What are the causes of chronic abdominal pain in patients with cystic fibrosis (CF)?

From the Guidelines

Chronic abdominal pain in patients with cystic fibrosis is most commonly caused by distal intestinal obstruction syndrome (DIOS), constipation, and pancreatic insufficiency, as well as other gastrointestinal complications such as gastro-esophageal reflux disease and liver disease 1. These causes can lead to a range of symptoms, including cramping pain, bloating, and discomfort, particularly after meals. Some key causes of chronic abdominal pain in CF patients include:

• Distal intestinal obstruction syndrome (DIOS), which occurs when thick intestinal contents partially or completely block the ileocecal region, causing cramping pain in the right lower quadrant
• Constipation, which presents with more diffuse pain and less acute symptoms
• Pancreatic insufficiency, which leads to maldigestion, bloating, and discomfort, particularly after meals, as noted in patients with virtually absent pancreatic enzyme activity 1 Other significant causes of chronic abdominal pain in CF patients include:
• Small intestinal bacterial overgrowth (SIBO), which develops due to altered gut motility and produces gas, bloating, and pain
• Gastroesophageal reflux disease (GERD), common in CF due to altered esophageal motility
• CF-related liver disease, which can cause right upper quadrant pain Management of chronic abdominal pain in CF patients typically involves treating the underlying cause with appropriate medications, such as pancreatic enzymes for insufficiency, polyethylene glycol or lactulose for constipation, and antibiotics for SIBO, along with proper hydration and dietary modifications 1.

From the FDA Drug Label

Fibrosing Colonopathy: Associated with high doses, usually over prolonged use and in pediatric patients with cystic fibrosis Colonic stricture reported in pediatric patients less than 12 years of age with dosages exceeding 6,000 lipase units/kg/meal. ADVERSE REACTIONS Most Common Adverse Reactions (6.1) Cystic fibrosis adult and pediatric patients: 7 years and older (≥4%): vomiting, dizziness, cough. Chronic pancreatitis or pancreatectomy patients: Adults (≥ 4%): hyperglycemia, hypoglycemia, abdominal pain, abnormal feces, flatulence, frequent bowel movements, nasopharyngitis.

The causes of chronic abdominal pain in patients with cystic fibrosis (CF) may include:

• Fibrosing Colonopathy associated with high doses of pancreatic enzyme replacement therapy
• Colonic stricture reported in pediatric patients less than 12 years of age with dosages exceeding 6,000 lipase units/kg/meal
• Abdominal pain as an adverse reaction to pancrelipase therapy 2 2

From the Research

Causes of Chronic Abdominal Pain in Patients with Cystic Fibrosis

• Distal intestinal obstruction syndrome (DIOS) is a common cause of chronic abdominal pain in patients with cystic fibrosis (CF) 3, 4, 5, 6, 7
• DIOS is characterized by the incomplete or complete intestinal obstruction by intestinal contents in the terminal ileum and proximal colon 3, 7
• The symptoms of DIOS include abdominal pain in the right lower quadrant, a palpable mass on examination, and signs of obstruction on plain radiography 3, 7
• Other causes of chronic abdominal pain in CF patients may include chronic constipation, intestinal blockage, and other gastrointestinal complications 4, 6

Diagnosis and Management of DIOS

• Diagnosis of DIOS is based on suggestive symptoms and confirmed by X-ray or other imaging studies 3, 7
• Treatment of DIOS consists of intensive laxative treatment with oral laxatives and enemas, and in some cases, surgery may be necessary 3, 5, 6
• Prophylactic laxative therapy is widely used to prevent further episodes of DIOS, but its effectiveness is not well established 7
• Management of DIOS requires a multidisciplinary approach, involving gastroenterologists, surgeons, and other healthcare professionals 4, 6