I haven’t done a post on Cystic Fibrosis for a while so thought I would do an explanation of one of the symptoms of Cystic Fibrosis that is not very well know about by a lot of people. When we discovered Joe had cystic fibrosis and we began explaining it to people, many people had heard of cystic fibrosis as a problem with lungs but did not realise about the multitude of other problems involved including pancreatic insufficiency.
As a consequence of CF Joseph suffers from Exocrine pancreatic insufficiency (EPI) which means he can’t digest food properly due to a lack of digestive enzymes made by the pancreas. EPI is caused by damage done to the pancreatic cells that make digestive enzymes. This leads to:
- maldigestion and malabsorption of nutrients
- poor growth
- bone loss
- inability to gain or maintain weight
- shortened lifespan if not treated
EPI causes a lot of nasty side effects including abdominal discomfort (very bad tummy ache), wind, bloating and a swollen tummy, and frequent oily, bulky smelly poops. This is because the body is not digesting and absorbing the vitamins, minerals and fats it needs to survive.
The pancreas is an organ in the digestive system that produces hormones -enzymes lipase, protease, and amylase- that are needed for food digestion in the first part of the small intestine, and bicarbonate to neutralize stomach acid. EPI means that due to damage done to the pancreas (in the case of Joe by sticky mucus) his pancreas cannot produce the hormones needed to digest food.
Almost 50% of infants with CF have EPI at birth, and another 25% develop pancreatic insufficiency within 6 months. An additional 15% will develop EPI sometime in childhood/adolescence or early adulthood. Unfortunately for Joseph he was born with EPI and has been on medication for this since around 6 weeks of age.
Joseph takes Creon Micro with every meal or food substance other than plain fruit and vegetables so that he can absorb fat from the food he eats. He also takes multitudes of vitamins and minerals as he finds it difficult to absorb them as well. You can find out more about Joes medicine here and here.
Before pancreatic enzyme replacement therapy, most children with CF failed to thrive due to maldigestion and inability to absorb nutrients and vitamins which resulted in severe malnutrition, inability to gain or maintain weight, and impaired growth, and ultimately death typically before the age of ten.
Children with CF may require anywhere from 20% to 50% more calories than other children and Joseph’s weight is monitored very closely at the hospital to ensure he does not lose too much weight. We feed him a high calorie diet which would seem very unhealthy to other moms but is helping to keep Joe alive.
Over their lifetime, many patients with CF will also develop diabetes, pancreatitis, liver disease or liver failure, osteoporosis, and heart failure. There are so many problems related to this disease that the general public are not aware of which makes CF such a draining and difficult condition to live with.
It is so much more than just a bad cough and some physio which unfortunately a lot of people think it is!
I hope that this post has given you a little more info about Cystic Fibrosis and the effect it has on the pancreas!