1. Cystic FibrosisPresented by Ofori-Addo, Eugene

2. The Disease – Cystic
Fibrosis
 Cystic fibrosis is known to be an inherited autosomal
(pattern of inherited genetic disease) chronic disorder
mostly seen in children that affects the lungs and the
digestive system.
 It is also known to be most common cause
of chronic lung disease mostly seen in children
and younger adults.
 Also known to be the most fatal hereditary disorder that affects
numerous Caucasians in the United States
 (Medline Plus, 2013, Cystic Fibrosis Foundation)

3. Statistics
 As the most common Chronic
lung disease in children and
some adults, this disorder
affects about-
 30, 000 children and young adults
 Where about 1200 children are diagnosed
every year
 About 70% of these patients are diagnosed
with the disease at age 2
 And about bout 40% of the population being
18 years of age and older.
 Life span of patients with Cystic Fibrosis is
about 37 years (Cystic Fibrosis Foundation,
2014)
 * Respiratory failure is mostly
the major consequence of
Cystic Fibrosis and usually
what causes death amongst
CF Patients
Population
Children
age 2- 70%
Young
Adults -
40%

4. Causes of Cystic Fibrosis
 This is an inherited diseases
complication so its is
genetically transmitted
 From parents
 through Genes
 2 copies of genes are needed to
signify inheritance of the disease

5. Pathophysiology
 Cystic Fibrosis becomes present
when there is a
 Defective gene i.e. related to
protein that is involved in chloride
ion transport
 Patients with the diseases usually
develop unusual thick sticky mucus
plugging that clogs the lungs and
causes obstruction which leads to life-
threatening lung infections and also
obstruction in the pancreas.
 Cystic Fibrosis Explained
 https://www.youtube.com/watch?v=LItSsV
JPQEY

6. Symptoms
 Cystic fibrosis have several
symptoms which include -
 A persistent cough that
produces thick spit (sputum)
and mucus
 Wheezing
 Shortness of breath
 Meconium ileus which
appears at birth
 Salty-tasting skin which is
usually greasy feeling, bulky
and has a bad smell
 Frequent lung infections

7. Diagnostic Testing
 Genetic Study (analysis)
 When a new born child has
signs and symptoms
confirmed, diagnosis of CF
can usually be done by using
Blood Test
 Since it is an inherited
diseases, family members
should be checked for
possible diagnosis
 The Sweat test
 This test is a very accurate
way of diagnosing CF.
 Test measures Sodium
and or chloride amounts
present in patient’s sweat.
(test not reliable in
newborns)

8. Other common diagnosis
 When a patient presents Cystic Fibrosis, they
are usually seen to have these further
complications-
 Inability for effective
airway clearance
 Inability to digest food
and absorb nutrients which
causes imbalanced nutrition

9. Cystic Fibrosis – X-RAY
 Young man with a history of
cystic fibrosis has
hyperinflation and
predominantly upper lobe
bronchiectasis.
 Bronchiectasis is a
condition in which damage to
the airways causes them to
widen and become flabby
and scarred.
 A chest x ray can show
areas of abnormal lung
and thickened, irregular
airway walls.

10. Therapy and Management
 Planning and intervention
usually include-
 Provision of respiratory
therapy treatments
 Chest physiotherapy to
clear secretions
 Administering medications
 Meeting nutritional needs
 Providing fat soluble
vitamins like A, D, E, K to
prevent deficiency
 Providing psychosocial
support

11. Treatments
 These help and aim at
relieving symptoms and
complications of CF
 Aerosolized antibiotics
 Mucus-thinning drugs that
thin out secretions and
makes it easier to cough up
 Use of bronchodilators
 As the purpose of relaxing
smooth muscles in the
airways
 Bronchial airway drainage
 Postural drainage

13. Drugs Contd..

14. Treatment contd..
 Enzymes taken orally for better
nutrition
 Should contain high calorie diet
since CF patients are usually
malnutritioned due to pancreatic
enzymes needed to digest are not
available
 Lung transplant at its worst
Significant when there is severe
breathing problems that has lead to
life-threatening infections of the lungs
 Pain relievers such as
 Ibuprofen- may help slow down
lung deterioration

15. Questions?
What do you call a nun with a lung infection and general mucus build-up?
Sister Fibrosis.
www.cysticfibrosisfoundation.com