Anthony Bui & Nhan Nguyen Jacobs Anatomy – 4 12 April 2011 Respiratory Diseases
What is Cystic Fibrosis? • Cystic Fibrosis is an inherited disease that causes the build-up of thick, sticky mucus in the lungs and digestive track. • The collection of the 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. =[
What causes it? • Cystic Fibrosis is caused by a defective gene which causes the body to produce abnormally thick and sticky fluid, called mucus. • The mucus builds up in the breathing passages of the lungs and in the pancreas. • There is little known about the CFTR protein that causes CF, because it is hydrophobic and difficult to crystallize.
Who will have it? • Millions of Americans carry the Cystic Fibrosis gene, but do not have any symptoms (they are simply carriers). • The disease is more common among those of Northern or Central European descent. • Most children with CF are diagnosed by age two, while a rare few are not diagnosed until age 18 or older.
What are some early signs? • Very salty-tasting skin; • Persistent coughing, at times with phlegm; • Wheezing or shortness of breath; • Frequent greasy, bulky stools or difficulty in bowel movements ; and • Ongoing diarrhea.
A few later signs… • Frequent lung infections; • Poor growth/weight gain in spite of a good appetite; (chronic malnutrition) • Inability to digest and absorb vital nutrients; (because of buildup in pancreas) and • Death, which is the most severe effect of Cystic Fibrosis.
Prognosis. • In the 1950s, few children with cystic fibrosis lived to attend elementary school. • Today, most children are fairly healthy until they reach adulthood. • They are able to participate in most activities and find employment easily. • BUT, the lung disease eventually worsens to the point where the person is disabled. Now, the average life span is 35 years, which is a dramatic increase since the 1950s.
How to diagnose it • The ImmunoreactiveTrypsinogen (IRT) test is a standard 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. • Chest x-ray or CT Scans • Fecal fat tests • Lung Function tests • Measurement of pancreatic function • Secretin Stimulation tests
How to treat Cystic Fibrosis Treatments include: • Antibiotics to prevent and treat infections • Inhaled medicines to help open airways • Special diet high in proteins and calories • Pancreatic enzymes to help absorb nutrients • Vitamin Supplements!
Preventative measures • Unfortunately, there is no way to prevent Cystic Fibrosis. • Cystic Fibrosis is totally hereditary. • The Cystic Fibrosis gene affects 1 in 29 Caucasian Americans, mostly of Northern European descent.
Works Cited! Content Sources • http://www.ncbi.nlm.nih.gov/pubmedhealth/PMH0001167/ • http://www.cff.org/AboutCF/ • http://www.ehow.com/facts_5006996_what-effects-cystic-fibrosis.html • http://cystic-fibrosis.emedtv.com/cystic-fibrosis/early-symptoms-of-cystic-fibrosis.html Image Sources • http://www.eradimaging.com/images/cystic_fibrosis_2008/cystic_fibrosis_fig1.jpg • http://www.wagerrun.com/wp-content/uploads/2010/06/CF-Logo.jpg • http://newcareerasacoach.com/wp-content/uploads/2011/03/cysticfibrosis_395.jpg • http://parenting.leehansen.com/downloads/clipart/halloween/images/skeleton.jpg • http://www.kdheks.gov/safekids/images/kidsHome.jpg • https://www.achooallergy.com/blog/images/mucus-rules.jpg