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