Preventing constipation in cystic fibrosis

It almost seems surprising that constipation is a major problem for children with Cystic Fibrosis (CF). After all, children and adults with CF take pancreatic enzymes to prevent malabsorption with its bulky, loose bowel movements. 

But it's understandable. Just like the way the thick mucus plugs up the lung and pancreas, the same is true in the intestine. Normally intestinal and digestive juices keep the intestine flowing but in CF, thick mucus contributes to thick bubbly stools that doesn't pass. The blockage is referred to as meconium ileus or the newer term, distal intestinal obstruction syndrome, or DIOS. 

DIOS can usually be prevented or treated using a common laxative, Miralax. When that doesn't clear the blockage, higher doses and other laxative or enemas may be used. Sometimes IV fluids and therapy are needed to dissolve the blockage and relieve the pain and cramping that result. 

Preventing constipation   

Constipation can often be prevented if children with CF by:  

• being well hydrated
• taking their salt supplements 
• eating adequate fiber in their diet
• taking the correct dose of enzymes
• on PEG, if needed

Not enough PERT gives them malabsorption and too much can cause constipation that can be bad enough to need hospitalization or even surgery.