THIS PRESENTATION INCLUDES DEFINITION, OVERVIEW, PATHOLOGY, CLINICAL FEATURES, ASSESSMENT AND PT MANAGEMENT OF CYSTIC FIBROSIS. THIS PPT WILL BE VERY USEFUL FOR FINAL YEAR BPT STUDENTS. IT COVERS BASIC KNOWLEDGE REGARDING THE DISEASE AND ALLOWS BETTER UNDERSTANDING. IT IS MADE ONLY FOR LEARNING AND EXAM PURPOSE.

1. CYSTIC FIBROSIS
DR.SAMIR JADAV (PT)

2. DEFINITION
OVERVIEW
PATHOLOGY
CLINICAL FEATURES
ASSESSMENT
PT MANAGEMENT

3. DEFINITION
Cystic Fibrosis is a chronic progressive and life limiting
autosomal recessive genetic disease characterized by chronic
respiratory disease, pancreatic insufficiency and elevation of fat
electrolytes & infertility.
It is most common hereditary disorder transmitted by recessive
gene.

4. OVERVIEW
CF is an inherited disorder that causes sever damage to the lungs,
digestive system and pancreas, liver & other organs.
CF affects the cells that produces mucus, sweat and digestive
juices.
These fluids are normally thin and slippery, but in CF, defective
gene causes these secretions to become sticky and thick.
Instead of acting as lubricants, this secretions plug up tubes, ducts
and passageways, in lungs and pancreas.
Life span:- 40s-50s, death is mostly due to lung complications.

5. PATHOLOGY
Mutation of gene results in defective chloricle transport
Decreased transport of Na+ and H2O in epithelial cells
Dehydration and Increased Viscosity of Secretions.

6. CLINICAL FEATURES
RESPIRATORY C/F :-
--A persistent cough that produces thick mucus.
(sputum).
--Wheezing
--Ex. Intolerance
--Repeated lung infections
--Inflammed nasal passages or a stuffy nose
--Recurrent sinusitis

7. DIGESTIVE C/F :-
--Foul smelling, greasy stools
--Poor weight gain & growth
--Intestinal blockage. (meconium ileus)
--Chronic or sever constipation, rectal prolapse.

8. ASSESSMENT (MOCK)
DEMOGRAPHIC DETAILS
CHIEF COMPLAINTS
-Patient complaints of cough, difficulty in breathing.
-patient also complaints of blood in sputum
sometimes.
HISTORY
H/O PRESENT ILLNESS:
- H/O of recurrent pulmonary infections.

9. PAST MEDICAL HISTORY
-No other medical conditions in past.
DRUG HISTORY
FAMILY HISTORY
-(hereditary disorder)
SOCIAL HISTORY
-patient may not fullfill the social requirement
of his/her family.
PERSONAL HISTORY
-(smoke, alcohol)

10. SUBJECTIVE ASSESSMENT
COUGH : Productive
DYSPNOEA : Present
Grade-1(NYHA scale)
SPUTUM : Purulent
HAEMOPTYSIS : Present
WHEEZE : Present
OTHER SYMPTOMS : Fatigue, pyrexia, etc.

11. OBJECTIVE ASSESSMENT
GENERAL OBSERVATION
- Patient seems to be breathless.
LEVEL OF CONSCIOUSNESS
- GCS scale
BODY BUILT
OBSERVATION OF CHEST
CHEST SHAPE :- Barrel chest
CHEST MOVEMENT :- Bilateral diminished
BREATHING PATTERN :- According to gender
TYPE OF BREATHING :- Obstructive breathing
POSTURE
- Exaggerated kyphotic and lordotic curve.

12. ON EXAMINATION
(VITALS)
TEMPERATURE :- Normal
RESPI. RATE :- Tachypnea
HEART RATE :-
BLOOD PRESSURE :-
ON PALPATION
TRACHEA : Normal/Central
TENDERNESS : Absent
TVF : Reduced
CHEST EXPANSION : Hyper-inflated

13. ON PERCUSSION
-Hyper resonant type on percussion.
ON AUSCULTATION
-BREATH SOUNDS : Vesicular
-FOREIGN SOUNDS : Wheeze, Crackles
INVESTIGATIONS

14. CHEST X-RAY : Shows hyperinflation and
bronchial wall thickening.
ABG ANALYSIS : Low PaO2 due to Va/Q
mismatch.
Increased PaCO2 as disease
become severe.
LUNG FUNCTION TESTS : Decreased FEV1/FVC ratio
Increased TLC, FRC, RV due to
overinflation of lungs.
DIAGNOSIS : CYSTIC FIBROSIS
PT MGMT - SEE ‘PULMONARY DISEASE PT MGMT’
PPT