Cystic fibrosis is a genetic disorder that affects the lungs and digestive system. It causes thick, sticky mucus to build up in the lungs and pancreas. Symptoms vary by individual but may include salty-tasting skin, poor growth, frequent lung infections, and infertility in males. Treatment focuses on airway clearance techniques, medications to thin mucus and treat infections, enzyme supplements to aid digestion, and exercise. The goals are to prevent and control lung infections, clear mucus from the lungs, treat intestinal issues, and ensure proper nutrition.

1. incidence
• The chances of your child having CF are about
1 in 3,000 if your child is Caucasian, 1 in 9,000
if he's Hispanic, 1 in 11,000 if he's Native
American, 1 in 15,000 if he's of African
heritage, and 1 in 30,000 if he's Asian
American.

2. • Cystic fibrosis (CF) is a genetic disorder that
affects mostly the lungs, but also
the pancreas, liver, kidneys, and intestine
• Cystic fibrosis can't be cured, but there are
new treatments that can not only prolong a
child's life but may also help make that life
more normal.

3. • salty-tasting skin
• poor growth, and poor weight gain
• accumulation of thick, sticky mucus
• requent chest infections, and coughing or shortness of
breath
• Males can be infertile due to congenital absence of the
vas deferens
• bowel obstruction
• growth failure are multifactorial and include chronic
lung infection, poor absorption of nutrients through
the gastrointestinal tract

4. Cystic fibrosis
Is a fatal autosomal recessive disease that
manifests itself in multiple body system .it
affect lungs ,pancreas, urogenital system
,skeleton and skin .
It is major cause of severe chronic lungs
disease in children .
Cystic fibrosis (CF) is a genetic disorder that
affects mostly the lungs, but also
the pancreas, liver, kidneys, and intestine

5. Causes of cystic fibrosis
• A defect in the CFTR gene causes cystic fibrosis
(CF).
• Cystic fibrosis is a genetic disease. This means
that people inherit it from their parents through
genes (or DNA).
• To have cystic fibrosis, a person must inherit two
copies of the defective CF gene, getting one copy
from each parent. Carriers are not affected.
•

7. Clinical manifestation

8. c/f
• Poor growth is one of the first signs of CF
• Pulmonary symptom like :- cough wheezing ,nasal
obstruction ,rhinorrhea, shortness of breath,
cynosis,hemoptysis,wheezing and exercise
intolerance. Due to blockage and congestion of
the respiratory passages eith thick secreations.
• Recurrent bronchitis,pneumonia
,bronchiectasis,nasal polyposis,chronic lungs and
respiratory failure.

9. • GI problems due to intestinal malabsorption
Diarrhoea, malnutrition ,failure to thrive
,arthritis, delay puberty ,diabetic , aspermia ,
azoospermia
big appetite with no weight gain, and large,
greasy stools.

10. complications
• Atelectasis
• Pneumothorax
• corpulmonale

11. Respiratory system complications
• Damaged airways (bronchiectasis).
• Chronic infections
• Growths in the nose (nasal polyps).
• Coughing up blood (hemoptysis).
• Pneumothorax.
• Respiratory failure.
• Acute exacerbations

12. Digestive system complications
• Nutritional deficiencies.
• Diabetes.
• Blocked bile duct
• Intestinal obstruction
• Distal intestinal obstruction syndrome
(DIOS).

13. Reproductive system complications
• Other complications
• Thinning of the bones (osteoporosis).
• Electrolyte imbalances and dehydration.

14. Diagnosis
cystic fibrosis
• complete medical history and physical exam
• Sweat (chloride) test.
• Genetic tests
• Blood tests eg ncluding pancreatic function
tests, can look at infection and to see which
organs may be affected.
• Chest X-rays.
• Pulmonary function tests

15. Cont..
• Sputum cultures ge to detect t infection
• Stool evaluations. For These are done to
measure stool fat absorption.

16. Treatment of cystic fibrosis
• Take pancreatic enzyme supplement capsules
with every meal and most snacks to help
digest fat and protein. Even babies who are
breastfeeding may need to take enzymes.
• Take multivitamins, especially the fat soluble
vitamins A, D, E and K
• Make sure they get plenty of salt by adding
salt to food or eating salty snacks.
• Eat a high calorie, high protein diet.

17. • "Airway Clearance
• nebulizer
• There are different types of aerosolized
medicines with different actions including
thinning the mucus, opening the airways and
fighting infection..

18. Medications
• Antibiotics to treat and prevent lung infections
• Anti-inflammatory medications to lessen
swelling in the airways in your lungs
• Mucus-thinning drugs to help you cough up
the mucus, which can improve lung function

19. • Inhaled medications called bronchodilators
that can help keep your airways open by
relaxing the muscles around your bronchial
tubes
• Oral pancreatic enzymes to help your digestive
tract absorb nutrients

20. • Chest physical therapy
• Pulmonary rehabilitation
Physical exercise that may improve your
condition
Breathing techniques that may help loosen
mucus and improve breathing
Nutritional counseling
Counseling and support
Education about your condition

21. Surgical and other procedures
• Nasal polyp removal.
• Oxygen therapy.
• Endoscopy and lavage
• Feeding tube.
• Bowel surgery.
• Lung transplant.

22. • immunizations up to date
• Exercise
• Eliminate smoke
• Encourage hand-washing

23. The goals of treatment include:
• Preventing and controlling infections that
occur in the lungs
• Removing and loosening mucus from the
lungs
• Treating and preventing intestinal blockage
• Providing adequate nutrition