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Cystic Fibrosis

Cystic fibrosis is a condition where there is abnormal production of thick and sticky mucus within the lungs and digestive tract. It is a genetic disorder that affects children and young adults. The thick mucus blocks the tiny openings in the lungs thereby trapping bacteria and bringing on various infections. The mucus also clogs the pancreatic passages. This prevents release of enzymes much needed for digestion. Consequently there is poor nutrition and possible diarrhea. This condition can also affect sweat glands and male reproductive system. Cases of cystic fibrosis are noticed more prominently among those Caucasians, especially of Northern or Central European descent. Often cystic fibrosis is diagnosed at birth where the infant's gut is blocked with thick meconium. While most cases of cystic fibrosis are detected in the first 3 years, some cases may be diagnosed in early teens. Other symptoms are delayed growth and failure to gain weight normally. Persons suffering from Cystic fibrosis are more susceptible to bacterial chest infections. Coughing, wheezing and excessive production of sputum are noticeable symptoms. A person suffering from cystic fibrosis is likely to suffer from oily and smelly stools, salty skin and abdominal pain. Other symptoms of cystic fibrosis is polyps in the nose, enlarged liver and spleen and fertility problems.


The first DNA-based blood test has been approved by the U.S. FDA in March 2005. This test helps in detecting the affected gene. Other diagnostic tools include sweat chloride test, fecal fat test and study of pancreatic function. Chest physiotherapy aids in loosening sticky mucus thereby bringing relief. Daily enzyme replacements are prescribed to facilitate digestion. Lung and respiratory infections are tackled with antibiotics. Other measures such as oxygen for easier breathing, postural drainage and vitamin supplements help in improving the quality of life for the patient.

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.


Vitamin K

Vitamin K helps the blood to clot to enable cuts and scrapes to stop bleeding. Persons suffering from liver disease, inflammatory bowel disease or cystic fibrosis are at risk of Vitamin K deficiency. Symptoms of Vitamin K insufficiency include heavy bleeding, anemia, bleeding gums and even osteoporosis. Low levels of Vitamin K do not allow sufficient use of calcium in bones. This vitamin is naturally found in green leafy vegetables, broccoli, and soya beans.

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Collection of Pages - Last revised Date: October 18, 2017