CLASS PRESENTATION ON:

CYSTIC FIBROSIS (CF)

PREPARED BY:
DEFINITION:
 Cystic Fibrosis (CF) is a disorder of the endocrine
glands that affects primarily the lungs, gastrointestinal
(GI) tract and sweat glands.
 CF is a genetic disorder and was thought to be just
a childhood disease, because most affected children
did not survive past puberty. However, with new
treatments, Patients with CF are lining longer and more
productive lives. Some CF patients now marry, have
careers and live well into their thirties.
CF is the most common fatal genetic disease of
children of European-American heritage.
PATHOPHYSIOLOGY:
 CF, affects primarily the lungs, GI tract and
sweat glands. The disease varies in severity;
some patients have no GI involvement.
 Abnormal sodium and chloride transport across
cell membranes, causing thick, tenacious
secretions, is responsible for many of the
characteristic symptoms.
Thick, sticky respiratory secretions that are
difficult to remove cause airway obstruction,
resulting in frequent respiratory infections.
CONT…..
 Similar abnormalities in the pancreas cause
blocked ducts and retained digestive enzymes.

These retained enzymes digest and destroy the

exocrine pancreas.

The absence of digestive enzymes in the

intestines causes:
CONT….

a) Malabsorption of essential nutrients

b) Frequent foul smelling Fatty stools
c) Excess flatus

Patients with CF secrete sweat that is high in

sodium and chloride because these
electrolytes are not reabsorbed as they pass
through the sweat ducts.
ETIOLOGY:

 Hereditary
 Both parents must be carriers of the
defective gene for CF to be present in a
child.
 Pancreatic insufficiency
CLINICAL MANIFESTATION:

Presentation usually occurs younger than age 6

months but may occur at any age. Signs and
symptoms and severity of the disease vary and
change over time as the disease progresses.
RESPIRATORY MANIFSTATION:
1. Recurrent pulmonary infections-H. influenza, S.
aureus, P. aeruginosa.
2. Cough, dry to productive. Chronic clearing of throat
may indicate increased mucus production.
CONT….

3. Wheezing, crackles on auscultation are indicative

of respiratory exacerbation.
4. Dyspnea
5. Barrel-shaped chest (increased anteroposterior
chest diameter).
6. Cyanosis
7. Clubbing of fingers and toes
8. Nasal polyps and pansinusitis
CONT…..

GI MANIFESTATION:
1. Meconium ileus found in neonates.

2. Failure to thrive and failure to gain weight in the

presence of a good appetite
3. Abdominal distention

4. Vomiting, dehydration and electrolyte imbalance

5. Maldigestion, steatorrhea (fatty stools, loss of fat

soluble vitamins).
6. Rectal polapse
CONT…

7. Distal intentional obstructive syndrome

8. Biliary cirrhosis, obstructive jaundice
9. Pancreatitis.
CONT…

OTHER MANIFESTATIONS:
1. Thin extremities, sallow skin, wasted buttocks.
2. Hyperglycemia, glucosuria, polyuria, weight loss.
3. Salty taste when parents kiss the skin.
4. Sterility in males
5. Hypoproteinemia and anemia6. Bleeding diathesis
7. Hyponatremia and heat prostration
8.Kyphosis
COMPLICATIONS:
Patients with CF are at high risk for a variety of
complications including:
-bronchiectasis
-pneumothorax
-corpulmonale
-respiratory failure
-bowel obstruction can occur as a result of thick mucus
binding with poorly digested fecal matter.
-Diabetes from pancreatic islet cell involvement may
be present late in the disease.
DIAGNOSTIC TESTS:
Quantitative sweat chloride test; pilocarpine
iontophoresis, performed at a CF foundation
accredited center by skilled personnel.
Measures sodium and chloride content in sweat.
a) Chloride level greater than 60mEq/L is
diagnostic.
b) Chloride of 4o to 60mEq/L is borderline and
should be repeated, followed by genotype for
the most frequent CFTR mutations.
c) Sodium level greater than 60mEq/L is
diagnostic.
CONT…..
 Measurement of trypsin concentration in
duodenal secretions; absence of normal
concentration virtually diagnostic.
 Analysis of digestive enzymes (trypsin and
chymotrypsin ) in stool- reduced, used for initial
screening for CF.
 Chest x-ray may be normal initially; later shows
areas of infection, overinflation, bronchial
thickening and plugging, atelectasis, fibrosis and
emphysema.
CONT…..
 Sinus radiograph or computed tomography shows
mucus plugging.
 Analysis of stool for steatorrhea.
 Sputum or throat cultures to rule out infection.
 Pulmonary function studies (after age 4)
a) Decreased vital capacity and flow rates.
b) Increased residual volume or increased total
lung capacity.
CONT….

 Present diagnostic tests- prenatal genetic

screening for families affected with CF.
 Neonatal screening: immunoreactive
trypsinogen ; if elevated, DNA assay for
single and multiple CFTR mutations.
MEDICAL TREATMENT:

 No cure for CF, Treatment is aimed at

relieving symptoms.
 Hydration, flutter mucous clearance device
and chest (CPT) physiotherapy up to 4
times/day.
 Regular exercise.

 Hot shower.

 Nebulize mist treatment using saline or

mucolytic medications before CPT.
CONT….
 High doses of ibuprofen.
 Breathing exercise, incentive spirometry and
effective coughing techniques.
 Lung transplant is potentially promising treatment.
 Antibiotics / prophylatic antibiotic therapy.
 Pancreatic enzyme replacement
 An increase in calories requirements necessitates a
high calorie, nutrient-dense diet.
ASSESSMENT
1. Check for family history of CF ,failure to thrive
;check for clients history and physical condition.
Carefully listen for subtle information that may
suggest CF.
2. Assess respiratory status; respiratory rate, presence
of tachypnea, wheeze, cough, character of sputum,
and oxygen saturation saturation level.
- Increased work of breathing.
- Quality of breath sounds by auscultation.
- Ability to participate in activities of daily living ,
exercise tolerance, quality of sleep.
- Assess for oxygen desaturation with sleep.
3. Assess nutritional status and characteristics
of stool.
NURSING DIAGNOSIS:
 Ineffective airway clearance related to thick pulmonary
secretion.
 Risk for infection related to thick , tenacious secretions.
 Imbalanced nutrition: less than body requirements
related to decreased appetite .
 Disturbed body image related to chronic disease
process.
 Interrupted family processes related to child with
a chronic disease.
NURSING INTERVENTIONS
Promoting Airway clearance:
1. Use intermittent nebulizer therapy three to four times
per day when client is symptomatic.
- Use pretreatment postural drainage.
- Administer bronchodilators and other medications,
diluted in normal saline, in aerosal form to penetrate
respiratory tract.
2. Perform CPT 3-4 times after nebulization if infection
is present.
- Perform prior to meals or 1hour after eating to prevent
vomiting or discomfort
- Place the client in a position that gives the greatest
access to affected lobes of lung and facilitate gravity
drainage of mucus from the lung area.
- Client with gastro- esophageal reflux should not use
head down position.
3. Help client to relax to cough more easily.
4. Suction the cough if necessary and monitor oxygen
saturation levels during procedures.
5. Monitor for haemoptysis and provide treatment for it
.
6. Maintain cautious oxygen therapy due to chronic
CO2 retention.
Preventing infection:
1. Provide frequent mouth care to reduce chance of
infection because mucus is present.
2. Administer antibiotics as prescribed if client is
symptomatic.
3. Monitor closely for deteriorating respiratory status.
4. Provide good skin care and position changes to
prevent skin breakdown.
Promoting adequate nutrition:
1. Encourage diet composed of high calories and
protein foods and moderate to high fat because
absorption of food is incomplete.
2. Administer fat soluble vitamin in water miscible
solution three to four times as prescribed.
3. Administer pancreatic enzymes with each
meals and snacks .dose is based on client’s
weight, weight gain, growth , food intake and
character of bowel movements.
4. Increase salt intake in hot weather, fever, or
excessive exercise to prevent sodium depletion.
5. Check weights at least weekly to assess
nutritional interventions.
Enhancing self esteem and Body image
1. Explain each procedure, medication and treatment
to client as appropriate to age.
2. Allow client to show frustration , fears and feelings by
talking , complaining or crying.
3. Provide diversional activities according to client’s
interest.
4. Help the client to indentify the strengths and
limitations and to feel good about self.
5. Encourage regular exercise and activity to foster a
sense of accomplishment and independence and to
improve pulmonary function.
Family education and Health maintenance:
1. Teach parents or family members to have through
understanding of the dietary regimen and special
need for calorie , fat and vitamins.
2. Help the family members to schedule care for client
within framework of family life.
3. Provide emotional support to client and family
members.
4. Stress the importance of regular medical care.
5. Stress the importance of follow up and regular
health care .
Discuss with family members about limitation
and expectation for client.
7. Investigate homecare facilities for families
and especially respite services for
caregivers.
EXPECTED OUTCOMES

 Tolerates CPT four times per day for 30

minutes with stable oxygen saturation.
 No signs of respiratory infection.

 Eats well with no vomiting ; weight stable.

 Family caretakers take part in care of their

client.