Cystic fibrosis- Ashley Verbeek

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  • 1. Cystic Fibrosis Ashley Verbeek EDU 214

2. Objective o Toinform the class about Cystic Fibrosis o Help future teachers know how to handle a student with Cystic Fibrosis in their classroom o To inform the public about Cystic Fibrosis and show them ways that they can help. 3. What is Cystic Fibrosis?? 4. Cystic Fibrosis also known as CF is an inherited chronic disease that effects the lungs and digestive system. The disease produces a thick sticky mucus, then this mucus the clogs the lungs which then can cause lung infections. It can also obstruct the pancreas and stops the natural enzymes from helping the body break down and absorb food. 5. What Causes Cystic Fibrosis? 6. Cystic Fibrosis is caused be a defect or mutation in the CFTR (Cystic Fibrosis Transmembrane Conductance Regulator) gene. The CFTR provides instructions for making a channel that transports negatively charged particles called chloride ions into and out of cells, if the CFTR mutates it disrupts the function of chloride channels and prevents them from regulating the flow of chloride ions and water across cell membranes. As a result, the passageways to the lungs, pancreas and other organs produce mucus that is thick, sticky and clogs airways and ducts. 7. How does one inherit Cystic Fibrosis? 8. In order to inherit CF, one must inherit both copies of the defective CFTR gene from their parents. If both parents are carriers, there is a 25% chance of inheriting the gene, 50% chance of having a copy and being a carrier, and a 25% of not having or carrying the gene. 9. Fun Facts.. Did you know Einstein had CF? 30,000 Children and Adults have CF in the U.S. Most common in Caucasians 1,000 new cases every year 70% are diagnosed by age 2 45% of people with CF are 18 or older Median age of survival is early 40s In the 1950s, the median age of survival was grade school. 1 in 31 Americans are carriers of CFTR There are 1,000 different mutations of the CFTR gene 1 child out of 3,500 are born with CF 10. Symptoms Salty Tasting Skin Lung Infections Wheezing/ Shortness of Breath Poor Growth / Weight Gain Persistent Coughing with Phlegm Upset Stomach Fatigue 11. Treatments There is NO cure for Cystic Fibrosis. Some treatments to lengthen and improve life: Specialized medical care Aggressive drug treatment and therapy Proper nutrition Exercise Lung transplant Clinical Trials 12. How to test for Cystic Fibrosis? Sweat Test- Sweat glands are stimulated and collected, if a person has a high level of chloride then they will most likely have CF. Genetic Testing- If sweat test is unclear, then they can test for the most common CF gene. They are also able to test your unborn child as well. 13. Day to Day. Take pancreatic enzyme supplements with every meal and snack. Multi Vitamins Airway clearance 1-4 times a day, sometimes more. Aerosolized medicine Nebulizers 14. Teaching Strategies Be aware of students IEPs Understanding students medical routines Give them access to the bathroom, because some students get upset stomachs and gas so allowing them to excuse themselves is not a big deal. Excuse students to cough, because coughing breaks up the thick mucus. Keep tissues and anti-bacterial handy at all times. If there are more than 1 student with CF in your school, they need to be as far away from each other as possible. Give them different classrooms, assign their own bathroom, assign water fountains, and designate different areas for them to go if theyre sick. 15. Current Laws & Programs Individuals with Disabilities Education Act (IDEA) - assist children in public schools Supplemental Security Income (SSI)- provides income to those not able to work Cystic Fibrosis Patient Assistance Program (CFPAF) Provides qualified patients medication, to reduce out of pocket cost Americans with Disabilities Act (ADA)- protected from being discriminated against in the work place. Health Insurance Portability and Accountability Act (HIPAA) - gives access to health insurance 16. Current Research Kalydeco- FDA approved in 2012, is the first drug available that targets the underlying cause of CF. Hypertonic Saline is an inhaled therapy that increases hydration of airway surface liquid with CF. The CF foundation is maintaining a robust pipeline of potential therapies that target the disease from every angle. 17. Resources Cystic Fibrosis Foundation- 75 chapters nation wide Maryland Chapter Cockeysville, MD 21030 p1: (410) 628-0795Cystic Fibrosis Care Center Johns Hopkins Hospital Johns Hopkins Adult CF Program (ADULT) Baltimore, MD 21205 Research Center The Johns Hopkins University School of Medicine Director: William B. Guggino. Ph.D., Baltimore, MD 21205-2185Appointments: (410) 502-7044 Director(s): Michael P. Boyle, M.D., F.C.C.P. Website: Johns Hopkins University (PEDIATRIC) Baltimore, MD 21287 Appointments: (410) 955-2795 Director(s): Peter J. Mogayzel, Jr., M.D., Ph.D., Pamela L. Zeitlin, M.D., Ph.D. Website: 18. How you can help. Make Donations to the Cystic Fibrosis Foundation Contact your local CF Chapter and Volunteer Become and advocate and raise awareness If have CF, join the clinical trials Participate in area Great Strides walk 19. References Cystic Fibrosis Foundation. (n.d). Retrieved from Davis, P.B ,American Journal of Respiratory and Critical Care Medicine, 2006, Vol.173: 475- 482, 10.1164/rccm.200505-840OE Genetic Home Reference. (2012, August). Retrieved from Pinkerton, K. (2013, June 11). Cystic Fibrosis Life Expectancy Statistics. Retrieved from Dear Friend lost her battle to CF in 2011..In loving memory