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Some schools do not have a full time nurse
Full time school nurses may not always be available
It is estimated that 30,000 people have cystic fibrosis
in the US
What is Cystic Fibrosis
CDC defines cystic fibrosis as an genetically inherited
disease that results in organ failure in the respiratory,
gastric, and other sites.
Cystic fibrosis is the most common genetic disorder in
children.
Cystic fibrosis is most common in Caucasians but
affects all races.
Cystic fibrosis is usually diagnosed at birth and the
mean age of survival is 37.4.
Symptoms of Cystic Fibrosis
“Salty” tasting skin
Bloody sputum (spit)
Thick, sticky mucous
Diarrhea
Foul smelling stools
Stomach pain
Poor weight gain and growth
Constipation
Frequent coughing
Tiredness/weakness
Fever
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 medication
administration would alleviate most of the symptoms experienced.
Interventions for the child with
Cystic Fibrosis
For frequent coughing- this could be disruptive to
classmates therefore an intervention would be to sit the
child next to the door in order for them to step out if need
be. Also assign another classmate to go with them to
monitor the student and seek help if further intervention is
needed.
Note for dehydration-allow student to have sports bottle
with appropriate beverage in the classroom. This is also
important is keeping secretions thin.
Tiredness/weakness- this is common in long hour school
days therefore an intervention would be to allow the child
frequent rest periods during school days to account for the
fatigue.
Factors that can trigger outbreaks
of Cystic Fibrosis
Smoking
Stress
Dehydration
Infection
Fear
Anxiety
Depression
Interventions for the child with
Cystic Fibrosis
To prevent infections-sure that the child washes their hands after
going to the bathroom and before eating. This is going to be
essential in preventing infection.
For the child experiencing an active infection-place the child’s
desk away from any sick kids that are in the classroom to try to
prevent any unnecessary infections. Again hand washing is
essential in preventing the spread of infections throughout your
classroom.
If the child is expressing fear, anxiety, or depression-with the
above symptoms tell the child that if they experience any of
these symptoms there is a guidance counselor at most schools
that they can talk to throughout their day to alleviate these
feelings. If a guidance counselor is not present question why they
are feeling like this and try to counsel the child.
Handwashing
Wash your hands, and suggest hand washing to
PREVENT infection
Wet hands with warm water
Apply hand wash or soap
Lather and wash for at LEAST 15 seconds and scrub
fingernails onto palms
Rinse both sides of hands with water
Dry hands and shut off faucet with towel
Important Facts
Symptoms vary from person to person and vary
overtime.
CF can cause severe breathing problems which can
result in permanent lung damage
CF affects almost all organs of the body
CF has NO cure, but treatment is improving
Common Treatment Plan
Chest physical therapy-to cough up secretions
Antibiotics to treat infections.
Anti-inflammatory medications to reduce swelling in
your airway
Bronchodilator medications-open up airways
Pancreatic Enzymes- to be able to digest fats and
proteins
High Calorie Shakes- for poor nutrition and growth
Supplements- for vitamin deficiency.
Common medications for Cystic
Fibrosis
Tobi –inhaled medication through a nebulizer , usually
taken every 12 hours and stored in a refrigerator and in
a dark room
Side 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
Common medications for Cystic
Fibrosis
Pulmozyme- inhaled medication through a nebulizer
that is used to break up mucous usually taken once a
day and stored in a refridgerator
Side Effects that are NORMAL-Changes in voice, sore
throat, stuffy or runny nose, dizziness, eye irritation,
rash
WARNING if seeing these side effects alert someone-
trouble breathing, hives, swelling of throat, chest pain
Common medications for Cystic
Fibrosis
Pancreatic Enzymes (Pancrease, Pancreacarb, Ultrase, Creon)-
aid in digestion of fats, carbohydrates, and proteins.
Should be taken before meals and snacks
Note: If taking multiple enzymes take some before the meal and
the rest at various points throughout the meal.
SHOULD NOT chew or crush
Comes in powder, capsule, or tablet
Should NEVER be stored in refrigerator
Side Effects that are NORMAL-nausea, vomiting, diarrhea,
cramping, constipation, bloating
WARNING if seeing these side effects alert someone-black tarry
stools, bloody diarrhea, pain or swelling in joints
Common medications for Cystic
Fibrosis
Azithromycin- antibiotic to treat a certain type of
bacteria called P. Aeuroginosa that causes infections
Can be taken orally, tablet, or liquid suspension
Usually taken 3X a week
Should be stored at room temperature
NORMAL side effects- nausea, diarrhea, abdominal
pain, unpleasant taste in mouth, headache, dizziness
WARNING if seeing these side effects alert someone-
bloody diarrhea, swelling of face, chest pain, rash,
yellowing of skin
References
http://www.nhlbi.nih.gov/health/dci/Diseases/cf/cf_treatm
ents.html
www.cdc.gov/
Moore, C.E. (2006). Cystic Fibrosis. In J. Selekman
(Ed.), School nursing: A comprehensive text. (pp. 747-
756). Philadelphia, PA: F.A. Davis Company.
Skidmore-Roth, Linda Mosby's Nursing Drug Reference.
St. Louis : Mosby, 2009 (ref RM138 .S59 2009)