Based on a case presented in September 2009
Glenda N. Bendiak
Pediatric Respiratory Medicine PGY5
Hospital for Sick Children
University of Toronto
A 12 year old girl was referred to the Hospital for Sick Children for lung transplant assessment. She had been diagnosed with cystic fibrosis at ten weeks of age following a history of failure to thrive, and severe left lower lobe pneumonia requiring admission to the Intensive Care Unit.
In regards to her respiratory history, she had had numerous admissions for respiratory exacerbation. Four admissions had occurred between 1997 to 2007, but in the year prior to referral, she had had six admissions. Her FEV1 percent predicted was in the mid-60s at the first measurement in 2001, but was only 24% predicted at the time of referral for transplant assessment. In 2008, during an admission, she was found to have oxygen saturations in the mid-70s in sleep, and thus supplemental oxygen was initiated. She had no history of allergic bronchopulmonary aspergillosis or air leak.
On review of her sputum microbiology, she had previously grown Pseudomonas aeruginosa, Burkholderia cepacia complex (genomovar IV), Staphylococcus aureus, Stenotrophomonas maltophilia, Aspergillus and Candida.
On review of her other systems, she had a history of poor weight gain, and had had a gastrostomy tube inserted in 2008 to optimize her nutrition. She had no history of distal intestinal obstructive syndrome, CF-related liver disease, CF-related diabetes, sinus disease or nasal polyps. She was on numerous medications including inhaled fluticasone, inhaled salbutamol, inhaled tobramycin, inhaled DNase, and replacement of pancreatic enzymes, and fat-soluble vitamins.
On examination, her weight was 28 kg (< 3 %ile), and height was 137 cm (< 3 %ile). Vital signs included heart rate of 80, respiratory rate of 20, and oxygen saturation 85% in room air. Coarse crackles were heard throughout lung fields, and she had indrawing and tracheal tug at rest. Cardiac examination revealed normal S1 and S2, normal P2, with no murmur heard. The liver edge was palpable, with no splenomegaly.
The next screen shows her pulmonary function test results
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