Cystic fibrosis (CF) is caused by mutations in the CFTR gene, which encodes for a protein involved in the transport of salts in and out of cells. The resulting defects in the transport of salts across cells lead to the build up of thick and sticky mucus in various organs, affecting their function.

Patients’ airways and lungs are often affected by this abnormal mucus accumulation, and the disease can also cause serious problems with the digestive system.

Symptoms

As the sticky mucus builds in digestive track organs, it can cause intestinal blockages in CF patients, especially in newborns and infants. This condition is called meconium ileus, and may need to be treated surgically. 

This mucus can also block the tubes or ducts within the pancreas, an organ that is responsible for producing both digestive enzymes that aid in the digestion of food and insulin. Without enough digestive enzymes, CF patients may have difficulty breaking down food and absorbing it properly, especially fats and proteins. This can cause patients to not absorb enough nutrients and calories from their food, and also lead to bouts of diarrhea or bulky, greasy stools.

Many CF patients, including children, have poor weight gain and growth because they are unable to get enough nutrients from their food.

Insulin works in the bloodstream to remove glucose or sugar, and transport to cells for energy. Patients who pancreas does not produce enough insulin can develop (insulin deficiency), or they can become insulin resistant. In either case, they are at risk of developing CF-related diabetes.

As the disease progresses, other digestive problems may occur, including:

  • Pancreatitis, where the pancreas becomes inflamed; it can be very painful.
  • Rectal prolapse, where frequent coughing or difficult bowel movements causes rectal tissue to move outside of the  rectum; it may require surgery to treat.
  • Liver disease, caused by inflamed or blocked ducts.
  • Diabetes, caused by insufficient insulin production or insulin resistance
  • Gallstones, or hard deposits of digestive fluid in the gallbladder, which may be painful and cause blockages.
  • Osteoporosis or brittle and weak bones, raising a risk of fractures. Difficulties patients have in absorbing necessary nutrition from their diet can affect their ability to maintain strong bones.

Treating digestive symptoms

A nutritionist can help people with CF and their caregivers ensure that the patient is getting the nutrients they need from their food. A good nutrition plan can also help patients stay active and strong, and better able to resist infections.

In addition to a healthy diet, patients may need:

  • Oral pancreatic enzymes to help digest fats and proteins, and absorb more vitamins
  • Medications to reduce stomach acid, helping the pancreatic enzymes to work better
  • Supplements of vitamins A, D, E, and K to replace the fat-soluble vitamins that CF patients have trouble absorbing
  • High-calorie shakes to provide extra nutrients
  • A high-salt diet or salt supplements that patients can take before exercising or during hot-weather months, as children and others with CF lose considerable salt when they sweat
  • A night-time feeding tube to help provide more calories while the patient is sleeping. A feeding tube may be threaded through the patient’s nose to their stomach, or be surgically connected to a port in the patient’s stomach
  • Enemas and mucus-thinning medications to treat intestinal blockages
  • Surgery to remove intestinal blockages

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Cystic Fibrosis News Today is strictly a news and information website about the disease. It does not provide medical advice, diagnosis or treatment. This content is not intended to be a substitute for professional medical advice, diagnosis, or treatment. Always seek the advice of your physician or other qualified health provider with any questions you may have regarding a medical condition. Never disregard professional medical advice or delay in seeking it because of something you have read on this website.