June 2014 Critical Care Case of the Month: Acute Exacerbation in Cystic Fibrosis

Seongseok Yun, MD PhD¹ 

Juhyung Sun, BS²

Laura Howe, MD¹

Roberto Bernardo, MD¹

Sepehr Daheshpour, MD¹

Department of Medicine¹

College of Medicine²

University of Arizona

Tucson, AZ 85724

History of Present Illness

A 28 year-old woman with a history of cystic fibrosis, presented with worsening shortness of breath and cough associated with productive secretions. She was diagnosed with cystic fibrosis when she was 14 months old, and has a history of multiple inpatient admissions for acute pulmonary exacerbation of cystic fibrosis. Her most recent hospitalization was a month prior to this admission, and sputum culture demonstrated methicillin-resistant Staphylococcus aureus, multidrug-resistant Pseudomonas aeruginosa, and Achromobacter xylosoxidans. She was treated with linezolide, meropenum, colistin, and azithromycin with significant symptom improvement, then, discharged home with ciprofloxacin, linezolide and zosyn. However, she developed worsening respiratory distress again and came back to hospital. In the emergency department she required 10 L/min of oxygen to maintain an SpO₂ above 90 %.

PMH

• Cystic fibrosis
• Seizure
• Kidney stone
• Portacath placement
• Gastrostomy tube placement

Medications

• Azithromycin 500 mg 3 times a day
• Dornase alpha 1 mg/ml nebulizer twice a day     
• Fluticasone-salmeterol 500-50 mcg/dose inhaler twice a day
• Lipase-protease-amylase 21,000-37,000-61,000 unit 4 caps a day
• Cholecalciferol  2,000 unit capsule daily
• Ferrous sulfate 325 mg PO twice a day
• Ascorbic acid 250 mg PO twice a day
• Oxycodone-acetaminophen  10-325 mg 4 times a day as needed

Social History

• No smoking
• No alcohol use
• No recreational drug use

Physical Examination

Vital signs: Temperature 37.3 °C, heart rate 114 beats/min, respiratory rate 20-24 breaths/min, blood pressure 99/69mmHg, SpO2 88-90 % on 10 L NC

General: Alert and oriented X 3, acutely distressed, tachypneic and dyspneic

Skin: Diaphoretic. No rash or lesions.

HEENT: Unremarkable.

Respiratory: Diffuse rales in all lung fields, no wheezing, no stridor

CVS: Tachycardic, regular rhythm, no murmur.

Abdomen: Soft, non-tender, no tenderness, no guarding, no hepato-splenomegaly, PEG tube placed

Lymphatics: No cervical or axillary lymphadenopathy

Extremities: No clubbing, no cyanosis, no peripheral edema, normal tone, normal range of movement

Neurological: Normal speech, no focal neurologic deficit, CN exam within normal range

Laboratory

CBC: WBC 11.9X 10³ /μL, Hb 9.8 g/dL, Hct 30.7%, Platelets 356,000 /μL.

Chemistries: Na⁺ 137 meq/L, K⁺ 4.1 meq/L, Cl^- 107 meq/L, CO₂ 22 mmol/L, blood urea nitrogen (BUN) 13 mg/dL, creatinine 0.7 mg/dL, glucose 106 mg/dL, calcium 8.0 mg/dL, albumin 2.6 g/dL, liver function tests within normal limits.

Prothrombin time (PT) 14.0 sec, international normalized ratio (INR)1.1, partial thromboplastin time (PTT) 37.2sec

Pulmonary Function Test

FVC 48 % (1.95 L), FEV₁ 36 % (1.25 L), FEF_25-75 14 % (0.55 L/sec)

Radiography

An old chest x-ray and thoracic CT scan were reviewed (Figure 1).

Figure 1. Previous PA (Panel A), lateral (Panel B) chest x-ray and representative image from the thoracic CT scan (Panel C).

Which of the following are findings of cystic fibrosis on chest x-ray? (Click on the correct answer to move to the next panel)

1. Bronchiectasis
2. Hyperinflation
3. Lobar collapse
4. Prominent pulmonary arteries
5. All of the Above

Reference as: Yun S, Sun J, Howe L, Bernardo R, Daheshpour S. June 2014 critical care case of the month: acute exacerbation in cystic fibrosis. Southwest J Pulm Crit Care. 2014;8(6):305-19. doi: http://dx.doi.org/10.13175/swjpcc047-14 PDF

March 2014 Critical Care Case of the Month: Interstitial Lung Disease

Seongseok Yun, MD PhD

Konstantin Mazursky, DO

Kahroba Jahan, MD

Enas Al Zaghal, MD

Department of Medicine

University of Arizona

Tucson, AZ 85724

History of Present Illness

An 80 year-old man with a history of chronic obstructive pulmonary disease, asbestosis and interstitial lung disease, presented to the outpatient clinic with cough, sinus congestion and mild sputum. He was sent home with amoxicillin for the treatment of a sinus infection. However, he came back to emergency department with worsening respiratory symptoms including shortness of breath and persistent cough. He required 8-10 L/min of oxygen to maintain an oxygen saturation above 90 %.

PMH

• COPD
• Asbestosis
• Interstitial lung disease
• Diabetes mellitus,  type II
• Hypertension
• Aortic valve replacement

Medications

• Fluticasone-salmeterol 250-50 mcg inhaler
• Mometasone 50 mcg/actuation nasal spray
• Furosemide 40 mg PO daily
• Felodipine 5 mg PO BID
• Warfarin 3 mg PO daily
• Insulin aspart 5 units SC injection before meals
• Insulin glargine 15 units SC injection night time

Social History

• 50 pack-year prior smoking history
• No ethanol or recreational drugs
• No recent travel history      

Physical Examination

Vital signs: temperature 37.2 °C, pulse 116 beats/min, respiratory rate 32-34 breaths/min, blood pressure 179/77 mmHg, SpO2 90 % on 10 L/ min non-rebreathing mask (NRB).

General: Alert and oriented but appearing distressed, tachypneic and dyspneic

Skin: Diaphoretic, no rash or lesions noted.

HEENT: Unremarkable.

Respiratory: Diffuse rales but no wheezing or stridor.

CVS: Tachycardic, regular rhythm, soft systolic murmur.

Abdomen: Soft, non-tender, no tenderness, no guarding, no hepato-splenomegaly

Lymph: No cervical lymphadenopathy

Extremities: No peripheral edema, normal tone, normal range of movement  

Laboratory

CBC: WBC 20.2 X 10³ /μL, hemoglobin 9.3 g/dL, hematocrit 29.8 %, platelets 272,000/μL.

Chemistries: Na⁺ 134 meq/L, K⁺ 4.8 meq/L, Cl^- 108 meq/L, CO₂ 19 mmol/L, blood urea nitrogen (BUN) 58 mg/dL, creatinine 1.7 mg/dL, glucose 272 mg/dL, calcium 9.7 mg/dL, albumin 2.2 g/dL, liver function test-within normal limits.

Prothrombin time (PT) 28.0 sec, international normalized ratio (INR) 2.5, partial thromboplastin time (PTT) 44.5 sec

An old chest x-ray and CT scan were reviewed (Figure 1).     

Figure 1. Old PA (panel A) and lateral (Panel B) and representative image from an old CT scan (panel C).

Which of the followings are the findings of asbestos related disease on chest x-ray? (click on correct answer to move to next panel)  

1. Reticular or patchy opacity
2. Calcified pleural plaque
3. Bilateral consolidation
4. Pulmonary edema
5. 1 + 2
6. 3 + 4

Reference as: Yun S, Mazursky K, Jahan K, Al Zaghal E. March 2014 ciritcal care case of the month: interstitial lung disease. Southwest J Pulm Crit Care. 2014;8(3):152-60. doi: http://dx.doi.org/10.13175/swjpcc013-14 PDF