Managing Acute Pulmonary Exacerbation in a Pediatric Cystic Fibrosis Patient: A Case Study Analysis

Write a detailed case review of a pediatric patient with cystic fibrosis, examining the interplay of pulmonary infections, pancreatic insufficiency, and nutritional management.

Case Study 15: Cystic Fibrosis

Patient’s Chief Complaints

T.B.’s mother brings her six-year-old son to the emergency room, concerned about his recent decline in energy. “A week ago, I noticed he was getting tired more easily when playing,” she explains. “But over the last two days, his cough and sputum production have worsened. He started having trouble breathing, so I brought him in immediately. Normally, he’s such a lively, bubbly boy—it’s clear he isn’t feeling well. I’m worried he has another infection.”

History of Present Illness (HPI)

T.B. is a six-year-old Caucasian male with a history of cystic fibrosis (CF), diagnosed at eight months old. Until recently, he had been doing well. Five days ago, his mother noticed he was becoming fatigued much faster than usual while playing. She also observed an increase in his cough, with dark-colored sputum, but no fever. Over the past week, his appetite has declined, leading to a weight loss of 2.5 pounds. His oxygen saturation was recorded at 87%, prompting immediate oxygen therapy via nasal cannula.

Past Medical History (PMH)

T.B. was born at 6 lbs, 7 oz to a 23-year-old mother following an uncomplicated pregnancy and vaginal delivery. Apgar scores at one and five minutes were 8 and 9, respectively. However, at 30 hours old, he developed abdominal distension and bile-stained vomiting, with no bowel movements since birth. A follow-up examination revealed a tense abdomen with faint bowel sounds. Radiographs showed distended loops of bowel without air, leading to an exploratory laparotomy. Surgeons discovered meconium ileus and atresia of the distal ileum, which they successfully resected. Despite an unsuccessful sweat chloride test at that time, he was later diagnosed with CF.

At eight months, T.B. exhibited signs of failure to thrive. He had been eating well but was experiencing frequent, pale, foul-smelling stools. Additionally, he had suffered multiple episodes of bronchitis. On examination, he appeared malnourished, frail, and pale, with minimal subcutaneous fat and a distended abdomen. Crackles were present in both lungs. His sweat chloride test was 99 meq/L, and a stool smear was positive for fat. During hospitalization, he developed pneumonia, requiring intensive intravenous antibiotic therapy. Following recovery, he was referred to a CF specialist center for ongoing management.

Clinical Course

Over the past six years, T.B. has been hospitalized multiple times for respiratory infections and one episode of hemoptysis. His infections required IV antibiotics and extended hospital stays. He was later diagnosed with bronchiectasis and pancreatic insufficiency. His mother administers postural drainage three times daily to help clear mucus. Although some positions are uncomfortable, T.B. never complains. His diet is high-calorie, high-protein, and unrestricted in fat, supplemented with fat-soluble vitamins and iron.

Family and Social History (FH & SH)

• Family History:
  • Mother knew she was a CF carrier before marriage; father was unaware.
  • Maternal uncle died at 16 from pneumonia related to CF.
• Social History:
  • Lives with both parents and attends first grade, where he is doing well.
  • Mother is a stay-at-home parent and a registered nurse; father works as an evening custodian.
  • The home is smoke-free, although the father smokes outside.
  • Household has city water, and there are no pets.

Review of Systems (ROS)

• Respiratory: Increased sputum production, cough, shortness of breath, chest pain when coughing.
• Gastrointestinal: Decreased appetite, no vomiting, no diarrhea or constipation.
• General: Fatigue, weight loss.

Medications

• Respiratory Therapies:
  • Aerosolized tobramycin 300 mg BID
  • Albuterol 2.5 mg via nebulizer TID
  • Dornase alfa 2.5 mg via nebulizer QD
  • Fluticasone propionate 100 μg, 1 puff BID
  • Prednisone 4 mg PO every 6 hours
• Digestive & Nutritional Support:
  • Pancrelipase with meals and snacks
  • Ferrous sulfate 15 mg PO every 8 hours
  • ADEK Multivitamin Pediatric Chewable Tablets BID

Physical Examination (PE)

• General Appearance: Thin, frail, pale six-year-old male, appearing fatigued, gasping for air when oxygen is removed.
• Vital Signs: BP: 105/68 mmHg | HR: 122 bpm | RR: 33 (labored) | SpO2: 95% on 1.5L O2 (88% on room air) | Temp: 98.4°F
• Lungs: Bilateral crackles in upper lobes, wheezing, decreased breath sounds in lower lobes, dullness to percussion in lower lobes.
• Heart: Tachycardic but regular rhythm, normal S1/S2, no murmurs.
• Abdomen: Soft, non-tender, non-distended, bowel sounds present.
• Extremities: Mild digital clubbing, normal strength and range of motion.
• Neurological: Alert, oriented, cooperative.

Diagnostic Findings

• Sputum Culture: Positive for Pseudomonas aeruginosa, Stenotrophomonas maltophilia, Staphylococcus aureus.
• Pulmonary Function Tests: FEV1 at 63% of predicted value.
• Chest X-Ray: Bilateral lower lobe consolidation, consistent with pneumonia.
• Laboratory Results:
  • WBC: 18,900/mm³ (elevated)
  • Hemoglobin: 11.8 g/dL (mild anemia)
  • Vitamin A: 40 mg/dL, Vitamin D: 43 ng/mL, Vitamin E: 0.2 mg/dL (deficiencies noted)

Clinical Questions

1. What is the primary cause of this patient’s CF diagnosis?
2. Why is the patient malnourished?
3. What is the purpose of postural drainage?
4. Which signs indicate developing cor pulmonale?
5. Which nutritional supplements should be prioritized?
6. Which lab findings suggest a bacterial infection?
7. What evidence suggests the absence of cirrhosis or diabetes?

References

References:

1. Rowe, S. M., Verkman, A. S., Denning, G. M., & Cutting, G. R. (2021). Cystic fibrosis. Nature Reviews Disease Primers, 7(1), 5. https://www.nature.com/articles/s41572-020-00239-9
2. Cystic Fibrosis Foundation. (2023). Patient Registry 2022 Annual Data Report. https://www.cff.org/Research/Researcher-Resources/Patient-Registry/2022-Patient-Registry-Annual-Data-Report/
3. Davies, J. C., Alton, E. W., Bush, A., Cuthbertson, L., Daniels, T., Hill, A. T., … & Bilton, D. (2022). Cystic fibrosis. The Lancet, 399(10322), 328-341. https://www.thelancet.com/journals/lancet/article/PIIS0140-6736(20)32531-6/fulltext