The Cause of Poor Weight Gain in Cystic Fibrosis

Cystic fibrosis is a potentially fatal genetic disorder that affects large numbers of people worldwide. The condition involves the failure of a particular gene to be expressed, known as the cystic fibrosis transmembrane conductance regulator. The failure to express this gene results in the failure of the normal secretion processes, and affects the lungs and pancreas in particular.

Effects of Pancreatic Problems

The pancreas is connected to the digestive tract by means of a duct, and it produces important digestive enzymes that travel through that passage into the duodenum. These pancreatic enzymes are essential for the proper digestion and absorption of nutrients. With cystic fibrosis, however, the pancreatic ducts are blocked, preventing the digestive enzymes from reaching the small intestine.

Also, the thickened secretions harm the pancreas itself, often causing irreparable damage. Beyond a certain extent, the organ will fail entirely, resulting in organ failure and followed soon by death if proper treatment is not forthcoming.

The blocking of the passage leading from the pancreas to the digestive tract leads to malabsorption. This is due to the inability of the digestive tract to break down and assimilate certain important nutrients and minerals without the digestive enzymes produced by the pancreas. Protein and fat in particular can’t be broken down and are not absorbed into the body. Malabsorption is followed by malnutrition, where the body does not get all of the minerals, nutrients, and other compounds that it requires in order to function properly. Younger patients usually suffer from normal growth rates and adults look often thin.

In addition, the disorder may also cause irreparable damage to the islets of Langerhans, which are glands responsible for the production of insulin. Once damaged, the lack of insulin leads to diabetes in patients.

Treatment Options

As mentioned, the only treatment for blocked enzyme release consists of taking pancreatic enzymes with food, especially fat and protein rich food.

Treatment of diabetes caused by cystic fibrosis usually involves injections of insulin, which is nothing more than an attempt to manage the symptoms of the condition. In patients where the genetic disorder has damaged too much of the pancreas, the only treatment option available to patients is an organ transplant in order for the body to continue functioning.

The one treatment method that shows potential to actually cure cystic fibrosis is known as gene therapy. This method of treatment actually involves replacing incorrect genes with correct genes, thereby resolving the problem. Gene therapy has yet to be fully tested, however, and scientists still struggle to find an effective method of inserting the correct copies of genes into the affected cells. Until gene therapy becomes a viable solution, treatment of pancreatic problems simply involves holding off the effects of the disease and keeping the patient healthy for as long as possible, with no hope of curing it.

By: Didier De Coster

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