Also called Mucoviscidosis. Use Pseudomonas Aeruginosa, Breathing Deep, and General Antiseptic programs. See Parasites General, and Roundworm programs if no progress.Encyclopedia Entry for Cystic Fibrosis :Cystic fibrosis - nutrition. The pancreas is an organ in the abdomen behind the stomach. An important job of the pancreas is to make enzymes. These enzymes help the body digest and absorb protein and fats. A buildup of sticky mucus in the pancreas from CF can lead to serious problems, including: Stools that contain mucus, are foul smelling, or float Gas, bloating, or distended belly Problems getting enough protein, fat, and calories in the diet Because of these problems, people with CF may have a hard time staying at a normal weight. Even when weight is normal, a person may not be getting the right nutrition. Children with CF may not grow or develop correctly.The following are ways for adding protein and calories to the diet. Be sure to follow other specific instructions from your health care provider. Enzymes, vitamins, and salt: Most people with CF must take pancreatic enzymes. These enzymes help your body absorb fat and protein. Taking them all the time will decrease or get rid of foul-smelling stools, gas, and bloating. Take enzymes with all meals and snacks. Talk to your provider about increasing or decreasing your enzymes, depending on your symptoms. Ask your provider about taking vitamins A, D, E, K, and extra calcium. There are special formulas for people with CF. People who live in hot climates may need a small amount of extra table salt. Eating patterns: Eat whenever you are hungry. This may mean eating several small meals throughout the day. Keep a variety of nutritious snack foods around. Try to snack on something every hour, such as cheese and crackers, muffins, or trail mix. Try to eat regularly, even if it is only a few bites. Or, include a nutrition supplement or milkshake. Be flexible. If you aren't hungry at dinner time, make breakfast, mid-morning snacks, and lunch your main meals. Getting more calories and protein: Add grated cheese to soups, sauces, casseroles, vegetables, mashed potatoes, rice, noodles, or meat loaf. Use whole milk, half and half, cream, or enriched milk in cooking or beverages. Enriched milk has nonfat dry milk powder added to it. Spread peanut butter on bread products or use it as a dip for raw vegetables and fruit. Add peanut butter to sauces or use on waffles. Skim milk powder adds protein. Try adding 2 tablespoons (8.5 grams) of dry skim milk powder in addition to the amount of regular milk in recipes. Add marshmallows to fruit or hot chocolate. Add raisins, dates, or chopped nuts and brown sugar to hot or cold cereals, or have them for snacks. A teaspoon (5 g) of butter or margarine adds 45 calories to foods. Mix it into hot foods such as soups, vegetables, mashed potatoes, cooked cereal, and rice. Serve it on hot foods. Hot breads, pancakes, or waffles absorb more butter. Use sour cream or yogurt on vegetables such as potatoes, beans, carrots, or squash. It can also be used as a dressing for fruit. Breaded meat, chicken, and fish have more calories than broiled or plain roasted. Add extra cheese on top of frozen prepared pizza. Add coarsely chopped hard cooked egg and cheese cubes to a tossed salad. Serve cottage cheese with canned or fresh fruit. Add grated cheeses, tuna, shrimp, crabmeat, ground beef, diced ham or sliced boiled eggs to sauces, rice, casseroles, and noodles.Egan ME, Green DM, Voynow JA. Cystic fibrosis.Encyclopedia Entry for Cystic Fibrosis :Cystic fibrosis - resources. Resources - cystic fibrosis.Encyclopedia Entry for Cystic Fibrosis :Cystic fibrosis. Cystic fibrosis (CF) is a disease that is passed down through families. It is caused by a defective gene that makes the body produce abnormally thick and sticky fluid, called mucus. This mucus builds up in the breathing passages of the lungs and in the pancreas. The buildup of mucus results in life-threatening lung infections and serious digestion problems. The disease may also affect the sweat glands and a man's reproductive system. Many people carry a CF gene, but do not have symptoms. This is because a person with CF must inherit 2 defective genes, 1 from each parent. Some white Americans have the CF gene. It is more common among those of northern or central European descent. Most children with CF are diagnosed by age 2. For a small number, the disease is not detected until age 18 or older. These children often have a milder form of the disease.Symptoms in newborns may include: Delayed growth Failure to gain weight normally during childhood No bowel movements in first 24 to 48 hours of life Salty-tasting skin Symptoms related to bowel function may include: Belly pain from severe constipation Increased gas, bloating, or a belly that appears swollen (distended) Nausea and loss of appetite Stools that are pale or clay-colored , foul smelling, have mucus, or that float Weight loss Symptoms related to the lungs and sinuses may include: Coughing or increased mucus in the sinuses or lungs Fatigue Nasal congestion caused by nasal polyps Repeated episodes of pneumonia (symptoms of pneumonia in someone with cystic fibrosis include fever, increased coughing and shortness of breath, increased mucus, and loss of appetite) Sinus pain or pressure caused by infection or polyps Symptoms that may be noticed later in life: Infertility (in men) Repeated inflammation of the pancreas ( pancreatitis ) Respiratory symptoms Clubbed fingers Clubbed fingers.A blood test is done to help detect CF. The test looks for changes in the CF gene. Other tests used to diagnose CF include: Immunoreactive trypsinogen (IRT) test is a standard newborn screening test for CF. A high level of IRT suggests possible CF and requires further testing. Sweat chloride test is the standard diagnostic test for CF. A high salt level in the person's sweat is a sign of the disease. Other tests that identify problems that can be related to CF include: Chest x-ray or CT scan Fecal fat test Lung function tests Measurement of pancreatic function Secretin stimulation test Trypsin and chymotrypsin in stool Upper GI and small bowel series.An early diagnosis of CF and treatment plan can improve both survival and quality of life. Follow-up and monitoring are very important. When possible, care should be received at a cystic fibrosis specialty clinic. When children reach adulthood, they should transfer to a cystic fibrosis specialty center for adults. Treatment for lung problems includes: Antibiotics to prevent and treat lung and sinus infections. They may be taken by mouth, or given in the veins or by breathing treatments. People with CF may take antibiotics only when needed, or all the time. Doses are often higher than normal. Inhaled medicines to help open the airways. Other medicines that are given by a breathing treatment to thin mucus and make it easier to cough up are DNAse enzyme. therapy and highly concentrated salt solutions (hypertonic saline). Flu vaccine and pneumococcal polysaccharide vaccine (PPV) yearly (ask your health care provider). Lung transplant is an option in some cases. Oxygen therapy may be needed as lung disease gets worse. Lung problems are also treated with therapies to thin the mucus. This makes it easier to cough the mucus out of the lungs. These methods include: Activity or exercise that causes you to breathe deeply Devices that are used during the day to help clear the airways of too much mucus Manual chest percussion (or chest physiotherapy), in which a family member or a therapist lightly claps the person's chest, back, and area under the arms Treatment for bowel and nutritional problems may include: A special diet high in protein and calories for older children and adults Pancreatic enzymes to help absorb fats and protein, which are taken with every meal Vitamin supplements, especially vitamins A, D, E, and K Your provider can advise other treatments if you have very hard stools Ivacaftor is a medicine that treats certain types of CF. It improves the function of one of the defective genes that causes CF. As a result, there is less buildup of thick mucus in the lungs. Other CF symptoms are improved as well. Care and monitoring at home should include: Avoiding smoke, dust, dirt, fumes, household chemicals, fireplace smoke, and mold or mildew. Giving plenty of fluids, especially to infants and children in hot weather, when there is diarrhea or loose stools, or during extra physical activity. Exercising 2 or 3 times each week. Swimming, jogging, and cycling are good options. Clearing or bringing up mucus or secretions from the airways. This must be done 1 to 4 times each day. Patients, families, and caregivers must learn about doing chest percussion and postural drainage to help keep the airways clear. Postural drainage.You can ease the stress of illness by joining a cystic fibrosis support group. Sharing with others who have common experiences and problems can help your family to not feel alone.Most children with CF stay in good health until they reach adulthood. They are able to take part in most activities and attend school. Many young adults with CF finish college or find jobs. Lung disease eventually worsens to the point where the person is disabled. Today, the average life span for people with CF who live to adulthood is about 37 years. Death is most often caused by lung complications.The most common complication is chronic respiratory infection. Other complications include: Bowel problems, such as gallstones, intestinal blockage, and rectal prolapse Coughing up blood Chronic respiratory failure Diabetes Infertility Liver disease or liver failure, pancreatitis, biliary cirrhosis Malnutrition Nasal polyps and sinusitis Osteoporosis and arthritis Pneumonia that keeps coming back Pneumothorax Right-sided heart failure (cor pulmonale).Call your provider if an infant or child has symptoms of CF, and experiences: Fever, increased coughing, changes in sputum or blood in sputum, loss of appetite, or other signs of pneumonia Increased weight loss More frequent bowel movements or stools that are foul-smelling or have more mucus Swollen belly or increased bloating Call your provider if a person with CF develops new symptoms or if symptoms get worse, particularly severe breathing difficulty or coughing up blood.CF cannot be prevented. Screening those with a family history of the disease may detect the CF gene in many carriers.CF.Enteral nutrition - child - managing problems Gastrostomy feeding tube - bolus How to breathe when you are short of breath Jejunostomy feeding tube Postural drainage.Clubbing Clubbing Postural drainage Postural drainage Clubbed fingers Clubbed fingers Cystic fibrosis Cystic fibrosis.Accurso FJ. Cystic fibrosis.