Cystic Fibrosis
(Mucoviscidosis)
Introduction
 CF
is an inherited disease of your mucus
and sweat glands
 It affects mostly the lungs, pancreas, liver,
intestines, sinuses and sex organs
 An abnormal gene causes mucus to
become extra thick and sticky
 This gene makes a protein that controls
the movement of salt and water not work
properly (retaining salt=thick secretions)
 This leads to mucus plugs
Introduction Continued

Mucus plugs lead to
collapsed lungs
(atlectasis)
 Increased mucus in
the lungs also allows
for more bacterial
growth which leads to
frequent pneumonia
 Constant infections
lead to inflammation
in the lung
Introduction Continued
Cystic fibrosis is the most common cause of
chronic lung disease in children and young
adults, and the most common fatal hereditary
disorder affecting Caucasians in the US.
CF is a multi-system disorder of exocrine glands
causing the formation of a thick mucus
substance that affects the lungs, intestines,
pancreas, and liver. The standard test for
diagnosis is a sweat test which evaluates the
level of chloride excreted by the body.
Diagnosis
The sweat chloride test is performed to determine the amount of
chloride that is excreted in sweat from the body during a certain
period of time. The test may be performed on infants to determine if
cystic fibrosis is present. Children with cystic fibrosis have
increased sodium and chloride concentrations in their sweat.
Normal Sweat
18 meq/L
Positive Test
60 meq/l
Diagnosis Continued






Detailed medical history is obtained (CF is
Hereditary)
Chest X-RAY to show scarring from frequent
inflammation
Sinus X-RAY
Pulmonary Function Test (CF is a COPD); used
only with individuals old enough to comply >
8years old usually
Sputum Cultures to determine certain bacteria
growth
Blood tests to find abnormal CF gene

Often the first sign of CF begins after birth, the
mother kisses the baby and they taste salty.
 Poor feeding occurs from blocked bile ducts (bile
released from pancreas helps digest food)
Diagnosis Continued
Symptoms
Symptoms
 Increased
WOB from plugged airways and
air trapping
 Tenacious Secretions
 Frequent productive cough
 Frequent bouts of bronchitis and
pneumonia
 Dehydration and malnutrition despite huge
appetite; failure to thrive
 Infertility (mostly in men)
 Ongoing Diarrhea and stomach pain
CF leads to…
 Sinusitis:
the sinuses have mucus build
up leading to head ache ear and
equilibrium problems
 Bronchiectasis: damaged lungs become
overly stretched and retain secretions and
gas
 Pancreatitis: Leads to inability to digest
food, leading to bowl obstruction and
sepsis
 Liver Disease, Diabetes, Gallstones and
low bone density from lack of Vitamin D
CF leads to Respiratory failure
 The
mucus plugs the airways causing
collapse and increased WOB
 Increased PaCO2, decreased PaO2 and
eventual death if not treated.
 Infections lead to inflamed and damaged
lung lining
 Blocked pancreas leads to vitamin
deficiencies
 There is no cure for CF only treatments;
average life span is 30 years
Treatments for CF
 Chest
physiotherapy (CPT) is the
traditional means of airway clearance in
CF. It uses postural drainage in various
positions, percussion, vibration, deep
breathing, and coughing to loosen and
move secretions out of the lungs. The
treatment time including an aerosol before
is about 45 minutes. Done so by using
manual percussion with hand, pneumatic
precursor with device or by Vest.
Treatment for CF
Chest Physical Therapy:
Using the “Vest” or manual
precursor. Helps loosen
secretions with percusion
Treatment Continued

PEP is a technique that uses a hand held device
which can be used with a nebulizer attached. It
has a restricted orifice. When exhaled into, this
creates pressure in the lungs. This pressure
allows air to enter behind areas of mucus
obstruction and keeps the airways open during
exhalation. As you exhale, mucus moves
towards the larger airways, so it can be more
easily coughed up with the huff technique. PEP
can be taught to children as young as 5 years,
and can be passively given to infants via a
mask. The treatment time is about 20 minutes.
PEP Device
Treatment Continued

Vibratory Positive Expiratory Pressure (Flutter®,
Acapella®)
Vibratory positive expiratory pressure is a hand held
device. Exhaling into this device results in oscillations
of pressure and airflow which vibrate the airway walls
(loosening mucus), helps hold the airway open (which
allows air to get behind secretions and keeps the
airways open during exhalation). It speeds up airflow
helping mucus move up to the larger airways where it
can be more easily coughed up. Vibratory PEP can
be taught to children as young as 2 years old by
mask, and to ages 5 and up via mouthpiece.
Treatment time is about 20 minutes.
Treatment Continued…
Intrapulmonary Percussive Ventilation.
The IPV is a pneumatic (air driven) device that
delivers both continuous airway pressure and mini
bursts of air. At the same time the IPV delivers a
dense aerosol.
The combination allows air to
enter behind mucus blockage,
and vibration to dislodge mucus
from the airway walls so it can
be more easily coughed up..
Treatment Continued

Active Cycle of Breathing
Active cycle of breathing is a series of breathing
techniques, consisting of thoracic expansion
exercises (deep breathing), breathing control
(using the diaphragm), and the forced expiration
technique (huff). These breathing cycles are
performed in various positions of drainage
similar to CPT positions but without the
percussion. This can be taught at about the age
of 8 years. Treatment time, including an aerosol
before, is about 45 minutes.
Treatments

Autogenic Drainage
Autogenic Drainage is a breathing technique
which involves 3 phases of breathing levels:
 The first phase is the unsticking phase which is
inhalation and exhalation of small amounts of
air.
 Phase two is the collection phase where medium
sized breaths are inhaled and exhaled.
 Phase three is the evacuation phase where
large amounts of air are inhaled and exhaled.
Treatments
 Hand
Held Nebulizers are used in
conjunction with PEP, IPV, CPT and
breathing techniques
 The nebulizer will nebulize medications
that bronchodilate and help
 Break up mucus, as well as
 antibiotics
Medications Used

Antibiotics: Tobramycin and azithromycin to fight
bacterial infection. Given by aerosol in nebulizer
or by IV
 Anti-Inflammatory Drugs: Steroids given inhaled
or by IV; also Ibuprofen is given
 Bronchodilators: Albuterol/Xopenex given to
relax smooth muscle
 Mucolytics: Given with bronchodilators to break
up thick secreations. Main one is Dornase Alfa
(Pulmozyne) made specifically for CF patients
More Treatments
 Oxygen
Therapy at low concentrations.
 Lung Transplantation; depends on severity
of illness and health of participate
 Nutritional therapy; oral pancreatic
enzymes to digest fats and proteins and
absorb vitamins.
 Vitamin supplements of A, D, E and K
 Feeding tube at night (G-Tube)
 Enemas and stomach meds to control acid
Conclusion
 CF
is a deadly hereditary disease that is
treatable but not curable
 CF causes abnormally thick mucus which
blocks bile ducts and plugs up the lung
and sinus
 May lead to respiratory failure, malnutrition
and frequent pnuemonias
 Treatment includes methods to remove
and thin mucus and medications to treat
digestive problems, and infections