinical Presentation: Presence of one or more characteristic clinical features, such as:
  • Sibling with diagnosed CF.
  • Positive newborn screening result.
  • Symptoms affecting respiratory, gastrointestinal, or other organ systems.
  • Chronic sinopulmonary disease (persistent lung and sinus problems).
  • Gastrointestinal or nutritional abnormalities (e.g., meconium ileus, malabsorption).
  • Salt loss syndromes (excessive salt excretion in sweat).
• Evidence of CFTR Dysfunction: Confirmed by at least one of the following:
  • Elevated Sweat Chloride Test: Sweat chloride concentration greater than 60 mEq/L on two separate occasions is a positive result.
  • Genetic Testing: Identification of two disease-causing CFTR mutations.
  • Abnormal Nasal Potential Difference (NPD): Measures the electrical potential difference across the nasal epithelium, reflecting CFTR function. (Less commonly used in routine diagnosis).

2. Prenatal, Neonatal, and Postnatal Screening: Early detection is crucial for timely intervention and management.

• Prenatal Screening:
  • Amniocentesis: Can be performed when both parents are known CF carriers to assess the fetus for CFTR mutations.
• Neonatal Screening:
  • Immunoreactive Trypsinogen (IRT) Test: A blood test performed as part of newborn screening programs in many regions. Elevated IRT levels require further confirmatory testing (sweat test, genetic testing).
• Postnatal Diagnostic Testing: Prompt diagnostic evaluation is essential for newborns presenting with meconium ileus or other signs suggestive of CF.

3. Sweat Chloride Test: Prepare the patient for this gold-standard diagnostic test. Explain the procedure:

• Pilocarpine Iontophoresis: A painless procedure using a mild electrical current to stimulate sweat production in a localized area of the skin.
• Sweat Collection and Analysis: Sweat is collected on filter paper or gauze and analyzed for chloride concentration.

4. Chest X-ray: Assist in obtaining and interpreting chest radiographs. Chest X-rays in CF patients may reveal:

• Pulmonary nodules
• Bronchiectasis (widening of the bronchi)
• Hyperinflation
• Infiltrates (indicating infection or inflammation)

5. Pulmonary Function Testing (PFT): Prepare the patient for PFT, which is vital for assessing and monitoring lung function in CF.

• Spirometry: Measures lung volumes and airflow rates. May not be reliable in very young children (under 5-6 years).
• Infant PFTs: Specialized techniques can assess airway obstruction even in infants.

6. Imaging Scans: Various imaging modalities are used for detailed evaluation:

• Chest CT Scanning: Provides high-resolution images of the lungs to detect bronchiectasis, mucus plugging, and other structural abnormalities.
• Abdominal Radiography: Can diagnose meconium ileus in newborns.
• Prenatal Ultrasound: Sonographic findings suggestive of hyperechoic masses in the fetal bowel may indicate increased risk of meconium ileus.

7. Genotyping: Discuss genetic testing (genotyping) especially for:

• Patients with a positive family history of CF.
• Couples planning pregnancy and known to be CF carriers or have a family history.

8. Pancreatic Enzyme Assessment: Evaluate pancreatic function.

• Fecal Elastase-1 Test: Measures the level of elastase, a pancreatic enzyme, in stool. Low levels indicate pancreatic insufficiency.
• Immunoreactive Trypsinogen (IRT): As mentioned earlier, also used in newborn screening for pancreatic involvement.

9. Sputum Culture and Analysis: Obtain sputum samples for analysis, particularly during pulmonary exacerbations.

• Bacterial Pathogens: Identify bacteria commonly colonizing the airways in CF, such as Pseudomonas aeruginosa, Staphylococcus aureus, and Burkholderia cepacia complex.
• Bronchoalveolar Lavage (BAL): Fluid obtained during bronchoscopy may show a high percentage of neutrophils and presence of Pseudomonas aeruginosa, supporting CF diagnosis in specific cases.

10. Contrast Barium Enema: Prepare for and assist with contrast barium enema if meconium ileus is suspected or seen on abdominal radiography in newborns. This can be both diagnostic and therapeutic in relieving meconium ileus.

Nursing Interventions for Cystic Fibrosis

Nursing interventions are crucial in managing the complex needs of patients with cystic fibrosis, focusing on symptom management, preventing complications, and improving quality of life.

1. Medication Administration: Administer medications as prescribed, which may include:

• Pancreatic Enzyme Supplements: Essential for digestion and nutrient absorption, taken with meals and snacks containing fat, protein, or complex carbohydrates.
• Multivitamins (especially fat-soluble vitamins A, D, E, K): To address malabsorption and prevent deficiencies.
• Mucolytics (e.g., dornase alfa, hypertonic saline): To thin and loosen thick mucus in the airways, facilitating airway clearance.
• Antibiotics (oral, inhaled, IV): To treat and prevent pulmonary infections, targeting common CF pathogens.
• Bronchodilators (e.g., albuterol): To open airways and improve airflow, often administered before mucolytics and airway clearance techniques.
• Anti-inflammatory Agents (e.g., inhaled corticosteroids, ibuprofen, azithromycin): To manage airway inflammation.
• CFTR Modulators (e.g., ivacaftor, lumacaftor/ivacaftor, tezacaftor/ivacaftor, elexacaftor/tezacaftor/ivacaftor): Medications that target the underlying CFTR gene defect, improving chloride transport and mucus hydration in eligible patients with specific CFTR mutations.
• Medications for CF-related complications: Such as insulin for CF-related diabetes (CFRD), bisphosphonates for osteoporosis.

2. Airway Clearance Techniques: Implement and educate patients and families on effective airway clearance techniques to remove thick mucus from the lungs:

• Mucolytic Medications: Administer as prescribed to reduce sputum viscosity.
• Chest Physiotherapy (CPT): Collaborate with respiratory therapists to perform or teach caregivers to perform CPT with postural drainage (positioning the patient to drain specific lung segments).
• Positive Expiratory Pressure (PEP) Devices (e.g., PEP mask, Flutter valve): Devices that create positive pressure during exhalation to help open airways and mobilize mucus.
• High-Frequency Chest Wall Oscillation (HFCWO) Vest: An inflatable vest that vibrates the chest wall at high frequencies to loosen mucus.
• Autogenic Drainage: A breathing technique to mobilize secretions through controlled breathing patterns.
• Active Cycle of Breathing Techniques (ACBT): Combines breathing control, thoracic expansion exercises, and forced expiratory techniques.

3. Management of Pulmonary Exacerbations: Educate patients and families on managing mild pulmonary exacerbations at home and recognizing when to seek medical attention:

• Increased Airway Clearance Techniques: Increase frequency and intensity of airway clearance therapies.
• Inhaled Bronchodilators: Administer as prescribed to alleviate bronchospasm.
• Chest PT and Postural Drainage: Perform more frequently.
• Increased Dornase Alfa (mucolytic) use: If prescribed.
• Oral or Inhaled Antibiotics: Administer as prescribed for early infection management.

4. Hypertonic Saline Administration: For patients aged six years and older, administer inhaled hypertonic saline as prescribed. It helps to hydrate airway surface liquid, improving lung function and reducing exacerbations.

5. Nutritional Management and Meal Planning: Collaborate with dietitians to develop individualized meal plans.

• High-Calorie, High-Fat Diet: Advise patients to consume a diet higher in calories and fat to compensate for malabsorption and increased energy expenditure.
• Unrestricted Fat Intake (unless other medical conditions warrant restriction): Due to pancreatic insufficiency, fat malabsorption is common, and restricting fat is generally not recommended without enzyme replacement.
• Nutritional Supplements: Recommend oral or enteral nutritional supplements to meet caloric and nutritional needs.
• Supplemental Vitamins: Ensure adequate intake of fat-soluble vitamins (A, D, E, K) and other vitamins and minerals as needed.

6. Pancreatic Enzyme Replacement Therapy (PERT) Education: Educate patients and families on the importance of PERT:

• Administer with all meals and snacks: Enzymes should be taken before consuming any food containing fat, protein, or complex carbohydrates.
• Dosage Adjustment: Emphasize the need for individualized dosage adjustments based on stool output, weight gain, and nutritional status, guided by a dietitian and physician.
• Capsule Administration: Instruct on swallowing capsules whole, or if unable to swallow capsules, how to open capsules and sprinkle the beads on acidic foods (e.g., applesauce) for children.

7. Surgical Interventions: Prepare patients for potential surgical procedures that may be necessary to manage CF complications:

• Respiratory Conditions:
  • Pneumothorax (air leak into the pleural space): Chest tube placement or surgery.
  • Persistent hemoptysis (coughing up blood): Bronchoscopy, embolization, or surgery.
  • Nasal polyps: Polypectomy (surgical removal).
  • Chronic sinusitis: Functional endoscopic sinus surgery (FESS).
  • End-stage lung disease: Lung transplantation (single or double lung).
• Gastrointestinal Conditions:
  • Meconium ileus (intestinal obstruction in newborns): Surgery to relieve obstruction.
  • Intussusception (telescoping of the intestine): Air enema or surgery.
  • G-tube placement (gastrostomy tube): For supplemental feeding when oral intake is insufficient.
  • Rectal prolapse: Manual reduction or surgery in severe cases.

8. Diabetes Management (CFRD): For patients who develop CF-related diabetes, implement diabetes management strategies, which may include:

• Insulin Therapy: Insulin is typically the primary treatment for CFRD.
• Blood Glucose Monitoring: Educate on self-monitoring of blood glucose levels.
• Dietary Management: Work with a dietitian to develop a diabetes-appropriate meal plan.
• Education on Hypoglycemia and Hyperglycemia: Instruct on recognizing and managing these conditions.

9. Long-Term Monitoring and Clinic Visits: Emphasize the importance of regular follow-up at a CF clinic every 2-3 months to:

• Promote growth and development (especially in children).
• Optimize lung function.
• Prevent lung disease progression.
• Provide nutritional support.
• Monitor treatment effectiveness and complications.
• Offer psychosocial counseling and support.

10. Vaccination and Infection Prevention: Advise on routine vaccinations:

• Influenza Vaccine (annual): Crucial to prevent seasonal influenza.
• Pneumococcal Vaccines (PPSV23, PCV13 or PCV15, PCV20): To prevent pneumococcal pneumonia.
• Routine childhood vaccinations: Follow recommended immunization schedules.
• Infection Control Measures: Educate patients and families on:
  • Avoiding crowds and contact with sick individuals.
  • Practicing meticulous hand hygiene.
  • Wearing masks in public places, especially during respiratory illness seasons.

Cystic Fibrosis Nursing Care Plans: Examples

Nursing care plans provide a structured framework for organizing and delivering patient care. They help prioritize nursing diagnoses, guide interventions, and establish measurable goals. Here are examples of nursing care plans for common nursing diagnoses in cystic fibrosis.

Nursing Care Plan: Imbalanced Nutrition: Less Than Body Requirements

Nursing Diagnosis: Imbalanced Nutrition: Less Than Body Requirements related to malabsorption, poor digestion, and nutrient deficiencies.

As evidenced by: Delayed growth, diarrhea or constipation, foul-smelling and greasy stools, poor weight gain, intestinal obstructions, abdominal distention or bloating.

Expected Outcomes:

• Patient will demonstrate improved nutritional status as evidenced by laboratory values within normal limits (e.g., prealbumin, vitamin levels).
• Patient will achieve and maintain a weight gain of [specific percentage or amount] within [timeframe], as appropriate for age and growth charts.
• Patient (or family/caregiver) will verbalize understanding of and adhere to prescribed pancreatic enzyme therapy and nutritional recommendations to optimize nutrition.

Nursing Assessments:

1. Assess weight and growth patterns: Rationale: Children with CF often exhibit failure to thrive or delayed growth due to malabsorption. Regular monitoring of height and weight is crucial to assess nutritional status and response to interventions.
2. Observe and document stool characteristics: Rationale: Steatorrhea (greasy, foul-smelling stools) is a hallmark of fat malabsorption due to pancreatic insufficiency. Changes in stool patterns can indicate the effectiveness of enzyme therapy.
3. Monitor relevant laboratory values: Rationale: CF can lead to complications such as liver disease and CF-related diabetes, which can impact nutritional status. Liver enzymes, glucose levels, and electrolyte levels provide objective data on metabolic function and nutritional status.

Nursing Interventions:

1. Administer pancreatic enzyme supplements as prescribed: Rationale: Pancreatic enzymes replace deficient endogenous enzymes, aiding in the digestion and absorption of fats, carbohydrates, and proteins. Timely administration before meals is essential for optimal effectiveness.
2. Provide education on a high-calorie, high-protein diet with unrestricted fat (unless contraindicated): Rationale: Patients with CF have increased caloric needs due to malabsorption and chronic infection. A diet rich in calories, protein, and fat helps meet these needs and promote weight gain and growth.
3. Provide comprehensive patient and family education on nutritional management: Rationale: Adherence to dietary recommendations and enzyme therapy is crucial for managing nutritional deficiencies. Education should address enzyme administration, dietary modifications, and strategies for managing feeding challenges, especially in children.
4. Collaborate with a registered dietitian: Rationale: A dietitian specialized in CF can provide expert guidance on individualized nutritional assessments, meal planning, enzyme dosage adjustments, and strategies to optimize nutritional intake and address specific dietary concerns.

Nursing Care Plan: Impaired Gas Exchange

Nursing Diagnosis: Impaired Gas Exchange related to accumulation of tenacious secretions, infectious process, and inflammation of airways.

As evidenced by: Abnormal arterial blood gases (ABGs), tachypnea, dyspnea, hypoxia, restlessness, tachycardia, altered respiratory pattern.

Expected Outcomes:

• Patient will demonstrate adequate ventilation and oxygenation as evidenced by ABGs within patient-specific normal limits or baseline.
• Patient will exhibit clear or improved lung sounds on auscultation and remain free from signs of respiratory distress (e.g., dyspnea, cyanosis).

Nursing Assessments:

1. Assess oxygen saturation using pulse oximetry: Rationale: Pulse oximetry provides a noninvasive measure of peripheral oxygen saturation (SpO2). Values below 95% may indicate hypoxemia and impaired gas exchange.
2. Monitor mental status and level of consciousness: Rationale: Changes in mental status, such as confusion, restlessness, irritability, or lethargy, can be early indicators of hypoxemia and inadequate cerebral oxygenation.
3. Continuously monitor respiratory rate, depth, and pattern: Rationale: Tachypnea, shallow breathing, and changes in respiratory rhythm can signal respiratory distress and impaired gas exchange. Early detection of changes allows for timely intervention.

Nursing Interventions:

1. Administer supplemental oxygen as prescribed: Rationale: Supplemental oxygen increases the inspired oxygen concentration (FiO2), improving oxygen delivery to the tissues and correcting hypoxemia. Humidified oxygen can help to liquefy secretions, aiding in airway clearance.
2. Cluster nursing care activities to maximize rest periods: Rationale: Minimizing interruptions and clustering care reduces oxygen demand and conserves energy, particularly during periods of respiratory distress or exacerbation.
3. Administer prescribed medications promptly: Rationale: Bronchodilators open airways, mucolytics thin secretions, and antibiotics treat pulmonary infections, all of which improve gas exchange by addressing underlying causes of respiratory compromise.
4. Monitor arterial blood gases (ABGs) as ordered and interpret results: Rationale: ABGs provide direct measurements of PaO2, PaCO2, and pH, offering a comprehensive assessment of gas exchange and acid-base balance. Serial ABGs monitor disease progression and response to therapy.

Nursing Care Plan: Ineffective Airway Clearance

Nursing Diagnosis: Ineffective Airway Clearance related to increased mucus production, infectious process, and inflammation of the bronchioles.

As evidenced by: Persistent cough, productive cough, wheezing, shortness of breath, nasal congestion, sinusitis, abnormal chest x-ray, adventitious breath sounds (crackles, rhonchi).

Expected Outcomes:

• Patient will maintain a patent airway as evidenced by absence of respiratory distress and ability to breathe comfortably.
• Patient will effectively expectorate or clear airway secretions, as evidenced by decreased cough and improved breath sounds.
• Patient will demonstrate improvement in airway clearance as evidenced by vital signs and chest x-ray findings returning toward baseline or within normal limits.

Nursing Assessments:

1. Auscultate lung sounds before and after airway clearance techniques: Rationale: Auscultation helps identify the presence of adventitious breath sounds (wheezing, crackles, rhonchi) indicating mucus accumulation and airway obstruction. Post-intervention auscultation assesses the effectiveness of airway clearance techniques.
2. Assess sputum characteristics (amount, color, consistency): Rationale: Changes in sputum production and characteristics can indicate infection, inflammation, and the effectiveness of mucolytic therapy and airway clearance techniques. Green or yellow sputum suggests bacterial infection.
3. Monitor respiratory rate, rhythm, and effort, and vital signs including oxygen saturation: Rationale: Tachypnea, labored breathing, and decreased oxygen saturation are signs of ineffective airway clearance and respiratory distress. Vital sign monitoring helps detect early changes and guide interventions.

Nursing Interventions:

1. Administer bronchodilators as prescribed, prior to airway clearance techniques: Rationale: Bronchodilators open airways by relaxing bronchial smooth muscle, maximizing the effectiveness of subsequent mucolytics and airway clearance techniques. Administering bronchodilators first optimizes airway patency for mucus removal.
2. Administer mucolytic medications as prescribed (e.g., hypertonic saline, dornase alfa): Rationale: Mucolytics reduce the viscosity of mucus, making it easier to mobilize and expectorate. Hypertonic saline hydrates airway secretions, while dornase alfa breaks down DNA in mucus, both contributing to improved airway clearance.
3. Assist with and educate on airway clearance techniques (chest physiotherapy, PEP devices, HFCWO): Rationale: Airway clearance techniques are essential for mechanically mobilizing and removing mucus from the airways. Tailoring techniques to individual patient needs and providing thorough education enhances adherence and effectiveness.
4. Position the patient strategically to promote secretion mobilization and drainage: Rationale: Proper positioning, guided by chest x-ray findings and lung segment involvement, utilizes gravity to facilitate drainage of secretions from specific lung lobes. Prone, supine, and side-lying positions can be used to target different lung segments for optimal drainage.

Nursing Care Plan: Risk for Infection

Nursing Diagnosis: Risk for Infection related to thick mucus accumulation in airways promoting bacterial growth, potential antibiotic resistance, and potential weakened immune system.

As evidenced by: (A risk diagnosis is not evidenced by actual signs and symptoms as the problem has not yet occurred. Nursing interventions are focused on prevention.)

Expected Outcomes:

• Patient will remain free from respiratory infections throughout hospitalization or designated timeframe.
• Patient will demonstrate behaviors and understanding of infection prevention strategies, including proper hand hygiene and avoidance of sick contacts.
• Patient will exhibit laboratory values (e.g., white blood cell count, CRP) within normal limits or baseline, indicating absence of active infection.

Nursing Assessments:

1. Monitor for early signs and symptoms of infection (fever, increased cough, change in sputum, fatigue, increased respiratory rate, tachycardia): Rationale: Early detection of infection allows for prompt intervention and treatment, preventing progression and complications. Regular assessment for subtle changes in clinical status is crucial.
2. Monitor laboratory values, particularly white blood cell count (WBC) and sputum cultures: Rationale: Elevated WBC count and positive sputum cultures indicate the presence of infection. Sputum cultures identify specific pathogens, guiding antibiotic selection and monitoring for antibiotic resistance.
3. Assess for risk factors for antibiotic resistance (history of frequent antibiotic use, previous resistant infections): Rationale: Frequent antibiotic use in CF patients increases the risk of developing antibiotic-resistant infections. Assessing risk factors helps guide antibiotic stewardship and infection control strategies.

Nursing Interventions:

1. Administer antibiotics as prescribed, ensuring appropriate dose, route, and duration: Rationale: Prompt and effective antibiotic therapy is crucial for treating pulmonary infections in CF patients. Adherence to prescribed regimens and monitoring for therapeutic response and adverse effects are essential.
2. Implement strict infection control precautions: Rationale: Meticulous infection control practices prevent transmission of pathogens, especially in healthcare settings. Hand hygiene, use of personal protective equipment (PPE), and appropriate patient room placement (single-patient rooms, spatial separation of CF patients) minimize cross-infection risks.
3. Promote and ensure adherence to recommended vaccination schedules: Rationale: Vaccination against influenza, pneumococcal pneumonia, and other vaccine-preventable illnesses reduces the risk of respiratory infections and associated complications in vulnerable CF patients. Annual influenza vaccination is particularly important.
4. Educate patient and family on infection prevention strategies: Rationale: Empowering patients and families with knowledge and skills for infection prevention is crucial for long-term management. Education should include hand hygiene techniques, avoidance of sick contacts, proper cough etiquette, and environmental hygiene measures to reduce infection risk in daily life.

These nursing care plans serve as examples and should be individualized based on a thorough assessment of each patient’s specific needs and clinical presentation. Continuous evaluation and modification of the care plan are essential to optimize patient outcomes in cystic fibrosis management.