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Clinical Considerations for Individuals with Cystic Fibrosis

Reviewed By Critical Care Assembly

Submitted by

Kriti Lonial, MD

Fellow

Division of Pulmonary, Critical Care and Sleep Medicine

University of Southern California, Keck School of Medicine

Los Angeles, CA

Pooja Patel, MD

Division of Pulmonary, Critical Care and Sleep Medicine

University of Southern California, Keck School of Medicine

Los Angeles, CA

A. Purush Rao, MD

Assistant Professor of Clinical Medicine

Division of Pulmonary, Critical Care and Sleep Medicine

University of Southern California, Keck School of Medicine

Los Angeles, CA

Submit your comments to the author(s).

History

A 38 year-old woman, with cystic fibrosis/bronchiectasis (genotype F508/F508) presented to the emergency department for sudden onset massive hemoptysis. She accounted an approximate volume of 6-8 ounces of blood.  She denied any associated chest pain, worsened productive sputum or dyspnea prior to the massive hemoptysis episode.  2 hours later, she complained of a non-radiated, stabbing right-sided chest pain with an intensity of 3 out of 10 level.

Her past medical history included bronchiectasis, cystic fibrosis associated pancreatic insufficiency, anxiety and recurrent massive hemoptysis requiring prior bronchial artery embolization (BAE) up to 37 times during her lifetime. The most recent BAE prior to the current episode was 6 weeks prior.

She was a lifetime non-smoker, with no history of illicit drug or alcohol use.

Her medications included fat soluble vitamins (ADEK), pancreatic enzyme supplements, recombinant DNase and hypertonic saline.  She denied any changes to her regimen prior to the most recent hemoptysis episode.

Physical Exam

A 38 year-old woman, with cystic fibrosis/bronchiectasis (genotype F508/F508) presented to the emergency department for sudden onset massive hemoptysis. She accounted an approximate volume of 6-8 ounces of blood.  She denied any associated chest pain, worsened productive sputum or dyspnea prior to the massive hemoptysis episode.  2 hours later, she complained of a non-radiated, stabbing right-sided chest pain with an intensity of 3 out of 10 level.

Her past medical history included bronchiectasis, cystic fibrosis associated pancreatic insufficiency, anxiety and recurrent massive hemoptysis requiring prior bronchial artery embolization (BAE) up to 37 times during her lifetime. The most recent BAE prior to the current episode was 6 weeks prior.

She was a lifetime non-smoker, with no history of illicit drug or alcohol use.

Her medications included fat soluble vitamins (ADEK), pancreatic enzyme supplements, recombinant DNase and hypertonic saline.  She denied any changes to her regimen prior to the most recent hemoptysis episode.

Lab

WBC: 6,200 per mm3

Hematocrit 37%

Platelet: 357,000 per mm3

PT/INR: 10.2/0.9

Electrolytes and liver function tests were normal

BUN was 10 mg/dL , and serum creatinine was 0.6 mg/dL

Sputum culture revealed mucoid pseudomonas aeruginosa, no burkholderia cepacia or staphloccus aureus

Sputum for AFB and fungi were negative

Figures


Figure 1: Chest radiograph showing an increased opacity in the right upper lobe

Figure 2: Chest computed tomography showing bronchiectasis and right upper lobe opacity suggesting the site of bleed

Question 1

All of the following are indications of lung transplant except:

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