Cystic fibrosis enzymes

Cystic fibrosis is a known inherited disease that affects both the respiratory and digestive systems of the sufferer. Individuals are usually diagnosed in childhood because of the symptoms which occur. CF will make children sick through the disruption of normal cell function in the sweat glands, lungs, liver, digestive tract and pancreas.

A defect in the DNA of these cells will cause an imbalance of salt and water in the body. This leads to an increase in mucus clogs the lungs and sets the stage for infections. In the digestive tract, cystic fibrosis will prevent a normal absorption of key nutrients and fat in the intestines, as well as pancreatic insufficiency. This results in poor digestion, slow growth and difficulty gaining weight.

The pancreatic insufficiency means that the enzymes created in the pancreas are not properly passed into the intestines which results in the poor absorption of nutrients. In much the same way that the mucus will block the lungs it also blocks the ducts in the pancreas and doesn\’t allow the enzymes to be delivered to the intestines.

Children and adults with pancreatic insufficiency will require enzymes in pill form to help digest their food appropriately. This will assist their body in gaining the correct nutrition and fat required for growth and weight gain. As children grow their dosage of enzymes will have to be adjusted based on their growth and age. Signs that this adjustment is necessary will include a failure to gain weight, despite a strong appetite, and frequent large bowel movements.

The doctor or dietitian will prescribe the amount of enzymes based on the individual\’s weight, growth and how much is eaten at one time. Enzymes must be taken with every meal and most snacks. They come in capsule form which are full of tiny beads and can be broken open for kids who are unable to swallow the entire capsule. These capsules should not be chewed or crushed and should only be mixed with foods that are acidic, like applesauce.

In some cases, individuals will choose to fast for a variety of reasons. In many physician practices patients will continue to take their enzymes as a prophylaxis against blockage in the intestines. Given every three to four hours it helps to break down the thick sticky mucus in the gut.

Some individuals with cystic fibrosis may continue to show a significant degree of malabsorption despite what appears to be an appropriate amount of enzymes treatment. In this case the primary care physician will recommend a full gastrointestinal investigation looking for other conditions that may be causing problems such as an anatomical abnormality, infection or other gastrointestinal issues.

In April 2004 the US Food and Drug Administration issued a new rule requiring the makers of pancreatic enzymes have their drugs approved by 2010. To receive approval the enzymes must be tested in clinical trials using individuals who have pancreatic diseases, including cystic fibrosis. The goal is to confirm the safety and effectiveness of the enzymes.

There are several different types of enzymes on the market currently which very in the amount of enzymes, how they are made and how many pills must be taken. The goal is to standardize the manufacturing process and ensure the consistency of enzyme from batch to batch. This new rule is currently active and clinical trials are under way.

Pancreatic enzymes were first made before the food and drug administration required testing in clinical trials. These older drugs were allowed to be \”grandfathered\” so they could be put on the market without approval. The Food and Drug Administration understands the importance of pancreatic enzymes for people with cystic fibrosis and while they will allow them to remain available, they are also requiring current clinical trials to prove efficacy and effectiveness.

Pancreatic enzymes are an essential treatment protocol for individuals who suffer from cystic fibrosis and any subsequent pancreatic insufficiency. Without the addition of the enzymes into the intestines with food, individuals are on able to absorb the nutrients, vitamins and minerals necessary to support adequate growth and development.

RESOURCES

Cystic Fibrosis Foundation: Pancreatic Enzyme Replacement

Cystic Fibrosis Foundation: FDA Review of Pancreatic Enzyme Products

Cystic Fibrosis Foundation: FAQs About Phthalates and Pancreatic Enzymes

Kids Health: Cystic Fibrosis and Nutrition

Journal of the Royal Society of Medicine: uses and Abuses of Enzyme Therapy in Cystic Fibrosis

The American Journal of Clinical Nutrition: Fat Malabsorption in Cystic Fibrosis Patients Receiving Enzyme Replacement Therapy