The GI System

The management of most causes of abdominal pain in CF is similar to the general population. Pancreatitis, GORD, gastritis/peptic ulcer disease, variceal bleeding and biliary disease are managed in line with recommendations for non–CF patients [4].

Non-opiate analgesia should be used whenever possible because opiates can precipitate DIOS. Antiemetics should be considered when appropriate.

Abdominal pain, which is common in CF, is often associated with anorexia and nausea; consequently, these patients are at risk of dehydration, which must be prevented or corrected if present; in particular, dehydration worsens DIOS.

Special gastrointestinal scenario in CF – Distal Intestinal Obstruction Syndrome (DIOS)

Different CF centres may have their own protocols for managing DIOS.  The goals of management are early recognition and prompt treatment to avoid the need for surgical intervention [4].

Mild cases, as well as uncomplicated constipation often respond to laxatives such as Lactulose, Senna or Movicol®. Careful attention to adequate hydration is important.

Oral or nasogastric diatrizoate (Gastrograffin®), N-acetylcysteine (NAC) or PEG solution is used to thin bowel contents4. NAC acts as a mucolytic and can help break up the protein matrix of the inspissate.

In more severe cases, phosphate enemas or Gastrograffin® directed into the lumen of the ascending colon by means of either an enema or colonoscopy may be successful.

If there are signs of peritoneal irritation or complete intestinal obstruction, then a surgical review is warranted. The patient should be kept NPO – other than a small dose of pancreatic enzymes every 3-4 hrs to avoid further obstruction. IV fluids and nasogastric (or PEG) drainage should be instituted [24].

Learning Bite

DIOS is managed medically in most cases, with surgical treatment generally resulting from failed, delayed or inappropriate medical therapy. CF Patients who are Nil by Mouth should still have a small dose of pancreatic enzyme, even in the post-op period.