From the Guidelines
A comprehensive treatment plan for infants with cystic fibrosis experiencing recurrent bacterial lung infections should include a combination of medications, respiratory therapy, and pulmonary hygiene, with the goal of reducing morbidity, mortality, and improving quality of life. The plan should address both Staphylococcus aureus and Pseudomonas aeruginosa infections, which commonly affect CF patients. For medication management, systemic antibiotics tailored to sensitivity testing are essential, as recommended by the European Respiratory Journal 1. For Pseudomonas, consider combination therapy with an aminoglycoside (such as tobramycin) and an anti-pseudomonal beta-lactam (like ceftazidime). For Staphylococcus aureus, options include oxacillin or cefazolin, with vancomycin reserved for MRSA. Inhaled antibiotics like tobramycin or colistin can provide targeted therapy with fewer systemic side effects. Respiratory medications should include bronchodilators (Xopenex/levalbuterol), anti-inflammatory agents (Pulmicort/budesonide), mucolytics (Pulmozyme/dornase alfa), and hypertonic saline. Xopenex should be administered before other nebulized medications to open airways and enhance medication deposition, as suggested by the American Journal of Respiratory and Critical Care Medicine 1. Hypertonic saline helps hydrate mucus secretions but may cause bronchospasm, so it's best given after Xopenex. Chest physiotherapy (CPT) is crucial and should be performed 3-4 times daily. CPT techniques include percussion, vibration, and postural drainage to mobilize secretions. For optimal effectiveness, perform CPT after bronchodilators but before hypertonic saline to open airways first, then after hypertonic saline to clear loosened secretions. A daily schedule would include:
- Xopenex at 0600,1200,1800, and 2400
- Pulmicort at 0000 and 1200
- Pulmozyme at 0900 and 1800
- Hypertonic saline at 0630,1230,1830, and 0030
- CPT should be performed at 0700,1300,1900, and 0100, approximately 30 minutes after hypertonic saline to allow time for mucus mobilization. This regimen aims to reduce inflammation, improve airway clearance, and control bacterial infections, ultimately helping to wean the infant from ventilator support and prevent future exacerbations, as recommended by the European Respiratory Journal 1.
The following table summarizes the treatment plan:
| Time | Medication |
|---|---|
| 0000 | Pulmicort |
| 0600 | Xopenex |
| 0630 | Hypertonic saline |
| 0700 | CPT |
| 0900 | Pulmozyme |
| 1200 | Pulmicort, Xopenex |
| 1230 | Hypertonic saline |
| 1300 | CPT |
| 1800 | Pulmozyme, Xopenex |
| 1830 | Hypertonic saline |
| 1900 | CPT |
| 2400 | Xopenex |
| 0030 | Hypertonic saline |
| 0100 | CPT |
From the Research
Treatment Plan for Cystic Fibrosis Infants with Recurrent Bacterial Lung Infections
The treatment plan for cystic fibrosis infants with recurrent bacterial lung infections, particularly those caused by Staphylococcus aureus and Pseudomonas aeruginosa, involves a combination of pulmonary nebulizers, systemic antibiotics, inhaled antibiotics, and pulmonary hygiene measures 2, 3, 4, 5.
- Pulmonary Nebulizers:
- Pulmicort nebulizer every 12 hours at 0000 and 1200 to reduce inflammation.
- Pulmozyme at 0900 and 1800 to help break down mucus.
- Xopenex and hypertonic saline nebs can be used as needed to help open airways and loosen mucus.
- Systemic Antibiotics:
- Used to treat acute infections, with the choice of antibiotic depending on the specific pathogen and its susceptibility pattern 2.
- Inhaled Antibiotics:
- Pulmonary Hygiene:
- Chest physiotherapy (CPT) is a crucial component of pulmonary hygiene, helping to loosen and clear mucus from the lungs.
- CPT can be performed before or after nebulizer treatments, depending on the individual's needs and response.
Chest Physiotherapy
Chest physiotherapy involves a series of techniques to help loosen and clear mucus from the lungs, including percussion, vibration, and drainage. It can be performed manually or with the aid of devices such as vibratory vests. The timing of CPT in relation to nebulizer treatments can vary:
- Before Nebulizer Treatments: Performing CPT before nebulizer treatments can help loosen mucus, making it easier for the nebulized medications to reach the lungs.
- After Nebulizer Treatments: CPT after nebulizer treatments can help clear mucus that has been loosened by the medications.
Schedule
The following schedule can be used as a guide, with adjustments made as needed based on the individual's response and clinical status:
| Time (hours) | Treatment |
|---|---|
| 0000 | Pulmicort nebulizer |
| 0900 | Pulmozyme |
| 1000 | Chest physiotherapy |
| 1100 | Xopenex and hypertonic saline nebs (as needed) |
| 1200 | Pulmicort nebulizer |
| 1800 | Pulmozyme |
| 1900 | Chest physiotherapy |
Considerations
The use of inhaled antibiotics, such as tobramycin, should be considered for chronic suppression of Pseudomonas aeruginosa, with the potential for alternating treatment regimens to improve outcomes 3, 4, 5. Systemic antibiotics should be used judiciously, taking into account the risk of resistance and side effects 2. Regular monitoring of lung function, microbiological cultures, and clinical status is essential to adjust the treatment plan as needed.