Pharmacotherapy Case: Cystic Fibrosis (CF) by: Dr. Nehad Ahmed

Age: 24 years

Gender: Male

Subjective Data

  • Chief Complaint: "I’ve had a worsening cough with thick mucus for the past month. I’m struggling to breathe and losing weight despite eating."

  • History of Present Illness:

    • Chronic productive cough with yellow-green sputum.

    • Increased fatigue and dyspnea during daily activities (e.g., walking to class).

    • Poor weight gain (unintentional 5 lb weight loss in 4 weeks).

    • Recurrent sinusitis and 2 pulmonary exacerbations in the past year requiring IV antibiotics.

    • Reports steatorrhea (greasy stools) despite pancreatic enzyme replacement therapy (PERT).

  • Past Medical History:

    • Diagnosed with CF at birth (F508del mutation homozygous).

    • CF-related diabetes (CFRD) managed with insulin.

    • Pancreatic insufficiency.

  • Medications:

    • Dornase alfa (Pulmozyme): 2.5 mg inhaled daily.

    • Pancrelipase (Creon): 36,000 units lipase with meals.

    • Inhaled tobramycin (TOBI): 300 mg BID (on monthly alternating cycles).

    • Insulin glargine: 20 units SC nightly.

    • Multivitamins: ADEKs (vitamins A, D, E, K) daily.

  • Adherence: Inconsistent with hypertonic saline due to time constraints; otherwise adherent.

  • Social History: College student, lives in dormitory. Non-smoker, no alcohol/drug use.

Objective Data

  • Vital Signs:

    • BP: 118/74 mmHg | HR: 88 bpm | RR: 20 breaths/min | SpO2: 93% (RA).

  • Physical Exam:

    • Respiratory: Coarse crackles and wheezing in upper lobes. Digital clubbing.

    • GI: Mild abdominal distension; BMI 18.5 (down from 19.8).

  • Diagnostic Testing:

    • Sputum Culture: Pseudomonas aeruginosa (chronic colonization), methicillin-sensitive Staphylococcus aureus (MSSA).

    • Pulmonary Function Tests (PFTs): FEV1 55% predicted (baseline 65%).

    • Chest CT: Bronchiectasis with mucus plugging in upper lobes.

    • Sweat Chloride Test: 110 mmol/L (confirmatory for CF).

    • HbA1c: 7.2% (goal <7%).

  • Nutritional Labs:

    • Vitamin D: 18 ng/mL (low), albumin: 3.2 g/dL (low).

Assessment

  • Diagnosis: Cystic Fibrosis (F508del homozygous) with:

    • Severe Lung Disease: FEV1 55%, recurrent exacerbations.

    • Pancreatic Insufficiency: Steatorrhea, low albumin/vitamin D.

    • CF-Related Diabetes (CFRD): HbA1c 7.2%.

  • Exacerbation Triggers: Non-adherence to airway clearance, Pseudomonas colonization.

Pharmacotherapy Plan

  1. Airway Clearance & Mucoactive Therapy:

    • Dornase alfa: 2.5 mg inhaled daily (continue).

    • Hypertonic Saline 7%: 4 mL inhaled BID + albuterol pre-treatment.
      Rationale: Improve mucus hydration and clearance.

  2. Chronic Infection Management:

    • Inhaled aztreonam (Cayston): 75 mg BID × 28 days (alternate with tobramycin).
      Rationale: Target Pseudomonas with rotating antibiotics to reduce resistance.

  3. CFTR Modulator Therapy:

    • Elexacaftor/Tezacaftor/Ivacaftor (Trikafta): 2 tablets PO daily (initiate after insurance approval).
      Rationale: Address F508del mutation to improve CFTR function.

  4. Pancreatic/Nutritional Support:

    • Pancrelipase (Creon): Increase to 48,000 units lipase per meal + snacks.

    • Vitamin D3: 50,000 IU weekly × 8 weeks, then 2,000 IU daily.

  5. CFRD Management:

    • Insulin aspart: Add 4 units SC before meals (carb ratio 1:15).

  6. Pulmonary Exacerbation Treatment:

    • IV ceftazidime/avibactam: 2.5 g every 8 hours × 14 days (for resistant Pseudomonas).

    • Oral prednisone: 30 mg daily × 7 days (for airway inflammation).

Non-Pharmacologic Interventions

  • Airway Clearance: High-frequency chest wall oscillation vest BID.

  • Nutrition: High-calorie, high-protein diet with CF dietitian consult.

  • Exercise: Referral to CF-specific pulmonary rehabilitation program.

  • Psychosocial Support: Counseling for anxiety related to disease progression.

Follow-Up/Monitoring

  • 2 Weeks: Assess cough, sputum volume, and weight. Check vitamin D and HbA1c.

  • 1 Month: Repeat PFTs; monitor Trikafta side effects (e.g., liver enzymes, rash).

  • 3 Months: Sputum culture, glucose tolerance test, bone density scan (due to low vitamin D).

Education

  • Adherence: Stress airway clearance, enzyme timing with meals, and insulin administration.

  • Infection Prevention: Avoid crowded spaces; hand hygiene; annual flu vaccine.

  • Trikafta Counseling: May cause rash or elevated LFTs; report abdominal pain or jaundice.

Outcome Goals

  • Short-Term: Increase FEV1 to ≥60%, gain 3–5 lbs in 8 weeks.

  • Long-Term: Reduce exacerbations to ≤1/year, achieve HbA1c <7%.

تعليقات

إرسال تعليق

المشاركات الشائعة من هذه المدونة

Tips on how to write a meta-analysis by Dr. Nehad Jaser

  Tips on how to write a meta-analysis   Writing a meta-analysis is a difficult endeavor that demands careful planning, strict methodology, and a clear presentation. A meta-analysis is a statistical approach that integrates the findings of several studies on a single topic or question to produce an overall assessment of the magnitude of the effect or the strength of the association between two variables. A meta-analysis can also investigate the sources of heterogeneity or variance among the studies, as well as assess the validity of various theoretical assumptions or hypotheses. Conducting and publishing a meta-analysis involves numerous steps, and various fields may have different standards and criteria. However, the following is a general framework for producing a meta-analysis: Specify the scope and research query of the meta-analysis. It is essential that the research query be precise, targeted, and pertinent to the field of study. Encompass the criteri...
قراءة المزيد

Centers for Disease Control and Prevention (CDC) categorizes germs into three main categories

The Centers for Disease Control and Prevention (CDC) categorizes germs into three main categories—urgent, serious, and concerning—based on their potential to cause public health threats and the urgency of action required to address them. Additionally, the CDC maintains a watch list of pathogens that are closely monitored due to emerging resistance or other factors. Here's an overview of each category along with examples: Urgent Threats : Urgent threats are pathogens that pose an immediate public health risk due to their high levels of antibiotic resistance, limited treatment options, and potential for widespread transmission. Examples include: Carbapenem-resistant Acinetobacter Drug-resistant Candida Auris Clostridioides difficile Carbapenem-resistant Enterobacteriaceae (CRE) Drug-Resistant Neisseria Gonorrhoeae Serious Threats : Serious threats are pathogens that are moder...
قراءة المزيد

The history of herbal medicine

  The history of herbal medicine is as ancient as human civilization itself, with evidence of medicinal plant usage dating back thousands of years. Herbal medicine, also known as botanical medicine or phytotherapy, involves the use of plants and plant extracts to prevent, alleviate, or treat various ailments. Throughout history, different cultures around the world have developed their own systems of herbal medicine, drawing upon indigenous plants and traditional knowledge. Ancient Civilizations : The roots of herbal medicine can be traced to ancient civilizations such as Egypt, China, India, and Mesopotamia. In ancient Egypt, for example, medicinal plants like aloe, garlic, and castor oil were documented in medical texts like the Ebers Papyrus (circa 1550 BCE). Similarly, traditional Chinese medicine (TCM) has a rich history of herbal remedies, as evidenced by texts like the "Shennong Ben Cao Jing," which dates back to around 300 BCE. Classical Greece and Rome : The Greek phy...
قراءة المزيد