Cystic  Fibrosis

Let's explore a disease so life-threatening that, about 60 years ago children with this disease never made it to school-age.

What is Cystic Fibrosis?

Cystic Fibrosis (CF) is a life-threatening genetic disease that primarily affects a persons lungs and digestive system.  This disease is a inherited condition, that affects the cells that produce mucus, sweat, and digestive juices. CF occurs when a defective gene causes the those secreted fluids to be thick and sticky , opposite of what they should be. These thick and sticky secretions begin to plug up tubes, ducts, and passageways, especially in the lungs and pancreas.

FACT ALERT: An estimated 30,000 children and adults in the United States (70,000 wordwide) have CF.

The Fine Print: Symptoms, Causes

The Symptoms vary from person to person as far as intensity. But the respiratory signs and symptoms are clear.The thicker mucus clog s the tubes we use to breath air in and out. Because of the clogs people with CF experience a persistent cough that produces thick spit and mucus, wheezing, breathlessness, repeated lung infections.  The mucus can also block the tubes that carry digestive enzymes from your pancreas to your small intestine. Without those digestive enzymes, your intestines can't fully absorb the nutrients in the food you eat. People with CF, often have foul-smelling, greasy stools, poor weight gain and growth, or severe constipation.

FACT ALERT:  People with CF often have very  salty-tasting skin.

The cause of Cystic Fibrosis stems from a mutation in a gene changes a protein that regulates the movement of salt in and out of cells. There are many different mutations that can occur in the gene, which determines the severity of the condition.

The mutated gene, which is inherited from a child's parent, is a recessive gene.The thing about this disease that makes it fairly rare , is that the child must inherit this gene from both parents. But, if the child inherits one copy then they will be a carrier of this recessive gene.

How do we fix this?

Every state in the United States, routinely screens newborns for cystic fibrosis. In this screening , a blood sample is checked for higher than normal levels of a chemical (IRT) released by the pancreas.

After diagnosis, there are many test that need to be done regularly. The imaging tests, lung function tests, sputum culture, and organ function tests all help monitor this condition.

What's new with CF

In October 2014, scientist where able to show that tannic acid may help ease the impact of bacterial lung infections in cystic fibrosis patients.  The test showed that tannic acid counteracts the harmful effect of an enzyme produced by the bacteria S. aureus. This is not a cure by a way to relieve the symptoms.

Gene Therapy is on the horizon! Gene therapy for CF is not yet possible but impressive progress is being made in developing ways to treat the gene abnormality.

What teachers should know about CF...

-Give the student frequent access to the bathroom

-Try to got draw attention to the students frequent farting.

-Encourage the student to keep a box of tissues to cough into and a trashcan to dispose of the tissue near their desk.

-Review the child's IEP

- Ensure the classroom is as germ free as possible. Students should wash hands more often and everyone should cough into tissues.

Need Resources?

*The Cystic Fibrosis Foundation Maryland Chapter

Call 1(800) FIGHT CF   or (410)

*The Cystic Fibrosis Foundation Patient Assitance Resource

www.cff.org/LivingWithCF/

*The Nationwide Childrens's Hopsital

http://www.nationwiechildrens.org/cystic-fibrosis..

References

Cystic Fibrosis Resources. (2014, December 1). Retrieved February 6, 2015, from http://www.nationwidechildrens.org/cystic-fibrosi...

Cystic Fibrosis Foundation - Teacher's Guide to CF. (2015, January 4). Retrieved February 8, 2015, from http://www.cff.org/LivingWithCF/AtSchool/Teachers...

Cystic fibrosis. (2012, June 13). Retrieved February 6, 2015, from http://www.mayoclinic.org/diseases-conditions/cys...

Researchers identify potential drug that could help treat cystic fibrosis. (2014, October 17). Retrieved February 8, 2015, from http://www.medicalnewstoday.com/releases/283943.p...

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