Pancreatic Enzyme Supplementation in CF Patients Before and After Fibrosing Colonopathy

ABSTRACT & COMMENTARY

Synopsis: There is a strong association between high doses of pancreatic enzyme and development of fibrosing colonopathy.

Source: Stevens JC, et al. J Pediatr Gastroenterol Nutr 1998; 26:80-84.

Nine children diagnosed with fibrosing colonopathy received high doses of pancreatic enzymes. Stevens and associates from the Indiana University School of Medicine reviewed the records of 258 patients with cystic fibrosis (CF) of the pancreas with pancreatic insufficiency and nine additional patients who had fibrosing colonopathy. In the year prior to the diagnosis of fibrosing colonopathy, these nine patients took a significantly larger pancreatic enzyme dose than the children who did not develop this complication. Stevens et al believe that there is a strong association between high doses of pancreatic enzyme supplement and fibrosing colonopathy. These high doses were not necessary for adequate nutrient absorption and growth.

COMMENT BY A. CRAIG HILLEMEIER, MD, FAAP

The vast majority of children with CF have fat malabsorption during the first year of life secondary to pancreatic insufficiency. These children with CF have improved fat absorption when they ingest pancreatic enzyme supplements, but they still fall far short of normal absorption because the lipase activity in the small intestine does not function normally. Approximately five years ago, pharmaceutical companies began to market pancreatic enzyme supplements with much higher amounts of pancreatic enzymes in each capsule (> 20,000 lipase units per capsule). This allowed the child with cystic fibrosis to ingest fewer pills and to achieve higher amounts of lipase. Within a year or two of the introduction of these high lipase enzyme supplements, patients with CF would occasionally present with cramps and bloody diarrhea. On barium enema, these children would often have relatively long colonic strictures (fibrosing colonopathy) and require surgical resection of these areas. Stevens et al examined the clinical characteristics of nine patients with CF who presented with fibrosing colonopathy and compared their course to more than 258 patients with CF who did not have this disorder. They found that the children who developed these inflammatory strictures in the colon were younger and received significantly more pancreatic enzyme, 18,000 units/kg/meal, compared to 8000 units/kg/meal in those who did not develop the colonic strictures. CF patients who received relatively high doses of pancreatic enzyme (12,700 units of lipase/kg/meal) were reduced to a lower dose (2600 units of lipase/kg/meal). There was no change in height or weight growth velocities during the next year.

Fortunately, the self defeating therapy of dietary fat reduction to treat diarrhea in patients with CF is long behind us. Gone also are the days of increasing pancreatic enzyme with disregard to the total dose. The relationship between high pancreatic enzyme dose and the entity of fibrosing colonopathy in patients with CF is well established, even though the precise mechanism is not well understood. The observation that most patients with CF can maintain good growth with 2000-3000 units/kg/meal is also worth noting. While some patients may require higher doses of pancreatic enzyme in order to avoid symptoms of diarrhea, there is good reason to maintain the total dose below 10,000 units/kg/meal. Often, patients are able to optimize their response to pancreatic enzyme therapy at lower doses if they are simultaneously treated with agents to reduce gastric acid secretion (i.e., H2 antagonists).