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          June 2000 Issue

          Welcome to the Second Edition of Cystic Fibrosis.
          By: Paige Osbourne

          In this portion, We will be defining some of the terminology associated with this disease, and what constitutes and emergency situation.

          Emergency Situations
          (criteria to potentially determine need for a call to the doctor, doctor visit or the Emergency Room at the hospital)

          • Fever

          • Increased cough frequency and severity followed by shortness of breath

          • Cough during the night

          • Increase in sputum or change in color of sputum

          • Blood in sputum

          • Weight loss

          • Poor Oral intake

          • Persistant vomiting

          • Abdominal distention, abdominal discomfort

          • Diarrhea with a concern about dehydration

          • Severe Constipation - lack of bowel movements for 2 or more days

          • Excessive thirst

          • Increased urination

          • Severe drug interactions/allergic reactions (i.e.; rash, hives, GI upset, joint pain, mental changes and others related to patients specific drug therapies)


          Glossary of Common CF Terminology
          Please note this is not a complete listing. Should you have any questions, please ask your doctor.

          Term Definition
          Acute Respiratory Failure A sudden, severe condition in which a sufficient supply of oxygen does not reach the bloodstream, and carbon dioxide is not adequately removed from the blood and lungs. Requires emergency care.
          Alpha-I-AntitrypsinDeficiency An inherited condition in which the lung tissue is destroyed through an abnormality of the body's immune system. Caused by a lack of Alpha-I- Antitrypsin, a protein in the blood which controls the action of the enzyme trypsin. When Alpha-I-Antitrypsin is not present, trypsin destroys the liver as well as lung tissue.
          Aminoglycoside A group of antibiotics that are used to combat bacterial infections. Tobramycin and gentamicin are examples of these antibiotics and are often used by people with CF.
          Anoxia The absence or lack of oxygen supply in the body.
          A-P Diameter Anterior-posterior (front to back) measurement of the chest. This measurement is usually increased in children with CF. It's related to the long-term mucous obstruction of the lungs and its effects on breathing patterns.
          Arrythmia Any variation from the normal rythm of the heartbeat.
          Aspirate The mechanical suctioning of liquids or gases from the lungs. Often used to remove excess mucous from the lungs of people with CF. Also means to inhale food orother material.
          Atelectasis A collapsed portion of the lung which does not contain air. Can be caused by excessive accumulations of mucous secretions which interfere with complete inhalation.
          Autosomal Recessive A genetic trait or disorder which appears only when an individual inherits a pair of chromosomes, each containing the gene for the trait.
          Barrel Chest Enlarged rib cage caused by some lung diseases,including CF.
          Bronchial DrainageA form of physical therapy used for people with CF in which the chest is pounded from several angles and positions to help loosen mucous in the lungs.
          Bronchial LavageA procedure in which large volumes of saline are rinsed through the lungs, followed by suction. It may be used in the treatment of CF to aspirate mucous secretions which cannot be removed through postural drainage.
          Bronchiectasis Long-term condition in which the bronchi of the lungs are stretched or dilated beyond their normal dimensions. This condition leads to abnormal breathing patterns, coughing, and coughing up mucus from the lungs.
          Bronchoscopy Internal examination of the lungs using an illuminated tube-like instrument (bronchoscope) which is inserted into thelungs through the throat, pharynx and trachea (windpipe). Allows direct examination of the interior of the bronchial tubes.
          Celiac Disease Intestinal disorder with symptoms (diarrhea, with bulky foul-smelling stools, failure to thrive, and protruding abdomen) similar to the digestive complications of CF. Celiac disease is a disorder of intestinal absorption cause by an intolerance to gluten, the protein found in wheat, barley and rye, and is apparently inherited.
          Chorionic Villius Biopsy Biopsy of a very small portion of the placenta. This is done during the early stages of pregnancy to obtain tissue for genetic diagnosis.
          Cirrhosis Fibrosis and scarring of the liver which can be caused by a number of diseases. A unique form of cirrhosis occurs in approximately one percent of individuals with CF when thick secretions block the liver's bile ducts.
          Cor Pulmonale Enlargement of the right ventricle of the heart due to resistance to the passage of blood through the lungs. Can often lead to right heart failure. This can be a major complication of chronic pulmonary diseases such as CF.
          CyanosisBluish coloration of the skin due to insufficient oxygen in the bloodstream. May occur in an individual with CF during acute respiratory failure.
          Hemoptysis Coughing up blood, often with sputum. Results from broken small blood vessels in the lungs.
          Hemothorax Accumulation of blood in the cavity around the lungs. This can be an advanced complication of CF.
          Hypoxia Low blood oxygen. May occur in lung diseases such as CF.
          Percussor A device which delivers short, sharp blows. In the management of CF, percussors may be used to perform chest physical therapy to help loosen mucous in the lungs.
          Pilocarpine IontophoresisA technique that stimulates sweat by applying the chemical pilocarpine on a small area of the skin, which then recieves a small electrical current. (Recommended procedure for a sweat test)
          Pneumothorax A sudden,partial or complete collapseof the lung. Results from the rupture of lung tissue, which allows air to escape from the lung and become trapped between the lung and the chest wall.
          SteatorrheaAn excessive amount of fats in the stool. In CF, this is caused by blockage of pancreatic ducts. Fats are not properly broken down and absorbed and are therefore excreted in the stool.
          ViscidThick and sticky. Usually refers to the consistancy of mucous in a CF patient.


          Below, you will find some great links to many things, such as more information, medical breakthroughs and support groups. I have started an e-mail support group as well.
          Please take a moment and subscribe.... the input you can give will be more valuable than you will ever know.

                 
          Subscribe to cf_support
                         
          Powered by www.egroups.com

          And now for some more links related to CF.

          CF Links

          Cystic Fibrosis Foundation A range of information on clinical trials, news, related links, public policy alliance, and other areas.

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          Canadian Cystic Fibrosis Foundation Latest news and research regarding cystic fibrosis in Canada, in French and English.

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             Cystic Fibrosis Index of On-Line Resources An exhaustive guide to all on-line web pages, news groups, FAQ's, chat rooms, and mailing lists associated with Cystic Fibrosis.

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          International Association of Cystic Fibrosis Adults IACFA International association with the purpose of improving the quality of life of all adults with cystic fibrosis, by identifying common problems, enhancing the exchange of information

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          Cystic Fibrosis.com Information on coping with the disease, and the CF Roundtable magazine.

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          Cystic Fibrosis Research Information on helping the organzation, newsletters, events, vehicle donation, and other areas.

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          CF-Web Online archive of CF information.

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          Mayo Clinic: Cystic Fibrosis Article on the improvements in the treatment of cystic fibrosis.

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          The Cystic Fibrosis Association of Ireland.      The Bridge to Life What is Cystic Fibrosis? Cystic Fibrosis is Ireland's most common life-threatening inherited disease.

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          Cystic Fibrosis - Doctor's Guide to the Internet The latest medical news and information for patients or friends/parents of patients diagnosed with Cystic Fibrosis and Cystic Fibrosis-related disorders.

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          A Family Guide to Cystic Fibrosis Genetic Testing Department of Pediatrics and Human Development Division of Genetics Michigan State University/College of Human Medicine Advances in technology are changing many aspects of our lives.

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          Cystic Fibrosis Awareness Page These pages are filled with pictures of people who fight this disease everyday. They represent the fight from every angle..as friends, family, and as those who have the diagnosis of Cystic Fibrosis.

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          Cystic Fibrosis 101 This site covers a range of cystic fibrosis issues, and offers 240 links to related sites.

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          Support Groups

          CF Support groups AOL, ICQ, Mailing Lists and the Florida CF Support group are all listed here.

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          Cystic-L - Cystic Fibrosis Info & Support CYSTIC-L is a free email service dedicated to the exchange of information and support specific to cystic fibrosis. Members include people with CF and those who share their lives:

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          Page 2
          (A Special Series on Caregiving)

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