Abstract
This chapter reviews the pathophysiology of cystic fibrosis and its effects on a host of organ systems. The author discusses the anesthetic implications of cystic fibrosis allowing the reader to formulate a safe perioperative plan for these patients.
A nine-year-old girl with cystic fibrosis and recurrent pulmonary infections is scheduled to undergo repair of an umbilical hernia. Her home treatment regimen includes chest physiotherapy, albuterol, tobramycin, and nighttime humidified continuous positive airway pressure (CPAP), 8 mmHg.
Preoperatively her vital signs include: blood pressure 105/68; heart rate 86, respiratory rate 20; temperature 36.8°C SpO2 97% on room air. Physical exam revealed a very thin young girl, cooperative with history and physical. Her respiratory effort is normal without the use of accessory muscles or retractions. She had coarse breath sounds bilaterally on auscultation. Airway exam was unremarkable.
What Genetic Factors Are Associated with Cystic Fibrosis?
Cystic fibrosis is caused by a mutation in the gene that encodes for the cystic fibrosis transmembrane regulator (CFTR) protein. This is a chloride channel that is found at the apical border of epithelial cells lining most exocrine glands. The mutation is the most lethal inherited disorder and displays an autosomal recessive pattern of inheritance.
What Are the Pulmonary Manifestations of Cystic Fibrosis?
Cystic fibrosis affects numerous organ systems although the primary cause of morbidity and mortality is related to pulmonary manifestations. Copious and thick secretions combined with mucociliary dysfunction lead to airway inflammation, atelectasis, and bacterial trapping. Bacterial overgrowth and inflammation cause biofilm formation and recurrent infection. With disease progression, airway obstruction from secretions causes further air trapping resulting in bronchiectasis. Patients are plagued by recurrent exacerbations leading to progressively reduced lung function, cor pulmonale, and hypercarbic, hypoxemic respiratory failure.
What Are the Nonpulmonary Clinical Manifestations of Cystic Fibrosis?
Common organ systems affected include: gastrointestinal, and genitourinary, endocrine, bone, skin, and reproductive systems (Table 13.1).
Table 13.1 Pathologic and clinical manifestations in cystic fibrosis by organ system
| Organ system | Pathology | Clinical manifestation |
|---|---|---|
| Upper respiratory tract | Copious nasal secretions | Chronic sinusitis, nasal polyps |
| Lower respiratory tract | Mucoid secretions, mucociliary dysfunction | Recurrent infections, bronchiectasis, chronic hypoxemia |
| Cardiac | Cor pulmonale | Right ventricular hypertrophy |
| Hepatobiliary | Bile duct obstruction | Biliary cirrhosis, portal hypertension |
| Gastrointestinal | Intestinal secretions | Neonatal meconium ileus, recurrent intestinal obstruction, malabsorption (vitamins A,D,E,K) |
| Endocrine | Obstruction and fibrosis of pancreatic ducts | Impaired pancreatic exocrine function and induced diabetes |
| Reproductive | Abnormal cervical secretions (females) Absence of vas deferens (males) | Decreased fertility (females and males) |
| Integumentary | Increased chloride levels in skin | Impaired thermoregulation |
| Bone | Impaired calcium, vitamin D absorption, increased bone catabolism | Early onset osteoporosis |
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