Internal Medicine

0521779407-C04 CUNY1086/Karliner 0 521 77940 7 June 14, 2007 20:37

Cystic Fibrosis 451

General Measures

■Adjust or avoid potentially hepatotoxic drugs

■Optimize pulmonary function; optimize nutritional status in those

with pancreatic insufficiency

■Evaluate stage of liver disease

■Sweat chloride test if diagnosis is not established

specific therapy

Indications for Treatment

■Medical therapy to improve nutritional status

■Liver transplantation for decompensated cirrhosis

Treatment Options

■Pancreatic enzyme replacement: lipase PO with each meal or snack

if pancreatic-insufficient

■Ursodeoxycholic acid: improves liver biochemistry and may improve

liver histology in early disease

■Liver transplantation: patients with end-stage liver disease and mild

pulmonary involvement

follow-up

During Treatment and Routine

■Monitor patient’s nutritional status, LFTs every 3 to 6 months

■Closely monitor whole blood cyclosporine levels after liver trans-

plantation; intestinal malabsorption and altered drug metabolism

may necessitate higher dosages

complications and prognosis

■Gallstones: in up to 25%; consider cholecystectomy if symptomatic

■Hepatic steatosis: frequently seen; improved nutrition appears to

reduce incidence

■Cirrhosis: develops in up to 8–10% of all cystic fibrosis patients at

a median age of 10 years; accounts for almost all non-pulmonary

causes of mortality

■Pulmonary: see Pulmonary section

■Pancreatic insufficiency: frequent

Prognosis

■Median life expectancy almost 30 years