Cystic Fibrosis

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Definition A. an inherited disease characterized by an abnormality in the glands that produce sweat and mucus B. thick, sticky mucus to form in the lungs, pancreas, and other organs C. mucus blocks the airways, causing lung damage, making it hard to breathe, and leading to serious lung infections Epidemiology A. second most common life-shortening, inherited disorder occurring in childhood in the United States B. Approximately 30,000 Americans have CF C. 1,000 new cases diagnosed each year D. equally in male & female and affect every race E. most commonly in caucasians of Northern European descent F. one in 2,500 caucasians are born affected each year Signs & Symptoms A. Vary depending on severity B. may worsen as time passes C. Respiratory S&S: 1. clogs the tubes that carry air in and out of your lungs a) A persistent cough that produces thick spit (sputum) and mucus b) Wheezing c) Breathlessness d) A decreased ability to exercise e) Repeated lung infections f) Inflamed nasal passages or a stuffy nose D. Digestive S&S: 1. The thick mucus can also block tubes that carry digestive enzymes from your pancreas to your small intestine. Without these digestive enzymes, your intestines can't fully absorb the nutrients in the food you eat a) Foul-smelling, greasy stools b) Poor weight gain and growth c) Intestinal blockage, particularly in newborns (meconium ileus) d) Severe constipation

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How is it diagnosed? A. newborn screening B. sweat test 1. measures amount of salt present

C. chemical test D. chest x-rays E. lung function test F. sputum cultures G. stool evaluations H. for babies who cannot produce enough sweat, blood may be used V. Treatment A. non curable B. Management of problems that cause lung obstruction, which may involve: 1. Physical therapy 2. Exercise to loosen mucus, stimulate coughing, and improve overall physical condition 3. Medications to reduce mucus and help breathing 4. Antibiotics to treat infections 5. Anti-inflammatory medications C. Management of digestive problems, which may involve: 1. Appropriate diet 2. Pancreatic enzymes to aid digestion 3. Vitamin supplements 4. Treatments for intestinal obstructions D. Medications: 1. Antibiotics a) treat and prevent lung infections 2. Mucus-thinning drugs a) help you cough up the mucus, which improves lung function 3. Bronchodilators a) help keep your airways open by relaxing the muscles around your bronchial tubes 4. Oral pancreatic enzymes a) help your digestive tract absorb nutrients VI. Prognosis A. survival age was 37.4 years B. on average people diagnosed with cystic fibrosis live to their mid to late 30 VII. Prevention

A. undergo genetic testing with your significant other to see if either of you carry the gene

Links: http://www.mayoclinic.org/diseases-conditions/cystic-fibrosis/basics/definition/con-20013731 http://www.hopkinsmedicine.org/healthlibrary/conditions/adult/respiratory_disorders/cystic_fibr osis_85,P01306/ http://www.lung.org/assets/documents/publications/solddc-chapters/cf.pdf http://www.webmd.com/children/tc/cystic-fibrosis-topic-overview