How CF affects the digestive system

There’s a number of different ways that CF can affect the digestive system.

Pancreas - The pancreas creates digestive enzymes that are released and transported to the digestive system where they are responsible for breaking down fats, proteins and carbohydrates so that they can be properly absorbed into the body. Some people with CF (around 90%) are pancreatic insufficient and this is because the small tubes that transport digestive enzymes out of the pancreas become blocked with mucus. The enzymes build up in the pancreas instead of reaching the digestive system (specifically, the lumen of the gut), causing the pancreas to become inflamed and resulting in malabsorption of fats, proteins and carbohydrates which eventually results in difficulty gaining weight and malnutrition. To rectify this, people with CF take creon micro which is an enzyme supplement with every meal they eat to make sure they can digest the fats, proteins and carbohydrates on their food. This means they must take creon with every breastfeed/formula bottle when they are babies too! As babies, creon is given as granules with an acidic purée like an apple purée. Not ideal at 2am when you have a crying baby and you just want to pop them on your boob!🙈And once they are older, people with CF take creon in the form of pills that contain the granules.

Sometimes one of the first indicators that a baby has CF is that they have very peculiar nappies - they tend to be mucousy (think stringy and slimey!!!). Symptoms of pancreatic insufficiency are as follows:
- Greasy and bulky stools
- Frequent and/or difficult bowel movements
- Constipation
- Nausea
- Swollen abdomen
- Loss of appetite

CF Related diabetes - The problems with the pancreas can also lead to CF related diabetes (CFRD). This is caused by inflammation and scarring of the pancreas which can prevent the effective production of insulin. The latest stats from the CF Trust UK website says that over one third of people with CF go on to develop CFRD. It is tested for throughout childhood and often develops in the teenage years. It’s described as having a mixture of the features from type 1 and type 2 diabetes. This is because the pancreas does not produce insulin effectively as with type 1 diabetes and the body does not respond to insulin in the right way just like type 2 diabetes.

Meconium ileus - One in every ten babies born with cystic fibrosis develops a bowel obstruction called meconium ileus at birth or within the first few days of life. In these cases, the meconium (a thick black material present in the bowels of all newborn babies) is so thick that it blocks the bowel instead of passing through. This results in babies needing urgent surgery to relieve the blockage. Thankfully Arlo was not affected by this but I know many mothers whose babies were rushed into surgery immediately or were blue lighted to hospital in the first few days and then had to spend a few months in hospital whilst their little ones recovered.

Distal intestinal obstruction syndrome (DIOS) - This is kind of like the version of meconium ileus that can happen once people with CF are older. It was previously known as meconium ileus equivalent (MIE). DIOS is a condition that is unique to cystic fibrosis and it results in blockages in the bowel that have to be rectified with medication (such as very strong laxatives) or in rare cases with surgery. It is caused by the build up of mucus along the intestinal tract, slowing down the emptying of food. (You should by now be recognising a theme - it’s all about the build up of mucus just in different parts of the body!) The symptoms of DIOS include stomach pain, constipation, bloating, nausea and weight loss.

GORD (Gastro oesophageal reflux disease) - GORD occurs when stomach acid frequently flows back into the tube connecting your mouth and stomach (esophagus). This backwash (acid reflux) can irritate the lining of your esophagus which can cause burning of the oesophagus or ‘heart burn’. In people with CF it is thought to provoke coughing and possibly contribute to lung damage.

GORD is more common in people with CF because of excessive coughing, obstructive lung disease, delayed emptying of the stomach, high-fat diet, a lax valve at the bottom of the oesophagus and other CF-specific complications.

It can be treated with antacids and other medicine that neutralises the acid in the stomach. But in rare cases it may also require surgery to strengthen the sphincter or valve at the bottom of the oesophagus.