Small Bowel Obstruction in CF Patients

Small bowel obstruction means the small intestine is partially or totally blocked. When this happens, the contents of the intestine cannot properly get out of the body. Stools, fluid, and gas build up inside the intestine. This is a potentially serious condition that requires urgent medical care.

Symptoms

The symptoms for “mechanical” small bowel obstruction include:

          Abdominal fullness and/or excessive gas

          Abdomen feels stretched out more than normal (abdominal distention)

          Pain and cramps in stomach area

          Vomiting

          Constipation (cannot pass gas or stool)

          Diarrhea

          Bad breath

The symptoms of paralytic ileus include:

          Abdominal fullness and/or excessive gas

          Abdominal distention

          Vomiting after eating

          Pain is often less severe than in “mechanical” small bowel obstruction

The diagnosis of a small bowel obstruction is initially performed through a physical exam. The exam will include listening for bowel sounds in your stomach. Very high pitched bowel sounds heard through a stethoscope suggest mechanical bowel obstruction, whereas paralytic ileus often produces no bowel sounds. Your doctor may recommend that you see a gastroenterologist or surgeon depending on the suspected diagnosis. These tests include:

  • CT scan
  • Abdominal ultrasound
  • Abdominal X-ray

For patients with cystic fibrosis, small bowel obstructions are much more common and potentially more dangerous. In cystic fibrosis, the build-up of thick, sticky mucus blocks the ducts leading from your pancreas to your bowel. When this happens, the amount of insulin that your body produces is reduced and digestive enzymes are stopped from aiding your digestion. Without these enzymes, you may not be able to digest enough essential nutrients and you may find it difficult to gain weight. Malnutrition can lead to poor growth, physical weakness and delayed puberty in children. Your feces may contain excess fat making them oily, smelly, large and difficult to flush away.

NYU Langone Medical Center

Bupa Health Center

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Pancreatic Enzyme Replacement Therapy in CF Patients

Cystic fibrosis is the most common fatal genetic disorder in North America. CF affects the epithelial transport in exocrine tissues. The disorder produces thick, sticky mucus secretions that may seriously impair the function of various organ systems. The organ systems that are affected include the respiratory tract, the gastrointestinal tract, the liver, the genitourinary system, and the sweat glands.

CF has three major consequences: chronic lung disease, pancreatic insufficiency, and abnormally high electrolyte concentrations in the sweat.

Cystic fibrosis causes pancreatic insufficiency in most cases, causing 85-90% of serious cases to require pancreatic enzyme replacement therapy (PERT). With age, damage to the pancreas becomes worse. The thick mucus obstructs the small pancreatic ducts and interferes with the secretion of digestive enzymes, pancreatic juices, and pancreatic hormones. Eventually, the pancreatic cells are surrounded by mucus and are gradually replaced by fibrous tissues. Malabsorption of many nutrients including fat, protein, vitamins, and minerals often leads to malnutrition. Additionally, the secretion of insulin may be affected resulting in glucose intolerance and diabetes.

With high energy needs, fat restrictions are inappropriate. Instead, pancreatic enzyme replacements are used to control Steatorrhea, relieve abdominal pain, and reduce the mass and frequency of stools passed. To improve the effectiveness of the enzyme replacements, H2-blockers are often provided as well. Even with enzyme replacements, from 10-20% of food energy is lost in the stools.

Pancreatic insufficiency has a strong influence on nutrition status and is a predictor of long-term outcome. The thickened secretions obstruct the pancreatic ducts and prevent the secretions of lipase, amylase, proteases, and bicarbonate. When pancreatic insufficiency is present, individuals are treated with pancreatic enzyme extracts. All enzyme products contain the various enzymes synthesized by the pancreas, including amylase, proteases and lipase in varying amounts. Commercial enzyme products vary in lipase activity from 4,000-25,000 U lipase/capsule. They are available in powder form as tablets that are acid labile or as enteric-coated microspheres- the enteric coating is designed to protect the enzyme from destruction by the acidic environment of the stomach.

Pancreatic enzymes are always taken when food or beverages are consumed. The dosage for enzymes in individualized based on the patient’s diet, nutritional status, degree of pancreatic insufficiency, intestinal pH, and GI anatomy and physiology. Because of inconsistencies in enzyme formulations, the Food and Drug Administration (FDA) has issued a rule requiring manufacturers of pancreatic enzyme supplements to obtain approval for their products. Prior to obtaining approval, manufacturers will need to test the enzymes in clinical trials and demonstrate that they are safe and effective. This rule means that the FDA now requires pancreatic enzymes to meet the same standards of testing as any other new drug.

CFF

Cystic-Fibrosis-World

Sixty Five Roses

Cystic fibrosis is the most common fatal genetic disorder in North America. The disorder produces thick, sticky mucus secretions that may seriously impair the function of multiple organs in the body. Most notably, these organs are the pancreas and lungs. Just a few decades ago, an infant born with CF seldom survived to adulthood. Today, the outlook is much brighter, with adults reaching their 30’s, 40’s, and some even into their 50’s.

Cystic fibrosis has three major consequences: chronic lung disease, pancreatic insufficiency, and abnormally high electrolyte concentrations in the sweat. Chronic lung disease develops because the airways in the lungs become congested with mucus, causing breathings to be strenuous. As the thick mucus stagnates in the bronchial tubes, bacteria multiply there. Lung infections are the usual cause of death in people with cystic fibrosis.

Cystic fibrosis causes some degree of pancreatic insufficiency in all cases, with about 90% of cases serious enough to require enzyme replacement therapy. With aging, damage to the pancreas deteriorates. The thick mucus obstructs the pancreatic ducts and interferes with the secretion of digestive enzymes, pancreatic juices, and pancreatic hormones. Eventually, the pancreatic cells are surrounded by mucus and are gradually replaced by fibrous tissues. Malabsorption of many nutrients including fat, protein, vitamins, and minerals often leads to malnutrition. Additionally, the secretion of insulin may be affected resulting in glucose intolerance and diabetes.

The therapy of cystic fibrosis aims to promote appropriate growth and development and prevent respiratory failure and complications. Treatment includes respiratory, diet, and drug therapy.

Nutrient losses through malabsorption, frequent infections, rapid turnover rate of protein and essential fatty acids, high protein catabolism, and high basal energy expenditures raise energy needs for people with cystic fibrosis to between 120%-150% of the RDA for gender and age. Extra energy is needed simply to breathe. RDs estimate individual energy requirements based on basal metabolic rate, activity level, pulmonary function, and degree of malabsorption.

Obtaining enough energy can be complicated, but because people with CF frequently experience a loss of appetite that is aggravated by repeated infections, emotional stress, and drug therapy. Coughing to clear the lungs may trigger vomiting or reflux of foods from the stomach. Thus the person with cystic fibrosis finds it difficult to take in enough food energy, protein, and other nutrients to meet needs.

NIH- Cystic Fibrosis

Cystic Fibrosis Foundation

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