Cystic Fibrosis Ashley Verbeek

EDU 214

Objectiveo To inform 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.

What is Cystic Fibrosis??

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.

What Causes Cystic Fibrosis?

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.

How does one inheritCystic Fibrosis?

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.

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 40’s

• In the 1950’s, 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

Symptoms•Salty Tasting Skin•Lung Infections

•Wheezing/ Shortness of Breath •Poor Growth / Weight Gain

•Persistent Coughing with Phlegm•Upset Stomach•Fatigue

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

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.

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

Teaching Strategies• Be aware of students IEP’s

• 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 they’re sick.

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

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.

ResourcesCystic Fibrosis Foundation- 75 chapters nation wide

Maryland Chapter Cockeysville, MD 21030

p1: (410) 628-0795

http://www.cff.org/Chapters/maryland/

Local Research Center

The Johns Hopkins University School of Medicine

Director: William B. Guggino. Ph.D.,

Baltimore, MD 21205-2185

Cystic Fibrosis Care Center

Johns Hopkins Hospital

Johns Hopkins Adult CF Program (ADULT)

Baltimore, MD 21205

Appointments: (410) 502-7044

Director(s): Michael P. Boyle, M.D., F.C.C.P.

Website: http://www.hopkinscf.org/

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: http://www.hopkinscf.org/

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

References• Cystic Fibrosis Foundation. (n.d). Retrieved from http://www.cff.org/

• 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 http://ghr.nlm.nih.gov/condition/cystic-fibrosis

• Pinkerton, K. (2013, June 11). Cystic Fibrosis Life Expectancy Statistics. Retrieved from http://www.disabled-world.com/health/respiratory/cystic-fibrosis/life-expectancy.php

My Dear Friend lost her battle to CF in 2011…..In loving memory