Exocrine pancreatic insufficiency

An inability to break down and in turn digest food properly is Exocrine Pancreatic insufficiency (EPI). In other words, a deficiency of the exocrine pancreatic enzymes leads to maldigestion or inability to digest food.

Exocrine pancreatic insufficiency or EPI can cause problems in how we digest our food. If our pancreas do not make enough enzymes that the body needs to break down and absorb nutrients, then EPI occurs. Enzymes speed up chemical reactions in our body. The enzymes made by our pancreas move into the small intestine, where they help break down the food we eat.

So much so, that a person who suffers from EPI does not get enough nutrients as the body cannot absorb fats, vitamins or minerals from food. That is the reason why he/she loses weight or has pain in the stomach. Many are prescribed drugs that help substitute a new supply of enzymes, so that they can get back to digesting food the right way. The exocrine pancreas produce three types of enzymes namely amylase, protease and lipase.

Symptoms of EPI

Symptoms of EPI can vary, but the doctor should be contacted if one or more of these signs persist as these symptoms could also possibly arise due to GI condition. A person with EPI may not exhibit all the symptoms first but once the pancreas gets damaged, it starts to hurt due to inability to absorb fat; some symptoms may appear:

Frequent diarrhea: EPI can cause diarrhea as undigested food moves too quickly through the digestive tract.

Weight loss: As those with EPI cannot digest fats, proteins and carbohydrates in the food they consume, this can result in weight loss.

Steatorrhea or foul smelling greasy stools: This is a type of bowel movement that is oily, floats, smells really bad and is difficult to flush. As these patients cannot absorb all the fat they eat, undigested fat is excreted, resulting in stools that look oily or greasy, though not many experience this symptom. It is better to contact the doctor if oil droplets are noticed in the toilet bowl or stools that float or stick to the sides of the bowl and are hard to flush.

Gas and bloating: As these people cannot properly digest the food they eat, it can result in gas and bloating symptoms which can be acutely uncomfortable in public.

Stomach pain: Due to gas and bloating caused by maldigestion, frequent stomach pain is possible. Fatigue, edema or hypoalbuminemia, anemia or deficiency of B-12, Iron, folic acid, bleeding disorders, metabolic bone disease due to Vitamin D deficiency and neurologic manifestations are other related symptoms.

Causes for exocrine insufficiency could be pancreatic or non pancreatic. Chronic Pancreatitis and cystic fibrosis are the result of longstanding inflammation of the pancreas, and they alter the organ's normal structure and function. These arise as a result of malnutrition, heredity or lifestyle behavior such as alcohol use or smoking. In children, this could be caused due to a rare autosomal recessive genetic disorder resulting from mutation of a gene.

Damage to pancreas which is the leading cause of EPI happens when our pancreas get inflamed often when enzymes made by the pancreas start working when they are still inside it, before they get to the small intestine. If a person is alcoholic, then he/she is at risk. Pancreas gets inflamed when the passage way is blocked, if he/she has high levels of triglycerides, due to some immune system disorder.

A surgery on pancreas, stomach or intestine can cause damage. Inherited diseases such as cystic fibrosis or Shwachman-Diamond syndrome can damage pancreas. In case of cystic fibrosis, the body makes unusually thick and sticky mucus which blocks the passage in the pancreas and stops enzymes from getting out. If you have Shwachman-Diamond syndrome, you may be missing cells in your pancreas that make enzymes. Crohn's disease and celiac disease can also lead to EPI in some people.

Diagnosis

The diagnosis of EPI is largely clinical and may go undetected because the signs and symptoms are similar to other Gastro-intestinal diseases. The signs and symptoms are also not always evident. Complete laboratory evaluation is required to diagnose EPI including the pancreatic function testing. The extent of malabsorption and manifestations underlying the disease has to be determined before treatment.

Tests to determine EPI include some blood tests to check if the person is getting enough vitamins and that pancreas are making enough enzymes, to check for celiac disease that can lead to EPI.

A 3-day fecal test is done to check the amount of fat in the bowel movements. Fecal elastase-1 is another test that is usually asked for. These tests can reveal if the pancreas is making enough enzymes. Other tests to check if the pancreas is inflamed include CT scan, MRI and Endoscopic ultrasound.

Treatment

Normally prescription pills that replace the pancreatic enzymes are prescribed. Apart from a healthy diet, the main treatment for EPI is pancreatic enzyme replacement therapy PERT. These enzymes break down your food so you can more easily digest and absorb it. You have to take them during your meals. If you take them before you eat, the replacement enzymes may move through your stomach before your food gets there. If you take the pills after you eat, you have the opposite problem.

An antacid to keep the stomach from breaking down pancreatic enzymes before they can start to work is given. There are six FDA-approved pancreatic enzyme products that are only available by prescription. These include Creon, Pancreaze, Pertzye, Ultresa, Viokace, Zenpep.

Medicines are also prescribed to treat pain such as acetaminophen or ibuprofen. Otherwise, stronger pain drugs such as hydrocodone and oxycodone are given.

Some tips to manage EPI

The right diet is essential for EPI. A dietician can be consulted to help choose the food that keep energy level up and nutrition that the body needs. Instead of traditional three, eat six small meals per day. A big meal may not appeal if he/she has EPI troubles of digestion.

Do not drink as alcohol makes it even harder for body to absorb fat and can damage pancreas over time.

Take vitamins A, D, E and K to replace ones that are not getting absorbed from the diet.

It is essential that you do not lose much weight and a nutritionist can be consulted to help choose foods that have enough protein and nutrients in them. Support from friends and family is by far more important than the treatment itself. A doctor can help to put you in support groups.

Preventing EPI

Many diseases such as pancreatic cancer, cystic fibrosis, diabetes, and pancreatic cancer that are related to EPI cannot be controlled. But some factors can such as intake of heavy continual alcohol, combining alcohol with high-fat diet and smoking - all these that increase the chances of Pancreatitis can be controlled. Those with Pancreatitis caused by heavy alcohol use tend to have severe stomach pain and can develop EPI more rapidly. In case there is a family history of cystic fibrosis or Pancreatitis, this can increase your chances of developing EPI.

Pancreatic Enzyme Replacement Therapy

Pancreatin is a mixture of pancreatic enzymes, lipase, amylase and protease. These enzyme supplements assist in digestion of fat, carbohydrates and proteins. Now, a replacement therapy for pancreatic enzyme involves taking the digestive enzymes that are needed in the form of a tablet or capsule to enable digestion.

Why Pancreatic enzyme replacement ?

Pancreas do not produce the enzymes that are needed to digest food and absorb the nutrients, if you suffer exocrine pancreatic insufficiency. In such cases, pancreatic enzyme replacement therapy (PERT) can help manage the condition and prevent malabsorption and gastric discomfort, if taken properly. This is more so in those with cystic fibrosis, the need for pancreatic enzyme replacement therapy to prevent malnutrition. Whenever food is consumed, enzyme preparation needs to be taken and the dose will be adjusted according to the food consumed.

What are pancreatic enzyme supplements?

Pancreatic enzyme supplements are extracts from pig pancreas glands. In fact, all enzyme replacement products are currently produced from pork derived ingredients. There are no alternatives available. While the Jewish Rabbi has approved pancreatic enzymes, for Muslim patients it has been advised by their religious heads that if no medicines are available, and the ones containing prohibited substances are suitable to cure illnesses or diseases, then that prohibited substance should be prescribed and taken by the patient.

Symptoms associated with pancreatic enzyme insufficiency

Patients with these symptoms should discuss with their medical team whether taking supplemental pancreatic enzymes may be beneficial for them.

• Indigestion / feeling of indigestion
  
• Cramping after meals
  
• Large amounts of gas
  
• Foul smelling stools
  
• Floating or greasy fatty stools
  
• Orange, yellow or light colored stools
  
• Frequent stools / loose stools
  
• Weight loss

How does PERT work?

Pancreatic supplements are capsules that contain a mixture of digestive enzymes. These include lipase to break down fat, protease to assist in digestion of protein and amylase for carbohydrates. Normally, two capsules are prescribed during the course of a meal and sometimes even three or more depending upon the patient's condition, the product and the concentration. Also, appropriate dosage of PERT tends to vary from one to another as other variables such as residual function of the pancreas, which can worsen over time, size and fat contents of meals taken, and goal of PERT for the patients, whether it is reducing bloating or eliminating diarrhea, are considered.

Doctors tend to start with a low dose of PERT and increase as and when necessary. As enzyme doses vary from person to person, a starting dose of 50,000 to 75,000 units of lipase with a meal and 25,000 units with a snack is given.

Preparation of Pancreatic enzyme

Pancreatic enzyme includes Creon, Nutrizym, Pancrease, and Pancrex. These come in capsule form, in two sizes, as 10,000 units for children and 25,000 units for adults. Each capsule sports a number and a letter to indicate the strength of the dose. Do not be alarmed by the high capsule units as it only relates to the amount of lipase unit it contains, and healthy pancreas will release about 720,000 lipase units during every meal.

How to use PERT?

Normally dieticians recommend the amount of intake of PERT and it starts on a low dose and is gradually increases until the symptoms exhibited are brought under control. Capsules should be taken with all food directly, with the first mouthful of food. This includes meals, snacks, and milky drinks, milk based supplements, over the counter shakes, homemade or prescription drinks.

If you skip taking enzymes with every meal or snack, the possibility is that malabsorption may recur. Capsules can be swallowed with a cold drink as swallowing them with hot drink can be less effective as the enzymes tend to get damaged at high temperatures. The capsules should be swallowed as a whole and should not be crushed or chewed. In case you are consuming a large meal, then take half the enzymes at the beginning and the other half in the middle of the meal.

As for the duration of taking pancreatic enzymes, if your pancreas are damaged by cancer and have been removed through surgery, then you have to take pancreatic enzymes for life. Most patients will need to take these enzymes for the rest of their lives as post surgery, the pancreatic function can decline. However, the dosage may be increased or decreased suitably. You should not take enzyme supplements on:

• An empty stomach
  
• With drinks that contain less than half milk, such as tea, non-milky coffee, fruit, squashes and fizzy drinks.
  
• With small quantities of fruit, vegetables, dried fruit, fat-free sweets and mints.
  
• With small quantity of food such as just a square of chocolate or a small plain biscuit.

Side effects of PERT

Some could experience side effects from enzyme replacement. The most frequent effects are constipation, diarrhea, nausea, and abdominal discomfort. These settle over a period of time, and if it does not settle down, then try a different enzyme brand. Remember, while using PERT:

• Pancreatic enzymes cannot withstand high temperatures and they can get damaged. They should be stored at room temperature.
  
  
• Do not store enzymes in warm places, near radiators, in cars or in trouser pockets. Carry them in a pill box or small containers as the whole bottle need not be carried.
  
  
• Never mix enzymes with hot drinks.