Ms. M is a 26-year-old woman who presented to the pulmonary clinic for the first time with complaints of dyspnea on exertion and a productive cough. She reports a productive

cough for many years and periodically developed yellow or green sputum and occasional hemoptysis. These symptoms were attributed to Bronchiectasis. When she was a child, her parents were told she had asthma. She also had many childhood respiratory infections requiring hospitalization. At age 11, she was told she had Bronchiectasis period since that time her therapy included inhaled albuterol and occasional antibiotic therapy. She had no regular pulmonary hygiene program. Three weeks before admission, an increasingly severe cough producing greenish sputum developed. She noticed increasing dyspnea on exertion and wheezing. Her family physician gave her a prescription for ciprofloxacin 750 milligrams twice daily, but her symptoms progressed despite this therapy. Her current symptoms increased to such a degree that she required sick leave from her job as a secretary. This is the third episode Ms. M has experienced in the last year. Her previous episodes were successfully treated with oral ciprofloxacin. She chronically heard wheezing and crackles with breathing, particularly when she went to bed at night. She also had difficulty maintaining weight and felt she was far below her ideal weight. She did not have fever, chills, pharyngitis, cordon, or purulent nasal discharge. She had no problems with steatorrhea (fatty stools), nasal polyps, or sinus infections. Ms. M never smoked. Her father had a chronic problem with Bronchiectasis that began as a teenager. Ms. M had been tested for cystic fibrosis using a sweat chloride concentration when she was first diagnosed with bronchiectasis at age 11. Her sweat chloride concentration was 57 mEq/L. 1. What features of this patient's history support the diagnosis of cystic fibrosis? 2. Which parts of Ms. M's history suggest that she does not have cystic fibrosis? 3. Does her sweat chloride concentration help to clarify whether or not she has cystic fibrosis? 4. What findings would you expect on physical examination if this patient has cystic fibrosis? 5. What additional testing would be appropriate to support or refute the diagnosis of cystic fibrosis? More on Ms. M... ● ● ● ● ● ● General: A thin, young, Caucasian woman with a productive cough; Uses accessory muscles of respiration that appears to be breathing comfortably and can converse without apparent dyspnea. Vital signs: Temperature 37.2°C (99.0°F) pulse 71/min, Respiratory rate 19/min, Blood pressure 105/72 mmHg. HEENT: nasal polyps seen in left nare; No sinus tenderness noted; Oral examination normal and mucous membranes moist; Eye and ear examinations normal. Chest: AP dimensions of chest increased; Diffuse inspiratory and expiratory crackles with polyphonic expiratory wheezing heard over both lungs; Hyperresonance noted with chest percussion. Heart: Regular rate with loud P2 component of S2 noted on cardiac auscultation; No murmurs or gallops noted; Point of maximum impulse (PMI) difficult to appreciate but located in the 5th interspace 2 to 3 cm lateral to sternal border. Abdomen: Bowel sounds active, with no guarding or tenderness; Liver 8 cm in the midclavicular line; No masses palpated. Extremities: Digital clubbing, but no cyanosis or edema; Pulses equal and symmetrical. 6. What is this significance of Ms. M's underweight appearance? 7. What is a nasal polyp, and what respiratory problems may it create? 8. What is indicated by an increase in the AP dimension of the chest? 9. What is the significance of the crackles heard over the lungs? 10. This patient has digital clubbing. What does clubbing of the fingers look like, and what may it indicate? ABGS (on room air) ABG pH Paco₂ Pao₂ HCO, P(A-a)0₂ O₂ content Complete Blood Count: WBCs/mm³ Red blood cells (M/mL) Hemoglobin (g/dL) Hematocrit (%) Differential Segmented neutrophils (%) Band neutrophils (%) Lymphocytes (%) Monocytes (%) Eosinophils (%) Basophils (%) Chemistry: Na* (mEq/L) K* (mEq/L) CI (mmol/dL) HCO, (mm/L) BUN (mg/dL) Creatinine (mg/dL) Calcium (mmol/L) Phosphate (mg/dL) Uric acid (mg/dL) Albumin (g/dL) Protein (g/dL) Value 7.42 34 mm Hg 67 mm Hg 21 mEq/L 39 mm Hg 17.5 mL/dL Observed 12,400 4.7 14.2 43 2 12 13 72 1 1 Observed 138 4.6 106 20 13 0.7 2.1 2.7 3.4 3.1 6.0 Normal 4,000-11,000 4.1-5.5 14-16.5 37-50 38-79 0-7 12-51 0-10 0-8 0-2 Normal 136-146 3.5-5.1 98-106 22-29 7-18 0.5-11 2.1-255 2.7-4.5 4.5-8.2 3.5-5.0 6.4-8.3 15 MAR 91 IYO B A 11. How would you interpret the ABG's? 12. Why are the WBC count and the number of band neutrophils elevated? 13. What additional diagnostic tests would be important to obtain? 14. If the physician decides to admit this patient to the hospital, what respiratory therapy orders would you suggest? ● FIGURE 5.4 Chest radiograph of Ms. M. (A) PA film. (B) Lateral view. Ms. M fails to improve on oral antibiotics, and her physician admits her to the medicine service for more aggressive treatment. The admitting orders include the following: ● Supplemental vitamins A, D, E, and K Aminophylline 1 g in 0.25 L normal saline: an initial IV infusion of 5 mg/kg over 30 minutes, then constant infusion at a rate of 0.5 mg/kg/hr Piperacillin IV 1g Q 6 hours. Chest physiotherapy and postural drainage Q 6 hours ● ● ● ● ● Theophylline level on the second hospital day Amikacin level before and after the third dose of amikacin Sputum Gram stain and culture Sweat chloride concentration Gene probe for the delta F508 gene 15. The admitting physician believes Ms. M may have cystic fibrosis. Why did the physician prescribe the supplemental A, D, E, and K vitamins? 16. What is albuterol? What are the potential side effects? 17. Is the prescribed chest physiotherapy and postural drainage important for this patient, or should you recommend that it be discontinued? Why? 18. If this patient is found to have cystic fibrosis, what organisms would you expect to find on the sputum culture? 19. What effect does inhaled DNase have on the sputum of patients with cystic fibrosis? Ms. M CONCLUSION... Ms. M is hospitalized for 5 days. During this time, she receives the antibiotics, bronchodilators, inhaled DNase, and chest physiotherapy. Her symptoms improved greatly, showing marked reduction in coughing and dyspnea. She produces far less sputum, and the color is light yellow. She is trained to administer her own IV antibiotics and to perform postural drainage; her husband is trained to deliver chest percussion. Ms. M is discharged with a follow-up appointment with a pulmonary specialist.