Patient Case: Cystic Fibrosis

PATIENT CASE: Cystic Fibrosis CC: Patients reports she is “experiencing shortness of breath, fast breathing, increasing cough and sputum production, and decreased energy, and she has a poor appetite” HPI: JL is a 19-year-old girl with a lifetime history of CF; she was diagnosed with CF at birth after presenting with meconium ileus. She had been doing well until 4 weeks ago, when she developed cold-like symptoms, with a runny nose, dry cough, sore throat, and subjective fever. She was seen by her primary care physician and prescribed a 5-day course of azithromycin 500mg po on Day 1 and 250mg po on Days 2-5 for possible pneumonia. After completing the antibiotic course, JO was not feeling any better. She called the pulmonary clinic regarding her symptoms and her pulmonologist called in a prescription to a local pharmacy for ciprofloxacin 500mg po BID, prednisone 40mg po daily, and increase her hypertonic saline schedule from one per day to twice daily with her vest treatments. The patient now presents to the pulmonary clinic for a follow-up to her outpatient treatment course. She describes worsening shortness of breath and chest pain, lung and sinus congestion, poor appetite, and severe fatigue. She reports increasing cough productive of very dark green sputum but no fever. The patient has lost 2 lb since her last clinic visit and has missed 7 days of work. Her oxygen saturation is 88% in clinic on room air, and she was immediately placed on 1 L of O2 by nasal cannula. PMH: CF (∆F508/G551D) 7 hospitalizations for APE of CF 2 hospitalizations for DIOS Pancreatic insufficiency Poor nutritional status Recurrent constipation/DIOS Seasonal allergies Asthma Hypovitaminosis D Clavicle fracture after falling from tree 2 months ago PSH: Sinus surgery x2, last 1 year ago FH: Father has hypercholesteromlemia Younger sister (age 2) with CF who was recently diagnosed with RSV bronchiolitis Two maternal uncles died at ages 13 and 17 from CF SH: Lives with mother, father, and younger sister approximately 50 miles from the nearest CF center. Father smokes but only outside of the home. Family is experiencing financial difficulties due to a job layoff and recently lost health insurance. Family is in the process of applying for state Medicaid assistance. MED: Ciprofloxacin 500mg po BID Prednisone 40mg po daily Inhaled tobramycin (TOBI podhaler) 300mg BID (every other month, currently “on”) Albuterol inhaler and nebulizers q4hr PRN with vest therapy Azithromycin 500mg po qMWF Dornase alfa (Pulmozyme) 2.5mg nebulizer once daily Sodium chloride 7% aerosol (Hyper-Sal) 4mL via nebulizer once daily Dulera 100mcg/5mcg 1 puff BID Loratadine 5mg po once daily Creon 12,000 2 caps with meals, 1 cap with snacks Omeprazole 40mg po once daily Ferrous sulfate 324mg po BID Cholecalciferol 5,000 units po once daily Fluticasone nasal spray 1 spray in each nostril daily ADEKs two tablets po once daily Polyethylene glycol 17 grams po once daily Ibuprofen 200mg po q4-6 hours PRN chest pain Vaccines: Routine pediatric immunizations complete Influenza vaccine received annually, most recent dose 10/5/2014 Allergies: Codeine (itching) Strawberries (anaphylaxis) ROS: Patient complains of chest pain when coughing and large amounts of expectorated green sputum. Reduced ability to perform usual daily activities and play because of SOB. No current hemoptysis, constipation, vomiting, or abdominal pain. Reports having 3-4 loose or partially formed stools each day. Patient usually has a large appetite but has not been able to finish a meal for the past week. PE GEN: Shy, thin, cooperative 19-year-old girl who has SOB with her oxygen cannula removed during examination VS: BP 100/65, HR 100, RR 35, T 37.8°C, Wt 40kg, Ht 5’0’’, oxygen saturation 95% on 1 L of oxygen, 88% RA HEENT: EOMI, PERRLA, nares with dried mucus in both nostrils, sinuses tender to palpation, no oral lesions but secretions noted in posterior pharynx LUNGS: Crackles heard bilaterally in upper lobes greater than in lower lobes, mild scattered wheezes, chest pain not reproducible with palpation CV: Tachycardic, regular rate without murmurs GI: (+) bowel sounds, abdomen soft/supple, mild bloating noted Other PE deferred LAB: BMP (CHEM-7) + Alb/Mag/Phos Na 149 mEq/L K 4.5 mEq/L Cl 108 mEq/L CO2 24 mEq/L BUN 18 mg/dL SCr 0.5 mg/dL Glu 195 mg/dL Alb 3.1 g/dL Phos 4.6 mEq/L Mg 2.1 mg/dL CBC Hgb 15.4 g/dL Hct 45.2% WBC 16.5 x 109/L LFTs AST 30 IU/L ALT 20 IU/L Total bilirubin 0.1 mg/dL Bands 10% Virology/Serology Results: Respiratory viral antigen panel: negative Influenza A/B PCR: negative B. pertussis PCR: negative Sputum Culture Results: Organism A: Pseudomonas aeruginosa Sensitive: piperacillin/tazobactam , ticarcillin/clavulanate, cefepime, ceftazidime, meropenem, aztreonam, tobramycin, amikacin Intermediate: ciprofloxacin Resistant: gentamicin Organism B: Pseudomonas aeruginosa, mucoid strain Sensitive: piperacillin/tazobactam, ticarcillin/clavulanate, cefepime, ceftazidime, meropenem, aztreonam, tobramycin Resistant: ciprofloxacin, gentamicin, amikacin Organism C: Staphylococcus aureus Sensitive: vancomycin, linezolid, trimethoprimsulfamethoxazole, minocycline Resistant: nafcillin, cefazolin, clindamycin, erythromycin PFTs: FEV1 65% of predicted, baseline is 80% Chest x-ray: Interstitial fibrotic changes consistent with CF High-resolution chest CT (HRCT): interval worsening of bronchiectasis in all lobes, increased mucus plugging in left lower lobe Instructions: Work up this patient USING SOAP FORMAT to submit on Examsoft and Canvas.