Pharmacotherapy Case: Cystic Fibrosis by Dr. Nehad Ahmed
Pharmacotherapy Case: Cystic Fibrosis
Patient Information:
- Age: 19
- Gender: Female
- Date of Visit: February 5, 2025
Chief Complaint:
"I’ve been coughing a lot more lately, and I feel like I can’t get enough air sometimes. I also have trouble with digestion and have been feeling more tired than usual."
Subjective Data:
History of Present Illness: A 19-year-old female with a long history of cystic fibrosis (CF) presents with worsening respiratory and gastrointestinal symptoms over the past few months. She reports a chronic cough that has been productive of thick, greenish sputum, which has worsened over the past month. She also describes an increase in shortness of breath and wheezing during physical activity, and states that she feels more fatigued than usual, even after a full night’s sleep. She has had occasional chest tightness and increased sinus congestion.
In addition to her respiratory issues, the patient mentions digestive problems—including frequent loose stools and abdominal discomfort. She notes poor appetite, and her weight has remained stable despite these symptoms. She states that she has been taking her prescribed medications, but often misses doses of her inhaled therapies due to the burden of the regimen.
Past Medical History:
- Cystic fibrosis (diagnosed in infancy)
- Chronic sinusitis, exacerbated by CF
- Pancreatic insufficiency, requiring enzyme replacement therapy
- Recurrent pulmonary infections (including pseudomonal infections)
Medications:
- Dornase alfa (Pulmozyme) inhalation 2.5 mg daily for airway clearance
- Tobramycin inhalation solution 300 mg twice daily for chronic pseudomonal infection (currently on a 28-day cycle)
- Pancrelipase (Creon) 10,000 units with every meal for pancreatic insufficiency
- Albuterol inhaler 90 mcg/puff, 2 puffs every 4-6 hours as needed for wheezing
- Vitamin D 1000 IU daily
- Azithromycin 250 mg three times a week (for chronic inflammation and infection prevention)
- Ibuprofen 400 mg twice daily for anti-inflammatory purposes in the lungs
Allergies:
- No known drug allergies
Family History:
- Father has hypertension and hyperlipidemia
- Mother has type 2 diabetes
Social History:
- Non-smoker
- Occasional alcohol use (1-2 drinks on weekends)
- Lives with parents and younger sibling
- Attends college part-time, living an active but often sedentary lifestyle due to fatigue
Review of Systems:
- Respiratory: Chronic cough, productive of thick sputum, shortness of breath on exertion, wheezing, increased sinus congestion
- Gastrointestinal: Loose stools, abdominal discomfort, poor appetite, no significant weight loss
- Neurological: Occasional headaches, no dizziness or syncope
- Musculoskeletal: No joint pain, but generalized fatigue
- Integumentary: No skin rashes or lesions
Objective Data:
Vital Signs:
- Temperature: 98.3°F (36.8°C)
- Blood Pressure: 110/68 mmHg (within normal limits)
- Heart Rate: 88 bpm (regular rhythm)
- Respiratory Rate: 20 breaths per minute (normal)
- Oxygen Saturation: 95% on room air
Physical Examination:
- General Appearance: The patient appears slightly fatigued but is alert and oriented. No signs of acute distress.
- HEENT: Mild nasal congestion and sinus tenderness upon palpation. No signs of conjunctival injection.
- Chest and Lungs:
- Inspection: No use of accessory muscles, but increased respiratory effort noted during auscultation.
- Palpation: No tenderness or palpable masses
- Percussion: Normal resonance, no dullness
- Auscultation: Wheezing and rales (fine crackles) in bilateral lower lung fields. Decreased breath sounds in the bases with prolonged expiration.
- Cardiovascular: Regular rhythm, no murmurs, no peripheral edema
- Abdomen: Mild tenderness in the epigastric region, no palpable masses, no hepatosplenomegaly
- Extremities: No cyanosis, no clubbing of the fingers or toes
- Neurological: No focal deficits, normal gait
Laboratory Results:
- Complete Blood Count (CBC): Normal WBC count, no signs of active infection
- Basic Metabolic Panel (BMP): Normal electrolyte levels, mild hypokalemia (3.3 mEq/L), normal renal function
- Liver Function Tests: Normal
- Sputum Culture: Positive for Pseudomonas aeruginosa, moderate growth
- Fecal Elastase: Low (consistent with pancreatic insufficiency)
- Vitamin D levels: Low at 15 ng/mL (normal range: 30-100 ng/mL)
Pulmonary Function Tests (PFTs):
- Forced Expiratory Volume in 1 second (FEV1): 60% of predicted value (moderate obstruction)
- Forced Vital Capacity (FVC): 75% of predicted value
- FEV1/FVC ratio: 60% (indicative of obstructive lung disease)
- Bronchodilator response: No significant improvement in FEV1 after bronchodilator administration
Chest X-ray:
- Mild hyperinflation of the lungs, consistent with obstructive lung disease
- No signs of pneumonia or other acute abnormalities
CT Chest (contrast-enhanced):
- Evidence of bronchiectasis and mucus plugging, characteristic of cystic fibrosis
- Thickening of the bronchial walls, with areas of air trapping
Assessment:
The patient is presenting with worsening respiratory symptoms, including cough, wheezing, and shortness of breath, which are indicative of pulmonary exacerbation due to chronic infection, most notably with Pseudomonas aeruginosa. Her pancreatic insufficiency is contributing to malabsorption and gastrointestinal distress, evidenced by loose stools and abdominal discomfort. The presence of bronchiectasis and mucus plugging on imaging, along with moderate pulmonary obstruction on PFTs, confirms a progression of cystic fibrosis (CF).
Plan:
Pharmacotherapy:
- Inhaled Antibiotics:
- Continue Tobramycin inhalation solution 300 mg twice daily (on 28-day cycle), as this is effective for chronic Pseudomonas aeruginosa infection.
- Mucolytic Therapy:
- Continue Dornase alfa (Pulmozyme) 2.5 mg daily to reduce sputum viscosity and promote airway clearance.
- Bronchodilators:
- Albuterol inhaler 90 mcg/puff, 2 puffs every 4-6 hours as needed, but encourage consistent use with a spacer for improved efficacy.
- Pancreatic Enzyme Replacement:
- Increase Pancrelipase (Creon) dose based on meals and snacks. Consider increasing to 15,000 units per meal for improved digestion and absorption.
- Vitamin D supplementation:
- Increase Vitamin D supplementation to 2000 IU daily to correct deficiency and promote bone health.
- Oral Antibiotics:
- Start Ciprofloxacin 500 mg twice daily for 14 days to treat the acute exacerbation of Pseudomonas aeruginosa infection.
- Anti-inflammatory therapy:
- Continue Azithromycin 250 mg three times a week to reduce inflammation and bacterial load.
- Inhaled Antibiotics:
Nutritional Support:
- Increase caloric intake and focus on a high-protein, high-calorie diet. Consider referral to a dietitian specialized in CF management.
- Monitor for signs of malnutrition or weight loss.
Pulmonary Rehabilitation:
- Refer to pulmonary rehabilitation to help improve exercise tolerance, airway clearance, and quality of life.
Follow-up:
- Schedule follow-up in 2 weeks for re-evaluation of respiratory status, treatment response, and review of laboratory results.
- Monitor weight, pulmonary function, and potential side effects of the new antibiotic regimen.
Patient Education:
- Educate the patient on the importance of adherence to daily airway clearance therapies, including inhaled medications and chest physiotherapy.
- Stress the need for early intervention with antibiotics for exacerbations to avoid further lung damage.
- Explain the importance of taking pancreatic enzymes with every meal to optimize nutrient absorption and improve gastrointestinal symptoms.
Discharge Instructions:
- Continue current medications as prescribed, including inhalers and enzymes.
- Monitor for signs of worsening respiratory distress or new symptoms such as increased sputum production or hemoptysis.
- Adhere to the antibiotic regimen for the full 14-day course to prevent complications from Pseudomonas infection.
Assessment Summary:
- Diagnosis: Cystic Fibrosis with respiratory exacerbation due to chronic Pseudomonas aeruginosa infection, pancreatic insufficiency, and vitamin D deficiency.
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