Exocrine pancreatic insufficiency is treated with enteric-coated pancreatic enzyme capsules, which contain lipase and proteases. Patients with CF are encouraged to follow high-calorie diets, often with the addition of nutritional supplements. Even with optimal pancreatic enzyme replacement, stool losses of fat and protein may be high. Fat should not be withheld from the diet, even when significant steatorrhea exists. Rather, pancreatic enzyme doses should be titrated to optimize fat absorption, although there is a limit to the doses that should be used. Lipase dosages exceeding 2500 U/kg/meal are contraindicated because they have been associated with fibrosing colonopathy. Fat-soluble vitamins (A, D, E, and K) are recommended, preferably in a water-miscible form.
Newborns with meconium ileus may require surgical intervention, but some can be managed with contrast (Gastrografin) enemas. Intestinal obstruction in CF patients beyond the neonatal period is often due to distal intestinal obstruction syndrome (DIOS), which may need to be treated with courses of oral laxatives (polyethylene glycol) or, in more refractory cases, with balanced intestinal lavage solutions. Pancreatic enzyme dosage adjustment, adequate hydration, and dietary fiber may help prevent recurrent episodes. Patients with CF-related diabetes are treated with insulin, primarily to improve nutrition and prevent dehydration, as ketoacidosis is rare. Although transaminase elevation is common in patients with CF, only 1% to 3% of patients have progressive cirrhosis resulting in portal hypertension. Cholestasis is treated with the bile salt ursodeoxycholic acid. Portal hypertension and esophageal varices due to cirrhosis of the liver are managed, when necessary, with portal vein shunting procedures or liver transplantation. Patients with symptomatic sinus disease and nasal polyps may require sinus surgical procedures.
The management of CF is complex and is best coordinated by personnel at accredited CF centers. As with other severe chronic diseases, management of CF patients requires a multidisciplinary team working with patients and their families to maintain an optimistic, comprehensive, and aggressive approach to treatment.
Thoracic scoliosis, when it is severe (curve >60°), can be associated with chest wall deformity and limitation of chest wall movement. This, in turn, can lead to decreased lung volumes (restrictive lung disease), ventilation/perfusion mismatching, hypoventilation, and even respiratory failure (see Chapter 202).Surgical correction of scoliosis may prevent further loss oflung function, but it rarely improves pulmonary function above presurgical levels.
Sternal concavity (pectus excavatum), a common chest wall deformity in children, is usually not associated with significant pulmonary compromise. However, occasionally, if severe, it may result in restrictive lung disease, obstructive defects, and/or decreased cardiac function. Often it comes to medical attention because of concerns over the appearance of the chest. Adolescents with pectus excavatum may complain of exercise intolerance. Routine spirometry is often normal but may show decreased vital capacity consistent with restrictive lung disease. The main reason for surgical correction is generally to improve appearance (cosmetic reasons), although in some cases surgical repair is justified to improve cardiac function and exercise tolerance.
Pectus carinatum is an abnormality of chest wall shape in which the sternum bows out. It is not associated with abnormal pulmonary function. Underlying pulmonary disease may contribute to the deformity. It can be observed after cardiac surgery performed via midsternal approach. Surgical correction of this condition is rarely indicated, but occasionally is done for cosmetic purposes.