Cystic Fibrosissss

8/7/2019 Cystic Fibrosissss

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Everything you need to know and more


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Why is this important to you?y Some schools do not have a full time nurse

y Full time school nurses may not always be available

y It is estimated that 30,000 people have cystic fibrosisin the US


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What is Cystic Fibrosisy CDCdefines cystic fibrosis as an genetically inherited

disease that results in organ failure in the respiratory,

gastric, and other sites.y Cystic fibrosis is the most common genetic disorder in

children.

y Cystic fibrosis is most common in Caucasians but

affects all races.y Cystic fibrosis is usually diagnosed at birth and the

mean age of survival is 37.4.


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Symptoms of Cystic Fibrosisy Salty tasting skiny Bloody sputum (spit)y Thick, sticky mucousy Diarrheay Foul smelling stoolsy Stomach painy Poor weight gain and growthy Constipationy Frequent coughingy Tiredness/weaknessy Fevery Note: Make sure you tell the child to alert you of any of the above symptoms

because interventions need to be taken. An intervention such as medicationadministration would alleviate most of the symptoms experienced.


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Interventions for the child with

Cystic Fibrosisy For frequent coughing- this could be disruptive to

classmates therefore an intervention would be to sit thechild next to the door in order for them to step out if need

be. Also assign another classmate to go with them tomonitor the student and seek help if further intervention isneeded.

y Note for dehydration-allow student to have sports bottlewith appropriate beverage in the classroom. This is alsoimportant is keeping secretions thin.

y Tiredness/weakness- this is common in long hour schooldays therefore an intervention would be to allow the childfrequent rest periods during school days to account for thefatigue.


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Factors that can trigger outbreaks

of Cystic Fibrosisy Smoking

y Stress

y Dehydrationy Infection

y Fear

yAnxiety

y Depression


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Interventions for the child with

Cystic Fibrosisy To prevent infections-sure that the child washes their hands after

going to the bathroom and before eating. This is going to beessential in preventing infection.

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For the child experiencing an active infection-place the childsdesk away from any sick kids that are in the classroom to try toprevent any unnecessary infections. Again hand washing isessential in preventing the spread of infections throughout yourclassroom.

y If the child is expressing fear, anxiety, or depression-with the

above symptoms tell the child that if they experience any ofthese symptoms there is a guidance counselor at most schoolsthat they can talk to throughout their day to alleviate thesefeelings. If a guidance counselor is not present question why theyare feeling like this and try to counsel the child.


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Handwashingy Wash your hands, and suggest hand washing to

PREVENT infection

y Wet hands with warm wateryApply hand wash or soap

y Lather and wash for at LEAST 15 seconds and scrubfingernails onto palms

y Rinse both sides of hands with watery Dry hands and shut off faucet with towel


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Important Factsy Symptoms vary from person to person and vary

overtime.

y CF can cause severe breathing problems which canresult in permanent lung damage

y CF affects almost all organs of the body

y CF has NO cure, but treatment is improving


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Common Treatment Plany Chest physical therapy-to cough up secretions

yAntibiotics to treat infections.

yAnti-inflammatory medications to reduce swelling inyour airway

y Bronchodilator medications-open up airways

y Pancreatic Enzymes- to be able to digest fats and

proteinsy High Calorie Shakes- for poor nutrition and growth

y Supplements- for vitamin deficiency.


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Common medications for Cystic

Fibrosisy Tobi inhaled medication through a nebulizer , usually

taken every 12 hours and stored in a refrigerator and in

a dark roomSide effects that are NORMAL-sore throat, headache,

unpleasant taste in mouth, hoarseness

WARNING if seeing these side effects alert someone-

coughing, wheezing, shortness of breath


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Fibrosisy Pulmozyme- inhaled medication through a nebulizer

that is used to break up mucous usually taken once a

day and stored in a refridgeratory Side Effects that are NORMAL-Changes in voice, sore

throat, stuffy or runny nose, dizziness, eye irritation,rash

yWARNING if seeing these side effects alert someone-trouble breathing, hives, swelling of throat, chest pain


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Fibrosisy Pancreatic Enzymes (Pancrease, Pancreacarb, Ultrase, Creon)-

aid in digestion of fats, carbohydrates, and proteins.

y Should be taken before meals and snacks

y Note: If taking multiple enzymes take some before the meal andthe rest at various points throughout the meal.

y SHOULD NOT chew or crush

y Comes in powder, capsule, or tablet

y Should NEVERbe stored in refrigerator

y Side Effects that are NORMAL-nausea, vomiting, diarrhea,cramping, constipation, bloating

y WARNING if seeing these side effects alert someone-black tarrystools, bloody diarrhea, pain or swelling in joints


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FibrosisyAzithromycin- antibiotic to treat a certain type of

bacteria called P. Aeuroginosa that causes infections

y C

an be taken orally, tablet, or liquid suspensiony Usually taken 3X a week

y Should be stored at room temperature

y NORMAL side effects- nausea, diarrhea, abdominalpain, unpleasant taste in mouth, headache, dizziness

y WARNING if seeing these side effects alert someone-bloody diarrhea, swelling of face, chest pain, rash,

yellowing of skin


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Referencesy http://www.nhlbi.nih.gov/health/dci/Diseases/cf/cf_treatm

ents.html

ywww.cdc.gov/

y Moore, C.E. (2006). Cystic Fibrosis. In J. Selekman(Ed.), School nursing: A comprehensive text. (pp. 747-756). Philadelphia, PA: F.A. Davis Company.

y Skidmore-Roth, LindaMosby's Nursing Drug Reference.St. Louis : Mosby, 2009 (ref RM138 .S59 2009)

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Cystic Fibrosissss