IOH Diabetes and related disorders - Cystic Fibrosis (CF)



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		Article disclaimer Edited by Lahle Wolfe Source NHLBI/National Institute of Health Publication edited for content, used with permission. Additional resources used: Cystic Fibrosis (MedlinePlus) Cystic Fibrosis Foundation Fast Links to Disorders Often Associated with Diabetes Addison's Disease Asthma and Allergies Celiac Disease (Sprue) Cyclic Vomiting Syndrome Cystic Fibrosis Eating Disorders Fibromyalgia Syndrome Frozen Shoulder Hashimoto's Thyroiditis Hemochromatosis (Iron Overload) Infertility Irritable Bowel Syndrome Polycystic Ovarian Syndrome Weight Gain Weight Loss Fast Click to Problems Associated with Diabetes Acanthosis Nigricans (AN) Acne Alopecia Areata (AA) Gum Disease Hirsutism Honeymooning Skin Tags Yeast Infections MedlinePlus - Health Information About CF Encyclopedia: Cystic Fibrosis Health Topic: Cystic Fibrosis Health Topic: Newborn Screening CF Information Links from NIH What is cystic fibrosis? How common is cystic fibrosis? What genes are related to cystic fibrosis? How do people inherit cystic fibrosis? Where can I find additional information about cystic fibrosis? What other names do people use for cystic fibrosis? What if I still have specific questions about cystic fibrosis? What glossary definitions help with understanding cystic fibrosis Resource Links to Cystic Fibrosis Sites Cystic Fibrosis.Com Cystic Fibrosis Foundation CF Chapter & Care Centers Books On Cystic Fibrosis Links to More Information About Cystic Fibrosis NHLBI Lung Diseases Information for the Public Other Cystic Fibrosis (MedlinePlus) Cystic Fibrosis Foundation Clinical Trials Current Research (NIH) Patient Recruitment for Studies Conducted by NHLBI, NIH
						main "Disorders" page Disorders Associated with Diabetes print this article Cystic Fibrosis - Part 2 of 2 (also known as CF, Cystic fibrosis of pancreas, Fibrocystic Disease of Pancreas, and Mucoviscidosis) Causes, Symptoms, Diagnosis, Treatment Join IOH Cystic Fibrosis Support Group Mini Site Index Treatment for CF Management of Digestive Problems Living With CF Section 1 What is Cystic Fibrosis (CF)? What Causes CF? What are Symptoms of CF? Tests and Diagnosis of CF CF Carrier Testing Treatment for Cystic Fibrosis There still is no cure for cystic fibrosis (CF), but treatments have improved greatly in recent years. The goals of CF treatment are to: Prevent and control infections in your lungs. Loosen and remove the thick, sticky mucus from your lungs. Prevent blockages in your intestines. Provide adequate nutrition. Treatment for Lung Problems The main treatments for lung problems in people with CF are: Antibiotics for infections of the airways Chest physical therapy Exercise Other medications Antibiotics Most people with CF have ongoing, low-grade lung infections. Sometimes, these infections become so serious that you may need to be hospitalized. Antibiotics are the primary treatment. You may be given several different types of antibiotics. The choice of antibiotics depends on: The strains of bacteria involved How serious your condition is Your previous history of antibiotic use The different types of antibiotics include: Oral antibiotics for relatively mild airway infections. Inhaled antibiotics, such as tobramycin. They may be used alone or with oral antibiotics. Intravenous antibiotics for severe infections or when none of the oral antibiotics work. Antibiotics, such as azithromycin, that also reduce inflammation. Chest Physical Therapy Chest physical therapy (CPT) is also called chest clapping or percussion. It involves pounding your chest and back over and over again to dislodge the mucus from your lungs so that you can cough up the mucus. CPT for cystic fibrosis should be done three to four times each day. CPT is also often referred to as postural drainage. This involves your sitting or lying on your stomach with your head down while you do CPT. This allows gravity to help drain the mucus from your lungs. Because CPT is hard or uncomfortable for some people, several devices have been developed recently that may help with CPT. The devices include: An electric chest clapper, known as a mechanical percussor. An inflatable therapy vest that uses high-frequency air waves to force the mucus out of your lungs. A "flutter" device, a small hand-held device that you breathe out through. It causes vibrations that dislodge the mucus. A positive expiratory pressure (PEP) mask that creates vibrations that help break the mucus loose from the airway walls. Several breathing techniques may also help dislodge the mucus. These techniques include: Forced expiration technique (FET)�forcing out a couple of breaths or huffs and then doing relaxed breathing Active cycle breathing (ACB)�FET with deep breathing exercises that can loosen the mucus in your lungs and help open your airways Exercise Aerobic exercise helps: Loosen the mucus Encourage coughing to clear the mucus Improve your overall physical condition If you exercise regularly, you may be able to cut back on your chest therapy. Check with your doctor before doing this. Other Medications Anti-inflammatory medications may help reduce the inflammation in your lungs that is caused by ongoing infections. These medications include: Inhaled or, sometimes, oral steroids. Steroids are the most effective anti-inflammatory medicines. Ibuprofen, a type of nonsteroidal, anti-inflammatory medicine. It may slow the progress of CF in young children with mild symptoms. Bronchodilators, which are inhaled drugs that relax the muscles around the airways so that the airways can open up. They should be taken just before CPT to help clear mucus. Mucus-thinning drugs reduce the stickiness of mucus in your airways. They include: Human DNase(Dornase Alfa), a drug that loosens the mucus in your lungs. It may lead to shorter hospital stays. Acetylcysteine and saline. Saline washes of your nasal passages, which may help clear your sinuses. Oxygen Therapy If the level of oxygen in your blood is too low, you may need oxygen therapy. Oxygen is usually given through nasal prongs or a mask. Lung Transplantation Surgery to replace one or both of your lungs with healthy lungs from a human donor may help you. Some of the factors that determine whether you can undergo lung transplantation include: The type of bacteria in your lungs Your age and weight The medications you are taking Whether you have other medical conditions, including osteoporosis How well your lungs are functioning Management of Digestive Problems Nutritional therapy can improve your growth and development, strength, and exercise tolerance. It may also make you strong enough to resist some lung infections. Nutritional therapy includes a well-balanced, high-calorie diet that is low in fat and high in protein. As part of your nutritional therapy, your doctor may: Prescribe oral pancreatic enzymes to help you digest fats and proteins and absorb more vitamins. The enzymes should be taken in capsule form before every meal, including snacks. Recommend supplements of vitamins A, D, E, and K to replace the fat-soluble vitamins that your intestines cannot absorb. Recommend that you use a feeding tube, called a gastrostomy (gas-TROS-to-me) tube or T-tube, to add more calories at night while you are sleeping. The tube is placed in your stomach. Before you go to bed each night, you attach a bottle with a nutritional solution to the entrance of the tube. It feeds you while you sleep. Other treatments for the digestive problems caused by CF may include: Enemas and mucus-thinning medications to treat intestinal blockages Medicines that reduce stomach acid and help the oral pancreatic enzymes work better Living With Cystic Fibrosis If you have cystic fibrosis (CF), you should learn as much as you can about the disease and work closely with your doctors to learn how to manage it. Ongoing medical care is important. You should seek treatment from a team of doctors, nurses, and respiratory therapists who specialize in CF. These specialists are often located at CF Foundation Centers in major medical centers. Good self-care includes: Eating a healthy diet Avoiding tobacco smoke Washing your hands often to reduce your chances of infection Exercising frequently Drinking lots of fluids Doing chest physical therapy every day Having annual flu and other appropriate vaccinations Taking your medicines as prescribed You can expect to have a normal sex life. Most men with CF are infertile, but they may be helped with modern reproductive techniques. Although most women with CF may be less fertile than women who don�t have CF, they usually can have children. Talk to your doctor before becoming pregnant. Having a positive attitude is also helpful. If you are a parent of someone with CF, do not feel guilty about passing it on to your child. And do not be overprotective; encourage your child to be active and self-reliant. Cystic Fibrosis Foundation 6931 Arlington Road Bethesda, MD 20814 Phone: 301�951�4422 or 1�800�FIGHT�CF Internet: www.cff.org, Robert Beall, President Purpose: Supports medical research, professional education, and a nationwide network of care centers to benefit patients with cystic fibrosis (CF). Supports services for young adults with CF. Publications: Information brochures.



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